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© 2005 WebMD, Inc. All rights reserved.                                                          ACS Surgery: Principles and Practice
4 THORAX                                                                                                    3 CHEST WALL MASS — 1



3 CHEST WALL MASS
John C. Kucharczuk, M.D., F.A.C.S.




Evaluation of Chest Wall Mass
Chest wall masses arise from a variety of different causes. The            a stand-alone test, it cannot differentiate between benign and
chest wall contains a number of distinct tissues, including skin, fat,     malignant chest wall lesions.
muscle, bone, cartilage, lymphatic vessels, and fascia. Each of               In general, given that neither physical examination nor diag-
these component tissues can give rise to either a benign or a malig-       nostic imaging can reliably distinguish benign from malignant
nant primary chest wall mass. In addition, the chest wall is also in       chest wall masses, it is important to move quickly to tissue diag-
intimate proximity to a number of organs (e.g., the breast, the            nosis.With very unusual tumors, it is often necessary to consult a
lung, the mediastinum, and the pleura) that may give rise to a             highly specialized pathologist to make the diagnosis and an oncol-
chest wall mass through extension of a malignancy or infection.            ogist to assist in treatment planning.
Finally, because of its large surface area, the chest wall can be the
                                                                           BIOPSY
site of a secondary mass caused by metastasis from a distant
malignancy (e.g., carcinoma or sarcoma) [see Table 1].                        Whether a tissue diagnosis is needed
   Chest wall masses, whether primary or secondary, are relative-          before initiation of definitive therapy de-
ly uncommon in clinical practice. Accordingly, many surgeons               pends on the size and characteristics of the
lack a solid working knowledge of the causes, evaluation, treat-           lesion. If the lesion is small (< 3 cm),
ment, and natural history of these lesions. Often, this unfamiliari-       regardless of whether it is believed to be
ty leads to inappropriate selection of diagnostic studies, unneces-        benign or malignant, excisional biopsy is
sary delays in treatment, and considerable frustration for patient         performed as both diagnosis and treat-
and surgeon alike. In what follows, I outline a focused clinical ap-       ment. If the lesion is larger (≥ 3 cm) and its resection will lead to
proach aimed at streamlining the evaluation and treatment of pa-           significant morbidity and necessitate extensive reconstruction, a
tients with chest wall masses. Appropriate diagnostic studies and          preoperative tissue diagnosis is obtained.
operative planning are discussed, and specific causes of chest wall            Whether fine-needle aspiration (FNA) is useful for tissue
masses are reviewed.                                                       diagnosis in this setting remains a subject of debate. FNA is a
                                                                           simple procedure that can be performed in the office during the
                                                                           initial patient evaluation, and several studies suggest that it is an
Clinical Evaluation                                                        effective technique for assessing chest wall masses.2 Never-
                                                                           theless, in routine clinical practice, cytologic analysis of a fine-
HISTORY AND PHYSICAL EXAMINATION
                                                                           needle aspirate from a primary chest wall mass frequently yields
   Initial evaluation of a patient with a chest                            nondiagnostic results, and additional tissue is often requested.
wall mass begins with a careful history that                               In such cases, a core-needle biopsy or an incisional biopsy is
notes the symptoms associated with the                                     performed. Both techniques provide tissue for histologic evalu-
mass and records the history of its growth.                                ation, and both must be performed in such a way that the biop-
Previously obtained radiographs, if available, are reviewed to             sy tract will be completely excised at the time of definitive sur-
determine how rapidly the mass has been growing. A complete                gical treatment. As a rule, in patients with a known primary
physical examination is performed to rule out other sites of disease       malignancy and a secondary chest wall mass, I perform FNA.
and to identify any comorbid medical conditions that may affect            In patients with a primary chest wall mass larger than 3 cm and
the patient’s candidacy for resection. If the mass is palpable, its size   no underlying diagnosis, I proceed directly to incisional biopsy
and its salient characteristics (i.e., hard versus soft and fixed ver-      for diagnosis.
sus mobile) are noted. By itself, physical examination will not
establish whether the lesion is benign or malignant.                                  Table 1 Classification of Primary
                                                                                      and Secondary Chest Wall Masses
Investigative Studies
                                                                                                   Benign
DIAGNOSTIC IMAGING                                                                                   Infectious masses
                                                                                                     Soft tissue neoplasms
   At presentation, most patients with                                     Primary masses of         Bone and cartilage neoplasms
chest wall masses have already undergone                                     chest wall
                                                                                                   Malignant
chest radiography and computed tomog-                                                                Soft tissue neoplasms
raphy. In the case of a primary chest wall                                                           Bone and cartilage neoplasms
mass, magnetic resonance imaging is useful for further character-
                                                                           Secondary masses of     Tumor invasion from contiguous organs
ization of the lesion. MRI allows precise delineation of tissue             chest wall             Metastasis from distant organs
planes and major adjacent neurovascular structures1; however, as
© 2005 WebMD, Inc. All rights reserved.                                                         ACS Surgery: Principles and Practice
4 THORAX                                                                                                    3 CHEST WALL MASS — 2




                                     Evaluation of Chest Wall Mass


                                          Patient presents with chest wall mass

                                          Obtain clinical history:
                                          • Growth of mass
                                          • Associated symptoms
                                          • Previous chest radiographs (if available)
                                          Perform physical examination:
                                          • Other disease sites or comorbid conditions
                                          • Size and salient characteristics of mass (if palpable)
                                          Determine whether mass is primary or secondary.




                               Chest wall mass is primary                  Chest wall mass is secondary

                               Consider MRI for further                   Control primary underlying disease process.
                               characterization of lesion                 Perform needle biopsy to confirm diagnosis.
                               if desired.
                                                                          Consider definitive treatment after primary
                               Assess size of mass.                       process is controlled.




      Mass is < 3 cm                                              Mass is ≥ 3 cm

      Perform excisional biopsy for diagnosis                   Perform incisional biopsy for diagnosis.
      and treatment.




                                 Mass represents benign lesion                            Mass represents malignant lesion

                                 Consider resection for confirmation of                  Perform radical resection with reconstruction.
                                 diagnosis or for management of symptoms.
© 2005 WebMD, Inc. All rights reserved.                                                     ACS Surgery: Principles and Practice
4 THORAX                                                                                                3 CHEST WALL MASS — 3


Management

PRIMARY BENIGN MASSES OF
CHEST WALL

   Infectious Masses
   Sternal infection Primary sternal
osteomyelitis may be seen in intravenous
drug abusers but is otherwise rare. Much
more common is osteomyelitis occurring after median sternoto-
my. Approximately 1% to 3% of median sternotomies for cardiac
procedures are complicated by sternal wound infection.3 Risk fac-
tors include diabetes, the use of bilateral internal mammary arter-
ies, and reoperation.4 Patients with poststernotomy osteomyelitis
present with pain, drainage, and, often, a palpable mass overlying
the incision. In most cases, the infection is not confined to the
sternum but extends deeply into the mediastinum as well. The
diagnostic study of choice is CT scanning of the chest, which will
                                                                         Figure 2 CT scan shows typical appearance of SCJ infection,
determine the extent of mediastinal soilage.
                                                                         including fluid collection around the joint and tissue stranding.
   After CT, the patient is taken to the operating room for wide
drainage and sternal debridement. A number of different tech-
niques have been employed to treat these infections; of these, aggres-
sive surgical debridement of the infected area with flap closure yields   is made on the basis of the history, the physical examination, and
the best overall clinical outcomes [see 4:9 Chest Wall Procedures].5     CT scanning of the chest (with particular attention paid to the
   The patient who presents with a pulsating sternal mass after          SCJ). Typical CT findings include tissue stranding and a collec-
sternotomy represents a special case. Such patients have a pseu-         tion around the joint [see Figure 2].
doaneursym of the underlying aorta and are at risk for exsan-               Treatment consists of wide resection of the SCJ and the proxi-
guination.They should undergo an emergency CT angiogram or               mal third of the clavicle, as well as debridement of the manubri-
aortogram to confirm the diagnosis and then be taken directly to          um [see 4:9 Chest Wall Procedures].6 Often, the proximal portion of
the OR, where they are placed on cardiopulmonary bypass                  the first rib is involved and also must be resected. Immediate
through femoral cannulation and cooled to a hypothermic state            reconstruction is performed by rotating a pectoralis muscle flap
before the sternum is opened.                                            into the resection cavity. After operation, I.V. antibiotics are ad-
                                                                         ministered for 6 weeks. Most patients require intensive postoper-
   Sternoclavicular joint infection Sternoclavicular joint               ative physical therapy to restore strength, function, and mobility
(SCJ) infections present as painful palpable masses overlying the        in the upper extremity.
joint [see Figure 1]. These infections are often associated with I.V.
drug abuse, infected indwelling subclavian catheters, or trauma.            Osteomyelitis of rib Osteomyelitis of a rib presents as a
Most patients also have an underlying risk factor (e.g., diabetes or     painful, swollen mass overlying an infected segment of rib. Often,
hepatic or renal insufficiency) or a history of sepsis.The diagnosis      a draining sinus tract is present.The diagnosis is made on clinical
                                                                         grounds. A CT scan of the chest is obtained to rule out an under-
                                                                         lying intrathoracic condition (e.g., empyema).
                                                                            Treatment consists of resection of the infected bone and soft
                                                                         tissue coverage of the defect [see 4:9 Chest Wall Procedures]. Care
                                                                         must be taken to avoid contamination of the underlying pleural
                                                                         cavity during rib resection.
                                                                            In children, the diagnosis is made on clinical grounds and may
                                                                         be facilitated by the use of ultrasonography, which demonstrates
                                                                         obliteration of the intermuscular planes adjacent to the infected
                                                                         rib and pericostal edema.7 Again, a CT scan is usually obtained to
                                                                         rule out other underlying pleura-based abnormalities. As with
                                                                         adults, the range of pathologic organisms that may be recovered is
                                                                         quite wide.
                                                                           Benign Neoplasms
                                                                            Benign neoplasms of the chest wall may be divided into those
                                                                         arising from soft tissue and those arising from bone and cartilage
                                                                         [see Table 2].

                                                                           Soft tissue neoplasms Benign soft tissue neoplasms of the
                                                                         chest wall usually present as slowly growing, painless masses. On
Figure 1 Sternoclavicular joint infections present as painful pal-       examination, the lesions usually are soft and freely movable. Plain
pable masses overlying the joint.                                        radiographs and a CT scan of the chest are obtained. The CT
© 2005 WebMD, Inc. All rights reserved.                                                     ACS Surgery: Principles and Practice
 4 THORAX                                                                                                3 CHEST WALL MASS — 4

          Table 2 Primary Benign Chest Wall                            PRIMARY MALIGNANT MASSES OF
                                                                       CHEST WALL
             Neoplasms by Tissue of Origin
                                                                         Like benign chest wall neoplasms, malig-
                                      Lipoma                           nant chest wall neoplasms may be divid-
                                      Fibroma                          ed into those arising from soft tissue and
                                      Hemangioma                       those arising from bone and cartilage [see
Benign soft tissue neoplasms          Granuloma                        Table 3].
                                      Neurofibroma
                                      Elastoma                           Soft Tissue Neoplasms
                                      Desmoid
                                                                          Sarcomas are the primary malignant soft tissue neoplasms of
                                      Osteochondroma                   the chest wall. Most of the published series addressing soft tissue
Benign bone and cartilage neoplasms
                                      Chondroma                        sarcoma of the chest wall have included small numbers of patients
                                      Fibrous dysplasia                and have also addressed lesions arising in bone and cartilage.The
                                      Eosinophilic granuloma           largest surgical series of patients with soft tissue sarcomas of the
                                                                       chest wall was published in 1991.10 This study included 149
                                                                       patients who had undergone resection at the Memorial Sloan-
                                                                       Kettering Cancer Center in New York.The overall 5-year survival
scan shows a homogeneous mass, with no necrosis and no infil-           rate was 66%. Unfortunately, the study also included 32 patients
tration of associated soft tissue or destruction of associated bone.   with desmoid tumors, which are not histologically classified either
Small soft tissue lesions (< 3 cm) are completely removed by           as sarcomas or as malignant neoplasms. In 2005, a large retro-
means of excisional biopsy, which provides definitive diagnosis         spective study from a single institution in Brazil reported on 55
and treatment. Larger lesions (≥ 3 cm) undergo incisional biopsy       patients who underwent surgical treatment of soft tissue sarcomas
first to rule out a malignant soft tissue neoplasm. If the lesion is    of the chest wall.11 Nearly 53% of the lesions were fibrosarcomas.
confirmed as benign, it is resected with close negative margins to      With wide surgical resection, the disease-free survival rate was
minimize the size of the surgical defect. If it is determined to be    75% at 5 years and 64% at 10 years. The histologic grade of the
malignant, an aggressive wide excision is performed with immedi-       tumor and the type of surgical resection performed were found to
ate reconstruction [see 4:9 Chest Wall Procedures].                    be independent prognostic factors for disease-free survival. These
   Desmoid tumors deserve special mention, in that they are bor-       findings are consistent with those of other studies that suggest that
derline neoplasms.8 These tumors generally arise in the muscle         age, gender, symptoms, and lesion size do not have a significant
and fascia around the shoulder. Although they are histologically       impact on survival.12
benign, they can infiltrate adjacent structures and exhibit a high         Sarcomas of the chest wall can be quite sizable [see Figure 3].
tendency for local recurrence. Desmoid tumors are best treated by      They are painless in about 50% of patients. The typical clinical
means of radical surgical excision. In patients with positive surgi-   finding is a hard, fixed mass. No calcifications are visible on CT
cal margins, the recurrence rate is 89%, whereas in those with neg-    chest scans, but bone invasion is common.The standard treatment
ative margins, the recurrence rate is less than 20%.9                  is wide surgical excision. Currently, the data are not sufficient to
                                                                       warrant recommendation of neoadjuvant therapy, which has
    Bone and cartilage neoplasms Osteochondromas are the               become routine in managing soft tissue sarcomas of the extremi-
most common bone tumors overall. These lesions are benign car-         ties. In 2001, the University of Texas M. D. Anderson Cancer
tilaginous neoplasms that may occur in any bone that undergoes         Center reported its multidisciplinary experience with primary chest
enchondral bone formation; essentially, they are hamartomas of         wall sarcomas.13 The retrospective review included patients with
the growth plate.The knee is the most common site of occurrence.       sarcomas of soft tissue, cartilage, and bone, as well as desmoid
In the chest, osteochondromas arise in the metaphyseal regions of      tumors. Nevertheless, the cumulative 5-year survival rate was 64%,
the anterior ribs. Most are asymptomatic and are found when            which is about the rate to be expected from surgery alone.
screening chest radiographs reveal an eccentric growth pattern at
the costochondral junction. The diagnosis is made on the basis of        Bone and Cartilage Neoplasms
a characteristic pattern on plain x-rays. In children, osteochondro-     A solitary plasmacytoma is a unique chest wall mass that is
mas are followed; in postpubescent and adult patients, they are        caused by a localized collection of monoclonal plasma cells. His-
resected to confirm the diagnosis and rule out malignancy.
    Chondromas are the next most common benign neoplasms of
the chest wall. They occur along the costochondral junctions                    Table 3 Primary Malignant Chest Wall
between the anterior ribs and the sternum. Unfortunately, it is not
possible to distinguish between benign and malignant cartilage neo-                 Neoplasms by Tissue of Origin
plasms on the basis of clinical or radiographic findings; therefore,
                                                                                                                Liposarcoma
excision is required for diagnosis. Excision results in a significant
                                                                                                                Leiomyosarcoma
surgical defect that usually necessitates complex reconstruction,
                                                                       Malignant soft tissue neoplasms          Rhabdomyosarcoma
including the use of prosthetic material to provide rigid structure                                             Malignant fibrous histiocytoma
and soft tissue coverage [see 4:9 Chest Wall Procedures].                                                       Angiosarcoma
    Fibrous dysplasia usually presents as a painless cystic bone
lesion that is found incidentally on a screening chest x-ray. Re-                                               Solitary plasmacytoma
                                                                                                                Chondrosarcoma
placement of the medulla by fibrous tissue creates a characteristic
                                                                       Malignant bone and cartilage neoplasms   Osteosarcoma
radiolucent appearance. These lesions are treated conservatively
                                                                                                                Ewing sarcoma
and are simply followed. Local resection is indicated if pain devel-                                            Synovial cell sarcoma
ops or if the lesion is seen to be enlarging on serial x-rays.
© 2005 WebMD, Inc. All rights reserved.                                                     ACS Surgery: Principles and Practice
4 THORAX                                                                                                3 CHEST WALL MASS — 5

                                                                        multimodality approach that includes preoperative chemothera-
                                                                        py followed by complete resection of residual disease.20 A 2003
                                                                        review of three multi-institutional trials from the Pediatric
                                                                        Oncology Group suggests that in patients with Ewing sarcoma
                                                                        and closely related primitive neuroectodermal tumors of the
                                                                        chest wall, the likelihood of achieving a complete resection is
                                                                        improved by employing neoadjuvant chemotherapy followed by
                                                                        delayed resection.21 The definitive resection is undertaken after
                                                                        four cycles of chemotherapy. If the resection is complete and the
                                                                        pathologic margins are negative, no radiation therapy is adminis-
                                                                        tered. Avoidance of radiation therapy may be particularly impor-
                                                                        tant in pediatric and adolescent patients, who are at significant
                                                                        (10% to 30%) risk for a radiation-induced malignancy over their
                                                                        lifetime.22
                                                                            Askin tumors are members of the Ewing family of tumors.They
                                                                        are small round cell tumors of the thoracopulmonary region that
                                                                        arise from the same primordial stem cell. They are best managed
                                                                        by means of diagnostic biopsy, preoperative chemotherapy, and
Figure 3 Shown is the appearance of a chest wall sarcoma on             complete surgical resection.23
physical examination. Such lesions can be quite large.                      Synovial sarcomas are very uncommon, but they occasionally
                                                                        arise on the trunk, where they present as palpable chest wall
                                                                        masses. Although these tumors are referred to as synovial cell
tologically, the plasma cells are identical to those seen in patients   sarcomas, this term is a misnomer, in that the lesions do not
with multiple myeloma; however, in a plasmacytoma, unlike mul-          arise from synovial cells or joint cavities. The name originally
tiple myeloma, these cells are confined to a single site. Patients       derived from the tumors’ resemblance to developing synovial tis-
generally present with pain and often have pathologic rib frac-         sue under light microscopy; however, synovial sarcomas are now
tures. When soft tissue becomes involved, a palpable mass be-           known to arise from primitive mesenchymal cells.24 The most
comes evident. The role of surgery is limited to obtaining tissue       important prognostic factor is tumor size.25 The limited data
for diagnosis via FNA, core-needle biopsy, or a small incisional        available at present suggest that although neoadjuvant chemo-
biopsy. Tissue is sent for analysis by flow cytometry, which con-        therapy may elicit an objective response, it apparently has no de-
firms the clonal nature of the cells.14 Definitive local radiother-       tectable beneficial effect on survival.26 The current therapeutic
apy is the treatment of choice for solitary bone plasmacytoma.15        recommendation is aggressive surgical resection. If the tumor is
Although local control is achieved in more than 90% of patients         very large or margins are positive, postoperative adjuvant treat-
with radiation therapy alone, about 50% of patients progress            ment is indicated.
to multiple myeloma within 2 years and require systemic
                                                                        SECONDARY MASSES OF CHEST WALL
treatment.16
   Chondrosarcomas are the most common primary malignant                   The secondary chest wall masses of sur-
tumors of the chest wall. They are found along the anterior ster-       gical interest arise as direct extensions of a
nal boarder or the costochondral arches and are substantially           malignancy in a contiguous organ. The
more common in males than in females. Pain is typically a pre-          breast and the lung are the most common
senting complaint. CT scanning is the primary imaging study;            primary sites. The initial evaluation centers
however, there are no distinguishing radiographic characteristics       on the underlying disease, not the chest wall
that establish a definitive diagnosis.17 Complete surgical resection     mass. For example, a patient with a chest
with adequate surgical margins and immediate reconstruction is          wall mass resulting from direct invasion by a primary lung cancer
the treatment of choice [see 4:9 Chest Wall Procedures]. A 2004         should undergo a staging workup to determine the extent of the
study from the Mayo Clinic reported a 5-year survival rate of           disease. If the patient is a stage-appropriate candidate for resection
100% and a recurrence rate of less than 10% in patients with ade-       and is medically fit for surgery, he or she should undergo pul-
quate surgical margins.18 In contrast, patients with inadequate         monary resection with en bloc chest wall resection.27 After resec-
surgical margins had a 50% 5-year survival rate and a 75% local         tion, the patient should be referred for four cycles of postoperative
recurrence rate.                                                        chemotherapy.The interventional intent is cure, and the outcomes
   Ewing sarcoma is an aggressive primary malignant bone tumor          are stage specific.
that occurs in children and adolescents. It is more common in              Unfortunately, most women who present with a chest wall
boys than in girls and usually develops between the ages of 10 and      mass arising from a breast neoplasm have a local recurrence.
20 years. Ewing sarcoma was initially distinguished from osteosar-      From a technical standpoint, resection with reconstruction is
coma on the basis of its sensitivity to radiation. The origin of        feasible in this setting; however, it is unclear whether it offers any
Ewing sarcoma remains unclear, but there appear to be several           real benefit. In a study from the Memorial Sloan-Kettering
tumors that share the same genetic translocation; these lesions are     Cancer Center, 38 women underwent extensive chest wall resec-
referred to as the Ewing family of tumors.19                            tion for recurrent breast cancer.28 The operative mortality was
   The initial role of surgery in managing primary Ewing sarco-         0%, but the 5-year survival rate was only 18%, and by 5 years,
ma of the chest wall consists of obtaining tissue for diagnosis.        87% of the patients had local recurrences. Currently, chest wall
With smaller rib lesions, this may be done by means of an exci-         resection for locally recurrent breast cancer must be considered
sional biopsy. Generally, Ewing sarcoma is best managed with a          on a case-by-case basis.
© 2005 WebMD, Inc. All rights reserved.                                                                                  ACS Surgery: Principles and Practice
4 THORAX                                                                                                                             3 CHEST WALL MASS — 6


References

1. Fortier M, Mayo JR, Swensen SJ, et al: MR imag-           10. Gordon MS, Hadju SI, Bains MS, et al: Soft tis-                tumors defined by specific chimeric transcripts. N
   ing of chest wall lesions. Radiographics 14:597,              sue sarcomas of the chest wall. J Thorac Cardio-               Engl J Med 331:294, 1994
   1994                                                          vasc Surg 101:843, 1991                                    20. Saenz NC, Hass DJ, Meyer P, et al: Pediatric chest
2. Gattuso P, Castelli MJ, Reyes CV, et al: Cutaneous        11. Gross JL,Younes RN, Haddad FJ, et al: Soft-tissue              wall Ewing’s sarcoma. J Pediatr Surg 35:550, 2000
   and subcutaneous masses of the chest wall: a fine-             sarcomas of the chest wall: prognostic factors.            21. Shamberger RC, LaQuaglia MP, Gebhardt MC,
   needle aspiration study. Diagn Cytopathol 15:374,             Chest 127:902, 2005                                            et al: Ewing sarcoma/primitive neuroectodermal
   1996                                                      12. King RM, Pairolero PC, Trastek VF, et al: Primary              tumor of the chest wall: impact of initial versus
3. Toumpoulis IK, Anagnostopoulos CE, DeRose JJ,                 chest wall tumors: factors affecting survival. Ann             delayed resection on tumor margins, survival and
   et al: The impact of deep sternal wound infection             Thorac Surg 41:597, 1986                                       use of radiation therapy. Ann Surg 238:563, 2003
   on long-term survival after coronary artery bypass        13. Walsh GL, Davis BM, Swisher SG, et al: A single-           22. Paulussen M, Ahrens S, Lehnert M, et al: Second
   grafting. Chest 127:464, 2005                                 institutional, multidisciplinary approach to prima-            malignancies after Ewing tumor treatment in 690
4. Ridderstolpe L, Gill H, Granfeldt H, et al: Super-            ry sarcomas involving the chest wall requiring full-           patients from a cooperative German/Austrian/
   ficial and deep sternal wound complications: inci-             thickness resections. J Thorac Cardiovasc Surg                 Dutch study. Ann Oncol 12:1619, 2001
   dence, risk factors and mortality. Eur J Car-                 121:48, 2001                                               23. Veronesi G, Spaggiari L, De Pas T, et al: Preoper-
   diothorac Surg 20:1168, 2001                              14. Jennings CD, Foon KA: Recent advances in flow                   ative chemotherapy is essential for conservative
5. DeFeo M, Gregorio R, Della Corte A, et al: Deep               cytometry: application to the diagnosis of hemato-             surgery of Askin tumors. J Thorac Cardiovasc
   sternal wound infection: the role of early debride-           logic malignancy. Blood 90:2863, 1997                          Surg 125:429, 2003
   ment surgery. Eur J Cardiothorac Surg 19:811,             15. Dimopoulos MA, Moulopoulos LA, Maniatis A,                 24. Miettinen M, Virtanen I: Synovial sarcoma: a mis-
   2001                                                          et al: Solitary plasmacytoma of bone and asymp-                nomer. Am J Pathol 117:18, 1984
6. Song HK, Guy TS, Kaiser LR, et al: Current pre-               tomatic multiple myeloma. Blood 96:2037, 2000              25. Deshmukh R, Mankin H, Singer S: Synovial sar-
   sentation and optimal surgical management of              16. Liebross RH, Ha CS, Cox JD, et al: Solitary bone               coma: the importance of size and location for sur-
   sternoclavicular joint infections. Ann Thorac Surg            plasmacytoma: outcome and prognostic factors                   vival. Clin Orthop Relat Res 419:155, 2004
   73:427, 2002                                                  following radiotherapy. Int J Radiat Oncol Biol            26. Singer S, Baldini EH, Demetri GD, et al: Synovial
7. Bar-Ziv J, Barki Y, Maroko A, et al: Rib osteomye-            Phys 41:1063, 1998                                             sarcoma: prognostic significance of tumor size,
   litis in children: early radiologic and ultrasonic find-   17. Murphey MD, Flemming DJ, Boyea SR, et al:                      margin of resection and mitotic activity for sur-
   ings. Pediatr Radiol 15:315, 1985                             Enchondroma versus chondrosarcoma in the ap-                   vival. J Clin Oncol 14:1201, 1996
8. Hayry P, Reitamo JJ, Totterman S, et al: The                  pendicular skeleton: differentiating features. Radio-      27. Burkhart HM, Allen MS, Nichols FC, et al: Re-
   desmoid tumor: II. Analysis of factors possibly               graphics 5:1213, 1998                                          sults of en bloc resection for bronchogenic carci-
   contributing to the etiology and growth behavior.         18. Fong YC, Pairolero PC, Sim FH, et al: Chondro-                 noma with chest wall invasions. J Thorac Cardio-
   Am J Clin Pathol 77:674, 1982                                 sarcoma of the chest wall. Clin Orthop Relat Res               vasc Surg 123:670, 2002
9. Abbas AE, Deschamps C, Cassivi SD, et al: Chest               427:184, 2004                                              28. Downey RJ, Rusch V, Hsu FI, et al: Chest wall resec-
   wall desmoid tumors: results of surgical interven-        19. Delattre O, Zucman J, Melot T, et al: The Ewing                tion for locally recurrent breast cancer: is it worth-
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Acs0403 Chest Wall Mass

  • 1. © 2005 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 4 THORAX 3 CHEST WALL MASS — 1 3 CHEST WALL MASS John C. Kucharczuk, M.D., F.A.C.S. Evaluation of Chest Wall Mass Chest wall masses arise from a variety of different causes. The a stand-alone test, it cannot differentiate between benign and chest wall contains a number of distinct tissues, including skin, fat, malignant chest wall lesions. muscle, bone, cartilage, lymphatic vessels, and fascia. Each of In general, given that neither physical examination nor diag- these component tissues can give rise to either a benign or a malig- nostic imaging can reliably distinguish benign from malignant nant primary chest wall mass. In addition, the chest wall is also in chest wall masses, it is important to move quickly to tissue diag- intimate proximity to a number of organs (e.g., the breast, the nosis.With very unusual tumors, it is often necessary to consult a lung, the mediastinum, and the pleura) that may give rise to a highly specialized pathologist to make the diagnosis and an oncol- chest wall mass through extension of a malignancy or infection. ogist to assist in treatment planning. Finally, because of its large surface area, the chest wall can be the BIOPSY site of a secondary mass caused by metastasis from a distant malignancy (e.g., carcinoma or sarcoma) [see Table 1]. Whether a tissue diagnosis is needed Chest wall masses, whether primary or secondary, are relative- before initiation of definitive therapy de- ly uncommon in clinical practice. Accordingly, many surgeons pends on the size and characteristics of the lack a solid working knowledge of the causes, evaluation, treat- lesion. If the lesion is small (< 3 cm), ment, and natural history of these lesions. Often, this unfamiliari- regardless of whether it is believed to be ty leads to inappropriate selection of diagnostic studies, unneces- benign or malignant, excisional biopsy is sary delays in treatment, and considerable frustration for patient performed as both diagnosis and treat- and surgeon alike. In what follows, I outline a focused clinical ap- ment. If the lesion is larger (≥ 3 cm) and its resection will lead to proach aimed at streamlining the evaluation and treatment of pa- significant morbidity and necessitate extensive reconstruction, a tients with chest wall masses. Appropriate diagnostic studies and preoperative tissue diagnosis is obtained. operative planning are discussed, and specific causes of chest wall Whether fine-needle aspiration (FNA) is useful for tissue masses are reviewed. diagnosis in this setting remains a subject of debate. FNA is a simple procedure that can be performed in the office during the initial patient evaluation, and several studies suggest that it is an Clinical Evaluation effective technique for assessing chest wall masses.2 Never- theless, in routine clinical practice, cytologic analysis of a fine- HISTORY AND PHYSICAL EXAMINATION needle aspirate from a primary chest wall mass frequently yields Initial evaluation of a patient with a chest nondiagnostic results, and additional tissue is often requested. wall mass begins with a careful history that In such cases, a core-needle biopsy or an incisional biopsy is notes the symptoms associated with the performed. Both techniques provide tissue for histologic evalu- mass and records the history of its growth. ation, and both must be performed in such a way that the biop- Previously obtained radiographs, if available, are reviewed to sy tract will be completely excised at the time of definitive sur- determine how rapidly the mass has been growing. A complete gical treatment. As a rule, in patients with a known primary physical examination is performed to rule out other sites of disease malignancy and a secondary chest wall mass, I perform FNA. and to identify any comorbid medical conditions that may affect In patients with a primary chest wall mass larger than 3 cm and the patient’s candidacy for resection. If the mass is palpable, its size no underlying diagnosis, I proceed directly to incisional biopsy and its salient characteristics (i.e., hard versus soft and fixed ver- for diagnosis. sus mobile) are noted. By itself, physical examination will not establish whether the lesion is benign or malignant. Table 1 Classification of Primary and Secondary Chest Wall Masses Investigative Studies Benign DIAGNOSTIC IMAGING Infectious masses Soft tissue neoplasms At presentation, most patients with Primary masses of Bone and cartilage neoplasms chest wall masses have already undergone chest wall Malignant chest radiography and computed tomog- Soft tissue neoplasms raphy. In the case of a primary chest wall Bone and cartilage neoplasms mass, magnetic resonance imaging is useful for further character- Secondary masses of Tumor invasion from contiguous organs ization of the lesion. MRI allows precise delineation of tissue chest wall Metastasis from distant organs planes and major adjacent neurovascular structures1; however, as
  • 2. © 2005 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 4 THORAX 3 CHEST WALL MASS — 2 Evaluation of Chest Wall Mass Patient presents with chest wall mass Obtain clinical history: • Growth of mass • Associated symptoms • Previous chest radiographs (if available) Perform physical examination: • Other disease sites or comorbid conditions • Size and salient characteristics of mass (if palpable) Determine whether mass is primary or secondary. Chest wall mass is primary Chest wall mass is secondary Consider MRI for further Control primary underlying disease process. characterization of lesion Perform needle biopsy to confirm diagnosis. if desired. Consider definitive treatment after primary Assess size of mass. process is controlled. Mass is < 3 cm Mass is ≥ 3 cm Perform excisional biopsy for diagnosis Perform incisional biopsy for diagnosis. and treatment. Mass represents benign lesion Mass represents malignant lesion Consider resection for confirmation of Perform radical resection with reconstruction. diagnosis or for management of symptoms.
  • 3. © 2005 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 4 THORAX 3 CHEST WALL MASS — 3 Management PRIMARY BENIGN MASSES OF CHEST WALL Infectious Masses Sternal infection Primary sternal osteomyelitis may be seen in intravenous drug abusers but is otherwise rare. Much more common is osteomyelitis occurring after median sternoto- my. Approximately 1% to 3% of median sternotomies for cardiac procedures are complicated by sternal wound infection.3 Risk fac- tors include diabetes, the use of bilateral internal mammary arter- ies, and reoperation.4 Patients with poststernotomy osteomyelitis present with pain, drainage, and, often, a palpable mass overlying the incision. In most cases, the infection is not confined to the sternum but extends deeply into the mediastinum as well. The diagnostic study of choice is CT scanning of the chest, which will Figure 2 CT scan shows typical appearance of SCJ infection, determine the extent of mediastinal soilage. including fluid collection around the joint and tissue stranding. After CT, the patient is taken to the operating room for wide drainage and sternal debridement. A number of different tech- niques have been employed to treat these infections; of these, aggres- sive surgical debridement of the infected area with flap closure yields is made on the basis of the history, the physical examination, and the best overall clinical outcomes [see 4:9 Chest Wall Procedures].5 CT scanning of the chest (with particular attention paid to the The patient who presents with a pulsating sternal mass after SCJ). Typical CT findings include tissue stranding and a collec- sternotomy represents a special case. Such patients have a pseu- tion around the joint [see Figure 2]. doaneursym of the underlying aorta and are at risk for exsan- Treatment consists of wide resection of the SCJ and the proxi- guination.They should undergo an emergency CT angiogram or mal third of the clavicle, as well as debridement of the manubri- aortogram to confirm the diagnosis and then be taken directly to um [see 4:9 Chest Wall Procedures].6 Often, the proximal portion of the OR, where they are placed on cardiopulmonary bypass the first rib is involved and also must be resected. Immediate through femoral cannulation and cooled to a hypothermic state reconstruction is performed by rotating a pectoralis muscle flap before the sternum is opened. into the resection cavity. After operation, I.V. antibiotics are ad- ministered for 6 weeks. Most patients require intensive postoper- Sternoclavicular joint infection Sternoclavicular joint ative physical therapy to restore strength, function, and mobility (SCJ) infections present as painful palpable masses overlying the in the upper extremity. joint [see Figure 1]. These infections are often associated with I.V. drug abuse, infected indwelling subclavian catheters, or trauma. Osteomyelitis of rib Osteomyelitis of a rib presents as a Most patients also have an underlying risk factor (e.g., diabetes or painful, swollen mass overlying an infected segment of rib. Often, hepatic or renal insufficiency) or a history of sepsis.The diagnosis a draining sinus tract is present.The diagnosis is made on clinical grounds. A CT scan of the chest is obtained to rule out an under- lying intrathoracic condition (e.g., empyema). Treatment consists of resection of the infected bone and soft tissue coverage of the defect [see 4:9 Chest Wall Procedures]. Care must be taken to avoid contamination of the underlying pleural cavity during rib resection. In children, the diagnosis is made on clinical grounds and may be facilitated by the use of ultrasonography, which demonstrates obliteration of the intermuscular planes adjacent to the infected rib and pericostal edema.7 Again, a CT scan is usually obtained to rule out other underlying pleura-based abnormalities. As with adults, the range of pathologic organisms that may be recovered is quite wide. Benign Neoplasms Benign neoplasms of the chest wall may be divided into those arising from soft tissue and those arising from bone and cartilage [see Table 2]. Soft tissue neoplasms Benign soft tissue neoplasms of the chest wall usually present as slowly growing, painless masses. On Figure 1 Sternoclavicular joint infections present as painful pal- examination, the lesions usually are soft and freely movable. Plain pable masses overlying the joint. radiographs and a CT scan of the chest are obtained. The CT
  • 4. © 2005 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 4 THORAX 3 CHEST WALL MASS — 4 Table 2 Primary Benign Chest Wall PRIMARY MALIGNANT MASSES OF CHEST WALL Neoplasms by Tissue of Origin Like benign chest wall neoplasms, malig- Lipoma nant chest wall neoplasms may be divid- Fibroma ed into those arising from soft tissue and Hemangioma those arising from bone and cartilage [see Benign soft tissue neoplasms Granuloma Table 3]. Neurofibroma Elastoma Soft Tissue Neoplasms Desmoid Sarcomas are the primary malignant soft tissue neoplasms of Osteochondroma the chest wall. Most of the published series addressing soft tissue Benign bone and cartilage neoplasms Chondroma sarcoma of the chest wall have included small numbers of patients Fibrous dysplasia and have also addressed lesions arising in bone and cartilage.The Eosinophilic granuloma largest surgical series of patients with soft tissue sarcomas of the chest wall was published in 1991.10 This study included 149 patients who had undergone resection at the Memorial Sloan- Kettering Cancer Center in New York.The overall 5-year survival scan shows a homogeneous mass, with no necrosis and no infil- rate was 66%. Unfortunately, the study also included 32 patients tration of associated soft tissue or destruction of associated bone. with desmoid tumors, which are not histologically classified either Small soft tissue lesions (< 3 cm) are completely removed by as sarcomas or as malignant neoplasms. In 2005, a large retro- means of excisional biopsy, which provides definitive diagnosis spective study from a single institution in Brazil reported on 55 and treatment. Larger lesions (≥ 3 cm) undergo incisional biopsy patients who underwent surgical treatment of soft tissue sarcomas first to rule out a malignant soft tissue neoplasm. If the lesion is of the chest wall.11 Nearly 53% of the lesions were fibrosarcomas. confirmed as benign, it is resected with close negative margins to With wide surgical resection, the disease-free survival rate was minimize the size of the surgical defect. If it is determined to be 75% at 5 years and 64% at 10 years. The histologic grade of the malignant, an aggressive wide excision is performed with immedi- tumor and the type of surgical resection performed were found to ate reconstruction [see 4:9 Chest Wall Procedures]. be independent prognostic factors for disease-free survival. These Desmoid tumors deserve special mention, in that they are bor- findings are consistent with those of other studies that suggest that derline neoplasms.8 These tumors generally arise in the muscle age, gender, symptoms, and lesion size do not have a significant and fascia around the shoulder. Although they are histologically impact on survival.12 benign, they can infiltrate adjacent structures and exhibit a high Sarcomas of the chest wall can be quite sizable [see Figure 3]. tendency for local recurrence. Desmoid tumors are best treated by They are painless in about 50% of patients. The typical clinical means of radical surgical excision. In patients with positive surgi- finding is a hard, fixed mass. No calcifications are visible on CT cal margins, the recurrence rate is 89%, whereas in those with neg- chest scans, but bone invasion is common.The standard treatment ative margins, the recurrence rate is less than 20%.9 is wide surgical excision. Currently, the data are not sufficient to warrant recommendation of neoadjuvant therapy, which has Bone and cartilage neoplasms Osteochondromas are the become routine in managing soft tissue sarcomas of the extremi- most common bone tumors overall. These lesions are benign car- ties. In 2001, the University of Texas M. D. Anderson Cancer tilaginous neoplasms that may occur in any bone that undergoes Center reported its multidisciplinary experience with primary chest enchondral bone formation; essentially, they are hamartomas of wall sarcomas.13 The retrospective review included patients with the growth plate.The knee is the most common site of occurrence. sarcomas of soft tissue, cartilage, and bone, as well as desmoid In the chest, osteochondromas arise in the metaphyseal regions of tumors. Nevertheless, the cumulative 5-year survival rate was 64%, the anterior ribs. Most are asymptomatic and are found when which is about the rate to be expected from surgery alone. screening chest radiographs reveal an eccentric growth pattern at the costochondral junction. The diagnosis is made on the basis of Bone and Cartilage Neoplasms a characteristic pattern on plain x-rays. In children, osteochondro- A solitary plasmacytoma is a unique chest wall mass that is mas are followed; in postpubescent and adult patients, they are caused by a localized collection of monoclonal plasma cells. His- resected to confirm the diagnosis and rule out malignancy. Chondromas are the next most common benign neoplasms of the chest wall. They occur along the costochondral junctions Table 3 Primary Malignant Chest Wall between the anterior ribs and the sternum. Unfortunately, it is not possible to distinguish between benign and malignant cartilage neo- Neoplasms by Tissue of Origin plasms on the basis of clinical or radiographic findings; therefore, Liposarcoma excision is required for diagnosis. Excision results in a significant Leiomyosarcoma surgical defect that usually necessitates complex reconstruction, Malignant soft tissue neoplasms Rhabdomyosarcoma including the use of prosthetic material to provide rigid structure Malignant fibrous histiocytoma and soft tissue coverage [see 4:9 Chest Wall Procedures]. Angiosarcoma Fibrous dysplasia usually presents as a painless cystic bone lesion that is found incidentally on a screening chest x-ray. Re- Solitary plasmacytoma Chondrosarcoma placement of the medulla by fibrous tissue creates a characteristic Malignant bone and cartilage neoplasms Osteosarcoma radiolucent appearance. These lesions are treated conservatively Ewing sarcoma and are simply followed. Local resection is indicated if pain devel- Synovial cell sarcoma ops or if the lesion is seen to be enlarging on serial x-rays.
  • 5. © 2005 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 4 THORAX 3 CHEST WALL MASS — 5 multimodality approach that includes preoperative chemothera- py followed by complete resection of residual disease.20 A 2003 review of three multi-institutional trials from the Pediatric Oncology Group suggests that in patients with Ewing sarcoma and closely related primitive neuroectodermal tumors of the chest wall, the likelihood of achieving a complete resection is improved by employing neoadjuvant chemotherapy followed by delayed resection.21 The definitive resection is undertaken after four cycles of chemotherapy. If the resection is complete and the pathologic margins are negative, no radiation therapy is adminis- tered. Avoidance of radiation therapy may be particularly impor- tant in pediatric and adolescent patients, who are at significant (10% to 30%) risk for a radiation-induced malignancy over their lifetime.22 Askin tumors are members of the Ewing family of tumors.They are small round cell tumors of the thoracopulmonary region that arise from the same primordial stem cell. They are best managed by means of diagnostic biopsy, preoperative chemotherapy, and Figure 3 Shown is the appearance of a chest wall sarcoma on complete surgical resection.23 physical examination. Such lesions can be quite large. Synovial sarcomas are very uncommon, but they occasionally arise on the trunk, where they present as palpable chest wall masses. Although these tumors are referred to as synovial cell tologically, the plasma cells are identical to those seen in patients sarcomas, this term is a misnomer, in that the lesions do not with multiple myeloma; however, in a plasmacytoma, unlike mul- arise from synovial cells or joint cavities. The name originally tiple myeloma, these cells are confined to a single site. Patients derived from the tumors’ resemblance to developing synovial tis- generally present with pain and often have pathologic rib frac- sue under light microscopy; however, synovial sarcomas are now tures. When soft tissue becomes involved, a palpable mass be- known to arise from primitive mesenchymal cells.24 The most comes evident. The role of surgery is limited to obtaining tissue important prognostic factor is tumor size.25 The limited data for diagnosis via FNA, core-needle biopsy, or a small incisional available at present suggest that although neoadjuvant chemo- biopsy. Tissue is sent for analysis by flow cytometry, which con- therapy may elicit an objective response, it apparently has no de- firms the clonal nature of the cells.14 Definitive local radiother- tectable beneficial effect on survival.26 The current therapeutic apy is the treatment of choice for solitary bone plasmacytoma.15 recommendation is aggressive surgical resection. If the tumor is Although local control is achieved in more than 90% of patients very large or margins are positive, postoperative adjuvant treat- with radiation therapy alone, about 50% of patients progress ment is indicated. to multiple myeloma within 2 years and require systemic SECONDARY MASSES OF CHEST WALL treatment.16 Chondrosarcomas are the most common primary malignant The secondary chest wall masses of sur- tumors of the chest wall. They are found along the anterior ster- gical interest arise as direct extensions of a nal boarder or the costochondral arches and are substantially malignancy in a contiguous organ. The more common in males than in females. Pain is typically a pre- breast and the lung are the most common senting complaint. CT scanning is the primary imaging study; primary sites. The initial evaluation centers however, there are no distinguishing radiographic characteristics on the underlying disease, not the chest wall that establish a definitive diagnosis.17 Complete surgical resection mass. For example, a patient with a chest with adequate surgical margins and immediate reconstruction is wall mass resulting from direct invasion by a primary lung cancer the treatment of choice [see 4:9 Chest Wall Procedures]. A 2004 should undergo a staging workup to determine the extent of the study from the Mayo Clinic reported a 5-year survival rate of disease. If the patient is a stage-appropriate candidate for resection 100% and a recurrence rate of less than 10% in patients with ade- and is medically fit for surgery, he or she should undergo pul- quate surgical margins.18 In contrast, patients with inadequate monary resection with en bloc chest wall resection.27 After resec- surgical margins had a 50% 5-year survival rate and a 75% local tion, the patient should be referred for four cycles of postoperative recurrence rate. chemotherapy.The interventional intent is cure, and the outcomes Ewing sarcoma is an aggressive primary malignant bone tumor are stage specific. that occurs in children and adolescents. It is more common in Unfortunately, most women who present with a chest wall boys than in girls and usually develops between the ages of 10 and mass arising from a breast neoplasm have a local recurrence. 20 years. Ewing sarcoma was initially distinguished from osteosar- From a technical standpoint, resection with reconstruction is coma on the basis of its sensitivity to radiation. The origin of feasible in this setting; however, it is unclear whether it offers any Ewing sarcoma remains unclear, but there appear to be several real benefit. In a study from the Memorial Sloan-Kettering tumors that share the same genetic translocation; these lesions are Cancer Center, 38 women underwent extensive chest wall resec- referred to as the Ewing family of tumors.19 tion for recurrent breast cancer.28 The operative mortality was The initial role of surgery in managing primary Ewing sarco- 0%, but the 5-year survival rate was only 18%, and by 5 years, ma of the chest wall consists of obtaining tissue for diagnosis. 87% of the patients had local recurrences. Currently, chest wall With smaller rib lesions, this may be done by means of an exci- resection for locally recurrent breast cancer must be considered sional biopsy. Generally, Ewing sarcoma is best managed with a on a case-by-case basis.
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