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![PET Scan
• assess metabolic function and can be used
to diagnose, stage, and monitor treatment
response for many cancers, and some soft
tissue tumors.
• One specific application is in the evaluation
of multifocal desmoid tumors, and more
specifically, response to treatment.
“ Kasper et al. demonstrated that PET imaging
might complement CT and improve the
assessment of patients with desmoid
tumors. Imatinib, a tyrosine kinase inhibitor,
has been shown to successfully stabilize
desmoid tumors, and PET imaging has been
used to monitor response to imatinib
treatment in patients with multifocal
desmoids [9]”](https://image.slidesharecdn.com/softtissuetumor-160706134441/85/Soft-tissue-tumor-22-320.jpg)
![Role of Radiotherapy in soft tissue
sarcoma
• enhance local control
• preserve function
• achieve acceptable cosmesis by contributing to tissue preservation
“Dagan et al. performed a retrospective review to evaluate the local control
and amputation-free survival in patients who received preoperative
radiation therapy prior to undergoing a marginal resection for a soft tissue
sarcoma of the extremity. The authors concluded that patients can expect
excellent rates of local control and limb preservation regardless of whether
they have a marginal, wide, or radical resection according to the classic
Enneking margin definitions [37].”](https://image.slidesharecdn.com/softtissuetumor-160706134441/85/Soft-tissue-tumor-31-320.jpg)
Uploaded bymanoj das
Soft tissue tumor
This document provides information about soft tissue tumors. It discusses the epidemiology, classification, etiology, diagnosis and treatment of both benign and malignant soft tissue tumors. Some key points include: - Benign soft tissue tumors are more common than sarcomas. Common benign tumors include lipomas, schwannomas and giant cell tumors of the tendon sheath. - Risk factors for soft tissue sarcoma include exposure to herbicides/pesticides, radiation exposure, genetic conditions and viral infections. - MRI is usually the best imaging modality for evaluating soft tissue tumors. Biopsy is needed for diagnosis. - Treatment depends on whether the tumor is benign or malignant. Benign tumors may
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Soft tissue tumor
- 1. SOFT TISSUE TUMOR DRMANOJ DAS Ortho resident Institute Of Medicine , TUTH , Nepal
- 2. Intoduction • Neoplastic conditionarising from extra-skeletal mesoderm -Fibrous tissue -Smooth muscle -Skeletal muscle -Adipose tissue -Blood and lymphatics vessels -Peripheral nerves
- 3. Epidemology -The annual clinicalincidence (number of new patients consulting a doctor) of benign soft tissue tumours has been estimated as up to 3000/million population -The annual incidence of soft tissue sarcoma is around 30/million i.e. less than 1 percent of all malignant tumours -Benign mesenchymal tumours outnumber sarcomas by a factor of at least 100.
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- 7. Aetiology • Unknown • Inrare cases, genetic and environmental factors, irradiation, viral infections and immune deficiency have been found associated with the development of usually malignant soft tissue tumours
- 8. Aetiology • Chemical carcinogens -Severalstudies, many of them from Sweden, have reported an increased incidence of soft tissue sarcoma after exposure to phenoxyacetic herbicides,chlorophenols, and their contaminants(dioxin) in agricultural or forestry work {607,608}. -Other studies have not found this association. -One explanation for different findings may be the use of herbicides with different dioxin contaminations {4,2333}.
- 9. Aetiology… Radiation -incidence of post-irradiationsarcoma ranges from some few per thousand to nearly one percent. -incidence estimates are based on breast cancer patients treated with radiation as adjuvant therapy. The risk increases with dose; most patients have received 50 Gy or more and the median time between exposure and tumour diagnosis is about 10 years. -Patients with a germline mutation in the retinoblastomas gene (RB1) have a significantly elevated risk of developing post- irradiation sarcomas, usually osteosarcoma
- 10. Aetiology… - Viral infectionand immunodeficiency Human herpes virus 8 plays a key role in the development of Kaposi sarcoma and the clinical course is dependent on the immune status of the patient {2232}. -Epstein-Barr virus is associated with smooth muscle tumours in patients with immunodeficiency {1368}. -StewartTreves syndrome, development of angiosarcoma in chronic lymphoedema, particularly after radical mastectomy, has by some authors been attributed to regional acquired immunodeficiency
- 11. Aetiology… Genetic susceptibility hereditary multiplelipomas (often angiolipomas) Desmoid tumours occur in patients with the familial Gardner syndrome (including adenomatous polyposis, osteomas and epidermal cysts) Neurofibromatosis (types 1and 2) is associated with multiple benign nerve tumours (and sometimes also nonneural tumours). In around 2% of the patients with neurofibromatosis type1 malignant peripheral nerve sheath tumours develop in a benign nerve sheath tumour Li-Fraumeni syndrome {954} is a rare autosomal dominant disease caused by germline mutations in the TP53 tumour suppressor gene, which seems to be of importance for sarcomagenesis. Half of the patients have already developed malignant tumours at age 30, among them, in more than 30% of cases, soft tissue and bone sarcomas .
- 12. Approach to diagnosis History -Age - Symptoms Mass Pain Numbness - Duration - Trauma - Family History - Constitution Symptoms
- 13. Approach to diagnosis… LocalExamination -site -Size -Depth -Consistency -Mobility -Pulsation -Tinel’s Sign -Neurovascular Status
- 14. • General Physical examination -café-lauit spot - Freckles • Lymph Node Examination
- 15. X -Ray • Providelittle information • Fat density radiolucencies • Phleboliths • calcification
- 16. CT Scan • Replacedby MRI in most institutions due to the superior soft tissue contrast achieved by MRI • still be useful for imaging soft tissues near the chest wall that are subject to motion artifact with MRI, and in cases where MRI is not an option
- 17. MRI - modality ofchoice for detecting, characterizing, and staging soft tissue tumours - distinguish tumour tissue from adjacent muscle and fat - define relationships to key neurovascular bundles
- 22. PET Scan • assessmetabolic function and can be used to diagnose, stage, and monitor treatment response for many cancers, and some soft tissue tumors. • One specific application is in the evaluation of multifocal desmoid tumors, and more specifically, response to treatment. “ Kasper et al. demonstrated that PET imaging might complement CT and improve the assessment of patients with desmoid tumors. Imatinib, a tyrosine kinase inhibitor, has been shown to successfully stabilize desmoid tumors, and PET imaging has been used to monitor response to imatinib treatment in patients with multifocal desmoids [9]”
- 23. Biopsy • open (incisionalor excisional) or closed (needle or trephine) Closed Biopsy Advantage - less invasive, -require smaller doses of anesthetic and analgesic agents -lower risk of tumor contamination, hemorrhage, and infection Disadvantage -Smaller amount obtained for pathologic review
- 24. Some rules forfor Biopsy “One should always approach tissue biopsy with the assumption that there is a possibility for malignancy, using a well-planned biopsy site” - Perform the biopsy through a longitudinal incision. -In the operative dissection, do not expose or contaminate important vascular or neural structures. - Do not violate anatomic compartments other than those containing the tumor. - Take special care around joints to prevent intraarticular contamination with tumor cells. - Biopsies of soft tissue should not involve major tendons (e.g., the patellar tendon) or their insertion -In a biopsy, do not use intramuscular or intranervous planes, as in more standard orthopaedic approaches. Instead, use a direct approach with the smallest possible incision directly through skin, subcutaneous tissue, fascia, and muscle down to tumor
- 25. Staging of softtissue tumor
- 26. Staging of softtissue sarcoma American joint committee on cancer for staging of soft tissue sarcoma
- 27. Treatment of BenignSoft tissue tumor Observation - Asymptomatic - <5cm - Superficial to fascia -Reevaluation every 6-12 weeks followed by every 3-6 months for 1 yr to document lack of growth Biopsy and Surgical Excision - not following natural history -Symptomatic
- 28. Treatment of Softtissue Sarcoma Goal of Treatment -minimizing local recurrence -maximizing function -improving patient survival Surgery- Mainstay of treatment
- 29. Limb Salvage SurgeryVS Amputation • Amputation rarely done nowadays • Limb sparing procedure are performed in 90-95% of cases “Rosenberg et al published a landmark article in 1982, a prospective randomized trial to evaluate the issue of amputation versus limb- sparing surgery plus radiation therapy in soft tissue sarcomas of the extremity. They found that although local recurrence was greater in the limb-sparing group, disease-free survival was no different”
- 31. Role of Radiotherapyin soft tissue sarcoma • enhance local control • preserve function • achieve acceptable cosmesis by contributing to tissue preservation “Dagan et al. performed a retrospective review to evaluate the local control and amputation-free survival in patients who received preoperative radiation therapy prior to undergoing a marginal resection for a soft tissue sarcoma of the extremity. The authors concluded that patients can expect excellent rates of local control and limb preservation regardless of whether they have a marginal, wide, or radical resection according to the classic Enneking margin definitions [37].”
- 32. Role of Chemotherapyin soft tissue Sarcoma • Controversial and still investigated • palliative and used to treat unresectable or metastatic disease to try and slow progression “ In 1997, the Sarcoma Meta-Analysis Collaboration published their results of doxorubicin-based chemotherapy regimens combined with surgery for local control. Disease-free survival improved from 45 % for control patients to 55 % for chemotherapy patients, an advantage of only 10 %. The authors were also unable to show a statistically significant benefit to improved survival at 10 years, with survival of patients undergoing chemotherapy at 54 %, compared to the control at 50 %” Favorable response to chemotherapy, -synovial sarcoma -Liposarcoma - pediatric rhabdomyosarcom
- 33. Surveillance Following Definitive Treatment •For potential development of local recurrence or metastatic disease • 40–60 % of patients will develop local or distant recurrence, and most recurrences occur within 2 years of treatment of the primary tumor • Current recommendations for surveillance include:- - clinical exam and chest radiograph or CT scan every 3 months for the first 2 years, every 4 months for the third year, every 6 months for the following 2 years, and annually thereafter • Local recurrence monitored with physical exams of the surgical site at routine intervals -If the patient is felt to be at high risk for local recurrence or there is concern on physical exam findings, then an ultrasound or MRI with and without gadolinium should be obtained.
- 34. Some important softtissue tumor
- 35. LIPOMA • the mostcommon soft tissue lesions • AGE:- 5th to 7th decades of life without a clear gender predilectioN • prevalence:- 2.1 per 100 people • Site:-upper back, neck, proximal extremities, and abdomen • Clinical Presentation -Painless mass -can be a/w local pain, limitation of range of motion, and nerve compression in approximately 25 % of patients -Usually Solitary • On physical Examination Mobile Doughy in consistency usually S/C Investgations X-ray - Soft tissue radiolucency MRI – Investigation of choice Isointense to s/c fat
- 37. LIPOMA… Tissue Diagnosis Gross -yellow toorange color with a greasy appearance Microscopic -mature adipocytes with uniform eccentric nuclei
- 38. Lipoma… Treatment Observation Excisionalbiopsy -increasing size -Symptomatic - Cosmetically undesirsble
- 39. Benign Peripheral NerveSheath Tumor Include -Schwannoma -Neurofiroma Age:- 20-50yr Schwannoma 40-50 yr Neurofibroma A/W Neurofibromatosis Type 2 Clinical Presentation - Painless mas - Neurological symptoms On Physical Examination -Firm - Mobile -Tender to touch and pressure ( Schannoma)
- 40. Benign Peripheral NerveSheath Tumor.. X-Ray - Soft tissue mass MRI -Fusiform mass, often displacing neurovascular bundle
- 42. Benign Peripheral NerveSheath Tumor… Tissue Diagnosis Gross -cut surface of a schwannoma has a yellow-gray color, sometimes with cystic regions Microscopic - Antoni A - Antoni B Neurofibromas- - not encapsulated - Histologically spindle-shaped cells in a myxoid stroma, with some collagen fibers.
- 43. Benign Peripheral NerveSheath Tumor… Treatment Observation with Serial Radiograph Surgical Excision Risk for neurological deficit after surgical excision??? - secondary to iatrogenic fascicular injury “Kim et al. evaluated postoperative neurological deficits and potential risk factors associated with this complication. They found an immediate neurological deficit in 76.7 % of their 30 patients, and at final followup, residual deficits persisted in 36.7 % of patients. Therisk for deficits was highest in patients with larger tumors.”
- 44. Giant Cell Tumorof Tendon Sheath • the most common benign neoplasm of the hand, often found on the fingers • fourth to fifth decade of life • On physical examination -firm -minimally mobile mass that runs along tendon -usually nontender Radiographs -nonspecific -cortical erosion Ultrasound -To distinguish this solid tumor from a ganglion cyst MRI - circumscribed soft tissue mass adjacent to a tendon, and is isointense with muscle on T1-weighted images
- 45. Giant Cell Tumorof Tendon Sheath… • On gross examination -dense capsule - C/S gray-white appearance with variegated pink, brown, or yellow discoloration • Histologically the predominant cells are mononuclear cells, epithelioid histiocyte like cells, giant cells, and xanthomatous cells, hemosiderin- laden macrophages Treatment -Excision Biopsy Recurrence Rate 10-20 %
- 46. Hemangioma • benign vascularlesions that histologically resemble normal blood vessels • Common tumor in 1st decade of life • Sites - Skin - Subcutaneus tissue - Intramuscular -Intraarticular -Bone Types - Capillary - Cavernous -Venous -Arteriovenous Malformation
- 47. Intramuscular Hemangioma • Moreoften larger than cutaneous hemangioma • Composed of morphologically benign vascular channels occuring within muscle • Age – Adolescents and adult • Sites –Lower limb m/c, Head neck, upper limb • Clinical presentation - Slowly enlarging mass - painful -pain worse after exercise -Changes size according to position of limb X-Ray -Phlebolith -Pressure erosion of bone or extension in to bone( Channel like radiolucencies )
- 48. Intramuscular Hemangioma… CT Scan -poorly defined lesion attenuation similar to muscle -Marked contrast enhancement of serpentine vascular channels -Subtle phlebolith MRI - hypointense, or isointense to muscle on T1-weighted sequences -hyperintense on T2-weighted sequences due to the vascular channels
- 49. Intramuscular Hemangioma… Gross - large,poorly demarcated, yelowish - Vascular and hemorhagic areas Microscopic - Mixed vessel type - Lined by normal appearing epithelial cells - Diffusely infiltrate muscle and entrap muscle cells - Prominent adipose component
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- 51. Desmoid Tumor • Benign, non metastasizing infiltrative fibrofroliferative neoplasm • Composed of fibrocytes, fibroblasts, and myofibroblasts within a collagenenous to myxoid stroma • Infiltration to muscle occur routinely • High propensity for local recurrence • A/W Familial Adenomatous polyposis
- 52. Desmoid Tumor… • Age15-40 • F>M • Sites – -shoulder girdle/upper arm(mc) -Buttock -Trunk -Head and Neck Clinical Presentation - painless/painful mass -When in close proximity to a joint, range of motion may be limited -Neurological symptoms if the tumor compresses or invades peripheral motor or sensory nerves.
- 53. Desmoid Tumor… X-ray -Soft tissuemass -Cortical erosion CT scan - Similar accentuation to muscle MRI -low signal intensity on both T1 and T2 Sequence - Frequently enhance after i/v contrast
- 54. Desmoid Tumor… Gross - Largetumor - Glistening with fibrous tissue - Infiltrates skeletal muscle at periphery Microscopic - Normal appearing sparse fibroblastic cells within dense fibrous stroma - Macrophage, lymphocyte, giant cells
- 55. Desmoid Tumor… Treatment - Observationfor asymptomatic and stable - Aggressive treatment for symptomatic lesion Surgical excision+ Adjuvant Radiotherapy
- 56. Glomus Tumor • Benignlesion arising from glomus bodies • 2 % of soft tissue tumors • Sites- hand especially within the subungual skin, palm, wrist, forearm, or foot • Age – adult • Clinical presentation: Triad of -hypersensitivity to cold - paroxysmal pain -pinpoint pain • Love’ s Pin test- 100% sensitive • Hildreth’s test- 100% specific and & 79% sensitive
- 57. MRI -increased signal intensityon T2- weighted images, and strong contrast enhancement Gross- small blue red nodules Microscopic- glomus cells , smooth cells, blood vessels Treatment -Excision biopsy
- 58. Synovial chondromatosis • disorderin which cartilaginous nodules are formed within a synovial joint • The nodules then detach from the synovium and may undergo calcification • Age- third and fifth decades • M:F 2:1 • Site- Usually monoarticular with the knee being the most common joint affected, but it can involve any synovial joint • Clinical presentation -pain, swelling, stiffness, and sometimes locking of the involved joint. On physical examination - effusion, tenderness, and decreased ROM.
- 59. Synovial chondromatosis… Radiograph -multiple intra-articulardensities when calcified - ‘‘ring-and-arc’’ chondroid mineralization pattern MRI -visualizing radiolucent nodules where they have low signal on T1-weighted images, and increased signal on T2- weighted images -erosive changes of the articular cartilage -in late stages, there is joint space narrowing, osteophytes, and sclerosis much like an osteoarthritic joint
- 60. Synovial chondromatosis… On gross inspection -thesynovial nodules, which are composed of hyaline cartilage, typically have a cobblestone appearance histologic appearance -benign hyaline cartilage, with low to moderate cellularity, and a synovial tissue lining outside Treatment -Nodule removal and Synovectomy -Open or arthroscopic approach
- 61. Liposarcoma • 20 %of all soft tissue sarcomas in adults • Ages 50–65 • Site-thigh and retroperitoneum • 1) Well-differentiated liposarcomas (atypical lipomatous tumor) 2)Dedifferentiated liposarcomas • 3)Myxoid or Round cell liposarcomas • 4)pleomorphic
- 62. Rhabdomyosarcoma • most commonsoft tissue sarcoma of infants and children, although they can occur in adults • Site – in areas lacking skeletal muscle- head, neck and genitourinary tract • Embryonal rhabdomyosarcoma –most common type -head , neck and genitourinary tract • Alveolar rhabdomyosarcoma - Aggressive type -arm, leg and trunk Pleomorphic rhabdomyosarcoma -common in adults - poor prognosis Clinical presentation depends on location
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Editor's Notes
- #13 patients with multifocal disease or those who have an established clinical syndrome. Exceptions include a familial history of lipomatosis, neurofibromatosis, Gardner's syndrome (colonic polyposis and desmoid tumor), Li-Fraumeni syndrome (familial breast cancer and soft-tissue sarcoma) (16), and Maffucci's disease (hemangiomas noted in conjunction with multiple bone enchondromas). patients with multifocal disease or those who have an established clinical syndrome. Exceptions include a familial history of lipomatosis, neurofibromatosis, Gardner's syndrome (colonic polyposis and desmoid tumor), Li-Fraumeni syndrome (familial breast cancer and soft-tissue sarcoma) (16), and Maffucci's disease (hemangiomas noted in conjunction with multiple bone enchondromas).
- #23 It may be less desirable in many circumstances due to its great expense, as well as the requirement for an injection of a radioactive substance into the patient.
- #27 Stage I tumors are low grade regardless of size or depth. Stage II tumors are high grade; they may be small and any depth or large and superficial. Stage III tumors are high grade, large, and deep. Stage IV tumors are tumors associated with metastases (including local lymph nodes) regardless of grade, size, or depth
- #29 The extent of surgery required and the use of radiotherapy and chemotherapy as an adjuvant continues to be a topic of controversy and is usually institution dependent.