This document discusses several cases of atypical chest neoplasms: 1. It describes bronchus-associated lymphoid tissue (BALT) lymphoma, a rare subtype of primary non-Hodgkin lymphoma that occurs in the lung. BALT lymphoma has nonspecific imaging findings but usually presents as nodules, masses, consolidation or ground-glass opacity. 2. It also discusses lymphangitic carcinomatosis, a tumor spread through the lungs' lymphatics most commonly seen with adenocarcinoma. On imaging, it demonstrates interlobular septal thickening, thickening of the bronchovascular interstitium, and often has a "dot in box" appearance

1. Atypical chest
neoplasms
DR. JAYANTH H KESHAVAMURTHY MD.

2. Bronchus-associated lymphoid
tissue lymphoma
 Primary pulmonary lymphoma is distinguished from
the more common secondary pulmonary lymphoma
by lack of extrapulmonary involvement.
 BALT lymphoma is the most common type of primary
pulmonary lymphoma.
 In a case of chronic multifocal consolidation or
multiple pulmonary nodules, BALT lymphoma should
be included in the differential diagnosis.

3.  Bronchus-associated lymphoid tissue (BALT) lymphoma is a rare subtype of
primary non-Hodgkin lymphoma that may occur within the lung.
 BALT lymphoma has variable imaging findings and can mimic other
malignancies such as lung adenocarcinoma on CT.
 The most common findings include parenchymal abnormalities such as
nodules, masses, consolidation, and ground-glass opacity.
 Intrathoracic (hilar or mediastinal) lymphadenopathy is usually absent.

4.  Mucosa-associated lymphoid tissue (MALT) lymphoma is a subtype of non-
Hodgkin B-cell lymphoma that can occur anywhere in the body, although it
most frequently affects the gastrointestinal system.
 When it presents in the lung, it is called BALT lymphoma.
 BALT lymphoma accounts for at least two-thirds of primary pulmonary non-
Hodgkin lymphoma but is overall very uncommon.
 The pathogenesis involves long-term antigenic stimulation from autoimmune,
inflammatory, or infectious etiologies.
 Associations between BALT lymphoma and smoking, Sjögren disease, multiple
sclerosis, rheumatoid arthritis, diffuse panbronchiolitis, chronic hypersensitivity
pneumonitis, and Hashimoto thyroiditis have been proposed.
 Most cases occur in patients over 50 years of age. Patients usually have no
symptoms at presentation, although chest discomfort, cough, breathing
difficulty, or hemoptysis may be present.

5.  On cross-sectional imaging with CT, BALT lymphoma typically
demonstrates nonspecific imaging findings, which include nodules,
masses, consolidation, and ground-glass opacity.
 Regions of consolidation may demonstrate indistinct borders and air
bronchograms. The lung may be involved in a focal or more diffuse
manner. Pleural effusions are uncommon. I
 ntrathoracic (hilar or mediastinal) lymphadenopathy is not a typical
feature of BALT. Differential considerations include such entities as lung
adenocarcinoma, organizing pneumonia,alveolar sarcoid, multifocal
pneumonia and pulmonary metastasis.
 Treatment usually consists of a combination of surgical resection,
chemotherapy, radiation therapy, and/or immunotherapy.
 Survival rates are relatively high because the disease is typically
diagnosed prior to extrapulmonary extension, and disease progression is
slow.

12.  Lymphangitic carcinomatosis, or lymphangitis
carcinomatosa, is the term given to tumour spread
through the lymphatics of the lung, and is most
commonly seen secondary to adenocarcinoma.

13. Pathology
 Lymphangitis carcinomatosa is most commonly seen
secondary to adenocarcinomas such as:
 breast cancer - most common
 lung cancer (bronchogenic adenocarcinoma)
 colon cancer
 stomach cancer
 prostate cancer
 cervical cancer
 thyroid cancer
 It can also be seen in numerous other primary cancers,
e.g. laryngeal cancer,pancreatic cancer, etc.

14. Radiographic features
 Radiographic appearances can most easily be divided
into those due to involvement of the peripheral
(interlobular septae) and central lymphatic system.
Involvement may be diffusely of both, or
predominantly of one or other compartment .
 Distribution of changes is variable, but most are
asymmetric and patchy .
 It is usually bilateral but may be unilateral especially in
cases of lung and breast cancer.

15.  CT, especially HRCT, is excellent at demonstrating both
peripheral and central changes.
 Typically the appearance is that of interlobular septal
thickening most often nodular and irregular, although
smooth thickening may also sometimes be seen . This
results in prominent definition of the secondary
pulmonary nodules, manifesting as tessellating
polygons.
 Thickening of the bronchovascular interstitium is
usually irregular and nodular, with changes seen
extending towards the hilum .
 The combination may give a characteristic "dot in box"
appearance.

16.  subpleural nodules, and thickening on the interlobar
fissures
 pleural effusion(s): pleural carcinomatosis
 hilar and mediastinal nodal enlargement (40-50%)
 relatively little destruction of overall lung architecture

17. Differential diagnosis
 Considerations include a differential for that
of thickened interlobular septae, with common entities
comprising of :
 sarcoidosis
 viral pneumonia
 pulmonary oedema - changes are commonly bilateral
and often have a gravitational distribution
 radiation pneumonitis
 lymphocytic interstitial pneumonitis (LIP)

20.  KS should be considered in the differential in patients
with AIDS who have localized bone pain or a febrile
illness of unknown origin.
 Tissue sampling is required to confirm the diagnosis.
 Even though the majority of the lesions are lytic they
are usually not seen on plain films. CT or MRI is the
preferred imaging modality.

21. KS is caused by Human Herpesvirus 8. It is a low-grade
mesenchymal neoplasm of blood and lymphatic origin primarily
affecting the skin. To date, four different types have been
described: AIDS-related, African, classic, and transplantation/
immunosuppression. KS usually involves the lungs,
gastrointestinal (GI) tract, liver, spleen, and skin. It rarely involves
the bone marrow, but there have been multiple biopsy-proven case
reports. Bone marrow involvement has been associated with a
poor prognosis.

22.  On chest plain films, KS usually presents as middle to
lower lung zone reticular opacities and parenchymal
nodules. On CT, the pulmonary findings are bilateral,
symmetric, and poorly marginated nodules arising from
the hila. They are characteristically referred to as
“flame-shaped” nodules. These nodules tend to
coalesce and can grow to measure more than 1 cm and
show surrounding ground-glass opacities—this is
known as the “halo” sign. Diffuse lymphadenopathy
and bilateral pleural effusions are common findings.

23. https://3s.acr.org/CIP/S
earchCaseView.aspx?Ca
seId=hATqEbi8jXk%3d

25.  Pancoast syndrome results from involvement
of brachial plexus and sympathetic chain by
tumour, or less commonly from other tumours
involving the superior pulmonary sulcus.
 The syndrome consists of:
 shoulder pain
 C8-T2 radicular pain
 Horner syndrome
 The classical syndrome is uncommon, with the Horner
syndrome present in only 25%.

30.  Hypertrophic osteoarthropathy is characterised by
periosteal reaction without an underlying bone lesion
involving the diaphysis and metadiaphysis of the long
bones of distal extremities.
 Clubbing of the fingers is seen most commonly in
patients with lung, liver, and gastrointestinal disorders.
 When associated with a pulmonary condition it is
termed hypertrophic pulmonary osteoarthropathy
(HPOA) and when associated with cancer is
considered a paraneoplastic syndrome.

31. Differential diagnosis
 General imaging differential considerations include:
 pachydermoperiostosis
 chronic venous insufficiency
 thyroid acropachy
 hypervitaminosis A

33. Ganglioneuromas
 Ganglioneuromas are benign tumors of the
sympathetic nervous system, most commonly found in
the posterior mediastinum
 They are typically homogeneously enhancing round or
oval masses which can contain punctate calcifications.

34.  Ganglioneuromas are tumors of the sympathetic nervous system that
originate from neural crest cells. Along with neuroblastomas and
ganglioneuroblastomas, ganglioneuromas are collectively known as
neurogenic tumors.
 These three are differentiated only by their stage of neuroblast
maturation. Ganglioneuromas are composed of mature ganglion cells and
are considered benign tumors. Ganglioneuroblastomas and
neuroblastomas are less mature and are considered more aggressive and
dangerous.
 The posterior mediastinum is the most frequent site of occurrence (38% of
cases), followed by the retroperitoneum. Tumors located in the central
nervous system are rare. Ganglioneuromas usually occur in adolescents
and young adults (60%) but patients of all ages can be affected. The mean
age of occurrence is 7 years. Patients are usually asymptomatic and these
lesions are typically discovered on routine radiographs,

35.  On imaging, ganglioneuromas are well defined masses which range
in appearance from round to lobulated. They show calcifications in
40% of cases and tend to grow around, rather than displace, adjacent
blood vessels.
 Tumors with intermediate to high signal intensity on T2 weighted
images have a higher degree of cellularity and more collagen,
whereas markedly high T2 signal signifies a high myxoid stroma
component and low cellularity.
 They also have characteristic curvilinear bands of low intensity on T2
weighted sequences, giving a whorled pattern to the tumor as a
result of intertwined schwann cells and collagen fibers.
 Ganglioneuromas homogeneously enhance.

38. Another case of right lower posterior Ganglioneuroma vs
plexiform neuroibroma

40. Multiple myeloma ribs

41. Back pain

43. Multiple myeloma

47. 1 yr post
treatment good
response

48.  IMPRESSION: Extensive sites of extramedullary soft tissue
plasmacytoma including bilateral lung pleura, bilateral mediastinum
including intensely in the inferior wall right atrium, head of the
pancreas, large tumor extending from the posterior wall of the
descending colon, lower para-aortic retroperitoneal, 2 mesenteric
nodules, large preaortic tumor beginning at the level of the
bifurcation extending inferiorly midline/left of midline into the lower
pelvis supra-vesicle region, bilateral pelvic sidewalls and
periacetabular regions, deep subcutaneous soft tissue nodule in the
right buttock.

50. Sickle cell disease patient 2
years apart

51. www.postersession.com
Pleural fibromatosis is a rare neoplasm of the pleura categorized as
a desmoid-type fibromatosis. Pleural fibromatosis is considered a
benign tumor with aggressive growth but no potential for metastasis.
Sickle cell disease has been associated with several malignancies
such as Wilms’ tumor, leukemia, lymphoma, and lung carcinoma (1).
Pleural fibromatosis has not been associated with sickle cell disease
to date.
Case
Conclusion
Pleural Fibromatosis and Sickle Cell Disease
Rebekah Anders, JMS; Abdullah Kutlar, MD; Paul Biddinger, MD; Jayanth
Keshavamurthy, MD.
Augusta University – Medical College of Georgia, Augusta, GA
References
Pleural fibromatosis is a rare neoplasm of the connective tissue. It
is also known as a desmoid tumor and is locally aggressive but
lacks metastatic potential. We present a case of a patient with
sickle cell disease with pleural fibromatosis. The patient’s
hydroxyurea therapy has been intermittent between 2011-present
due to pregnancy.
Genetics:
• The etiology of the majority of desmoid tumors is a sporadic
activating mutation of the β-catenin or loss of function of the APC
gene.
• These mutations block the breakdown of β-catenin and leads to
its accumulation within the nucleus.
• Excess β-catenin within the nucleus will result in activation of the
Wnt pathway and excessive cell proliferation.
• 70-80% of desmoid tumors will be positive for β-catenin and
negative for desmin and S100. Other genetic causes of desmoid
tumors include trisomies of chromosomes 8 and 20.
Pathology:
• Histopathology reveals bland spindle cells in bundles in a
collagenized background surrounded by small blood vessels and
a few chronic inflammatory cells.
• Mild nuclear atypia may be present with a few distinct nucleoli.
• The lesions are ill-defined and infiltrate the surrounding soft
tissue (2).
Demographics:
• There is an increased incidence of desmoid tumors in familial
adenomatous polyposis, but no connection between pleural
fibromatosis and sickle cell disease has been made to date.
• Desmoid tumors affect an equal number of males and females
within the pediatric population and occur outside the abdomen.
• Patients with desmoid tumors between the ages of puberty to
forty tend to be female and occur within the abdominal wall, most
commonly from the internal oblique and rectus musculature and
connective tissue, or in the pelvis.
• After the age of forty, desmoid tumors occur equally in males and
females and tend to occur outside the abdomen.
• The most common extra-abdominal locations are the chest wall,
shoulder, back, thigh, and buttock regions (3).
• At times, desmoid tumors may arise from previous thoracotomy
sites and could be the source of the pleural fibromatosis in our
patient.
Presentation and Prognosis:
• Most desmoid tumors will cause minimal pain, and patients will
present with a mass.
• Despite surgical resection attempts, many desmoid tumors will
recur independently of complete resection.
Introduction Imaging
1.Schultz, W. H., & Ware, R. E. (2003). Malignancy in patients with sickle cell disease.
American journal of hematology, 74(4), 249-253.
2.Barnes, L., Eveson, J. W., Reichart, P., & Sidransky, D. (2005). World Health
Organization classification of tumours: pathology and genetics of head and neck
tumours. Word Health Organization Classification of Tumours: Pathology and
genetics of head and neck tumors.
3.Rock, M. G., Pritchard, D. J., Reiman, H. M., Soule, E. H., & Brewster, R. C. (1984).
Extra-abdominal desmoid tumors. J Bone Joint Surg Am, 66(9), 1369-1374.
Conflicts Of Interest
• All authors have disclosed no conflicts of interest
Figure 1 - Radiographs
A.Anteroposterior chest radiograph demonstrates a left upper and lower lobe
pleural opacity. This prompted biopsy of both lesions. The left lower lesion
was found to be a loculated pleural effusion and the left superior lesion
demonstrated aggressive fibromatosis.
B.Anteroposterior chest radiograph two years later reveals a decrease in size
of both the left upper pleural fibromatosis and left lower pleural effusion as
well as fibrotic scarring in the right costophrenic angle.
• Pleural fibromatosis is a rare neoplasm of the connective tissue.
• No association between sickle cell disease and fibromatosis
has yet been established. Further investigation of the effects of
hydroxyurea therapy on pleural fibromatosis may be warranted.
• Desmoid tumors may be fatal due to local mass effect and
involvement of vital structures despite absence of malignant
metastatic potential.
Pathology
Figure 2 – Pathology
A. Low power hematoxylin and eosin stained sections of needle core biopsy
of lesion demonstrating bland spindle cells in bundles.
B. High power hematoxylin and eosin stained sections of needle core biopsy
of lesion.
C. β-catenin immunostained section demonstrating nuclear positivity.
A B
C
A B

54. Metastatic Papillary
thyroid cancer

58. Primary Germ Cell Tumor Presenting As Anterior Mediastinal Mass - A Rare Entity!
T. Kukkadapu , S. Gupta , M. Wallace , C. Forseen , J. Keshavamurthy Augusta, GA,
Augusta, GA, Georgia .
Corresponding author's email: tarunkukkadapu@gmail.com
Extra gonadal germ cell tumors (EGCTs) are rare, representing only 2- 5% of adult
germ cell tumors.
Anterior mediastinum is the most common site of EGCTs, comprising 50–70% of
all extra gonadal tumors.
We report a case of primary mediastinal non-seminomatous germ cell tumor
presenting with complete lung collapse

59.  Primary germ cell tumors of the anterior mediastinum are unusual.
 Seminomas, embryonal carcinomas, teratocarcinomas, and
choriocarcinomas account for the majority of reported cases .
 Even less common is a histologically distinctive germ cell tumor
called an endodermal sinus tumor or a yolk sac tumor.
 A recent publication summarized 1 2 reported cases of this tumor
primary in the mediastinum (all single case reports).
 Radiologists should include germ cell tumors in their differential
diagnosis of anterior mediastinal tumors.

60.  42 year old male with no prior medical history presented to emergency
department with complaints of dyspnea on exertion, orthopnea and
pleuritic chest pain of 2 months duration.
 He had no cough, hemoptysis, fever, night sweats, weight loss, and
hypoxia at rest or other constitutional symptoms. He never smoked
tobacco.
 Chest radiograph showed complete opacification of left hemithorax. CT
imaging showed a large (10.6 cm x 7.7 cm x 5.1 cm) left side anterior
mediastinal mass with mediastinal shift to right side, causing compression
of left main stem bronchus leading to complete left lung collapse and
large left pleural effusion (Figure 1).
 Ultrasound guided drainage of hemorrhagic pleural fluid showed
lymphocyte predominance but cytology negative for any malignant cells.
 Serum AFP level is elevated and beta-HCG level is with in normal limits.
CT-guided anterior mediastinal mass biopsy showed non seminomatous
germ cell tumor (pure yolk sac type) with AFP negative in tumor cells

61.  Immunohistochemical studies demonstrated positive
staining for SALL4 and GATA3, and negative staining
for OCT 3 / 4 consistent with a yolk sac tumor.
 Findings confirmed by PET-CT which was obtained for
staging purposes which showed intensely
hypermetabolic partially necrotic large bilateral
anterior, left middle, left posterior mediastinal and left
hilar mass with extension into the left pleural space and
mildly active left supraclavicular lymph node (Figure 2).
 He is currently undergoing chemotherapy with
Vinblastine, Ifosfamide and Cisplatin.

62.  Our case illustrates the importance of considering primary
mediastinal non seminomatous germ cell tumors as a
differential diagnosis for anterior mediastinal masses in
young patients since they are associated with poor
prognosis and 40-50% overall survival rate.
 Diagnosis of these tumors is often times challenging to a
pathologist due to morphologic variability, unusual
location, less studied immunophenotypes and choice of
diagnostic technique.
 These tumors behave aggressively presenting as bulky
masses with predisposition to developing hematological
malignancies like AML - M7 type and myelodysplastic
syndromes.
 More research is needed for developing standard
guidelines for managing these rare non seminomatous
germ cell tumors.

65. Nodular pleural thickening
 Essentially all common causes of nodular pleural thickening are malignant and
include:
 metastatic pleural disease, particularly from adenocarcinomas, e.g:
 bronchogenic adenocarcinoma
 breast cancer
 ovarian cancer
 prostate cancer
 gastrointestinal adenocarcinoma
 renal cell carcinoma
 mesothelioma
 lymphoma
 invasive thymoma
 Thoracic splenosis is a rare benign cause of pleural nodularity.

67. Pathologic fracture from
sacral chordoma