This document discusses several unknown thoracic imaging cases presented by different physicians. It describes the patient histories and imaging findings of each case. The cases include a 51-year-old female with epithelioid hemangioendothelioma, a 71-year-old female who developed a tracheo-mediastinal fistula from previous tuberculosis, a 34-year-old woman with generalized cystic lymphangiomatosis, a 50-year-old female with diffuse panbronchiolitis, and a 35-year-old male found to have a rib osteochondroma on CT scan. Differential diagnoses and treatment options are discussed for each unknown case.

1. NERRS Chest 2012
Unknown Cases
October 19, 2012
Terrance Healey MD
Associate Professor of Diagnostic Imaging
Director, Thoracic Imaging

2. Dr. Oliva

3. History
 51 year old female presented to ER in
respiratory distress
 CT pulmonary angiogram in ER (July 2012)

6. 2003

7. DDX Calcified nodules slowly
growing over 9 years
 Granulomas
 Hamartomas
 Osteosarcoma mets
 Thrombosed AVMs
 Obscurity

8. Epitheloid
Hemangioendothelioma
 Rare vascular tumor
 Dail & Liebow 1975
“intravascular bronchoalveolar tumor” (IVBAT)
 Lung, pleura, liver, soft tissue, bone
 Round/spindle shaped endothelial cells abundant cytoplasm
 CD31, CD34, FVIII, FLI-1
 1-15 year survival
 Intermediate hemangioma / angiosarcoma
 Pleural effusion & hemoptysis poor outcomes
 Age 35-55
 F>M
 Asymptomatic>dyspnea/cough

9. Treatment
 Variety of chemotherapeutic cocktails
have shown minimal response
 Surgery for local disease

10. Dr. Abbott

11. History
 65 year old female who has been
“coughing up rocks”

15. Additional History
 71 yr old retired pediatric ID treated for TB
when arriving in US 10 yrs earlier
 Now sputum positive for TB
 Rare late complications of TB
 Tuberculoma
 Bronchial stenosis/ broncholiths
 Broncho-esophageal fistula
 Fibrosing mediastinitis
 Not previously described tracheo-
mediastinal fistula

16. Dr. Bankier

17. History
 34 woman presents with cough, sore
throat, and low grade fever

18. Case c/o Anand S. Patel MD

24. Generalized cystic
lymphangiomatosis
 Rodenber 1828
 Rare congenital lymphatic malformation
 Dilated chyle-filled lymphatics
 May invlove all tissues (except CNS)

25. Lymphangiomatosis
 3% of mediastinal tumors
 Typically in children; rarely reported in adults
 Typically assymptomatic
 Dyspnea
 Cough
 Hemoptysis
 Dysphagia
 Hoarseness
 Gorham’s Disease: bony dissemination

26. Treatment
 Surgical resection is only proven treatment
 Complete resection in this case not possible given
location & extent of disease

27. Reference
 Wunderbaldinger P, Paya K, Partik
B, Turetschek K, Hörmann M, Horcher
E, Bankier AA. CT and MR imaging of
generalized cystic lymphangiomatosis in
pediatric patients. AJR Am J Roentgenol.
2000 Mar;174(3):827-32. PubMed PMID:
10701634.

28. Dr. Kazerooni

29. History
 50 year old female presented to the ER
with hemoptysis.
 PMHX: “COPD”, sinusitis, cough

32. You find a study from 7 yrs ago!

33. Additional History
 DDX very long
 Wegners, Churg Strauss, Ig deficiencies,
ciliary dysplasia, CF, ABPA, HIV, IBD
 Flares have been treated successfully for
7 years with Macrolide antibiotics

34. Diffuse Panbronchiolitis
 Rare clinicopathologic syndrome
 Bronchiolitis & Chronic sinusitis
 DIFFUSE= involves all lobes
 PAN= involves entire bronchiole
 Genes: HLA-B54, HLA-A11, MUC5B
deletion
 Environment: 1st noted in East Asia
 Lymphocytes
 Macrolides reduce # of lymphocytes

35. Dr. Mandell

36. History
 35 year old male presented to the ED with
“pre-syncope”

38. What next? Protocol?
 A nodule is present in the left mid-
lung, which likely contains a focus of
calcification
 If no priors are available, unenhanced
chest CT is recommended

39. HU=neg 50

40. DDX?
 A circumscribed nodule is present
containing macroscopic fat and
calcification
 The nodule abuts the anterior subpleural
surface
 Fat within a circumscribed nodule is nearly
pathognomonic of a hamartoma. DDX
includes focal lipoid pneumonia or
metastatic liposarcoma

42.  Magnified view in bone window shows that
the nodule is contiguous with the anterior
rib. There is the suggestion of marrow
continuity between the rib and the
“nodule.” The calcification is peripheral.
 This most likely represents a rib
osteochondroma. The fat attenuation is
marrow and the calcification is a calcified
cartilage cap.

43. Osteochondroma
 Cortical and medullary bone (direct
continuity) & hyaline cartilage cap
 50% bone lesions in the chest and
presents as a painless mass on external
surface of rib metaphysis
 20-50% of all benign bone tumors
 Malignant transformation < 1% (pain)

44. NERRS Chest 2012
Unknown Cases
October 19, 2012
Terrance Healey MD
Associate Professor of Diagnostic Imaging
Director, Thoracic Imaging