Dr. Leslie Saturday Seminar Unknown Case Case History A 47 year - - PowerPoint PPT Presentation

• Dr. Leslie

Saturday Seminar Unknown Case

A 47 year old woman is found to have several lung nodules in the right lower lobe. Three (3) were excised, all with similar histopathology. Questions to consider: Is this a neoplasm? If a neoplasm is it benign or malignant? Is there any other information regarding the patient that would help you resolve 1 or 2 above? What, if any, immunohistochemical stains might you perform in resolving a definitive diagnosis?

Case History

The patient has Cowden Syndrome…

Case History

Surgery

• On excision the lesion was well

circumscribed but did not seem to have a defined capsule

• On sectioning, the lesion was tan with

areas of cystic degeneraton

• Representative sections were taken

PanCK PanCK TTF-1 TTF-1

Differential diagnosis

• Alveolar adenoma
• Carcinoid tumour
• Carcinoma, primary or metastatic
• Papillary adenoma of Type 2 cells
• Pneumocytoma
• Salivary gland tumor

DIAGNOSIS

Pneumocytoma

(Pulmonary sclerosing hemangioma)

Pneumocytoma

• A well-circumscribed but un-

encapsulated lesion

• The diagnosis is made through the

recognition of a multitude of growth patterns

Pneumocytoma

Four growth patterns are identified consistently:

1) Papillary 2) Sclerotic 3) Solid 4) Hemorrhagic

Pneumocytoma

• Two cell types occur in all lesions:
• One is a cuboidal cells that lines the

papillary structures and blood-filled cysts

Pneumocytoma

• The other is bland polygonal/round cell

with abundant pale cytoplasm with growth in solid sheets (always look in the solid areas)

Pneumocytoma

• Calcifications and ossification can occur

along with peculiar giant lamellar bodies

Immunohistochemistry

• Both the surface cells and pale round

stromal cells are positive for TTF-1 and express selected epithelial markers (EMA, surfactant apo-protein B)

• Cytokeratin is positive only in the surface

cells (negative in the round stromal cells)

Immunohistochemistry

• Pneumocytoma is the only

adult neoplasm that expresses TTF-1 in the absence of pan- cytokeratin.

Histogenesis

On the basis of the immuno- histochemistry analysis it is believed that the cells arise from primitive type 2 pneumocytes

Prognosis

• Sclerosing hemangioma is

considered a benign lesion despite a few rare cases that have been reported with regional lymph node metastases

• The presence of the lymph node

metastases did not affect prognosis

COWDEN SYNDROME

• A “PTEN-opathy” resulting from inherited

dysfunction of PTEN (Phosphatase and TENsin homolog deleted on chromosome TEN)

• “PTEN hamartoma tumor syndrome”

(PHTS) can be used to describe any patient with germline PTEN mutation regardless of phenotype.

A R T I C L E

When Overgrowth Bumps Into Cancer: The PTEN-opathies

JESSICA MESTER AND CHARIS ENG American Journal of Medical Genetics Part C (Seminars in Medical Genetics) 163C:114–121 (2013)

References

• 1.

Bardenstein DS, McLean IW, Nerney J, Boatwright RS. Cowden's disease. Ophthalmology. 1988; 95(8): p. 1038-1041.

• 2.

Dacic S, Sasatomi E, Swalsky PA, et al. Loss of heterozygosity patterns of sclerosing hemangioma of the lung and bronchioloalveolar carcinoma indicate a similar molecular

• pathogenesis. Arch Pathol Lab Med. 2004; 128(8): p. 880-884.
• 3.

Kalhor N, Staerkel GA, Moran CA. So-called sclerosing hemangioma of lung: current concept. Ann Diagn Pathol. 2010; 14(1): p. 60-67.

• 4.

Keylock JB, Galvin JR, Franks TJ. Sclerosing hemangioma of the lung. Arch Pathol Lab Med. 2009; 133(5): p. 820-825.

• 5.

Liebow AAHubbell DS. Sclerosing hemangioma (histiocytoma, xanthoma) of the lung. Cancer. 1956; 9(1): p. 53-75.

• 6.

Lloyd KM, 2ndDennis M. Cowden's disease. A possible new symptom complex with multiple system involvement. Ann Intern

• Med. 1963; 58: p. 136-142.
• 7.

Shimosato Y. Lung tumors of uncertain histogenesis. Semin Diagn Pathol. 1995; 12(2): p. 185-192.

Questions