Case # 1

Presenter: Iffat A. Choudhry, MD

Attending: Charles D. Sturgis, MD

Diagnosis: Primary Pulmonary Mucinous Cystadenocarcinoma

Essentials of diagnosis:

No prior/subsequent evidence of primary extra pulmonary adenocarcinoma Multiloculated cystic neoplasm

Transition of normal respiratory epithelium into neoplastic epithelium (in situ change)

Complex glandular/papillary architecture with true cribriforming

Intra and extracellular mucin production

Ovarian type stroma

Differential diagnosis:

Nonneoplastic lesions:

Congenital/acquired/infectious

Neoplastic (benign):

Mucinous gland adenoma

Pleomorphic adenoma

Neoplastic (malignant):

Mucinous (colloid) adenocarcinoma

Well differentiated mucinous bronchoalveolar carcinoma

Pulmonary mucoepidermoid carcinoma

Solitary metastasis of mucinous carcinoma

References:

1. Higashiyama, M. Doi O, Kodama K, Yokouchi H, Tateishe R: Cystic mucinous adenocarcinoma of the lung: Two cases of cystic variant of mucus producing lung adenocarcinoma. Chest 1992; 101: 763-766.

2. Gaeta M. Blandino A, Scribano E, Ascenti G, Minutoli F, Pandolfo I: Mucinous cystadenocarcinoma of the lung: CT-pathology correlation in three cases. J Comput Assist Tomogr 1999;23:641-643.

3. Dixon AY, Moran JF, Wesselius LJ, McGregor DH: Pulmonary mucinous cystic tumor: Case report with review of the literature. Am J Surg Pathol 1993; 17: 722-728

4. Devaney K, Kragel P, Travis W: Mucinous cystadenocarcinoma of the lung: A tumor of low malignant potential (abstr). Am J Clin Pathol 1989; 92: 524

5. Gowar, Sambrook FJ: Thorax: An unusual mucous cyst of the lung. British Medical Assn., London 1978 Dec; 33(6): 796-9

Case # 2

Presenter: Lena Kubba, D.O.

Attending Pathologist: Robert Goldschmidt, M.D.

Diagnosis: Gangliocytic Paraganglioma

Differential Diagnosis:

Carcinoid tumor Ganglioneuroma Neurofibroma

Paraganglioma Schwannoma Gastrointestinal stromal tumor (GIST)

Key Morphologic Features:

Gross: sessile or pedunculated polypoid submucosal lesions, frequently with an ulcerated mucosal surface; Not encapsulated; Infiltrative pattern; Range 2 - 13 cm

Sites of Tumor: ~95% arise in 2^nd part of duodenum/Ampulla of Vater, rarely reported in 3^rd and 4^th part of duodenum, jejunum, ileum, pylorus, bronchus, nasopharynx, appendix

Microscopic: Exhibits features of ganglioneuroma, paraganglioma, and carcinoid tumor.

3 cell components in varying proportions: spindle cells, ganglion and ganglion-like cells, and epithelioid cells

Immunohistochemistry:

EPITHELIOID CELLS: chromogranin, synaptophysin, neurofilament protein, NSE, PP, somatostatin, gastrin, VIP, serotonin, leu-enkephalin, insulin, glucagons

GANGLION CELLS: NSE, neurofilament protein, synaptophysin, leu-enkephalin, PP, somatostatin, glucagons, S-100 protein

SPINDLE CELLS: S-100 protein, neurofilament protein, NSE, synaptophysin

Treatment and Outcome: Follows a benign course. With complete excision, surgical therapy should be curative. Regional lymph node metastases in a few cases.

References:

1. Kee A, Forrest CH, Brennan BA, Papadimitriou JM, Glancy RJ. Gangliocytic Paraganglioma of the Bronchus: A Case Report with Follow-up and Ultrastructural Assessment. Am J Surg Pathol 2003;27:1380-1385.

2. Sundarajan V, Robinson-Smith TM, Lowy AM. Duodenal Gangliocytic Paraganglioma with Lymph Node Metastasis: A Case Report and Review of the Literature. Arch Pathol Lab Med. 2003;127:e139-e141.

3. Girgis PA, Henthorne WA. Mass at the Ampulla of Vater in a 43-Year-Old Man. Arch Pathol Lab Med. 2002; 126: 1239-1240.

4. Sinkre P, Lindberg G, Albores-Saavedra J. Nasopharyngeal Gangliocytic Paraganglioma: A Case Report with Emphasis on Histogenesis. Arch Pathol Med. 2001;125:1098-1100.

5. Sakhuja P, Malhotra V, Gondal R, Dutt N, Choudhary A. Periampullary Gangliocytic Paraganglioma. J Clin Gastroenterol 2001;33(2):154-156.

6. Dookhan DB, Miettinen M, Finkel G, Gibas Z. Recurrent Duodenal Gangliocytic Paraganglioma with Lymph Node Metastases. Histopath 1993;22:399-401.

7. Burke AP, Helwig EB. Gangliocytic Paraganglioma. Am J Clin Pathol 1989;92:1-9.

Case # 3

Presenter: Jason R. Weiss, D.O.

Diagnosis: Serous Cystic Tumor of Borderline Malignancy

Differential Diagnosis:

Carcinoma of the Rete Testis

Mesothelioma

Serous cystadenoma

Serous cystadenocarcinoma

Common Clinical Feature:

Palpable scrotal or testicular mass; there may be an associated hydrocele

Key Morphologic Features:

A cystic lesion lined by pseudostratified serous epithelial cells with micropapillae.

Epithelial cells with mild atypia and prominent nucleoli.

Cilia are present.

No invasion is identified

Ancillary Studies:

Special Stains: Positive cytokeratin 7 (CK7), estrogen Receptor (ER), progesterone receptor (PR), CD15/LeuM1 staining. Negative cytokeratin 20 (CK20), carcinoembryonic antigen, calretinin, and HER-2/neu staining.

Treatment:

Complete excision

References:

1. McClure RF, et al. “ Serous Borderline Tumor of the paratestis: a Report of Seven Cases.” American Journal of Surgical Pathology 25(3);373-8, 2001

2. Young RH, Scully RE. “Testicular and Paratesticular Tumors and Tumor-Like Lesions of Ovarian Common Epithelial and Mullerian Types.” American Journal of Clinical Pathology 86:146-52, 1986.

3. Remmele W, et al. “ Serous Papillary Cystic Tumor of Borderline Malignancy with Focal Carcinoma Arising in Testis; Case Report with Immunohistochemical and Ultrastructural Observations.” Human Pathology 23(1):75-9, 1992

4. Ulbright TM, Amin MB, Young RH. Armed Forces Institute of Pathology Atlas of Tumor Pathology: Tumors of the Testis, Adnexa, Spermatic Cord and Scrotum. Washington, DC: AFIP Press, 1999.

Case #4

Presenter: Lin Liu, M.D.

Diagnosis: Gastric Schwannoma

Differential Diagnosis:

Gastrointestinal stromal tumor (GIST)

Leiomyoma

Neurofibroma

Ganglioneuroma

Common Clinical features:

4% of GI stromal tumors

2-11 cm well circumscribed tumors that are confined to the muscularis propria.

Female more than male.

Benign clinical course.

Key Morphologic Features:

Prominent peripheral lymphoid cuff.

Ancillary Studies:

Positive for S-100, and GFAP

Negative for CD 117, CD34, actin and desmin.

References:

1. Daimaru Y. et al. “Benign Schwannoma of the Gastrointestinal Tract: A

Clinicopathologic and Immunohistochemical Study” Hum Pathol 1988,19; 257-64

2. Sarlomo-Rikala M. et al “Gastric Schwannoma-a clinicopathological analysis of

six cases’ Histopathology 1995, 27, 355-360

3. Prevot S. et al. “Benign Schwannoma of the Digestive Tract” Am J Surg Pathol

23(4): 431-436, 1999.

4. Rudolph P. et al. “Gastrointestinal Mesenchymal Tumors - Innunophenotypic

Classification and Survival Analysis” Virchows Arch 2002, 441: 238-248.

5. Miettinen M. et al. “Gastrointestinal Stromal Tumors (GISTs): Definition,

Occurrence, Pathology, differential diagnosis and Molecular Genetics” Pol J

Pathol 2003, 54, 1, 3-24.

6. Lasota J. et al. “Evaluation of NF2 and NF1 Tumor Suppressor Genes in Distinctive

Gastrointestinal Nerve Sheath Tumors Traditionally diagnosed as Benign

Schwannomas: A Study of 20 Cases” Laboratory Investigation 2003, vol. 83, No 9,

p.1361-1371.

Case #5

Presenter: Monica Goswami, MD

Attending: C Hall

Diagnosis: Interdigitating Dendritic cell Sarcoma

Differential Diagnosis:

Follicular dendritic cell sarcoma

Malignant spindle cell neoplasms

Histiocytic sarcoma

Langerhan’s cell histiocytosis

Key Morphologic features:

Spindle cell proliferation in T zone of lymph nodes with areas of preserved architecture. Architecture is whorled or storiform. Cells demonstrate eosinophilic to amphophilic cytoplasm. Nuclei are indented with vesicular chromatin and prominent nucleoli.

Immunohistochemistry:

S100 strong positive, CD68 focal positive.

Treatment:

Excision, chemotherapy with CHOP regimen or surgical excision.

Reference:

1. Erich M Gartner et al. Interigitating dendritic cell sarcoma, a report of four cases. Hematopathology. 2001; 115:589-597
2. Shigeo Nakamura et al. Interdigitating cell sarcoma: A morphological and immunologic study of lymph node lesions in four cases. Pathology International 1994; 44: 374-386
3. N Barwell et al Interdigitating dendritic cell sarcoma of salivary gland associated lymphoid tissue not associated lymphoid tissue not associated with HHV-8 or EBV infection. J Clin Pathol 2004; 57-87-89
4. S A Pileri et al Tumours of histiocytes and accessory dendritic cells. Histopathology 2002, 41,1-29.
5. M.J Olnes et al Interdigitating Dendritic Cell Sarcoma: A rare malignancy responsive to ABVD therapy. Leukemia and Lymphoma, 2002, Vol. 43 (4), pp. 817-821.

Case # 6

Resident: Ajay Rawal, MD Attending: Mohamed Eldibany, MD

Diagnosis: Primary cutaneous marginal zone lymphoma with extreme plasmacytic differentiation

Clinical features:

• Primary cutaneous marginal zone lymphoma constitutes ~25% of all primary cutaneous B-cell lymphomas
• Most patients present with nodules/plaques on head and neck or trunk
• Median age: 55 years; predominate in females

Histology:

• Normal epidermis; preserved Grenz zone
• Dense heterogenous lymphoid infiltrate- “top” or “bottom” heavy
• Marginal zone cells infiltrate; hyperplastic germinal centers that may be colonized by marginal zone cells.
• Plasmacytic differentiation, often monotypic, is common; may be extreme on rare occasions, like in the index case

Immunophenotype:

• CD 20+, bcl2+, Ig chain restriction, CD5-, CD10-, bcl6 –

Differential diagnosis:

• Cutaneous lymphoid hyperplasia (pseudolymphoma)
• Primary cutaneous follicular lymphoma
• Plasmacytoma and lymphoplasmacytic lymphoma, in patients with extensive plasmacytic differentiation

Pathogenensis:

• Unknown for most cases; Borrelia burgdorferi infections associated in a subset of cases in European studies

Behavior:

• Indolent; cutaneous recurrences common; systemic dissemination and death from the disease is rare

References:

1. Connors JM, Hsi ED, Foss FM. Lymphoma of the skin. ASH Education Book 2002; 263-82.
2. Baldassano MF, Bailey EM, Ferry JA, Harris NL, Duncan LM. Cutaneous lymphoid hyperplasia and cutaneous marginal zone lymphoma. Am J Surg Pathol 1999; 23:88-96
3. de Leval L, Harris NL, Lontine J, Ferry JA, Duncan LM. Cutaenous B-cell lymphomas of follicular and marginal zone types. Am J Surg Pathol 2001; 25:732-41.
4. Cerroni L, Signoretti S, Hofler G et al. Primary cutaneous marginal zone B-cell lymphoma: a recently described entity of low-grade malignant cutaneous B-cell lymphoma. Am J Surg Pathol 1997; 21:1307-15.
5. Chunmei L, Inagaki H, Kuo T et al. Primary cutaneous marginal zone B-cell lymphoma: a molecular and clinico-patholgic study of 24 Asian cases. Am J Surg Pathol 2003; 27:1061-69.
6. Kiyohara T, Kumakiri M, Kobayashi H, Nakamura H, Okhawara A. Cutaneous marginal zone B-cell lymphoma: a case accompanied by massive plasmacytoid cells. J Am Acad Dermatol. 2003; 48: 582-5
7. Goodlad JR, Davidson MM, Hollwood K et al. Primary cutaneous B-cell lymphoma and borrelia burgdorferi infection in patients from the Highlands of Scotland. Am J Surg Pathol 2000; 24:1279-85.

Case #7

Presenter: Bernard Ng, MD

Atttending: Mark Dieterich, MD

Diagnosis: Hydatid Cyst of the Spleen

Synonyms: Hydatidosis/hydatid disease – infection in humans by metacestodes

echinococcosis – infection in nonhuman carnivores by the adult tapeworm

Key Morphologic Features:

Gross:

White, spherical, fluid-filled cyst that varies from a few millimeters to many centimeters in diameter. Cyst may contain daughter cysts.

Microscopic:

Histopathologic demonstration of hydatid structures from any site is diagnostic. The laminated cyst wall is pathognomonic. Examination of the cysts may reveal daughter cysts, brood capsules, protoscolices, and hooklets; any of which are diagnostic.

Differential Diagnosis:

Epithelial cysts of the spleen, false cysts of the spleen

Clinical Features:

Variable depending on size and location of the cyst. Usually asymptomatic if small. Must have high index of suspicion especially if the patient is from an endemic area or with exposure to animal vectors. Hydatid cysts are most commonly seen in the liver and lung. Solitary splenic hydatid cysts are rare.

Ancillary Studies:

Imaging (including radiographs, ultrasound, MRI and CT), serologic tests

References:

1. Al-Mohaya S et al. Hydatid cyst of the spleen. Am J Trop Med Hyg. 1986 Sep; 35(5): 995-999.

2. Barzilai A et al. Splenic Echinococcal Cyst Burrowing into Left Pleural Space. Chest 1977 October; 72(4): 543-545.

3. Bhandarwar AH et al. Splenic Hydatidosis A Review of Literature. Bombay Hosp J 2002 October; 44(4):656-663.

4. Gregg RO and Halsey WS. Echinococcal cyst of Spleen. N Y State J Med 1973 Mar 1; 73(5): 693-694.

5. Hira PR et al. Cystic hydatid disease: pitfalls in diagnosis in the Middle East endemic area. J Trop Med Hyg 1993 Dec; 96(6): 363-369.

6. Klompmaker IJ et al. Splenic Vein Obstruction due to a Solitary Echinococcal Splenic Cyst, resulting in Gastric Fundus Varices: An unusual case of variceal bleeding. HPB Surgery 1993: 6: 229-233.

7. Laajam MA and Nouh MS. Hydatidosis: Clinical Significance and Morbidity Patterns in Saudi Arabia. East African Med J 1991; 68(1): 57-63.

8. Marty AM et al. Pathology of Infectious Diseases: Vol. 1-Helminthiases. Washington D.C.: AFIP, 2000.

9. Pedrosa I et al. Hydatid Disease: Radiologic and Pathologic Features and Complications. Radiographics. 2000; 20:795-817.

10. Singh H and Arora S. Primary Hydatid Cyst of the Spleen. MJAFI 2003; 59:169-170.

11. Wurtele LH et al. Ultrasonograpic appearance of splenic echinococcal cyst. Penn Med. 1982 October: 55-56.