Small bowel (small intestine) - Follicular dendritic cell sarcoma

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Small bowel (small intestine)

Other malignancies

Follicular dendritic cell sarcoma

Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 22 December 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Rare tumor, first described in 1986 (Am J Pathol 1986;122:562)
● Usually presents as painless, indolent mass (occasionally pain but without constitutional symptoms)
● May be associated with Castleman’s disease-hyaline vascular type
● Need immunostains for diagnosis

Sites
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● Usually lymph nodes, predominantly cervical, axillary or mediastinal (Am J Hematol 1998;59:161)
● Extranodal sites include oral cavity, spleen, liver, small intestine, pancreas, peritoneum, soft tissue, skin

Poor prognostic factors
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● Large tumor size (6 cm or more), intraabdominal location and coagulative necrosis (Cancer 1997;79:294)

Case reports
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● 36 year old man with nausea, vomiting, diarrhea and abdominal pain (Case of the Week #81)

Treatment
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● Excision; often recurs locally, occasional distant metastases to liver or lung

Gross images
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Duodenal mass 10.5 x 10 cm

Micro description
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● Storiform arrangement of oval or spindled cells, lymphocytic infiltrate

Micro images
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Various images

CD35

Positive stains
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● CD21, CD35; occasionally S100

Negative stains
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● Desmin, smooth muscle actin, CD117/c-kit

Differential diagnosis
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● Fibroblastic reticulum cell sarcoma: vimentin+, smooth muscle actin+, desmin+, CD21-, CD35-
● Gastrointestinal stromal tumor: CD117+, CD34+, CD21-, CD35-
● Interdigitating dendritic cell tumor: S100+, vimentin+, CD21-, CD35-
● Melanoma or other sarcomas

End of Small bowel (small intestine) > Other malignancies > Follicular dendritic cell sarcoma

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