Indeterminate Dendritic Cell Tumor (IDCT) of GI Tract, Lymph Node and Spleen Masquerading as Refractory Inflammatory Bowel Disease

Abstract Introduction/Objective Indeterminate dendritic cell tumor (IDCT) is an extremely rare neoplasm, most frequently presenting in skin with cells resembling precursors to Langerhans cells. We report an exceptional case of IDCT occurring in GI tract, spleen, and lymph nodes of a 66-year-old lady...

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Bibliographic Details
Published in:American journal of clinical pathology 2020-10, Vol.154 (Supplement_1), p.S108-S108
Main Authors: Huang, W, Bayerl, M G
Format: Article
Language:English
Subjects:
Biopsy
Cell differentiation
Chromatin
Colon
Crohn's disease
Cyclin D1
Cytoplasm
Dendritic cells
Diarrhea
Gastrointestinal tract
Histiocytosis
Ileostomy
Immunohistochemistry
Inflammatory bowel disease
Inflammatory bowel diseases
Intestine
Langerhans cell histiocytosis
Langerhans cells
Leukocytes (eosinophilic)
Leukocytes (neutrophilic)
Lymphadenopathy
Lymphatic system
Lymphocytes
Mimicry
Ostomy
Patients
Spleen
Tumor cells
Ulcerative colitis
Ulcers
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Summary:Abstract Introduction/Objective Indeterminate dendritic cell tumor (IDCT) is an extremely rare neoplasm, most frequently presenting in skin with cells resembling precursors to Langerhans cells. We report an exceptional case of IDCT occurring in GI tract, spleen, and lymph nodes of a 66-year-old lady, mimicking inflammatory bowel disease (IBD). Methods Four years prior to the diagnosis of IDCT, she was diagnosed with ulcerative colitis (UC) based on bloody diarrhea and pan-colitis. Her colitis became refractory to medical treatments and she developed pancytopenia, splenomegaly and abdominal lymphadenopathy. A FNA of abdominal lymph node and a colonoscopic biopsy both showed non-necrotizing granulomas, which along with her multiple oral ulcers suggested Crohn’s disease. Due to failure to medical treatments, she underwent proctocolectomy and ileostomy, followed later by subtotal colectomy with excision of a splenule and lymph nodes. Results All specimens from the final operation showed involvement by IDCT characterized by polygonal cells with abundant eosinophilic cytoplasm, oval nuclei with occasional nuclear grooves, open chromatin and eosinophilic nucleoli. By immunohistochemistry, the tumor cells expressed S100, CD1a, cyclin D1, BRAFV600E. Langerin staining was observed in
ISSN:0002-9173
1943-7722
DOI:10.1093/ajcp/aqaa161.236