Primary pancreatic sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): an unusual extranodal manifestation clinically simulating malignancy.

Archives of Pathology & Laboratory Medicine
Mark PodberezinSujata Gaitonde

Abstract

Abstract Sinus histiocytosis with massive lymphadenopathy (SHML), also called Rosai-Dorfman disease, is a rare entity. Its etiology and pathogenesis are still essentially unclear. The histologic hallmark of this disease is proliferation of distinctive histiocytes within lymph node sinuses and in extranodal sites. Approximately 23% of patients with SHML, documented in the SHML Registry, presented with disease primarily in extranodal sites, and very few cases of SHML (<1%) involving the gastrointestinal system have been described in the literature. We report an unusual case of primary pancreatic SHML with infiltration of the process into peripancreatic, perinephric, and perisplenic adipose tissue, simulating malignancy.

References

Nov 4, 1998·Cytopathology : Official Journal of the British Society for Clinical Cytology·V Deshpande, K Verma
Nov 25, 2004·Hematology·Kenneth L McClainSteven H Swerdlow
Jul 10, 2007·Archives of Pathology & Laboratory Medicine·Sujata Gaitonde
Nov 21, 2008·Journal of Gastrointestinal Surgery : Official Journal of the Society for Surgery of the Alimentary Tract·Sean P ZivinGerard V Aranha

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Citations

Mar 19, 2013·World Journal of Surgical Oncology·Mahathi KomaragiriRonald S Chamberlain
Oct 16, 2020·The American Surgeon·Emily NoggleEvan S Glazer

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