Interdigitating Dendritic Cell Sarcoma: A Rare Malignancy Responsive to ABVD Chemotherapy

Abstract

Interdigitating dendritic cell sarcoma (IDCS) is an aggressive neoplasm of which fewer than 25 cases have been reported in the world literature. This malignancy is difficult to diagnose because of its rarity, and because of the subtle histopathologic features that distinguish IDCS from similar tumors arising from reticular cells. To date, there exists no consensus on a standard chemotherapeutic regimen for IDCS. Patients with this malignancy have been treated with chemotherapy regimens used against non-Hodgkin's lymphomas. Responses to these regimens have been variable, but mostly unsuccessful. In this article we describe a case of IDCS occurring in a 44 year old female who presented with abdominal pain and inguinal adenopathy. Staging of the tumor with CT scan, PET scan, and bone marrow biopsy demonstrated inguinal and abdominal lymphadenopathies, a large mass encasing the small bowel, and extensive liver infiltration. Morphologic and cytochemical analysis of biopsies from the abdominal mass and inguinal node were consistent with a diagnosis of IDCS, and immunohistochemical stains of the lymph node were positive for CLA, Kp-1, S-100, while negative for CD1a, CD3, CD20, CKER, and HMB45. Treatment of this patient with ABVD chemotherapy resulted in rapid clinical improvement with a marked decrease in tumor burden after two cycles of ABVD, and a complete response after six cycles of therapy.

Interdigitating Dendritic Cell Sarcoma Following Adult Liver Transplantation: Case Report and Literature Review

“…Most treatment approaches reported for advanced disease have been primarily non-Hodgkin's lymphoma chemotherapeutic regimens, with or without adjuvant radiation [3,16], but with unpredictable response. Recently, a report of a patient with wide metastatic disease to the liver and abdomen showed complete respondse to 6 cycles of ABVD chemotherapy (adriamycin, bleomycin, vinblastine, dacarbazine) [3,16]. However, there is no standard therapy for treating this rare and aggressive malignancy.…”

Braf mutation in interdigitating dendritic cell sarcoma: a case report and review of the literature

“…5,6,33 In 3 patients high-dose chemotherapy and autologous bone marrow transplantation have been tried without any benefit, 34-36 whereas a case report has described one complete radiological response after a chemotherapy regimen for Hodgkin lymphoma (ABVD) in metastatic disease. 37 Ultimately, the most used chemotherapy combinations contain alkylating agents and anthracyclines; in this context chemotherapy combinations indicated for sarcomas such as epirubicin and ifosfamide thus appear as a reasonable option.…”

Dendritic cell neoplasms: An overview

“…Several chemotherapeutic regimens have been tried including CHOP, ABVD, DHAP, EPOCH, ICE, Cisplatin/Epirubicine, ABO, BACO, and p Lasp Vn Dn, with limited responses [27,29,30,32,42,47,79,80]. A case of IDDC presenting with inguinal and abdominal lymphadenopathies as well as extensive liver infiltration was treated successfully with ABVD [81].…”