Interdigitating dendritic cell sarcoma

Interdigitating dendritic cell sarcoma: case report and literature review

RARE HEMATOLOGICAL TUMORS AND SYNDROMES , ,

P.A. Zejnalova1, А.I. Pavlovskaya1, T.T. Valiev2, and N.V. Kokosadze1

1 Clinical Oncology Research Institute, N.N. Blokhin Cancer Research Center, RAMS, Moscow, Russian Federation

2 Pediatric Oncology and Hematology Research Institute, N.N. Blokhin Cancer Research Center, RAMS, Moscow, Russian Federation

ABSTRACT

We present the literature data and the authors’ observation of interdigitating dendritic cell sarcoma (IDCS), a very rare tumor consisting of histiocytic and dendritic cells. Due to variability in clinical presentation with nodal and/or extranodal involvement and immunomorphological characteristics of this sarcoma, differential diagnosis with non-Hodgkin’s lymphomas, melanoma, follicular dendritic cell sarcoma, and Langerhans cell histiocytosis is necessary. Here, we describe a clinical case of IDCS with cervical lymph node involvement and successful treatment with a CHOP program and subsequent irradiation. No generally accepted treatment strategy exists so far. Currently used methods include surgery, irradiation, and chemotherapy, but treatment outcomes need to be optimized.

Keywords: Interdigitating dendritic cell sarcoma, diagnosis, treatment.

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