Jacobs Journal of Cancer Science and Research

Atypical Axillary Mass: Follicular Dendritic Cell Sarcoma within Castleman’s Disease: A Case Report and Review of Literature

*Maite Lopez
Department Of General Surgery, Clínica Asunción, Guipúzcoa,, Spain

*Corresponding Author:
Maite Lopez
Department Of General Surgery, Clínica Asunción, Guipúzcoa,, Spain
Email:lopezdeogracias@gmail.com

Published on: 2016-02-26

Abstract

Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm (0.4% of soft tissue sarcomas) originating from follicular dendritic cells. On the other hand, Castleman’s disease (CD) is a rare lymphoproliferative disorder first described in 1956 by Castleman et al. as a benign, localized enlargement of hyperplastic lymph nodes. Castleman’s disease has been found in association with FDCS in a minority of cases, suggesting that it may represent a precursor lesion. Combination of both FDCS and unicentric CD is extremely rare. As far as we know, there are no documented cases of a follicular dendritic cell sarcoma within a hyaline-vascular Castleman’s disease localized in the axilla. There is no consensus about treatment strategy in this situation; surgical resection when possible seems to be the best option, with or without complementary radiotherapy. We present the case of a 42-year-old male with a single axillary mass identified as a FDCS in a unicentric CD in the axilla.

Keywords

Introduction

Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm (0.4% of soft tissue sarcomas) originating from follicular dendritic cells; they usually present as coalescent nodal mass of variable size. Due to its rarity, most of the data on FDCS are based on case reports or small case series. Castleman´s disease has been found in association with FDCS in a minority of cases, suggesting that it may represent a precursor lesion. FDCS generally affect young to middle-aged adults, with a mean age of 43 years with no sex predilection and they usually present as painless coalescent nodal mass (60%) of variable size (1-20cm diameter). The optimal treatment for FDCS is yet to be found due to the limited experience but it has a slight recurrent and metastatic potential. Castleman’s disease (CD) is a rare lymphoproliferative disorder first described in 1956 by Castleman et al as a benign, localized enlargement of hyperplastic lymph nodes. It can occur at any age with a peak incidence in the third and fourth decades. It is divided into two groups depending on its histologic and clinical features. In 1972, Keller et al sub classified CD into a hyaline-vascular type an a plasma-cell type based on their histologic features. Some patients have a mixed form. Clinically, CD can be divided into a localized form, which refers to a unicentric (UC) disease, and a widespread disease (multicentric) (MC).