Histiocytic Sarcoma and Tumor Lysis Syndrome: An Unexpected Sequela of Neck mass

Abstract

1. Vos, et al. “Histiocytic sarcoma: a study of five cases including the histiocyte marker CD163” Modern pathology. Jan 2005 (18)p693-704 2. De Vos, FYFL, Gerding M, Ardens J, Wegmen J. Histiocytic sarcoma localised in the thyroid—a case report. Ann Hematol 2008;87(8): 681682. 3. World Health Organization Classification of Tumours (2001) Pathology and genetics of tumours of hematopoietic and lymphoid tissue. International Agency for Research on Cancer (IARC), Lyon, pp 274–289 . 4. Cairo M, Bishop M. Tumor lysis syndrome: new therapeutic strategies and classification. British J Haematology 2004;127:3-11. 5. Gokmen Akoz A, Yildirim N, Engin H, et al. An unusual case of spontaneous acute tumor lysis syndrome associated with acute lymphoblastic leukemia: A case report and review of the literature. Acto Oncol 2007; 46(8):1190-2. The patient continued to rapidly deteriorate, developing disseminated intravascular coagulation (DIC), multi-system organ failure, and decreased neurologic function. The patient was pronounced dead early the following morning, just two days after admission. Her final pathology was consistent with histiocytic sarcoma. Histology revealed sheets of large atypical cells with abundant eosinophilic cytoplasm, large irregular nuclei and prominent nucleoli (Figure 4). Staining was positive for CD4, CD45 and CD68, and negative for B and T cell markers as well as keratin and HMB-45 (Figures 5,6). Her cause of death was felt to be spontaneous tumor lysis syndrome caused by massive tumor burden. Approximately 1/3 of cases present in lymph nodes, 1/3 in skin and 1/3 in extranodal sites, with the GI tract being most common.3 Treatment of histiocytic sarcoma consists of wide local excision when possible with adjuvant radiotherapy. Chemotherapeutic regimens have been proposed without evidence-based data.2 Patients have a very poor prognosis with poor response to therapy except in the case of very limited disease where complete excision is possible.1,2 Our case is unique in that histiocytic sarcoma does not often present as a neck mass and therefore does not typically enter into the differential diagnosis of otolaryngologists.