Pediatric multifocal histiocytic sarcoma- a fatal diagnosis not to miss!

Abstract

Histiocytic sarcoma (HS) is a rare hematolymphoid malignant neoplasm with an aggressive clinical course. It can arise de novo or from low-grade B-cell lymphoma. We describe the case of a 16-year-old boy referred to our hospital with generalized lymphadenopathy, weight loss, and decreased appetite for one month. The patient died undiagnosed on the 7 th day of hospitalization. Lymph node and bone marrow biopsies were performed one day before the patient died. The lymph node biopsy revealed an architectural effacement with a diffuse proliferation of large pleomorphic neoplastic cells containing large, multilobulated nuclei, coarse vesicular chromatin, prominent nucleoli, and a moderate amount of eosinophilic cytoplasm. The bone marrow aspiration smears and biopsy also showed evidence of infiltration by these above-mentioned cells. Based on the morphology, along with the exclusion of many differential diagnoses by an extensive panel of immunohistochemical markers, a diagnosis of HS was made. This case report aims at evaluating all the clinical and immunophenotypic features of a case of HS with multifocal presentation and an aggressive clinical course in order to give a correct and definite diagnosis at the proper time.

Histiocytic Sarcoma of Bone Marrow With Partial Cohesive Neoplastic Cells and Hemophagocytosis: a Case Report

“…Histiocytic sarcoma, also formerly known as "true histiocytic lymphoma", is rare because of its prevalence rate less than 1% of all non-Hodgkin's lymphomas [4]. The differential diagnosis of HS involves various lymphomas, histiocytic and dendritic cell neoplasms, melanomas, metastatic carcinoma and pleomorphic sarcomas [5], which is confronted with challenges of misdiagnosis and requires pro cient recognition of clinic features, the atypical-morphology of tumor cell, the expression of histiocyte-associated makers.…”

“…HS has a wide patient age range from infancy to old age with median age of 52 years and a slight male predominance [2]. Clinically, HS could affect solitary organs such as lymph nodes, gastrointestinal tract, soft tissue, skin, bone marrow, spleen and even central nervous system, usually associated with other analogical hematologic malignancies of follicular lymphoma, mantle cell lymphoma and acute/chronic lymphocytic leukemia [3,4]. Given its rarity, complicated organs symptoms and histologic overlap with diverse mimics, the diagnosis of histiocytic sarcoma had been extremely challenged.…”