Shangpliang Darilin, Baishya Pakesh, Raphael Vandana, Dey Biswajit, Jagtap Vikas Kantilal
North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences (NEIGRIHMS), Department of Pathology. Shillong, India.
North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Department of Radiotherapy and Oncology. Shillong, India.
Autops Case Rep. 2020 May 6;10(2):e2020170. doi: 10.4322/acr.2020.170.
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 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.
组织细胞肉瘤（HS）是一种罕见的血液淋巴系统恶性肿瘤，临床病程侵袭性强。它可原发发生，也可由低度B细胞淋巴瘤演变而来。我们报告一例16岁男孩，因全身淋巴结肿大、体重减轻和食欲减退1个月转诊至我院。患者在住院第7天未明确诊断即死亡。在患者死亡前一天进行了淋巴结和骨髓活检。淋巴结活检显示结构破坏，大量多形性肿瘤细胞弥漫性增殖，这些细胞具有大的、多叶核，粗糙的泡状染色质，明显的核仁，以及中等量的嗜酸性细胞质。骨髓穿刺涂片和活检也显示有上述细胞浸润的证据。根据形态学表现，结合通过广泛的免疫组化标志物排除多种鉴别诊断，最终诊断为HS。本病例报告旨在评估一例多灶性表现且临床病程侵袭性强的HS病例的所有临床和免疫表型特征，以便在适当的时候做出正确明确的诊断。
