Departments of Pathology, All India Institute of Medical Sciences, New Delhi, India.
Departments of Neuroradiology, All India Institute of Medical Sciences, New Delhi, India.
Neurol India. 2022 May-Jun;70(3):1254-1259. doi: 10.4103/0028-3886.349657.
Histiocytic sarcoma (HS) is an aggressive hematolymphoid malignancy that arises from non Langerhans histiocytes and usually involves the skin, lymph nodes, and intestine. The involvement of the central nervous system (CNS) is a rare occurrence with around 30 cases being reported in English literature. Morphological and immunohistochemical evidence of histiocytic differentiation is essential for diagnosis. Prognosis is very poor and consensus on treatment is not available mainly due to its rarity. We report two cases of HS with varied clinical presentation and pathological findings and elucidate the diagnostic challenges of this rare entity.
组织细胞肉瘤(HS)是一种源自非朗格汉斯细胞的侵袭性血液淋巴系统恶性肿瘤,通常累及皮肤、淋巴结和肠道。中枢神经系统(CNS)受累较为罕见,英文文献中报道约有 30 例。形态学和免疫组织化学的组织细胞分化证据对诊断至关重要。由于其罕见性,预后非常差,且治疗方法尚无共识。我们报告了两例具有不同临床表现和病理发现的 HS 病例,并阐明了这一罕见实体的诊断挑战。