Salazar Miguel Fdo, Escalante Abril Paola Andrea, Velasco Vales María Verónica, Martínez Ruiz Celene, Gómez Apo Erick, Chávez Macías Laura G
Pathology Unit, Neuropathology Service, Mexico General Hospital, 06726 Cuauhtémoc, DF, Mexico.
Case Rep Pathol. 2016;2016:6749590. doi: 10.1155/2016/6749590. Epub 2016 May 25.
Haemangioblastomas are neoplasms of uncertain histogenesis with cellular and reticular variants advocated in current lore. Herein we describe an intriguing cerebellar specimen with unusual traits including spindle cell morphology and CD34 positivity. A thirty-nine-year old man had an infratentorial tumour discovered incidentally and resected three times. In all the instances, histopathological diagnosis was haemangioblastoma; nonetheless, he had neither physical stigmata nor family history of von Hippel-Lindau disease. By histology, the lesion was composed of areas of conventional stromal cells admixed with territories populated by short-spindled cells packed in lobules, sometimes giving the appearance of gomitoli. Immunoperoxidase-coupled reactions confirmed the expression of inhibin A, neuron-specific enolase (NSE), PS100, and CD57 but also revealed focal immunolabeling for CD34, CD99, and FXIIIa. This case highlights the potential phenotypical diversity that can be found within these neoplasms. Rather than uncertain histogenesis, it may in fact reflect multiple lines of differentiation-histomimesis-prone to adopt unusual morpho- and immunophenotypes in a subset of haemangioblastomas.
血管母细胞瘤是一种组织发生尚不明确的肿瘤,目前普遍认为存在细胞型和网状型两种变体。在此,我们描述了一个具有有趣特征的小脑标本,其具有包括梭形细胞形态和CD34阳性等不寻常特征。一名39岁男性偶然发现幕下肿瘤,并接受了三次切除手术。在所有病例中,组织病理学诊断均为血管母细胞瘤;然而,他既没有身体特征,也没有冯·希佩尔-林道病的家族史。通过组织学检查,病变由传统基质细胞区域与由短梭形细胞组成的小叶区域混合而成,有时呈现出肾小球样外观。免疫过氧化物酶偶联反应证实了抑制素A、神经元特异性烯醇化酶(NSE)、PS100和CD57的表达,但也显示了CD34、CD99和FXIIIa的局灶性免疫标记。该病例突出了这些肿瘤中可能存在的潜在表型多样性。与其说是组织发生不明确,实际上它可能反映了在一部分血管母细胞瘤中易于出现多种分化谱系——组织模仿——并采用不寻常的形态和免疫表型。