Broggi Giuseppe, Piombino Eliana, Altieri Roberto, Romano Chiara, Certo Francesco, Barbagallo Giuseppe Maria Vincenzo, Vigneri Paolo, Condorelli Dario, Colarossi Lorenzo, Colarossi Cristina, Magro Gaetano, Tirrò Elena
Department of Medical and Surgical Sciences and Advanced Technologies "G.F. Ingrassia", Anatomic Pathology, University of Catania, Catania, Italy.
Pathology Unit, Department of Experimental Oncology, Mediterranean Institute of Oncology, Catania, Italy.
Front Neurol. 2022 Feb 9;13:823015. doi: 10.3389/fneur.2022.823015. eCollection 2022.
It has been reported that in-frame fusions confer to glioblastomas, -wild type (GBMs, wt) some unusual morphologic features, including monomorphous rounded cells with ovoid nuclei, nuclear palisading, endocrinoid network of "chicken-wire" vessels, microcalcifications and desmoplastic stroma, whose observation may predict the molecular profile of the tumor. We herein present a case of recurrent GBMs, wt, exhibiting some of the above-mentioned morphological features and a molecularly-proven fusion. A 56-year-old man presented to our hospital for a recurrent GBM, wt, surgically treated at another center. Histologically, the tumor, in addition to the conventional GBM morphology, exhibited the following peculiar morphologic features: (1) monomorphous neoplastic cells with rounded nuclei and scant pale cytoplasm; (2) thin capillary-like vessels with "chicken-wire" pattern; (3) nuclear palisading; (4) formation of vague perivascular pseudorosettes; (5) spindled tumor cells embedded in a loose, myxoid background. Based on this unusual morphology, molecular analyses were performed and an exon17- exon 10 fusion was found. The present case contributes to widening the morphologic spectrum of -fused GBM, IDHwt and emphasizes that pathologists, in the presence of a GBM, wt with unconventional morphology, should promptly search for this fusion gene.
据报道,框内融合赋予野生型胶质母细胞瘤(GBMs,wt)一些不寻常的形态学特征,包括具有卵圆形核的单形圆形细胞、核栅栏状排列、“铁丝网”样血管的内分泌样网络、微钙化和促纤维增生性基质,观察这些特征可能预测肿瘤的分子特征。我们在此报告一例复发的野生型GBMs病例,其表现出上述一些形态学特征且存在分子证实的融合。一名56岁男性因复发的野生型GBMs到我院就诊,该肿瘤在另一中心接受了手术治疗。组织学上,除了传统的GBM形态外,肿瘤还表现出以下特殊形态学特征:(1)具有圆形核和少量淡染细胞质的单形肿瘤细胞;(2)呈“铁丝网”样的细毛细血管样血管;(3)核栅栏状排列;(4)形成模糊的血管周围假菊形团;(5)梭形肿瘤细胞嵌入疏松的黏液样背景中。基于这种不寻常的形态,进行了分子分析,发现了外显子17-外显子10融合。本病例有助于拓宽融合型野生型IDH的GBM的形态学谱,并强调病理学家在遇到具有非传统形态的野生型GBM时,应及时寻找这种融合基因。