MELANOTIC NEUROECTODERMAL TUMOR OF INFANCY - CLINICOPATHOLOGICAL, IMMUNOHISTOCHEMICAL, AND FLOW CYTOMETRIC STUDY
MELANOTIC NEUROECTODERMAL TUMOR OF INFANCY - CLINICOPATHOLOGICAL, IMMUNOHISTOCHEMICAL, AND FLOW CYTOMETRIC STUDY
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DOI：
10.1097/00000478-199306000-00004
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发表时间：
1993-06-01
影响因子：
5.6
通讯作者：
POPEK, EJ
中科院分区：
文献类型：
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作者：
KAPADIA, SB;FRISMAN, DM;POPEK, EJ
本文报告20例婴儿黑色素性神经外胚层瘤。 患者（13名女性，7名男性）的年龄范围为1至9个月（平均5个月），通常表现为快速增长的肿块。肿瘤部位包括上颌骨（13例）、下颌骨（3例）、硬脑膜（2例）、脑（1例）和颅骨/眼眶（1例）。平均肿瘤大小为3.5 cm（范围，1.0-10.0 cm）。对12例病例进行了随访。五个肿瘤（45%）在诊断后4个月内复发，但没有转移。1例手术死亡，组织学表现独特，在较小的神经母细胞巢周围有管状或泡状的含黑色素的细胞，细胞质稀少或呈纤维状。12个肿瘤进行了免疫化学研究，肿瘤细胞角蛋白阳性12例，HMB 45阳性12例，波形蛋白阳性7例，上皮膜抗原（EMA）阳性4例，主要是大细胞。神经元特异性烯醇化酶（NSE）（7/12）和Leu 7（9/12）在小细胞和大细胞中呈阳性;一些肿瘤还表达突触素（4/12）、胶质细胞酸性蛋白（GFAP，3/12）或S-100蛋白（2/12）。嗜铬粒蛋白、结蛋白或癌胚抗原（CEA）均未染色。研究的10个肿瘤中有8个在流式细胞术（FCM）上具有可解释的结果（4个DNA二倍体，3个DNA非整倍体和1个具有突出肩部的DNA二倍体）。在5例随访病例中，有2例局部复发，我们无法证明FCM在预测复发方面的有用性。进一步的研究是必要的，以更好地确定FCM在预测攻击行为的潜在有用性。独特的形态学和多表型（上皮，神经，黑色素细胞）表达MNTI从黑色素瘤和转移性神经母细胞瘤区分。
Twenty cases of melanotic neuroectodermal tumor of infancy (MNTI) are reported. The patients (13 females, seven males), whose ages ranged from 1 to 9 months (mean, 5 months), typically presented with a rapidly growing mass. Tumor sites included the maxilla (13 cases), mandible (three cases), dura (two cases), brain (one case), and skull/orbit (one case). The mean tumor size was 3.5 cm (range, 1.0-10.0 cm). Follow-up was obtained on 12 cases. Five tumors (45%) recurred within 4 months of diagnosis, but none metastasized. One surgical death occurred.Histologic appearance was distinctive, with tubular or alveolar formations of large melanin-containing celts around nests of smaller neuroblastic cells possessing scant or fibrillar cytoplasm. Twelve tumors were studied immunohistochemically; tumor was positive for cytokeratin in 12 of 12, for HMB 45 in 12 of 12, for vimentin in seven of eight, and for epithelial membrane antigen (EMA) in four of nine tumors, mainly in the large cells. Neuron-specific enolase (NSE) (seven of 12) and Leu 7 (nine of 12) were positive in small and large cells; some tumors also expressed synaptophysin (four of 12), glial fibrillary acidic protein (GFAP, three of 12 tumors), or S-100 protein (two of 12 tumors). No staining was found for chromogranin, desmin, or carcinoembryonic antigen (CEA). Eight of 10 tumors studied had interpretable results on flow cytometry (FCM) (four DNA diploid, three DNA aneuploid, and one DNA diploid with a prominent shoulder). Tumor recurred locally in two of five cases with follow-up, and we were unable to demonstrate the usefulness of FCM in predicting recurrences. Further studies are necessary to define better the potential usefulness of FCM in predicting aggressive behavior. Distinctive morphology and multiphenotypic (epithelial, neural, melanocytic) expression distinguish MNTI from melanoma and metastatic neuroblastoma.