婴儿黑色素神经外胚瘤

婴儿黑色素神经外胚瘤是一种临床上罕见的原始神经嵴来源性肿瘤,好发于上颌骨,多见于1岁以内的婴儿。临床上大部分患儿以局部缓慢增大的无痛性肿块就诊,肿块大体观察为实性,呈淡蓝色或灰黑色。大部分婴儿黑色素神经外胚瘤为良性,复发者易发生恶变,临床治疗多采取手术扩大切除方式以避免肿瘤复发。下面就该肿瘤临床表现、影像学特征、组织病理学特点、治疗、预后等方面的认识和研究进展作一综述。     

婴儿黑色素神经外胚瘤

婴儿黑色素神经外胚瘤(melanotic neurocetodermal tumor of infancy,简称MNTI)为发生于婴幼儿时期,尤其是好发于半岁以内的婴儿的一种较少见的,良性的神经源性肿瘤。 1918年Krompecher首先报告了这种肿瘤,称为先天性黑色素癌。此后,陆续有MNTI文献报告。在早期文献中,各作者将MNTI称为黑色素上皮牙瘤(melanotic epithelial odontoma)色素性畸胎瘤(pigmented teratoma)、非典型性黑色素细胞瘤(atypical melanoblastoma)、黑色素牙釉质溜(melanoctic odamantinoma),黑色素造釉细胞瘤(melanotic ameloblastoma)、色素性龈瘤(pigmented epulis)、视网膜始基瘤(retinal anlage tumor)、黑色素胚前瘤(melanotic prognoma)国际卫生组织肿瘤命名委员会正式命名为婴儿黑色神经外胚瘤。现将MNTI的临床病理综述如下:     

婴儿黑色素神经外胚瘤1例

目的探讨黑色素神经外胚瘤的临床特征及治疗方法。方法通过报告病例和文献回顾分析其组织发生、病理表现、临床特征、治疗方法及预后。结果本例患者的发病年龄、临床表现以及病理表现符合黑色素神经外胚瘤的特点。结论婴儿黑色素神经外胚瘤是罕见的肿瘤疾病,属于良性肿瘤,需经组织病理确诊,临床上需与骨肉瘤、骨巨细胞瘤等鉴别诊断,治疗方法以手术切除为主,预后较好。     

婴儿色素性神经外胚瘤的临床病理及免疫组化观察

目的 研究婴儿色素性神经外胚瘤的临床病理和免疫组化特点。方法 6例婴儿色素性神经外胚瘤行HE染色组织学观察，并对其中4例行多种抗体的免疫组化观察。结果 6例婴儿色素性神经外胚瘤中，组织学上显示大而淡染并含不等量色素颗粒的上皮样细胞和小而深染的淋巴样细胞。免疫组化显示CK、Vimentin、NSE、SY、HMB45、PCNA、CyclinD1、CyclinD1mRNA和CDK4在上皮样细胞呈阳性表达。NSE、SY和Vimentin在淋巴样细胞呈阳性或弱阳性表达，S-100在两种细胞均不表达。结论 婴儿色素性神经外胚瘤有特征性临床病理表现，它来源于神经嵴，其上皮样细胞为肿瘤的增殖成分。     

婴儿色素性神经外胚层瘤的临床病理观察

目的：结合文献探讨婴儿色素性神经外胚瘤的临床病理特征。方法：对患儿左上颌骨肿物组织标本进行HE染色,并采用免疫组化S—P法检测肿瘤细胞的CK、S-100、Syn、NF、GFAP、HMB45、Ki67的表达情况。结果：肿瘤含两种主要成份：立方形上皮样细胞和神经母细胞。上皮样细胞呈腺管、腺泡样或条索状、片状,胞浆内含（中枢神经）黑色素颗粒。神经母细胞呈巢状,细胞间有多少不等神经微丝。免疫组化显示：神经母细胞Syn（＋）,S-1130（＋）,GFAP（＋）,NF（＋）,上皮样细胞CK（＋）,Ki67（＋）阳性细胞约占5％～10％,HMB45（-）。结论：婴儿色素性神经外胚层瘤是非常罕见的肿瘤,是属于PNET类肿瘤的一种,具有神经内分泌的表现,属于良性肿瘤,但具有局部侵袭性。     

婴儿黑色素神经外胚瘤1例

婴儿黑色素神经外胚瘤１例山东省桂平县人民医院（２５２１００）韩金友山东医科大学附属医院王旭霞张立新婴儿黑色素神经外胚瘤在临床上极少见，我科遇１例，现报告如下。患者男，６个月。因右侧上颌牙龈黑色肿物一周来诊。检查：右侧上颌相当于第一乳磨牙处牙龈偏颊侧有...     

婴儿色素性神经外胚瘤的组织病理及超微结构观察

作者对３例婴儿色素性神经外胚瘤进行了超微结构和免疫组化等研究，结果表明，肿瘤呈局部浸润性生长，并主要由神经母细胞样小细胞和含色素细胞组成；超微结构示小细胞胞浆内含膜性分泌颗粒，花环状多聚核糖体，可见核分裂相，色素细胞含大量黑色素体和微丝；免疫组化结果显示两种瘤细胞对ＮＳＥ及Ｖｉｍｅｎｔ均呈阳性反应，而Ｓ－１００蛋白阴性，表明该肿瘤细胞较幼稚且生长活跃，提示其有复发和恶变的潜能。本文还对肿瘤细胞和胚胎眼杯细胞的同源性进行了讨论。     

婴儿黑色素神经外胚瘤的临床,X线及超微结构观察

作者对3例婴儿黑色素神经外胚瘤进行了临床、X线及超微结构观察。其临床特点是:①6个月内婴儿居多;②好发于上颌前部;③肿瘤生长迅速,病期较短。X线特点是骨质破坏与增生相并存。超微结构特点是具有神经轴突样结构,支持神经源性学说。手术治疗时,不但考虑根治肿瘤,还应考虑患儿术后继续生长发育。作者对该瘤的组织来源及治疗原则进行了讨论。     

婴儿黑色素神经外胚瘤一例及文献回顾

婴儿黑色素神经外胚瘤（MNTI）是一种少见的、增长迅速的神经脊来源良性肿瘤,多发生于1岁以内婴幼儿。好发部位为上颌骨前部,下颌骨的病例少见。本文报道1例发生于下颌骨并行手术治疗的MNTI病例,并通过文献回顾探讨MNTI的病例特点及诊断治疗要点。口腔婴儿MNTI具有典型的发病部位与发病年龄,临床检查时应注意肿物的颜色。肿瘤常生长迅速,具有局部侵袭性。影像学检查表现为边界清楚的透射影,典型的CT影像为边缘不规则的低密度团块,偶有高密度的报道。MRI的T1加权和T2加权上均表现为低信号团。病理检查常由大的并含不等量色素颗粒的上皮样细胞和小的神经母细胞样细胞组成。目前常用的治疗方法为手术切除,发生恶变及转移的可能性较低,复发率约10% - 20%,且多发生于术后6个月以内,定期随访非常重要。     

婴儿黑色素神经外胚瘤2例

婴儿黑色素神经外胚瘤（melanotic neuroectodermal tumor of infancy，MNTI）是发生于婴幼儿期的罕见肿瘤，自1918年至今报道300余例。我科收治2例，现报道如下。     

Melanotic neuroectodermal tumor of infancy (MNTI) of the head and neck: A French multicenter study

Objectives

Melanotic neuroectodermal tumor of infancy (MNTI) of the head and neck is a rare entity with uncertain clinical behavior. Radical surgical resection is the current recommended treatment, however this can cause severe aesthetic and functional sequelae. The aim of this study was to clinically characterize MNTIs and to stratify risk factors that may influence locoregional recurrence.

Melanotic neuroectodermal tumor of infancy: A narrative review of dental aspects

Melanotic neuroectodermal tumors of infancy (MNTI) are a rare type of benign tumor that affects the head and neck region. MNTI represents (68–80%) of the tumors in the maxillary region. This tumor is usually located in the alveolar crest, skull, mandible, and brain. Although this tumor is considered benign, it can grow rapidly, with a high risk of recurrence and interference with functions of infancy, such as feeding and breathing. It is also frequently harmful to the surrounding soft and hard tissues or adjacent sensitive vital structures. This study aimed to review the pathological, clinical presentation, and treatment of melanotic neuroectodermal tumors in infancy and the role of dentists in these cases.     

Recurrent melanotic neuroectodermal tumor of infancy: a proposal for treatment protocol with surgery and adjuvant chemotherapy

The case of a 4-month-old male infant treated with combined surgery and chemotherapy for an aggressive recurrent melanotic neuroectodermal tumor of infancy (MNTI) on the top of the alveolar process of the mandible with a long-term follow-up is presented. Initial treatment comprised conservative local excision and curettage of the mandible. After several local recurrences and because radical surgical excision would give gross functional and aesthetic mutilation, finally complete, long-lasting remission was achieved with adjuvant chemotherapy, according to a neuroblastoma protocol (10-year follow-up). The reason for this protocol was because molecular genetic studies of this tumor showed loss of heterozygosity of chromosome 1p and gain of chromosome 7q analogue to neuroblastomas. A combination of surgery and chemotherapy should be the preferred treatment in case of a recurrence MNTI because optimal functional and aesthetic outcome.     

The melanotic neuroectodermal tumor of infancy. Report of two cases and review of the literature

The melanotic neuroectodermal tumor of infancy (MNTI) is a rare childhood neoplasm with an alarming but classical clinical presentation. It appears as a rapidly enlarging mass in the jaws of skull of infants and unless MNTI is considered in the differential diagnosis, the lesion can easily be mistaken for a malignant neoplasm. Although possessing an aggressive growth rate and radiographic appearance, the MNTI almost always behaves in a benign fashion and can be treated with local excision. However, recent reports of malignant behavior as well as of occasional recurrences make close follow-up important. Approximately 139 cases of the MNTI have been reviewed and tabulated with respect to age at discovery, sex, tumor location, length of follow-up, and whether recurrence occurred, 2 additional cases, 1 of which was in the mandible, are presented along with theories of origin and recommended therapy.     

Melanotic neuroectodermal tumor of infancy: evidence for multicentricity

The melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that is believed to be derived from cells of the neural crest. This case demonstrates a multicentric distribution of the MNTI. The possibility of a multicentric variant of benign MNTI is discussed with respect to both the present case and previously described reports. Suggestions for evaluation and surgical management are given.     

Ultrastructural characteristics of a cell line derived from a melanotic neuroectodermal tumor of infancy

Thin section and freeze-fracture transmission electron microscopy were used to examine and identify the cytoplasmic and membrane structures in a cell line derived from a melanotic neuroectodermal tumor of infancy (MNTI). The cultured cells had a uniform appearance after 70 population doublings characterized by long dendritic processes and evidence of melanin production. The cytoplasm contained numerous melanosomes in various stages of development, vesiculated rough endoplasmic reticulum, microfilaments and uncoated as well as coated vesicles. The membrane specializations included caveoli, coated pits, gap junctions, microfilaments, desmosome-like structures and lamellipodia. The ultrastructural appearance of the cultured MNTI cells was similar to features previously seen in electron micrographs of MNTI tumor specimens. However, correlated freeze-fracture and thin section micrographs permitted further identification of structures previously described. The MNTI cell line represents one of the cell types of the tumor and provides an opportunity for further study of the pathogenesis of this rare tumor.     

Melanotic neuroectodermal tumour of infancy: Refining the surgical approach

Melanotic neuroectodermal tumours of infancy (MNTI) are particularly rare and although predominantly benign, are infiltrative and locally aggressive. Presenting in the first year of life, prompt diagnosis and effective management are critical in minimizing morbidity and the risk of recurrence.A retrospective review of 11 MNTI managed at Great Ormond Street Hospital (GOSH) from 2000 to 2017 was undertaken. Eight tumours presented in the maxilla, two in the skull and one in the mandible. The primary modality of treatment was surgery in 10 cases with one patient receiving neoadjuvant chemotherapy. In spite of microscopically incomplete resection in seven cases, only three recurred. Overall, there was a local recurrence rate of 27% with no distant metastases noted.Disease-free survival was 100% with a follow-up ranging from 0.75 to 17 years (median 5 years). Taking our results in conjunction with the available literature, there is a role for conservative initial surgery of MNTI and this should be coupled with delayed reconstruction and intensive short-term follow-up. We propose an adapted treatment algorithm that aims to balance the risk of recurrence and malignant change with surgical morbidity in an infant population.