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ArticleHead and neck pathology2025

Melanotic Neuroectodermal Tumor of Infancy: Clinicopathological Evaluation of a 10-Year Consecutive Case Series from a Tertiary Cancer Center.

Divakar Sharma, Sajid Qureshi, Nehal Khanna, Jifmi Manjali, Siddhartha Laskar, Akshay Baheti, Vasundhara Patil, Poonam Panjwani, Mukta Ramadwar

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Article in Head and neck pathology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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5 · Who and what money

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9 authors.

Divakar SharmaDepartments of Pathology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, India.
Sajid QureshiDepartment of Paediatric Surgical Oncology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, India.
Nehal KhannaDepartment of Radiation Oncology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, India.
Jifmi ManjaliDepartment of Radiation Oncology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, India.
Siddhartha LaskarDepartment of Radiation Oncology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, India.
Akshay BahetiDepartment of Radio-Diagnosis, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, India.
Vasundhara PatilDepartment of Radio-Diagnosis, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, India.
Poonam PanjwaniDepartments of Pathology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, India.
Mukta RamadwarDepartments of Pathology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, India. muktaramadwar@gmail.com.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

purposeMelanotic Neuroectodermal Tumor of Infancy (MNTI) is a rare, locally aggressive tumor with distinct pathological features and treatment paradigms commonly occurring in the head and neck region. Microscopically, it consists of a biphasic population of small neuroblast-like cells and larger melanin-containing epithelioid cells. The main purpose of this study is to characterize clinicopathological and immunohistochemical features of MNTI at a single institution and discuss challenges in the differential diagnosis.

methodsWe performed a retrospective analysis of MNTI cases diagnosed at our center during a 10-year period and discussed the differential diagnoses.

resultsEleven MNTI cases were identified. Median patient age was 5 months. Male to Female ratio was 1.75:1. Tumor distribution was in the Maxilla (n = 8), Mandible (n = 1) greater wing of Sphenoid (n = 1), and Temporal bone (n = 1). All tumors revealed classic biphasic morphology in the resection specimens. By immunohistochemistry, 9/9 (100%) cases were positive for both AE1/AE3 and HMB45 in the larger epithelioid cells and 6/6 (100%) were positive for Synaptophysin in the smaller neuroblast-like cells. One patient had unique nested areas composed of mature glial tissue. One patient who had incomplete resection was given adjuvant radiotherapy. One patient developed a solitary ipsilateral lymph nodal metastasis. Follow-up period ranged from 1 to 93 months. All the patients were alive with no evidence of disease at the last follow-up (median: 16 months).

conclusionsLack of consideration of MNTI in the differential diagnosis can lead to misdiagnosis and undue exposure to cytotoxic therapies. Awareness of the classic biphasic morphology and distinct immunoprofile of MNTI is essential.

Indexed as

Head and Neck NeoplasmsNeuroectodermal Tumor, MelanoticBiomarkers, TumorFemaleHumansInfantMaleRetrospective StudiesTertiary Care CentersBiomarkers, TumorDifferential diagnosisHead and neckMelanotic neuroectodermal tumor of infancyMNTIRare

Identifiers

PMID40278957
PMCPMC12031676

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