Melanotic Neuroectodermal Tumor of Infancy of the Upper Arm

Kirigin, Monica Stephany; Džombeta, Tihana; Seiwerth, Sven; Mesić, Marko; Giljević, Jasminka Stepan; Krušlin, Božo

doi:10.1159/000485744

Cited by 4 publications

(3 citation statements)

References 9 publications

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“…3,4 Rare, non-head and neck sites include epididymis, [5][6][7] testis, 8,9 ovaries, 10 and soft tissue and bones of the extremities. [11][12][13][14] The largest retrospective study 2 that reviewed 472 reported cases within the literature showed mainly maxillary involvement in 279 cases (62.2%), followed by the skull (70; 15%), mandible (35; 7%), male reproductive system (23; 5%), central nervous system (20; 4%), soft tissue (7; 1%), and female reproductive system (3; ,1%). They also determined that the median age of diagnosis was 5 months.…”

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confidence: 99%

Melanotic Neuroectodermal Tumor of Infancy

Soles¹,

Wilson²,

Lucas³

et al. 2018

Archives of Pathology &Amp; Laboratory Medicine

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Context.— Melanotic neuroectodermal tumor of infancy, albeit rare and generally regarded as benign, is an important tumor to recognize because of its rapid growth, potential for local recurrence, and small round blue cell morphology, which can lead to misdiagnosis of a malignant neoplasm. Objective.— To review its clinical presentation and immunomorphologic findings, and discuss common entities in the differential diagnosis. Data Sources.— The study involved PubMed searches, including multiple review articles, case studies, retrospective studies, selected book chapters, and University of Michigan cases. Conclusions.— Melanotic neuroectodermal tumor of infancy most commonly occurs in the bones of the head and neck region during the first year of life, but it can also present in other locations, including the central nervous system, testes, ovaries, and subcutaneous soft tissues. Histologically, it is composed of a biphasic population of cells, consisting of epithelioid melanin-producing cells and primitive neurogenic cells in a fibrocollagenous stroma. These microscopic findings, especially in small biopsies, can lead to a broad differential diagnosis that includes malignant small round blue cell tumors and malignant melanoma. Melanotic neuroectodermal tumor of infancy commonly has an infiltrative growth pattern, and anatomic constraints often lead to incomplete resection and local recurrence, requiring multiple surgical operations. Because melanotic neuroectodermal tumor of infancy can mimic a more aggressive and aggressively treated malignancy, recognition of this rare tumor is very crucial for pathologists.

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confidence: 99%

Melanotic Neuroectodermal Tumor of Infancy

Soles¹,

Wilson²,

Lucas³

et al. 2018

Archives of Pathology &Amp; Laboratory Medicine

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“…More than 500 cases of MNTI have been reported worldwide by Rachidi et al In addition to the 472 cases reported between 1918 and 2013 as a part of the largest retrospective study, [5] we found 49 cases reported between January 2014 and June 2021 by searching PubMed [3,4,9–32,36–38] . In a systematic review of 472 MNTI cases, [5] most cases developed within 1 year of age, with more than 90% of cases occurring in the craniofacial region, such as the maxilla (60.3%), skull (18.1%), and mandible (10.3%).…”

Section: Discussionmentioning

confidence: 75%

Melanotic neuroectodermal tumor of infancy in the mandible

et al. 2021

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Rationale: Melanocytic neuroectodermal tumor of infancy (MNTI) is a rare benign pigmented neoplasm that arises from the neural crest and has an aggressive growth pattern. It is predominantly seen in infants under 1 year of age, and the most common site of involvement is the maxilla. The currently accepted treatment is removal by surgical resection. Herein, we report a case of MNTI that involved the anterior alveolar ridge of the mandible in a 6-month-old infant. Patient concerns: A case of a 6-month-old male child with a huge mass in the anterior alveolar ridge of the mandible. Diagnosis: The tumor was diagnosed using histopathological and immunohistochemical techniques on the biopsy specimen obtained following incisional biopsy. Based on the findings, a final diagnosis of MNTI was established. Interventions: Radical resection of the tumor was performed, after determining the extent of resection by referring to the mandibular 3D model created using the pre-operative CT data. Outcomes: The postoperative course was uneventful, and no recurrence has been observed to date for more than 4 years after surgery. Lessons: This case emphasizes that early diagnosis and radical surgery are critical to the effective treatment, as MNTI exhibits rapid and destructive growth. It also requires careful and close follow-up because of high recurrence rates.

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“…The tumor is localized most often in the head and neck region, with maxilla (62.2%), skull (15.6%) and mandible (7.8%) being the most common anatomical sites [1]. Localizations of MNTI within epididymis, extremities, ovary and endolymphatic sac have also been reported [3][4][5][6][7][8][9][10].…”

Section: Introductionmentioning

confidence: 99%

The Role of Chemotherapy in Management of Inoperable, Metastatic and/or Recurrent Melanotic Neuroectodermal Tumor of Infancy—Own Experience and Systematic Review

Krawczyk

Brecht

Haug

et al. 2021

Cancers

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Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a very rare pediatric neoplasm of neural crest origin. In most cases, it develops in infants as a localized tumor of the maxilla, and surgery is usually curative. In less than 10% of patients with inoperable, metastatic or persistently recurring MNTI, chemotherapy (CHT) may be considered; however, its role is still unclear. The aim of our study was to assess the efficacy of CHT in children with large, inoperable, metastatic and/or recurrent MNTI. Four such infants, treated with CHT in Polish and German centers of pediatric oncology, were presented. Additionally, a systematic literature search of the PubMed/MEDLINE, Scopus and Web of Science databases was performed, yielding 38 similar cases within the last 42 years. Neoadjuvant CHT, based mainly on the protocols for neuroblastoma, was often effective, allowing for complete delayed surgery in most cases. However, the role of adjuvant CHT in preventing recurrences after incomplete resection of MNTI remains unclear. Disseminated inoperable MNTI was almost universally associated with poor response to CHT and unfavorable outcome. Further investigations to elaborate standards of management in patients with inoperable, metastatic or persistently recurring MNTIs are necessary to improve outcomes.

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Melanotic Neuroectodermal Tumor of Infancy of the Upper Arm

Cited by 4 publications

References 9 publications

Melanotic Neuroectodermal Tumor of Infancy

Melanotic Neuroectodermal Tumor of Infancy

Melanotic neuroectodermal tumor of infancy in the mandible

The Role of Chemotherapy in Management of Inoperable, Metastatic and/or Recurrent Melanotic Neuroectodermal Tumor of Infancy—Own Experience and Systematic Review

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