Giant melanotic neuroectodermal tumor of infancy (melanotic progonoma) of the head and neck: report of a malignant case

Abstract

<jats:p>Melanotic neuroectodermal tumor of infancy is a rare congenital pigmented neoplasm of neural crest origin, locally aggressive, growing rapidly and developing during the 1st year of life. It most commonly arises from the maxilla, cranial vault, and mandible. Occasionally, it exhibits malignant behavior with local lymph nodes involvement. Cases misdiagnosed and left untreated for a long time can present challenges due to the tumor mass and infiltration. In these cases, adjuvant chemotherapy can be extremely helpful before radical excision.</jats:p><jats:p>Authors of this report describe a 4-year-old boy from a developing country who was referred to their hospital with an ulcerated bulging lesion in the midline/right parietooccipital region, extending to the right laterocervical and parotid regions, resulting in significant craniofacial deformation. Magnetic resonance imaging of the brain revealed a highly enhancing tumor with intracranial and extracranial development extending mainly at the level of the right parietooccipital region, with lytic and hypertrophic alterations of the skull. The patient was managed with neoadjuvant and adjuvant chemotherapy and radically resective surgery on metastatic lymph nodes and the primary tumor of the skull. Scheduled radiotherapy was not performed, according to the parents' wishes. The patient returned to his native country where the lesion recurred, and he ultimately died approximately 10 months after the end of the treatment.</jats:p><jats:p>The literature indicates that tumor removal alone has been the treatment of choice in most isolated cases, but in cases of highly advanced tumor with involvement of the skull and cervical lymph nodes, it is preferable to proceed with preoperative chemotherapy with the aim of reducing the tumor volume, allowing better technical conditions for complete surgical removal, and decreasing the risk of local recurrence or metastasis.</jats:p>

Melanotic Neuroectodermal Tumor of Infancy

“…40 Metastatic spread has been documented to lymph nodes and the central nervous system. 3,41,42 Complete local surgical excision is considered the treatment modality of choice. 20,22 Chemotherapy and/or radiation have only been used in a limited number of cases.…”

Melanotic Neuroectodermal Tumor of Infancy: A Rare Case Report

“…The levels of VMA returned to normal following surgery and chemotherapy. The variable expression of IHC markers in different studies suggests that MNTI is a primitive neuroectodermal tumor with a polyphenotypic expression of neural and epithelial markers, and melanin production [5,9,10].…”

“…However, multiple lesions have also been reported [11]. Recurrence may occur due to the invasion of the tumor edge into the bone and difficulty in complete resection due to tumor with no envelope [8,10,12].…”

Melanotic neuroectodermal tumor of infancy: A rare case report

“…The variable expression of immunohistochemical markers in different studies suggests that MNETI is a primitive neuroectodermal tumor with polyphenotypic expression of neural and epithelial markers, melanin production, occasional rhabdomyoblastic, glial, ganglionic and osseous differentiation and no photoreceptor differentiation. [ 5 6 7 ]…”