title: Giant melanotic neuroectodermal tumor of infancy (melanotic progonoma) of the head and neck: report of a malignant case.
abstract: 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. 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. 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.
authors.affiliation: Departments of 1 Paediatric Neurosurgery.|Department of Neurosurgery, University of L'Aquila, Italy.
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Nicosia
authors.affiliation: Departments of 1 Paediatric Neurosurgery.
authors.forename: Pietro
authors.initials: P
authors.lastname: Spennato
authors.affiliation: Departments of 1 Paediatric Neurosurgery.
authors.forename: Ferdinando
authors.initials: F
authors.lastname: Aliberti
authors.affiliation: Neuroradiology.
authors.forename: Daniele
authors.initials: D
authors.lastname: Cascone
authors.affiliation: Oncology, and.
authors.forename: Lucia
authors.initials: L
authors.lastname: Quaglietta
authors.affiliation: Pathology, Santobono-Pausilipon Children's Hospital.
authors.forename: Maria Elena
authors.initials: ME
authors.lastname: Errico
authors.affiliation: Department of Neuroradiology, Antonio Cardarelli Hospital.
authors.forename: Mario
authors.initials: M
authors.lastname: Muto
authors.affiliation: Division of Maxillo-Facial and ENT Oncologic Surgery, Istituto Nazionale dei Tumori, IRCCS Fondazione Pascale, Naples; and.
authors.forename: Franco
authors.initials: F
authors.lastname: Ionna
authors.affiliation: Departments of 1 Paediatric Neurosurgery.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Cinalli
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging / Q000601:surgery; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D006801:Humans; D008207:Lymphatic Metastasis; D008297:Male; D017600:Neuroectodermal Tumor, Melanotic / Q000175:diagnosis* / Q000473:pathology* / Q000628:therapy; D012888:Skull Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery
keywords: HVA = homovanillic acid; MNTI; MNTI = melanotic neuroectodermal tumor of infancy; VMA = vanillylmandelic acid; chemotherapy; endovascular treatment; melanotic neuroectodermal tumor of infancy; melanotic progonoma; oncology; preoperative embolization; skull