ABSTRACT
Abstract Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that affects mainly children under 1 year of age. A 4-month-old boy was referred for evaluation of a lesion with 1 month of evolution. Intra-oral examination detected a firm upon palpation submucosal nodular mass, measuring 1.5 cm in diameter, affecting the anterior maxillary alveolar ridge and covered by a slightly blue mucosa with evident telangiectasia. The patient underwent an incisional biopsy and histological and immunohistochemical analyses revealed nests of AE1/AE3 positive epithelioid cells with abundant melanin pigmentation. Other cell types, resembling neuroblasts, were also present and positive for CD56, synaptophysin and enolase. The diagnosis of MNTI was established and the patient was referred for treatment. Conservative surgical resection was performed along with 3 adjacent teeth under general anesthesia. The patient is in follow-up for 1,5 year without recurrence. Conservative surgical management of MNTI may be an alternative to maxillectomy, contributing to the patient´s quality of life.
Resumo Tumor neuroectodérmico melanótico da infância (TNMI) é um neoplasma raro que afeta principalmente crianças com idade abaixo de 1 ano. Um menino de 4 meses foi referenciado para avaliação de uma lesão com 1 mês de evolução. O exame intra-oral detectou uma massa nodular submucosa firme à palpação, medindo 1,5 cm de diâmetro, afetando rebordo alveolar anterior da maxila e recoberta por mucosa de coloração levemente azulada com telangiectasia evidente. O paciente foi submetido à biopsia incisional e as análises histológica e imunohistoquímica revelaram ninhos compostos por células com abundante pigmento de melanina, positivas para AE1/AE3. Outro tipo celular, semelhante à neuroblastos, também estava presente e foram positivas para CD56, sinaptofisina e enolase. O diagnóstico de TNMI foi estabelecido e o paciente encaminhado para tratamento. Ressecção cirúrgica conservadora sob anestesia geral ao longo de 3 dentes adjacentes foi realizada. O paciente está em acompanhamento há 1 ano e meio sem sinais de recorrência. O tratamento cirúrgico conservador do TNMI pode ser uma alternativa à maxilectomia, contribuindo para a qualidade de vida do paciente.
Subject(s)
Humans , Male , Infant , Neuroectodermal Tumor, Melanotic/diagnosis , Biopsy , Immunohistochemistry , Neuroectodermal Tumor, Melanotic/surgery , Neuroectodermal Tumor, Melanotic/metabolism , Neuroectodermal Tumor, Melanotic/pathology , CD56 Antigen/metabolism , Melanins/metabolismABSTRACT
O objetivo do presente artigo é relatar a abordagem odontológica com obturador palatino imediato diferenciado em um caso clínico de tumor neuroectodérmico melanótico da infância, ocorrido no Instituto Nacional de Câncer José Alencar Gomes da Silva, realizando uma revisão da literatura e abordando o atendimento multidisciplinar como garantia do cuidado integral ao paciente. Lactente, 4 meses, sexo masculino, tumoração em maxila esquerda, foi encaminhado à seção de Odontologia para confecção de obturador palatino com projeção maxilar. O paciente que é submetido a um tratamento mutilador na tentativa de cura de alguma neoplasia de cabeça e pescoço necessita de conforto, bem-estar e um cuidado integral da equipe multidisciplinar, sendo o cirurgião-dentista parte dessa equipe.
The aim of this paper is to report a dental strategy with a differentiated immediate palatal obturator in a case of melanotic neuroectodermal tumor of infancy, held at the National Cancer Institute José Alencar Gomes da Silva, by performing a literature review and addressing the multidisciplinary treatment as a guarantee of integral care for the patient. Infant, 4 months, male, tumor in the left maxilla, was referred to the Odontology section for a palatal obturator confection with maxillary projection. The patient who is submitted to a mutilating treatment in an attempt to cure any head and neck neoplasm needs comfort, wellness and integral care from the multidisciplinary team, being the dentist part of it.
Subject(s)
Palatal Obturators , Neuroectodermal Tumor, Melanotic , Neoplasms , Sex , World Health Organization , Health Services Needs and DemandABSTRACT
A melanotic neuroectodermal tumor of infancy (MNTI) is a uncommon osteolytic pigmented neoplasm that primarily affects the jaws of newborn infants. Most patients (> 90%) present with the tumor in the first year of life. Approximately 65% form in the maxilla, 11% in the mandible, 5% in the brain and elsewhere. MNTI is normally benign, but up to 15% may recur and a few have metastasized. Approximately 200 cases of MNTI have been reported but only 2 of them presented as multifocal. A case of MNTI in a 7 month old boy was encountered. The chief complaint was maxillary anterior ridge swelling. The incisional biopsy findings were MNTI. Two months after the first operation, mild swelling of another site was observed. The infant was examined periodically since undergoing two procedures with no recurrence. This case demonstrates the possibility of a multicentric MNTI. We report a multicentric MNTI with a review of the relevant literature.
Subject(s)
Humans , Infant , Infant, Newborn , Biopsy , Brain , Jaw , Mandible , Maxilla , Neuroectodermal Tumor, Melanotic , Polyenes , RecurrenceABSTRACT
Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm known by a variety of names including melanotic progonoma and retinal anlage tumor, and currently thought to be neural crest derivation. The vast majority develop within the first year of life. The behavior is generally benign, but recurrent and metastatic cases have been documented. It arises in the head and neck in over 90%, mainly in the maxilla, but is extremely rare in the epididymis. We report a case of MNTI together with a review of literature, which occurred in the right epididymis of a 6-month-old boy who presented with a painless, slowly growing, scrotal mass and was treated with radical orchiectomy.
Subject(s)
Humans , Infant , Male , Epididymis , Head , Maxilla , Neck , Neural Crest , Neuroectodermal Tumor, Melanotic , OrchiectomyABSTRACT
The melanotic neuroectodermal tumor of infancy(MNTl) is a rare, usually benign, pigmented lesion which most frequently involves the maxilla. Skull, brain, and mandible are other craniofacial sites; this anatomic region accounts for 87% of all cases. The vast majority of MNTl, nearly 95%, present in infants of both sexes within the first year of life. Most cases of MNTl follow a benign course, with a 15% propensity for local recurrence. MNTl is regarded as a primitive neuroectodermal tumor with polyphenotypic differentiation. We report a classical MNTl which occurred in left lower maxilla in 6-month old female infant. She developed an ill defined, firm swelling on the left alveolar ridge and gum during 3 weeks. Mass excision reveals ill defined, grayish-tan hard mass, measuring 2.0x1.6 cm. Microscopically, the tumor reveals irregular alveolar pattern of melanin-containing cuboidal cells and nests of small round cells. The immunohistochemical staining for melanoma-associated antigen, neuron-specific enolase and cytokeratin show positive reactions. Ultrastructural examination showed intracytoplasmic melanosome, tonofilament and desmosome.