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1/19. Subtotal maxillectomy for melanotic neuroectodermal tumor of infancy.

    Melanotic neuroectodermal tumor of infancy is a rare pigmented neoplasm occurring in infants before 1 year of age. It is a rapidly growing tumor that most frequently affects the craniofacial skeleton. Although melanotic neuroectodermal tumor of infancy is benign in the vast majority of cases, inadequate excision, occasional multicentricity, and a small malignant potential result in a fairly high recurrence rate. On the basis of data obtained from the literature and our clinical experience, we advocate an aggressive surgical approach consisting of complete surgical excision when vital structures are not involved. Histopathologic confirmation of complete excision is mandatory to minimize the risk of recurrence and provide the patient with curative treatment and minimal morbidity.
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2/19. Melanotic neuroectodermal tumor of infancy: report of two cases and review of literature.

    Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, distinctive neoplasm containing melanin; it primarily affects the maxilla of the infants during the first year of life. Approximately 150 instances of this tumor are reported in the medical literature. Genesis of the tumor is obscure and the diagnosis is challenging for the pathologist. Two cases operated by the first author are presented, and the diagnostic features and treatment alternatives of MNTI discussed.
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3/19. Benign melanocytic tumor in infancy: discussion on a rare case and review of the literature.

    Meningeal melanocytoma is an infrequent neoplasm of the central nervous system (CNS), especially in childhood and infancy. It was first described as an entity different from pigmented meningiomas and schwannomas in 1972, and few cases have been published so far. In this article, a 5-month-old male patient with meningeal melanocytoma is presented. This midline lesion was localized in the posterior fossa and manifested by hydrocephalus. The entire dural origin and extradural growing pattern in addition to the destruction of the adjacent occipital bone were the unexpected presentations since these tumors usually tend to locate on leptomeninges and to extend into the adjacent neural compartment rather than the outside. On the other hand, this case is the only one which had identical lesions in both surrenal glands and the left renal capsule, the structures containing neural-crest-derived cells outside the CNS. The prognostic criteria, differential diagnosis and its embryological aspects are discussed with an extensive review of the related existing literature.
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4/19. Melanotic neuroectodermal tumor of infancy discovered after head trauma.

    Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that generally arises in the maxilla during the first year of life. Involvement of bones of the cranial vault or brain is extremely rare. We describe a 7-month-old black female who presented after falling out of bed onto a concrete floor. Subsequently, she developed an anterior frontal mass that enlarged over several days. Radiographs of the skull at her local hospital showed a depressed right frontal skull fracture. However, computerized tomography of the head (reviewed at our institution) revealed a slightly hyperdense extra-axial mass which crossed the anterior frontal midline, widening the metopic suture and extending into the anterior subgaleal scalp. hyperostosis of the adjacent frontal calvarium was also present. A craniotomy revealed a dark, 1.5-cm calcified epidural lesion with some features of an unusual hematoma. Microscopic evaluation revealed a chronic hematoma and MNTI. The tumor recurred within a year. MNTI should be included in the differential diagnosis of epidural and skull lesions in infants.
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5/19. Melanotic neuroectodermal tumor of infancy in the soft tissues of the arm: fine needle aspiration biopsy and histologic correlation-a case report.

    A case of melanotic neuroectodermal tumor of infancy (MNTI) presenting as a soft tissue mass in the right arm of a 6-mo female child is discussed. The mass was diagnosed by fine-needle aspiration biopsy (FNAB) and confirmed by histological examination. This communication emphasizes the characteristic cytology of MNTI and discusses its significance in the context of this tumor occurring in the soft tissues. The cytology smears were distinctive in showing a dual population of small, rounded, undifferentiated cells and larger melanin-containing epithelial-like cells. It is the identification of the latter cells in the smears which differentiates this tumor from other round cell tumors of infancy and helps in the correct diagnosis by FNAB.
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keywords = soft
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6/19. Management of melanotic neuroectodermal tumor of infancy.

    Melanotic neuroectodermal tumor of infancy is a rare congenital neoplasm involving the head and neck in young patients. The clinical assessment, histologic diagnosis, and management is reviewed, with an emphasis on different treatment alternatives in two new case reports.
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7/19. Melanotic neuroectodermal tumor of infancy in the skull: case report and review of the literature.

    BACKGROUND: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that develops during the first year of life and grows rapidly. early diagnosis and radical surgery are critical for a long-term cure. We report a rare case of MNTI in the skull and discuss the importance of the radical surgery and the long-term follow-up results. CASE PRESENTATION: We describe a case of a 4-month-old girl with an MNTI in the skull who underwent the operation 11 years ago. The mass in the frontotemporosphenoid region grew rapidly after birth. The patient underwent a craniotomy. By referring to the histological findings of frozen section during surgery, a total excision of the tumor including its adjacent hypertrophic bone was performed. The patient has remained well without evidence of recurrence or neurological abnormality for 11 years. CONCLUSION: Radical surgery for MNTI provides complete cure. According to the literature including our case, there should be follow-up for at least 2 years after surgery. Especially in cases in which tumors recur, follow-up should be for longer periods because of the possibility of its malignant change.
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8/19. December 2004: one-year-old girl with aggressive skull tumor.

    December 2004. Twelve-month old girl presented with recurrent subcutaneous lesion in the left parietal region, one year after excision of a "benign" tumor. An MRI demonstrated left temporo-parietal skull tumor infiltrating the soft tissue, surrounding craniotomy flap, and extending to the brain parenchyma. biopsy revealed biphasic neoplasm displaying nests of poorly differentiated neuroblastic cells positive for synaptophysin and pigmented cuboidal epithelioid cell positive for keratins, epithelial membrane antigen and MHB-45. In addition, some neoplastic cells were immunoreactive for synaptophysin as well as HMB-45 and epithelial markers, suggestive of their origin from a common progenitor. Interestingly, cell with the neuroblastic immunophenotype displayed 80% nuclear MIB-1 reactivity indicating that the aggressiveness of the neoplasm was confined mostly to this pattern of differentiation. The overall histological features are consistent with a rare malignant variant of a melanotic neuroectodermal tumor of infancy.
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ranking = 2.0250498594882
keywords = neoplasm, soft
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9/19. Melanotic neuroectodermal tumor in a newborn.

    Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon, fast-growing, pigmented neoplasm of neural crest origin; it primarily affects the maxilla of the infants during the first year of life. Approximately 200 instances of this tumor are reported in the medical literature. We present a case of newborn with MNTI on the left maxillary alveolar ridge. The patient underwent complete surgical resection without maxillectomy. There has been no recurrence in a follow-up period of 1 year. The diagnostic features and management alternatives of MNTI are discussed.
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10/19. Melanotic neuroectodermal tumor of infancy: systematic review of the literature and presentation of a case.

    Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, distinctive neoplasm of early infancy with rapid expansile growth and a high rate of recurrences. Most commonly the lesion affects the maxilla of infants during the first year of life, but it may also occur in the mandible, skull, brain, epididymis, and other rare locations. The origin of the tumor is the neural crest. The expansive, destructive, and rapid growth of MNTI and its effects on the surrounding tissues are the most obvious clinical features. Microscopically, large polygonal epithelioid cells resembling melanocytes, with variable deposits of melanin, and smaller neuroblast-like round cells characterize MNTI. Malignant transformation may occur. Since the first description in 1918, only 215 cases were reported up to the last extensive review in 1992. The present review supplements another 140 published cases of MNTI up to 2004, including an original case report. Clinical features, treatment alternatives, and follow-up are discussed.
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