No abstract available.
Neuroectodermal Tumor, Melanotic

No abstract available.
Neuroectodermal Tumor, Melanotic

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.
Epididymis* ; Head ; Humans ; Infant ; Male ; Maxilla ; Neck ; Neural Crest ; Neuroectodermal Tumor, Melanotic* ; Orchiectomy

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.
Biopsy ; Brain ; Humans ; Infant ; Infant, Newborn ; Jaw ; Mandible ; Maxilla ; Neuroectodermal Tumor, Melanotic ; Polyenes ; Recurrence

The melanotic neuroectodermal tumor of infancy (MNTI) is a rare osteolytic pigmented neoplasm that most frequently arises from the anterior maxillary alveolar ridge. It presents in the first few months of life, and usually follows a benign course. We present a 5-month-old girl with a melanotic neuroectodermal tumor of infancy of maxilla.
Alveolar Process ; Female ; Humans ; Infant ; Maxilla ; Maxillary Neoplasms ; Neuroectodermal Tumor, Melanotic*

The term "pineal anlage tumor" has been recently proposed and few cases have been reported. We report the first Korean case of pineal anlage tumor in a 6-year-old girl who complained of headache and vomiting for 2 months. Brain MRI revealed a well defined, lobulated, calcifying mass in the pineal region. Tumor was totally removed. Pathological examination revealed a primitive pineal parenchymal tumor with melanotic epithelial component that was similar to histologic findings of melanotic neuroectodermal tumor of infancy, so-called retinal anlage tumor and of the developing pineal gland. The tumor was composed mostly of small, undifferentiated cells, Flexner-Wintersteiner rosettes, and ganglionic differentiation. The tumor also contained the cartilage and skeletal muscle cells.
Brain ; Cartilage ; Child ; Female ; Ganglion Cysts ; Headache ; Humans ; Magnetic Resonance Imaging ; Muscle, Skeletal ; Neuroectodermal Tumor, Melanotic ; Pineal Gland ; Pinealoma ; Vomiting

The melanotic neuroectodermal tumor of infancy is a rare neoplasm arising in the first year of life. A 33-day-old female infant had an enlarged alveolar ridge on the right anterior maxilla. Intraoral examination revealed a nonulcerative swelling at the site. An intraoral radiograph showed an ill-defined radiolucency on the right anterior maxilla and displacement of primary incisors from their alveolar sockets. CT scans revealed an expansion of the surrounding bone and partial destruction of the anterior wall of the premaxilla. Histopathologic examination showed the cytoplasm of neuroblastic cells and eosinophilic, epithelioid cells frequently contained a dark brown granular pigment that stained positively to vimentin and HMB45, focally positive to NSE and cytokeratin. Four weeks after the operation, CT scans showed a rapidly growing soft tissue mass occupying right maxillary sinus encroaching to the orbit and nasal cavity. The final diagnosis was made as a malignant melanotic neuroectodermal tumor of infancy.
Alveolar Process ; Cytoplasm ; Diagnosis ; Eosinophils ; Epithelioid Cells ; Female ; Humans ; Incisor ; Infant ; Keratins ; Maxilla ; Maxillary Sinus ; Nasal Cavity ; Neuroectodermal Tumor, Melanotic* ; Neuroectodermal Tumors ; Orbit ; Tomography, X-Ray Computed ; Vimentin

The melanotic neuroectodermal tumor of infancy is a rare neoplasm arising in the first year of life. A 33-day-old female infant had an enlarged alveolar ridge on the right anterior maxilla. Intraoral examination revealed a nonulcerative swelling at the site. An intraoral radiograph showed an ill-defined radiolucency on the right anterior maxilla and displacement of primary incisors from their alveolar sockets. CT scans revealed an expansion of the surrounding bone and partial destruction of the anterior wall of the premaxilla. Histopathologic examination showed the cytoplasm of neuroblastic cells and eosinophilic, epithelioid cells frequently contained a dark brown granular pigment that stained positively to vimentin and HMB45, focally positive to NSE and cytokeratin. Four weeks after the operation, CT scans showed a rapidly growing soft tissue mass occupying right maxillary sinus encroaching to the orbit and nasal cavity. The final diagnosis was made as a malignant melanotic neuroectodermal tumor of infancy.
Alveolar Process ; Cytoplasm ; Diagnosis ; Eosinophils ; Epithelioid Cells ; Female ; Humans ; Incisor ; Infant ; Keratins ; Maxilla ; Maxillary Sinus ; Nasal Cavity ; Neuroectodermal Tumor, Melanotic* ; Neuroectodermal Tumors ; Orbit ; Tomography, X-Ray Computed ; Vimentin

Adult ; Diagnosis, Differential ; Follow-Up Studies ; Humans ; Infant ; Keratins ; metabolism ; Male ; Mandibular Neoplasms ; metabolism ; pathology ; surgery ; Melanoma ; metabolism ; pathology ; Neuroblastoma ; metabolism ; pathology ; Neuroectodermal Tumor, Melanotic ; metabolism ; pathology ; surgery ; Rhabdomyosarcoma, Embryonal ; metabolism ; pathology ; Synaptophysin ; metabolism