Melanotic neuroectodermal tumor of infancy frequently affects the maxilla. A communication between the oral and nasal cavities can be created by surgery. The authors rehabilitated a young patient with obturator prostheses to correct feeding. The association of the obturators with orthodontic devices provided proper maxillary growth and eruption of teeth. The outcomes were very satisfactory afte...

A 13-year-old boy presented with a primitive neuroectodermal tumor (PNET) with unusual extracranial extension. Precontrast computed tomography showed the tumor as a ring-shaped high-density area which was enhanced postcontrast, with a low-density center. Magnetic resonance imaging showed the tumor as a low-intensity area on the T1-weighted images with marked enhancement by gadolinium-diethylene...

The concept of primitive neuroectodermal tumors (PNETs) has been evolving for many years, as has been its nomenclature. A 5 year old boy presented with pain in lower cervicodorsal region and left leg. Preoperative MRI of the spine and paravertebral region revealed a hyperintense lobulated lesion extending from D1-D4 with a large intraspinal and thoracic component. A total removal of tumor was a...

The melanotic neuroectodermal tumor of infancy (MNTI) is a rare benign neoplasm of neural crest origin most commonly found in the anterior region of the maxilla. The tumor almost always develops during the first year of life, although in some cases it can be present at birth. MNTI's present as a rapidly growing, painless expansile, partly pigmented mass. They are usually unencapsulated, with a ...

We report the case of a 12-year-old girl, who consulted us with one-year history of an 8 mm nose lesion that was painless and firm upon palpation. The lesion was resected conservatively. Immunohistochemistry was in favor of a primitive neuroectodermal tumor (PNET)/Ewing's sarcoma lesion, excluding epithelial, lymphoid, and other tumors. After a second resection, our patient was referred to chem...

Primitive neuroectodermal tumors (PNETs) are presented as rare malignant neoplasms. In unusual cases, those neoplasms may arise in solid organs containing neuroendocrine cells, such as the pancreas. Herein the case of a 28-year-old patient that underwent gastroduodenopancreatectomy after the diagnosis of a huge mass (PNET) located in both head and body of the pancreas is reported. This is the 1...

Peripheral primitive neuroectodermal tumor of the chest wall is a rare malignant tumor usually occurring in children. The tumor shares a similar histology, immunohistology and cytogenetics to Ewing's sarcoma. The location of the tumor in the chest wall poses a major challenge with respect to the diagnostic workup and treatment which involves multidisciplinary management. Neoadjuvant chemotherap...

A case of primary primitive neuroectodermal tumor (PNET) of the chest wall in an adolescent is presented with imaging findings including 18F-fluorodeoxyglucose (18F-FDG) positron-emission computed tomography (PET-CT). To date, radiological description of the PNET of the chest wall has mainly been carried out by radiographic analysis, computed tomography (CT), and magnetic resonance imaging (MRI...

Melanotic neuroectodermal tumour of infancy (MNTI)/progonoma is a rare lesion affecting infants. Although it is slow growing and appears benign, it may have malignant potential. Evidently, surgery is the main stay of treatment and close follow-up is recommended for all cases. The literature shows that radiotherapy and chemotherapy may be indicated especially in cases where total surgical extirp...

Chicken beta globin locus contains four genes, two of which, rho and epsilon, are expressed from the earliest stage of primitive hematopoiesis. Here we show that the transcription of these two genes in the nucleus engages in "on/off" phases. During each "on" phase, cotranscription of rho and epsilon in cis is favored. We propose that these two chicken beta globin genes are transcribed not by co...