retinoblastoma is the most common intraocular neoplasm in children. glial tumor of the retina and optic nerve head are considered to be congenital and are therefore classified as hamartomas. concurrent occurrence of these tumors in one eye is uncommon and by reviewing the studies, a few cases have been reported. we report a 9 years old boy with eye enucleation and concurrent occurrence of retin...

PURPOSE To investigate the role of proton MR spectroscopy in pediatric cerebellar tumor diagnosis. METHODS Single voxel pulse sequences with long echo time (135 or 270 milliseconds, voxel size 8 to 19 cm3), were used to obtain proton spectra of primary pediatric cerebellar tumors. Eleven primitive neuroectodermal tumors (patient age, 2 to 12 years; mean, 7 years), 11 low-grade astrocytomas (a...

Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) are high-grade malignant tumors typically found in children and adolescents. These tumors belong to the family of small round cell tumors and are of neuroectodermal origin. Primary Ewing sarcoma of the kidney is rare and because of that is an infrequent differential diagnosis in urologic malignancies. Renal PNET mostly presents...

Melanotic neuroectodermal tumor of infancy (MNTI) is a relatively uncommon osteolytic-pigmented neoplasm that primarily affects the jaws of newborn infants. Here, we are reporting a case of MNTI presented at the age of 12 years. The objective of this report is to review the incidence, presentation, investigation protocols, management options and outcomes.

The rare pediatric embryonal tumors retinoblastoma, medulloblastoma and neuroblastoma derive from neuroectodermal tissue and share similar histopathological features despite different anatomical locations and diverse clinical outcomes. As metabolism can reflect genetic and histological features, we investigated whether the metabolism of embryonal tumors reflects their similar histology, shared ...

The N-glycolylated ganglioside NeuGc-GM3 has been described in solid tumors such as breast carcinoma, nonsmall cell lung cancer, and melanoma, but is usually not detected in normal human cells. Our aim was to evaluate the presence of NeuGc-GM3 in pediatric neuroectodermal tumors by immunohistochemistry. Twenty-seven archival cases of neuroblastoma and Ewing sarcoma family of tumors (ESFT) were ...

Five pathologically proved melanotic neuroectodermal tumors of infancy are reported. These rare neoplasms of infancy exhibit a distinct predilection for the maxillary bone. Three tumors originated in the maxilla, one in the calvaria, and one in the cerebellar vermis. Those occurring in bone did not metastasize but were locally invasive, as reflected in their radiologic appearance. Bone erosion,...

Primitive neuroectodermal tumors (PNETs) are small round-cell tumors that often show neuroectodermal differentiation. They can be either central or peripheral. Central PNETs are derived from the neural tube predominantly involving the brain or spinal cord. Peripheral PNETs (pPNETs) are derived from the neural crest involving the sympathetic nervous system, bone, or soft tissue [1]. The most com...

primitive neuroectodermal tumor (penets) is an uncommon malignancy of bone and soft tissue witch rarely occurs in the kidney. in more than 90% of the cases, the tumor cells relieves a balanced translocation (11; 22) (q24; q12). immunohistochemical staining may be required for diagnosis of penet. the cells of tumor express cd99, vimentin, nse, fl1 but do not express ck, lca, myogenin, and wt1. w...