Extraosseous Ewing’s sarcoma/primitive neuroectodermal tumor is a rare malignant tumor with poor outcome. It is mostly reported in the second decade of life with equal in males and females. It is an aggressive tumor with unavoidable multiple recurrences and relatively poor prognosis. These tumors can be easily misdiagnosed for different tumors due to lack of established diagnostic pathological ...

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare condition that occurs normally in the anterior maxilla of infants aged <1 year. The use of clinical and imaging tools for MNTI is extremely important to prevent problems with function as well as the aesthetic features in a breastfeeding child. Brazil is a multiethnic country with a poor epidemiological policy and little data to track t...

This chapter will review the current knowledge about the molecular genetic alterations associated with the initiation and progression of the most common forms of primary nervous system tumors. These include glial tumors, primitive neuroectodermal tumors (PNETs), meningiomas, and schwannomas. We will briefly review recent epidemiological data and the present histopathological classification of p...

OBJECTIVES: To characterize primitive neuroectodermal tumor of the kidney as a differential diagnosis for Wilms tumor, and to emphasize the severity of the disease. METHODS: We report the case of a patient with a diagnosis of primitive neuroectodermal tumor of the kidney who underwent nephrectomy and complete tumor resection combined with chemotherapy. RESULTS: Initially, the response of the pa...

central nervous system atypical teratoid/rhabdoid tumor during infancy is a rare, highly aggressive tumor most commonly seen in the cerebellar area. herein we describe the case of a 4-month-old baby who presented with convulsions. pathologic examination of her cerebellar mass showed an atypical teratoid/rhabdoid tumor. the patient died 5 days after surgery despite complete excision of the mass ...

TO THE EDITOR: In the April issue of the Journal of Clinical Oncology, Timmermann et al suggest that radiotherapy plays a major role in the treatment of young children with supratentorial primitive neuroectodermal tumors (stPNET) and conclude that a delay in irradiation may compromise survival. Although stPNET are undoubtedly aggressive brain tumors and results of treatments are still disappoin...

Primitive neuroectodermal tumor (PNET) arise from Kulchitsky cells and are rarely seen in the female genital tract. Differential diagnosis of PNET can be made based on immunohistochemical profiles and genetic analyses. Genital tract pNETs are very aggressive pathologies with different clinical and molecular manifestations and there are no standard guidelines for treatment. We aimed to present t...

Primitive neuroectodermal tumor in adults is extremely rare. It belongs to the Ewing’s sarcoma family that usually occurs in children and adolescents with a predilection for the truncal and axial soft tissue. Primitive neuroectodermal tumor of the posterior urethra is even less common with no prior reported cases in the Englishlanguage literature. We reported here a case of primitive neuroectod...