18F-FDG PET/CT for Staging and Response Evaluation in a Rare Case of Childhood Ovarian Primitive Neuroectodermal Tumor.

ABSTRACT: Primitive neuroectodermal tumor (PNET) is an aggressive small cell malignancy. Apart from the central nervous system, they can arise from peripheral sites as well. Ovarian PNETs are extremely rare neoplasms. We here present a case of childhood ovarian PNET, where 18F-FDG PET/CT was efficiently used for staging and response evaluation.

[Lens subluxation as a sign of medulloepithelioma in 13 years boy--case report]. / Podwichniecie soczewki jako objaw nabloniaka rdzeniowego (medulloepithelioma) u 13-letniego chlopca--opis przypadku.

The paper presents the case of the 13 years old boy with the deterioration of vision acuity of the right eye, caused by lens subluxation. The condition was caused by tumour of ciliary body. Additional tests: USG, UBM, CT did not resolve diagnostic doubts. After the biopsy medulloephitelioma was diagnosed and the decision of the eye enucleation was made. Histopathological examination confirmed the diagnosis.

Primitive neuroectodermal tumor of the prostate: Case report from China.

Peripheral primitive neuroectodermal tumor (PNET) in the prostate is one of the most aggressive tumors and a rare, uncommon clinical disease entity with a very poor prognosis. We reported a case of PNET in the prostate of a 49-year-old man and diagnosed through a biopsy. The patient underwent chemotherapy followed by adjuvant external radiation therapy without cystoprostatectomy as recommended, and 2 years later there is no sign of recurrence or distant metastasis. The patient had a good recovery and satisfactory outcomes in the follow-up. The successful treatment of PNET in the prostate in our case without surgery will provide a good therapeutic regime for reference until now.

Pigmented malignant medulloepithelioma of the ciliary body.

Medulloepitheliomas are usually amelanotic. Pigmented medulloepitheliomas are unusual and only two cases have been reported. We report an additional case of pigmented malignant medulloepithelioma of the ciliary body with histopathologic correlation.

Primary epidural peripheral primitive neuroectodermal tumor of the thoracic spine.

A 25-year-old male patient presented with an extremely rare primary spinal peripheral primitive neuroectodermal tumor (pPNET) manifesting as acutely progressive paraparesis and back pain. Neuroimaging and intraoperative examination showed that the tumor was confined to the epidural space of the thoracic spine. The patient was treated successfully by gross total resection of the tumor followed by chemotherapy and local radiotherapy. The present case illustrates the unexpected occurrence and important differential diagnosis of primary epidural pPNET of the thoracic spine in young patients presenting with progressive paraparesis and back pain.

Precocious puberty because of a pancreatic neuroectodermal tumor.

Primitive neuroectodermal tumors (PNETs) are uncommon, representing approximately 1% of all sarcomas. Pancreatic PNETs have been reported only 12 times in the literature. This report presents the first case of a patient with a PNET exhibiting precocious puberty. The clinical presentation, surgical management, and pathology of this rare pancreatic PNET found in a 2-year-old girl with precocious puberty are discussed.