Primitive neuroectodermal tumors (PNETs) are a heterogeneous group of malignant neoplasms found primarily in childhood and early adulthood. In this paper, we described the case of a 64-year-old male with primary spinal PNET, successfully treated with surgery, craniospinal radiotherapy, and concurrent chemotherapy. This is the case of a 64-year-old male who presented with a 2-month history of bilateral lower extremity weakness and numbness associated with urinary and bowel incontinence. Work-up was done, and the spine's plain magnetic resonance imaging (MRI) revealed a heterogeneously enhancing intradural lesion with an extradural component at the right T9/T10 level, causing mild to moderate cord compression. The patient underwent laminectomy and gross total resection of the said tumor. Histology and immunohistochemistry were consistent with a primitive neuroectodermal tumor of the spine. The tumor recurred three months after the surgery, and the patient was then referred for radiation therapy with concurrent chemotherapy. Repeat spinal MRIs with three- to six-month intervals after treatment showed no tumor recurrence as of August 2021. Primary spinal PNETs are rarely found in adults, especially in the elderly. These tumors currently have no recommendations or guidelines regarding their management. Thus, most cases are presently being managed based on studies on children and central nervous system (CNS) PNETs. This paper presented a case of a successfully treated primary spinal PNET in the elderly. The management was primarily based on studies done on that of the pediatric population and CNS PNETs.
Neuroectodermal Tumors, Primitive ; Neuroectodermal Tumors, Primitive, Peripheral

No abstract available.
Neuroectodermal Tumors, Primitive, Peripheral*

Schwannoma (neurilemmoma) is a common, benign, nerve-sheath tumor, and has classically been described as having a biphasic histologic appearance with alternating areas of cellular (Antoni A) and loose myxoid (Antoni B) tissues. Neuroblastom-like schwannoma is an extremely rare variant, which shows giant rosette-mimicking neuroblastoma. It must be differentiated from neuroblastoma, hyalizing spindle-cell tumors with giant rosettes, peripheral primitive neuroectodermal tumors, dendritic cell neurofibroma with pseudorosette, and malignant changes of schwannoma. To the best of our knowledge, there have been no reports of neuroblastoma-like schwannoma in Korea. We herein report a case of neuroblastoma-like schwannoma, along with a review of pertinent literature.
Dendritic Cells ; Korea ; Neurilemmoma* ; Neuroblastoma ; Neuroectodermal Tumors, Primitive, Peripheral ; Neurofibroma

Neuroblastoma occurs most frequently in children. It, however, may occur in adults. The peripheral neuroblastoma in adult is a primitive neuroectodermal tumor arising from peripheral tumor arising from peripheral nerve. It is exceedingly rare and estimated less than 1% of all malig-peripheral nerve tumors. We report a case of adult peripheral neuroblastoma arising in left lower extremity, who was treated with local excision and radiation.
Adult ; Child ; Humans ; Lower Extremity ; Neuroblastoma ; Neuroectodermal Tumors, Primitive ; Peripheral Nerves

The authors presented one case of Dysembryoplastic neuroepithelial tumor revealed at a 23 years old woman with a 6 years epileptic. The histological and immunohistochemical aspect identified a “nonspecific” DNT. The authors discussed about the clinical, CT scanner, MRI and pathologic aspects and reviewed in literature
Neuroectodermal Tumors, Primitive, Peripheral ; Neoplasms, Neuroepithelial

No abstract available.
Neuroectodermal Tumors, Primitive, Peripheral* ; Ulnar Nerve*

Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/PPNET) arises from bone or soft tissue of many sites of body, but ES/PPNET, as primary lesion, in the soft tissue of neck is very uncommon. The authors experienced a case of ES/PPNET in a 17-year-old girl, which was manifested as isolated cervical mass in the soft tissue without any other suspected primary lesion or metastasis. We report the case with a brief review of the literature.
Adolescent ; Humans ; Neck ; Neoplasm Metastasis ; Neuroectodermal Tumors, Primitive ; Neuroectodermal Tumors, Primitive, Peripheral ; Sarcoma, Ewing

Child ; Family ; Humans ; Neuroectodermal Tumors, Primitive/pathology* ; Neuroectodermal Tumors, Primitive, Peripheral/pathology* ; Oropharynx

Primary intraspinal peripheral primitive neuroectodermal tumors (pPNET) are extremely rare. We report a 10-month-old girl presented with rapidly progressive paraparesis and raised cerebrospinal fluid protein. A magnetic resonance image demonstrated an intramedullary mass from C3 to T3 level and intradural extramedullary nodules in the lumbosacral area. Surgery was performed with partial tumor removal and histologic examination revealed a small round cell tumor and immunohistochemical characteristics of pPNET. She died due to tumor progression at four months after initial diagnosis. This case has been reported to raise awareness among clinicians to include the possibility of intraspinal tumors in the differential diagnosis of progressive neurological deficits mimicking Guillain-Barre syndrome.
Diagnosis, Differential ; Guillain-Barre Syndrome ; Humans ; Infant ; Magnetic Resonance Spectroscopy ; Neuroectodermal Tumors ; Neuroectodermal Tumors, Primitive ; Neuroectodermal Tumors, Primitive, Peripheral ; Paraparesis

OBJECTIVETo study clinicopathologic features, immunohistochemical profile, diagnosis and differential diagnosis of childhood central nervous system primitive neuroectodermal tumors (CNS PNETs) with the features of ependymoblastoma and neuroblastoma.

METHODSThe clinical data, morphologic and immunohistochemical features were analyzed in 4 cases of pediatric CNS PNETs with features of ependymoblastoma and neuroblastoma. EnVision method immunohistochemistry was applied.

RESULTSFour patients including three boys and one girl presented at the age from 12 month to 4 years and three tumors located in cerebrum, one in brain stem. All tumors showed typical combined histological patterns of ependymoblastoma and neuroblastoma, demonstrating zones of true rosettes, occasional pseudovascular rosettes, and undifferentiated neuroepithelial cells in a prominent background of mature neuropils. There was focal expression of glial fibrillary acidic protein (GFAP) consistent with glial differentiation and epithelial membrane antigen (EMA) consistent with ependymal differentiation. Necrosis was seen in three cases and calcification was present in one case. Immunohistochemically, the rosettes and undifferentiated neuroepithelial cells were positive for vimentin, partially positive for GFAP and EMA but negative for synaptophysin. The tumor cells were also positive for synaptophysin in neuropils. The Ki-67 label index ranged from 20% to 60%.

CONCLUSIONSCNS PNETs with the features of ependymoblastoma and neuroblastoma is a rare tumor with poor prognosis. The tumor primarily occurs in childhood, especially infant and belongs to the family of embryonal tumors of the CNS. The morphologic, immunohistochemical and genetic features are important in differential diagnosis from other tumors of the CNS.

Antigens, Neoplasm ; metabolism ; Central Nervous System ; pathology ; Child ; Female ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Immunohistochemistry ; Infant ; Male ; Mucin-1 ; metabolism ; Neuroblastoma ; diagnosis ; pathology ; Neuroectodermal Tumors, Primitive ; diagnosis ; pathology ; Neuroectodermal Tumors, Primitive, Peripheral ; diagnosis ; pathology ; Synaptophysin ; metabolism ; Vimentin ; metabolism