Primitive neuroectodermal tumor of ulnar nerve: A rare case

Primitive neuroectodermal tumor (PNET) is a highly aggressive tumor and mostly develops in children and young adults. PNETs of peripheral nerves are uncommon. Ulnar nerve, in particular, is an extremely peculiar origin for PNET and to the best of our knowledge only few well-documented cases have been yet reported.

Primary intracranial peripheral primitive neuroectodermal tumor in an adult patient with aphasia: a rare case report

Primary intracranial primitive neuroectodermal tumors (PNETs) are extremely rare malignancies, affects children and adolescents with only 10 cases has been reported over 33 years old. we present a case of PNET in a 36 years old female patient with the chief complaint of aphasia for the first time

MPC-7 Clinical features of Diffuse hemispheric glioma, H3 G34-mutant in children and young adults

INTRODUCTION: H3F3A G34R/V mutated gliomas are seen predominantly in children and young adults, and have been proposed as “Diffuse hemispheric glioma, H3 G34-mutant” in cIMPACT-NOW Update 6. However, the clinical features of the tumor have not been fully elucidated. METHODS: We retrospectively reviewed 4 cases with H3G34R mutation among 40 cases diagnosed as glioblastoma under 30 years old or primitive neuroectodermal tumor (PNET) in our hospital. RESULTS: There were one male and three female patients with a median age of 21.5 years (range: 17–27 years). All lesions were localized in the cerebral hemispheres, and the initial symptoms were headache in two cases and seizures in two cases. On imaging, there was one case with poor contrast, and unlike the infiltrative growth pattern of the other three contrasted cases, it showed a well-defined mass lesion. DWI showed high signal in all four cases, reflecting the high cell density in histopathology. All cases were IDH-wildtype. CONCLUSION: Although the patient background and genetic characteristics of the glioma with H3 G34R/V mutation at our institution were generally consistent with previous reports, there were some cases with atypical imaging findings. Further investigation is required for a deeper understanding of the clinical features of this tumor.

Autonomic dysfunction in postoperative primitive neuroectodermal tumor of heart

We present a patient with a primitive neuroectodermal tumor arising from the right atrium who experienced multiple syncope episodes daily, which had first appeared 1 month after surgery. The symptoms continued to worsen over the course of chemotherapy, and the autonomic function test (AFT) was performed after the 14th chemotherapy cycle. The AFT revealed orthostatic hypotension and reduced baroreflex-dependent sympathetic reactivity. Physical counterpressure techniques were applied with a visual biofeedback intervention, and were found to be effective in reducing the syncope episodes.

Isolated High-Grade Malignancy of the Spinal Cord Presenting as Longitudinally Extensive Transverse Myelitis

This article characterizes 2 cases of longitudinally extensive transverse myelitis (LETM) that did not respond to immunotherapy and were diagnosed by biopsy as primary central nervous system (CNS) malignancies. Diffuse H3 K27M-mutant glioma is a recently described entity with very few cases of isolated spinal disease described in adults. Primitive neuroectodermal tumor is similarly uncommon in the spinal cord. Malignancies should be considered in patients who fail to improve with immunomodulatory therapy. We believe the experiences of our center will raise awareness about that point, broaden the existing understanding of the diagnostic approach to LETM, and highlight the need for additional studies.

Snoring as main symptoms of peripheral primitive neuroectodermal tumor in oropharynx:a case report with review of literature

IntroductionIn children, snoring is often associated with hypertrophied tonsils and adenoids. There was a case in a girl who did not suffer from hypertrophy adenoids, but from a peripheral primitive neuroectodermal tumor (pPNET) in the oropharynx. pPNET often occurs in the deep soft tissues of the trunk, paravertebral, lower limbs and retroperitoneum. It is rarely located in children's oropharynx and makes snoring.Patient concernsWe report a case of a 4-years-old girl who complained of slurred speech and snoring. Her serum showed normal results, computed tomography (CT) and magnetic resonance imaging (MRI) revealed a mass lesion in oropharynx. By using histology and immunohistochemistry, cluster of differentiation 99 (CD99), vimentin, friend leukemia integration 1 (FLI-1), integrase interactor-1 and α-smooth muscle actin were positive, but epithelial membrane antigen, erythroblast transformation-specific (ETS) related gene (ERG), NK homeobox 2.2, Wilms’ tumor 1 and S100-protein were negative. And the rearrangement of the Ewing sarcoma breakpoint region 1 (EWSR1) gene was proved in the test of fluorescent.DiagnosisShe was diagnosed as a peripheral primitive neuroectodermal tumor.Interventions and outcomeThe girl had been treated with surgery and chemotherapy. She has been well for 24 months without any signs of disease.ConclusionIn children who snore, we should not only think of hypertrophied adenoids, but also the possibility of pPNETs in oropharynx. CT scan or MRI should be conducted in time, as well as histology and immunohistochemistry to avoid misdiagnosis.

Primary primitive neuroectodermal tumor of the adrenal gland: A unique tumor at an unusual site

Ewing sarcoma/Primitive Neuroectodermal Tumor (ES/PNET) is a malignant small round blue cell tumor of neuroectodermal origin that affects bones and soft tissue in children and young adults. ES/PNET is very uncommon in parenchymal organs. We report a case of primary adrenal PNET in a young female having pregnancy induced persistent hypertension. She underwent right adrenalectomy for a large adrenal mass. On histopathology, it was typical malignant round cell tumor (MRCT). Immunohistochemistry confirmed the diagnosis of ES/PNET with expression of CD99, FLI-1 and NKX2.2. Until now, only 38 cases of primary adrenal ES/PNET have been reported in the English literature with just 5 cases from India. Adrenal ES/PNET can be mistaken with other MRCTs like Non-Hodgkin lymphoma and neuroblastoma. Since the biology, treatment and prognosis of these tumors is entirely different, we emphasize that pathologists must be aware of this possibility and try to differentiate them using ancillary techniques.