Intramedullary Primitive Neuroectodermal Tumor Presenting With Rapidly-Progressive Cauda Equina Syndrome -Case Report-

Abstract

A 39-year-old male presented with gait disturbances with rapid deterioration for 2 weeks. Neurological examination found paraparesis, sensory loss in the L1-S5 dermatomes, and vesicorectal dysfunction. Magnetic resonance (MR) imaging revealed a fusiform intramedullary tumor at T12-L1 levels with heterogeneous enhancement. The patient underwent microsurgical tumor resection. A myelotomy exposed a highly vascular tumor that was subtotally resected. Histological examination demonstrated hypercellular tumor accompanied by significant cell atypism and mitotic figures. Immunohistochemical staining was positive for glial fibrillary acidic protein, S-100 protein, synaptophysin, and INI-1, consistent with primitive neuroectodermal tumor (PNET). Postoperatively, the patient underwent irradiation to the whole craniospinal axis. He experienced local recurrence 7 months after surgery. MR imaging performed at 10 months revealed holocord progression and intracranial dissemination. The patient died 13 months after the onset of the disease. PNET should be considered in the differential diagnosis of an intramedullary spinal cord tumor.

Metastatic thoracic and lumbar intramedullary and extramedullary Ewing’s sarcoma: a rare case report and literature review

“…The identified cases showed that intramedullary Ewing sarcoma was located mainly in the thoracic and lumbar regions, although cervical and sacral locations were also reported. 4 – 36 The current case had a thoracic intramedullary lesion and a lumbar extramedullary lesion, both causing neural compression and deficits.…”

“… 2 The prognosis and survival rate for patients with spinal cord Ewing sarcoma is not well known because of the rarity of the condition; however, based on previous cases ( Table 1 ), the survival rate was 45% to 50% in primary cases and 30% to 35% in recurrent and metastatic cases. 4 – 36 In surviving cases, rehabilitation and physical therapy should be considered part of the treatment program to improve the patient’s neurological status. In the current case, the patient improved markedly after 4 months of physical therapy and rehabilitation.…”

The vaccine injection sites mimicking metastases of malignant melanoma on FDG PET

“…Bu nedenle uzun dönemli kontrolü amaçlamaktan çok hastanın hayat kalitesini ön plana alarak, tedaviye bağlı toksisiteden kaçınarak tedavi planlamak önerilebilir. [24] Sonuç olarak, daha az agresif tedavi ile toksisiteden kaçınmak bir alternatif olarak değerlendirilmelidir. Hastamızda total rezeksiyon sonrası kraniyospinal radyoterapi uygulanmıştır.…”

Primary intramedullary primitive neuroectodermal tumor

“…[ 4 ] Primary spinal PNETs are uncommon and can be extradural, intradural extramedullary, and intramedullary, of which primary intramedullary PNETs are extremely rarer. [ 5 , 6 ] Till now, only 24 cases of primary intramedullary PNETs have been reported. [ 3 , 5 – 24 ] Here we describe an unusual case of primary intramedullary PNET with unique clinical features and poor prognosis.…”