H3K27M-mutant glioma in thoracic spinal cord and conus medullaris with pilocytic astrocytoma morphology: case report and review of the literature.

BACKGROUND: The H3K27M-mutant spinal cord gliomas are very aggressive with a dismal prognosis, very few cases have been reported in the thoracic spinal cord and conus medullaris, and it is extremely rare with morphological features of pilocytic astrocytoma. CASE PRESENTATION: A 20-year-old man presented with thoracolumbar pain, progressive paraparesis, and urinary incontinence. Magnetic resonance imaging revealed an intramedullary solid-cystic lesion from D9 to conus medullaris. Subtotal resection was performed, restricted by the indistinct margins and the decline of the motor evoked potential during the surgery. Pathologic findings revealed a pilocytic astrocytoma with anaplastic features. However, a further assessment determined a diffuse midline glioma H3K27M-mutant, and adjuvant chemoradiotherapy was administered. After seven months of progression-free survival, the paraparesis worsened; at twelve months of follow-up, the patient developed paraplegia, and at 24 months the patient remains alive without any neurologic functions distal to the tumor and he is still under adjuvant treatment. CONCLUSIONS: The H3K27M-mutant spinal cord glioma is a very infrequent tumor with a wide variety of histological presentations even as indolent as pilocytic astrocytoma, which should be considered in spinal cord tumors, especially if there are clinical, histological, or radiological data that suggest aggressiveness. On the other hand, the fast progression led to the loss of complete neurological function distal to the tumor, in spinal tumors could explain a not so poor prognosis as it is in functionally and vital structures.

Solitary fibrous tumor: A clinical case. / Tumor fibroso solitario extra-intramedular de localización dorsal.

INTRODUCTION: Solitary fibrous tumor (TFS) is a rare tumor of mesenchymal origin, located mainly in the pleura. It is extraordinarily infrequent find it at the intraespinal level, being the thoracic region the most frequent. CASE PRESENTATION: We present the case of a 48-year-old patient with progressive ascending lower limb and myelopathy of one month of evolution, with intraspinal location at the D3-D4 level. It was surgically operated by posterior dorsal approach and D3-D4 laminoplasty, with an intradural tumor with an intramedullary component of approximately 18×12mm. The resection was complete and the pathological anatomy gave the diagnosis of solitary fibrous tumor. The patient is currently asymptomatic. DISCUSSION: Complete tumor resection and histopathological features are the main prognostic factors. Surgery have a main role in this type of neoplasia. CONCLUSION: There are few case published of solitary fibrous tumor with intraspinal localization. We apport another case to the literature.

Intraneural cyst of the supraescapular nerve: Atypical cause of peripheral nerve entrapment syndrome. Case report and literature review. / Quiste intraneural del nervio supraescapular: causa atípica de síndrome de atrapamiento de nervio periférico. Presentación de un caso y revisión de la literatura.

OBJECTIVE: Intraneural cysts are benign lesions located within the epineurium of some peripheral nerves and their aetiopathogenesis is controversial. Most are located at the level of the lower limbs. In the upper limbs, the most frequently affected nerve is the ulnar nerve. Suprascapular nerve entrapment syndrome due to the formation of an intraneural cyst is rare. In this article, we show a new case and perform a literature review of intraneural cysts located in the suprascapular nerve. METHODS: We present a 49-year-old woman with pain in the lateral shoulder region of several months' evolution. A brachial plexus MR showed a tumour of approximately 2×1.5cm, with a cystic appearance, in relation to the upper trunk of the right brachial plexus. RESULTS: We used a supra-infraclavicular approach. The cystic tumour affected the suprascapular nerve. After locating a zone on the surface without nervous fascicles, we performed a partial resection of the capsule and emptying of the cyst, with a xanthochromic gelatinous content. The anatomopathological examination confirmed the diagnosis of intraneural cyst. CONCLUSION: The suprascapular nerve is a mixed nerve, coming from the upper trunk. It provides the motor branches to the supraspinatus and infraspinatus muscle. Compression of the suprascapular nerve leads to atrophy of these muscles. This entity is one of the differential diagnoses in a patient with pain irradiating to the shoulder, and its correct treatment often results in complete remission of symptoms.

[Spinal sinovial cysts: Surgical treatment and clinical outcomes in a series of 18 cases]. / Quistes articulares espinales: evolución clínica y resultados según intervención quirúrgica. Revisión de 18 casos.

OBJECT: A series of 18 patients with symptomatic synovial cysts was analysed from May 2009 to November 2013. Different approaches were performed for their removal. MATERIAL AND METHODS: The study included 18 patients, 8 men and 10 women, aged between 50 and 77 years. An analysis was made of the variables including age, gender, symptoms, imaging studies, histopathology, surgery, follow-up, complications, and clinical outcome. RESULTS: An articular synovial cyst was diagnosed in 17 cases, and a ganglion in one cases. The most common symptom was back pain with radiculopathy (94%). Motor deficits occurred in 4 cases (22%), and 1 case (5%) presented with urinary incontinence. The most common level was L4- L5 (67%), with one atypical case observed in the D12 -L1 location. Hemi-laminectomy was performed in 14 cases, with 9 of them having an interspinous spacer (ISP) device inserted. A laminectomy with a fusion procedure was performed in 3 patients and 1 patient had a bilateral decompression using a unilateral approach. The patients were followed-up for between 6 months to 2 years. CONCLUSIONS: Synovial cysts are a cause of radiculopathy/neurogenic claudication. Spinal cysts are commonly found at the L4-L5 level. MRI is the tool of choice for diagnosis. The most common symptom was back pain with radiculopathy. Synovial cysts resistant to conservative therapy should be treated surgically. In our series, surgical resection of symptomatic juxtafacet cysts showed a good clinical outcome, but the optimal approach for patients with juxtafacet cysts remains unclear.

[Hemangioblastoma of the right L4 nerve root with radicular extraforaminal involvement]. / Hemangioblastoma radicular extraforaminal de la raíz L4 derecha.

Hemangioblastomas are benign hypervascular tumours, which are frequently located at the posterior fossa or the spinal cord. Nerve root hemangioblastomas account for less than 2% of total cases. Sometimes the initial diagnosis can be inaccurate and the final diagnosis is not reached until the surgical procedure is performed. Given the high vascularisation of this particular kind of tumour, preoperative embolisation of the tumour is recommended to reduce surgical bleeding and minimise the risk of injuries to motor nerve roots. In this particular case, the patient presented with radicular pain in the right L4 nerve root territory originated by a radicular extraforaminal hemangioblastoma. After tumour devascularization and under neurophysiological control, total excision of lesion was achieved without posterior neurological deficit.