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Primary Spinal Intradural Mesenchymal Chondrosarcoma with Several Local Regrowths Treated with Osteoplastic Laminotomies: A Case Report.

ABSTRACT: Mesenchymal chondrosarcomas (MCSs) are rare malignant tumors of the bone and soft tissues. Only a few cases of such tumors originating from the spinal canal meninges have been described in the literature. The authors report on a case of a 22-year-old woman with MCS of the arachnoid at the T12-L1 level with a 14-year-long observation. The tumor was totally resected using osteoplastic laminotomy with reconstruction of laminar roof. This small spindle cell tumor was initially microscopically suspected of synovial sarcoma, but correctly verified with widened immunophenotyping and molecular studies as MCS. At its first recurrence, the neoplasm showed microscopically a typical bimorphic pattern of small round cell component with foci of hyaline cartilage. The patient experienced three local recurrences: 4, 6, and 10 years after the initial resection, respectively. The techniques of laminotomy and relaminotomy were also used during three following operations. The repeated surgical removal, radiotherapy, and chemotherapy were the methods of complex oncological treatment. The patient remains now in complete remission, fully self-dependent with slight motor disturbance, and mild sensory deficits. Current views on the clinicopathological characteristics and treatment modalities of the chondrosarcomas of the spinal canal are discussed.

SUBMITTER: Derenda M

PROVIDER: S-EPMC5553510 | biostudies-other | 2017 Jul

REPOSITORIES: biostudies-other

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Project description:Introduction Hereditary transthyretin amyloidosis (ATTRv) is an autosomal-dominant disorder, where a TTR mutations lead to amyloid fibril deposits in tissues and consecutively alter organ function. ATTRv is a multisystemic disorder with a heterogeneous clinical presentation. Spinal leptomeningeal depositions are described only scarcely in the literature. Research question We present a rare case of surgically treated intradural, extra-medullary amyloidosis with respective clinical, diagnostic and surgical features to raise awareness of this rare entity. Material and methods Clinical, radiological and operative characteristics were retrieved from the electronical patient management system. Additionally, a scoping literature review on leptomeningeal spinal manifestations of ATTRv was performed. Results A 45-year-old man with a known ATTRv presented with gait disturbance and paresis of the lower extremities. He had been treated with the siRNA therapeutical Patisiran for 13 months under which his symptoms worsened. An MRI of the spine revealed spinal cord compression with myelopathy at the level of T2 with anterior dislocation of the spinal cord due to an intradural, extramedullary lesion. A laminectomy and opening of the dura with a complete resection of the lesion was performed. The histological examination of the biopsy showed amyloid deposits. At six-month follow-up the patient showed complete normalization of the paresis, gait, sensory and urinary disturbances and resumed his work. Discussion and conclusion Spinal leptomeningeal deposition of amyloid is a rare occurrence within the framework of ATTRv. Micro-neurosurgical complete resection of the lesion is feasible in patients with preoperative myelopathic symptoms and resulted in complete symptom relief in this case. Highlights • Spinal leptomeningeal manifestation of hereditary transthyretin amyloidosis is a very rare cause of spinal myelopathy.• Its awareness is important as it can be easily misinterpreted with other intradural spinal lesions.• Multidisciplinary workup is necessary and resection is feasible in symptomatic patients and can improve or resolve symptoms.• Online intraoperative video material illustrates the intraoperative findings and lesion characteristics.

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Primary Spinal Intradural Mesenchymal Chondrosarcoma with Several Local Regrowths Treated with Osteoplastic Laminotomies: A Case Report.

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