Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1762617 (weakness)
 37,932 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 79-year-old female presented with difficulty ambulating and was found to have weakness and hyperreflexia in the lower extremities. Magnetic resonance imaging (MRI) revealed a large T8-T9 intraspinal tumor. She underwent a thoracic laminectomy, and excision of an intradural extramedullary lesion. The surgical specimen was soft, black tissue that consisted of a moderately cellular, deeply pigmented tumor. The neoplastic cells proved to be melanocytic, and were devoid of overt features of anaplasia, i.e., prominent nuclear pleomorphism, necrosis, significant mitotic activity, and high proliferation indices. Four months postoperatively, MRI demonstrated focal areas of enhancement in the conus medullaris and in the fourth ventricle, indicating leptomeningeal spread. Subsequently, the patient underwent whole brain radiation. On repeat imaging, there was nodular enhancement of the fourth ventricle and throughout the spinal cord. Despite chemotherapy and radiation therapy, the disease advanced and the patient expired. Meningeal melanocytoma is a rare, histologically benign tumor with good prognosis. However, local aggressive behavior has been recorded, especially in cases of subtotal gross resection. On a literature review, there was one case of cranial posterior fossa meningeal melanocytoma with associated lesions in both suprarenal glands and the left kidney, but there were no cases with distant metastasis. In this report, we present an unusual case of spinal meningeal melanocytoma with diffuse spread throughout the craniospinal axis that proved to be fatal.
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PMID:Meningeal melanocytoma: an aggressive course for a benign tumor. 1455 2

Meningeal melanocytoma is a benign lesion arising from leptomeningeal melanocytes that at times can mimic its malignant counterpart, melanoma. Lesions of the spine usually occur in extramedullary locations and present with spinal cord compression symptoms. Because most reported spinal cases occur in the thoracic region, these symptoms usually include lower extremity weakness or numbness. The authors present a case of primary intramedullary spinal meningeal melanocytoma presenting with bilateral lower extremity symptoms in which the patient had no known supratentorial primary lesions. Gross total surgical resection allowed for full recovery, but early recurrence of tumor was detected on close follow-up monitoring, allowing for elective local radiation without loss of neurological function. Case reports of such tumors discuss different treatment strategies, but just as important is the close follow-up monitoring in these patients even after gross total surgical resection, since these tumors can recur.
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PMID:Intramedullary spinal melanocytoma. 2113 26

Meningeal melanocytoma is a rare benign tumor, most frequently located in the posterior fossa and spinal canal. The aim of this study was to investigate the clinical manifestations, radiological features, management, and follow-up data of spinal meningeal melanocytomas. We present the clinical data and long-term outcomes from a consecutive surgical series of 16 patients with pathologically diagnosed spinal meningeal melanocytomas. All of the patients underwent surgical resection. Pre and postoperative MRI was performed. Follow-up data and neurological functional assessment is presented and discussed. The mean age at diagnosis was 42.0 years, with a significant male predominance. The primary clinical symptoms were weakness or numbness of the extremities. The appearance of melanocytoma on MRI is typically isointense to hyperintense on T1-weighted images, hypointense on T2-weighted images, and contrast enhancement tends to be remarkable and homogeneous. In most cases, gross total resection is achievable; however, in rare cases with dumbbell-shaped tumors involving the extraspinal region, a staging operation and subtotal resection should be attempted. During a mean follow-up period of 58.1 months, the symptoms were completely relieved in all the patients, and no tumor progression or recurrence was noted. Melanocytic tumors of the central nervous system have a typical appearance on MRI scans, varying with the content and distribution of melanin. However, the differential diagnosis between malignant melanoma and melanocytoma still depends on pathological criteria. Spinal meningeal melanocytoma has a benign course, and it is amenable for gross total resection. The outcome is favorable following complete resection.
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PMID:Spinal meningeal melanocytomas: clinical manifestations, radiological and pathological characteristics, and surgical outcomes. 2694 Sep 7