Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:Q86TM3 (cage)
 29,987 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neurofibrosarcomas are rare tumors usually arising in somatic soft tissues or peripheral nerves. Four cases of metastatic neurofibrosarcoma to the spine have been reported before. The current case is unusual because of the presence of two distinct, metachronous spinal metastasis and lung metastasis. A 30-year-old woman with neurofibromatosis and a history of previous neurofibrosarcoma resection presented with back pain. Radiologic evaluation revealed a lytic lesion of the eleventh thoracic vertebra. A transthoracal corpectomy, reconstruction by Harms' cage and posterior instrumentation, and fusion were carried out. After the completion of adjuvant chemotherapy, a solitary pulmonary nodule was detected. Shortly after resection of the metastatic pulmonary nodule, the patient complained of acute onset low-back pain. Radiologic assessment revealed another lytic lesion in the L5 vertebra after 6 months. Again, a corpectomy, anterior and posterior instrumentation, and fusion were carried out. Eight months after the second spinal resection, another solitary pulmonary metastasis was diagnosed and resected. The patient's health status suddenly deteriorated 26 months after the initial spinal metastatectomy, and she died. Though local control can be achieved in more than 80% of the patients with neurofibrosarcoma by wide surgical resection followed by adjuvant chemo- and radiotherapy, most patients die of systemic metastasis. The current patient survived 50 months after the initial resection of a forearm neurofibrosarcoma. Despite achieving local control, she died due to systemic recurrence. Prolonged survival with the help of chemo- and radiotherapy justifies our aggressive surgical strategy for the treatment of spinal metastasis in order to achieve neurologic cure and spinal stability.
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PMID:Metastatic spinal neurofibrosarcoma. 1188 Sep 13

We report a case of fatal evolution of neurofibromatosis in a young boy. A laminectomy was performed when he was 9 years old. A secondary hyperkyphosis led to many surgeries resulting in recurrent malunions. When he was 23 years old, a breakage of his rods was treated by a new instrumentation and a T12-L1 interbody cage fitted with rh-BMP. Five months later, he developed a huge posterior tumour on his back. The biopsy diagnosed a neurofibrosarcoma. The growth of the tumour was extremely rapid. He died after several months from a septic shock. NF1 is characterised by neurofibromas that have a possibility of malign degeneration and conversion to a sarcoma. However, the chronology, rapidity of evolution and the exceptional volume of the tumour made us wonder whether the BMP had a part of responsibility as osteoinductor in the malignant degeneration, in this particular case, of neurofibromatosis. It seemed important to point out this case to the medical community.
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PMID:Could an osteoinductor result in degeneration of a neurofibroma in NF1? 2044 13