Intramedullary spinal cord metastasis arising from papillary thyroid carcinoma: A case report and review of literature.

BACKGROUND: Intramedullary spinal cord metastases (IMSCM) are typically drop lesions from intracranial metastases and are a rare manifestation of systemic malignancy (8.5% of central nervous system metastases). They arise from primaries such as the lungs, breast, kidney, melanoma, or lymphoma. On the other hand, they arise very rarely from papillary thyroid carcinoma (PTC), even though it is the most common type of primary thyroid malignancy. CASE DESCRIPTION: A 54-year-old male presented with pain in the lower back along with tingling, numbness, and weakness in the bilateral lower limbs. This was associated with urine incontinence for 1½ months. In the previous month, he developed a left-sided solitary thyroid nodule. Fine needle aspiration cytology and ultrasonography were suggestive of metastasis. Furthermore, the thoracolumbar magnetic resonance imaging showed T1-hypo and T2-hyper-intense D11-D12 level intramedullary lesion, with intense enhancement, which was consistent with an intramedullary lesion involving the conus. At surgery, a firm, brownish yellow, friable, vascular tumour was removed en toto. Upon discharge, the patient was neurologically intact except for residual bladder incontinence. CONCLUSION: In an extensive literature review (pubmed), IMSCM metastasis from PTC primary is confirmed as a rarity and this may be the fourth documented case. Moreover, this may be the first report of a case of PTC metastatic neurological deterioration "even before the treatment of the primary was undertaken." Early diagnosis and microsurgical resection can result in improvement of neurological deficits and in the quality of life of patients with IMSCM.

Trochlear Nerve Neurofibroma in a Clinically NF-1-Negative Patient; A Case Report and Review of Literature.

BACKGROUND: Isolated orbital neurofibroma unassociated with systemic neurofibromatosis is relatively rare and may be difficult to clinically differentiate from other orbital tumors. Sensory branches of the trigeminal nerve-namely lacrimal, nasociliary, and frontal-are the most common nerves of origin for intraorbital neurofibroma, but we discovered a neurofibroma arising out of the right trochlear nerve, in absence of clinical stigmata of neurofibromatosis type 1, which is rare. CASE DESCRIPTION: A 41-year-old adult presented with painless progressive proptosis of the right eye for 10 years without history of visual problems or diplopia. The right eye had axial proptosis with periorbital swelling. On magnetic resonance imaging (MRI), a right orbital extraconal, expansile, lobulated, cystic space-occupying lesion was seen with an enhancing component, pushing the lateral rectus with T1 isointensity and T2 hyperintensity, suggesting a preoperative working diagnosis of pseudotumor or lymphoproliferative tumor. Intraoperatively, a rudimentary slender, white, elongated structure was passing through the length of the tumor. The elongated tumor engulfing the trochlear nerve was traced up to the lateral part of the superior orbital fissure. The tumor was excised completely and was found to be a neurofibroma. CONCLUSION: Isolated trochlear nerve neurofibromas, in the absence of clinical stigmata of NF1, are rare. Multiplicity, multilobulation, ring-configured contrast enhancement, and heterogenous MRI signal intensities help in the accurate preoperative imaging diagnosis. A possible cure is thus achievable with complete excision without damaging important adjacent neurovasculo-musculotendinous structures in the orbit. To the best of our knowledge, this is the fourth reported case of isolated trochlear nerve neurofibroma.

Primary extradural spinal ganglioneuroblastoma: a case report.

We report a case of ganglioneuroblastoma of the spinal cord in a 25-year-old man. Clinical history was short with paraparesis and bladder involvement. The MRI picture was that of an extradural solid tumor with extension to both intervertebral foramina, more suggestive of nerve sheath tumour rather than malignant embryonal tumour. Diagnosis was established by histopathological study. We could not find such a presentation of spinal ganglioneuroblastoma as an extradural, primary tumour in the literature. We discuss the radio-pathological features, surgical management and post-operative outcome.

Review of spinal neuroectodermal tumor.

Primary spinal primitive neuroectodermal tumors (PNETs) are rare tumors. Most of these tumors occur in children and young adults. To date, 47 cases of primary spinal PNET have been reported in the literature. These tumors are highly aggressive with rapid growth. Review of the literature shows that the overall prognosis of PNETs of spinal cord is very poor even with adequate surgery, radiotherapy and chemotherapy. All the cases reported to date are reviewed in terms of surgical treatment, adjuvant therapy and outcome and the experience with two of these cases are described. Both cases were thoracic extradural ones with intrathoracic extension through intervertebral foramina resembling neurofibroma. Both cases underwent gross total removal of intraspinal and thoracic component. Post-operatively both underwent cranio-spinal radiotherapy. One patient died after a post-operative period of 4 months and the other one is still alive 8 months after surgery, radiotherapy and chemotherapy.