Paraganglioma is a rare tumor arising from extra-adrenal paraganglia and accounts for 0.3 % of all neoplasms. Less common sites include the retroperitoneum, the para-arotic region, bladder, fi lum terminale, skull, larynx and others. According to Zileli et al. 2008 , paragangliomas are tumors that arise from the paraganglia cells of the autonomic nervous system, ultimately derived from the neural crest. The major­ity of paragangliomas are nonfunctional and benign; however, they may produce excess catecholamines or have malignant potential. Paragangliomas arise within or near the carotid bod or the glomus jugulare in 80–90 % of cases. Other locations are the middle ear, thyroid, GI tract and pancreas. In the CNS paragangliomas have been seen in the pineal region, the petrous ridge, and the sella turcica. Paragangliomas of the spine are uncommon and usually present as intradural tumors within the cauda equina, thoracic tumors are even more rare.

Spinal paragangliomas are rare. According to Rosai ( 2011 ), spinal paragangliomas are rare and most reported cases occurred in the cauda equina or fi lum terminale. A series of 30 spinal cases were reported by Marcin et al. 1997, of which 19 were in the lumbar region, six in the cauda equina, two in the fi lum terminale, two in the thoracic region and two in the cervical spine. For pheochromocytoma and func­tional paraganglioma, please see Roman 2004 .

According to Zibilli et al. “the majority of paraganglioas are nonfunctional and benign. However, they may produce catecholamines in excess or may be protein malignant (20–49 %). They occur within or near the carotid bodies or the glomus jugulares in 80–90 % of cases. They occur in other sites as well (see incidence above). Extra-adrenal pheochromocytomas are the paragangliomas, which account for 15 % of total pheochromocytomas. They are rarely functional and 80–90 % as mentioned above are in the glomus jugulares or carotid bodies. Retroperitoneal paragangliomas are higher in malignancy and may be manifested by metastasis. Pheochromocytomas may occur as isolated tumor or as part of familial syndromes, e.g. von Hippel Landau, multiple endocrine neoplasia, von Recklinghausen's dis­ease, and Surge-Weber syndrome; paragangliomas show 14 %. Microscopically: the tumor consists of polyhedral chief cells organized in in nests, surrounded by a deli­cate fi brovascular stroma. The tumor have a granular eosinophilic cytoplasm and round or ovoid nuclei. There is positive staining for synaptic physin, chromagranin, S-100, tumor-specifi c antigen. Metastatic spinal lesions represent 35 % of the spinal paragangliomas. According to Falavigna et al. The paraganglion systems are a com­ponent of the neuorendrocrine system and comprises the chromaffi n-positive cells

1. History of cord or cauda equina compression

2. Neurological fi ndings

5. Biochemical tests:

• Local radiotherapy for the incomplete removal and recurrent cases, as well as malignant cases.

Plasma cell leukemia (PCL) is a leukemic variant of multiple meyloma (MM) 2–4 %, which formed an intraspinal mass compressing the spinal cord leading to paraplegia.