Paragangliomas of the spine

Abstract

Clinical presentations and courses of spinal paragangliomas are varied, and there are no standard principles of treatment to date (1,8). In this article, we report the clinical data of 7 consecutive cases with complete medical records and followup results that were treated in our institutions, in an attempt to explore the diagnosis, treatment and prognosis. █ mATERIAl and mEThODSA retrospective analysis was performed in 7 patients with spinal paraganglioma in our hospital from October 2000 to October 2011. No patient was lost in the follow-up period. There were 6 cases of primary spinal paraganglioma and one case █ INTRODUCTION P aragangliomas are rare tumors that arise from the paraganglia cells of the neuroendocrine system and account for 0.3% of all neoplasms (2,4). More than 80% of this kind of tumor develops within or near the glomus jugulare and carotid body. Paragangliomas of spinal involvement are extremely rare. Primary spinal paragangliomas usually take the form of intradural compression of the cauda equina or intra/extradural compression of the thoracic and cervical spinal cord (1). The first case of metastatic spread of a paraganglioma to the spine was reported in 1948. So far, less than 30 cases of spinal metastases by paragangliomas have been reported in English.AIm: Paragangliomas of the spine are rare tumors. Clinical presentations and courses of spinal paragangliomas are varied, and there are no standard principles of treatment to date. The purpose of this study was to explore the diagnosis, treatment and prognosis of spinal paragangliomas. mATERIAl and mEThODS:The clinical data of 7 consecutive cases, with complete medical records and follow-up results that were treated in our institutions from October 2000 to October 2011, was retrospectively reviewed.RESUlTS: There were 6 males and 1 female with a mean age of 40 years (range, 16-61). The follow-up period ranged from 40 to 98 months (mean, 72 months). Of the 6 primary spinal paragangliomas, one lesion was in the cervical intradural extramedullary space, one in the thoracic epidural space and four in the lumbar intradural extramedullary space. All tumors were totally resected and no recurrence was detected during the follow-up period. Of the metastatic case, the lesion of the spine was located in the first lumbar epidural space and vertebra. The patients underwent surgical resection two times with radiotherapy, but the tumor recurred and the patient suffered from the paraplegia of lower limbs and urine and stool incontinence during the follow-up period. No patient died.CONClUSION: Spinal paragangliomas are rare lesions and seldom considered in the presurgical differential diagnosis due to its rarity and non-specific clinical symptoms and imaging features. Clinical follow-up was necessary to determine the outcome. Complete resection is necessary to prevent recurrence. The role of radiotherapy in the management of these lesions needs further assessment.

Nonfunctional extradural thoracic spine paraganglioma in a pediatric patient: a case report

“…Continued follow-up for these patients shows a recurrence rate of 2.2% in the literature. Multiple studies reported a recurrence after complete resection in their case series in at least one patient, whereas others reported none [ 4 , 5 , 9 ]. Subtotal resection, however, can increase the figure substantially.…”

Froin's Syndrome due to Spinal Paraganglioma - A Case Report

“…Paraganglioma's can be secretory secreting catecholamines, and patients presenting with hypertension, tachycardia or diaphoresis which weren't present in our case so we excluded functional or malignant paragangliomas 5 and we didn't go for catecholamines levels or other investigations in that direction. On the basis of location they can also present with mass effect like numbness, pain or weakness [6][7][8] . Our patient's MRI lumbosacral had also revealed a lesion extending from T12-L1 levels, so we thought that patient's symptoms of numbness, pain and weakness were due to that mass.…”

“…Our patient's MRI lumbosacral had also revealed a lesion extending from T12-L1 levels, so we thought that patient's symptoms of numbness, pain and weakness were due to that mass. Radiologically they appear hyperintense on T2 weighted images and iso or hypointense on T1 images of MRI 6,7,8 . These findings are consistent with MRI findings of our case which also showed a hyperintense area on T2 weighted images and hypointense on T1 weighted images.…”

“…Complete surgical resection was used as the mode of treatment in our case, and has been found effective as the patient was symptom free after the operation and was found symptom free on follow-ups as well. As their prognosis is excellent they should be differentiated from other spinal tumors and in case of malignant forms or recurrent tumors neoadjuvant therapy should be implicated 8 and follow up imaging and radiological assessment is also required.…”

Surgical treatment of malignant paraganglioma with spinal invasion in a juvenile patient

“…Clinical studies looking at malignant spinal paragangliomas in juvenile patients is lacking due to the extremely low incidence rate, and only 3 cases of juvenile patients have been reported in literature. [9,25] In a review of the literature, we discuss the common perioperative considerations in patients with malignant paragangliomas in the thoracic spine level and how to manage the perioperative hemodynamic complications. The epidemiology, origins, diagnosis, treatment, and prognosis of malignant spinal paraganglioma are also discussed.…”