Paragangliomas are benign neoplasms that arise from the autonomic nervous system and the associated paraganglia. Although benign, they have been shown to possess metastatic potential. Involvement of the spine is rare. Even rarer is con- sidered the involvement of the cervical spine. We report a case of a patient with a history of an extra-adrenal non-functional paraganglioma of the jugular foramen which was initially treated with intra-arterial embolization. After a 3-year disease- free follow-up, the patient was presented with symptoms of spinal cord compression due to spinal metastases in C2 and C3 vertebrae. The patient was then treated with surgical decompression and external beam radiation. Therapeutic manage- ment with additional treatment options is now under discussion by a multidisciplinary team. Paraganglioma of the jugular foramen with spinal metastasis is an uncommon presentation where increased physician awareness and long-term follow- up are mandatory for all patients with history of paraganglioma. INTRODUCTION to possess metastatic potential [1, 2]. There have been several reports of metastatic paraganglioma in the literature, but rarely do Paragangliomas are highly vascular neuroendocrine tumors that arise from chromafﬁn cells of the adrenal medulla (in the case of such tumors metastasize to the spine . In this report, we present a patient with symptoms of spinal cord compression caused by pheochromocytomas) and from neuroendocrine cells of the extra-adrenal autonomic paraganglia. Most paragangliomas are spinal metastases in C2 and C3 vertebra from a primary non- functional extra-adrenal paraganglioma of the jugular foramen. In described as benign in nature, but almost 10% have been shown Received: October 28, 2017. Accepted: January 7, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact firstname.lastname@example.org Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy005/4837047 by Ed 'DeepDyve' Gillespie user on 16 March 2018 2 S. Kapetanakis et al. Figure 1: (A) CT shows a destructive lesion in right petrous bone arising at the level of jugular bulb extending anteriorly to the carotid canal (arrow) (B) CT at the level of C3 reveals mottled osteolytic lesion with destruction of the vertebral body and involvement of posterior elements. MRI demonstrates the lesion to be hypointense on sagittal T1-weighted image (C) isointense on T2-weighted (D) and with avid enhancement on post-contrast sagittal (E) and axial (F) TI fat-sat images and epidural extension of the tumor with spinal cord compression. Note avid enhancement of the metastatic lesion of C5 vertebrae body as well. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy005/4837047 by Ed 'DeepDyve' Gillespie user on 16 March 2018 Metastatic paraganglioma of the jugular foramen 3 Figure 2: (A) Osteolytic invasion by paraganglioma (B) tumor cells expressing chromogranin A. addition, we review the relevant literature and discuss on the Malignant paraganglioma is considered a rare presentation, topics of imaging and treatment of this challenging condition. sinceinmostcases themassisasymptomatic and slow-growing. Its malignancy is diagnosed by local recurrence after resection of the primary mass, or ﬁndings of distant metastases. Vertebral CASE REPORT metastases are rare and even rarer are considered the cervical A 52-year-old man presented to our hospital with increasing neck metastases . In our case, the paraganglioma was non-functional pain and upper extremity weakness. The patient had a history of without any symptomatology of paroxysmal hypertension, a right jugular paraganglioma which had been discovered by palpitation, headache or sweating. It became symptomatic, chance and he had been treated 3 years earlier with intra-arterial due to mechanical compression of the spinal cord, when it embolization. The patient had been found to be disease-free at metastasized to the cervical spine. follow-ups. On computed tomography (CT), a permeative destruc- Awareness of imaging manifestations of spinal metastatic tive lesion in the right petrous bone arising from the upper por- disease is essential for both the diagnosis and the follow-up. tion of the jugular bulb and extending anteriorly to the carotid Bone scintigraphy has been the standard initial imaging meth- canal was depicted (Fig. 1A). CT scan and magnetic resonance od for screening of skeletal metastases. CT scans can recognize imaging (MRI) of the patient’s cervical spine showed a destructive a bony metastatic lesion earlier than an X-ray. Both bony mass lesion involving the C2 and C3 vertebral bodies with severe involvement and neural compression from epidural tumor are canal stenosis and spinal cord compression. A mottled osteolytic demonstrable by MRI, however, MRI cannot distinguish this lesion with destruction of C2 and C3 vertebral bodies and involve- lesion from other spinal tumors [4, 6]. Thus the diagnosis is ment of posterior elements was observed (Fig. 1B). MRI showed a conﬁrmed on the histology and immunohistochemical analysis hypointense expansile lesion involving the C2 and C3 vertebral of the intraoperative tissue . In our report, the presence of an bodies with extension into the epidural space compressing the inﬁltrating mass with tumor cells arranged in solid nests, spinal cord. The lesion enhanced homogeneously on post- osteolytic invasion, increased number of mitoses and tumor contrast T1-weighted MR (Fig. 1C–F). Laboratory tests were nor- cells positive for chromogranin A conﬁrmed the diagnosis. mal and did not indicate elevated serum normetanephrine/nor- Regarding the primary site, the combined therapeutic approach epinephrine. The patient underwent decompressive surgery with with preoperative selective embolization followed by surgical an uncomplicated postoperative course. Histopathological exam- resection is the safest and most effective method for complete ination revealed an inﬁltrating neoplasm with tumor cells excision of tumors with a reduced morbidity rate. Irradiation arranged in solid nests with increased number of mitoses. Tumor (gamma knife) is an effective therapy method for residual cells were positive for chromogranin A. The histologic and immu- head and neck paraganglioma . At ﬁrst, our patient was only nohistochemical assays were compatible with paraganglioma treated with intra-arterial embolization without surgical resec- (Fig. 2). Following his discharge, the patient underwent an out- tion. This happened due to the fact that the initial site appeared patient external beam radiation. No family history of paraganglio- to be benign and surgically challenging due to its location. The ma was reported. patient remained disease free at the follow-ups. However, metastases appeared and the therapeutic strat- egy changed completely. It is believed that the treatment of DISCUSSION choice for malignant paragangliomas with vertebral metasta- Generally, paragangliomas account for only about 0.3% of all neo- sis and cord compression is surgical decompression and plasms . Most paragangliomas are described as benign in adjuvant external beam radiotherapy. Chemotherapy and nature, but almost 10% have been shown to possess metastatic I -MIBG have also been used therapeutically in malignant potential . Usually, paragangliomas are seen within the adrenal paraganglioma . Our patient underwent decompressive medulla giving rise to pheochromocytoma and only 10% arise surgery without any complications and outpatient external outside the adrenal glands. In the head and neck region, the beam radiation. Treatment with alternative therapies includ- carotid body is the most common site of occurrence followed in ing combination chemotherapy is now under discussion by a frequency by the jugulotympanic and vagal paragaglioma . multidisciplinary team. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy005/4837047 by Ed 'DeepDyve' Gillespie user on 16 March 2018 4 S. Kapetanakis et al. It is challenging to make the appropriate initial choice regard- REFERENCES ing the management of such cases. Wait-and-see policy may be 1. Chrisoulidou A, Kaltsas G, Ilias I, Grossman AB. The diagno- considered for very slow-growing tumors in patients with multiple sis and management of malignant phaeochromocytoma medical problems and old age . While the individual behavior of and paraganglioma. Endocr Relat Cancer 2007;14:569–85. paragangliomas is unpredictable the primary management should 2. 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Combined surgical and radiosurgical treatment for a of data and data analysis, drafting of the article and critical symptomatic cervical metastasis in a case of malignant evaluation, and approval of the version to be published. paraganglioma: a casereport. BMC Res Notes 2013;6:494. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy005/4837047 by Ed 'DeepDyve' Gillespie user on 16 March 2018
Journal of Surgical Case Reports – Oxford University Press
Published: Feb 1, 2018
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