A rare case of spinal cord compression due to cervical spine metastases from paraganglioma of the jugular foramen—how should it be treated?

Kapetanakis, Stylianos; Chourmouzi, Danai; Gkasdaris, Grigorios; Katsaridis, Vasileios; Eleftheriadis, Eleftherios; Givissis, Panagiotis

doi:10.1093/jscr/rjy005

Cited by 6 publications

(5 citation statements)

References 11 publications

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“…Paragangliomas generally behave like benign tumors with the majority of recurrences occurring locally [11]. Paragangliomas often exhibit a prolonged interval to the development of recurrence or metastasis, with recurrences and/or distant metastasis being reported to occur 0-20 years from diagnosis [11,[17][18][19][20][21]]. An estimated 10% of paraganglioma tumors will demonstrate malignant potential, spreading to regional lymph nodes, bone, lung, and liver [11,19].…”

Section: Discussionmentioning

confidence: 99%

“…Paragangliomas often exhibit a prolonged interval to the development of recurrence or metastasis, with recurrences and/or distant metastasis being reported to occur 0-20 years from diagnosis [11,[17][18][19][20][21]]. An estimated 10% of paraganglioma tumors will demonstrate malignant potential, spreading to regional lymph nodes, bone, lung, and liver [11,19]. Despite distant metastasis being a rare occurrence, the patient described in the case above demonstrated multiple vertebral metastases at T6-T7 and T11, resulting in a Bilsky grade 2 posterior to anterior cord compression.…”

Section: Discussionmentioning

confidence: 99%

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Carotid body paraganglioma metastatic to spine causing cord compression: a case report

et al. 2023

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Background Carotid body tumors (CBTs) are rare neuroendocrine neoplasms arising near the carotid bifurcation with a reported incidence of 1 to 2 cases in 100,000 patients. Most CBTs are sporadic, benign, slow-growing, and non-secreting, but untreated CBTs can grow locally to compress the nearby blood vessels, esophagus, and airway. Regional metastases can occur in 5% to 10% of cases, but distant metastases are exceedingly rare, occurring in roughly 1–2% of cases. As such, the optimal treatment for metastatic CBTs is not well-defined. We report a rare case of a patient with CBT distant metastases causing spinal cord compression. Case presentation A 40-year-old African American female presented with a right neck mass, headaches, vertigo, tinnitus, hoarseness, and dysphagia. Imaging demonstrated a Shamblin II right neck mass; subsequent transcervical resection and pathology showed a carotid body paraganglioma. The patient recurred locally near the carotid bifurcation, so she underwent Stereotactic Body Radiation Therapy to the recurrent right neck disease. She later re-presented with new onset bilateral lower extremity weakness, dysmetria, and numbness. She was found to have metastatic disease to the thoracic spine causing spinal cord compression. She underwent laminectomy, tumor resection, and posterior fixation followed by adjuvant radiation therapy. She was started on systemic therapy with sunitinib. She eventually progressed with metastatic disease to the right iliac bone, which was treated with palliative radiotherapy. Second line systemic therapy with capecitabine and temozolomide was started. At last follow up, the patient was asymptomatic with stable persistent disease. Conclusions Paragangliomas often exhibit a prolonged interval to the development of progression; locoregional recurrences or rare distant metastases have been reported to occur as many as 20 years from diagnosis. The natural course of CBTs in other cases as well as the present case call into question the idea that CBTs are truly benign; instead CBTs may be indolent tumors with metastatic potential. Treatment choices for CBTs include surgical resection, radiation therapy, and systemic therapy, though the optimal treatment regimen for metastatic CBTs is not well-defined. A more advanced understanding of CBT pathophysiology, disease classification, risk stratification, and treatment options is needed to improve outcomes for patients.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Carotid body paraganglioma metastatic to spine causing cord compression: a case report

et al. 2023

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“…However, CVD therapy only provides short-term management of metastatic paragangliomas and does not increase overall patient survival [16]. In patients with vertebral metastases and spinal cord compression, it is believed that the treatment of choice is surgical decompression coupled with external beam radiotherapy [5]. Patient prognosis with metastatic paragangliomas is variable.…”

Section: Discussionmentioning

confidence: 99%

“…They are typically benign neuroendocrine tumors arising from the autonomic paraganglia in the neck, thorax or abdomen [4]. An estimated 10% of these tumors will demonstrate malignant potential, with possible spread to the bone, lung and liver [3,5].…”

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confidence: 99%

Multiple Spinal Metastases from a Malignant Paraganglioma

Calderon¹,

Villafuerte²,

Lalla³

et al. 2020

Neurosurg Cases Rev

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“…[1–3,26,27] Due to its exceptional rarity, malignant paraganglioma of thoracic spine have not been well recognized and commonly appear as an ill-circumscribed lesion that occupies the spinal region. [26–29]…”

Section: Discussionmentioning

confidence: 99%

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

Liu¹,

Zhou²,

Song

et al. 2019

Medicine

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Rationale:Paragangliomas are rare neuroendocrine tumors that originate in specialized cells derived from the neural crest with metastasis to the thoracic spine being among the rarest forms. Here, we are presenting a detailed analysis of a case of malignant paraganglioma in the thoracic spinal region in a 14-year-old boy. Our focus is to emphasize the importance of considering malignant paraganglioma as a diagnosis and guiding the perioperative management upon surgical treatment. The management of these unique cases has yet to be well-documented.Patient concerns:A 14-year-old boy presented with a 5-month history of continuous and progressive elevated blood pressure and back pain. The patient, who had been diagnosed of malignant paraganglioma in the left posterior mediastinum for 3 months, received surgical resection of paraganglioma in the left posterior mediastinum, which had involved the left intervertebral foramen of T4. However, the tumor was not completely resected during the first operation.Diagnoses:Magnetic resonance imaging of spine and positron emission tomography-computed tomography showed spinal cord compression secondary to the epidural component of the T4 mass, with increased marrow infiltration of the left T4 intervertebral foramen, which was difficult to be removed. Postoperative pathology confirmed the diagnosis of spinal involvement of malignant paraganglioma.Interventions:The patient underwent biopsy and percutaneous vertebroplasty of T4 and paravertebral lesions, and needle-track cement augmentation via a posterior approach.Outcomes:The patient's neurological deficits improved significantly after the surgery, and the postoperative period was uneventful at the 10-month follow-up visit. There were no other complications associated with the operation during the follow-up period.Lessons:Combined efforts of specialists from orthopedics, neurosurgery, thoracic surgery, and medical oncology led to the successful diagnosis and management of this patient. Malignant paraganglioma of thoracic spine, although rare, should be part of the differential diagnosis when the patient has a history of paraganglioma and presents with back pain and radiculopathy. We recommend the posterior approach for spinal decompression of the malignant paraganglioma when the tumor has caused neurological deficits. Osteoplasty by cement augmentation is also a good choice for surgical treatment. However, we need to take the potential risk of complications in bone cement applications into full consideration.

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A rare case of spinal cord compression due to cervical spine metastases from paraganglioma of the jugular foramen—how should it be treated?

Cited by 6 publications

References 11 publications

Carotid body paraganglioma metastatic to spine causing cord compression: a case report

Carotid body paraganglioma metastatic to spine causing cord compression: a case report

Multiple Spinal Metastases from a Malignant Paraganglioma

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

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