Paragangliomas can generally develop at various body sites, but spinal paragangliomas are rare cases in which radiculopathy occurs in the lumbar lesion. We evaluated 2 cases of intradural extramedullary tumor associated with gait disturbance and radiating pain in both lower extremities. We performed surgical resection and biopsy of the masses. Although our first impression was schwannoma for one and ependymoma for the other, pathologic diagnosis was paraganglioma for each. Symptoms were relieved after surgery.
Paragangliomas were first described by Kohn
A 37-year-old female patient complained of radiating pain in both legs causing gait disturbance for a month prior to admission. In addition, she complained of lower back pain in any position. The patient underwent lumbar spine magnetic resonance imaging (MRI) at another hospital (
The patient was placed in the prone position. We performed a laminectomy on L2–3 and then incised the dura to find an encapsulated mass in the caudal direction. We started to remove the mass using a microscope; during the removal, the amplitude of the right vastus anterior MEP on the IOM was reduced by 50% and the left tibia anterior amplitude was reduced by 50% to 80%. However, after gross total removal, the MEP returned to normal.
A 59-year-old female patient was admitted complaining of radiating pain in both legs for a month prior to admission; this radiating tube was more severe in the right than in the left leg. The patient underwent a lumbar spine MRI at another hospital (
After surgery, the radiating pain was improved and the tingling sensation was reduced. The patient visited the outpatient department a month after discharge, and she was examined via EMG and NCV. The results showed that radiculopathy remained but in a much improved state. Additional follow-up was needed because approximately 50% of patients with recurrent disease experience distant metastasis. Histopathological examination of the mass revealed that the paraganglioma was a relatively well-circumscribed highly vascularized tumor of monotonous cells, showing cell ball or papillary pattern.
The patient’s symptoms improved dramatically after surgery, and follow-up MRI images showed no residual tumor. Against the initial expectation, histopathological examination of the mass revealed that it was a paraganglioma (
Paragangliomas are rare neuroendocrine tumors that produce catecholamines, and 80% to 90% of cases happen in the carotid body and jugular tissue
In about half of paraganglioma tumors, abnormal cells produce hormones such as catecholamines or adrenaline, which may induce high blood pressure, rapid heartbeat, flushed skin, sweating, headache, and tremors. However, spinal paraganglioma causes back pain, radiculopathy, and sometimes myelopathy in cervical or thoracic areas. Therefore, it is very difficult to diagnose the disease solely by symptoms.
Preoperative diagnosis is difficult with radiological tools. MRI of paragangliomas in the cauda equina region are nonspecific and relatively isointense on T1-weighted images (WIs) and hyperintense on T2-WIs
The treatment for paragangliomas is usually surgical resection. After total removal, the prognosis is relatively good, however, paragangliomas are highly vascularized tumors, which makes total surgical resection difficult. The preoperative embolization of tumor-feeding vessels may be performed to minimize intraoperative bleeding
We described 2 cases of cauda equina paragangliomas. In each, against the initial diagnosis, paraganglioma was diagnosed through histologic examination; it should be noted that paragangliomas are difficult to diagnose radiologically. Therefore, we must not forget that paraganglioma may be the cause of cauda equina.
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The 3.1 cm size well-defined enhancing solid mass at intradural space of central spinal canal at the level of L2/3. Adjacent 2.4 cm size non-enhancing cystic lesion with fluid-fluid level, suggesting hemorrhage at superior to the enhancing mass at level of L2.
The 1.6×1.7×1.2-cm well-defined enhanced solid mass in the intradural space of the central spinal canal at the L2/3 level with dense calcification of about 1.1 cm.
Microscopically, the tumor shows an encapsulated solid mass with ossification (A) and an anastomosing trabecular pattern with prominent vascularity (B). The tumor is composed of small, relatively uniform nests of cells (C). Immunohistochemically, the tumor cells disclose diffuse positive staining for CD56 (D).