Malignant pheochromocytoma with multiple vertebral metastases causing acute incomplete paralysis during pregnancy: Literature review with one case report

Abstract

Rationale: We present a rare case of malignant pheochromocytoma with thoracic metastases during pregnancy that presented with symptoms of myelopathy and was treated with circumferential decompression, stabilization, and radiation. The management of this unique case is not well documented. The clinical manifestations, imaging results, pathological characteristics, treatment and prognosis of the case were analyzed.

Patient concerns: A 26-year-old pregnant woman with a history of paroxysmal hypertension during the second trimester presented with lower extremity weakness, numbness, urinary incontinence, and back pain. Imaging studies revealed a right adrenal pheochromocytoma, multiple metastases at T8, T11, T12, and the pelvis girdle causing significant multilevel cord compression and significant osteolytic lesions at T11 and T12.

Diagnoses: We believe this is the first reported case of metastatic pheochromocytoma of the thoracic spine presenting with symptoms of myelopathy during pregnancy.

Interventions: A healthy neonate was delivered by emergency caesarean section at 34 weeks. Subsequently, the patient underwent a circumferential spinal cord decompression and a stabilization procedure.

Outcomes: The patient's neurological deficits improved significantly after the surgery, and the postoperative period was uneventful at the 6-month follow-up visit.

Lessons: This article emphasizes that metastatic pheochromocytoma of the spine, although rare, should be part of the differential when a patient presents with elevated blood pressure, weakness, and urinary incontinence.

Figure 1

A, Preoperative sagittal T2-weighted MRI scan revealing several vertebral metastases, pathological vertebral fractures, and multilevel thoracic spinal cord compressions caused by metastatic malignant pheochromocytoma. B, Preoperative sagittal T2-weighted fat-saturated MRI scan revealing vertebral metastases, vertebral fractures, and thoracic spinal cord stenosis. C and D, Preoperative transverse T2-weighted MRI images showing vertebral metastasis and metastatic adrenal pheochromocytoma. MRI = magnetic resonance imaging.

Figure 2

A-H, Positron emission tomography-computed tomography revealed adrenal pheochromocytoma of the right side and multiple metastases of the spine, right kidney, pelvis, and left erector spinae.

Figure 3

A, Intraoperative photography depicting the exposed spinal cord. B, Intraoperative photography depicting partially resected metastatic tumor. C, Posteroanterior (PA) x-ray image of the thoracic spine obtained postoperatively. D, Lateral x-ray image of the thoracic spine obtained postoperatively.

Figure 4

Pathologic histology of spinal metastases. A and B, Microphotography showing characteristic nests of tumor cells separated by vascular septa (Zellballen) with cells showing significant nuclear pleomorphism with prominent nucleoli (Hematoxylin and Eeosin, original magnification ×20 and ×40). C, Chromogranin A immunostaining is strongly positive in the chromaffin cells. Chromogranin A is present in the secretory granules. D, Synaptophysin immunostaining shows strong, diffuse cytoplasmic staining in the tumor cells. E, The sustentacular cells of the spinal metastases of pheochromocytoma showing characteristic staining of S100. F, P-53 immunostaining is sporadically positive. G, Ki-67 immunostaining shows 3% Ki-67 positive cells. Ki-67 staining is localized in the tumor nuclei.