Pigmented extraadrenal paragangliomas

Abstract

Pigmented extraadrenal paragangliomas are unusual neoplasms that have rarely been reported in the literature. The clinical, pathologic, and immunohistochemical features of five cases of pigmented extraadrenal paragangliomas were reviewed. The patients were 2 women and 3 men within the ages of 17 and 56 years (mean age: 36.5). Two neoplasms were located in the lumbar spine, one in the urinary bladder, one in the anterior mediastinum, and one in the retroperitoneum. Clinically, one patient with spinal paraganglioma presented with symptoms of numbness and weakness of the lower extremities whereas the second patient had low back pain of several weeks' duration. The paraganglioma of the bladder occurred in a pregnant woman who had symptoms of dysuria and microscopic hematuria whereas the patient with an anterior mediastinal tumor presented with chest pain. No clinical history was obtained from the patient with the retroperitoneal tumor. None of the patients had a history of hypertension. Grossly, the tumors were described as well-circumscribed, soft, and slightly hemorrhagic, and measured from 2 to 9 cm in greatest dimension. Histologically, the five tumors displayed characteristics similar to those described in these tumors, mainly the presence of an organoid or zellballen growth pattern. In addition, they contained moderate amounts of intracellular melanin pigment that focally obscured the true nature of the lesion. Immunohistochemically, four cases were positive for chromogranin whereas S-100 protein was detected in the sustentacular cells in four cases. Follow-up information ranging from 6 months to 18 years for 3 patients revealed that the patients were alive and well without recurrence or metastasis. One patient with spinal paraganglioma was lost to follow-up, and the patient with mediastinal paraganglioma was a recent case and therefore the behavior of the paraganglioma could not be assessed. The current study expands the morphologic spectrum of extraadrenal paragangliomas and emphasizes the need to consider these tumors in the differential diagnosis of pigmented neoplasms. These findings suggest that the presence of melanin pigment does not alter the behavior of these neoplasms.