We report a case of extraadrenal retroperitoneal paraganglioma in a 24-year-old female. This case is unusual in that the neoplasm showed extensive calcification, osseous metaplasia, and marrow elements. Although calcification may occur in paragangliomas, to the best of our knowledge, osseous metaplasia with marrow elements have not been previously reported in the English literature. Int J Surg Pathol 4(3):00- 00, 1997 Extraadrenal retroperitoneal paraganglioma is a distinct neoplasm that has characteristic clinical and pathological features. Approximately 10-20% of retroperitoneal paragangliomas arise outside the adrenal gland, and a majority of these tumors arise from the organ of Zuckerkandl located near the inferior mesenteric artery [ 1 ] . These neoplasms may produce catecholamines and present with hypertension, arrythmias, headache, and diaphoresis. Nonfunctional tumors commonly present with pain or abdominal mass, and correct diagnosis is infrequently made before surgery. We present an unusual case of extraadrenal paraganglioma with extensive calcification, osseous metaplasia, and marrow formation.
Case ReportA 24-year-old female presented with complaints of right flank and lower abdominal pain that began 1 month after completion of an uncomplicated fullterm pregnancy. She denied weight loss, anorexia, hematuria, flushing, headaches, or palpitations. The patient was normotensive, and no abdominal masses were palpable. Intravenous pyelogram, a computed tomography (CT) scan, and magnetic resonance imaging revealed a IOx6 cm calcified mass located medial to the upper pole of the right kidney, lateral to the inferior vena cava and posterior to the head of the pancreas (Fig. 1). No visceral or bone metastases were present. Preoperative urine catecholamine, vanillylmandelic acid (VMA), and metanephrine levels were normal. She undervent surgery 5 months after the onset of her symptoms.The tumor was approached through a flank incision and found to be highly vascular. It was fairly well demarcated and not adherent to adjacent structures and was removed in its entirety. The patient remained hemodynamically stable throughout the operation. Her postoperative course was uneventful, and she was discharged home on the sixth postoperative day.Gross pathology revealed an oval, slightly lobulated firm mass that was covered by a gray-white capsule and measured 8x4x4 cm and weighed 42 g. The cut surface was gray-yellow, lobulated, and contained large areas of calcification (Fig. 2). A positive chromaffin reaction was observed by demonstration of dark brown discoloration of the tumor upon immersion in dichromatc solution. Histologiat UNIVERSITE DE MONTREAL on August 17, 2015 ijs.sagepub.com Downloaded from