Abstract
A 41-year-old man without clinical symptoms was referred for treatment of an enlarging retroperitoneal tumor. Enhanced computed tomography showed a well-defined and heterogeneously enhanced tumor, 4 cm in size, in the dorsal portion of the pancreas. A low-density nodule was detected in the left adrenal gland, 10 mm in diameter. Retroperitoneal sarcoma and nonfunctional left adrenal tumor were suspected, and surgical treatment was performed. During excision of the retroperitoneal tumor, blood pressure was extremely elevated when the tumor was compressed. Blood pressure normalized after excision of the tumor; thus, a diagnosis of paraganglioma was favored over that of retroperitoneal sarcoma. The left adrenal gland was resected together with the adrenal tumor. Microscopically, the tumor cells of the retroperitoneum had round to oval nuclei, and abundant granular amphophilic cytoplasm proliferated in nest-like fashion. Extra-adrenal retroperitoneal paraganglioma was considered, and the adrenal tumor was diagnosed as cortical adenoma. In patients with retroperitoneal tumor, even in the absence of clinical symptoms, we should keep in mind the possibility of extra-adrenal paraganglioma.
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References
Rosai J. Adrenal gland and other paraganglia. In: Rosai and Ackerman’s surgical pathology. 9th ed. St. Louis: Mosby; 2004. p. 1115–62.
Tohya T, Yoshimura T, Honda Y, Miyoshi J, Okamura H. Unsuspected extra-adrenal pheochromocytoma simulating ovarian tumor. Eur J Obstet Gynecol Reprod Biol. 1999;82:217–8.
Ghidirim G, Rojnoveanu G, Mishin I, Gutsu E, Iakovleva I. Extra-adrenal nonfunctional retroperitoneal paraganglioma: case report and review of the literature. Int Surg. 2005;90:275–8.
Yamaguchi T, Tada M, Takahashi H, Kagawa R, Takeda R, Sakata S, et al. An incidentally discovered small and asymptomatic para-aortic paraganglioma. Eur Surg Res. 2008;40:14–8.
Thompson L. Pheochromocytoma of the adrenal gland scaled score (pass) to separate benign from malignant neoplasms: a clinicopathologic and immunophenotypic study of 100 cases. Am J Surg Pathol. 2002;26:551–6.
Lack EE. Paragangliomas. In: Mills SE, Carter D, Greenson JK, Oberman HA, Reuter V, Stoler MH, editors. Sternberg’s diagnostic surgical pathology. Philadelphia: Lippincott Williams & Wilkins; 2004. p. 679–96.
Disick G, Palese M. Extra-adrenal pheochromocytoma: diagnosis and management. Curr Urol Rep. 2007;8:83–8.
Hayes W, Davidson A, Grimley P, Hartman D. Extraadrenal retroperitoneal paraganglioma: clinical, pathologic, and ct findings. AJR Am J Roentgenol. 1990;155:1247–50.
Motta-Ramirez G, Remer E, Herts B, Gill I, Hamrahian A. Comparison of ct findings in symptomatic and incidentally discovered pheochromocytomas. AJR Am J Roentgenol. 2005;185:684–8.
Sahdev A, Sohaib A, Monson J, Grossman A, Chew S, Reznek R. Ct and mr imaging of unusual locations of extra-adrenal paragangliomas (pheochromocytomas). Eur Radiol. 2005;15:85–92.
Cadden I, Atkinson A, Johnston B, Pogue K, Connolly R, McCance D, et al. Cyclooxygenase-2 expression correlates with pheochromocytoma malignancy: evidence for a bcl-2-dependent mechanism. Histopathology. 2007;51:743–51.
Feldman J. Surgery in patients with unsuspected pheochromocytomas. J Surg Oncol. 1989;41:65–9.
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Uchiyama, S., Ikenaga, N., Haruyama, Y. et al. Asymptomatic extra-adrenal paraganglioma masquerading as retroperitoneal sarcoma. Clin J Gastroenterol 3, 13–17 (2010). https://doi.org/10.1007/s12328-009-0118-4
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DOI: https://doi.org/10.1007/s12328-009-0118-4