Prostatic Paraganglioma: 5-YEAR Followup

0022-5347/99/1616-1909/0 THEJOURNALOF UROLOGY Copyright 0 1999 by AMERICAN UROLOCICAL ASSOCIATION, INC

Vol. 161, 1909-1910.June 1999 Printed in U S A .

PROSTATIC PARAGANGLIOMA: 5-YEAR FOLLOWUP RAFAEL E. JIMENEZ, M I TIGUERT, JOHN F. HAFtB, WAEL SAKFt, J . EDSON PONTES

AND

DAVID J. GRIGNON From the Departments of Pathology and Urology, Harper Hospital, The Karmanos Cancer Institute and Wayne State University, Detroit, Michigan

KEY WORDS: prostatic neoplasms, paraganglioma, pheochromocytoma Paraganglioma of the prostate is an uncommon entity, with only 8 cases reported in the literature.'-3 All patients have been younger than 37 years old. We report a new case of this unusual entity. CASE REPORT

A 17-year-old man complaining of perineal pain during defecation and urination for 6 months was found to have an indurated left prostatic lobe on digital rectal examination. The urinary level of vanillylmandelic acid was 3 times higher than normal, and serum levels of dopamine, norepinephrine and epinephrine were elevated. Endorectal ultrasound identified a 3 cm. heterogeneous mass involving the entire left aspect of the prostate and biopsy of the mass revealed paraganglioma with no feature that could exclude or confirm malignancy. Pelvic lymph node dissection and radical retropubic prostatectomy were performed after 3 weeks of a-adrenergic blocker phenoxybenzamine to prevent intraoperative complications due to catecholamine release. The 48 gm. radical prostatectomy specimen showed a well circumscribed tumor involving the left side and extending from the apex to the base (fig. 1). Microscopic examination revealed an infiltrative neoplasm composed of cells with abundant, granular cytoplasm and rounded nuclei with minor pleomorphism arranged in well demarcated nests (fig. 2). Focally a more infiltrative pattern, multiple microscopic tumor satellite foci and prominent perineural invasion were present. Mitoses were uncommon. Extraprostatic extension of the tumor as well as involvement of the margin of resection was documented. Examination of multiple regional lymph nodes revealed 3 left iliac nodes with metastatic tumor. The patient underwent a course of adjuvant chemotherapy with vincristine, cyclophosphamide and cisplatin. At 5-year followup evaluation was negative for any recurrent disease. DISCUSSION

Extra-adrenal pheochromocytoma, or paraganglioma, is an uncommon tumor in the genitourinary tract and most commonly found in the bladder. The 8 previously described patients with prostatic paraganglioma have been young adults or children (mean age 23.6 Metastases t o the retroperitoneal lymph nodes have been reported in 2 cases,3 but distant metastases have not been described. Diagnosis was based on imaging andor serological studies in 6 cases and on biopsy in 2. Radical or partial resection has been the most common therapy, except for malignant cases in which themotherapy was used. Urethral paragangliomas are also unusual lesions, with only 6 cases reported in the literature: 5 of which have occurred in the prostatic urethra. They usually Occur in elderly men who present with obstructive symptoms. Although centered in the lamina propria, invasion of the underlying prostatic tissue was described in 1case. Metastases have not been reported to date. These cases warrant separation from prostatic Paragandiorna as they appear to OCCUr in different age populations and may have different Clinical behavior. Accepted for publication January 15,1999.

FIG.1. Left side of prostate is mostly replaced by ill-defined tumor is present from to base. (arrows).

paranganglioma of the prostate appears to occur in a relatively young population, which facilitates its differentiation from prostatic adenocarcinoma. Thus, the diagnosis of prostatic adenocarcinoma should not be rendered in a young patient without excluding the possibility of paraganglioma. To our bowledge our case represents the third report of paraganglioma of the prostate with metastatic disease, and the first report of the longest followup. Since it is generally believed that in paraganglioma no histopathological feature reliably predicts biological behavior, especially on biopsy, lymph node sampling at the time ofexcision may be indicated for accurate staging and removal of any clinically nondetected metastatic lesion. Additionally, close clinical followup is mandatory to detect early recurrence of metastasis. Our patient has no evidence of disease 5 years after treatment.

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FIG.2. A, paraganglioma cells on left are closely associated with benign prostatic glands on right side. Reduced from x50. B , characteristic abundant granular cytoplasm of paraganglioma cells and occasional mitosis (arrow). Reduced from x200. REFERENCES

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and Wilson, D. D.: Bladder pheochromocytoma with ring calcification. Abdom. Imag., 21: 275, 1996.

3. Shapiro, B., Gonzalez, E., Weissman, A., McHugh, T. and Markel, S. F.: Malignant paraganglioma of the prostate: case

report, depiction by meta-iodobenzylguanidine scintigraphy and review of the literature. J . Nucl. Biol. Med., 41: 36, 1997. 4. Boyle, M., Gaffney, E. F. and Thurston, A.: Paraganglioma of the prostatic urethra. A report of three cases and a review of the literature. Brit. J . Urol., 77: 445,1996.