Testicular adrenal rest tumors in patients with congenital adrenal hyperplasia: prevalence and sonographic, hormonal, and seminal characteristics. (9/13)

OBJECTIVE: Testicular adrenal rest tumors have been described in patients with congenital adrenal hyperplasia (CAH). The aim of this work was to (1) evaluate the prevalence of testicular adrenal rest tumors in patients with CAH; (2) study the hormonal profile; (3) define the sonographic features; (4) assess the seminal profile; and (5) initiate a longitudinal study on the possible role of corticotropin (ACTH) plasma levels in the induction and persistence of testicular adrenal rest tumors. METHODS: Eighteen patients affected by CAH, aged 21 to 41 years, were studied. These were all patients referred to our endocrinology unit for the first time to undergo a clinical evaluation. All of the patients were taking long-term cortisone acetate and fludrocortisone replacement therapy. The study included (1) a physical examination, (2) testis sonography, (3) a hormonal profile, (4) semen analysis. RESULTS: Sonography showed testicular adrenal rest tumors in 11 patients (61.1%); of these, 9 cases (50.0%) were bilateral, and 2 (11.1%) were unilateral. The diameter ranged from 4 to 38 mm. In 9 patients, the lesions were hypoechoic, whereas in 2, they were hyperechoic. High plasma ACTH levels were detected in all of the patients with tumors despite long-term therapy. Semen analysis found 2 cases of azoospermia and 6 cases of oligoasthenoteratozoospermia; the 3 remaining patients were normospermic. The preliminary longitudinal study has shown 3 patients with a disappearance or reduction of the tumors after 6 months of modified treatment. CONCLUSIONS: This study confirms the high prevalence of testicular adrenal rest tumors in patients with CAH and the major role played in its pathogenesis by high plasma ACTH levels.  (+info)

Testicular adrenal rest tumors in patients with congenital adrenal hyperplasia. (10/13)


Macroorchidism: consequence of untreated congenital adrenal hyperplasia. (11/13)

Testicular adrenal rest tumors (TART) are consequences of delayed diagnosis and/or undertreatment in patients with congenital adrenal hyperplasia (CAH). We describe a case of CAH with TART who presented with bilateral macroorchidism. He was managed with glucocorticoids which led to decrease in testicular size without restoration of spermatogenesis.  (+info)

Non-functioning adrenocortical carcinoma arising in an adrenal rest: immunohistochemical study of an adult patient. (12/13)

Adrenal rest is uncommon in adults, and usually represents a small lesion incidentally detected during surgery or autopsy. The adrenal rest can be detected anywhere along the path of embryonic migration of adrenal cortex, including celiac axis, genitals and broad ligament, and may be formed with the separation of cortical fragments by the migration of medullary elements from the sympathochromaffin tissue into the preformed cortical primordium. In addition, even primary adrenocortical carcinoma is a rare tumor with incidence 0.5-2 per million annually; therefore, adrenocortical carcinoma arising in adrenal rests is extremely rare. We encountered a patient with non-functioning ectopic adrenocortical carcinoma in retroperitoneum. A 34-year-old female presented with an incidentally discovered retroperitoneal mass revealed by abdominal ultrasound in her regular health examinations. She did not have any clinical abnormalities and underwent hand-assisted laparoscopic resection of the tumor. A dark-brown tumor, measuring 65 x 56 x 45 mm, was identified in the retroperitoneal space between lower pole of right kidney and inferior vena cava. Histologically, the tumor was predominantly composed of compact eosinophilic cells forming nest-like arrangements and diffusely positive for the steroidogenic factor-1. The tumor met four of the criteria of Weiss used in histological diagnosis of adrenocortical carcinoma (eosinophillic cytoplasm, nuclear atypia, atypical mitosis, and sinusoidal invasion). The tumor cells were immunohistochemically positive for 17alpha-hydroxylase, dehydroepiandrosterone sulfotransferase and 3beta-hydroxysteroid dehydrogenase, each of which is involved in the synthesis of adrenocortical steroids. Therefore, based on these findings, we diagnosed this tumor as ectopic adrenocortical carcinoma arising in adrenal rest of retroperitoneum.  (+info)

Lipid cell tumor of the ovary. (13/13)

A 32-year-old nulligravida, with a history of abdominal hysterectomy for multiple leiomyomas of the uterus, was seen initially with signs of masculinization. Normal values for 17-ketosteroids, 17-hydroxycorticosteroids, and plasma cortisol eliminated the adrenal as the source of the excess androgen as well as Cushing syndrome. Increased plasma testosterone and androstenedione levels in the peripheral blood as well as in the right ovarian vein sampling implicated the right ovary, which revealed a lipid cell tumor (0.5 to 1.3 cm in maximum dimension).  (+info)