Extraovarian Steroid Cell Tumor ‘Not Otherwise Specified’ as a Rare Cause of Virilization in Twelve-Year-Old Girl

Abstract

Background: We present a 12-year-old girl with a 5-year history of progressive virilization. Results: Regarding elevated plasma levels of 17-hydroxyprogesterone (17-OHP) and androgens, normal ultrasound and CT scan of ovaries and adrenal glands, the nonclassic form of congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency was presumed the cause of virilization. As the glucocorticoid therapy did not normalize high levels of 17-OHP and androgens, and the DNA analysis did not demonstrate a mutation causing CAH, a laparotomy was performed. Near the right ovary a tumor was found and extirpated. Pathohistological studies determined it to be a rare steroid cell tumor, ‘not otherwise specified’. Within the next months the signs of virilization resolved and menarche occurred. Conclusions: Steroid cell tumor should be considered in differential diagnosis of virilization in childhood. Regarding the age of our patient and pathohistological findings of the tumor, her prognosis is favorable.

Hyperandrogenism, Elevated 17-Hydroxyprogesterone and Its Urinary Metabolites in a Young Woman with Ovarian Steroid Cell Tumor, Not Otherwise Specified: Case Report and Review of the Literature

“…ACTH stimulation test was performed in 10 cases (including the current case) with elevated baseline 17-OHP levels. No response or insignificant changes were seen in eight cases (80%), including the current case [11, 25, 27, 29, 33–35], while positive 17-OHP responses to ACTH stimulation were observed in two cases (20%) (see legend of Table 3 for the definition of a positive response) [30, 31]. The expression of ACTH receptor may explain the positive 17-OHP response to ACTH stimulation in the minority of cases of SCT–NOS, as the expression of ACTH receptor mRNA was demonstrated in the tumoral tissue of one reported case of SCT–NOS with slightly elevated 17-OHP but not in the ovarian control tissue, although no ACTH stimulation test was performed [24].…”

“…Alternatively, it is possible that the 17-OHP levels in these cases were spuriously increased in response to ACTH stimulation due to cross-reactivity of immunoassays with cortisol and/or other cortisol precursors. Therefore, while a significant increase in 17-OHP in response to ACTH stimulation is typical of CAH [38], this may also be seen in ovarian tumors with 17-OHP hypersecretion, predisposing one to make a wrong diagnosis of CAH in these patients [30, 31].…”

Steroid Cell Ovarian Neoplasm, Not Otherwise Specified: A Case Report and Review of the Literature

“…Indeed, prior case reports have demonstrated steroid cell, NOS, as the root cause of heterosexual precocious puberty incorrectly diagnosed as late onset congenital adrenal hyperplasia. Only after the workup for 17-hydroxyprogesterone levels and ACTH stimulation tests were negative and attempted management with glucocorticoid supplementation had failed, was attention turned to the ovary as the possible source of the hyperandrogenism [4]. …”

A rare occurrence of a steroid cell tumor of the pelvic mesentery: a case report

“…The steroid cell tumors could be of ovarian or ectopic adrenal origin, whereas few cases of extraovarian and extra-adrenal origin have been reported in the literature [1,4,5,7]. However, in the majority of the cases, the exact origin of the tumor remains undecided [1].…”