Cushing’s syndrome caused by an ACTH-producing ovarian steroid cell tumor, NOS, in a prepubertal girl

Abstract

Ectopic ACTH syndrome is a very rare cause of pediatric Cushing's syndrome. And if present, bronchial or thymic carcinoids predominate as causes. We hereby demonstrate a first case report of ACTH-producing ovarian steroid cell tumor, NOS, causing ectopic ACTH syndrome in a prepubertal girl.

Ovarian steroid cell tumor, not otherwise specified: A clinicopathological and immunohistochemical experience of 12 cases

“…Seidman et al . described an immunohistochemical study of 28 SCT and a substantial number of SCT were positive for cytokeratin Cam 5.2 (46%) and AE1/3 (37%) which was not seen in our observation . Jones et al .…”

“…Seidman et al described an immunohistochemical study of 28 SCT and a substantial number of SCT were positive for cytokeratin Cam 5.2 (46%) and AE1/3 (37%) which was not seen in our observation. 17 Jones et al also reported that 36% of tumors showed positive expression for AE1/AE3. 18 Although many other published studies have also shown variable diffuse positivity for cytokeratin Cam 5.2 and EMA, none of our cases showed positivity for these immunostains, 13,[17][18][19] and even cytokeratin AE1/AE3 was focally positive in only two cases.…”

Ovarian Steroid Cell Tumor Associated to Endometrial Hyperplasia and Presenting as Postmenopausal Vaginal Bleeding

“…The mean age of diagnosis is 58 years for stromal luteomas and Leydig cell tumors [2], and usually affects younger women for adrenal cortical SCT [2,[8][9][10] and SCT NOS [2,11]. Adrenal-cortical and SCT NOS can present prior to puberty [2,3,8,11], and have even been described in fetuses [12]. In prepuberal girls it can cause isosexual precocity [13,14], with hirsutism, acne, growth-acceleration, clitoral enlargement and pubic hair.…”

A rare occurrence of a malignant ovarian steroid cell tumor not otherwise specified: A case report and literature review

“…The malignant cells show marked atypia, mitoses, necrosis and hemorrhage. The literature indicates that 25–43% of steroid cell tumor cases are clinically malignant, with 20% of cases found to exhibit metastases outside of the ovary during surgery (2,7). If metastasis is present, ~20% of metastatic lesions usually occur within the peritoneal cavity and rarely occur at distant sites.…”