46, XY Disorders of Sex Development ; complications ; genetics ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Middle Aged ; Ovarian Neoplasms ; pathology ; surgery ; Sex Chromosomes ; Sex Cord-Gonadal Stromal Tumors ; pathology ; surgery

The association of intramuscular myxoma and fibrous dysplasia is a rare disease known as Mazabraud's syndrome. We present a case of Mazabraud's syndrome coexisting with a uterine tumor and resembling an ovarian sex cord tumor (UTROSCT). This uterine tumor showed a high mitotic index and cytological atypia. To the best of our knowledge, the coexistence of the two different entities has not been reported in the literature.
Aged ; Biopsy ; Buttocks/pathology/surgery/ultrasonography ; Diagnosis, Differential ; Female ; Fibrous Dysplasia, Monostotic/complications/*diagnosis/surgery ; Humans ; Magnetic Resonance Imaging ; Myxoma/complications/*diagnosis/surgery ; Ovarian Neoplasms/*diagnosis ; Rare Diseases ; Sex Cord-Gonadal Stromal Tumors/*diagnosis ; Syndrome ; Uterine Neoplasms/complications/*diagnosis/surgery

We report here on the multiple genital tract neoplasms in a 41-yr-old Korean woman with Peutz-Jeghers Syndrome (PJS). The patient presented with lower abdominal pain. Her previous medical history was PJS and breast cancer. Pelvic ultrasound showed a multilocular cyst at the right adnexal region, diagnosed as bilateral ovarian mucinous borderline tumors. An ovarian sex cord tumor with annular tubules was incidentally diagnosed together with a minimal deviation adenocarcinoma of the uterine cervix and mucinous metaplasia of both the Fallopian tubal mucosa and the endometrium. Although the cases of multiple genital tract tumors with PJS has rarely been reported, the present case appears to be the first in Korea in which the PJS syndrome was complicated by multiple genital tract tumors and infiltrating carcinoma of the breast. The clinical significance of the multiple genital tract tumors and breast cancer associated with PJS is reviewed.
Uterine Cervical Neoplasms/complications/*pathology ; Sex Cord-Gonadal Stromal Tumors/complications/pathology ; Peutz-Jeghers Syndrome/complications/*pathology ; Ovarian Neoplasms/complications/*pathology ; Metaplasia ; Korea ; Humans ; Female ; Fallopian Tubes/pathology ; Endometrium/pathology ; Carcinoma, Ductal, Breast/complications/pathology ; Breast Neoplasms/complications/*pathology ; Adult ; Adenocarcinoma/complications/pathology

Adenocarcinoma ; complications ; metabolism ; pathology ; Adult ; Carcinoembryonic Antigen ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Ki-67 Antigen ; metabolism ; Ovarian Neoplasms ; complications ; metabolism ; pathology ; Peutz-Jeghers Syndrome ; complications ; metabolism ; pathology ; Proliferating Cell Nuclear Antigen ; metabolism ; Sex Cord-Gonadal Stromal Tumors ; complications ; metabolism ; pathology ; Uterine Cervical Neoplasms ; complications ; metabolism ; pathology

Steroid cell tumor, not otherwise specified (NOS), are rare ovarian tumor, in addition, it is more rare in children. The majority of these tumors produce several steroid hormones, particularly testosterone. Estrogen also secreted by steroid cell tumor, NOS, but it is uncommon. Furthermore, hypertension is an infrequent sign in steroid cell tumor, NOS. An 8.5-yr-old girl with hypertension and frequent vaginal spotting visited at our clinic. On laboratory evaluation, secondary hypertension due to an elevated plasma renin level and isosexual pseudoprecocious puberty was diagnosed. Right solid ovarian mass was detected in radiologic tests. She underwent a right ooporectomy and it revealed renin and progesterone receptor positive steroid cell tumor, NOS. After operation, her blood pressure returned to normal level and vaginal bleeding disappeared. Even though this case is very rare, when hypertension coincides with virilization or feminization, a renin-secreting ovarian steroid cell tumor, NOS, should be considered.
Child ; Female ; Humans ; Hypertension/*etiology ; Ovarian Neoplasms/complications/*diagnosis/pathology ; Puberty, Precocious/enzymology/*etiology ; Receptors, Cell Surface/metabolism ; Receptors, Progesterone/metabolism ; Renin/blood ; Sex Cord-Gonadal Stromal Tumors/complications/*diagnosis/pathology ; Steroids/biosynthesis ; Tomography, X-Ray Computed ; Vacuolar Proton-Translocating ATPases/metabolism