Uterine tumors resembling ovarian sex cord tumors are rarely reported with limited imaging findings.The current study reported two case of uterine tumors resembling ovarian sex cord tumors and described the detailed MRI findings,which would provide valuable imaging evidence for the diagnosis of such tumors.
Female ; Humans ; Uterine Neoplasms/diagnostic imaging* ; Ovarian Neoplasms/pathology* ; Sex Cord-Gonadal Stromal Tumors/pathology* ; Magnetic Resonance Imaging

Female ; Humans ; Ovarian Neoplasms/surgery* ; Sex Cord-Gonadal Stromal Tumors/surgery* ; Myxoma/surgery*

Uterine tumors resembling ovarian sex-cord tumors (UTROSCT) are very rare tumors that occur mainly in the uterine fundus of women in reproductive age. These tumors can be classified into group 1 and group 2 by histological results. In group 1, epithelial-like differentiation is partially observed in the tumors. In group 2, sex-cord elements are predominant in uterine mural mass. We experienced UTROSCT group 1 in a 29-year-old woman who complained of severe abdominal pain that started one week after delivery and UTROSCT group 2 case in a 49-year-old woman who complained of dysfunctional uterine bleeding. We report two different types of UTROSCT cases that we experienced.
Abdominal Pain ; Adult ; Female ; Humans ; Metrorrhagia ; Middle Aged ; Sex Cord-Gonadal Stromal Tumors ; Uterine Diseases ; Uterine Neoplasms

The primary function of intraoperative frozen consultation is to provide an as accurate and prompt diagnosis as possible during surgery and to guide the surgeon in further management. However, the evaluation of frozen section (FS) is sometimes difficult because of suboptimal tissue quality and frozen artifacts compared with routinely processed tissue section. The pathologist responsible for the FS diagnosis requires experience and good judgment. Ovarian tumors are a heterogeneous group of tumors including primary surface epithelial tumors, germ cell tumors and sex cord-stromal tumors, secondary tumors, and other groups of tumors of uncertain histogenesis or nonspecific stroma. Intraoperative FS is a very important and reliable tool that guides the surgical management of ovarian tumors. In this review, the diagnostic key points for the pathologist and the implication of the FS diagnosis on the operator’s decisions are discussed.
Artifacts ; Diagnosis ; Frozen Sections ; Judgment ; Neoplasms, Germ Cell and Embryonal ; Sex Cord-Gonadal Stromal Tumors

Gynandroblastoma is an extremely rare sex cord-stromal tumor with both female (granulosa cell tumor) and male (Sertoli-Leydig cell tumor) elements. Juvenile granulosa cell tumors are also very rare and are so named because they usually occur in children and adolescents. A 71-year-old woman with right upper quadrant abdominal pain visited our hospital. Pelvic computed tomography showed a large multilocular cystic mass, suspected to be of ovarian origin. We performed a total abdominal hysterectomy (total abdominal hysterectomy was performed) with bilateral salpingo-oophorectomy. A 13-cm multilocular cystic mass with serous fluid was observed in her right ovary. Upon microscopic examination, the solid component of the mass showed both Sertoli-Leydig cell and juvenile granulosa cell differentiation, which we diagnosed as gynandroblastoma. Gynandroblastoma with a juvenile granulosa cell tumor component is extremely rare and, until now, only six cases have been reported in the English literature. We report the first gynandroblastoma with a juvenile granulosa cell tumor component diagnosed in an elderly patient, along with a literature review.
Abdominal Pain ; Adolescent ; Aged ; Child ; Female ; Granulosa Cell Tumor ; Granulosa Cells ; Humans ; Hysterectomy ; Male ; Ovary ; Postmenopause ; Sex Cord-Gonadal Stromal Tumors

OBJECTIVETo investigate the clinicopathological features of testicular malignant Leydig cell tumor (TMLCT) and improve the non-invasive diagnosis of the disease.

METHODSWe retrospectively analyzed the clinicopathological data on a case of TMLCT, detected the circulating tumor cells (CTC) in the peripheral venous blood, and reviewed the related literature.

RESULTSThe patient, a 47-year-old male, underwent radical orchidoepididymectomy under general anesthesia. Postoperative pathology confirmed the lesion to be TMLCT, which was mainly composed of Leydig cells and suspected with vessel carcinoma embolus. Immunohistochemistry showed the tumor cells to be positive for α-inhibin, Ki67, CD30, vimentin, EMA, and PLAP, but negative for CK, CK7, S100, CD10, SMA, Des, AFP, hCG, CEA, CK19, CD117, Oct-4, LCA, CD20, Pax-5, CD3, and CD43. Two CTCs were detected in the peripheral venous blood. The patient received 3 courses of chemotherapy for retroperitoneal multiple lymph nodes metastasis post-operatively. Subsequent CT imaging manifested no obvious reduction of the retroperitoneal lymph nodes and consequently the patient again underwent retroperitoneal lymphadenectomy and cryoablation. At 8 months after treatment, CT examination revealed notably enlarged retroperitoneal lymph nodes with the right adrenal gland evidently invaded.

CONCLUSIONTMLCT is an extremely rare sex-gonad stromal tumor with high malignancy and poor prognosis, and CTCs may be used for its early diagnosis and prognostic prediction.

Biomarkers, Tumor ; metabolism ; Humans ; Immunohistochemistry ; Leydig Cell Tumor ; drug therapy ; pathology ; surgery ; Lymph Node Excision ; Lymphatic Metastasis ; Male ; Middle Aged ; Neoplastic Cells, Circulating ; Prognosis ; Retrospective Studies ; Sex Cord-Gonadal Stromal Tumors ; drug therapy ; pathology ; surgery ; Testicular Neoplasms ; drug therapy ; pathology ; surgery

Granulosa cell tumors (GCTs) are rare sex cord-stromal tumors that have been studied for decades. However, their infrequency has delayed efforts to research their etiology. Recently, mutations in human GCTs have been discovered, which has led to further research aimed at determining the molecular mechanisms underlying the disease. Mouse models have been important tools for studying GCTs, and have provided means to develop and improve diagnostics and therapeutics. Thus far, several genetically modified mouse models, along with one spontaneous mouse model, have been reported. This review summarizes the phenotypes of these mouse models and their applicability in elucidating the mechanisms of granulosa cell tumor development.
Animals ; Female ; Granulosa Cell Tumor* ; Granulosa Cells* ; Humans ; Mice* ; Models, Animal ; Phenotype ; Sex Cord-Gonadal Stromal Tumors

Ovarian tumors are relatively rare in children and adolescent. The incidence of malignancies in these groups is 1% to 1.5%. The common histologic type is non-epithelial type such as germ cell tumors or sex cord-stromal tumors and only 10% to 17% of those are epithelial tumors. It is important to accurately diagnose in the early these rare tumors for proper staging and treatment to save the patient's life and fertility. We present a case of a 13-year-old girl with a giant ovarian mucinous borderline tumor.
Adolescent ; Child ; Female* ; Fertility ; Humans ; Incidence ; Mucins* ; Neoplasms, Germ Cell and Embryonal ; Ovarian Neoplasms ; Ovary* ; Sex Cord-Gonadal Stromal Tumors

Carcinoma ; pathology ; Endometrial Neoplasms ; pathology ; Female ; Humans ; Neoplasms, Multiple Primary ; pathology ; Ovarian Neoplasms ; pathology ; Sex Cord-Gonadal Stromal Tumors ; pathology

Sclerosing stromal tumor (SST) was first delineated as a distinct ovarian sex cord stromal tumor in 1973 by Chalvardjian and Scully. It is a benign neoplasm, distinguished from other ovarian stromal tumors by the production of collagen and a pseudolobular pattern, and it tends to occur in the second and third decades of life in diagnosed patients. We discovered two rare cases of SST in post-menopausal women which are the topic of this report. These case studies are accompanied by a brief review of the literature.
Collagen ; Female ; Humans ; Menopause ; Ovarian Neoplasms ; Ovary* ; Sex Cord-Gonadal Stromal Tumors