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 salpingooophorectomy. 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.
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Case of Gynandroblastoma of the Ovary with Raised AFP and Associated DICER 1 Mutation Dipak Limbachiya, Rajnish Tiwari, Rashmi Kumari, Priti Trivedi The Journal of Obstetrics and Gynecology of India.2024;[Epub] CrossRef
Ovarian cancer in children and adolescents: A unique clinical challenge Marina Jakimovska Stefanovska, Aleksandar Celebic, Jean Calleja-Agius, Kristina Drusany Staric European Journal of Surgical Oncology.2024; : 108785. CrossRef
Ovarian Gynandroblastoma with a Juvenile Granulosa Cell Tumor Component in a Postmenopausal Woman Soohyun Hwang, Byoung-Gie Kim, Sang Yong Song, Hyun-Soo Kim Diagnostics.2020; 10(8): 537. CrossRef
Clinical and histological criteria for sex cord ovarian stromal tumors A. М. Beishembaev, K. I. Zhordania Obstetrics, Gynecology and Reproduction.2020; 14(3): 261. CrossRef
Juvenile granulosa cell tumor is rare but one of the common congenital testicular neoplasms. Although histological features are typical of its ovarian counterpart, testicular juvenile granulosa cell tumor has a distinctly different clinical presentation. We report a case of juvenile granulosa cell tumor arising in the cryptochid testis of a 4-day-old newborn. A 6 5 5 cm sized multilocular cyst containing thick, mucinous fluid was found in the peritoneal cavity. The external surface of the cyst was smooth and the septae were relatively thin. The cyst consisted of numerous mucin-filled, cystic follicles lined by cells having vacuolated cytoplasm and round to oval dark nuclei without grooves. Cells resembling granulosa cells of an ovarian follicle were also observed in the intervening stroma forming irregular solid nests.
Granulosa cell tumor of the testis is extremly rare in adult males and granulosa cell tumor occurring in the paratesticular area has not been reported. We report a paratesticular granulosa cell tumor in a 34-year-old man who presented with a 5.5 cm sized scrotal mass. The tumor was present in the paratesticular area near the head of epididymis. It was located in the tunica and completely separated from the testis by thick fibrous tissue. Microscopically, the tumor exhibited solid, microfollicular, and trabecular patterns. The tumor cells had ovoid to elongated nuclei with longitudinal intranuclear grooves and one or two nucleoli and scanty cytoplasm. Mitoses were relatively frequent with an average number of 9/10 HPFs. The tumor extended into the visceral tunica vaginalis and showed multiple lymphatic tumor emboli. Immunohisto chemical stains revealed diffuse strong positivity for inhibin, vimentin, and keratin and diffuse weak positivity for estrogen and progesteron receptor. Placental alkaline phosphatase (PLAP) and epithelial membrane antigen (EMA) were negative. On electron microscopic examination, tumor cells were polygonal and had large indented nuclei. The cytoplasm contained a moderate number of small round mitochondria, abundant rough and smooth endoplasmic reticula, and a few lipid droplets.
Small aggregates of intermediate filaments and intercellular junctions were observed. The patient was alive and well 5 months after orchiectomy. This is the first case of adult granulosa cell tumor arising in the paratesticular area.
Unilocular cystic granulosa cell tumors (UCG) are extremely rare. Due to the relatively small mass of the tumor available for histologic examination, diagnosis of UCG is not easy. Here we present a case of UCG in a 54-year old female. A 12x10 cm unilocular cystic mass was identified in the right ovary. The tumor was thin-walled and consisted of a single large cavity with a smooth internal surface. In most areas there were no cells lining the cyst, however, in focal areas the unilocular cyst was lined by one or more layers of uniform granulosa cells, forming Call-Exner bodies. A diagnosis of UCG was made after multiple sections were examined. A careful histologic examination is required for the correct diagnosis of UCG.
A case of Juvenile Granulosa Cell Tumor (JGCT) of the ovary with unusual pleomorphic histologic and malignant biologic behaviour is described. The tumor occurred in a 10-year-old girl and was associated with clinical features of isosexual pseudoprecosity and a marked elevation of serum estradiol.
The mass manifested initially in the right ovary and subsequently involved the contralateral ovary. A multi-organ metastasis developed during a 6-month-interval despite chemotherapy. She received two operations at 6-month interval, and tissues were obtained from the tumor mass. A marked histologic difference was observed between these two samples. The second biopsy showed profound cellular pleomorphism with numerous multinucleated tumor giant cell formation and hyaline bodies. The differential diagnosis from germ cell tumor and the possible factors for the pleomorphism are discussed.