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Juvenile granulosa cell tumor associated with Maffucci syndrome in pregnancy: A case report.

08:00 EDT 13th March 2019 | BioPortfolio

Summary of "Juvenile granulosa cell tumor associated with Maffucci syndrome in pregnancy: A case report."

Juvenile granulosa cell tumor (JGCT) is an extremely rare ovarian tumor that has been associated with Maffucci syndrome. It both secretes hormone and has been postulated to grow in response to hormone. We present a case of a 33-year-old G1P0 asymptomatic woman with a history of Maffucci syndrome found to have a left adnexal mass on routine ultrasonography at 13 weeks gestation. This case demonstrates the sonographic and magnetic resonance imaging (MRI) features of JGCT, as well as the natural progression of the tumor during pregnancy. A follow-up ultrasound 3 weeks after initial diagnosis demonstrated marked growth in size and vascularity of the tumor, prompting unilateral salpingo-oophorectomy. Histopathological findings confirmed the diagnosis of JGCT.

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This article was published in the following journal.

Name: Clinical imaging
ISSN: 1873-4499
Pages: 77-80

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Medical and Biotech [MESH] Definitions

A neoplasm composed entirely of GRANULOSA CELLS, occurring mostly in the OVARY. In the adult form, it may contain some THECA CELLS. This tumor often produces ESTRADIOL and INHIBIN. The excess estrogen exposure can lead to other malignancies in women and PRECOCIOUS PUBERTY in girls. In rare cases, granulosa cell tumors have been identified in the TESTES.

Neoplasms derived from the primitive sex cord or gonadal stromal cells of the embryonic GONADS. They are classified by their presumed histogenesis and differentiation. From the sex cord, there are SERTOLI CELL TUMOR and GRANULOSA CELL TUMOR; from the gonadal stroma, LEYDIG CELL TUMOR and THECOMA. These tumors may be identified in either the OVARY or the TESTIS.

Gonadal neoplasm composed entirely of SERTOLI CELLS or may have a component of GRANULOSA CELLS. Some of the Sertoli cell tumors produce ESTROGEN or ANDROGENS, but seldom in sufficient quantity to cause clinical symptoms such as FEMINIZATION or masculinization (VIRILISM).

A rare syndrome characterized by UROGENITAL ABNORMALITIES; GONADAL DYSGENESIS; PSEUDOHERMAPHRODITISM; and WILMS TUMOR. It is caused by a mutation in the Wilms tumor suppressor gene (GENES, WILMS TUMOR) on chromosome 11.

Arthritis in children, with onset before 16 years of age. The terms juvenile rheumatoid arthritis (JRA) and juvenile idiopathic arthritis (JIA) refer to classification systems for chronic arthritis in children. Only one subtype of juvenile arthritis (polyarticular-onset, rheumatoid factor-positive) clinically resembles adult rheumatoid arthritis and is considered its childhood equivalent.

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