ABSTRACT
Resumen Introducción: Los tumores de las células de la granulosa de tipo juvenil (TCGJ) son muy poco fre cuentes, especialmente en menores de 1 año. Los signos de pubertad precoz constituyen la presenta ción clínica más importante. Objetivo: Presentar una lactante con pubertad precoz periférica, con diagnóstico de TCGJ, discutiendo las claves de su tratamiento y seguimiento. Caso Clínico: Lactante de 10 meses que presentó telarquia, vello púbico y tumor abdominal palpable acompañado de niveles plasmáticos de Estradiol aumentados, gonadotrofinas muy bajas e imágenes que mostraban masa ovárica gigante. Se realizó salpingooforectomía, obteniéndose regresión absoluta de signos y síntomas. La biopsia demostró TCGJ por lo que se tomó inhibina B (InB) como marcador después de la cirugía. Esta hormona estaba alta inicialmente, pero descendió rápidamente. El seguimiento se basó en InB, Hormona antimulleriana (AMH) y estradiol como se describe en este tipo de tumores. Conclusiones: Los TCGJ son muy infrecuentes en pediatría; deben sospecharse en niñas con puber tad precoz periférica. El tratamiento quirúrgico en la gran mayoría es curativo, pero debe mantenerse un estricto control con marcadores tumorales, siendo los más específicos la InB y la AMH y en menor escala los niveles de Estradiol.
Abstract Introduction: Juvenile granulosa cell tumors (JGCT) are very rare, especially in infants under the age of one. The most frequent presentation is with signs of precocious puberty. Objective: Present an in fant with peripheral precocious puberty, diagnosis of JGCT and follow up. Clinical case: 10-month-old female infant with thelarche, pubic hair and palpable abdominal mass accompanied with eleva ted levels of estradiol, very low gonadotrophins and images that show a very large ovarian mass. A sapingooforectomy was carried out with full regression of symptoms and signs and improvement of laboratory exams. The biopsy showed TCGJ so inhibin B (InB) was taken as tumoral marker after surgery. This hormone was high initially, but rapidly declined. Follow-up was based on InB, antimu-llerian Hormone (AMH) and estradiol as described in this type of tumors. Conclusions: Juvenil gra nulosa cell tumors are very infrequent in pediatric age, but should be suspected in girl with peripheral precocious puberty. The majority of cases improve with surgery, but strict surveillance of tumoral markers is needed. The most specific markers are inhibin B and anti mullerian hormone (AMH), followed by estradiol levels.
Subject(s)
Humans , Female , Infant , Ovarian Neoplasms/diagnosis , Puberty, Precocious/etiology , Granulosa Cell Tumor/diagnosis , Ovarian Neoplasms/complications , Granulosa Cell Tumor/complicationsABSTRACT
To determine the clinicopathologic prognostic factors in adult granulosa cell tumors [GCTs] of the ovary. This retrospective study was carried out over a period of 10 years [1995-2005] in the Gynecology Department of Shengjing Hospital, China Medical University, Shenyang, China. Forty-six patients with GCT were enrolled in this study. Demographic data, pathologic findings, treatments, and survival time were reviewed and analyzed for prognostic significance. It was found that International Federation of Gynecology and Obstetrics [FIGO] stage [p=0.0003], presence of nuclear atypia [p=0.036], and increased mitoses [p=0.002] were the 3 factors that impacted significantly on survival. Age, residual tumor disease, parity, and size of the tumor had no significant effect on survival. The only factor associated with risk of recurrence was rupture of the tumor [p=0.038]. Patients who received chemotherapy had a better median disease-free survival than those who did not [105 versus 78 months], however, this did not reach statistical significance [p=0.080]. The FIGO stage, nuclear atypia, and increased mitoses are the statistically significant prognostic factors, and may be used for selecting patients for adjuvant therapy. A prolonged follow-up is necessary due to risk of recurrences, late, and exceptional for the adult ovarian GCT, especially when the tumor ruptured before, or at operation
Subject(s)
Humans , Female , Granulosa Cell Tumor/complications , Ovarian Neoplasms , Prognosis , Adult , Retrospective StudiesABSTRACT
Ovarian tumor with clinical manifestations like hirsutism, atrophic uterus, flattened breasts and absence of post-menopausal bleeding and atrophic endometrium was reported as an adult granulosa cell tumor (GCT) on histopathological examination, is discussed.
Subject(s)
Female , Granulosa Cell Tumor/complications , Hirsutism/diagnosis , Humans , Middle Aged , Ovarian Neoplasms/complicationsABSTRACT
Os tumores de estroma ovariano são responsáveis por 5 a 8 por cento dos tumores ovarianos. Sua manifestação clínica inicial mais comum em meninas pré-puberais é o desenvolvimento de puberdade precoce. Neste artigo discutimos o caso de uma menina de 7 anos e 2 meses com tumor de estroma ovariano do tipo misto - cordões-sexuais-células germinativas -, cuja manifestação inicial foi puberdade precoce isossexual, de evolução rápida e progressiva. Os níveis séricos de testosterona, estradiol e 17alfaOH-progesterona encontravam-se elevados. A ecografia pélvica-abdominal demonstrou massa anexal direita. Salpingo-ooforectomia unilateral foi realizada com completa ressecção tumoral. A paciente encontra-se bem 7 anos após a cirurgia com crescimento e desenvolvimento puberal normal, sem evidência de recidiva tumoral. São revistas as principais manifestações clínicas dos tumores ovarianos, sua classificação e o estadiamento dos tumores de estroma ovariano/cordão sexual, seu seguimento empregando diversos marcadores tumorais e hormonais. Finalmente é analisado o tratamento e prognóstico destes pacientes.