Tumor de células de la granulosa de tipo juvenil en ovario izquierdo / Juvenile granulosa - cells tumor in the left ovary

RESUMEN Se presentó el caso de una adolescente de 14 años, en el servicio de Cirugía Pediátrica del Hospital Pediátrico Docente "Pedro Agustín Pérez" de Guantánamo, que refirió aumento de volumen de todo el abdomen y dolor abdominal difuso. Al examinarla se constató una tumoración visible y palpable en hemiabdomen inferior. Los estudios complementarios imagenológicos mostraron una masa ecogénica, heterogénea que ocupaba hipogastrio, más lateralizada hacia la izquierda. Tras discusión colectiva multidisciplinaria se le realizó oforectomía izquierda y los estudios anatomopatológicos confirmaron la presencia de tumor de células de la granulosa de tipo juvenil en ovario izquierdo. Técnicas quirúrgicas que permitan preservar la capacidad reproductiva a niñas con neoplasias malignas, resultan usadas ahora con frecuencia y garantizan una mayor calidad de vida.

ABSTRACT A 14-year-old girl presented to the pediatric surgery department at the Pediatric Teaching Hospital "Pedro Agustín Pérez" in Guantanamo. She reported a difuse abdominal pain and distention. The examination revealed a visible and palpable tumor in the lower hemiabdomen. Complementary imaging studies showed an ecogenic and heterogeneous mass situated in the left hypogastrium. After a multidisciplinary team meeting, an ophthalmectomy was performed, and anatomopathological studies confirmed the presence of juvenile granulosa cells tumor on the left ovary. Surgical techniques that allow girls with malignant neoplasms to preserve their reproductive capacity are now frequently used and they guarantee higher life quality.

Tumor de las células de granulosa: pubertad precoz en lactante menor de 1 año. Caso clínico / Granulosa cell ovarian tumor: precocious puberty in infant less than 1 year of age. Case report

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.

Tumor de células granulares de la vulva: caso clínico / Granular cell tumor of the vulva: a clinical case

El tumor de células granulares es una neoplasia de la piel y los tejidos blandos muy poco frecuente, benigna y de crecimiento lento, pero con altas tasa de recurrencia. La localización más frecuente en el aparato genital es en la vulva. Se debe realizar diagnóstico diferencial con otras lesiones vulvares, debido al manejo distinto de esta tumoración. El diagnóstico generalmente es histológico, ya que clínicamente es muy difícil diferenciarlo de otras tumoraciones a nivel vulvar. El tratamiento recomendado es la exéresis quirúrgica, con márgenes de seguridad. En caso de bordes afectos, se recomienda realizar una reescisión, por la frecuencia de recurrencia local y porque ésta puede ser el primer indicador de una conducta agresiva. Presentamos un caso clínico a nivel vulvar, con el objetivo de destacar la importancia de realizar un diagnóstico correcto, para un buen manejo clínico y seguimiento. Las formas malignas suponen menos del 3% de estos tumores y son muy agresivas

The granular cell tumor is a neoplasm of the skin and soft tissue very rare, benign and slow growing, but with high recurrence rate. The most frequent location in the genital tract is in the vulva. Differential diagnosis should be made with other vulvar lesions, due to differences in the management of this tumor. The diagnosis is usually histological, since it is clinically difficult to differentiate it from other tumors at the vulvar level. The recommended treatment is surgical excision, with safety margins. In case of affected borders, it is recommended to perform a resection, due to the local recurrence and this may be the first indicator of aggressive behavior. We present a clinical case at the vulvar level, in order to emphasize the importance of making a correct diagnosis, for a good clinical management and follow-up. Malignant forms account for less than 3% of these tumors and are very aggressive.

Tumores malignos sincrónicos de ovario y endometrio / Ovary malignant synchronous tumors and endometrium

Los tumores malignos sincrónicos primarios de ovario y endometrio son raros y se reportan en alrededor de un 5 por ciento y pueden tener muy buen pronóstico cuando son detectados tempranamente. El objetivo del presente trabajo es dar a conocer a la comunidad científica el caso de un paciente con tumores malignos concurrentes de ovario y endometrio poco frecuentes. Se presenta una paciente femenina de 67 años de edad, multípara, de color de piel blanca, a la cual se le diagnosticó un adenocarcinoma de endometrio y en el curso de la cirugía se encontró un tumor sólido de ovario y el diagnóstico histopatológico arrojó un tumor de las células de la granulosa. Los tumores sincrónicos de ovario y endometrio parecen tener mejor pronóstico y sobrevida cuando son de bajo grado y diagnosticados en etapas tempranas(AU)

The primary synchronous malignant ovarian and endometrial tumors are rare and they are reported in around 5 percent. They can have very good prognosis when they are early detected. The aim of this paper is to inform the scientific community the case of a patient with concurrent malignant tumors of ovarian and endometrial infrequent. We present a 67 year old female patient, multiparous, white skin color, who was diagnosed with an endometrial adenocarcinoma. In the course of surgery, a solid ovarian tumor was found. Her histopathologic diagnosis yielded a granulose-cell tumor. Synchronous ovarian and endometrial tumors appear to have a better prognosis and survival when they are low grade and diagnosed at early stages(AU)

Metástasis pulmonar de tumor de células de la granulosa como recurrencia del tumor primario después de diez años del tratamiento quirúrgico / Pulmonary metastasis of granulosa cell tumor as recurrence of primary tumor after 10 years of surgical treatment

Los tumores de células de la granulosa son neoplasias de bajo grado, que corresponden al 2-5% de los tumores malignos del ovario y presentan una baja incidencia. Las manifestaciones clínicas dependerán del tamaño tumoral y de la exposición prolongada a estrógenos producidos por las células neoplásicas. Habitualmente, son tumores unilaterales, sólido-quísticos con focos de hemorragia, constituidos por células pálidas con su característico pliegue nuclear, en un trasfondo fibrotecomatoso, con cuerpos de Call Exner sólo en el 30-60% de los casos. El diagnóstico se realiza con los niveles séricos de estradiol y con exámenes imagenológicos como ecotomografía ginecológica, tomografía axial computarizada o resonancia nuclear magnética. El tratamiento quirúrgico es la elección. Las recurrencias pueden ocurrir años posteriores al diagnóstico inicial y en general son pélvicas. El factor pronóstico más determinante en la evolución de la enfermedad es el estadio clínico al momento del diagnóstico. Se presenta el caso clínico de una paciente postmenopáusica con metástasis pulmonar de tumor de células de la granulosa después de diez años del tratamiento quirúrgico.

The granulosa cell tumors are low grade neoplasias that correspond to 2-5% of all malignant tumors of the ovary with a low incidence in the population. The clinical presentation depends on the tumor size and the long term exposition to estrogens produced by neoplastic cells. They are usually unilateral tumors exhibiting a mixture of cystic and solid areas with bleeding, constituted by pale cells with their characteristic nuclear groove in a fibrotecomatous background, with Call Exner bodies only in 30-60% of cases. The diagnosis is done with serum levels of estradiol and gynecological imaging analysis such as echotomography, Computerized axial tomography and magnetic nuclear resonance. The surgical treatment is the choice. The recurrences could happen years after the first diagnosis and usually are in pelvic area. The most important prognostic factor in the disease progression is the clinical stage at diagnosis. It is presented a case report of a postmenopausal patient with lung metastasis of granulosa cell tumor after ten years of surgical treatment.

Tumor de células de la granulosa asociado a hiperandrogenismo femenino rápidamente progresivo / Granulosa cell tumour associated with rapidly progressive female hyperandrogenism

Reportamos el caso de una mujer de 21 años con hiperandrogenismo rápidamente progresivo de origen tumoral ovárico. La biopsia informó tumor de células de la granulosa y la resección fue curativa. Se analizan los posibles mecanismos por los que un tumor de origen en células de la granulosa pueda sintetizar andrógenos.

We report a 21 year old woman with rapidly progressive hyperandrogenism of ovaric tumoral origin. The biopsy of the tumor reported a granulosa cell tumor and the surgery was curative. We analyze the possible mechanisms implied in the androgen production in the granulosa cells of the tumor.

Clinicopathological aspects and their relation to prognosis in adult-type granulosa cell tumor of the ovary / Aspectos clinicopatológicos e sua relação com o prognóstico em tumor de células da granulosa do tipo adulto do ovário

INTRODUCTION AND OBJECTIVE: The adult granulosa cell tumors (AGCT) correspond to less than 5 percent of ovarian neoplasias. They are considered low malignant potential tumors and may recur after many years. The differential diagnosis must be made with other primary or metastatic ovarian neoplasias. The aim was to analyze clinical and pathological aspects of AGCT and relate them to its evolution. METHOD: in a 10- year (1995-2004) review of the files from University of Campinas Clinical Hospital, Brazil, 20 AGCT cases were found. The clinical records and slides were reviewed and age, symptoms, macro and microscopic aspects, diagnostic staging and recurrence were considered. When there was intraoperative biopsy, its accuracy was evaluated. RESULTS: Age ranged from 27 to 79 years (mean: 53) and the follow-up from 12 to 96 months (mean: 42). The main symptoms were post-menopause bleeding (45 percent), abdominal pain (35 percent) and palpable mass (25 percent). Most tumors were yellowish (60 percent) and the solid aspect (40 percent) was more common than the cystic or solid-cystic. The histological patterns were 40 percent solid, 15 percent macrofollicular and 45 percent combined forms. All of them with low mitotic index. Only three out of nine intraoperative frozen sections were accurately diagnosed. The clinical staging was 13 cases in Ia (65 percent), one case Ic and 6 IIIc. In three out of 14 hysterectomies there was simple endometrial hyperplasia with no atypia. Only the disease staging was significantly associated with recurrence (p < 0.0001). CONCLUSION: ACGT generally occurs after menopause and intraoperative biopsies are commonly inconclusive. Only advanced staging was related to the worst prognosis.

INTRODUÇÃO E OBJETIVO: O tumor de células da granulosa tipo adulto (TCGA) corresponde a menos de 5 por cento das neoplasias ovarianas. São de baixo potencial de malignidade, podem recorrer depois de muitos anos, e o diferencial deve ser feito com outras neoplasias primárias ou metastáticas. Analisamos os aspectos clínicos e patológicos do tumor, relacionando-os à evolução. MÉTODOS: Na revisão de 10 anos dos arquivos do laboratório de Anatomia Patológica do Hospital das Clínicas da Universidade de Campinas (UNICAMP), 20 casos de TCGA foram encontrados. Os prontuários e as lâminas foram revisados e considerados: idade, sintomas, aspectos macro e microscópicos, estádio ao diagnóstico e à recidiva. Quando houve biópsia intraoperatória, sua acurácia foi avaliada. RESULTADOS: A idade variou de 27 a 79 anos (média: 53); o seguimento de 12 a 96 meses (média: 42). Os sintomas principais: sangramento pós-menopausa (45 por cento), dor abdominal (35 por cento) e massa palpável (25 por cento). A maioria era amarelada (60 por cento), o aspecto sólido mais comum (40 por cento) que o cístico ou sólido-cístico. Os padrões histológicos foram: 40 por cento sólido, 15 por cento macrofolicular e 45 por cento de formas combinadas, todos com baixo índice mitótico. Apenas três de nove casos submetidos à biópsia intraoperatória foram diagnosticados corretamente. O estádio clínico foi: 13 casos Ia (65 por cento), um caso Ic e seis, IIIc. Em três de 14 histerectomias analisadas, havia hiperplasia endometrial simples sem atipia. Apenas o estádio da doença foi significativamente associado à recidiva (p < 0,0001). CONCLUSÃO: TCGA geralmente ocorre após a menopausa, as biópsias intraoperatórias são mais comumente inconclusivas e apenas o estádio avançado esteve relacionado com o pior prognóstico.

GnRH Agonist Therapy in a Patient with Recurrent Ovarian Granulosa Cell Tumors

A 65-yr-old woman presented 17 yr status post-hysterectomy with bilateral ovarian salpingo-oophorectomy, attributable to ovarian cancer. She was admitted to our hospital, with multiple cystic liver masses and multiple large seeded masses in her abdomen and pelvic cavity. Histological examination of the pelvic masses demonstrated granulosa cell tumors. After two courses of systemic combination chemotherapy, with paclitaxel and carboplatin, the masses in the abdomen and pelvic cavity increased, and debulking surgery also failed because of peritoneal dissemination with severe adhesion. Finally, she underwent palliative radiotherapy for only the pelvic masses obstructing the urinary and GI tracts, and monthly hormonal therapy with a gonadotrophin-releasing hormone agonist; leuprorelin 3.75 mg IM. Subsequently, multiple masses beyond the range of the radiation as well as those within the radiotherapy field partially decreased. This partial response had been maintained for more than 8 months as of the last follow-up visit. Owing to its long and indolent course and the low metabolic rate of the tumors, advanced or recurrent granulosa cell tumor (GCT) requires treatment options beyond chemotherapy, surgery, and radiotherapy. Hormonal agents may provide another treatment option for advanced or recurrent GCT in those who are not candidates for surgery, chemotherapy, or radiotherapy.

Giant Ovarian Tumor Presenting as an Incarcerated Umbilical Hernia: A Case Report

We report a rare case of a giant ovarian tumor presenting as an incarcerated umbilical hernia. A 61-yr-old woman was admitted to the hospital with severe abdominal pain, an umbilical mass, nausea and vomiting. On examination, a large, irreducible umbilical hernia was found. The woman underwent an urgent operation for a possible strangulated hernia. A large, multilocular tumor was found. The tumor was excised, and a total abdominal hysterectomy and bilateral salphingo-oophorectomy were performed. The woman was discharged 6 days after her admission. This is the first report of incarcerated umbilical hernia containing a giant ovarian tumor within the sac.

Testicular granulosa cell tumor, adult type.

Granulosa cell tumor (GCT) of the adult type is a rare and slow-growing neoplasm in the testis. This tumor may be associated with endocrine manifestations and resemble classic GCT of the ovary morphologically. It has a potential for distant metastasis or recurrence late in the clinical course. We report a case of granulose cell tumor of the testis, adult type.

Ovarian Granulosa Cell Tumor presenting as Meigs' Syndrome with elevated CA125

Herein, a rare case of ovarian granulosa cell tumor, presenting as Meigs' syndrome, with elevated carbohydrate antigen 125 (CA125), is reported. A 69-year-old woman was admitted for the investigation of abdominal fullness and dyspnea. A preoperative examination revealed a huge pelvic tumor and an abdominopelvic magnetic resonance image (MRI) assumed ovarian cancer. A chest computed tomography (CT) scan revealed pleural effusion. A laparotomy confirmed the huge mass to be an ovarian tumor. A total abdominal hysterectomy (TAH), with a bilateral salpingo-oophorectomy (BSO) and partial omentectomy, was performed. Although short-term intrathoracic drainage was required, the hydrothorax and ascites rapidly resolved in the postoperative period.

Cystic granulosa cell tumor of the ovary--a case report.

A case of cystic granulosa cell tumor of ovary in a 32 year old woman is described. The patient presented with abdominal mass without any signs and symptoms of hyperestrinism and virilization. Ultrasonographically and grossly a diagnosis of benign cystadenoma was considered. Histopathology confirmed the diagnosis of cystic granulasa cell tumor.

Granulosa Cell Tumor of Scrotal Tunics: A Case Report

We report a case of adult granulosa cell tumor arising in the scrotal tunics. The patient was a 34-year-old man who presented with right scrotal swelling, first noticed four months previously. Under the initial clinical impression of epididymo-orchitis, antibiotic treatment was instituted but there was no response. The paratesticular nodules revealed by ultrasound and magnetic resonance imaging mimicked intratesticular lesion, and radical orchiectomy was performed. Although several cases of adult testicular granulosa cell tumor, have been reported, the occurrence of this entity in the paratesticular area has not, as far as we are aware, been previously described.

Extraovarian Granulosa Cell Tumor

A 54-year-old woman was admitted to our hospital complaining of postcoital bleeding. Sonography of the abdomen showed a 8.2 x 8.9 cm-sized solid heterogeneous mass occupying the cul-de-sac, which appeared to be in no way connected with the ovary. On exploratory laparotomy, the tumor mass protruded from the posterolateral retroperitoneum of the pelvic cavity and severely replaced the uterus and adnexa with the outer surface being grossly intact. It grossly measured 10 cm in maximal diameter. The histologic features closely resembled those of ovarian granulosa cell tumor. The primary extraovarian granulosa cell tumor is extremely rare such that in the English literature only 7 cases have been reported to date. Of those granulosa cell tumors are especially rare and only two cases have been reported to arise from retroperitoneum. We herein present a case of retroperitoneal granulosa cell tumor with special regard to differential diagnosis from other solid tumors with similar histology.

Un caso de tumor de células de la granulosa del ovario derecho sincrónico con un tumor carcinoide del apéndices cecal / A case of granulosa cell tumour of left ovarian sineronic with carcinoid tumor of cecal appendix

Se trata de un caso de una paciente femenina de 49 años, intervenida con el diagnóstico de tumoración del ovario derecho. En la laparatomía a que fue sometida, se encontró la tumoración confirmando el diagnóstico clínico, pero al hacer una exploración exhautiva del abdomen y sus vísceras, también se detectó una lesión tumoral de aproximadamente un centímetro en el extremo distal del apéndice cecal; se pensó que se trataba de una metátasis del tumor primario del ovario derecho, estableciendo una clasificación quirúrgica como Estadio III. El resultado definitivo de anatomía patológica determinó que se trataba de un tumor de células de la Granulosa del ovario derecho y otro tumor primario carcinoide del apéndice. Es de hacer notar que al revisar la bibliografía nacional, no hemos encontrado ningún trabajo publicado hasta ahora que reporte otro caso similar

Tumor de la célula granulosa juvenil del ovario / Young granulous cell tumor of the ovary

Los tumores derivados del estrome gonadal diferenciado corresponden aproximadamente al 5 por ciento de las neoplasias ováricas y dentro de éstos, los tumores de las células de la granulosa (TCG) son los más frecuentes. Existen dos variedades de TCG, siendo la variedad juvenil una entidad histopatológicamente diferente y la menos común. El propósito de éste trabajo es mostrar nuestra experiencia en tres casos de tumores de las células de la granulosa juvenil (TCGJ), tanto en los aspectos clínicos, terapéuticos, como asimismo en los hallazgos estructurales e inmunohistoquímicos encontrados

Tumor de células granulares primitivo do colédoco / Granular cell tumor of the common bile duct

We report a case of a rare bile duct tumor in a 28 year old black woman. The clinical features as well as the biochemical profile were those of an obstructive jaundice. The histological examination disclosed a bile duct granular cell tumor (42 cases in the medical literature). The patient is doing well after a segmentar resection and a Roux-en-y hepatico-jejunostomy

Tumor de células granulosas doble localización en lengua / Granular cell tumor: double localization on the tongue

El tumor de células granulosas es una neoformación de curso habitualmente benigno. Entidad clínica infrecuente y de histología definida. Su localización principal es en mucosa bucal, sobre todo en lengua, piel y tejido celular subcutáneo; menos frecuente en distintos órganos. Se presenta como un tumor único, noduloide, asintomático,duro y pequeño. Presentamos un caso de sexo masculino, caracterizado por tener dos tumores aislados, localizados en ambos bordes de la lengua; uno de aspecto noduloide ulcerado, y el otro tipo placa indurada, de dos años y medio de evolución, doloroso al roce y a la palpación. Ambos fueron extirpados quirúrgicamente, sin recaídas

Forma juvenil de tumor de células de la granulosa en niña lactante / Juvenile granulosa cell tumor in a 1 year old girl

Una niña de 12 meses de edad fue traída a consultar a causa de una gran masa abdominal palpable, pseudopubertad precoz y aumento significativo de los estrógenos totales en el suero, producidas por un tumor ovárico que fue resecado en su totalidad. La masa pesaba 1000 g y el examen histológico demostró que se trataba de un tumor de células de la granulosa juvenil, que constituye aproximadamente 5 por ciento de todos los tumores ováricos en las primeras dos décadas de la vida