RESUMO
Granulosa cell tumor (GCT) is a rare ovarian malignancy that represents only 2-3% of all cases. There are two subtypes of GCT: juvenile/JGCT (5% of cases) and adult/AGCT (95% of cases). This study aimed to describe a series of 6 GCT cases. The 6 study patients were managed from June 2011 to November 2022 in a private oncology clinic located in Teresina (PI), Brazil. At diagnosis, the mean patient age was 47 years, and symptoms in 5 patients (83%) were pelvic pain and/or increased abdominal volume. The majority of the patients (N=4/67%) had no comorbidities or findings related to GCT on physical examination. The mean tumor size was 11 cm. Five (83%) tumors were stage Ia and one tumor (17%) was stage III. Regarding tumor subtype, 5 (83%) were AGCT and 1 (17%) was JGCT. Surgical treatment consisted of unilateral salpingo-ophorectomy in 2 patients (33%), total hysterectomy and bilateral salpingo-ophorectomy in 3 patients (50%), and cytoreduction (suboptimal) in 1 patient (17%). After a mean follow-up period of 62.7 months, 5 patients (83%) are still alive and free of disease. One (17%) died from disease progression after 126 months. In the current study, disease-free overall survival was 83%, in a mean follow-up period of 62.7 months.
Assuntos
Tumor de Células da Granulosa , Estadiamento de Neoplasias , Neoplasias Ovarianas , Humanos , Feminino , Tumor de Células da Granulosa/patologia , Tumor de Células da Granulosa/diagnóstico , Tumor de Células da Granulosa/cirurgia , Pessoa de Meia-Idade , Adulto , Neoplasias Ovarianas/patologia , Neoplasias Ovarianas/diagnóstico , Brasil , Histerectomia , Seguimentos , Procedimentos Cirúrgicos de Citorredução/métodos , Idoso , Estudos Retrospectivos , Dor Pélvica/etiologiaRESUMO
Los tumores de células de la granulosa son tumoraciones ováricas infrecuentes. Hay dos tipos histológicos: adulto y juvenil. Los tumores de células de la granulosa juvenil representan el 5 %, y solamente un 3 % ocurre en mayores de 30 años. Ante la sospecha, el diagnóstico definitivo intraoperatorio es complejo dada su rareza y su fácil confusión con otras neoplasias ováricas. El patrón quístico con células de la granulosa inmaduras, con frecuentes mitosis, la ausencia de cuerpos de Call-Exner y el estudio inmunoquístico lo confirman. Su baja prevalencia dificulta su diagnóstico. El estadio de la enfermedad es el factor pronóstico más importante, y resulta imprescindible una completa resección. El papel de la terapia complementaria no está bien establecido, además los estudios disponibles solamente incluyen un número mínimo de casos, que no diferencian mujeres adultas. El adecuado seguimiento para la detección precoz de una posible recidiva tardía supone un reto clínico(AU)
Granulosa cell tumors are rare ovarian tumors. There are two histological types: adult and juvenile. Juvenile granulosa cell tumors account for 5%, with only 3% occurring in people over 30 years of age. Given the suspicion, the definitive intraoperative diagnosis is complex given its rarity and its easy confusion with other ovarian neoplasms. The cystic pattern with immature granulosa cells, with frequent mitosis, the absence of Call-Exner bodies and the immunocystic study confirm this. Its low prevalence makes it difficult to diagnose. The stage of the disease is the most important prognostic factor, and complete resection is essential. The role of complementary therapy is not well established, and the available studies include only a minimal number of cases, which do not differentiate between adult women. Adequate follow-up for the early detection of a possible late recurrence is a clinical challenge(AU)
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Tumor de Células da Granulosa/diagnóstico por imagem , Exame FísicoRESUMO
O sarcoma de partes moles mais comum na infância é o rabdomiossarcoma. Entretanto a localização ovariana é extremamente rara. Acredita-se que este tumor se origina de células imaturas destinadas a compor o músculo esquelético, porém pode surgir em locais onde tipicamente não há músculo esquelético. O diagnóstico do Rabdomiossarcoma primário de ovário pode causar um dilema entre os clínicos, cirurgiões e patologistas, por se tratar de um tumor muito raro. Após o diagnóstico, é necessária a investigação de possíveis metástases. Este caso trata de uma paciente de 17 anos, submetida a parto cesáreo e, no intraoperatório, foi observado aumento de volume, inespecífico, de ovário direito sendo optado por não abordar naquele momento. De antecedentes pessoais, apresentava ooforectomia esquerda aos 13 anos, por Tumor de células da granulosa juvenil e lobectomia inferior esquerda por malformação adenomatosa cística aos 7 anos. Deu entrada no Pronto Socorro 17 dias após dar à luz com queixa de febre, vômitos e dor abdominal. Foi realizada ultrassonografia de urgência, onde foi visualizada massa sólida em fossa ilíaca direita medindo 14,0 x 11,2 x 10,8 cm. Realizada laparotomia exploradora com anexectomia direita e cito-redução subótima do tumor. O resultado anátomo-patológico demonstrou neoplasia maligna fusocelular com áreas de necrose em ovário. A complementação com o estudo imunohistoquímico concluiu rabdomiossarcoma embrionário. Ela voltou a procurar atendimento no Pronto Socorro dois meses após a abordagem com queixa de vômitos biliosos e epigastralgia. Realizou tomografia computadorizada que identificou recidiva do tumor. Durante a internação, evoluiu com quadro de tromboembolismo pulmonar agudo. Diante disso, foi iniciada terapia com enoxaparina em dose plena e quimioterapia com esquema VAC (Vincristina, Doxorrubicina e Ciclofosfamida). Entretanto, ela apresentou insuficiência de múltiplos órgãos, que culminou com o óbito da paciente. O curso clínico desse caso mostra a rápida progressão e letalidade dessa neoplasia. Além da histopatologia, a idade, o tamanho do tumor, a ressecabilidade, o subtipo histopatológico, a presença de metástase no momento do diagnóstico e a invasão linfonodal influenciam no curso clínico da doença. Palavras-chave: Neoplasias ovarianas. Rabdomiossarcoma. Ovário.
Assuntos
Humanos , Feminino , Adolescente , Neoplasias Ovarianas/cirurgia , Ovário/anormalidades , Rabdomiossarcoma/classificação , Sarcoma/complicações , Vincristina/administração & dosagem , Doxorrubicina/administração & dosagem , Rabdomiossarcoma Embrionário/diagnóstico , Enoxaparina/administração & dosagem , Ciclofosfamida/administração & dosagem , Tumor de Células da Granulosa/mortalidade , Metástase Neoplásica/fisiopatologia , Neoplasias/cirurgiaRESUMO
Estrogen modulates platelet activation and aggregation, and it increases the levels of the von Willebrand factor, factors II, VII, VIII, and X, and of fibrinogen, all of which increase the risk for thromboembolism. We report the case of a 59-year-old woman, postmenopausal for 4 years, not using hormone replacement therapy, who was admitted into the emergency room with shortness of breath and increased abdominal volume. After physical examination and imaging and biochemical tests, she was diagnosed with pulmonary thromboembolism and a large left adnexal tumor. The patient was promptly received full anticoagulation therapy for the pulmonary thromboembolism. High levels of estradiol (810.4 pg/mL), anti-Mullerian hormone (16.39 ng/mL), inhibin (11250 pg/mL), and suppressed FSH (<0.16 IU/L) led to a suspicion of granulosa-cell tumor. After clinical stabilization, she underwent to an exploratory laparotomy with total hysterectomy and bilateral adnexectomy to treat the pelvic tumor. Pathologic report confirmed a granulosa-cell tumor.
Assuntos
Tumor de Células da Granulosa , Neoplasias Ovarianas , Embolia Pulmonar , Estradiol , Feminino , Tumor de Células da Granulosa/complicações , Tumor de Células da Granulosa/cirurgia , Humanos , Inibinas , Pessoa de Meia-Idade , Neoplasias Ovarianas/patologia , Embolia Pulmonar/etiologiaRESUMO
The objective of this study was to describe a case of a granulosa cell tumour (GCT) of incipient formation and to characterize it by its immunohistochemical pattern and hormonal profile. The case presented corresponds to a 7-year-old Holstein cow without reproductive disorders. No alterations were observed at rectal palpation, neither in the ultrasonography nor in the hormonal profile. A GCT concomitant with normal follicular development was diagnosed. Through a panel of immunohistochemical markers, a highly differentiated pattern could be determined in the GCT, which preserves the expression of steroid receptors (ESR1, ESR2 and PR) typical of granulosa cells, but does not express the enzymes for the synthesis of androgens (CYP17A1) and oestrogens (CYP19A1). In addition, the expression of co-regulators of steroid hormone receptors and neuroendocrine markers was described for the first time in a GCT in cattle. These results increase the information about GCTs in cattle before the ovarian function is compromised.
Assuntos
Doenças dos Bovinos , Tumor de Células da Granulosa , Neoplasias Ovarianas , Animais , Bovinos , Doenças dos Bovinos/diagnóstico , Feminino , Tumor de Células da Granulosa/veterinária , Células da Granulosa , Hormônios , Neoplasias Ovarianas/veterinária , EsteroidesRESUMO
Extraovarian granulosa cell tumors (GCTs) develop from ectopic gonadal tissue situated along the embryonal route of the genital ridge. Primary retroperitoneal tumors are extremely rare, with an incidence of 02% -06% and 80-85% probability of malignancy. Only eight such case reports have been published previously. We herein, report a rare case of extraovarian retroperitoneal GCT in a 55-year-old woman who presented with intermittent left lumbar region pain of one-year duration. She had a history of hysterectomy and bilateral salpingo-oophorectomy 8 years ago for uterine leiomyoma. Laparotomy revealed a retroperitoneal mass measuring 8cm x 10cm x 20cm in size, solid cystic with areas of necrosis and hemorrhage. The gross features, classical histopathology, and positive immunostaining of the retroperitoneal mass with inhibin, calretinin, PR, WT1 and immunonegativity for EMA were characteristic of adult-type GCT. Excluding any previous history of primary ovarian GCT in this patient, a de-novo retroperitoneal diagnosis was established.
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Neoplasias Retroperitoneais/patologia , Tumor de Células da Granulosa/patologiaRESUMO
Humanin (HN) is a short peptide involved in many biological processes such as apoptosis, cell survival, inflammatory response, and reaction to stressors like oxidative stress, between others. In the ovary, a correct balance between pro- and anti-apoptotic factors is crucial for folliculogenesis. In the follicular atresia, survival or death of granulosa cells is a critical process. The goal of this study was to evaluate the action of HN on granulosa cell fate. To explore endogenous HN function in the ovary, we used a recombinant baculovirus (BV) encoding a short-hairpin RNA targeted to silence HN (shHN). HN downregulation modified ovarian histoarchitecture and increased apoptosis of granulosa cells. HN was also detected in a granulosa tumor cell line (KGN). Transduction of KGN cells with BV-shHN resulted in HN downregulation and increased apoptosis. On the other hand, treatment of KGN cells with exogenous HN increased cell viability and decreased apoptosis. In summary, these findings indicate that HN is a cytoprotective factor in granulosa cells of antral follicles, suggesting that this peptide would be involved in the regulation of folliculogenesis. Also, this peptide is a cytoprotective factor in KGN cells, and therefore, it could be involved in granulosa tumor cell behavior.
Assuntos
Citoproteção , Tumor de Células da Granulosa/patologia , Células da Granulosa/citologia , Peptídeos e Proteínas de Sinalização Intracelular/metabolismo , Folículo Ovariano/citologia , Ovário/citologia , Fragmentos de Peptídeos/metabolismo , Animais , Feminino , Tumor de Células da Granulosa/metabolismo , Peptídeos e Proteínas de Sinalização Intracelular/antagonistas & inibidores , Peptídeos e Proteínas de Sinalização Intracelular/genética , Folículo Ovariano/metabolismo , Ovário/metabolismo , Estresse Oxidativo , Fragmentos de Peptídeos/antagonistas & inibidores , Fragmentos de Peptídeos/genética , RNA Interferente Pequeno/genética , Ratos , Ratos WistarRESUMO
Granulosa cell tumour, an ovarian neoplasm of stromal origin, is an important tumour related to oestrogenic dominance syndrome and cystic endometrial hyperplasia-pyometra complex. In order to analyse ovarian tumour´s malignant potential, immunohistochemical markers can be used, such as anti-HER2 and anti-Ki-67. The aim of this study was to evaluate the expression of immunohistochemical markers HER-2 and Ki-67 in granulosa cell tumour from bitches´ ovaries. In HER-2 immunomarker analysis using the HercepTest® method, most tumours were classified as 2+ (moderate labelling). Concerning Ki-67 immunomarker, only one case was described as having a high proliferative index. An association was found between immunostained cell percentage by anti-HER-2 antibodies and high pleomorphism, represented by the pattern of follicular/trabecular tumour arrangement. There was no correlation between anti-Ki-67 and anti-HER-2 antibody immunostaining intensities, probably due to only one case with a high Ki-67 index. With an effective protocol for HER-2 and Ki-67 immunohistochemical identification in granulosa cell tumours in bitches, it was possible to characterize this neoplasm proliferation profile.
Assuntos
Tumor de Células da Granulosa/veterinária , Antígeno Ki-67/metabolismo , Neoplasias Ovarianas/veterinária , Receptor ErbB-2/metabolismo , Animais , Biomarcadores Tumorais , Doenças do Cão/patologia , Cães , Feminino , Tumor de Células da Granulosa/patologia , Imuno-Histoquímica , Antígeno Ki-67/genética , Neoplasias Ovarianas/patologia , Receptor ErbB-2/genéticaRESUMO
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.
Assuntos
Adolescente , Neoplasias Ovarianas/diagnóstico , Tumor de Células da Granulosa/diagnóstico , OvariectomiaRESUMO
BACKGROUND Granulosa cell tumor of the ovary is very rare in childhood; its most common clinical manifestation is isosexual precocious puberty. Clinical presentation as acute abdomen due to pain and ovarian torsion is rare, but a granulosa cell tumor must be suspected in a patient with this acute presentation and signs of early puberty. Adult-type granulosa cell tumor is an even rarer occurrence in children. CASE REPORT We report a case of torsion of adult-type granulosa cell tumor of the ovary in a 5-year-old patient with acute abdominal pain and ovarian torsion and highlight the importance of histological diagnosis of this tumor for the therapeutic plan and progression of these patients. CONCLUSIONS Precocious puberty, pain, abdominal distension, and an ultrasonography with suspicion of ovarian torsion are warning signs that may indicate the presence of a gonadal stromal tumor in pediatric patients seen at an emergency unit. These patients require long-term follow-up by a pediatrician and gynecologist because of the potential for late recurrence.