Disgerminoma ovariano na gestação: relato de caso / Ovarian dysgerminoma in pregnancy: a case report

Os disgerminomas são tumores malignos de células germinativas ovarianas, são raros, geralmente acometem mulheres em idade fértil e têm bom prognóstico e sobrevida elevada. Paciente de 20 anos, primigesta com 26 semanas de gestação, foi admitida no centro obstétrico da Fundação Hospitalar Santo Antônio em Blumenau- SC com quadro de dor abdominal intensa refratária à analgesia e desconforto respiratório. Ressonância magnética demonstrou derrame pleural, moderada ascite e volumosa lesão expansiva de aspecto sólido-cístico em anexo direito. Foram realizadas salpingo-oforectomia à direita e omentectomia parcial e coletado lavado peritoneal. Anatomopatológico evidenciou disgerminoma. A paciente seguiu acompanhamento gestacional nos serviços de pré-natal de alto risco e oncologia. Devido à imaturidade fetal, manteve-se conduta expectante e, após o parto normal com 37 semanas, foi realizado estadiamento e iniciada quimioterapia adjuvante. Devido à baixa incidência e à raridade de tumores de células malignas ovarianas, relatos de casos como este são importantes para discutir as melhores estratégias de manejo clínico.(AU)

Dysgerminomas are rare malignant ovarian germ cell tumors that generally affect adolescence and early adulthood, have a good prognosis and high survival. Patient 20 years old, gestation 1, at 26 weeks of gestation, was hospitalized at the obstetric center of Fundação Hospitalar Santo Antônio in Blumenau-SC, with severe abdominal pain refractory to analgesia and respiratory discomfort. Magnetic resonance showed pleural effusion, moderate ascites and a massive expansive lesion with a solid cystic aspect in the right ovary. Right salpingoophorectomy, partial omentectomy and peritoneal lavage were collected. Anatomopathological evidence showed dysgerminoma. Patient followed gestational follow-up at high-risk prenatal and oncology services. Due to fetal immaturity, expectant management was maintained and after vaginal delivery at 37 weeks, staging was performed and adjuvant chemotherapy was started. Due to the low incidence and rarity of ovarian malignant cell tumors, case reports such as this one are important to discuss the best clinical management strategies.(AU)

Germ cell tumours of the ovary.

Germinal cell tumours represent only 2-5% of all cancers of the ovary. However, the characteristics of the tumour and the patients have some special qualities as high rates of healing goes together with a strong desire to keep fertility intact because this condition occurs in female children and adolescent girls. Neither the prognosis nor the treatment of these tumours is homogeneous; the low incidence is the reason it is hard to develop prospective studies for establishing prognostic factors and specific treatments. The introduction of adjuvant chemotherapy into initial surgery has improved the prognosis of these patients. The BEP scheme has proved to be equally efficient and less toxic than PVB, and for this reason it has become the standard scheme despite the fact that no randomised studies have been carried out. The surgical treatment demands the application of the same principles seen in cytoreduction surgery of epithelial cancers of the ovary (maximum possible cytoreduction), though in many cases hysterectomy and double adnexectomy may be obviated. In view of the rarity of these tumours, it is advisable to work within cooperative groups that may have subgroups for the treatment of rare tumours. This will probably be the only way to move forward in the prospective knowledge of prognostic factors for these tumours.

[Clinical experience with chemotherapy of the malignant tumor of germinal cells (dysgerminoma) of the ovary]. / Experiencia clínica con el tratamiento a base de quimioterapia del tumor maligno de células germinales (disgerminoma) del ovario.

OBJECTIVE: To evaluate two alternative plans of chemotherapy with EP (etoposide, platinum) and BEP (bleomycin, etoposide, platinum) after oncological surgery. MATERIAL AND METHODS: A clinical, longitudinal and descriptive study was done, where the cases with pure dysgerminoma diagnosed in the gynecology-oncology service during the years from 1992 to 2003 were included. Information was recollected in a precoding survey that included sociodemographic characteristics, tumor size, free survival of disease, general survival, recurrence index and the index of fertility. RESULTS: The age of the group studied was of 22.1 +/- 6.5 years, with stratification to 9 patients in phase I (50%), phase III in 7 patients (38.9%) and phase IV in 2 patients (11.1%). Histological study confirmed the pure dysgerminoma, with a mean duration of the symptoms of 5.2 months, the free survival of disease was of 39.6 months and general survival was of 49.5 months. It was administered early chemotherapy in 11 patients, from which 7 received EP and the other 4 received BEP. Eight patients were found in advanced phases and with metastasis. There were 4 (22.2%) recurrences, of which 3 corresponded to phase III and a case to phase IV with tumors all of them over 15 cm. There were 3 patients rescued with chemotherapy of second line and a patient with radiotherapy to central nervous system. The statistical analysis showed that size of tumor among the recurrent group (24.2 cm) and the group without recurrence (14.5 cm) had significant differences (p = 0.018), the size of the tumor and the free survival of disease did not have significant correlation (p = 0.99), but upon comparing the general survival with the phase of the disease, a significant correlation was found (p = 0.03), where the survival to 5 years was observed in 6 cases (33.3%), of which a case was treated with surgery and without chemotherapy, 4 cases with EP and a case with BEP. Out of the 11 cases treated with conservative surgery, two patients got pregnancy in three occasions.

Ovarian germ cell cancer: clinicopathologic analysis and outcome of 31 cases.

AIMS: The aim of the study was to evaluate some clinicopathologic characteristics and the outcome of patients with ovarian germ cell cancer (OGCC) treated with cisplatin-based chemotherapy. METHODS: It was a clinical retrospective study. The clinical charts of 31 patients with OGCC assisted at the Department of Obstetrics and Gynecology of the State University of Campinas, Brazil, from January 1986 to June 1997 were reviewed. RESULTS: Ten patients had dysgerminoma and 21 patients nondysgerminomatous tumors. Women with dysgerminoma and nondysgerminomatous tumors did not present differences regarding surgical staging, age, ascites or residual tumor after the initial surgery. Frozen section, performed in 16 patients, showed some discrepancy with paraffin histology diagnosis in 8 patients. Platinum-based chemotherapy was used in 5/10 patients with dysgerminoma and in 17/21 patients with nondysgerminomatous tumors, with a 5-year survival of 100% for the dysgerminoma and 53% for the nondysgerminomatous group. CONCLUSIONS: Women with dysgerminoma and nondysgerminomatous tumors did not present differences regarding clinicopathologic characteristics. The prognosis for patients with dysgerminoma was better than for those with nondysgerminomatous tumors. Frozen section had a high error rate in diagnosing OGCC intraoperatively.

Etoposide y displatino (EP) en el tratamiento de tumores germinales de testículo / Etoposide and cisplatin (EP) in the treatment o germinal testicular tumors: factors which affect tumoral reaction to treatment

Entre 1986 y 1990 se estudió un grupo de 51 enfermos con tumores germinales del testículo, tratados con etoposide y cisplatino, con el propósito de conocer la reacción tumoral a este esquema de tratamiento y establecer que factores clínicos e histopatológicos influyen en ella

Cáncer embrionario de ovario / Embryonary neoplasms of the ovary

De 1965 al 1990 se estudian 33 casos de cáncer embrionario de ovario en los hospitales Belén y Regional de Trujillo, con la finalidad de determinar su incidencia y complicaciones. Este grupo de tumores representó el 17.6 por ciento de todas las neoplasias ováricas malignas, siendo el disgerminoma el tumor más frecuentemente encontrado (16 casos). Se apreció un predominio en las pacientes menores de 30 años (29 casos), que acudieron por dolor y tumoración abdominal (33 casos), presentando 25 de ellas un tiempo de evolución menor de 7 meses. Asimismo, 23 llegaron en estadíos III y IV. Todas fueron operadas a excepción de una que falleció el día de su ingreso al Hospital. La complicación más frecuente fué la anemia (15 casos), habiéndose registrado un caso con perforación de colon. Fueron derivadas al INEN 25 pacientes, 6 no regresaron a su control y 2 fallecieron víctimas de la enfermedad

Malignant germ cell sacrococcygeal tumors in children. Improved prognosis after introduction of cisplatin-containing multiple drug treatment.

The survival of children with malignant germ cell sacrococcygeal tumors has improved during the last few years after introduction of a multidrug protocol including cisplatinum. Treatment for 10 patients registered in 1965-1978 was not uniform and consisted of surgical resection or biopsy and radiotherapy with or without multiple drug chemotherapy (methotrexate + actinomycin D + cyclophosphamide). Only one of these patients is alive. Fifteen patients registered between 1978 and 1986 were treated with actinomycin D + cyclophosphamide + vincristine + doxorubicin + bleomycin + cisplatinum. Four patients also received radiotherapy. Seven out of these 15 children are alive without evidence of disease.

Treatment of advanced seminoma dactinomycin, cyclophosphamide,vinblastine, bleomycin and cisplatinum the VAB-6 protocol

Eighteen ppatients with advanced seminoma (stages IIc-III) were with: cytoxan 600 mg/m2, vinblastine 4 mg/m2, actinomycin D 1 mg/m2, bleomycin 30 mg, i. v. on day 1, followed by bleomycin 30 mg/day by infusion, days 1-3, and cis-platinum 120 mg/m2 on day 4. 9/18 pts. achieved complete remission and have been followed clinically; no recurrences have occurred in this group. The other 9 pts. had residual masses after 3 cycles of chemotherapy; they were taken to surgery and no viable tumor was found on surgical specimens. Thera were 3 recurrences in this group, always distant from the site of original disease. These patients were successfully treated with salvage chemotherapy and radiotherapy. One of these patients died of hepatic failure un related to his seminoma. All the other patients are alive, at a median follow-up of 50 months. This chemotherapy regimen is effective in curing advanced seminoma

Tumores germinativos avançados: tratamento com PVB / Advances germ-cell tumors: treatment with PVB

Treze pacientes portadores de tumores germinativos avançados foram tratados nos últimos três anos no Hospital de Oncologia - INAMPS com esquema quimioterápico PVB. Destes, um paciente näo é avaliavel visto ter recebido dois ciclos incompletos e somente um tem apresentaçäo extragonadal, sendo os demais primitivos do testículo. Oito pacientes apresentaram RC (66,7%) e quatro RP (33,3%). Duas recidivas ocorreram 12 e 16 meses após início da quimioterapia. Houve três óbitos, após RP, dois por falha ao esquema de 2ª linha (VP16 + CCDP) e um por evoluçäo de doença após abandono de tratamento durante esquema de 2ª linha. Somente um paciente recebeu dois ciclos de quimioterapia que corresponde a um tumor metacrônico. O período médio de acompanhamento é de 21 meses (oito a 39 meses). A média de ciclos é 3,8. O esquema é eficaz, a tolerabilidade muito boa, porém é necessário maior para confirmarmos o percentual de remissäo

Tumores de celulas germinales, situación actual y perspectivas futuras / Germinal cells tumors, current status and future perspectives

El tratamiento médico de los tumores de células germinales (TCG) en general y de los testiculares en particular supone uno de los más importantes logros de la moderna Oncología. Aunque ha sido el desarrollo de la quimioterapia la principal causa de este avance, la práctica oncológica multidisciplinaria que agrupa a la cirugía y a la radioterapia junto con la quimioterapia, es al igual que sucede con otors tumores curables, la base fundamental de estos logros

Vinblastine, actinomycin D, bleomycin, cyclophosphamide and cis-platinum for advanced germ cell testis tumors: Brazilian experience.

Disseminated germ cell testicular cancer proved to be highly sensitive to platinum-containing chemotherapy regimens. We present data concerning the treatment of advanced seminoma and nonseminomatous tumors in a developing country. We treated 30 patients with advanced germ cell testis tumors with 3 or 4 cycles of vinblastine, actinomycin D, bleomycin, cyclophosphamide and cis-platinum. Surgical resection of residual masses was done 30 days after completion of chemotherapy in 18 patients. The histology of the primary tumor was seminoma in 13 patients and nonseminomatous tumors in 17. Toxicity was mild and no treatment-related deaths occurred. All 13 patients (100 per cent) with seminoma and 12 of 17 patients (71 per cent) with nonseminomatous tumors had a complete response to chemotherapy, and 1 of 17 patients was free of disease after a debulking operation and additional chemotherapy. A total of 3 patients with seminoma and 2 with nonseminomatous tumors had recurrences 5 to 8 months after an initial complete response and received additional chemotherapy (VP-16 regimen) with or without radiotherapy. Complete clinical response was achieved in 4 of 5 patients. Median followup was 24 months (range 8 to 38 months) in the 13 patients with seminoma and 28 months (range 9 to 58 months) in those with nonseminomatous tumors, and 13 (100 per cent) and 12 (71 per cent), respectively, are alive without evidence of disease. These data suggest that the protocol of vinblastine, actinomycin D, bleomycin, cyclophosphamide and cis-platinum is highly effective and minimally toxic in the treatment of disseminated germ cell testicular cancer, inducing an 83 per cent long-lasting clinical remission. Seminomas seem to be equally or even more sensitive than nonseminomatous tumors to this platinum-containing chemotherapy regimen. Recurrence after initial complete response can be treated successfully with regimens containing VP-16.

Remissäo completa em câncer avançado do testículo com o emprego do esquema VAB6: experiência com 40 casos / Total remission of advanced cancer of the testis with the use of the VAB6 schedule: esperience with 40 cases

O prognóstico dos pacientes com câncer disseminado do testículo melhorou de forma significativa com o advento de esquemas de quimioterapia citotóxica contendo cis-platinum, vinblastina e bleomicina. O objetivo do presente trabalho foi o de avaliar a eficiência desta abordagem em pacientes com tumores germinativos metastáticos do testículo em estádios IIc e III. Quarenta pacientes, 14 portadores de seminoma e 26 portadores de tumores näo-seminomatosos, foram tratados com quimioterapia sistêmica seguida de cirurgia cito-redutiva. Nestes casos foi empregado o esquema VAB6, representado pela administraçäo de vinblastina, actinomicina-D, bleomicina, cis-platinum e ciclofosfamida por 3 ou 4 ciclos de 21 dias. Nos pacientes com persistência ou recorrência posterior da neplasia foram feitas novas reinduçöes com esquemas contendo VP-16-213 e cis-platinum. A análise deste material, após um período de seguimento que variou de 8 a 46 meses (mediana = 29 meses) em seminomas e de 3 a 64 meses (mediana = 33 meses) em tumores näo-seminomatosos, evidenciou remissäo completa e prolongada da neoplasia em 34 dos 40 pacientes (85%), incluindo todos os 14 pacientes com seminoma (100%) e 20 dos 26 pacientes com tumores näo-seminomatosos (77%). Os dados do presente estudo demostram que o cancêr do testículo representa doença consistentemente curável, mesmo em fases de apresentaçäo mais avançada