Sacrococcygeal teratoma: A case report

Sacrococcygeal teratomas (SCTs) are the most common extragonadal germ cell tumors, comprised different types of tissues that come from at least two of three germ cell layers. Depending on the tissues that are included, they are divided into mature, immature, and malignant. The incidence of SCT in infants and children is 1 in 35,000–40,000 live births. We are reporting a case of type I SCT. The patient was gravida 3, para 1, and abortion 1, which was diagnosed during antenatal ultrasound examination at 22 weeks of gestation and the termination was done after counseling the parents. A female fetus with a tumor in the sacrococcygeal region, weighing 800 g was delivered. The baby was sent to the department of anatomy. SCTs develop at the base of the coccyx and are thought to be derived from Henson’s node a rounded and elevated area at the cranial end of the primitive streak. This primitive streak consists of totipotent cells, which are able to transform into any type of cells.

Three cases of mediastinal germ cell tumors and concurrent hematologic malignancy and literature review / 中华实用儿科临床杂志

To analyze the clinical characteristics, treatment and prognosis of mediastinal germ cell tumors (GCTs) with concurrent hematologic malignancy (HM). The clinical features, treatment and prognosis of 3 cases of HM associated with mediastinal GCTs treated in the Department of Medical Oncology, Beijing Children′s Hospital from November 2014 to September 2018 were retrospectively analyzed.Meanwhile, relevant cases were searched in the PubMed and Wanfang database from their establishment to December 2019.Three male cases of HM associated with mediastinal GCTs aged from 12 to 16 years.The pathogenesis of mediastinal masses suggested teratoma or yolk sac tumor.All of them were treated with surgery and chemotherapy.Acute myeloid leukemia (AML) or myelodysplastic syndromes (MDS) was diagnosed respectively at 5 months, 9 months and 31 months after initial GCTs in the 3 cases.Two patients died and 1 child survived at the last follow-up.A total of 135 cases of concurrent GCTs and HM (or leukemia) were reviewed in online databases, involving 127 cases (94.1%) with the mediastinal GCTs associated with HM and 8 cases(5.9%) with GCTs related HM from another original sites.One hundred and twenty-six cases (99.2%) were male and the median age of GCTs diagnosis was 22 (10-48) years.Fifty-three cases (41.7%) were teratoma and 94 cases (74.0%) were GCTs containing teratoma with or without yolk sac tumor.Among the types of HM, 72 cases (56.7%) were AML and 31 cases (24.4%) were AML-M7.The median interval between GCTs and HM was 3 (0-122) months.Forty-six cases (36.2%) presented 2 malignancies simultaneously.HM were diagnosed within 12 months of GCTs in 85 cases (66.9%). The survival data were known in 107 cases, involving 94 (87.9%) deaths and 13 (12.1%) survivors.The median survival time after diagnosis of HM was 2 (0-48) months.The tendency of HM must be highly concerned in adolescent male patients with primary mediastinal GCTs, especially those with yolk sac tumor or teratoma.Their prognoses are very poor.Allogeneic hematopoietic stem cell transplantation is an alternative treatment.

Analysis of therapeutic effect of pediatric patients with intracranial primary non-germinomatous germ cell tumors / 中华放射肿瘤学杂志

Objective:To investigate the clinical features of pediatric patients with intracranial primary non-germinomatous germ cell tumors (NGGCT) and evaluate the treatment outcomes and prognostic factors of NGGCT.Methods:Clinical data of 40 children with NGGCT who were treated with radiotherapy (RT) at our department between November 2008 and June 2019 were retrospectively analyzed. Ninety percent of them received craniospinal irradiation (CSI). All children received platinum-based chemotherapy. Survival analysis was conducted using the Kaplan-Meier estimate. The prognostic factors were analyzed by log-rank test.Results:The primary sites were pineal gland, sellar / suprasellar region and basal ganglia. The median age of onset was 108 months (20-204 months). The median follow-up time was 33 months (8-131 months), and the 3-year and 5-year overall survival (OS) rates were 82.0%. The 3-year and 5-year progression-free survival (PFS) rates were 78.6% and 73.0%. Univariate analysis showed that increased alpha-fetoprotein (AFP) ( P=0.02), age at first diagnosis>10 years ( P=0.006), metastasis at first diagnosis ( P<0.001), and the pathological type (choriocarcinoma, yolk sac tumor and / or embryonal carcinoma) ( P=0.036) were independent adverse prognostic factors. Conclusions:Increased AFP, age>10 years at first diagnosis, tumor metastasis and pathological type were independent adverse prognostic factors of NGGCT. The overall prognosis of NGGCT children is worse than that of their counterparts with germinoma, and multidisciplinary intensive therapy is needed to improve survival.

Testicular germ cell tumors type 2 have high RNA expression of / 亚洲男科学杂志(英文版)

This study analyzed RNA expression of genes for three serum tumor markers, alpha fetoprotein (AFP), human chorionic gonadotropin (hCG), and lactate dehydrogenase (LDH), in patients with testicular germ cell tumors (TGCT) type 2. The gene AFP encodes AFP, the gene for chorionic gonadotropin beta polypeptide 5 (CGB5) encodes a major part of the specific beta subunit of hCG, and the genes for LDH subunit A (LDHA), LDH subunit B (LDHB), and LDH subunit C (LDHC) encode three different subunits of LDH. LDHB encodes the LDHB subunit present as a tetramer in LDH isoenzyme 1 (LDH-1). We examined three datasets with 203 samples of normal testis tissue (NT) and TGCT type 2. Yolk sac tumor (YST) expressed RNA of AFP fourteen thousand times higher than seminoma (SE), embryonal carcinoma (EC), and teratoma (TER) combined (P = 0.00015). In the second microarray, choriocarcinoma (CC) expressed RNA of CGB5 ten times higher than other histologic types of TGCT combined. EC expressed RNA of LDHB twice higher than SE, YST and TER combined (P = 0.000041). EC expressed RNA of LDHB higher than that YST expressed RNA of AFP and that CC expressed RNA of CGB5. In conclusion, TGCT type 2 expressed RNA of LDHB markedly higher than the RNA of 23 other candidate genes for TGCT type 2.

Preoperative differentiation of benign and malignant non-epithelial ovarian tumors: clinical features and tumor markers / Diferenciação pré-operatória de tumores ovarianos não epiteliais benignos e malignos: características clínicas e marcadores tumorais

Abstract Objective To evaluate the role of clinical features and preoperativemeasurement of cancer antigen 125 (CA125), human epididymis protein(HE4), and carcinoembryonic antigen (CEA) serum levels in women with benign and malignant non-epithelial ovarian tumors. Methods One hundred and nineteen consecutive women with germ cell, sex cordstromal, and ovarian leiomyomas were included in this study. The preoperative levels of biomarkers were measured, and then surgery and histopathological analysis were performed. Information about the treatment and disease recurrence were obtained from the medical files of patients. Results Our sample included 71 women with germ cell tumors (64 benign and 7 malignant), 46 with sex cord-stromal tumors (32 benign and 14 malignant), and 2 with ovarian leiomyomas. Among benign germ cell tumors, 63 were mature teratomas, and, amongmalignant, fourwere immatureteratomas. Themost common tumors in the sex cordstromal group were fibromas (benign) and granulosa cell tumor (malignant). The biomarker serum levels were not different among benign andmalignant non-epithelial ovarian tumors. Fertility-sparing surgeries were performed in 5 (71.4%) women with malignant germ cell tumor. Eleven (78.6%) patients with malignant sex cord-stromal tumors were treated with fertility-sparing surgeries. Five women (71.4%) with germ cell tumors and only 1 (7.1%) with sex cord-stromal tumor were treated with chemotherapy. One woman with germ cell tumor recurred and died of the disease and one woman with sex cord-stromal tumor recurred. Conclusion Non-epithelial ovarian tumors were benign in the majority of cases, and the malignant caseswere diagnosed at initial stages with good prognosis. Themeasurements of CA125, HE4, and CEA serum levels were not useful in the preoperative diagnosis of these tumors.

Resumo Objetivo Avaliar o papel das características clínicas e a medida pré-operatória dos níveis séricos de CA125, HE4, e CEA em mulheres com tumores de ovário não epiteliais benignos e malignos. Métodos Cento e dezenovemulheres consecutivas comtumores ovarianos de células germinativas, do cordão sexual-estroma, e miomas ovarianos foram incluídas neste estudo. Os níveis pré-operatórios dos biomarcadores foram medidos, a cirurgia e a análise histopatológica foram realizadas. Informações sobre tratamento e recorrência da doença foram obtidas dos prontuários médicos das pacientes. Resultados Nossa amostra incluiu 71 mulheres com tumores de células germinativas (64 benignos e 7 malignos), 46 com tumores do cordão sexual-estroma (32 benignos e 14 malignos), e 2 com leiomiomas ovarianos. Entre os tumores benignos de células germinativas, 63 eram teratomas maduros, e, entre os malignos, quatro eram teratomas imaturos. Os tumores mais comuns do grupo do cordão sexual-estroma foram fibromas (benignos) e tumores de células da granulosa (malignos). Os níveis séricos dos biomarcadores não diferiram entre os tumores de ovário não epiteliais benignos e malignos. A cirurgia preservadora de fertilidade foi realizada em 5 (71,4%) mulheres com tumores malignos de células germinativas. Onze (78,6%) mulheres com tumores do cordão sexual-estromamalignos foram tratadas comcirurgia preservadora de fertilidade. Cinco (71,4%)mulheres com células germinativas e apenas 1 (7,1%) com tumor do cordão sexual-estroma foram tratadas com quimioterapia. Uma mulher com tumor de células germinativas recidivou e morreu da doença. Uma mulher com tumor do cordão sexual-estroma recidivou. Conclusão Os tumores de ovário não epiteliais foram benignos namaioria dos casos e os malignos foram diagnosticados em estágios iniciais, com bom prognóstico. A medida dos níveis séricos de CA125, HE4, e CEA não foram úteis no diagnóstico préoperatório desses tumores.

Non seminomatous mixed germ cell tumor of the testis with predominant component of somatic type malignancy (Rhabdomyosarcoma) – A rare occurrence

Testicular germ cell tumors (GCT) are a diverse group of neoplasms, broadly divided into seminomatous and non seminomatous types, with varying histomorphology. Mixed germ cell tumors express more than one germ cell component. Somatic type malignancy occurring in testicular GCT is rare. Most often these components present as metastasis, particularly following chemotherapy, rather than primarily involving the testis. We describe a rare case of a young adult with no significant past history, who presented with testicular mass which on histology revealed a non-seminomatous mixed germ cell tumor with additional somatic type malignancy component of a rhabdomyosarcoma.

Progress in the treatment of intracranial germ cell tumors / 中国肿瘤临床

Intracranial germ cell tumors (IGGCTs) are rare intracranial embryonal tumors with various histological types. Most types of germ cell tumors are sensitive to radiotherapy and chemotherapy and can be cured with early treatment. The 5-year overall survival rate (OSR) of people with IGGCT has been reported as high as 90%, while the 5-year OSR of other malignant non-germinomatous germ cell tumors (NGGCTs) has been reported as less than 70%. Due to developments in surgery, imaging, nuclear medicine, pharmaceuticals, and other disciplines, the therapeutic effect of IGGCTs has improved in the last decade. However, due to the slow progress in researching its pathological genes, a treatment plan has not been standardized, and there is no standardized and clear clinical pathway or diagnosis and treatment guide. Here, the treatment experience and progress of IGGCTs is reviewed.

Hipertiroidismo como clave diagnóstica de un coriocarcinoma avanzado: Una asociación inusual / Hyperthyroidism as a diagnostic key to advanced choriocarcinoma. An unusual association

El hipertiroidismo es una condición relativamente frecuente con múltiples etiologías. La más común es la enfermedad de Graves, seguida del bocio multinodular y el adenoma tóxico. La asociación entre hipertiroidismo y cáncer es infrecuente en la práctica clínica. Presentamos el caso de un varón de 42 años con síntomas de hipertiroidismo de dos meses de evolución. Al examen físico se constató una marcada hepatomegalia de consistencia duro pétrea. El examen de testículos se reveló normal. Se llevó a cabo el diagnóstico de hipertiroidismo a través del dosaje hormonal. Los estudios por imágenes mostraron la presencia de múltiples lesiones sólidas compatibles con metástasis hepáticas. Luego de descartar las causas habituales de hipertiroidismo y las neoplasias primarias de la glándula tiroides, se consideró la posibilidad de mimetismo molecular a través de la producción ectópica de gonadotrofina coriónica humana. Se obtuvieron valores críticamente elevados de esta hormona y en un segundo tiempo se confirmó el diagnóstico histológico de coriocarcinoma a través de una biopsia hepática. Consideramos que el reconocimiento de este mecanismo poco frecuente de hipertiroidismo, puede ser una clave diagnóstica para arribar rápidamente al diagnóstico correcto, particularmente en los tumores extragonadales.

Hyperthyroidism is a relatively frequent condition with multiple causes. The most common cause is Graves' disease; followed by hyperthyroid multinodular goiter and toxic adenoma. Association between hyperthyroidism and cancer is infrequent in daily practice. We present the case of a 42-year-old man who developed severe symptoms of hyperthyroidism within a period of two months. Physical examination revealed significant hepatomegaly. Testicular examination proved normal. Imaging studies showed the presence of multiple hepatic solid lesions consistent with metastases. After discarding the most common causes of hyperthyroidism and primary thyroid gland neoplasm, the possibility of molecular mimicry was considered through human chorionic gonadotrophin production. Critical high values of this hormone were found and choriocarcinoma histological diagnosis was confirmed through a liver biopsy. We consider that the recognition of this rare mechanism of hyperthyroidism may be a clue permitting a faster diagnosis, particularly when extragonadal tumors are present.

Testicular Teratomas In Children- Case Series

Testicular tumors are very uncommon in children and teratomas are the commonest of these tumors. We are reporting our experience with five cases of testicular teratomas in the last 25 years.

Possibility of women treated with fertility-sparing surgery for non-epithelial ovarian tumors to safely and successfully become pregnant-a Chinese retrospective cohort study among 148 cases / 医学前沿

This study was performed to evaluate the oncological and reproductive outcomes of childbearing-age women treated with fertility-sparing surgery (FSS) for non-epithelial ovarian tumors in China. One hundred and forty eight non-epithelial ovarian tumor women treated with FSS between January 1, 2000 and August 31, 2015 from two medical centers in China were identified. Progression-free survival (PFS) was 88.5%, whereas overall survival (OS) was 93.9%. Univariate analysis suggested that delivery after treatment is related to PFS (P = 0.023), whereas histology significantly influenced OS. Cox regression analysis suggested that only histology was associated with PFS and OS (P < 0.05). Among the 129 women who completed adjuvant chemotherapy (ACT), none developed amenorrhea. Among the 44 women who desired pregnancy, 35 (79.5%) successfully had 51 gestations including 35 live births without birth defects. Non-epithelial ovarian tumors can achieve fulfilling prognosis after FSS and chemotherapy. Histology might be the only independent prognostic factor for PFS and OS. FSS followed by ACT appeared to have little or no effect on fertility. Meanwhile, postoperative pregnancy did not increase the PFS or OS. Use of gonadotropin-releasing hormone agonist was not beneficial for fertility.

Study on the clinical outcomes of children with stage Ⅳ malignant extracranial germ cell tumors / 临床儿科杂志

Objective To evaluate the outcomes of children with stage Ⅳ malignant extracranial germ cell tumors. Methods Twenty-five patients were enrolled in the retrospective analysis. Event-free survival (EFS) and overall survival (OS) rates were estimated by Kaplan-Meier method with SPSS 13.0. Results Of the 25 children, there were 13 males and 12 females. The mean age at diagnosis was 2 years old (ranged 1 to 11). Five patients receiving chemotherapy in another hospital before (n=1), or giving up treatment after confirmed diagnosis (n=1), or giving up effective treatment after received less than 2 cycles (n=3) were excluded from this analysis. Of the 20 patients, 90.0% (18/20) achieved complete remission and 5.0% (1/20) achieved partial remission after treatment. The 5-year EFS rate and 5-year OS rate were 70.0%±10.2% and 82.4%±9.2% respectively. There was no death occurred due to complications. Conclusions The effect of this treatment program is positive. The cumulative dose of the drugs is not high, compared with other schemes such as PEB, but there are more drugs involved. Whether these drugs may cause long-term adverse reactions needs further research.

The 18F-fluorodeoxyglucose positron emission tomograhy in predicting residual retro-peritoneal tumor after chemotherapy of testis germ cell tumors / 中国肿瘤临床

Objective:To determine the value of 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) scans in identify-ing the residual retroperitoneal tumor after chemotherapy of testis germ cell tumors. Methods:Sixteen testis germ cell tumor patients with metastasis of retroperitoneal lymph nodes who were treated in our hospital from February 2014 to December 2016 were select-ed for the study from February 2014 to December 2016. After 4-6 cycles of chemotherapy, their CT scans showed residual masses with diameters greater than 2 cm. The retroperitoneal lymph nodes were dissected after the 18F-FDG PET exam. The post-surgery pathology results were compared with the results of the 18F-FDG PET exam. Results:Residual tumors were found in 5 of 10 patients with 18F-FDG PET positive. Residual tumor was absent in 4 of 6 patients with 18F-FDG PET negative, while residual mature teratoma tumors were found in two patients. The accuracy rate, sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of the 18F-FDG PET exam were 56.25%(9/16), 71.42%(5/7), 44.44%(4/9), 50.00%(5/10), and 66.67%(4/6), respectively. Conclusion: 18F-FDG PET is highly sensitive. However, many factors influence the result of 18F-FDG PET. Mature teratoma leads to a false negative re-sult, whereas massive tissue inflammation leads to a false positive result. Therefore, more clinical examinations should be made.

Clinical outcome of postchemotherapy retroperitoneal lymph node dissection and predicting retroperitoneal histology in advanced nonseminomatous germ cell tumours of the testis / 中华外科杂志

Objective@#To explore the clinical outcome of advanced testicular nonseminomatous germ cell cancer patients undergoing post chemotherapy retroperitoneal lymph node dissection (PC-RPLND), and to analyze the relevant prognostic factors of lymph node pathological.@*Methods@#A total of 43 consecutive testicular nonseminomatous germ cell cancer patients underwent PC-RPLND between March 2001 and December 2014 in Department of Urology at Sun Yat-sen University Cancer Center were retrospectively reviewed. The average age of the patients was (29.0±11.5) years (ranging from 12 to 58 years). Before PC-RPLND, 22 patients were classified as phase Ⅱ, while 21 were phase Ⅲ. Primary tumor histology revealed seminomatous elements in 19 cases, embryonal cell carcinoma in 22 cases, yolk sac tumor in 13 cases, chorionic carcinoma in 3 cases, mature teratomatous elements in 11 and immature teratomatous elements in 2 cases. Patients were treated with cisplatin-based chemotherapy after orchectomy and then underwent surgical resection of retroperitoneal lymph nodes.After PC-RPLND, all patients underwent a periodic review including the blood routine, biochemistry routine and computed tomography or ultrasonograph of the chest, the abdomen and the pelvis. The association of pathological data with patient′s clinic features and the correlations between molecular features detected with each other were assessed by the t test, χ2 and Fisher′s exact test. Multivariate logistic regression were used to assess prognostic factors.@*Results@#The median operative time was 278 minutes (ranging from 50 to 715 minutes). Median blood loss was 425 ml (ranging from 50 to 5 000 ml). Eight patients received blood transfusion intra-operatively, 2 patients underwent adjunctive surgical procedures, 4 patients developed ileus and 4 had an ascites chylosus following PC-RPLND, 1 patient had a postoperative hyperthermia and retrograde ejaculation was present in 10 patients. The transverse diameter of the residual tumor in patients ranged from 0.8 to 18.2 cm. Necrosis, teratoma and viable germ cell tumors were found in 15, 17 and 11 of all patients. The median follow-up time was 46 months (ranging from 6 to 169 months). There were 39 patients had no tumor recurrence, 7 patients were found recurrence after PC-RPLND, 5 died of malignant germ cell tumor. The normal serum lactate dehydrogenase (LDH) level before chemotherapy (HR=25.811, 95%CI: 0.678 to 982.624, P=0.017) and relative changes more than 50% in retroperitoneal lymph node size (HR=0.016, 95%CI: 0 to 0.698, P=0.032) were statistically significant prognostic factors of the presence of necrosis.@*Conclusions@#Since most residual masses are not sensitive to chemotherapy, PC-RPLND is still an essential part of the treatment of metastatic testicular nonseminomatous germ cell cancer. Patients with the normal serum LDH level before chemotherapy and a shrinkage of 50% or more in retroperitoneal mass have a considerably chance of having necrosis in the retroperitoneum resection. This may help to refine the selection of candidates for PC-RPLND.

Imaging manifestations of testicular germ cell tumors / 实用放射学杂志

Objective To discuss the imaging features of germ cell tumors on CT and MRI.Methods Preoperative CT and MRI data of 11 pathologically proved germ cell tumors were analyzed retrospectively.A total of 11 cases were enrolled in the study,4 cases received plain and contrast-enhanced CT scan,1 received plain and contrast-enhanced MRI scan,and 6 received both CT and MRI scan.Results Histological examinations revealed 11 testicular neoplasms,among which 4 cases were seminoma,1 was embryonal carcinoma,1 was immature teratoma,3 were mixed germ cell tumors,1 was spindle cell rhabdomyosarcoma and 1 was carcinoid.Most germ cell tumors were iso density or low density with or without clear boundary,mild to moderate enhancement on CT scan.On MRI scan,most germ cell tumors showed iso-hyper intensity on T1WI,hypointensity,isointensity or hyperintensity on T2 WI,heterogeneous mild to moderate enhancement,high signal intensity on DWI,and low or high signal intensity on ADC.Conclusion Typical testicular germ cell tumor imaging performance has a certain specificity,but the atypical germ cell tumor lacks specificity.So the final diagnosis still depends on the pathology.

Malignant extra-cranial germ cell tumors in children and adolescents: Results following the guidelines of SFOP/SFCE 95 Protocol

Between September 1995 and December 2010, 99 new consecutive assessable patients with extra-cranial MGCT were treated according to SFOP/SFCE TGM95 Protocol. A "watch and wait" strategy for completely resected stage I-II was observed in cases with preoperative high tumor markers levels. Metastatic disease or alpha fetoprotein levels > 15 000 ng/ml cases were treated by VIP chemotherapy (etoposide, ifosfamide and CDDP) 4-6-courses. All other cases were treated by VBP (vinblastine, bleomycin, and CDDP) 3-5 courses. Median age for the whole group was 11.1 (r: 0-17) years. Males: 49, females: 50. Stage I: 19 patients, stage II: 16, stage III: 31 and stage IV: 3. Gonadal disease occurred in 77 cases. Of 21 completely resected stage I-II patients with MGCT who did not receive chemotherapy after surgery, 6 presented disease progression and were successfully treated by chemotherapy and remained disease-free. There were no significant differences in outcome according to age, gender, initial site, staging, and histological variant or high levels of alpha-fetoprotein. Initial non-responsiveness to VIP chemotherapy was the only significant unfavorable prognostic feature. With a median follow-up of 64 (r: 5-204) months, at 10 years EFS and OS estimates for the whole group were 0.82 (SE = 0.05) and 0.90 (SE = 0.03) respectively. Therapy results of MGCT treated with the SFOP/SFCE 95 strategy were excellent. Initial non-response to front line chemotherapy was the only significant adverse prognostic feature. The "watch and wait" strategy for completely resected disease with initial positive markers proved to be safe with optimal outcome.

Entre septiembre de 1995 y diciembre 2010 se registraron 99 nuevos pacientes evaluables consecutivos con tumores germinales malignos (TGM) extra-cerebrales. Los pacientes fueron tratados prospectivamente según los lineamientos del Protocolo SFOP/SFCE TGM95. Se siguió una estrategia de watch and wait para la enfermedad estadio I-II completamente resecada. La enfermedad con metástasis y los casos con niveles de alfa fetoproteína > 15 000 ng/ml fueron tratados con etopósido, ifosfamida y CDDP, 4-6 cursos. El resto fue tratado con vinblastina, bleomicina y CDDP, 3-5 ciclos. La mediana de edad fue de 11.1 (r: 0-17) años. Varones: 49, niñas: 50. Estadio I: 19 casos; II: 16; III: 31y IV: 33. De 21 enfermos con estadios tumorales I y II con resección completa inicial que no tuvieron tratamiento adyuvante, seis progresaron, todos fueron exitosamente tratados con quimioterapia y permanecieron libres de enfermedad. No hubo diferencias significativas en los resultados de supervivencia según edad, género, sitio inicial, estadificación, variante histológica o niveles elevados de alfa-fetoproteína. La resistencia primaria a la quimioterapia VIP fue el único factor pronóstico desfavorable significativo. Con una mediana de seguimiento de 64 (r: 5-204) meses, a 10 años las probabilidades de supervivencia libre de eventos y supervivencia global para todo el grupo fueron respectivamente de 0.82 (EE = 0.05) y 0.90 (EE = 0.03). Los resultados con la estrategia SFOP/SFCE 95 fueron excelentes. La ausencia de respuesta a la quimioterapia de primera línea fue el único factor pronóstico adverso significativo. La estrategia de watch and wait probó ser segura y eficaz.

Ovarian tumors in children: 10‑year experience from a tertiary care center in South India.

BACKGROUND: Ovarian tumors are uncommon in childhood and constitute around 1% of childhood malignancies. Two thirds of pediatric ovarian tumors are germ cell tumors. Epithelial ovarian tumors and stromal tumors are less frequent. We share our experience in childhood ovarian cancers, analyzing a series of cases with respect to the clinical profile, treatment and survival. METHODS: All newly diagnosed ovarian tumors in children up to 14 years of age, registered in our Pediatric Oncology Division between January 2000 and December 2009 were retrospectively reviewed. OBSERVATIONS: There were 47 patients with newly diagnosed ovarian tumors. The mean age at presentation was 10.0 ± 3.4 years. The most common symptoms at presentation were acute abdominal pain (48.9%) and abdominal mass (40.4%). Precocious puberty was uncommon (6.3%). Histology was germ cell tumors in 44 cases and nongerm cell tumors in three cases. The benign teratomas (mature and immature grade 1 and 2; n=9) underwent complete surgical resection alone; none had recurrence on follow up. Of the remaining 35 GCTs, 31 patients were given chemotherapy and 4 refused treatment.26 out of the 31 patients completed chemotherapy with BEP (bleomycin, etoposide, cisplatin) regimen with acceptable toxicities. 5 children i.e.; (19.2%) developed recurrence. At a median follow up of 80 months, 10 year disease free survival was 80.8 ± 7.7% and 10 year overall survival was 92.7 ± 4.9%. CONCLUSION: Germ cell tumors are the most common ovarian malignancy in children. With surgery and chemotherapy using BEP, good outcome can be expected in these patients.

Intensive chemotherapy as salvage treatment for solid tumors: focus on germ cell cancer

Germ cell tumors present contrasting biological and molecular features compared to many solid tumors, which may partially explain their unusual sensitivity to chemotherapy. Reduced DNA repair capacity and enhanced induction of apoptosis appear to be key factors in the sensitivity of germ cell tumors to cisplatin. Despite substantial cure rates, some patients relapse and subsequently die of their disease. Intensive doses of chemotherapy are used to counter mechanisms of drug resistance. So far, high-dose chemotherapy with hematopoietic stem cell support for solid tumors is used only in the setting of testicular germ cell tumors. In that indication, high-dose chemotherapy is given as the first or late salvage treatment for patients with either relapsed or progressive tumors after initial conventional salvage chemotherapy. High-dose chemotherapy is usually given as two or three sequential cycles using carboplatin and etoposide with or without ifosfamide. The administration of intensive therapy carries significant side effects and can only be efficiently and safely conducted in specialized referral centers to assure optimum patient care outcomes. In breast and ovarian cancer, most studies have demonstrated improvement in progression-free survival (PFS), but overall survival remained unchanged. Therefore, most of these approaches have been dropped. In germ cell tumors, clinical trials are currently investigating novel therapeutic combinations and active treatments. In particular, the integration of targeted therapies constitutes an important area of research for patients with a poor prognosis.

Hipogonadismo hipogonadotropo en paciente con síndrome de Klinefelter y tumor hipotálamo-hipofisario / Hypogonadotropic hypogonadism in Klinefelter syndrome and hypothalamic-pituitary tumor

El síndrome de Klinefelter es la causa más frecuente de hipogonadismo hipergonadotropo en el varón. La supresión en la respuesta al estímulo con hormona liberadora de la hormona luteinizante en estos pacientes debe hacer sospechar como posible etiología una tumoración a nivel hipotalámico. Se presenta el caso de un paciente diagnosticado a los 4 meses con síndrome de Klinefelter mediante cribado neonatal, con cariotipo 47 XXY, en el que se realizan controles clínicos y analíticos seriados y se encuentran, a los 17 años, valores suprimidos de hormona luteinizante y hormona folículo estimulante. Inicia, posteriormente, cefalea y amaurosis de ojo izquierdo, y se encuentra, en una resonancia magnética cerebral, un tumor germinal mixto a nivel hipotalámico, que precisa tratamiento quirúrgico, quimioterapia y radioterapia, con respuesta favorable.

Klinefelter Syndrome is the most frequent cause of hypergonadotropic hypogonadism in men. A flat response at luteinizing hormone releasing hormone stimulation test could be the first sign of hypothalamic tumor in these patients. We report the case of a patient diagnosed by neonatal screening with Klinefelter Syndrome, 47 XXY, that at 17 years follow-up presents analytical modification of the response to luteinizing hormone releasing hormone stimulation test with suppressed luteinizing hormone and follicle-stimulating hormone values; lately he presents with headache and loss of left eye vision. A magnetic resonance imaging of the brain showed a mixed germ cell hypothalamus tumor, requiring surgery, chemotherapy and radiotherapy with optimal response.

CT and MRI diagnosis of intracranial germ cell tumors / 中国医师杂志

Objective To study the features of CT and MRI diagnosis of intracranial germ cell tumors ,and to improve the un-derstanding of its imaging findings .Methods Fifteen cases of intracranial germ cell tumors proved by pathology and follow-up were retrospectively analyzed .Results Among the 15 cases with intracranial germ cell tumors ,9 cases in pineal body , tumours showed like-round masses,the margin of the masses were well defined .CT was slightly high density,MRI revealed isointense or hypointense signals on T1 weighted and homogeneous hyperintense signals on T 2 weighted image without edema around tumors and homogeneous en-hancement.One cases in CSF spread.Four cases were identified in the saddle-up area, among the 2 cases was cystic,1 cases was sol-id-cystic and 1 cases was solid,CT was isointense or high density in solid,low density in cystic,without edema around tumors.MRI re-vealed slight-hypointense signals on T 1 weighted and hyperintense signals on T 2 weighted image clearly enhanced in solid , cystoid with-out enhance .In the other 2 cases of intracranial germ cell tumors in the basal ganglia region ,CT was inhomogeneous high density ,inho-mogeneous signals on T2 and T1 weighted image, inhomogeneous enhancement .Conclusions Intracranial germ cell tumors show char-acteristic manifestations on CT and MRI,according to location,shape,signal,age characteristic,which is helpful in diagnosis and differ-ential diagnosis and guide treatment plan .

Research progress of octamer-binding transcription factor 4 and sal-like gene 4 in germ cell tumors / 肿瘤研究与临床

Tumor marker research is a hot field of oncology.Previous studies of tumor markers such as placental alkaline phosphatase,alpha-fetoprotein,c-kit and CD30 and so on,lack sufficient sensitivity and specificity for the early diagnosis of germ cell tumor.Recent research demonstrated that octamer-binding transcription factor 4 (OCT4),sal-like gene 4 (SALL4) can be used as new germ cell tumor markers.This paper reviewed the research progress on the OCT4 and SALL4 in recent years,to provide the reference about the early diagnosis,tumor typing,condition estimation,prognosis and targeted therapy of germ cell tumors.