Ansari Sh, Vossogh P, Tabarok A,
Volume 64, Issue 11 (10-2006)
Abstract
Background: Germ cell tumor (GCT) account for approximately 2-3% of all malignancies in childhood. About 20% of patients with GCT are still resistant to therapy.
Methods: This study was undertaken on 57 patients with Germ cell tumor who were admitted to Ali Asghar Children’s Hospital during 1990-2004. Through this study, information about sex, age type of pathology, clinical sign, treatment and survival (5-year period) was gathered in order to have better treatment and follow up. This study was carried out as across-sectional survey and the obtained data was analyzed via Spss 10 soft ware.
Results: The findings showed that the mean age of patients was 4/9 ± 0/1 (1mo -14y), male 54%, female 46%, male/female, ratio=1/1. Site of tumor: saccrococcygeal 57/8 %( 33), gonadal 42% (24). Pathological type is yolk sac 61/4% (35), dysgerminoma 12/2% (7), malignant teratoma 14% (8), embryonal carcinoma 10/5% (6). The most common clinical sign were buttock mass 31/5% (18), abdominal pain 10/5% (6), abdominal mass 17/5%(10), testicular mass 28% (16). All of the patients were treated with chemotherapy (bleomycine, vinblastin, cisplatinum) mean of duration follow up were 48/4 months. In all of patients 31/5% (18) of the cases were alive and 70% (40) of patients were relapse and 15/7% (9) no information, 52/6% (30) of cases were expired. Five years survival of patients was 62%.
Conclusion: The analysis of the patients treated shows that extragonadal location of primary tumor (specially sacrococcygeal), level of AFP above 10 ng/ml in patients ,6 or more months of age and metastatic disease were the most unfavorable factors for overall survival.
Zohreh Yousefi , Sima Kadkhodayan , Shohre Saeed , Amirhossein Jafarian , Fatemeh Mirzamarjani ,
Volume 74, Issue 3 (6-2016)
Abstract
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Background: Swyer syndrome is a type of hypogonadism with 46,XY karyotype. This syndrome was named by Gerald Swyer, an endocrinologist. It leads to a female with normal internal genitalia (uterus, fallopian tubes, cervix, vagina), but instead of ovaries, they have non functional ovary (streak gonads). Also, they have absence of puberty because of gonadal digenesis. The current practice is to proceed gonadectomy once the diagnosis is made due to the fact that the risk of malignant transformation is high in dysgenetic gonad. In addition, hormonal replacement therapy after surgery is acceptable.
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Case Presentation: We present a case of gonadoblastom in right ovary in a Swyer syndrome who referred to the department of Gynecology Oncology at Ghaem Hospital, Mashhad University, Iran in 2015 for evaluation of abdomino-pelvic distention. She was a 18-year-old female with 46, XY karyotype and poor secondary sexual character and normal external genitalia. She suffered of abdominal pain. In palpation of the abdomen, an irregular mobile mass was detected in left lower quadrant. The ultrasound revealed uterine size approximate dimensions 3×2 cm (infantile) and a 19 cm pelvic mass heterogeneous and multi-loculated in left side of the pelvic cavity with possible origin of the left ovary. In addition, in right pelvic fossa, a mass about 6 cm was detected. CT-Scan showed a pelvic mass with overall dimensions of 10 cm with vicinity to the left iliac vessels, modest amounts of ascities along with evidence of peritoneal dissemination (seeding). In laparotomy we observed massive ascities and a 20 cm solid mass in left ovary and a small mass in right ovary and involvement para aortic lymph node. Pathological report indicated as stage III of dysgerminoma in left ovary and gonadoblastom in right ovary.
Conclusion: This case is presented because it could have excellent prognosis if not missed opportunities of early recognizing and furthermore adequate treatment with gonadectomy.
