Primary ovarian tumors in children: a single center experience of 124 patients.

BACKGROUND: Primary ovarian tumors are rare in the pediatric age group. We reviewed our 40-year experience with ovarian tumors to evalute the clinical features and treatment results in a single institution. METHODS: Between January 1975 and October 2015, 124 girls with primary ovarian tumor were diagnosed and treated in our center. Tumors were identified with biopsy or total resection and/or serum markers. Seventy four children were included in the treatment analysis. RESULTS: Median age for 124 children was 11.0 years (0.73-17.63). The main complaint was abdominal pain in 85 patients (68.5%). One hundred and five patients (84.6%) had total one-sided salpingo-oophorectomy and five patients had bilateral salpingo-oophorectomy. Amongst 124 cases, 29 patients had mature teratoma, which was the most common tumor in this study. Dysgerminoma (n=21) was the most common malignant histopathologic type. Stage I disease was diagnosed in 57.2% and stage IV in 6.6% of the patients. Five year overall survival (OS) and event-free survival (EFS) for 124 children were 82.5% and 76.3% respectively. For 74 children who received treatment, 5-year OS and EFS were 75.2% and 67.1%, respectively. Age (p < 0.017), histopathological subgroup (p < 0.001), stage (p =0.003) and chemotherapy protocols (p =0.049) were significant prognostic factors for OS. CONCLUSIONS: The survival rates in children with ovarian tumors were comparable with studies in the literature. Although patients treated with platin based regimens had better survival rates, prognosis was still poor for the patients in advanced stages. This should be the focus for further studies and improvements.

Liver transplantation from a deceased donor with ß-thalassemia intermedia is not contraindicated: A case report.

The use of extended criteria donors who might have previously been deemed unsuitable is an option to increase the organ supply for transplantation. This report presents a pediatric case of a successful liver transplantation from a donor with ß-thalassemia intermedia. A patient, 6-year-old female, with a diagnosis of cryptogenic liver cirrhosis underwent deceased donor liver transplantation from a thalassemic donor. Extreme hyperferritinemia was detected shortly after transplantation. The most probable cause of hyperferritinemia was iron overload secondary to transplantation of a hemosiderotic liver. Hepatocellular injury due to acute graft rejection might have contributed to elevated ferritin levels by causing release of stored iron from the hemosiderotic liver graft. Iron chelation and phlebotomy therapies were started simultaneously in the early postoperative period to avoid iron-related organ toxicity and transplant failure. Follow-up with monthly phlebotomies after discharge yielded a favorable outcome with normal transplant functions. Thalassemia intermedia patients can be candidates of liver donors to decrease pretransplant waitlist mortality. After transplantation of a hemosiderotic liver, it is important to monitor the recipient in terms of iron overload and toxicity. Early attempts to lower iron burden including chelation therapy and/or phlebotomy should be considered to avoid organ toxicity and transplant failure.

Simple cyst of the testis: a rare and benign cause of testicular swelling in infancy.

We report a five-month-old boy who was referred to the hospital with a presumptive diagnosis of hydrocele of the cord. Ultrasonography revealed a cystic mass within the left testis. A complete removal of the cystic mass was done. Histopathological diagnosis was simple cyst of the testis. This rare lesion should be considered in the differential diagnosis of cystic testicular lesions in infancy. The treatment of choice is simple enucleation of the cyst with sparing of the testis and related structures.

Solid tumors in the neonatal period.

From 1972 to 2000, 123 patients with solid tumors whose complaints had started in the first 28 days of life were retrospectively evaluated. Fifty-five patients were diagnosed in the first 28 days and 68 patients were diagnosed after 28 days. In the former group, 85.5% of patients had symptoms in the first day of life. In the latter group, 77.9% had the onset of symptoms in the first day. Tumor subgroups in the neonatal period included teratoma (34), neuroblastoma (11), rhabdomyosarcoma (3), Wilms tumor (1), and retinoblastoma (3), and the others (3). Three patients had other, less common tumors. In the second group the numbers were the following: for teratoma (32), neuroblastoma (15), germ cell tumors other than teratomas (8), rhabdomyosarcomas (4), the other soft tissue sarcomas (3), Wilms tumor (1), retinoblastoma (1), and other, rare tumors (4). There were 22 malignant tumors in the first group, and 44 in the second group. Fourteen patients in the first group died in the early postoperative period or with progressive disease. Nineteen of 44 patients died in the second group. Overall survival rates were 24.9% and 51.6% in first and second groups, respectively (p = 0.015). Event-free survival rates were 14.7% and 47.7% in these groups, respectively (p = 0.0063). This is the first report comparing clinical features and prognosis of tumors diagnosed in the first 28 days of the life with those diagnosed after 28 days. The prognosis was worse in infants diagnosed in the first 28 days of life.