Pediatric germ cell tumors; a 10-year experience

The aim of this study was to evaluate the outcome of germ cell tumors in patients admitted to our center during a ten year period. In a retrospective descriptive study, patients with the pathological diagnosis of germ cell tumor [GCT] were included. All records were evaluated and patients followed by personal visit in clinic or phone call. Data regarding age, sex, tumor site, bio-chemical assay, pathology, treatment and outcomes were gathered. For qualitative variables we computed frequency and percentage and for quantitative variables, mean and standard deviation. Survival analysis was performed using Kaplan-Meier. All statistical analyses were performed by SPSS version 6.0. Forty four patients consisted of 32 girls [72.7%] and 12 boys [27.3%]. Their median age was 23 months. The most common pathological tumor types were 18 [40.9%] mature teratomas and 14 [31.8%] yolk sac tumors. Extra gonadal tumors were more prevalent [32 cases] and consisted of 21 [47.7%] sacrcoccygeal, 7 [15.9%] retroperitoneal, 2 [4.4%] mediastinal and 2 [4.4%]cervical tumors. In gonadal tumors 9 patients had ovarian and 3 patients testicular involvement. Staging at the time of diagnosis revealed stage one in 23 [52.3%] cases. All patients were treated surgically and the most common procedure was total resection in 41 [93.2%] patients. Fifteen [34.1%] patients received chemotherapy. In follow-up 31 [77.5%] patients were in complete remission, 9 [22.5%] had died, and 4 cases did not appear to follow-up visits. The median survival was 16 months [IQR 4-49 months]. The highest mortality rate was found in patients with yolk sac tumors [8 of 13 cases]. The patients with extra-gonadal GCT and a high AFP level have the worst prognosis and lower survival rate. Combination of surgery and chemotherapy can lead to a better prognosis

Congenital nasopharyngeal teratoma in a neonate

Congenital germ cell tumors are uncommon. The most common site of teratoma is in the sacrococcygeal region. Teratoma arising from the head and neck comprises less than 10% of reported cases and of these, nasopharyngeal lesions are rare. Teratomas are generally benign, and have a well recognized clinical and histopathological entity. We present a case of nasopharyngeal teratoma [NPT] associated with a wide cleft palate. A 20 day old female neonate with a teratoma of the nasopharyngeal area, and wide cleft palate was referred to our center. The protruded mass which measured 6x4x3cm, was of soft consistency, blocked the airway, and prevented oral feeding. Preoperative evaluation and imaging was performed and mass was excised 2 days after admission. Pathology revealed a well-differentiated mature solid teratoma [hairy polyp]. The patient had no complication in the post-operative period. Cleft palate was surgically repaired when 2 years old. She is now a six year old girl with normal development. Congenital nasopharyngeal teratomas are usually benign. Surgery is the treatment of choice, and should be undertaken on an urgent basis, especially in a patient who presents with signs and symptoms of airway obstruction

Liver hydatid cyst in children [a 14-year review]

Hydatid disease is still an important health hazard in the world. This disease is a parasitic infestation which is endemic in many sheep and cattle raising areas such as in Iran. The aim of this study was to evaluate the clinical appearance, diagnosis, and treatment of liver hydatid cyst in children. This retrospective study evaluated 100 patients who were referred to Mofid Children's Hospital with liver hydatid cyst from March 1996 to March 2010. Medical records of 1 to 14 year old patients who had definitive liver hydatid cyst were included and analysis of variables such as age, gender, symptoms, diagnostic investigation, operative technique, hospital stay, mortality, morbidity and outcome of treatment were evaluated. The patients consisted of 54 boys [54%] and 46 [46%] girls with an age range of 1-14 years [mean 11.8 +/- 4.6]. The incidence rate increased by age. The patients had totally 110 cysts, right pulmonary lobe 81 [73%] cysts and left side had 29 [27%]. Abdominal mass was the most common [50%] symptom. Abdominal sonography gave correct diagnosis in 94 [94%] patients. Conservative surgical treatment was carried out in 98 children. Two patients were treated medically as the cysts were small and calcified. The most common complication was wound infection in 3 cases. Mean length of hospitalization was 9 days. In 100% of our patients the type of parasite was Echinoccocus granulosus. The morbidity rate was 12% [prolonging external catheter drainage in 12 patients]. There was only one [1%] mortality and 2 [2%] recurrences. Due to the less invasive and high accuracy of liver sonography in diagnosis of hydatid cyst, we recommend it as the method of choice for the diagnosis in endemic regions. Surgery is the method of choice for treatment

Diphallus with imperforate anus and complete duplication of recto-sigmoid colon and lower urinary tract

Diphallus is a rare anomaly and accompanying anomalies vary from bifid scrotum, bladder exstrophy, imperforate anus and colo-rectal anomaly such as duplication, and | other associated anomalies. A 2-day old infant is reported with imperforate anus and complete duplication of recto-sigmoid colon, rectal pouch, doubling of the genitalia with completely formed penis [diphallus], double bladder, urethra and hypospadias. No family history of abnormalities was noted. The patient underwent several operations: laparatory and colostomy at 3rd day of life, and after clinical and paraclinical investigations, cystoplasty, ureteral reimplantation and resection of left phallus were carried out when 4 months old. At the age of 1 year, after colostogram and total colon evaluation, laparatomy, resection of duplicated recto-sigmoid colon, and pull-through was carried out; 3 months later colostomy closure was performed and the patient discharged without complications. The patients with diphallus have to be examined carefully because of the high incidence of other systemic anomalies. Treatment of diphallus usually includes excision of the duplicated penile structure, its urethra, and repair of associated anomalies