Pediatric Germ Cell Tumors; A 10-year Experience.

OBJECTIVE: 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. METHODS: 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 version16.0. Findings : 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). CONCLUSION: 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 rhabdomyosarcoma of shoulder.

A 16-day-old female was referred with congenital swelling on her right shoulder. On examination, there was a hard, round, ecchymotic, nontender, slightly movable, warm and shiny 10x15 cm mass on the right axillary pits which was extended to the right side of neck and chest wall. The mass separated the shoulder from the chest wall causing paralysis of right hand. Chest X-ray, ultrasound and MRI with contrast demonstrated a soft tissue mass suspected to be a hemangioma. The mass rapidly increased in size despite aggressive steroid therapy with rupture and bleeding. On the 45th post natal day the baby was taken to operating room to control the bleeding and if possible total excision of the mass. The mass was separated easily from the surrounding tissue and was excised along with right upper extremity. At the end of surgery the baby had cardiac arrest, and apparently died of Disseminated Intravascular Coagulation (DIC). The final pathology report was Rhabdomyosarcoma (RMS).

Complete colonic duplication in children.

BACKGROUND: Complete colonic duplication is a very rare congenital anomaly that may have different presentations according to its location and size. Complete colonic duplication can occur in 15% of gastrointestinal duplication. We report two cases of complete colonic duplications, and their characteristics. CASE PRESENTATION: We present two patients with complete colonic duplication with different types and presentations. Case 1: A 2- year old boy presented to the clinic with abdominal protrusion, difficulty to defecate, chronic constipation and mucosal prolaps covered bulging (rectocele) since he was 6 months old. The patient had palpable pelvic mass with doughy consistency. Rectal exam confirmed perirectal mass with soft consistency. The patient underwent a surgical operation that had total tubular colorectal duplication with one blind end and was treated with simple fenestration of distal end, and was discharged without complication. After two years follow up, he had normal defecation and good weight gain. Case 2: A 2 -day old infant was referred with imperforate anus and complete duplication of recto-sigmoid colon, diphallus, double bladder, and hypospadiasis. After clinical and paraclinical investigations, he underwent operations in several stages in different periods, and was discharged without complications. After four years follow up, he led a normal life. CONCLUSION: The patients with complete duplication have to be examined carefully because of the high incidence of other systemic anomalies. Treatment includes simple resection of distal common wall, fenestration, and repair other associated anomalies.

Tracheoesophageal fistula following disc battery ingestion and foreign body impaction.

BACKGROUND: Ingestion of foreign bodies may result in the formation of a tracheoesophageal fistula (TEF), which causes severe morbidity in children. We describe four cases of TEF, who underwent emergent surgery for repair. CASE PRESENTATION: In this report, we present about four patients aged between 9 months to 2.5 years, who referred due to disc battery ingestion. There were two boys and two girls. The common symptoms were cough, cyanosis, and dysphagia, choking and vomiting. The diagnosis was performed through an x-ray, barium swallow and CT Scan. All batteries were impacted in the esophagus, two in upper, one in the middle, and one in lower esophagus position. All disc batteries were removed endoscopically, but had tracheoesophageal fistula (TEF). All the patients underwent TEF repaired surgically. There was no morbidity in four patients, but one patient developed moderate esophageal stenosis, which was repaired by staged dilatation. There was no mortality in our cases. CONCLUSION: Long-term impaction of foreign bodies may result in tracheoesophageal fistula. This complication may be seen earlier with alkaline disc batteries. Removal of these foreign bodies should be followed carefully for the diagnosis and treatment of these fistulas.

The prevalence of polyp in colon of patients with acromegaly.

Several studies have shown that patients with acromegaly are at increased risk of developing colorectal polyps and cancer. The prevalence of polyp in colon of patients with acromegaly was variously reported between 23% and 53%. This study was conducted to determine the prevalence of polyp and cancer of colon in patients with acromegaly. Twenty-three patients with acromegaly were evaluated with colonoscopy for the presence of colonic polyps and cancer. Three patients were found to have colonic polyps. This translates to a prevalence rate of 13%. We recommend that screening colonoscopy be carried out once every three years in all acromegalic patients over the age of 40 years.