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

Survey on childhood solid malignant tumors in cases admitted to Mofid pediatric hospital from 1996-2010: a single-center study

As children comprise a considerable proportion of our population, the importance of local epidemiologic research and geographic and racial differences can't be disputed on childhood malignancies. In this descriptive retrospective study, we extensively reviewed the medical records of patients younger than 15 years of age, diagnosed with solid malignant tumors, from 1996 to 2010, using the last version of International Classification of Childhood Cancers. In our study the order of incidence of solid malignancies was relatively similar to the other national studies, with lymphomas and Central Nervous System [CNS] tumors as the most common, followed by Sympathetic Nervous System [SNS] tumors, soft tissue sarcomas and renal tumors. The peak age of diagnosis was between 1 and 4 years old. In our study, the overall male to female ratio was 1.38, with a trend towards male dominance in the older age groups. We also observed a disturbing trend of childhood solid malignancies. The total number of cases almost doubled from 2009[54[6.9%]] to 2010[96[12.2%]] .This trend was particularly detected in CNS and SNS tumors. Further analysis showed that malignant CNS tumors had played a more pronounced role in this change. Changes in trends of some tumor categories have illustrated a desperate need to further research in regional and national levels. Also the gathered data can be used to make more accurate programs for a better control of cancer and to help policymakers to allocate more evidence-based resource for hospitals

Clinical evaluation of disc battery ingestion in children

The purpose of this study was to evaluate the characteristics, management, and outcomes of disc battery ingestion in children. We reviewed the medical records of children admitted to Mofid Children's Hospital due to disc battery ingestion from January 2006 to January 2010. Clear history, clinical symptoms and results of imaging studies revealed diagnosis of disc battery ingestion in suspected patients. The clinical data reviewed included age, gender, clinical manifestation, radiologic findings, location of disc battery, duration of ingestion, endoscopic results and surgical treatment. We found 22 cases [11 males and 11 females] of disc battery ingestion with a mean age of 4.3 years [range: 9 months to 12 years]. Common symptoms were vomiting, cough, dysphagia, and dyspnea. The mean duration of ingestion was 2.7 days [4 hours to 1.5 months]. A total of 19 patients had histories of disc battery ingestion, but three cases referred with the above symptoms, and the batteries were accidentally found by x-ray. Only three cases had batteries impacted in the esophagus. Twelve batteries were removed endoscopically, 6 batteries spontaneously passed through the gastrointestinal [GI] tract within 5 to 7 days, and 4 patients underwent surgery due to complications: 3 due to tracheo-esophageal fistula [TEF] and 1 due to intestinal perforation. There was no mortality in our study. Most cases of disc battery ingestion run uneventful courses, but some may be complicated. If the battery lodges in the esophagus, emergency endoscopic management is necessary. However, once in the stomach, it will usually pass through the GI tract

Treatment of bilateral macrostomia [lateral lip cleft]: case report

Macrostomia as a rare facial deformity is classified among facial clefts. It originates from failure in union of maxillary and mandibular prominences of first brachial arch during 7[th] embryonic week. We report a case of bilateral macrostomia [bilateral lip cleft] in a female newborn as a sole entity without other skeletal and facial deformities. The cleft was repaired by a simple linear triangular flap using extra oral landmarks to locate lip commissures. Patient was followed through a six-month period. Acceptable results were gained in mouth appearance as well functional aspects. Commissural repair through a linear flap can result in minimal visible scar with satisfying results in both esthetics and functional aspects

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]

Abdominal inflammatory myofibroblastic tumor: report on four cases and review of literature

The Abdominal Inflammatory Myofibroblastic Tumor [AIMT] is a rare tumor with unknown etiology which usually occurs in children and adolescents. It is composed of myofibroblastic spindle cells intermixed with inflammatory cells. We present four cases of AIMT. We herein present four cases of AIMT in different ages [range: 3.5 to 13 years] and in different organs [stomach, periduodenal, mesenteric, and colon]. There were two females and two males. The main symptoms were abdominal pain/mass/obstruction, vomiting, and weight loss. In all four patients, diagnosis was made by laparatomy and pathologic examination of excised mass lesion. Three patients underwent complete excision and no residual disease was present, one patient received chemotherapy due to tumor recurrences. The patients were followed up in average for four years. As the imaging and laboratory tests are non-specific, the diagnosis of AIMT is rarely made before surgery. AIMT should, therefore, be considered when a mass arises in an unusual location in the pediatric age group. Complete surgical resection should be performed whenever possible and the child should be kept on long-term follow-up

Can we rely on frozen sections of a rectal biopsy for one-stage trans-anal pull-through operation in Hirschsprung's disease?

Successful results after one-stage trans-anal pull-through [OSTAPT] operation for Hirschsprung's disease [HD] depend on accurate identification of the aganglionic segment in intra-operative frozen section [FS]. Misinterpretation of the findings of the rectal biopsy is an anxiety-evoking pitfall for the surgeon. This study aims to describe our experiences in comparing results of FS and permanent Section [PS] rectal biopsies in children with HD who were candidates for OSTAPT in a single-step operation. Subjects under the age of 14 years, admitted from March 2000 to July 2008 in a university-affiliated children's hospital for open rectal biopsy to diagnose HD were included in the study. All biopsies were taken 2-3 cm above the dentate line. 210 specimens of full-thickness rectal biopsy were obtained for both frozen section and permanent biopsy from all patients, examined by two well experienced pediatric pathologists for ascertaining the presence of ganglion cells, and the results were compared. Analysis was performed by SPSS Software version 11.5. Two-hundred one infants and children underwent FS rectal biopsy to exclude HD. Positive results were seen in 63.8% of the specimens examined as PS and in 58.3% of FS samples. 93.9% of positive results in FS studies were confirmed by PS studies. 6.1% of FS reports were false positive and 21.7% were false negative [P<0.001]. The sensitivity of FS was 85.8% and specificity 90.2%. Positive predictive value [PPV] was 93.9% and negative predictive value [NPV] was 78.3% in FS studies [P<0.001]. The accuracy of FS was 80.4%. Although FS of the rectal biopsy is useful in defining the aganglionic segment during operation, according to this study, it cannot be used as the sole base for performing primary pull-through operation before the results of the permanent section are on hand

Early exploration in the management of acute scortum in children

Acute scrotal conditions are a common clinical setting that present with pain and swelling of the hemiscrotum. The aim of our study has been to evaluate the findings in boys operated on acute scrotum. A descriptive study was conducted on 100 patients with acute scrotum admitted to Mofid Children's Hospital from March 1993 to March 2007. Data included history, age, primary symptoms, definite diagnosis, side involvement, paraclinical tests, imaging modalities, medical or surgical management and type of the surgery. Diagnosis was made mainly by clinical signs and symptoms and surgical exploration. Torsion of testis [n=31] was the most common cause of acute scrotum followed by incarcerated inguinal hernia [n=30], torsion of testicular appendage [n=27], epididymo-orchitis [n=7], idiopathic scrotal edema [n=4] and hematocele [n=1]. Most [34%] of the patients were in the first year of life and the mean age was 5.4 years. The commonest signs were pain and swelling [62%] followed by pain, swelling and redness [21%] and pain alone [16%]. 83 patients consisting of 31 with torsion of testis, 14 with torsion of testicular appendage, 30 with incarcerated hernia and 7 with epididymo-orchitis underwent surgical exploration after careful physical examination. 10 of 31 patients with torsion of testis had orchiectomy and orchiopexy of contrea-lateral testis and the rest had detorsion and bilateral orchiopexy. 80% of patients were referred to the hospital after 12 hours of clinical onset of symptoms. Early exploration of scrotum based on careful physical examination excludes the risk of misdiagnosis by diagnostic procedures and unnecessary delay by diagnostic techniques. Exploration of scrotum is a relatively safe and simple procedure with good cosmetic results, it also allows an accurate diagnosis to be made

Thyroid nodules in childhood: a single institute experience

Thyroid nodules are rare in children. Multiple diagnostic modalities are used to evaluate the thyroid mass. The aim of this study was to determine results of management of thyroid nodules in children with special attention to the role of fine needle aspiration biopsy, [FNAB] in diagnosis. Thirty-two children who underwent surgery for thyroid nodules in Mofid Children's Hospital within 10 years [1996 to 2005] were retrospectively studied. From clinical records we [obtained data about demographic characteristics, clinical manifestations, ultrasonography [USG] findings, and FNAB results, pathological reports, surgical therapy and complications. Data was analyzed statistically for association with thyroid cancer. Twenty-five patients [78.1%] were girls, and 7 [21.9%] boys. Mean age was 10.9 [range 8 to 14] years. 24 [75%] patients had benign and 8 [25%] malignant tumors. 18 [56.25%] nodules were located in the right lobe. Statistical analysis revealed sensitivity, specificity, accuracy, and positive and negative predictive values as follows: 80%, 65%, 25%, and 86% for USG; 35%, 41%, 40%, 18%, and 66% for RNS; 91%, 94%, 90%, 74%, and 96% for FNAB respectively. Clinical judgment as determined by serial physical findings with USG continues to be the most important factor in the management of thyroid nodules in children. FNAB is the most accurate method of investigation and its accuracy is improved by USG guidance

results of posterior sagittal anorectoplasty in anorectal malformations

Posterior sagittal anorectoplasty [PSARP] is the newest and most-widely-used technique for correction of anorectal malformations [ARMs]. In the present investigation, the functional outcomes of PSARP and also the relationship between the complications and the sacral ratio [SR] have been studied. The study group included 49 patients [25 males and 24 females] with ARMs, operated on by the PSARP technique during 1995 - 1999. The patients had been followed up for at least 4 years. Those with mental retardation were not included in this retrospective study. For objective evaluation of the sacrum, the SR was calculated in all of the patients. In the female patients, rectovestibular fistula [46%] and in the males, perineal fistula and imperforate anus without fistula, each with equal frequency [28%] were the most common anomalies. The mean SR in the study group was 0.72 + 0.04. SR was 0.67 + 0.03 among patients who had associated urogenital anomalies, 0.69 + 0.04 in patients suffering from soiling, and 0.67 + 0.02 in patients who had postoperative fecal incontinence. The most common complication following PSARP was soiling [44.9%] and then constipation and fecal incontinence in order of frequency. None of the cases developed urinary incontinence or other urinary complications after PSARP. Seventy-three percent of the patients had voluntary bowel movements [VBMs] and 51% were totally continent. Although the PSARP has a negligible complication rate, the success and outcome of the surgical correction in view of the bowel function depend on the development of the sacral nerves. SR reflects the sacral bone development and can be easily calculated by a pelvic AP film. Considering the lower SR in patients suffering from postoperative soling and fecal incontinence, in comparison to the normal group [0.77], SR could be used as a prognostic index to predict the probability of achieving total continence following PSARP

Perinatal testicular torsion: a case report

Torsion of the testicles is typically seen in children of prepubertal age. This condition is extremely rare in perinatal period. The perinatal diagnosis of spermatic cord torsion is often difficult. Here, we report a case of a male newborn baby who was born with swollen and erythematous scrotum. Ultrasonography showed an enlarged left testis, with a nonhomogeneous texture and a regular border, bilateral hydrocele, and septate fluid collection on the left side. Following the diagnosis of acute scrotal condition, surgical exploration was performed. The left testis was gangrened with a blue coloration because of complete extravaginal torsion. Left orchidectomy, along with a fixation of right testis were carried out. Perinatal extravaginal testicular torsion, although a rare condition, is a true immediate emergency that needs early and quick medical attention, and surgical intervention to save testicular tissue