Myxoid Liposarcoma In The Extremities: A Case Report

Liposarcoma is among the most common sarcomas in the adult population. On the contrary, their incidence in the pediatric population is rare. The most common subtype affecting extremities is the myxoid liposarcoma. The preferred method of surgery for the limb myxoid liposarcoma is the limb-preserving surgeries. In the current study, we report the case of a patient who was affected by myxoid liposarcoma and who underwent conservative management with the impression of lipoma several months before the definite diagnosis was obtained. After diagnosis, he underwent radical resection in a single procedure and was referred to the oncology ward for further adjunct treatment.

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

Treatment outcome of neurogenic bladder dysfunction in children; a five-year experience

A neurogenic bladder is one which functions abnormally due to disorders of sacral nerves that control the bladder's ability to fill, store and empty urine. Abnormal bladder function can cause the bladder to be underactive or overactive. This study was planned to evaluate the treatment outcome of our patients with neurogenic bladder dysfunction [NGBD]. Thirty three patients who have been treated for NGBD were evaluated. Diagnosis was confirmed by voiding-cysto-urethrography [VCUG] and urodynamic study. The patients were treated medically and all had clean intermittent catheterization [CIC]. Data regarding age, sex, clinical and paraclinical findings, sonography, imagings, renal scan, associated anomalies, treatment and outcomes were collected and entered in SPSS software version 18 and analyzed by descriptive statistical. Totally 33 patients aged three days to four years [mean 6.8 months] were included in this study. There were 20 [61%] males and 13 [39%] females. Mean follow-up period was 3.4 +/- 1.2 years [1.5 months to 5 years]. Eighty two precent cases had bilatral and 18% unilatral hydronephrosis and bilatral vesicouretral reflux [VUR] existed in 67% and unilatral in 33% of the patients. Treatment consisted of antibiotherapy and CIC in all patients, which was only in 33% of the cases succesful. The most common associated anomaly was meningomyelocle in 8 patients. Vesicostomy was performed in 22 [67%] cases. Kidney scan showed scar in 10 patients at follow-up study. Complete continence on follow-up was achieved in 24 [71%] patients, and it was improved in 6 [18%] cases. Mortality rate was 9% [3 cases]. Cure rate was 85% in urinary tract infection, 82.7% in hydronephrosis, 80% in VUR and 86.5% in kidney function. Anticholinergic medications was not effective in all our patients. We believe that permanent vesicostomy is an effective and acceptable surgical intervention for protection of upper urinary tract decompression, especially in those who do not respond to medical treatment and have high risk position

Posterior urethral valves; a single center experience

Posterior urethral valves [PUV] are the most common cause of bladder outlet obstruction in infancy that impair renal and bladder function. This study was planned to evaluate and record the various clinical presentations and management, complications, and surgical management and long-term outcome of PUV. In a retrospective study, 98 patients who have been treated for PUV are evaluated in Mofid Children's Hospital from January 2007 to December 2012. Detailed history taken and paraclinical examinations were performed in each patient and diagnosis was confirmed by voiding-cysto-urethrography [VCUG]. PUV had been ablated in 62 patients by electric hook, and diversion was performed in 42 [42.85%] cases. Data were analyzed by SPSS software versionlS. Totally 98 patients with mean age at diagnosis 62 [ +/- 13] days were included in this study. Fifty seven cases had been catheterized within one to 6 days of life [mean age one day], PUV was ablated in 62 patients by electric hook, and dive rsion was performed in 42 cases. The most common symptom in our group was dribbling poor stream 51% and urinary tract infection [UT1] 40.8%. There was vesicoureteral-reflux [VUR] in 61.2%, and hydronephrosis in 82.6%. Most common associated anomaly was kidney anomalies [multicystic kidney disease and renal agenesis/dysplasia] in 8 [8.2%] patients. Twenty patients had prenatal diagnosis of PUV. Complication occurred in three [3.1%] patients. Mortality occurred in 5 [5.1%] patients. Mean follow-up period was 3.4 +/- 1.2 years [1.5 months to 5 years]. Urinary drainage by feeding tube in early days of infancy, followed by valve ablation is the best treatment in PUV, and urinary diversion improves the outcome. VCUG is still the gold-standard imaging modality for documenting PUVs. The factors like renal dysplasia and UTI have their role in final outcome

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]

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

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

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

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

Penile agenesis: report on 8 cases and review of literature

Penile agenesis [PA] is an extremely rare anomaly with profound urological and psychological consequences. The opening of the urethra could be either over the pubis or at any point on perineum or most frequently in anterior wall of the rectum. The aim of treatment is an early female gender assignment and feminizing reconstruction of the perineum. We report 8 cases of penile agenesis with urination and defecation through the rectum, apparently normal scrotum, bilateral descended testis, normally located anus, urethral opening in anus, 46xy karyotype and associated anomalies. In 2 cases parents refused any surgical interventions, but in 6 cases we did perform different operations [transforming five cases to females and one case to male gender]. We recommend feminizing operations in newborns or infants, but in older patients, regarding the child's psychology, it is advised to perform masculinizing operations, and finally, no surgical intervention should be undertaken before counseling the parents

Urogenital tract abnormalities associated with congenital anorectal malformations

Genitourinary anomalies in patients with imperforate anus are a frequent source of significant morbidity. Variability of reports on the incidence of associated anomalies with imperforate anus mandates investigation on this issue. The case records and imaging studies of 105 patients who underwent surgery for imperforate anus over a 10-year period are retrospectively reviewed. Voiding cystouretherography, intra venous pyelography [IVP] and ultrasound were performed in patients with intermediate or high level anorectal lesions. During 10 years, there were 48 boys [45.7%] and 57 girls [54.3%] with imperforate anus. Genitourinary anomalies were seen in 34 [48.6%] patients with intermediate or high level anorectal lesions. Eighteen of them [52.9%] were shown to have vesicouretral reflux, only 6 of them required surgical correction. Vesicoureteral reflux was the most prominent urologic anomaly; other anomalies such as ureteropelvic junction [UPJ] stenosis, hydronephrosis, hypospadias, renal agenesis and undescended testis were seen in these series Patients with anorectal malformations should be evaluated for urinary tract and spinal anomalies