Non-hematological tumors of head and neck region in the pediatric age group in a tertiary care cancer center.

PURPOSE: To analyze the clinical profile, management and outcome of non-hematological tumors in head and neck region in children less than 18 years of age. PATIENTS AND METHODS: Medical records of patients (0-18 years) presenting with non-hematological tumors of head and neck region from 2000 to 2010 were reviewed. RESULT: Out of the 78 patients identified, 50 were males with median age of 14 years (3 months to 18 years). Histologically, 58 out of the 78 patients had malignant tumors and 20 were metastatic at presentation. Common site of tumor origin was noted as thyroid (23/78), salivary glands (21/78) and nasopharynx (13/78). Out of 78 patients, 44 patients were treated by surgery, 26 patients received radiation and 13 patients received chemotherapy, while 9 patients received radio-iodine (I-131) treatment. Median follow up of all cases was 22 months (range 1 month to 8 years). The event free survival was 74.1% and overall survival was 89.6%. Subset analysis revealed patients with thyroid and salivary gland malignancies have excellent survival, while those with squamous cell carcinoma and high risk neuroblastoma had poor outcome. CONCLUSION: This study reveals the interesting profile of head and neck tumors in children, and important role of surgery for favorable outcome.

Outcome of multimodality treatment of Ewing's sarcoma of the extremities.

BACKGROUND: The management of Ewing's sarcoma family of tumors (ESFT, Ewing's sarcoma/primitive neuroectodermal tumor) has been established as a multimodality treatment. Advances in imaging and diagnostics, chemotherapy, surgical techniques, radiotherapy and prosthetic technology have resulted in drastic changes in the outcome of this disease, with most of the recent studies having 5-year survival rates of more than 60%. The Indian patients present at a more advanced stage and the compliance of treatment is suboptimal. While there is plenty of data in the world literature on the outcome of Ewing's sarcoma, there is paucity of data in Indian patients. Therefore, we conducted the present study to analyze the outcome of multimodality treatment of ESFT of the extremities at a tertiary nonprofit institute over a decade. MATERIALS AND METHODS: 34 patients who had histopathologically proven diagnosis of Ewing's sarcoma of the extremities and had received treatment at our institute from 1997 through 2007 were included for analysis. The majority of patients had involvement of the femur (35%), followed by tibia (17%), fibula and foot (15% each), humerus (12%) and soft tissue of thigh (6%). Twenty-nine patients presented with localized disease (Enneking stage II B) while five patients presented with metastases (Enneking stage III). All patients received Vincristine, Actinomycin D, Cyclofosfamide + Ifosfamide and Etoposide (VAC+IE)-based chemotherapy and local treatment was offered to all but three patients having multicentric disease. The local treatment offered were, radiation (n= 15), surgery (n= 12) both surgery and radiation (n=4). All patients were analyzed for oncological outcome (event-free and overall survival, local and systemic relapses) by clinical and imaging evaluation and functional outcome by using the musculoskeletal tumor society (MSTS) score. These outcomes were correlated with age, sex, size of tumor, stage at presentation, modality of local treatment and site of relapse. RESULTS: At the final follow-up (mean, 26 months; median, 17 months; range, 3-97 months), the overall and event-free survivals were 47 ± 12% and 34 ± 9%, respectively. Sixty-two percent of the patients presented with a tumor size more than 8 cm. On correlation with age, sex, size of tumor, stage at presentation, modality of local treatment and site of relapse, no correlation of survival was seen with any of the variables except event-free survival with size of the tumor. The functional outcome of all the patients was satisfactory (MSTS score >16 out of 30). No patient underwent amputation. CONCLUSION: Although the demographic profile, stage at presentation and the local and systemic treatment regimen followed in our study was similar to the world literature, the outcome of Ewing's sarcoma in Indian patients were found to be inferior to that reported in the western literature. Larger multicentric studies with longer follow-up are required to exactly determine the key areas crucial in improving this outcome.

Diagnosing ruptured appendicitis preoperatively in pediatric patients.

BACKGROUND: Over the past decade, pediatric patients with ruptured appendicitis (RA) have been successfully treated with IV antibiotics and an interval appendectomy. Because the treatment of acute appendicitis (AA) and RA in children is now diverging, distinguishing between these two conditions preoperatively is critical. STUDY DESIGN: A prospective cohort study was conducted. Clinical data were collected, and the attending surgeon's preoperative diagnosis was recorded. Accuracy of the pediatric surgeon's diagnosis was determined. Univariable and multivariable logistic regression were then used to determine independent clinical predictors of RA. Using the relative beta coefficients of these predictors, a scoring system was constructed to aid in the diagnosis of RA. RESULTS: Two hundred forty-seven patients were evaluated: 98 AA (40%), 53 RA (21%), and 97 not appendicitis (39%). Median age was 10 years old. The overall accuracy of the pediatric surgeon's preoperative diagnosis was 92%. Sensitivity and specificity for the diagnosis of RA were 96% and 83%, respectively. Multivariable regression analysis identified generalized tenderness on examination, duration of symptoms longer than 48 hours, WBC>19,400 cells/microL, abscess, and fecalith on CT scan as independent predictors for RA. A novel scoring system was developed with these variables, and, when applied to the study population, the specificity for the diagnosis of RA improved to 98%. CONCLUSIONS: Pediatric surgeons differentiate AA from RA and not appendicitis preoperatively with high accuracy and sensitivity, but the specificity for diagnosing ruptured appendicitis is lower. The scoring system improved the specificity of the preoperative diagnosis. The validity and utility of this scoring system should be examined in future studies in larger patient populations.

Surgical management of necrotizing enterocolitis and isolated intestinal perforation in premature neonates.

Necrotizing enterocolitis (NEC) and isolated intestinal perforation (IP) are two relatively common disease conditions that require neonatal surgical therapy. The early mortality rate approaches 50%, and survivors frequently have growth and neurodevelopmental impairment. Much discussion has occurred regarding whether initial drain placement alone or laparotomy with resection of diseased intestine should be the initial surgical therapy. Several recent prospective studies have shown that the early mortality rate is likely not significantly different after either of these surgical approaches. Major morbidity, especially the likelihood for neurodevelopmental impairment, may be different in the two treatment groups. Further prospective trials are needed to further explore this question and are planned. Studies focusing on prevention of NEC and IP and also on improved medical treatment are needed to allow a major advance in the outcomes of infants with NEC and IP. As these studies are being performed, trials evaluating existing medical and surgical therapies are also needed.

Clinical implications and surgical management of intussusception in pediatric patients with Burkitt lymphoma.

BACKGROUND/PURPOSE: Intussusception as a presenting feature of Burkitt lymphoma may be associated with early stage disease, which is curable with less intensive therapy. We determined the incidence, presentation, stage, and outcome of children with Burkitt lymphoma presenting with intussusception. METHODS: The medical records of patients with Burkitt lymphoma treated at our hospital from 1962 to 2005 were reviewed, and the patients presenting with intussusception were then further analyzed. RESULTS: Of 189 patients with primary abdominal Burkitt lymphoma, 33 (17.5%) presented with intussusception. Their median age at diagnosis was 10 years (range, 3-19 years). Most patients presented with abdominal pain (88%) and/or nausea/vomiting (42%). Twenty-three (70%) of these 33 patients as compared with 10 of the other 156 patients with abdominal lymphoma could have complete resection of their tumor (P < .0001) and hence had low stage disease (stage II). Only 10 patients with intussusception had stage III (n = 7) or stage IV (n = 3) unresectable disease. Twenty-five of the patients remained alive in continuous complete remission for 3 months to 31 years (median, 14 years). CONCLUSION: Pediatric patients with Burkitt lymphoma presenting with intussusception often have completely resectable disease and are older than general pediatric patients with intussusception.

Significance of pleural effusion in neuroblastoma.

BACKGROUND: Pleural effusion is uncommon at diagnosis of neuroblastoma in children. Because the presence of malignant cells in pleural fluid may significantly change the management and outcome of patients with neuroblastoma, we retrospectively analyzed a cohort of neuroblastoma patients who presented with pleural effusion at the time of diagnosis to determine the incidence, presentation, stage, treatment, and outcome of these patients. METHODS: We reviewed the presenting features of 295 patients with the diagnosis of neuroblastoma who received treatment at St. Jude Children's Research Hospital between 1991 and 2005. Patients were chosen for further analysis if pleural effusion had been identified on chest radiographs or computed tomography (CT) scans at diagnosis RESULTS: Thirty-one out of 295(10.5%) patients with neuroblastoma had pleural effusion identified at time of presentation. International neuroblastoma staging system (INSS) risk stratification was high risk in 29 cases and intermediate risk and low risk in 1 case each. The primary site of disease was abdomen in 26 patients; mediastinum in 5. We conducted cytologic analysis of pleural fluid of nine patients; the specimen of seven contained malignant cells. Eighteen of 31 patients died of progressive or recurrent disease. CONCLUSION: In patients with neuroblastoma, pleural effusion is usually associated with unfavorable biologic features and high-risk disease. Pleural fluid should be examined cytologically and at a time when the results would change the risk stratification. There was no statistically significant difference in the survival rate of the patients with high-risk neuroblastoma with or without malignant pleural effusion.

Evaluation of pediatric oncology patients with previous multiple central catheters for vascular access: is Doppler ultrasound needed?

BACKGROUND/PURPOSE: Pediatric oncology patients who have undergone placement of multiple central venous catheters may have thrombosis or stenosis in the upper venous system. The purpose of this study was to identify factors that predict venous thrombosis or stenosis and to evaluate the role of Doppler ultrasonography in assessing the upper venous system of pediatric patients requiring multiple central vascular catheters. METHODS: The medical records of eligible patients were reviewed with regard to demographics, primary disease, type of catheter, duration of previous central venous access, association with infection, operative notes, and Doppler ultrasonographic findings. RESULTS: Our evaluation criteria were met in 50 cases (47 patients). In 10 cases, Doppler ultrasonography revealed abnormality in the upper venous system. Patient demographics, primary disease, type of catheter, duration of previous central venous access, or association with infection were not found to significantly predict the abnormality in the upper venous system. Placement of central venous access device was performed without difficulty when the site of placement was chosen on the basis of ultrasonographic findings. CONCLUSION: Doppler ultrasonography is useful in diagnosing thrombosis or stenosis in asymptomatic pediatric patients requiring placement of multiple central venous catheters.

Neonatal epithelioid sarcoma: a distinct clinical entity?

Epithelioid sarcoma is a rare soft tissue sarcoma with a propensity for local aggressiveness, regional nodal spread, and pulmonary metastases. We report a case of epithelioid sarcoma in a neonate with bilateral optic nerve hypoplasia who developed liver, kidney, and bone metastases. The unusual presenting features and pattern of disease progression in this patient suggest that a different disease evaluation strategy should be considered for infants with epithelioid sarcoma.