Customized approach for upfront or delayed resection using radiological criteria in unilateral, nonmetastatic pediatric renal tumors: A prospective study.

BACKGROUND: The availability of robust, equivalent data regarding outcomes for upfront or delayed surgery for renal tumors in children leads to a dilemma in selecting the initial treatment. Imaging criteria associated with the probability of rupture or incomplete resection may provide a more objective assessment for customization for the timing of surgery. PROCEDURE: Eighty-three children with unilateral, nonmetastatic renal tumors were enrolled between January 2012 and April 2018. Upfront nephrectomy was performed in the absence or delayed surgery (after a biopsy and chemotherapy) in the presence of one or more imaging-based high-risk features, including perinephric spread or adjacent organ infiltration, tumors crossing the midline, intravascular thrombus, and extensive adenopathy. Post hoc analysis for interobserver concordance for high-risk imaging features was also performed. RESULTS: The upfront surgery group (19) had predominantly stage I or II diseases (89%) and the histological types were Wilms (13), non-Wilms (5) renal tumor, and an inflammatory lesion. The delayed surgery group had 60% with stage I or II diseases and the histological types were Wilms (60) and non-Wilms (4) tumor. In addition, high-risk pathology was identified in nine patients. Overall, 27 patients with Wilms tumors required radiotherapy and anthracycline because of stage III disease, including one in the immediate surgery group. The event-free and overall survival (OS) at a median follow-up of 39 months for Wilms tumor are 88% (95% confidence interval [CI]: 78.5-94.9%) and 89% (95% CI: 81.4-96.6%), 85.1% (95% CI: 73.8-93.4%) and 86.5% (95% CI: 77.4-95.8%) for the delayed, and 100% event-free survival as well as OS (P = .1) in the upfront surgery group. CONCLUSION: A customized approach pivoted on image-based high-risk features facilitates identification of patients with early-stage renal tumor when the timing of surgery is tailored. Moreover, non-Wilms tumor and high-risk pathology are also identified.

Outcomes and complications of surgery in patients with intermediate-risk neuroblastoma: experience from an Indian tertiary Cancer Centre.

PURPOSE: The treatment of intermediate risk (IR) neuroblastoma has evolved with the focus now on reducing the drugs, dosage, and duration of chemotherapy. The aim of this study is to present the outcomes of treatment and the complications of surgery in patients with IR neuroblastoma treated at a tertiary cancer center in India. METHODS: All eligible patients with IR neuroblastoma treated between April 2005 and August 2016 were identified. The presence and number of image-defined risk factors (IDRF) before and after neoadjuvant chemotherapy were retrospectively analyzed as were the extent of surgery, complications, and outcomes. RESULTS: Of 282 neuroblastoma patients treated during the study period, 54 had IR neuroblastoma. Complete excision was achieved in 25 patients. There were 26 surgical complications in 22 patients with a similar incidence in patients with complete (n = 13) or incomplete (n = 13) resection (p = 0.78). After a median follow-up of 47 months, the 4-year overall and event-free survival was 91.5% and 75%, respectively. There was no difference in survival between patients who underwent complete resection versus those with incomplete resection (p = 0.9). CONCLUSION: Outcomes of IR neuroblastoma are favorable. The extent of resection does not affect the survival and complications can occur even when the resection is incomplete.

Rhabdomyosarcoma of the breast: Report of two cases with the review of literature.

Rhabdomyosarcoma (RMS) is a common soft-tissue neoplasm in the pediatric age group. Common locations are head and neck, genitourinary areas, trunk, and extremities. Two pathologic variants of this malignancy are embryonal and alveolar. The involvement of breast is rare. Herein, we report two cases of alveolar RMS of the breast of which one is an isolated breast metastasis from an orbital primary, whereas the other is a primary RMS of the breast. Both the patients were treated with surgery followed by adjuvant chemotherapy and radiotherapy and are currently disease free at six and three year respectively, following completion of treatment.

Pancreatoblastoma masquerading as hepatoblastoma: A diagnostic dilemma.

Clinicoradiological and histopathological differentiation of pancreatoblastoma from hepatoblastoma can often be a challenge in clinical practice owing to their peculiar resemblance. We report a case of a 4-year-old boy with a right hypochondriac region mass, which was diagnosed as hepatoblastoma on the basis of imaging, raised tumor marker, and biopsy; however, pancreatic origin of the mass was ascertained on exploration and pancreatoblastoma was confirmed on histopathology.

Aggressive cervical neuroblastoma with a rare paraneoplastic syndrome: A therapeutic dilemma.

Neuroblastoma is infrequently associated with paraneoplastic syndromes. Amongst the few, opsomyoclonus (Kinsbourne syndrome) is the most common neurological paraneoplastic syndrome and diarrhea secondary to increased secretion of vasoactive intestinal peptide (Kerner-Morrison syndrome), hormonal paraneoplastic syndrome. Hypothalamic dysfunction (HD) is a rare disorder and its manifestation as a paraneoplastic syndrome of neuroblastoma is uncommonly reported. We present an interesting case of an unrelenting cervical neuroblastoma associated with HD, which posed a therapeutic challenge.

PRETEXT II-III multifocal hepatoblastoma: significance of resection of satellite lesions irrespective of their disappearance after chemotherapy.

PURPOSE: To determine the outcomes of resection of satellite lesions in PRE-treatment Tumor EXTension (PRETEXT) II and III multifocal hepatoblastoma irrespective of their disappearance after chemotherapy. To compare the overall outcomes of multifocal and unifocal hepatoblastoma. METHODS: Fourteen patients with PRETEXT II (n = 7) and III (n = 7) multifocal hepatoblastoma treated between April 2006 and July 2014 were analyzed and their outcomes were compared with PRETEXT II and III unifocal hepatoblastoma treated in the similar period. RESULTS: Satellite lesion or the affected segments with disappeared satellite lesions were resected in 11 patients. Amongst them, all relapses were distant except one in the liver. In contrast, two of three patients developed liver relapse when the affected segments were not resected. None of the patients receiving intensive chemotherapy based on SIOPEL-3 guidelines developed a relapse. The 3-year event-free and overall survival were 38.6 and 42.9% in multifocal hepatoblastoma and 86.4 and 92.4% in unifocal hepatoblastoma (p = 0.001). Multifocality (p = 0.002) and AFP >10,000 after induction chemotherapy significantly affected event-free survival (p = 0.01). CONCLUSION: Multifocal hepatoblastoma is associated with poor outcomes as compared to unifocal hepatoblastoma. These preliminary observations of relapse and the role of chemotherapy intensification deserve further study in a multicenter controlled trial setting.

Benign liver tumors in children: outcomes after resection.

PURPOSE: Benign liver tumors in children are rare and data regarding the surgical outcomes are deficient. We reviewed our experience in the management of these tumors particularly the extent, safety, and effectiveness of surgical resection. METHODS: Between March 2005 and March 2014, 10/90 liver resections were performed for benign liver tumors. Three other patients received conservative treatment. Demographic characteristics, operative management, morbidity, and mortality were analyzed. RESULTS: The median age of the patients was 12 months. The distribution of pathology was infantile hepatic hemangioendothelioma/hemangioma (n = 7), mesenchymal hamartoma (n = 4), adenoma (n = 1) and focal nodular hyperplasia (n = 1). Median tumor size was 12.25 cm (range 3.5-21 cm) with a median tumor volume of 576.64 cm(3) (range 13.9-1822.64 cm(3)). Non-anatomic resection was performed in 6/10 patients and the median blood loss was 100 ml (range 10-850 ml). Median length of hospital stay was 7 days (range 5-9 days). There were no mortality, none of the patients had a local recurrence, and all are alive at a median follow-up of 33 months. CONCLUSIONS: Two-third patients with benign liver tumors were managed with surgical excision, which comprised 11% of our resectional practice. Non-anatomical resection whenever feasible can be performed safely and does not compromise the oncological outcomes.

Non-rhabdomyosarcoma soft-tissue sarcomas in children: Contemporary appraisal and experience from a single centre.

Nonrhabdmyosarcoma soft tissue sarcomas (NRSTS) include a cluster of different types of soft tissue sarcomas clubbed together due to the rarity of individual subtypes. The diagnostic accuracy is lately reinforced due to the availability of immunohistochemical and molecular markers. Surgery is the central modality of treatment since many of them are insensitive to chemotherapy. With the availability of rational risk stratification system, efforts are in progress to evaluate the role of neoadjuvant chemotherapy and radiotherapy to improve outcomes especially for the locally advanced disease. The survival remains dismal for metastatic disease. This review highlights the current status of NRSTS and also describes the experience from a single centre in treatment of NRSTS.

Primary non-metastatic Ewing sarcoma of the jaw in children: results of surgical resection and primary reconstruction.

BACKGROUND AND OBJECTIVE: The rarity of Ewing sarcoma (ES) of the jaw coupled with the technical challenge of resection and associated functional and cosmetic impairment has resulted in deficient data on surgical management of these tumors. The purpose of this study is to describe the results of surgical excision and reconstruction of primary non-metastatic ES of the mandible and maxilla in children. METHODS: Consecutive patients (mandible = 6, maxilla = 5) treated with surgery from August 2005 to January 2013 were selected. All patients received induction chemotherapy and were selected for surgical resection based on the presence of specific criteria for operability. RESULTS: The median age was 11.5 years (range 5-16 years). Free fibular osteocutaneous flap was commonly used for reconstruction. There were no complications related to microvascular anastomosis or flap loss. Five patients had 100% tumor necrosis and did not receive radiotherapy. Teeth alignment, chewing, swallowing, and speech were normal in all and donor site morbidity occurred in one. The 5-year overall, event-free survival, and local control are 87.5%, 72.9%, and 90%, respectively. CONCLUSION: In eligible patients, surgery with contemporary reconstruction results in optimal oncological and functional outcome. Surgery also has the added advantage of identifying patients who may not need radiotherapy.

Utility of immunologic testing in suspected rheumatologic disease.

The use of diagnostic testing in the clinical practice of medicine has been a shifting landscape from the time that the first blood test was utilized. This is no different in the field of immunology and in particular rheumatology. As the field of immunology is relatively young, the clinical tests are not well established and therefore guidelines for use are still under debate. In this review, we seek to look at some of the key autoantibodies, as well as other tests that are available to diagnose suspected rheumatologic disease, and examine how to best use these tests in the clinic. In particular, we will focus on the anti-nuclear antibodies, anti-neutrophil cytoplasmic antibodies, complement, cryoglobulins, rheumatoid factor, and anti-citrullinated protein antibodies.

Anti-IL-5 therapy reduces mast cell and IL-9 cell numbers in pediatric patients with eosinophilic esophagitis.

BACKGROUND: Eosinophilic esophagitis (EoE) is a clinicopathologic entity of increasing worldwide prevalence. IL-5 is essential for eosinophil trafficking, and anti-IL-5 therapy decreases esophageal eosinophilia. EoE is associated with prominent mast cell infiltration. OBJECTIVE: We investigated whether anti-IL-5 (mepolizumab) treatment reduced esophageal mast cell accumulation in biopsy specimens from pediatric patients with EoE from a previous randomized anti-IL-5 trial. METHODS: A subanalysis was completed for children treated with 0.55, 2.5, or 10 mg/kg mepolizumab monthly for 12 weeks followed by no treatment until week 24. Quantitative immunochemistry was used to assess the numbers of eosinophils, tryptase-positive mast cells, IL-9(+) cells, and mast cell-eosinophil couplets before and after treatment. RESULTS: Forty-three biopsy specimens had adequate tissue for paired analysis. Forty percent of subjects responded to anti-IL-5 (defined as <15 eosinophils per high-power field [hpf] after mepolizumab therapy), and 77% of all subjects had decreased numbers of mast cells after anti-IL-5. In responders epithelial mast cell numbers decreased from 62 to 19 per hpf (P < .001), were significantly lower than in nonresponders after therapy (P < .05), and correlated with eosinophil numbers (r = 0.75, P < .0001). Mast cells and eosinophils were found in couplets before therapy, and these were significantly decreased only in responders after anti-IL-5 (P < .001). Esophageal eosinophils comprised the majority of cells that made the mast cell growth factor IL-9. IL-9(+) cell numbers decreased from 102 to 71 per hpf (P < .001) after anti-IL-5. CONCLUSIONS: Pediatric patients with EoE had significantly fewer mast cells, IL-9(+) cells, and mast cell-eosinophil couplets in the esophageal epithelium after anti-IL-5 therapy. Because eosinophils were one source of IL-9, they might support esophageal mastocytosis.

Anemia and Nutritional Status in Tuberculosis Patients.

INTRODUCTION: Anemia is a common complication of pulmonary tuberculosis (TB). Nutritional deficiency and malabsorption syndrome can deepen the severity of anemia. The aim of the present study was to study anemia and nutritional status in patients with TB at a tertiary care center. MATERIALS AND METHODS: All patients diagnosed with TB (pulmonary and extrapulmonary), registered withRevised National TB Control Programme (RNTCP), taking DOTS regimen, attending the outpatient department as well as those admitted in the medicine and chest units of a tertiary care hospital, were enrolled in the present study. RESULTS: Anemia was more common between the age groups of 51 and 60 years, with distribution of males (87 [58%]), patients with diabetes (49 [32%]), and hypertensive patients (29 [19.3%]). In our study, 48 (85.7%) out of 56 patients diagnosed with extrapulmonary TB were found to have anemia; similarly, 77 (88.5%) out of 87 patients diagnosed to have pulmonary TB were anemic. Anemia of chronic disease (128 [97.17%]) was higher when compared to that of those with iron-deficiency anemia (3 [2.29%]). According to body mass index (BMI), 135 (90%) patients were underweight; according to mid-arm circumference (MAC), 131 (87.3%) patients had severe malnutrition; and according to waist-hip ratio [WHR], 96 (64%) patients were underweight. CONCLUSION: Anemia was common in males and alcoholics, and there was a high prevalence of anemia of chronic disease. In addition, anemia was associated with high erythrocyte sedimentation rate and C-reactive protein. High proportions of TB patients (pulmonary and extrapulmonary) were classified as underweight and malnourished on the basis of different parameters (BMI, MAC, and WHR); in addition, the degree of malnutrition was higher in patients with anemia than in those without.

Intravascular extension of Wilms tumor: Characteristics of tumor thrombus and their impact on outcomes.

BACKGROUND: Studies describing intravascular involvement in Wilms tumor have focused on illustrating individual institutional experience and the elements of surgical management. Thrombus characteristics like extent, patterns of regression, and correlation with the surgical findings, intraluminal adhesion, and viable tumor in the thrombus, and patency of the inferior vena cava (IVC) have not been systematically described. OBJECTIVES: The aim of this study is to evaluate these thrombus characteristics and explore their impact on the overall outcomes. METHODS: All patients with histologically confirmed Wilms tumors with intravascular thrombus diagnosed in the pediatric oncology unit of Tata Memorial Hospital registered from 2006 to 2019 were included. Data regarding clinical, radiological, and surgical particulars were retrieved from the prospectively maintained institutional database. Specific data for the thrombus included: distal extent before and after neoadjuvant chemotherapy, correlation of extent with the surgical findings, completeness of thrombectomy, the presence of a viable tumor in the thrombus, and the patency of the IVC. Survival analysis was performed utilizing the Kaplan-Meier method on SPSS software version 25. RESULTS: The study included 43 (9.9%) of the 432 patients with Wilms tumor having intravascular extension. Retrohepatic IVC (33.3%) followed by atrioventricular (26%) formed the frequent levels of thrombus with maximum regression occurring after chemotherapy in the latter (Summary figure). The overall concordance rate between computed tomography (CT) scan and surgical findings for the presence of thrombus was 86% and 4 patients had the thrombus limited to a lower level than the preoperative scan. At a median follow-up of 5-years, the 5-year event-free and overall survival was 81% and 82.2% respectively. Atrioventricular thrombus (p = 0.003) and postoperative patency of IVC (p = 0.02) were significantly associated with inferior survival, while the extent of regression, thrombus fracture, and viability was not significant. DISCUSSION: The findings of this study bring forth the characteristics of intravascular tumor thrombus affecting the outcomes which can be validated in future prospective studies. Although the ideal method for radiological assessment of the intravascular thrombus is elusive, CT scan provided adequate information for the presence and level of the intravascular thrombus with reasonable accuracy in this study. Study limitations include small sample size, the limited number of events, and lack of multivariate analysis to rule out confounding factors that could influence the observed findings. CONCLUSION: Atrioventricular thrombus and occlusion of IVC represent adverse prognostic factors. The extent of regression, fracture, and viability of thrombus did not affect survival in this study.

Incidence, treatment, and outcomes of primary and recurrent Non-Wilms renal tumors in children: Report of 109 patients treated at a single institution.

INTRODUCTION: Non-Wilms renal tumors represent a compelling subset of childhood renal tumors. However, their relative rarity renders accurate diagnosis, and therapy challenging which in some instance is inferred from their adult counterparts. OBJECTIVE: To describe the incidence and analyze the diagnostic challenges, therapies and, outcomes of non-Wilms renal tumors at the largest tertiary cancer centre in India. METHODS: All patients with histologically confirmed non-Wilms renal tumours diagnosed in the paediatric oncology unit of Tata Memorial Hospital between 2006 and 2019 were included. Data regarding clinical and radiological features and treatment outcomes were retrieved from the prospectively maintained institutional database. At the outset, histological types were categorised into a high and low-risk group depending on anticipated survival. Survival analysis was performed utilising the Kaplan-Meier method on SPSS software version 24.0. RESULTS: Of the 569 patients with renal tumors, 109 (19%) patients with primary (n = 97) or recurrent (n = 12) non-Wilms renal tumors were included. Histological high-risk group included clear cell sarcoma (CCSK) (39.4%), renal cell carcinoma (RCC) (19.3%), malignant rhabdoid tumor (MRTK) (12.8%), Ewing's sarcoma (rES) (15.6%), synovial sarcoma (2%), and undifferentiated sarcoma (2%). The low-risk group comprised of congenital mesoblastic nephroma (CMN) (4.6%), cystic partially differentiated nephroblastoma (2%), and other rare tumors (3%). Diagnostic error occurred in 2 patients in the high-risk group. All low-risk tumours were treated with surgery alone and most (97%) high-risk tumors were operated either upfront (61.5%) or after preoperative chemotherapy (38.4%). Adjuvant therapy based on histology was offered to 70%. The recurrent tumors received various salvage treatments including chemotherapy; radiotherapy; surgery and immunotherapy, however, only 2 patients could be salvaged. The 3-year overall survival for the entire cohort with primary tumors was 59%, and the survival rates were 76.7%, 77.9%, 0.0%, and 52% for CCSK, RCC, MRTK, and rES (summary figure). Low-risk tumors had 100% survival while the recurrent tumors had a median survival of 10.5 months. CONCLUSIONS: Non-Wilms renal tumors constitute a heterogeneous group of tumors, accounting for less than 20% of all renal tumors. Low-risk tumors are associated with excellent outcomes following surgery alone while the high-risk tumours have a variable outcome. MRTK and recurrent non-Wilms tumour have the worst survival. Favourable outcomes for CCSK and RCC and worst outcomes for MRTK were observed in this study. Renal ES have higher incidence of treatment failure and unsatisfactory outcomes. Recurrent non-Wilms tumours have an extremely poor outcome and more alternative or innovative approaches are needed for their treatment.

Standardizing lymph nodal sampling for Wilms tumor: A feasibility study with outcomes.

BACKGROUND: Despite being mandated by cooperative groups, omission of nodal sampling is the most frequent protocol deviation in surgery for Wilms tumor. The stations as well as the number of nodes that should be sampled are not clearly defined resulting in a marked variation in practices among surgeons. We propose a systematic method for nodal sampling intending to reduce interoperator variation. In this study, we have assessed the feasibility and yield of systematic lymph node sampling and also evaluated the factors influencing nodal metastasis. METHODS: Prospective evaluation of 113 Wilms tumor patients operated at a single tertiary cancer center between 2015 and 2019. All these patients underwent a systematic 5-station nodal sampling. RESULTS: Median lymph node yield was 8 and 13.2% (15/113) patients harbored a histologically positive nodal disease. Of the patients with positive nodal disease, interaortocaval nodes had metastasis in 46.7% (n = 7). They represented isolated sites of nodal disease (skip metastases) in 28.6% (n = 4) of patients. Right-sided tumors had more frequent involvement of interaortocaval nodes and skip disease. Tumors with high-risk histology had 12.5 times more odds of harboring nodal disease as compared to low and intermediate-risk histology Wilms tumor. CONCLUSIONS: The proposed method of systematic station wise sampling provides a template to guide surgeons in performing lymph node harvesting. Interaortocaval nodes sampling should be performed routinely as the incidence of disease at this station is sufficiently high and metastasis may skip hilar nodes. STUDY OF DIAGNOSTIC TEST: Level III evidence.

Vasoconstrictor responses in the upper and lower limbs to increases in transmural pressure.

The purpose of this study was to examine upper and lower limb vasoconstrictor responses to changes in transmural pressure in humans. Brachial and femoral blood mean blood velocity (MBV) and vessel diameter (Doppler ultrasound) were measured in 20 supine healthy subjects (10 men and 10 women; 27 +/- 1 yr; mean +/- SE) during four levels of limb suction at -25, -50, -75, and -100 mmHg, respectively. Limb suction led to an initial rise in MBV followed by a rapid fall in flow velocity to a level below MBV baseline, indicating a vasoconstriction effect. Femoral compared with brachial vessels exhibited a greater fall in flow velocity at all levels of suction (-89 +/- 17 vs. -10 +/- 2, -142 +/- 11 vs. -14 +/- 2, -156 +/- 22 vs. -13 +/- 2, and -162 +/- 29 vs. -12 +/- 2 ml/min for -25, -50, -75, and -100 mmHg, respectively; interaction effect, P < 0.05). Even at low tank suction levels (i.e., -10 and -20 mmHg), significant brachial flow velocity vasoconstriction from baseline values was demonstrated, reflecting downstream resistance vessel changes (n = 14). Brachial and femoral diameters did not change during changes in negative tank pressure. During suction, changes in limb volumes were significantly greater in the forearm (1.4 +/- 0.5%, 2.4 +/- 0.8%, 3.5 +/- 1.0%, and 4.3 +/- 1.1%) compared with the calf (0.9 +/- 0.5%, 1.4 +/- 0.7%, 2.0 +/- 0.8%, and 2.8 +/- 1.1%) at all levels of negative tank pressures (-25, -50, -75, and -100 mmHg, respectively). Simultaneous measurements of both upper limbs and both lower limbs suggested that the majority of the reduction in flow was due to myogenic influences except when -100 mmHg of suction was applied to the lower limb. The greater vasoconstriction responses in the leg compared with the arm with suction appear to be influenced by both myogenic and sympathetic mechanisms.

Portal Vein Embolization for Future Liver Remnant Enhancement and Combined Modality Treatment for the Management of Post-hepatic Resection Biliary Fistula in an 18-Month Old Child With Hepatoblastoma.

Hepatic resection is the mainstay of treatment for hepatoblastoma. However, the presence of adequate future liver remnant (FLR) is essential to prevent postoperative liver failure. Portal vein embolization (PVE) is commonly utilized in adults for promoting hypertrophy of FLR, however, it is sparingly used in children. Secondly, bile leak after liver resections is a well-defined complication. Apart from conservative treatment such as drainage and antibiotic, several management strategies including endoscopic, percutaneous, and surgical approaches have been described for its management. We present an 18-month old child with hepatoblastoma for whom PVE was performed to enhance the FLR so that an extended right hepatectomy could be accomplished. The same patient endured delayed postoperative biliary leak wherein the conservative, and non-operative interventional procedure failed, however, surgery combined with intraoperative interventional radiology procedure was utilized with a favorable outcome.

A cross-sectional study of the distribution of pediatric solid tumors at an Indian tertiary cancer center.

CONTEXT: Pediatric solid tumors include a heterogeneous group of tumors, and the burden of these tumors, especially from resource-challenged countries, is not well described. AIMS: The aim of this study was to describe the distribution of solid tumors in children and the treatment outcome of Wilms tumor and hepatoblastoma. PATIENTS AND METHODS: All patients under 15 years of age with histologically confirmed tumors presenting at a tertiary cancer center from January 2012 to December 2016 were identified from the hospital database. Patients with lymphomas, bone, and central nervous tumors were excluded. The demographic profile including age, sex distribution, and the treatment received were recorded for all patients. RESULTS: The mean age of the eligible 1944 patients was 5.7 years with majority (57.3%) in the 0-4 years age group. The male-to-female ratio was 1.4:1 with a male predominance in all tumors except germ cell tumors. Soft tissue tumors were the most common tumors followed by neuroblastoma and renal tumors, whereas liver tumors formed only 6.7% of all tumors. Seventy percent of the patients received treatment completely or partially at our institute, whereas 18.3% had no cancer-directed treatment. The 3-year overall survival of patients with Wilms tumor and hepatoblastoma was 85.4 and 78.5%, respectively. CONCLUSIONS: Extracranial and extraosseous pediatric solid tumors include a wide range of tumors with a predilection for male sex and children below 4 years of age. Soft tissue tumors, neuroblastoma, and renal tumors are the most common; the outcomes of Wilms tumor and hepatoblastoma are favorable.

Re-excision after unplanned resection of nonmetastatic nonrhabdomyosarcoma soft tissue sarcoma in children: Comparison with planned excision.

BACKGROUND: It is not exceptional to come by children with initial unplanned surgical intervention for nonrhabdomyosarcoma soft tissue sarcomas (NRSTS). The aim of this study was to evaluate the presence of residual disease in these patients after re-excision and compare the treatment outcomes with patients who had planned upfront excision. METHODS: The data of patients with primary nonmetastatic NRSTS with initially unplanned excision who underwent re-excision between March 2006 and December 2014 were analyzed and the results compared with patients having planned upfront excision in the similar period. RESULTS: Of the 84 patients, 40 (48%) had an unplanned excision elsewhere; 35 of these patients had a re-excision. Twenty-one of the remaining 44 patients underwent upfront planned excision. A residual tumor was present in 16 (45.7%) patients. There was no significant difference in the local recurrence, distant metastases, or deaths in patients with re-excision or planned excision. The 5-year overall, disease-free survival and local control rates were, respectively, 93.5%, 90.2% and 96.6% for the re-excision group and 84.9%, 65.2% and 88.5% in the planned excision group (p=NS). CONCLUSIONS: The probability of residual disease following unplanned excision of NRSTS is high. The outcomes following re-excision are similar to that with planned excision. PROGNOSIS STUDY: Level II evidence.

Clinical characteristics and treatment outcomes of primary and recurrent malignancy involving the salivary glands in children.

BACKGROUND: Malignant salivary gland neoplasms are rare in children. The purpose of this study was to present our experience in the treatment of primary and recurrent salivary gland neoplasm and contribute to the fractional available data. METHODS: All patients <18 years, treated between June 2005 and April 2014 were included in this study. RESULTS: The parotid gland was the most common site for both primary (n = 18) and recurrent (n = 6) tumors and mucoepidermoid carcinoma, low or intermediate grade, main histological type. Surgical excision was performed in all patients, of which 13 patients received radiotherapy. Lymph node metastasis was present in 3 patients with primary tumor, which were assessed accurately on frozen section examination. Local recurrence occurred in 1 patient with primary tumor and all patients are alive. CONCLUSION: Surgery is the prime modality of treatment for malignant salivary gland neoplasm and is associated with excellent outcomes. The role of lymph node sampling and frozen section examination needs further evaluation in identifying lymph node metastasis. © 2015 Wiley Periodicals, Inc. Head Neck 38: 852-856, 2016.