Monica Bhagat

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

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.

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

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.

Recurrent abdominal wall dermatofibrosarcoma protuberans in a child: a challenging reconstruction

IntroductionDermatofibrosarcoma protuberans is an uncommon low-grade soft tissue sarcoma with a high potential for recurrence as it has irregular finger like extensions.Case descriptionWe report a case of a large, recurrent dermatofibrosarcoma protuberans in a child involving the anterior abdominal wall, which posed a challenge for reconstruction. Peritoneum sparing full thickness resection of the anterior abdominal wall, meshplasty and a free anterolateral thigh flap was performed for reconstruction of the defect.Discussion and evaluationLarge composite defect, involving more than half of the anterior abdominal wall, necessitate a free flap reconstruction. Although these reconstructions are technically challenging in children, they are the only option available.ConclusionComplete surgical excision is essential for DFSP of the abdominal wall, which may result in large challenging defects. Free flaps remain the only option in this scenario and hence it is essential to have expertise for microvascular flap reconstruction.

Chyle leak following surgery for abdominal neuroblastoma

BACKGROUND The incidence and optimal management of postoperative chylous leak of neuroblastoma is inadequately documented. We analyzed the risk factors, management, and the implication of chyle leak following surgery for abdominal neuroblastoma. METHODS One hundred sixty patients who underwent surgery for abdominal neuroblastoma between September 2004 and August 2014 were evaluated. To find the oncological implication we evaluated the delay in starting further treatment, local control, event free and overall survival. RESULTS Chyle leak was the most common complication (20%). The median measure of leakage was 100ml/day and it persisted for a median of 12days. All patients were managed conservatively except one who needed exploration for wound dehiscence. Number of lymph nodes resected was the only factor associated with the risk of chyle leaks (p=0.013). Adjuvant chemotherapy was not delayed in any patient because of chyle leaks per se and the local control, event free and overall survival were not different for patients with and without chyle leak. CONCLUSION Chylous leakage is a common postoperative complication of abdominal neuroblastoma, predisposed by the number of lymph nodes resected. It responds to conservative management and does not compromise further the oncological treatment and outcome hence; it should not be a deterrent to complete surgery.

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.

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

PurposeThe 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.MethodsAll 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.ResultsOf 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).ConclusionOutcomes of IR neuroblastoma are favorable. The extent of resection does not affect the survival and complications can occur even when the resection is incomplete.

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.

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.

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.

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.