OBJECTIVES:Primitive neuroectodermal tumors (PNET) are rare highly aggressive neoplasms. The diagnosis is made by histopathology with the support of immunohistochemistry (IHC) and cytogenetics. The aggressive multimodality treatment is recommended for the management of these tumors. The purpose of our study is to review our experiences in the diagnoses and treatment of PNET of the kidneys.MATERIALS AND METHODS:We retrospectively reviewed the data of all the patients, who were treated for the PNET of the kidneys at our institute between April and March 2011 and compared with the available literature.RESULTS:A total of eight patients were treated for PNET of the kidney. Out of the eight patients, four were males and four females. Nearly 50% of our patients had inferior vena caval thrombus at the time of presentation. The diagnosis was made on histopathology supported by IHC. Out of the eight patients, one patient had intraoperative death due to massive pulmonary thromboembolism and another died on the 7th post-operative day due to disseminated intravascular coagulation and multiorgan failure. Rest six patients were treated with post-operative chemotherapy or a combination of chemotherapy and radiotherapy. For these six patients, overall median survival was 45 months with a 3 year disease-free survival of 66% and 5 year survival of 44%.CONCLUSIONS:PNET of the kidneys are rare peripheral neuroectodermal tumors with an aggressive clinical course. These tumors carry a very poor prognosis. An aggressive treatment approach using a combination of surgery, chemotherapy and radiotherapy is recommended for a reasonable survival in these tumors.

Background A Ewing sarcoma family tumor (ESFT) of vertebral column is a rare malignancy. Data on outcome and prognostic factors about this entity is scarce in literature. Methods This is single institutional data review of 68 consecutively treated patients of ESFT of vertebral column between June 2003 and November 2015 with uniform chemotherapy protocol. All patients received neoadjuvant chemotherapy, surgery and/ or radiotherapy as local treatment followed by adjuvant chemotherapy, except a few who received induction chemotherapy after upfront decompression surgery. abstracts Results Median age was 16 years (range: 2-53) with male: female ratio 50:18. Sites of disease were cervical spine in 7 (10%), dorsal spine in 14 (21%), lumbar spine in 21 (38%) and sacro-coccigeal in 26 (38%) patients; 29 patients (43%) had baseline metastasis. Median symptom duration was 3.5 months (range: 0.1-24). 48 (70%) patients presented with weakness of limbs. Fourteen (21%) patients received anti- tubercular pharmacotherapy before diagnosis of ESFT. Twenty-six (38%) patients had upfront decompression surgery due to spinal cord compression and 18 patients received adjuvant radiotherapy after induction chemotherapy. Of the remaining 42 patients, 26 were eligible for local therapy: 25 received definitive radiotherapy and one patient underwent surgery with adjuvant radiotherapy. After median follow-up of 29.5 months (range: 3.2-81.1), 5-year event-free-survival (EFS) and overall survival (OS) was 23.266.9% and 25.368.5%, respectively. Multivariate analysis showed that baseline hemoglobin (:::11 g/dl) and lactate dehydrogenase (2: 2 x normal) independently predicted inferior outcome for EFS (p < 0.001 & 0.003, respectively) and baseline serum albumin of ::: 3.5 g/dl for OS (p = 0.005) in whole cohort. Conclusions This is the largest data set of vertebral ESFT from Asia with high rate of metastases at presentation. Majority of patients had delayed presentation and were overtly morbid. One-fifth of patients were mistreated as Koch's before the actual diagnosis of ESFT. Outcome was dismal with multimodality management with limitation of quality of life data. Serum albumin and hemoglobin emerged as novel prognostic factor affecting outcome. Legal entity responsible for the study Akash Kumar Funding All India Institute Of Medical Sciences, New Delhi Disclosure All authors have declared no conflicts of interest.

Background Primitive neuroectodermal tumor (PNET) of kidneys is a rare entity. Treatment strategies are extrapolation of skeletal PNET. We evaluated clinical characteristics and outcome in 20 patients of renal PNET registered at our centre from January 2005 to December 2015. Methods This is a single institutional data review of renal PNET patients. All patients were treated with uniform chemotherapy protocols. Baseline clinical characteristics, histological features, treatment details ad survival outcomes were collected from case files. Overall survival (OS) was calculated from time of diagnosis to death due to any cause. Results Median age was 24.5 years (range: 14-67 years). Sixty five percent were males. Most common symptoms were pain abdomen (N = 15, 75%), hematuria (N = 11,55%) and systemic symptoms (N = 6,30%). Anemia (haemoglobin<12g/dl), leukocytosis (total leucocyte count>11000cells/ll), hypoalbuminemia (serum albumin<3.5g/dl) and lactate dehydrogenase above upper limit of normal occurred in 9(45%), 2(10%), 6(30%), 10(50%) patients, respectively. Tumor thrombus occurred in 8(40%) cases. Ten patients (50%) had metastases. Lungs (N = 10, 50%) and bones (N = 4, 20%) were most common metastatic sites. A preoperative diagnosis was made in 8(40%) patients. Of these, 7 received neoadjuvant chemotherapy (NACT) comprising vincristine, adriamycn, ifosfamide, etoposide and cyclophosphamide. Overall, 19 (95%) patients received chemotherapy. Fifty percent patients with metastases and 90% patients with localized disease underwent local treatment. Surgery alone, combined modality and radiotherapy alone were performed in 11(55%), 2 (10%) and 1 (5%) cases, respectively. Median follow-up duration was 32 months (range: 0.13-101 months). Five year OS was 40%. Due to small sample, multivariate analysis was not done to find prognostic factors. Conclusions Renal PNET was difficult to identify preoperatively which converted into underutilisation of NACT. A preoperative diagnosis and combined modality treatment is required to improve survival in these patients. Legal entity responsible for the study AIIMS Funding AIIMS Disclosure All authors have declared no conflicts of interest.

Aims: Data on metastatic Ewing's sarcoma family of tumours (ESFT) with uniform chemotherapy protocol are minimal.Materials and methods: This was a single institutional patient review of patients treated between June 2003 and November 2011 and evaluated on an intent-to-treat analysis. All patients received uniform chemotherapy: neoadjuvant chemotherapy (NACT), surgery and/or radiotherapy as local treatment followed by adjuvant chemotherapy. Local treatment was offered if the patient achieved a complete response and/or a partial response at both the primary and the metastatic site.Results: In total, 150/374 (40%) ESFT patients were metastatic, with a median age of 15 years (range: 2-50); a tumour diameter of 10 cm (range: 1.8-26). Most common metastatic sites were lung only (53; 35%), bone only (35; 23%) and combined bone/lung (25; 17%). Twenty patients underwent surgery; 55 patients received radical radiotherapy after NACT. After a median follow-up of 26.1 months (range: 1.6-101.6), 5 year event-free survival (EFS), overall survival and local control rate (LCR) were 9.1 +/- 3.3%, 16.9 +/- 5.2% and 31.8 +/- 7.9%, respectively. Univariate analysis showed serum albumin <= 3.4 g/dl (P < 0.001) to predict inferior EFS. Tumour size >8 cm (P = 0.05), haemoglobin <= 10 g/dl (P = 0.04), hypoalbuminaemia (P = 0.003) and radical radiotherapy as local treatment (P = 0.03) predicted inferior overall survival. No factor significantly predicted LCR, although age < 15 years (P = 0.08) and radical radiotherapy as local treatment (P = 0.09) had a trend towards inferior LCR. Hypoalbuminaemia was the only prognostic factor to predict EFS on multivariate analysis.Conclusion: This was the largest study of metastatic ESFT from Asia and identified a unique prognostic factor. In view of dismal prognosis with conventional chemotherapy in metastatic ESFT with hypoalbuminaemia, palliative intent therapy may be a potential therapeutic alternative for this subgroup of patients, especially in resource-challenged situations. (C) 2014 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.