Local therapy in non-metastatic primary Ewing sarcoma of the mandible and maxilla in children.

Ewing sarcoma (ES) of the jaw bones comprises a small fraction of ES at all sites. Due to their rarity, a specific policy for local treatment is lacking. The aim of this study was to evaluate the local therapy for ES and recommend measures to individualize treatment options. Patients with primary non-metastatic ES of the jaw bones treated between August 2005 and February 2015 were analyzed. All patients received primary induction chemotherapy, following which lesions amenable to resection based on specific radiological criteria were resected; those with unresectable lesions were offered definitive radiotherapy. The maxilla was the primary site in 13 patients and the mandible in eight. The median age of patients was 11.6 years (range 5-17 years). Overall, surgery was performed in 17 patients and definitive radiotherapy was used in four patients. Postoperative radiotherapy was administered to 12 patients and was avoided in five patients with 100% tumour necrosis. The 3-year overall survival, event-free survival, and local control were 68.1%, 63.6%, and 80.2%, respectively. Mandible primary and a histological response to chemotherapy were significant prognostic factors. The stratification of patients based on radiological criteria aids in selecting local therapy. In eligible patients, surgery with contemporary reconstruction results in optimal oncological and functional outcomes. Surgery also has the added advantage of identifying patients who may not need radiotherapy.

Sunitinib in patients with imatinib-resistant gastrointestinal stromal tumor: A single center experience study.

AIM: The outcome of patients with advanced gastrointestinal stromal tumor (GIST) has improved with the use of imatinib. Despite high response rates with this drug resistance eventually develops in nearly all patients. We present an analysis of prospectively collected data on sunitinib efficacy and safety in patients with imatinib-resistant GIST. SUBJECTS AND METHODS: Between November 2006 and October 2007, patients with GIST were accrued in an approved sunitinib patient access protocol. Key eligibility criteria included tumor resistance to imatinib and/or patient intolerance to this drug. Patients received sunitinib at a starting dose of 50 mg once daily for 4 weeks in a 6 week cycle, with standardized dose modification titrated to toxicity. Patients were continued on sunitinib until disease progression or unacceptable toxicity. The endpoints were safety, overall survival (OS) and objective response rate (ORR). RESULTS: Fifteen patients, all of whom had imatinib resistance and none intolerance, with median age of 48 (26-69) years, were treated on the protocol. The most common sites of primary disease were small intestine (40%), stomach (26.7%) and retroperitoneal (26.7%). A median of 10 (1-47) cycles of sunitinib were delivered, 9 (60%) patients required dose reductions due to toxicity whereas dose delay of > 2 weeks was required in only one (6.7%) patient. There were no toxicity-related drug discontinuations. Hypothyroidism (n = 4; 26.7%) and hand-foot syndrome (n = 3; 20%) were the most common toxicities. There were no complete and 4 (26.7%) partial responses while prolonged disease stability was seen in 8 (53.3%) patients. At a median follow-up of 81 months in surviving patients, the median progression-free and overall survivals were 15.5 and 18.7 months, respectively. CONCLUSIONS: Sunitinib appears to be an effective and well-tolerated treatment for Indian patients with imatinib-resistant GIST with outcomes similar to that reported previously. Adverse effects can be reasonably well managed using a dose modification strategy.

Prospective observational study on diagnostic accuracy of whole-body MRI in solid small round cell tumours.

AIM: To assess the diagnostic accuracy of whole-body magnetic resonance imaging (WB-MRI) for metastatic disease in patients with solid small round cell tumours (SRCT) by comparing it with routine staging procedures (standard of care). MATERIALS AND METHODS: Eligible cases of neuroblastoma, primitive neuroectodermal tumour, and rhabdomyosarcoma were enrolled in the study after obtaining informed consent. WB-MRI was undertaken using overlapping coronal T1 and short-tau inversion recovery (STIR) sequences. Lesions were classified into skeletal, pulmonary, and soft-tissue types. Conventional staging, which consisted of combined positron-emission tomography & computed tomography (PET-CT), bone scintigraphy & bone marrow biopsy for bone metastases, CT thorax for lung metastases, combined PET-CT, metaiodobenzylguanidine (MIBG) scintigraphy (in neuroblastoma) for soft tissue metastases and clinical evaluation was used as the reference standard. Parameters for diagnostic accuracy were calculated. RESULTS: Thirty-four out of forty patients enrolled were included in final analysis, half of them having metastatic disease. The sensitivity, specificity, positive and negative predictive value, and the diagnostic accuracy of WB-MRI and PET-CT for skeletal metastases as compared to reference standard were 91.9%, 99.8%, 97.4%, 99.6%, and 95.5% and 99.1%, 99.9%, 99.1%, 99.9%, and 99.9%, respectively. The sensitivity of MRI, only PET and PET-CT with plain CT thorax was 30%, 40%, and 100%, respectively, for lung metastases. The sensitivity of MRI for soft-tissue lesions was 76.9%. CONCLUSION: WB-MRI is a radiation-free tool with high diagnostic accuracy for the evaluation of metastatic disease to the marrow. The rate of detection of soft-tissue metastases, such as nodal metastases, is less when WB-MRI is compared with conventional staging using coronal STIR images. CT thorax is essential for accurate evaluation of lung metastases.

Escalated radiation dose alone vs. concurrent chemoradiation for locally advanced and unresectable rectal cancers: results from phase II randomized study.

PURPOSE: This trial was undertaken to compare the rates of resectability between patients treated with neoadjuvant concurrent chemoradiation vs. boosted radiotherapy alone. MATERIALS AND METHODS: Patients with clinically unresectable rectal cancer were randomized to receive external beam radiation therapy (EBRT) to pelvis (45 Gy) with concurrent oral Capecitabine (CRT group; Arm 1) or EBRT to pelvis (45 Gy) alone followed by 20 Gy dose of localized radiotherapy boost to the primary tumor site (RT with boost group, Arm 2). All patients were assessed for resectability after 6 weeks by clinical examination and by CT scan and those deemed resectable underwent surgery. RESULTS: A total of 90 patients were randomized, 46 to Arm 1 and 44 to Arm 2. Eighty seven patients (44 in Arm 1 and 41 in Arm 2) completed the prescribed treatment protocol. Overall resectability rate was low in both the groups; R0 resection was achieved in 20 (43 %) patients in Arm 1 vs. 15 (34 %) in Arm 2. Adverse factors that significantly affected the resectability rate in both the groups were extension of tumor to pelvic bones and signet ring cell pathology. Complete pathological response was seen in 7 and 11 %, respectively. There was greater morbidity such as wound infection and delayed wound healing in Arm 2 (16 vs. 40 %; p = 0.03). CONCLUSION: Escalated radiation dose without chemotherapy does not achieve higher complete (R0) tumor resectability in locally advanced inoperable rectal cancers, compared to concurrent chemoradiation.

Understanding the principles in management of Wilms' tumour: can imaging assist in patient selection?

The management of Wilms' tumour has evolved through thorough systematic research, predominantly lead by two groups: the Wilms' Tumour Study Committee of the International Society of Paediatric Oncology (SIOP) and National Wilms' Tumor Study Group (NWTSG) of North America. These two groups differ in their approach: SIOP advocates initial chemotherapy of 4-6 weeks followed by surgery, whereas the NWTSG advocates upfront surgery, with certain exceptions. This review briefly discusses the principles, and pros and cons of each approach. Both the treatment approaches have equivalent outcomes (in the form of event-free survival and overall survival), when compared stage-wise. With this knowledge, modern imaging can be used for individualizing treatment in anticipation of minimizing complications. The review identifies critical imaging features and discusses the reliability of imaging based on current reports in the literature.

A cystic hepatic lesion: when to worry? / La lesión cística del hígado: ¿cuándo preocuparse?

A 16-year old female presented to hospital with abdominal pain. Features on computed tomography raised the possibility of biliary cystadenoma or cystadenocarcinoma. She underwent a liver resection, and histopathology confirmed a serous biliary cystadenoma. This case is presented to highlight the radiological features of this uncommon pre-malignant condition as well as to summarize a management algorithm for cystic liver lesions.

Una mujer de 16 años de edad acudió al hospital con un dolor abdominal. Las características observadas con tomografía computarizada apuntaban a un cistoadenoma biliar o un cistoadenocarcinoma como diagnósticos diferenciales. La paciente fue sometida a una resección del hígado, y la histopatología confirmó un cistoadenoma biliar seroso. Presentamos este caso para resaltar los rasgos radiológicos de esta condición premaligna rara, así como para resumir un algoritmo de tratamiento para las lesiones císticas de hígado.

A cystic hepatic lesion: when to worry?

A 16-year old female presented to hospital with abdominal pain. Features on computed tomography raised the possibility of biliary cystadenoma or cystadenocarcinoma. She underwent a liver resection, and histopathology confirmed a serous biliary cystadenoma. This case is presented to highlight the radiological features of this uncommon pre-malignant condition as well as to summarize a management algorithm for cystic liver lesions.

Simultaneous gallbladder and bile duct cancers: revisiting the pathological possibilities.

The pathogenesis of gallbladder cancer presenting synchronously with malignancy of the bile duct has not been clearly understood. The possible causes for the simultaneous presence of these tumors could be due to local spread, metastases, de novo multifocal origin, or as part of a field change in the extrahepatic biliary apparatus. In this article, we discuss the cases of four patients with simultaneous gallbladder and bile duct malignancies and analyze their individual pathologies to provide an explanation into the mechanisms that may play a role in such conditions.

Primitive neuroectodermal tumour of the prostate gland: ultrasound and MRI findings.

We report a rare case of primitive neuroectodermal tumour (PNET) of the prostate occurring in a 26-year-old man with a mass replacing the prostate gland in the absence of any lesion involving the bone and soft tissues. To our knowledge, there is no radiological literature that has described the imaging findings in a case of PNET of the prostate. Imaging findings in cases of PNET of the kidney are described in the literature. On ultrasound, the mass appeared multilobulated and multinodular with intratumoural heterogeneity. No brightly reflective echoes with posterior acoustic shadowing to suggest calcification were noted. MRI revealed a heterogeneous lobulated mass with irregular septae within. The mass showed low signal intensity on T(1) weighted images and appeared heterogeneous on T(2) weighted images. Haemorrhage and necrosis were also seen in the tumour.

Is there a role for estrogen and progesterone receptors in gall bladder cancer?

BACKGROUND/AIMS: The concept of metaplastic and non-metaplastic types of gall bladder cancer and the likelihood of hormone receptor expression in the nuclei of tumour cells raised the possibility of a potential role for anti-estrogen therapy in gall bladder cancer. This study was carried out to determine the hormone receptors (ER/PR) expression level in gall bladder cancer using specific immunohistochemical assays and correlate it with patient and tumour histopathological characteristics. PATIENTS AND METHODS: Histopathological tumour specimens of 62 patients who underwent a radical cholecystectomy were analysed. Pronase pretreatment and primary monoclonal antibodies were used to perform immunohistochemical analysis for ER and PR. RESULTS: The histology was adenocarcinoma--predominantly, moderately to poorly differentiated (91%). Gallstones were present in 90% of the individuals. Of the 62 specimens analysed, 62 (100%) and 61 (98%) were negative for ER and PR, respectively. CONCLUSION: The high incidence of gallstone-related gall bladder cancer in India is associated with metaplasia and a tendency to poorer differentiation in the tumour histology. These tumours are consequently less likely to express hormone receptors. Thus, there does not seem to be a role for anti-hormone therapy in patients with histogenesis similar to that seen in India.

Solid pseudopapillary neoplasm of the pancreas: a single institution experience of 14 cases.

Solid pseudopapillary neoplasm of pancreas (SPN) is a rare entity; therefore proper diagnosis, evaluation and formulation of treatment protocols is difficult. A total of 14 cases were diagnosed and treated at our institute over a 10-year period (1992-2002). A retrospective study of all these cases was performed with respect to age group most frequently affected, sex ratio, common presenting symptoms and signs, investigative protocols, pathological features, treatment offered, outcome and prognosis. All the 14 patients were females, with a median age of 20 years (range 13-45 years). The commonest presenting symptom was abdominal pain. All these patients underwent surgical exploration; 13 patients underwent resection of the tumour. The average length of postoperative hospital stay was 10.3 days. Significant postoperative morbidity was seen in only one case. There was no postoperative mortality. All these patients who underwent resection were disease-free on follow-up ranging from 6 to 96 months (median 36 months).