Neuroendocrine neoplasms are defined as epithelial neoplasms with predominant neuroendocrine differentiation. They can arise in almost every organ of the body although they are most commonly found in the gastrointestinal tract and respiratory system. Nasal cavity and paranasal sinuses are a rare site for neuroendocrine carcinoma. In contrast to the other regions, neuroendocrine tumours of the sinuses have been reported to be recurrent and locally destructive. Very few cases of paranasal sinus neuroendocrine carcinoma have been reported till date. Difficulty in pathologic diagnosis and rarity of this malignancy have hindered the progress in understanding the clinical course and improving outcomes. We herein report a case of poorly differentiated neuroendocrine tumour of ethmoid and sphenoid sinus with invasion of orbit and intracranial extension. The patient had complete response at the end of chemoradiation and he was disease-free for 9 months duration after which he developed bone metastasis without regional recurrence.

Brain tumors are the second most common cancers after hematological malignancies accounting for approximate 21% of all childhood malignancies in children between ages of 0 and 14 y. The present study was undertaken to determine the spectrum of the brain tumors diagnosed in a tertiary cancer center in South India. A retrospective analysis of the data of pediatric brain tumors diagnosed between 2003 and 2009 was done and data was classified according to the age, gender and histology types. Out of 2,844 pediatric patients, 341 (11.99%) were diagnosed as having brain tumors. Most of the patients were in the age group of >5-14 y. Male to female ratio was 1.58:1. The most common pediatric brain tumor was medulloblastoma followed by astrocytoma and ependymoma. Glioblastoma multiforme was the most common subtype of astrocytoma. Other common tumors were glioma, oligodendroglioma, periphereral neuroectodermal tumor and germ cell tumor. As compared to western data, incidence of brain tumors in children was found to be less in the present study.

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Involvement of oral cavity by lymphoma is rare. Plasmablastic lymphoma was the most common oral cavity NHL in our patients. Although number of cases in present study was less, our data suggests that oral NHL has aggressive course with less favorable outcome. Further large sample studies incorporating rituximab-based chemotherapy and more aggressive chemotherapy for plasmablastic lymphoma will be helpful for better understanding of treatment outcome.

The distribution of malignancy in infants is quite different from that which is found in older children. Although neuroblastoma is the most common infantile tumor in western countries, in our study leukemia is the most common infantile malignancy. Embryonal tumors such as neuroblastoma, Wilms tumor, retinoblastoma, and hepatoblastoma were more prevalent in infants. Solid tumors were the most common malignancy in infants which is followed by leukemia.

Objective: Eosinophilia associated with FIP1L1-PDGFRA rearrangement represents a subset of chronic eosinophilic leukemia and affected patients are sensitive to imatinib treatment. This study was undertaken to learn the prevalence and associated clinicopathologic and genetic features of FIP1L1-PDGFRA rearrangement in a cohort of 26 adult patients presenting with profound eosinophilia (>1.5x109/L).Materials and Methods: Reverse-transcriptase polymerase chain reaction and gel electrophoresis were used for the detection of FIP1L1-PDGFRA rearrangement. Results: Five male patients with splenomegaly carried the FIP1L1-PDGFRA gene rearrangement. All patients achieved complete hematological response within 4 weeks of starting imatinib. One patient had previous deep vein thrombosis and 1 patient had cardiomyopathy, which improved with steroids and imatinib. Conventional cytogenetics was normal in all these patients. No primary resistance to imatinib was noted.Conclusion: This study indicates the need to do the FIP1L1-PDGFRA assay in patients with hypereosinophilic syndrome. Prompt treatment of this condition with imatinib can lead to complete hematological response and resolution of the organ damage that can be seen in this setting.

Background:Triple negative breast cancers (TNBCs) are a diverse and heterogeneous group of tumors that by definition lack estrogen and progesterone receptors and amplification of the HER-2 gene. The majority of the tumors classified as TNBCs are highly malignant, patients are usually young and only a subgroup of patients responds to conventional chemotherapy with a favorable prognosis. Various studies have been reported in western literature on TNBCs, all highlighting the poor prognosis of this subtype. However, extensive data from India is lacking.Aim:The aim of this study was to analyze the epidemiological and clinical profile of TNBCs at our institute.Materials and Methods:This was the retrospective study carried out in Tertiary Cancer Care Center in South India. Case files of all breast cancer patients were reviewed from the hospital database registered in 1 year and TNBC patients were selected for the study. Patient's characteristic, treatment, and histological features were analyzed.Results:A total of 322 patients were registered during the period of 1 year and 26% (84/322) of total patients were TNBC. Median age of presentation was 44.5 years. About 94% (79/84) of patients had first full-term delivery before the age of 30 years. The most common presenting symptom was left sided breast lump. Locally advanced and early breast cancer (EBC) was 51% (43/84) and 42% (36/84), respectively. Metastatic breast cancer was seen in five patients. The highest numbers of patients were node negative disease (36.9%) [31/84], followed by N1 30.95% (26/84). Most of the patients had high-grade tumor. 94% (34/36) of cases of EBC had undergone upfront modified radical mastectomy. Invasive ductal carcinoma was the predominant histology except one who had medullary carcinoma. Twenty-four patients received neoadjuvant chemotherapy (NACT). There was no pathological complete remission, but all patients responded to NACT. Metastatic disease was seen in five patients. All patients had bone metastasis.Conclusions:TNBCs are highly aggressive subtype, with high grade with limited treatment options and very poor prognosis. Incidence is more in our country than the western literature. Even in our country also the incidence is varies in different region. TNBCs are significantly associated with young aged patients. There was a lack of association between tumor size and lymph node positivity.

Background Bilateral breast cancer (BBC) is an unusual clinical entity. Unlike unilateral breast cancer, there is fewer data regarding clinicopathological aspects and treatment guidelines in BBC. The present study was carried out at a tertiary oncology center in South India to analyze the clinicopathological profile of patients diagnosed with BBC.Methods This was a retrospective observational study of patients diagnosed with BBC in the department of medical oncology from August 2012 to July 2013.Results Out of a total of 300 breast cancer patients during a 1-year period, 15 had BBC. Synchronous and metachronous breast cancers were seen in six and nine patients, respectively. The median age at presentation for BBC was 40 years. Family history of breast cancer was present only in two cases. The median age of menarche and first child birth was 13 and 22 years, respectively. History of breast feeding was positive in all except one who was nulliparous. Out of the 15 patients, 11 were premenopausal and 4 postmenopausal. Contralateral breast cancer was detected mammographically in 4 patients and by clinical examination in 11. Out of 30 tumors, all were invasive ductal carcinomas (IDC). Nine patients had bilateral mastectomy, and five had unilateral mastectomy. Out of 30 tumors, 14 were triple negative, 9 estrogen receptor (ER)/progesterone (PR) positive, and 7 Her2neu positive. Neoadjuvant chemotherapy (NACT) was given in four synchronous and three metachronous breast cancer cases for contralateral breast cancer. All patients received chemotherapy during the course in the form of either adjuvant or palliative chemotherapy.Conclusions BBC is an uncommon clinical entity. For women with younger premenopausal age, the incidence of BBC is higher compared with older women. As the prognosis of BBC is poor, it is crucial to be aware of this entity, and every patient with breast cancer should be regularly followed up. These patients require individualized treatment planning based on the tumor factors and treatment factors of the primary lesion.