Background:Langerhans cell histiocytosis (LCH) is a disease that primarily affects bone but can be associated with a clinical spectrum that ranges from a solitary bone lesion with a favorable natural history to a multisystem, life-threatening disease process.Aim:We analyzed our single institutional experience of managing children with LCH.Settings and Design:A total of 40 children of LCH, managed in tertiary cancer center in South India in the period from 2001 to 2005, were evaluated retrospectively.Materials and Methods:Clinicopathological features, laboratory findings, treatment modalities and long-term outcome were analyzed.Results:Children were aged between 2 months and 12 years, with a mean of 3 years. Majority of the children were below 5 years of age. Group B constituted a bulk of children. Disseminated cases were less (five patients). Liver function dysfunction was seen in four (10%) children. Pulmonary interstitial infiltrates were seen in two (5%) cases. Diabetes insipidus manifested in three patients. There was one death.Conclusion:A better understanding of the etiology and pathogenesis of LCH will result in more directed and efficacious treatment regimens.

Background:Neuroendocrine carcinoma (NEC) is a rare tumor arising from the diffuse neuroendocrine system. Most of these present in the advanced stage and palliative chemotherapy remains the only option. The prognosis remains poor with the standard chemotherapy regimen of platinum and etoposide (EP) providing modest survival benefit.Methods:The study was done for 3 years at a tertiary cancer center in South India. Patients with a diagnosis of metastatic NEC were analyzed for clinical and pathological characteristics. The treatment outcomes and prognostic factors were evaluated using appropriate statistical test.Results:A total of 114 patients of metastatic NEC satisfied the inclusion criteria and were analyzed. Gastrointestinal including hepatobiliary tract (33%) was the most common site of primary disease followed by lung (26%), genitourinary (15%), head and neck (14%), and unknown primary (9%). On analysis of pattern of metastasis, liver (65%) was the most common site followed by bone (54%) and lung (42%). The median overall survival was 11 months with a statistically significant difference between pulmonary and extrapulmonary disease (8 vs. 13 months; P = 0.003). Ki67% value was strongly associated with prognosis (hazard ratio 0.517, 95% confidence interval; 0.318–0.840, P = 0.008) whereas age, sex, and lactate dehydrogenase level did not show any relation with survival.Conclusion:The outcome of advanced NEC with standard chemotherapy remains poor. Larger studies with other therapeutic and novel agents are warranted to improve the treatment outcomes.

Background: Invasive mucinous carcinoma (IMA) is a distinct histopathologic variant of adenocarcinomas of lung comprising about 2 -10%. A large proportion of IMAs carry KRAS mutations and only rarely EGFR mutations or ALK/ROS translocations, thus most cases are not amenable for targeted therapy at present. This study was conducted to elicit the unique clinico-pathological characteristics of IMA. Methods: Medical records of patients diagnosed with IMA by needle biopsy at Kidwai Cancer Institute, Bangalore from 2013 to 2018 were retrieved, clinical presentations and treatment outcomes were reviewed. Statistical analysis was performed using SPSS version 23.0. Results: 490 cases of needle biopsy of lung were done at our institute between 2013 and 2018. Nine cases (1.8%) were diagnosed as IMA. The median age was 59 years (range 49 -76 years). The male female ratio was 2:1. Six (66.7%) were current smokers with pack year greater than 20. The median symptom duration before diagnosis was 3 months. Three (33.3%) of the cases were initially misdiagnosed as pneumonia in view of CT findings of pneumonia-like multifocal consolidative appearance. Lung was the most common site of metastasis (77.8%). Serum CEA was elevated in six cases (66.7%). None of the cases had any mutations in EGFR gene or ALK and ROS1 translocations. All cases were treated with Pemetrexed-Carboplatin followed by Pemetrexed maintenance till progression. 5 (55.6%) patients had stable disease, 3 (33.3%) had partial response and 1 (11.1%) had progressive disease after three months of therapy. Median PFS was 12 months (Range 3 -18 months). Docetaxel was given as second line chemotherapy in all progressed patients. Best response noted was stable disease; seen in 4 (57.1%) cases. Median PFS with docetaxel was 6 months (range 3-8 months). Median overall survival 16 months (range 9 -27 months). Patients with progressive disease had serial rise in Serum CEA. Conclusions: IMA is rarely diagnosed on needle biopsies due to insufficient tissue for characterisation. They mimic pneumonia on imaging thus delaying diagnosis. EGFR mutations, ALK and ROS1 translocations are usually negative. Response to chemotherapy is modest. Serum CEA may be used as a tumor marker for response assessment.

The rate of the tumor regression can predict the overall outcome in patients with buccal cancers treated with radiation. Completion of the planned course of radiation in patients who do not show a substantial reduction in size by 4.5 weeks of conventional radiation does not improve the results.