Pancreatic neuroendocrine tumor in an individual with Von Hippel Lindau syndrome. A case report and review literature

Von Hippel Lindau (VHL) disease is a heritable cancer syndrome characterized by VHL gene mutation in 3p chromosome. Mutations occur at the exons. Rarely mutations at introns have been reported. It leads to accumulation of Hypoxic ischemic factor α (HIF α) which in turn leads to uncontrolled cellular proliferation. Few reports of pancreatic neuroendocrine tumors (PNETs) are there in the literature. Most of them are associated with a mutation in the exon of VHL gene. Association with intron mutation is not there. Here we are reporting a case of PNET in a patient who has a simultaneous mutation both at the exon and the intron

Acute Philadelphia Chromosome Positive Biphenotypic Leukemia Presenting with Bilateral Orbital Chloroma: A Rare Case Report

Introduction: Chloromas are characteristically formed by the extramedullary soft tissue infiltration by the immature myeloid malignant cells. Such extramedullary masses are most commonly seen in acute myeloid leukemia usually in the M2, M4, M5 subtypes of the AML FAB classification. However, it has been reported to rarely present only in pediatric patients with acute lymphoblastic leukemia.Presentation of the case: We encountered an unusual case of a young male, who presented with proptosis of both eyes followed by fever and fatigue. On evaluation, he was diagnosed to have bilateral orbital chloroma which was due to infiltration by leukemic cells of acute leukemia. Flowcytometry revealed features confirming an acute biphenotypic leukemia. Subsequently, cytogenetic evaluation revealed the leukemic cells to be Philadelphia chromosome positive.Conclusion: To our knowledge, this is the first case of bilateral orbital choloroma due to Philadelphia positive biphenotypic acute lymphoblastic leukemia

Pancreatic neuroendocrine tumor in an individual with Von Hippel Lindau syndrome. A case report and review literature

Von Hippel Lindau (VHL) disease is a heritable cancer syndrome characterized by VHL gene mutation in 3p chromosome. Mutations occur at the exons. Rarely mutations at introns have been reported. It leads to accumulation of Hypoxic ischemic factor α (HIF α) which in turn leads to uncontrolled cellular proliferation. Few reports of pancreatic neuroendocrine tumors (PNETs) are there in the literature. Most of them are associated with a mutation in the exon of VHL gene. Association with intron mutation is not there. Here we are reporting a case of PNET in a patient who has a simultaneous mutation both at the exon and the intron

Decitabine Compared with Low-Dose Cytarabine for the Treatment of Older Patients with Newly Diagnosed Acute Myeloid Leukemia: A Pilot Study of Safety, Efficacy, and Cost-Effectiveness

Introduction. The incidence of Acute Myeloid Leukemia (AML) increases progressively with age and its treatment is challenging. This prospective case control study was undertaken to compare the safety, efficacy, and cost-effectiveness of decitabine with those of cytarabine in older patients with newly diagnosed AML who are not fit for intensive chemotherapy. Materials and Methods. 30 eligible patients above 60 years old with newly diagnosed AML were assigned to receive decitabine or cytarabine. The primary end point was overall survival (OS). The secondary objective was to compare adverse events and cost-effectiveness of therapy in the two study groups. Results. In this study, 15 patients received decitabine and 15 patients received cytarabine. The median OS was 5.5 months for each of the treatment groups. The hazard ratio between the treatment groups was 0.811 with 95% CI of 0.390 to 1.687. Toxicity profile was similar in both groups. Cost per cycle of chemotherapy in INR was 24,200 for decitabine and 1,600 for low-dose cytarabine group. Median of simplified cost-effectiveness ratio was 0.00022 for decitabine group and 0.0034 for low-dose cytarabine group. Conclusions. For elderly patients with AML, decitabine and low-dose cytarabine should be chosen based on the patient’s choice and affordability. Our study has shown that both of these agents have similar OS and toxicity. Low-dose cytarabine scores over decitabine in developing countries as it is more cost-effective

Pharmacoeconomic benefit of cisplatin and etoposide chemoregimen for metastatic non small cell lung cancer: An Indian study

Background: The incidence of lung cancer is rising in developing countries like India. Due to unaffordability among the low socioeconomic status (SES) patients, there is a significant delay in seeking appropriate medical treatment due to which a high proportion of patients present in an advanced/metastatic stage and the outcomes are poor. Objective: In this study, we studied the progression-free survival (PFS) and the pharmacoeconomic benefits with the cisplatin plus etoposide (EtoP) chemo regimen and compared it with the current generation chemo regimen. Materials and Methods: We performed a retrospective analysis of metastatic nonsmall cell lung cancer patients who received one or more cycles of platinum-based chemotherapy between 2011 and 2014. Results: Of the 304 patients, 56.6% of the patients were of the low SES. Of the low socioeconomic group patients, 67.45% and 31.4% received etoposide and paclitaxel platinum doublet combination regimen as first line, respectively. The mean PFS with the etoposide, paclitaxel, pemetrexed, and gemcitabine platinum-based doublet regimens were 9.35, 10, 10.76, and 9.83 months, respectively. Kaplan–Meier survival curve analysis showed a statistically significant initial survival with the first line EtoP cisplatin regimen for the initial 6 months of starting chemotherapy in comparison with the other regimens. Conclusions: This study showed a substantial pharmacoeconomic benefit with the cisplatin and etoposide chemo regimen in the lower socioeconomic group of patients. We believe that this is the first pharmacoeconomic study on metastatic non small cell lung treatment of great relevance to countries with limited resources

Prognostic significance of bone only metastasis compared to visceral metastasis in patients with carcinoma cervix treated with platinum-based chemotherapy

Context: Carcinoma cervix is a leading cause of cancer in Indian females where 15%–60% of the cases eventually metastasize. Bone only metastasis is rare, and data on its response and survival with systemic therapy as compared to other visceral metastasis are limited. Settings and Design: The study design was a retrospective analysis. Materials and Methods: We retrospectively analyzed our data between May 2013 and April 2015 to identify the cases of bone only metastasis and visceral metastasis and tried to analyze their outcomes with paclitaxel- and carboplatin-based chemotherapy and bisphosphonates (for bone metastasis only). Results: Totally, 12 cases with bone only metastasis (Group 1) and 43 cases with visceral metastasis (Group 2) were identified. Most common sites of bone metastasis were vertebrae (66.67%) and pelvis (25%) while that of visceral metastasis was liver (44.18%) and lung (34.88%). Only 33.33% and 34.88% of cases in Group 1 and Group 2, respectively, could complete all six cycles of chemotherapy. Overall, response rates were 41.67% and 30.32% in Group 1 and Group 2, respectively. Median progression-free survival and overall survival (OS) were 10 months and 14 months, respectively, in Group 1 as compared to 4 months and 9 months, respectively, in Group 2. The difference in survival was statistically significant. Statistical Analysis Used: It was carried out by SPSS software version 20. Conclusion: Bone only metastasis is a rare and distinct entity with favorable outcomes as compared to visceral metastasis. However, disease remains aggressive and poor OS emphasizing the need of further research

Primary bone lymphoma: A report of two cases and review of the literature

Primary bone lymphoma (PBL) is an uncommon tumor accounting for approximately 4-5% of extra nodal lymphoma and less than 1% of all non-Hodgkin\u2032s lymphoma. Disease may be complicated at presentation by pathological fracture or spinal cord compression. Diffuse large-B-cell lymphoma (DLBCL) accounts for the majority of cases of PBL. Owing to its rarity, only a few retrospective studies have been published addressing the prognosis and treatment of primary bone lymphoma. In this paper, we report our experience with two cases of PBL treated with chemotherapy and radiotherapy and review literature to elucidate the optimal treatment of primary bone lymphoma

Clinicopathological profile and utility of prognostic tools in peripheral T-cell lymphoma

Introduction: With the availability of three prognostic scores in Peripheral T-Cell Lymphoma (PTCL)-International Prognostic Index (IPI), International Peripheral T-Cell Lymphoma Project (IPTCLP), and Prognostic Index for PTCL-Unspecified (PIT), we intended to find out usefulness of each of them in prognostication of patients. Objectives were to know the clinicopathological profile and to analyze the effectiveness of these prognostic scores and their individual parameters in predicting Complete Remission (CR) rates, Progression Free Survival (PFS), and Overall Survival (OS) in PTCL. Materials and Methods: All 55 patients were treated as per institute chemotherapeutic protocols. Response assessment was done by Cheson’s criteria. At baseline, patients were risk stratified with the three scores into low, low- and high-intermediate, and high-risk groups. Outcomes − CR and survival − PFS and OS were analyzed and compared in the risk groups as stratified by the three prognostic scores. Clinical and pathological details were also analyzed. Results: For PFS estimation, IPI and IPTCLP scores proved be the best prognostic tools. For OS, IPTCLP score emerged to be the single best prognostic tool. CR correlated with Ann Arbor stage of disease and IPI score. Conclusion: PTCL NOS (Not Otherwise Specified) was the most common histological subtype. IPI score correlated the best with CR reflecting as PFS, but was not related statistically with OS in the local population. IPTCLP score has consistent correlation with prognosis more so with OS than PFS, but no relationship with CR rate

Plasmablastic lymphoma in immunocompetent and in immunocompromised patients: Experience at a regional cancer centre in India

Introduction: Plasmablastic lymphoma (PBL) is a rare lymphoma associated with immunosuppression. It is strongly associated with immunosuppression (human immunodeficiency virus [HIV]) and often occurs within the oral cavity. PBL is also seen in patients receiving immunosuppressive therapy; however, despite its predisposition for the immunocompromised patients, PBL has been diagnosed in immunocompetent patients. Aim: This study aims to prognostic factors and outcome of PBL in immunocompromised and in immunocompetent patients. Materials and Methods: We conducted a retrospective study at our institute from the year 2008 to 2015. Results: A total of 13 patients (8 males and 5 females) with PBL were identified. Eight patients (61.5%) had extraoral PBL (median age 30.2 years) and 5 patients (38.5%) had oral PBL (median age 44 years). Most common extraoral site was gastrointestinal tract. Eight (61.5%) out of 13 patients were HIV positive. More than 50% of patients had Ann Arbor Stage III or IV. All the cases were CD20 negative and CD138 positive. Seven out of 13 patients had Ki-67 more than 80%. Nine patients received cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy. Three patients were on best supportive care due to poor performance status (PS). One patient received intensive chemotherapy with CODOX-M/IVAC. The median overall survival was 9 months in HIV-positive patients and 6 months in HIV-negative patients. The prognosis was worse in patients with Ki-67 of> 80%. Statistical Analysis: Survival curves were generated using the Kaplan–Meier method and analyzed using log-rank test and Fisher's t-test. Conclusion: The present study confirms that PBL in both HIV-positive and in HIV-negative patients has an overall unfavorable outcome. The most important prognostic factors are stage, ki-67, and the Eastern Cooperative Oncology Group PS of the patient at the time of presentation

Primary bone lymphoma: A report of two cases and review of the literature

Primary bone lymphoma (PBL) is an uncommon tumor accounting for approximately 4-5% of extra nodal lymphoma and less than 1% of all non-Hodgkin′s lymphoma. Disease may be complicated at presentation by pathological fracture or spinal cord compression. Diffuse large-B-cell lymphoma (DLBCL) accounts for the majority of cases of PBL. Owing to its rarity, only a few retrospective studies have been published addressing the prognosis and treatment of primary bone lymphoma. In this paper, we report our experience with two cases of PBL treated with chemotherapy and radiotherapy and review literature to elucidate the optimal treatment of primary bone lymphoma