Kn Lokesh

Primary Diffuse large B-Cell lymphoma of testis: A single centre experience and review of literature

Background: Primary testicular lymphoma constitutes 1-2% of Non-Hodgkins lymphomas affecting elderly men >60 years of age. Most often it is a Diffuse large B cell lymphoma (DLBCL) and treatment involves multimodality approach involving surgery, chemotherapy and radiotherapy. Outcome remains poor in spite of aggressive therapy. Materials and Methods: We retrospectively reviewed 286 registered cases of DLBCL (aged >14 years) from 2007 to 2011 and found nine primary testicular involvement patients. These cases were analyzed for baseline clinical features, investigations, staging, treatment and outcome. Results: Median age was 58 (46-76) years. All patients presented with testicular swelling, two had the presence of B symptoms, and three with abdominal lymphadenopathy. Six had stage IE disease and three patients had stage IIE. All patients underwent orchiectomy. Eight patients received combination chemotherapy and six completed three or more cycles. Four achieved complete response, among these three relapsed after 32, 42, 70 months and one was lost to follow up. Two had a progressive disease, among these one died of disease and one alive with disease. Complete follow up was available from five patients and median survival was 36 months (11-78 months). Conclusion: Primary testicular DLBCL is uncommon, needs multimodality treatment and central nervous system prophylaxis to improve the survival. The outcome needs to be further investigated using biological approaches (Rituximab based) and/or more aggressive management.

Current status of systemic therapy for recurrent and/or metastatic squamous cell carcinoma of the head and neck

Head and neck squamous cell carcinoma (HNSCC) is now the seventh most common cancer worldwide. The median overall survival for patients with recurrent and/or metastatic (R/M) HNSCC remains <1 year despite modern systemic chemotherapy and targeted agents. Palliative systemic therapy for patients with R/M HNSCC typically includes a platinum-based doublet, with an understanding that the increase in efficacy compared with single agents is primarily related to improved response rate, and not survival. Till date, the only systemic therapy regimen to demonstrate survival superiority over platinum-5-fluorouracil (5-FU) doublet is platinum, FU, and cetuximab. Epidermal growth factor receptor inhibitors, including monoclonal antibodies and tyrosine kinase inhibitors, have achieved only a modest success in R/M HNSCC. Immunotherapy represents an attractive treatment option for R/M HNSCC, with encouraging preliminary data from studies involving immune checkpoint inhibitors (e.g., pembrolizumab, nivolumab) and toll-like receptor agonists (e.g., motolimod). Given the poor prognosis of R/M HNSCC, enrollment of patients into clinical trials to investigate novel systemic agents, is necessary for further improvement of oncologic outcomes in this patient population.

Acute Coronary Syndrome Manifesting as an Adverse Effect of All-trans-Retinoic Acid in Acute Promyelocytic Leukemia: A Case Report with Review of the Literature and a Spotlight on Management.

Background. Acute promyelocytic leukemia is characterized by t(15;17). This leads to the formation of PML/RARα which blocks the differentiation of blasts at the stage of promyelocytes. This is reversed by all-trans-retinoic acid (ATRA), a vitamin A derivative. Acute myocardial ischemia is a rare side effect of ATRA. Case Report. We report a case of acute coronary syndrome manifesting as an adverse effect of ATRA in a lady with APL who had no other risk factors for cardiovascular disease. Conclusions. We emphasize the need for high index of suspicion for the diagnosis of this entity. In the light of this case, the rare instances of ATRA associated acute myocardial ischemia recorded in the literature and the options available for treatment of acute promyelocytic leukemia sans ATRA have been reviewed.

Primary pulmonary rhabdomyosarcoma in children: Report of three cases with review of literature

Primary pulmonary rhabdomyosarcoma in children is rare. Three children aged three, nine and three years were evaluated for abnormal shadows on radiological examination with pneumothorax in two cases. Resection and histopathological examination revealed embryonal rhabdomyosarcoma in all and cystic malformation in first case. All the three children were treated with surgery and first two received adjuvant chemotherapy. The disease free duration was 160 months, 19 months and seven months respectively. The literature on primary pulmonary rhabdomyosarcoma in children was reviewed.

Correlation of BMI with breast cancer subtype and tumour size

Background Breast cancer is a heterogeneous disease which is divided broadly into luminal, HER2 and basal type based on molecular profiling. Increased body mass index (BMI) has been associated with the risk of developing breast cancer but the association based on molecular subtype remains conflicting. Methods This was an observational study carried out over a period of 2 years. Nonmetastatic breast cancer patients were evaluated for the tumour subtype based on surrogate markers (ER, PR and HER2). The BMI of these patients was correlated with the tumour subtype and size. Results We studied 476 patients with breast cancer with the median age of 46 years (range, 25–86) and 58% were premenopausal. The mean BMI of the cohort was 24.1, which was significantly higher in postmenopausal women (24.9 versus 23.6, p < 0.05). Overall, only 10% of patients were obese. The mean BMI in the luminal, HER2 and TNBC subtypes was 24.7, 22.4 and 23.9, respectively (p < 0.01). Also, the mean tumour size in luminal, HER2 and TNBC subtype was 4.02, 3.80 and 4.27 cm, respectively (p = 0.158). Conclusion The average BMI was higher in patients with luminal subtype followed by TNBC and lowest for HER2 at the time of diagnosis. The mean tumour size was numerically higher for TNBC and lowest for HER2 subtype although the difference was not statistically significant. Larger studies may provide clarity of association between the BMI and tumour subtype.

A rare case of primary synovial sarcoma of lung

Synovial sarcoma of lung is a very rare tumor accounting for 0.5% of all primary lung malignancy. It presents clinically with cough, chest pain, shortness of breath, or hemoptysis, with a mass lesion on X-ray and computerized tomography scan. Diagnosis is made by histopathology and immunohistochemistry. Here, we report a case of 48-year-old male, who presented with right-sided chest pain, cough with blood-tinged sputum, and found to have primary pulmonary synovial sarcoma of lung.

Study of clinical, haematological and cytogenetic profile of patients with acute erythroid leukaemia.

Background Acute erythroid leukaemia (AEL) is a rare subtype of acute myeloid leukaemia (AML), constituting <5% of all the cases of AML. The World Health Organization (WHO) in 2001 classified AEL into two types: (1) erythroid/myeloid leukaemia which required ≥50% erythroid precursors with ≥20% of the non-erythroid cells to be myeloid blasts and (2) pure erythroleukemia (pEL) with ≥80% erythroblasts. The WHO 2008 classification kept these subcategories, but made erythroleukemia a diagnosis of exclusion. There are very few studies on the clinico haematological and cytogenetic profile of this disease, considering the rarity of its occurrence and poor prognosis. Materials and methods This study was done by retrospective analysis of data from 32 case files of patients diagnosed with AEL. Clinical details noted down were the demographic profile, peripheral blood smear details and bone marrow examination details: (1) blasts-erythroblasts and myeloblasts, (2) dysplasia in the cell lineages and (3) cytogenetic abnormalities. Results The most common presenting symptom was fever. Pancytopenia at presentation was seen in 81.25% of patients. Dysplasia was observed in bone marrow in 100% of erythroblasts and in 40% of myeloblasts in erythroid/myeloid subtype. In pure myeloid subtype, myeloid and megakaryocytic dysplasias were not obvious. Complex karyotype was noticed only in patients of pEL. Conclusion AEL is a rare group of heterogeneous diseases with many neoplastic and non-neoplastic conditions mimicking the diagnosis. The clinical presentation and cytogenetics are also non-specific, presenting additional challenges to the diagnosis.

Primary breast angiosarcoma – a single institution experience from a tertiary cancer center in South India

INTRODUCTION Primary angiosarcoma of the breast is a rare entity with incidence of less than 0.05% of all malignant breast neoplasms. It occurs in young females without any associated risk factors. The tumor behaves aggressively and has a poor prognosis compared to invasive ductal carcinoma. METHOD It was a retrospective observational study done at a tertiary cancer center from January 2012 to December 2016. The medical records of patients diagnosed with primary breast angiosarcoma were reviewed for the study. Clinicopathological profile, treatment, and the outcomes were analyzed. RESULTS Four patients were diagnosed with primary breast angiosarcoma out of 2560 breast cancer patients seen over a period of 5 years. Two had metastatic disease at presentation. Among four patients, two underwent surgery of the primary tumor, whereas, all received chemotherapy either as adjuvant or palliative setting. One patient received adjuvant radiation therapy. Three patients received 2nd line and one received 3rd line chemotherapy on disease progression. After a median follow-up of 18 months one patient was surviving on 3rd line chemotherapy with trabectedin. Other three succumbed to disease after progression. CONCLUSION Due to a small number of this malignancy randomized studies are difficult to perform and optimum treatment strategy still need to be defined.

Primary breast lymphoma in males-a report of two cases with a review of the literature.

Primary breast lymphoma (PBL) in males is a rare clinical entity and has been reported in anecdotal case reports up until now. We report two cases of PBL from a tertiary care centre in Southern India. A 46-year-old male presented with a lump in the right breast with right axillary lymphadenopathy; a biopsy with immunohistochemistry showed neoplastic cells positive for CD 20 and negative for CD 30, epithelial membrane antigen, anaplastic lymphoma kinase, suggestive of diffuse large B cell lymphoma stage IIEA. He received three cycles of CHOP (cyclophosphamide, adriamycin, vincristine, and prednisolone) chemotherapy, then developed a cerebrovascular accident, and died. The other patient was a 60-year-old male with a left breast lump with left axillary lymphadenopathy. The biopsy with immunohistochemistry showed neoplastic cells positive for CD 23 and CD 5, suggestive of small lymphocytic lymphoma stage IIEA. Initially he received three cycles of cyclophosphamide, vincristine, and prednisolone (COP) and defaulted. One year later, he received six cycles of COP chemotherapy, developed progressive disease, and was lost to follow-up. The literature on PBL in males was reviewed. To conclude, PBL in males is an extremely rare disease and can mimic breast cancer. A strong index of suspicion with early diagnosis by biopsy with immunohistochemistry and treatment with rituximab- and anthracycline-based chemotherapy followed by radiotherapy will improve overall survival.

Clinical profile and treatment outcomes of metastatic neuroendocrine carcinoma: A single institution experience

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.