Umesh Das

Paranasal Sinus Neuroendocrine Carcinoma: A Case Report and Review of the Literature

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.

A study of triple negative breast cancer at a tertiary cancer care center in southern India

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.

Breast Cancer in Women of Younger than 35 Years: A Single Center Study

Aim: Breast cancer in women younger than 35 years old is uncommon and only accounts for 2% of all breast cancers diagnosed in Western countries. However, there is paucity of data on breast cancer in women younger than 35 years old in India. The aim of this study was to assess clinicopathological parameters in these young breast cancer patients. Methods: This retrospective study summarized data on women younger than 35 years with breast cancer between August 2012 and July 2013 from tertiary cancer center in India. Results: Out of a total of 320 patients, 36 were younger than 35 years old in this period of time and the median age was 32 years. A breast lump was the most commonly presented symptom with an average symptom duration of four months. The median age of the first childbirth was 22 years. Most of the patients had a stage III disease (52%) followed by stage II (33%). All patients had invasive ductal carcinoma and 86% of patients had high-grade tumors. 51% of patients were lymphnode-positive, and lympho vascular emboli were present in 43% of cases. Estrogen receptor (ER)/progesterone receptor (PR) and human epidermal growth factor receptor-2 (HER2) positivity occurred in 38.9% and 22.2% of patients, respectively and 50% of patients were triple negative. Conclusion: Breast cancer occurring in women younger than 35 years old constituted 11.3% of all breast cancer cases in Kidwai Memorial Institute of Oncology, India. They had high tumor grade with a larger size and an increased incidence of lympho vascular emboli, which lead to aggressive behaviors and poor prognosis. This study suggest that early onset of breast cancer must be considered as a diagnostic possibility in Indian patients presenting palpable masses in the mammary glands.

FIP1L1-PDGFRA-Positive Chronic Eosinophilic Leukemia: A Low-Burden Disease with Dramatic Response to Imatinib - A Report of 5 Cases from South India.

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.

An unusual case of pulmonary granulocytic sarcoma treated with combined chemotherapy and radiation.

We report an unusual case of a 6-year-old male child who presented with fever and a cough of one month’s duration. A bone marrow aspiration and cytogenetics were suggestive of acute myeloid leukaemia with t(8;21)(q22;q22). A chest x-ray and computed tomography of the thorax showed a soft tissue lesion in the right lung. The fine needle aspiration cytology (FNAC) of this lesion was suggestive of pulmonary granulocytic sarcoma. The patient was successfully treated with induction chemotherapy (cytosine arabinoside + daunomycin), followed by consolidation with high-dose cytosine arabinoside. In view of the persistent lesion in the right lung, the patient was given external beam radiotherapy (EBRT), which resulted in near total resolution of the lung granulocytic sarcoma. We report this case in view of its rarity and clinical importance, and to highlight the treatment options in this scenario.

Linezolid induced vasculitis: An unusual case report with review of the literature

We report a case of a 17-year-old male with precursor B acute lymphoblastic leukemia who presented with erythematous palpable rash over all extremities and trunk following initiation of linezolid for febrile neutropenia. This rash resolved on discontinuation of linezolid. We report this case with emphasis on review of the literature to highlight this unusual adverse effect and make clinicians aware and create a high index of suspicion in such a situation.

Aflibercept as a second-line therapy in metastatic colorectal cancer: A limited Indian experience

Introduction: Aflibercept in combination with FOLFIRI has been shown to improve overall survival in the pivotal VELOUR study. Aflibercept has not yet been marketed in India. Sanofi has made available this drug for Indian patients under a program called Named Patient Access Program (NPP). We present a limited clinical experience with the use of aflibercept at our center. Materials and Methods: We analyzed the data of the patients who received aflibercept under NPP. Aflibercept was given in combination with FOLFIRI as second-line for patients who progressed on oxaliplatin based therapy. Aflibercept was given at 4 mg/kg intravenous (IV) every 15 days. Chemotoxicities were assessed as per CTCAE. Response evaluation was done every four cycles. Results: Five patients were enrolled. The median age was 34 years. The median number of aflibercept cycles administered was 12. Common grade 2/3 toxicities were mucositis, diarrhea, neutropenia thrombocytopenia, and hypertension seen in three (60%), three (60%), two (40%), two (40%), and one patient respectively. After four cycles, the response was assessed as: One complete remission (CR), three partial remissions (PR), and one progressive disease (PD). Three patients completed 12 cycles of chemotherapy and aflibercept. At the end of 12 cycles, one patient still in CR and two patients were in PR. Four patients were alive till date. Conclusion: As we had very less number of patients, it was very difficult to compare it with VELOUR data. It is one of option as second-line in metastatic colorectal cancer (mCRC) who progressed on oxaliplatin chemotherapy. Mucositis, diarrhea, and hematological toxicity were the most common toxicity in our patient.

A clinical study of hemoglobin E with special reference to serum ferritin level

Introduction: The spectrum of anaemia is modified in many parts of world, due to prevalence haemoglobinopathies. The common haemoglobinopathies includes HbS, HbC, D (Punjab) and E. In Assam with its population consisting of diverse ethnic groups, is a rich reservoir of abnormal haemoglobin and thalassaemia. The incidence of HbE from various population survey of Assam showed that 20% among Indig Assamese, 57.4% among Ahoms and as high as 80% among Bodo-Kacharis. Iron therapy is hazardous in haemoglobinopathies due to risk of tissue iron toxicity, so needs evaluations of iron status before iron therapy. The size of the stored iron in the body is accurately indicated by serum ferritin estimation, which is single most sensitive and non-invasive method for iron status evaluation. Keeping all in minds the aim of the our study is to study the serum ferritin level of patients with HbE attending AMCH, Dibrugarh. Materials and Methods: This study was conducted in the department of medicine, AMCH for 1-year. 80 HbE patients of various religions and tribes were selected for study. Patients with HbE having malignancy, end stage renal disease, arthritis, chronic liver disease, and sepsis were excluded from the present study by careful history taking and laboratory investigations. Serum ferritin level was estimated by IRMA technique. Result and Observation : The incidence of HbE disease (E/E), trait (A/E) and HbE-thalassaemia (E/F) was 47.5%, 42.5%& 10% respectively. Most of the patients were in their second and third decade (57.5%). Male female ratio was 13:7, most being Ahom (60%) and Kochari (12.5%). The haemoglobin concentration in A/E, E/E and E/F were 8.94±0.928, 8.04±0.8840 and 6.02±0.1500; serum ferritin level 24.95±11.90, 162.26±59.5 and 1017±322.19 respectively. E/E patients on blood transfusions have a higher level of serum ferritin (122.85±4.95) than those without transfusions (95.75±9.54). Again those cases of E/F on regular blood transfusions have a higher ferritin (1260±188.14) level than those with occasional transfusions (715±155.80). Conclusion : Serum ferritin assay IRMA technique is simple and suitable for evaluation of serum iron status. Iron deficiency may be seen among HbE trait but normal or higher serum ferritin in E/E and E/F cases in this study suggests that indiscriminate iron therapy could be harmful in most of the patients without prior evaluation of serum ferritin.

Oncological emergencies for the internist

An oncologic emergency is defined as any acute, potentially life-threatening event, either directly or indirectly related to a patient′s cancer (ca) or its treatment. It requires rapid intervention to avoid death or severe permanent damage. Most oncologic emergencies can be classified as metabolic, hematologic, structural, or side effects from chemotherapy agents. Tumor lysis syndrome is a metabolic emergency that presents as severe electrolyte abnormalities. The condition is treated with aggressive hydration, allopurinol or urate oxidase to lower uric acid levels. Hypercalcemia of malignancy is treated with aggressive rehydration, furosemide, and intravenous (IV) bisphosphonates. Syndrome of inappropriate antidiuretic hormone should be suspected if a patient with ca presents with normovolemic hyponatremia. This metabolic condition usually is treated with fluid restriction and furosemide. Febrile neutropenia is a hematologic emergency that usually requires inpatient therapy with broad-spectrum antibiotics, although outpatient therapy may be appropriate for low-risk patients. Hyperviscosity syndrome usually is associated with Waldenstrφm′s macroglobulinemia, which is treated with plasmapheresis and chemotherapy. Structural oncologic emergencies are caused by direct compression of surrounding structures or by metastatic disease. Superior vena cava syndrome is the most common structural oncological emergency. Treatment options include chemotherapy, radiation, and IV stenting. Epidural spinal cord compression can be treated with dexamethasone, radiation, or surgery. Malignant pericardial effusion, which often is undiagnosed in ca patients, can be treated with pericardiocentesis or a pericardial window procedure.

Is pregnancy wise after 12-year chemotherapy of chronic myeloid leukemia woman?

Diagnosis of leukemia during pregnancy is a dramatic event that poses challenges to the pregnant woman, the family, and physicians. Chronic myeloid leukemia (CML) comprises up to 10% of pregnancy-associated leukemia. There is no specific guideline for CML management in pregnant women. This study reported a case of successful pregnancy after 12 years of chemotherapy including tyrosine kinase inhibitor for CML. Pregnancy after 12 years of continuous chemotherapy is rare, which also led a challenge for medical oncologists and patient as well. This study described the assessment of the risk balance and benefit and management of such a patient.