Vishwanath Sathyanarayanan

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.

Human Immunodeficiency Virus – Associated Lymphomas: A Neglected Domain

Background: Human immunodeficiency virus (HIV) associated lymphoma is an important public health concern; however, the epidemiological data available from India is sparse. Aims: The present study was carried out at a tertiary cancer care center in South India to analyze the scenario of HIV-associated lymphoma. Materials and Methods: This was a retrospective observational study conducted at our center, on consecutive patients diagnosed with HIV-associated lymphoma, from January 2008 to December 2012. Results: A total of 44 patients were diagnosed with HIV-associated lymphoma, of which 18 opted for treatment. There were 11 males and 7 females in the study population. Median interval from the diagnosis of HIV infection to diagnosis of lymphoma was 18 months. Median CD4 count at the time of lymphoma diagnosis was 218/mm3. Five patients had Hodgkins lymphoma, and the rest had non-Hodgkins lymphoma. Five out of 18 (28%) patients in the present study expired during treatment. Ten (55.5%) patients are alive and lymphoma free, with a median follow up of 18 months. Conclusions: More than half of our treated patients are lymphoma free with a median follow up of 18 months; hence treatment of patients with HIV-associated lymphoma should be encouraged.

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.

Diffuse large B‑cell lymphoma of the thyroid: Seven cases with review of the literature from India

Background: Diffuse large B-cell lymphoma (DLBCL) is the most frequent subtype of nonHodgkin′s lymphoma globally with frequent extra nodal involvement. There is sporadic data available on thyroid DLBCL in Indian population and hence we undertook this retrospective observational study at Kidwai Memorial Institute of Oncology, a tertiary care oncology center in India to analyze the clinico biological features of this disease. Methodology: A total of 7 consecutive cases diagnosed as DLBCL by appropriate lymph node biopsy with evidence of thyroid involvement on fine-needle aspiration cytology of thyroid or thyroid histopathology (following thyroidectomy) were confirmed by immunohistochemistry (WHO classification) between January 2008 and September 2013 were included in this study. Results: A total of 7 patients were included in the study. Median age was 65-years (range: 50-72 years), and all were females. Six out of seven were in stage IIAE and one was in stage IVAE. The distribution according to the international prognostic index was as follows: 3 were in low risk, 1 in low-intermediate, 2 in high-intermediate and 1 in high risk group. Regarding treatment, 5 received CHOP, 1 received COP and 1 received no treatment. 5 patients received radiotherapy. 2 patients underwent subtotal thyroidectomy. Of the 6 patients who received chemotherapy ΁ radiotherapy, 4 had complete response 1 was lost to follow-up at 3 months and 1 alive with no disease at 32 months), 2 had partial response. The median survival was 15 months (3-32 months). Conclusions: Extranodal thyroid DLBCL presents common in elderly age group and in females. A multimodality treatment approach is used to treat this entity and may improve survival compared with unimodality approach.

A Single Center Experience in 266 Patients of Infantile Malignancies

Introduction: The diagnosis and management of cancer in infantile age group is a significant challenge to pediatric oncologists. Malignancies occurring in infants often have different clinical and biological behavior in comparison to older children. This study was performed with an aim to find out the profile of infantile cancers at a tertiary care cancer center in South India. Methods: The present study was a retrospective analysis of infants presenting with malignancy between 2003 and 2012 to our center in South India. Result: A total of 4588 pediatric patients were registered in the Department of Pediatric Oncology at our institute between 2003 and 2012. Among those, 266 (5.79%) of the patients were infants (0–1 years). There were 65.75% males and 34.25% females. Solid tumors were the most common malignancy in this age group (72.56%). Leukemias were observed in 67 (25.19%) infants. ALL was the most common hematological malignancy (17.29%) followed by AML (5.64%). Common solid tumors in descending orders were neuroblastoma, soft tissue sarcoma, renal tumors, germ cell tumors, retinoblastoma and hepatoblastoma. Thirteen (4.89%) neonates were seen in our study. The most common malignancy in neonates was neuroblastoma. Conclusion: 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.

A case of tonsillar anaplastic large cell lymphoma-anaplastic lymphoma kinase negative: An unusual site of involvement with review of literature

We present this unusual case of the clinical importance of a 50-year-old male patient who presented with foreign body sensation in the throat and halitosis of 20 days duration. On examination, there were no palpable lymph nodes and oral cavity revealed an ulcero proliferative growth over the right tonsil. Computed tomography of the paranasal sinuses and neck revealed a heterogeneously enhancing mass involving the right tonsil measuring 3.8 cm × 3 cm. Biopsy of the tonsillar mass was suggestive of anaplastic large cell lymphoma (ALCL) with neoplastic large cells positive for CD30, epithelial membrane antigen and CD3 and negative for Tdt, CD56, anaplastic lymphoma kinase (ALK) and cytokeratin. A diagnosis of ALK negative ALCL Stage IA was made and the patient was started on chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone every 3 weeks. He received six cycles of chemotherapy followed by 33 gray involved region radiotherapy and reassessment showed total regression of the tonsillar lesion. The patient is in complete remission and now under follow-up for the last 2 years

Gefitinib-induced skin ulceration in metastatic adenocarcinoma lung

We report a case of gefitinib-induced skin ulceration in a 50-year-old female with metastatic adenocarcinoma of lung who developed this adverse effect 2 weeks following initiation of gefitinib at a dose of 250 mg/day. The ulcer improved with stopping gefitinib for 2 weeks and also addition of topical steroids and antibiotics. We are reporting this case to create awareness among treating oncologists of this adverse effect and also prompt interruption of therapy and topical steroids/antibiotics is useful to treat this adverse event.

Primary central nervous system lymphoma: An Indian Perspective

Context: Primary central nervous system lymphoma (PCNSL) is an unusual entity comprising around 5% of extra nodal lymphomas with a dismal prognosis in spite of a multi-modality treatment protocol involving chemotherapy and radiotherapy. There is sporadic data from this part of the world and hence we took up this study to analyze the outcome with various modalities of treatment. Materials and Methods: We undertook this study between January 2007 and January 2013 on 33 consecutive patients diagnosed with PCNSL to analyze the clinical profile and treatment outcomes of PCNSL at a tertiary care oncology center in South India. Twelve patients received the DeAngelis protocol and the remaining 21 patients received either radiotherapy or a combination of radiotherapy and anthracycline based therapy or steroids. This study also compared the outcomes with protocols of DeAngelis et al. and cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) regimen with/out radiotherapy. Results: A total of 33 patients with PCNSL were studied. Median age was 40 years (range 22-75 years). Male: Female ratio was 3.1:1. All 33 were histologically diffuse large B-cell lymphoma (DLBCL). HIV was positive in 1. The most common presenting symptom was focal neurological deficits in 19 (58%). Cerebrospinal fluid was positive in 2 (6%). The area of involvement was mainly cerebral hemispheres 20 (61%). The treatment protocols followed were DeAngelis in 12, cyclophosphamide, doxorubicin, vincristine and prednisolone with radiotherapy (CHOP + radiation therapy [RT]) in 12, RT alone in 6, CHOP in 2 and 1 received no therapy. On a median follow-up of 17 months, the median overall survival was 15 months (range 1-60 months) with DeAngelis protocol and 12 months (range 8-24 months) with CHOP + RT. Conclusions: DeAngelis protocol has improved the prognosis in patients with PCNSL compared with other protocols as shown in our study. Newer chemotherapy regimens or targeted therapies need to be evaluated to further improve the survival.

Extranodal diffuse large B-cell lymphoma: Experience from a tertiary care oncology center in South India

Aims: Diffuse large B-cell lymphoma (DLBCL) is the most common non-Hodgkins lymphoma (NHL) with frequent extra nodal (EN) presentation. The overall occurrence of lymphomas has been increasing; however, those of EN-NHL have been increasing much more rapidly. There is limited data found on EN-DLBCL in the Indian population and hence we carried out this retrospective observational study of primary EN-DLBCL at our center in Southern India. Materials and Methods: A total of 90 consecutive cases diagnosed as EN-DLBCL (according to the standard criteria) by tissue biopsy confirmed by immunohistochemistry between 2007 and 2011 were included. Staging workup including computed tomography of neck, thorax and abdomen and pelvis, bone marrow aspiration and biopsy was done and International Prognostic Index (IPI) calculated. Staging was according to Cotswolds modification of Ann Arbor. The actuarial survival analysis was performed by Kaplan-Meier. Data were analyzed using the SPSS (version 16) statistical software. Results: The median age in this study was 49 years (18-88) with results showing EN-DLBCL to be 1.36 times more common in males. Advanced stages were seen in 15 subjects (16.6%) and bulky disease in 13 subjects (14.4%). CD20 was positive in 89 (98.8%) while 32 had high serum lactate dehydrogenase. According to the IPI most were low-risk-56 (66.6%). Overall response rate for the various combination chemotherapies was 85.7% with complete response in 62.3%. The overall survival range spanned from 2 to 123 months. Univariate analysis showed only bulky disease was associated with inferior survival. Conclusions: EN-DLBCL was present at an early age compared to nodal DLBCL, present more often in early stage and low IPI score. Chemoimmunotherapy with radiotherapy to the EN or bulky site is the standard treatment at present.