Geetha Narayanan

Multiple myeloma (MM) is a disease characterised by the clonal expansion of malignant plasma cells in the marrow, leading to anaemia...

BACKGROUND: Lymphoblastic lymphomas (LBL) are a rare, aggressive type of non-Hodgkin lymphomas and constitute 2% of all lymphomas. It is classified as T-lymphoblastic lymphoma (T-LBL) and B lymphoblastic lymphoma (B-LBL) and resembles acute lymphoblastic leukemia (ALL), with no or limited bone-marrow involvement. LBL rarely present as soft-tissue swellings. Soft-tissue involvement is more common in B-LBL than in T-LBL. They occur predominantly in adolescents and young adults and have a prognosis similar to ALL.OBJECTIVE: p>The aim was to study the clinical characteristics, treatment response, and survival of patients with lymphoblastic lymphoma presenting with soft-tissue swelling.SETTINGS AND DESIGN: This was a retrospective study of eight adolescent and adult patients with lymphoblastic lymphoma presenting with soft-tissue swelling conducted in the Department of Medical Oncology at a tertiary cancer center in India.STATISTICAL ANALYSIS: The baseline patient characteristics, treatment details, and response assessment were analyzed using descriptive statistics. Overall survival (OS) and progression-free survival were obtained by the Kaplan–Meier method, using the SPSS version. 11.MATERIALS AND METHODS: We present the clinical features, imaging, diagnosis, treatment, and outcome of eight cases of lymphoblastic lymphoma presenting with soft-tissue swellings. Patients above 14 years of age treated over 12 years were included in the study.RESULTS: There were five males and three females. The median age at diagnosis was 24 years. Common presenting complaints were swelling, pain, and paraparesis. The sites of involvement were the epidural mass, thigh, calf, breast, and anterior chest wall. The diagnosis was confirmed by immunohistochemistry. Seven patients were diagnosed with B-LBL and one with T-LBL. They were treated with intensive chemotherapy (six patients with Berlin–Frankfurt–Munster protocol and two with hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone). Three patients received consolidation radiotherapy and one received palliative radiotherapy. Seven patients attained remission after induction chemotherapy and four of them are currently alive at a median follow-up of 48 months. The 2-year OS was 71.4%.CONCLUSION: Even though rare, lymphoblastic lymphoma should be considered a differential diagnosis in patients presenting with soft-tissue swelling and should be managed with systemic chemotherapy similar to ALL.

Background: Waldenstroms macroglobulinemia (WM) or Lymphoplasmacytic lymphoma is a rare B cell lymphoproliferative malignancy characterized by serum monoclonal immunoglobulin M (IgM) protein and lymphoplasmacytic infiltration in the bone marrow. Patients usually present in their seventh decade with symptoms related to the infiltration of the marrow or the effects of monoclonal IgM in the blood. Objective: Was to study the clinical characteristics and treatment outcome of patients diagnosed with WM. Materials and Methods: This is a retrospective analysis of 26 cases of WM treated at a tertiary cancer centre. Results: WM constituted 0.68% of our nonHodgkin’s lymphoma. The median age at presentation was 67 years with a male to female ratio of 2:1. Four patients had lymphadenopathy, six had splenomegaly and four patients had evidence of hyper viscosity. All patients had IgM paraproteinemia, the M band was IgM-kappa in 19 patients and IgM-lambda in seven patients. All patients had a histopathological confirmation. According to IPSS-WM, 12 patients each were in the intermediate and high risk group and 2 in the low risk group. Among the 26 patients, 20 patients received upfront treatment and four patients were kept under observation. The indications for treatment were cytopenia in ten patients, constitutional symptoms in five, hyper viscosity in four and symptomatic lymphadenopathy in one patient. Sixteen patients received rituximab-based chemotherapy. Four patients with features with hyper viscosity underwent plasmapheresis. The three year progression free survival and overall survival were 69.4% and 78 % respectivel. Conclusions: WM is a low grade B cell lymphoproliferative disorder having an indolent course and do not require treatment for prolonged period. Diagnosis is challenging due to lack of distinct diagnostic features. Rituximab containing regimens are the standard of care. Newer targeted treatment options may improve the outcome of this incurable disease.

Background: The extramedullary disease usually occurs in the advanced stages of multiple myeloma and predicts poor survival. Commonly involved extramedullary sites in multiple myeloma include the nasal cavity, lymph nodes, lung, central nervous system, liver, spleen, skin, and orbit. Pleural effusion in multiple myeloma is unusual and rarely due to multiple myeloma itself. Myelomatous pleural effusions occur in less than 1% of cases of multiple myeloma. Material and methods: Eight patients were diagnosed with myelomatous pleural effusion in the Department of Medical Oncology at a tertiary cancer care center in India, during the 7-year period (2012 to 2018). Clinical presentation, diagnosis, treatment, and survival details were abstracted from medical records. Results: We report eight patients with myelomatous pleural effusion, constituting 0.45 % of all multiple myeloma cases treated during the study period. Five out of the eight patients were females with a median age of 58 years. Three patients had myelomatous pleural effusion at the time of initial diagnosis and five developed pleural effusion at disease progression. IgG was the common immunoglobulin subtype. The International staging system stage was stage I in one patient and stage III in seven patients. After the diagnosis of multiple myeloma, all patients developed myelomatous pleural effusion at a median of 7 months and all patients died within 2 weeks.Conclusion: Pleural effusions in multiple myeloma should be investigated to rule out myelomatous pleural effusion. Myelomatous pleural effusion is rare, having a poor prognostic outlook and an aggressive natural course.

Castleman disease (CD) describes a group of rare heterogeneous lymphoproliferative disorders characterized by enlarged hyperplastic lymph nodes. It is classified into unicentric CD (UCD) and multicentric CD (MCD). The present retrospective study examined the data of 11 patients with CD diagnosed and treated at a tertiary cancer center from 2017 to 2022. The median age of the study group was 41 years (range, 24 to 68 years). There were 8 males and 3 females. In total, 7 patients were diagnosed with UCD and 4 patients with MCD. The hyaline‑vascular variant was the most common histology in both UCD and MCD. Among the 7 patients with UCD, 5 patients underwent excision, 1 patient underwent debulking followed by radiotherapy and 1 patient received single agent rituximab. Of the patients with UCD, 6 had a complete response (CR) and 1 patient had a partial response (PR). All 4 patients with MCD received systemic treatment, which included single agent rituximab (2 patients), rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone (RCHOP) (1 patient) and CHOP (1 patient). Among the patients with MCD, 1 patient attained a CR, 2 patients had a PR and 1 patient succumbed. The 3‑year survival rate for the study population was 91%. In summary, CD is a rare disease occurring in immunodeficient patients. UCD is more common and is associated with better outcomes. Surgery is the mainstay of management in UCD whereas MCD requires combination chemotherapy.

Telangiectatic osteosarcoma is an uncommon subtype of osteosarcoma accounting for less than 4% of all cases of osteosarcoma. It is characterized by distinctive radiographic, pathologic features, and prognostic implications. Radiologically and microscopically, it mimics aneurysmal bone cyst. The objective of this study was to study the clinical profile and treatment outcome of patients with telangiectatic osteosarcoma. Thirteen patients were diagnosed with telangiectatic osteosarcoma in the department of medical oncology at a tertiary cancer center in India during a 12-year period. All patients were above 15 years of age. The median age at presentation was 20 years, males were predominant, and the commonest sites of involvement were lower end of femur and upper end of humerus. Ten patients underwent treatment that consisted of neoadjuvant and/or adjuvant chemotherapy with ifosfamide, doxorubicin, cisplatin regimen and limb sparing surgery or amputation. Currently, eight out of ten patients are alive in remission at a median follow-up of 50 months with survival ranging from 18 to 138 months.

Introduction Burkitt's lymphoma (BL) is a highly aggressive B cell non-Hodgkin lymphoma (NHL) having three distinct subtypes: endemic, sporadic, and immunodeficiency-associated BL. Sporadic BL accounts for only 1 to 2% of adult NHL. Objectives The objective of this article was to study the clinical profile and treatment outcome of patients with BL. Materials and Methods This was a retrospective study of 60 patients with BL conducted in the department of medical oncology at a tertiary cancer center in India during a 10-year period. Patients with BL/leukemia above 14 years of age diagnosed during the study period were included and their clinical presentation, treatment details, and outcome were studied. Results Among 60 cases with BL, there were 41 males and 19 females. The median age at presentation was 42 years (range: 14–81 years). The main symptoms were lymphadenopathy, abdominal pain, and abdominal distension. Two patients each had paraparesis, breast lump, and jaw swelling and one patient had involvement of the cervix. Thirteen patients had features of tumor lysis at presentation. The Ann Arbor stage was I in 17, II in 16, III in 5, and IV in 22. Fifty-five patients received combination chemotherapy that included hyper-cyclophosphamide, vincristine, adriamycin, dexamethasone ± rituximab (hyper-CVAD ± R; 35), cyclophosphamide, adriamycin, vincristine, prednisolone/ cyclophosphamide, vincristine, prednisolone ± rituximab CHOP ± R (13), Berlin-Frankfurt-Munich protocol (4), and others (3). Thirty-four patients attained remission, 13 patients had progressive disease, and 8 patients died during chemotherapy. At a median follow-up of 113 months, 58% patients were alive. Conclusions BL accounts for 1.57% of NHL above the age of 14 years with male preponderance. Intensive, short-duration chemotherapy is the standard treatment. Treatment with hyper-CVAD ± R gives 8-year progression-free survival and overall survival (OS) of 60%. Treatment with CHOP ± R is an alternative option in elderly frail patients with an 8-year OS of 46%.

Background: Ewing’s sarcoma (EWS) is an aggressive small round cell tumor, affecting bone and soft tissues and is mostly seen in childhood and second decade of life. EWS accounts for 10–12% of bone tumors in more than 15 years age group and is even rarer after 40 years of age. Materials and Methods: This retrospective analysis was conducted among patients aged more than 15 years with histologically proven EWS. Results: Among 240 cases of EWS treated at our center during 2001–2010, 130 (54%) were more than 15 years of age. The median age was 20 years with a male: female ratio of 2.4:1. Ninety percent had skeletal EWS, 10% had extra skeletal EWS, and 37% patients were metastatic at presentation. Eighty-two received curative treatment with chemotherapy (vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide (VAC/IE)) along with local treatment, radiotherapy (RT) in 61, surgery alone in seven, and RT plus surgery in 14. Two- and 5-year overall survival (OS) was 43.3% and 25.5%, respectively, for the entire series. The OS for the non-metastatic group was 63.2% at 2 years and 36.5% at 5 years, and the progression free survival was 53.7% at 2 years and 37.8% at 5 years. High lactate dehydrogenase was found to be a significant poor prognostic factor ( P = 0.001). Median OS for localized central EWS was 49.2 months and that for peripheral EWS was 24 months. Patients more than 20 years of age with non-metastatic disease had better OS compared to those with 15–20 years of age. Conclusion: Treatment of EWS requires a multidisciplinary approach with radical surgery and/or radiation to control local disease and multiagent chemotherapy to control systemic disease. Long-term follow-up is essential because of disease relapse and treatment-related complications.