Plasmablastic lymphoma of the oral cavity with breast recurrence: a case report.

BACKGROUND: Plasmablastic lymphoma is an aggressive variant of diffuse large B cell lymphoma, mostly found in the oral cavity and associated with human immunodeficiency virus. There are no clear guidelines for its treatment. Therapies more intensive than cyclophosphamide, doxorubicin, vincristine, and prednisone are not associated with a prolonged survival. Lymphomas of the breast are rare, in one series representing 0.14% of all female breast malignancies, with diffuse large B cell lymphoma comprising up to 55% of all cases. Only one case of plasmablastic lymphoma involving the breast has been reported in the literature. CASE PRESENTATION: A 30 year old Pakistani woman, presented with a small nodule in the floor of the mouth. An excisional biopsy revealed CD20, CD3, and CD117 negative and CD138, CD79a, CD56, MUM1/IFR4 and CD30 positive lesion with Ki-67 of 60% with cells which were plasmablastic in appearance. The morphological and immunohistochemistry features were consistent with plasmablastic lymphoma. The staging scans did not reveal any lymphadenopathy and the bone marrow biopsy and human immunodeficiency virus test were both negative. After treatment with four courses of CHOP and later radiation to the floor of the mouth, her disease was in complete remission. Two months later, she presented with velvety red lesions in both breasts and its trucut biopsy was consistent with plasmablastic lymphoma. Her CT scans revealed multiple nodules involving both breasts with no lymphadenopathy. The bone marrow was now positive for disease. Her disease continued to progress despite second and third line chemotherapy with DHAP (dexamethasone, cisplatin and cytarabine) and ICE (ifosfamide, carboplatin and etoposide) respectively. Her last CT scans revealed progressive disease with new lung lesions. The patient decided to opt for best supportive care. CONCLUSION: To our knowledge this is the second report of plasmablastic lymphoma involving the breast. The patient who was human immunodeficiency virus negative and immune competent had progressive disease despite three lines of chemotherapies with an overall survival (to date) of 15 months.

Ewing's sarcoma arising from the adrenal gland in a young male: a case report.

BACKGROUND: Ewing's sarcoma uncommonly arises from extraosseous soft tissue or parenchymal organs. Primary adrenal Ewing's Sarcoma, although very rare, is extremely aggressive and commonly fatal. CASE PRESENTATION: A 17 year old Pakistani male was referred to the outpatient oncology clinic at our center with a three month history of concomitant pain, swelling and dragging sensation in the right hypochondrium. Abdominal examination revealed a large, firm mass in the right hypochondrium extending into the right lumbar region and epigastrium. His genital exam was unremarkable and there were no stigmata of hepatic or adrenal disease.Computed tomography scans revealed a large peripherally enhancing mass in the hepatorenal area, biopsy of which showed a neoplastic lesion composed of small round blue cells which exhibited abundance of glycogen and stained diffusely positive for CD99 (MIC2 antigen). Fluorescence in situ hybridization demonstrated gene rearrangement at chromosome 22q12 which confirmed the diagnosis of Ewing's sarcoma. Staging scans revealed pulmonary metastasis and hence he was commenced on systemic chemotherapy. CONCLUSION: This case report highlights the importance of keeping Ewing's sarcoma in mind when a young patient presents with a large non-functional adrenal mass.

The financial burden of cancer: estimates from patients undergoing cancer care in a tertiary care hospital.

INTRODUCTION: The emotional burden associated with the diagnosis of cancer is sometimes overshadowed by financial burden sustained by patient and the family. This is especially relevant for a developing country as there is limited state support for cancer treatment. We conducted this study to estimate the cost of cancer care for two major types of cancer and to assess the perception of patients and families regarding the burden of the cost for undergoing cancer treatment at a private tertiary care hospital. METHODS: This cross-sectional study was conducted at day care and radiotherapy unit of Aga Khan University, Hospital (AKUH) Karachi, Pakistan. All adult patients with breast and head & neck cancers diagnosed for 3 months or more were included. Data was collected using a structured questionnaire and analysed using SPSS. RESULTS: Sixty seven patients were interviewed during the study period. The mean and median monthly income of these patients was 996.4 USD and 562.5 USD respectively. Comparatively the mean and median monthly cost of cancer care was 1093.13 USD and 946.42 USD respectively. The cost of the treatment either fully or partially was borne by the family in most cases (94%). The financial burden of cancer was perceived as significant by 28 (42%) patients and unmanageable by 18 (27%) patients. This perceived level of burden was associated significantly with average monthly income (p = <0.001). CONCLUSION: Our study indicates that the financial burden of cancer care is substantial and can be overwhelming. There is a desperate need for treatment support programs either by the government or other welfare organisations to support individuals and families who are already facing a difficult and challenging situation.

Outcome of febrile neutropenic patients on granulocyte colony stimulating factor in a tertiary care hospital.

INTRODUCTION: Febrile neutropenia is a relatively frequent event in cancer patients treated with chemotherapy and improvement in absolute neutrophil count (ANC) has been linked directly to improved outcome. Evaluation of granulocyte colony stimulating factors (GCSFs) for treatment has shown reduced incidences of episodes of prolonged neutropenia and protracted hospitalization. To determine absolute neutrophil counts with GCSF in febrile neutropenic cancer patients admitted to a tertiary care centre and to co-relate the improvement in ANC with mortality and hospital discharge. METHODS: A prospective cross sectional study was carried at an oncology ward at Aga Khan University hospital from January 2010 to June 2011. All adult patients who were admitted and treated with GCSF for chemotherapy induced febrile neutropenia were included. Multivariable regression was conducted to identify the factors related with poor outcomes. RESULTS: A total of 131 patients with febrile neutropenia were identified with mean age of 43.2 (18-85) years, 79 (60%) being ≤ 50. Seventy-five (57%) had solid tumors and 56 (43%) hematological malignancies, including lymphoma. Fifty seven (43.5%) had an ANC less 100 cells/mm(3), 34 (26%) one between 100-300 cells/mm(3) and 40 (31%) an ANC greater than 300 cells/mm(3). Thirty (23%) patients showed ANC recovery in 1-3 days, and 74(56%) within 4-7 days. Thirteen (10%) patients showed no recovery. The overall mortality was 18 (13.7%) patients. The mean time for ANC recovery seen in hematological malignancies was 6.34 days whereas for solid tumors it was 4.88 days. Patients with ANC <100 cells/mm(3) were more likely to die than patients with ANC >300 cells/mm(3) by a factor of 4.3. Similarly patients >50 years of age were 2.7 times more likely to die than younger patients. CONCLUSION: Our study demonstrated that use of GCSF, in addition to intravenous antibiotics, in treatment of patients with chemotherapy induced febrile neutropenia accelerates neutrophil recovery, and shortens antibiotic therapy and hospitalization. We propose to risk classify the patients at the time of admission to evaluate the cost effectiveness of this approach in a resource constrained setup.

Factors influencing the opinion of individuals in determining tumour spread after biopsy.

BACKGROUND: People often have concerns regarding tumour spread after biopsy which leads to a delay in seeking expert medical advice. The data regarding this perception is scanty. Therefore, we conducted this cross sectional study to explore the beliefs and perceptions of individuals regarding tumour spread after biopsy and the basis of those beliefs. METHODS: The survey was conducted in outpatient areas of two different tertiary care hospitals of Karachi namely Aga Khan University Hospital Karachi (AKUH) and Karachi Institute of Radiotherapy and Nuclear Medicine (KIRAN). We interviewed 600 individuals and documented their responses on a questionnaire. There were 400 responders from Aga Khan's Consulting Clinic and 100 each from Aga Khan's Oncology Clinic and KIRAN. RESULTS: Only 50% of the respondents chose biopsy as the best test for diagnosis of cancer. The level of education was statistically significant in making this choice of answer (p = 0.02) only in univariate analysis. Those individuals who were involved in the work up of cancer patients irrespective of their educational status gave more intelligent answers (p = 0.003). The tumour disturbance after biopsy was regarded as a major factor among 127 respondents (53%) who believed that biopsy could lead to spread of tumour. CONCLUSIONS: Our study revealed that awareness regarding cancer diagnosis and biopsy is lacking among general public and it does not co-relate well with the level of formal education. These misconception and taboos need to be addressed in public seminars and in the media in order to increase the awareness which could facilitate prompt diagnosis.

Four year experience of sarcoma of soft tissues and bones in a tertiary care hospital and review of literature.

BACKGROUND: Sarcoma encompasses an uncommon group of cancer and the data is insufficient from Pakistan. We report our four years experience of Sarcoma of soft tissues and bones. METHODS: This cross sectional study was carried out at Aga Khan University Hospital from 2004 to 2008. The patients were divided into two groups from the outset i.e. initially diagnosed and relapsed group and separate sub group analysis was conducted. RESULTS: Out of 93 newly diagnosed patients, 58 belonged to bone sarcoma and 35 to soft tissue sarcoma group. While for relapsed patients, 5 had soft tissue sarcoma and 9 had bone sarcoma. Mean age was 32.5 years. At presentation, approximately two third patients had localised disease while remaining one third had metastatic disease. The Kaplan Meier estimate of median recurrence free survival was 25 months, 35 months, and 44 months for Osteogenic sarcoma, Ewing's sarcoma and Chondrosarcoma respectively. For Leiomyosarcoma and Synovial sarcoma, it was 20 and 19 months respectively. The grade of the tumour (p = 0.02) and surgical margin status (p = 0.001) were statistically significant for determination of relapse of disease. CONCLUSION: The median recurrence free survival of patients in our study was comparable to the reported literature but with significant lost to follow rate. Further large-scale, multi centre studies are needed to have a more comprehensive understanding of this heterogeneous disease in our population.

Career goals of trainee physicians in internal medicine.

OBJECTIVE: To survey Internal Medicine trainees' future career choices and factors influencing their decision-making. DESIGN: Cross-sectional study. PLACE AND DURATION OF STUDY: The Aga Khan University Hospital, Karachi, from November 2006 to January 2007. SUBJECTS AND METHODS: A standard questionnaire was administered to the residents and Interns working in the Department of Internal Medicine of The Aga Khan University Hospital. The questionnaire covered demographic details, specialty choice, work-time distribution and factors influencing career choice. Descriptive statistics were applied. RESULTS: A total of 49 doctors participated with 57% females; and 43% males, having 33% interns and 67% residents. A minority (22%) opted for internal medicine, while 78% selected sub-specialties with cardiology (20%) being the most sought- after sub-specialty. Majority (69%) wanted to spend more time in clinical activities compared to administration (18%) or research (13%). Majority (92%) wanted to work in an academic setting. Availability of a structured training program (61%), ability to practice broad area of medicine (41%) and prestige of the specialty (41%) highly influenced their career choices. Forty one (82%) wanted to proceed abroad for further training and 80% wanted to eventually practice in Pakistan. CONCLUSION: Most of the interviewed under-training physicians wanted to pursue sub-specialty. Clinical work was more attractive compared to research. A structured training program was detrimental in affecting their choices. Majority wanted further training abroad but eventual settlement in Pakistan. The study results can help us create the basis for reforming the current training programs.