Latest update on the clinical features and management of Merkel cell carcinoma.

Merkel cell carcinoma (MCC), is a rare, highly malignant skin tumour, with a poor prognosis. Though the aetiology of MCC is not known, but there are several features that it shares with melanoma. These include the natural history, clinical features and behaviour, e.g. an early spread to nodal sites, high local recurrence rate and early metastasis. Incidence of MCC is seen to be increased in immunosuppressed transplant patients, in patients with rheumatoid arthritis and in B cell malignancies, with a strong male predominance. Despite the ongoing research and advancement, MCC yet poses a challenge to the clinicians because of its rarity. The purpose of this paper is to review the most salient and clinically relevant updates of MCC since its first publication in July 2007 in JPMA. In order to expedite an improved understanding of the new diagnostic modalities, treatment and preventive measures, along with the new staging system established in 2009 after an extensive literature review, and an analysis of over 5,000 patients using the National Cancer Database has all been included in our article.

General physicians' perspective of sleep apnea from a developing country.

To assess the knowledge of general physicians about the diagnosis and management of obstructive sleep apnea (OSA), a self-administered questionnaire, containing 15 questions, was distributed to 160 doctors attending a pulmonary CME program in March 2002. After 15 minutes of response time, the questionnaires were collected. The data were entered and analyzed using SPSS (Version 10.0) software. One hundred and twenty (75%) questionnaires were returned. Only 41% of responders had ever read an article about OSA and 36% had suspected it at least once in their practice. The majority (61-77%) of responders were aware of the common symptoms of OSA, but 55% did not recognize its association with hypertension. A significant number of doctors were not aware that OSA could occur in non-obese individuals (33%), women (42%) and children (39%). Only 25% of responders recognized that a history and blood tests were insufficient to make a reliable diagnosis of OSA. Half of the responders were aware of CPAP therapy for OSA, whereas 18% would have prescribed sedatives to treat sleep disturbances in OSA.

Nasopharyngeal carcinoma - an update of treatment and acute radiation induced reactions from a tertiary-care hospital in Pakistan.

INTRODUCTION: Nasopharyngeal carcinoma (NPC) is a diverse entity with a multi-factorial etiology and a distinct racial and geographical distribution. It is curable if diagnosed and treated early. METHODS: This descriptive study covered 30 patients who underwent radiotherapy (RT) for nasopharyngeal malignancies during February 2006 till November 2010 at the Department of Radiation Oncology, Aga Khan University Hospital (AKUH), Karachi. RESULTS: Thirty cases of nasopharyngeal tumors were registered; a case of embryonal rhabdomyosarcoma and another of diffuse large B-cell lymphoma were not included in the final analyses. There were 20 (71.0%) males and 8 (29.0%) females, 2 and 5 being observed in adolescents. The mean age of the male and female patients was 43.7 [SD 20.1] and 30.3 years [SD 17.9], respectively. Nodal involvement was seen in 23 cases and cranial in 8. Almost two-thirds the patients presented with a stage IV disease and all but two received chemotherapy. Electrons or photons were used for 23 cases; low anterior neck field was used in 25 cases. The doses of radiotherapy delivered ranged between 2000cGy to 7400cGy, the lower ones being given for palliation only. Once spinal cord tolerance was reached, electron boost fields were used in order to treat the posterior neck. A nasogastric tube was required for feeding in 2 (10.0%) male and 2 (25.0%) female patients; gastrostomy was needed for 1 (5.0%) patient. Radiotherapy (RT) induced reactions were observed in 90% of the patients, but were mild in most cases. CONCLUSIONS: In the presence of clinician based expertise and technical constraints in Pakistan, nasopharyngeal tumors are still being treated optimally with the need of creating a better awareness in public for early detection.

Lymphoma cases referred to the radiation oncology service of a tertiary referral university hospital in Karachi, Pakistan: a retrospective study.

INTRODUCTION: Radiation therapy is an important component of curative therapy for Lymphoma [Hodgkin's disease (HD) and non Hodgkin's Lymphoma (NHL)]. The current study was conducted to give us an overview of lymphoma patients presenting to a tertiary care hospital for complementary adjuvant RT. METHOD: Data of lymphoma patients who underwent radiotherapy during February 2006 till August 2009 at the department of radiation oncology, Aga Khan University, Hospital, Karachi was retrieved from the medical records and analyzed using SPSS (version 16.0). RESULTS: A total of 1,678 cancer patients were registered, 75 (0.45%) were lymphoma patients (25.3% HD; 74.7% NHL). HD and NHL were both seen predominantly in males, with a male:female ratio of 2 and 3 respectively. Nodal HD comprised 94.7% and nodal NHL comprised 41.1%. Extranodal NHL (EN-NHL) comprised 53.6% whereas 5.4% cases had both nodal and EN-NHL; 6.7% of EN-NHL were primary CNS lymphomas. Stages of presentation for HD were IIA (52.6%), 1A (21.1%), IIB (10.5%) and IB, IIIB and IVA collectively 15.9%. The ages of HD patients ranged from 11 to 54 years (median 23.5 years 13.2 SD). Response to therapy for HD was 52.6% complete remission, 36.8% partial response, and 5.3% each with stable and progressive disease. Almost all patients (94.7%) received radical treatment with radiation doses (between 1950 cG to 5404 cGy) with a median of 40 Grays.Stages at presentation for NHL were II (23.2%), IV (21.4%) and IE (17.9%); I, IIE, and III were found to be 12.5 %. NHL patients ranged from 15 to 88 years. It was more commonly observed amongst the elderly with 25% patients diagnosed in the fifth decade of life; 50% patients aged at or above 50 years, 41.1% belonged to the 25-49 years age group and 8.9% below 25 years of age. Majority of the cases were diffuse NHL (82.1%), follicular NHL (7.1%), Primary CNS unclassified type (8.9%), and unclassified other than CNS variety (1.8%). Response to chemotherapy for NHL was 51.8% complete remission, 25.9% progressive disease, 20.4% partial response. Forty (71.4%) patients with NHL received radical treatment with radiation doses between 2340 cG to 6600 cGy; 28.6% received palliative RT. CONCLUSION: Radiation therapy though important for curative lymphoma therapy, is relatively underutilized in our population, despite encouraging outcomes.