Chemotherapy in adult soft tissue sarcoma.

Soft tissue sarcomas (STSs) are rare and histologically diverse neoplasms. Recent results of various meta-analyses and development of newer drugs have changed the medical management of soft tissue sarcoma. This review gives an outline of chemotherapy and the newer targeted therapies for the same. We have carried out an extensive search in PubMed, Medline for almost all relevant articles concerning chemotherapy of soft tissue sarcoma. The available data from the literature is mainly composed of the most recent reviews, meta-analyses, phase II, and randomized phase III trials published in various peer reviewed journals and various international conferences. The role of neoadjuvant and adjuvant chemotherapy has been found to be controversial. The recent meta-analysis for adjuvant therapy in STSs has shown an increase in the overall survival with combination of ifosfamide and adriamycin. In locally advanced and metastatic STSs, single agent adriamycin remains the basic standard of medication. The combination of ifosfamide and adriamycin may also be used for rapid symptom relief and in patients planned for curative resection for metastases. Newer combinations of docetaxel and gemcitabine appear promising in selected subgroups, especially in leiomyosarcoma and malignant fibrous histiocytoma. Some recent developments include the European Union's approval of trabectedin for advanced STSs patients who had progressed on adriamycin and ifosfamide therapy. The future of mTOR inhibitors, insulin like growth factor receptor inhibitors and anti-angiogenic drugs appear quite promising. Newer methodologies such as, Bayesian adaptive randomization and inclusion of newer end points like progression-free rate, time of progression rate, and tumor growth rate will improve the results of sarcoma trials. At the end of each section we have also presented recommendations from *European Society of Medical Oncology and **National Comprehensive Cancer Network guidelines v.1.2009 for better correlation with the present literature.

Waldenstrom's macroglobulinemia.

Waldenstrom's macroglobulinemia is an uncommon lymphoplasmacytic lymphoma presenting with hyperviscocity and autoimmune phenomenon. Disease is characterized by bone marrow infiltration by lymphoplasmacytic cells and raised IgM. Bone marrow morphology and immunohistochemistry is important for diagnosis. Course is indolent and anemia and age are most important prognostic factors. Treatment options include alkylating agents, anti-purine anti-metabolites, which though not curative but offer valuable responses. Newer agents like Rituximab and autologous transplant are being tried.

Chronic myeloid leukemia.

Chronic myeloid leukemia is one of the commonest hematological malignancies seen in clinical practice. It is the result of abnormal and excess cell proliferation due to de-regulated bcr-abl tyrosine kinase activity as a result of Philadelphia chromosome. The present article discusses the various options available to treat the disorder. Allogeneic stem cell transplant remains the gold standard and the only curative option. Hydroxyurea and Busulfan helps in controlling the total leukocyte count but fail to impact on survival. Interferon especially when combined with cytarabine is curative in minority of patients though a substantial number of patients achieve functional cure. Imatinib, a molecular targeted oral therapy, against bcr-abl tyrosine kinase is the latest addition to various treatment options. Early results appear very promising and can be considered as non- transplant standard of care.

Gastrointestinal stromal tumour--a paradigm shift in management of solid tumours.

Gastrointestinal stromal tumour (GIST) till recently were non-responsive to all chemotherapy agents. With the advent of c-kit, diagnosis of GIST has become more specific. STI-571, a tyrosine kinase, has become one of the first targeted therapeutic agent tobe active in solid tumour. At present it is the only agent with substantial activity in GIST.

The rheumatological prodrome: an unusual inaugural manifestation of acute myeloblastic leukemia.

Uncommon patterns of presentation of acute leukemia pose diagnostic problems. A rheumatological prodrome in acute myeloblastic leukemia is very rare. We describe one such patient who had a normal haemogram. Bone marrow examination done later revealed acute myeloblastic leukemia. The case is discussed with reference to literature.

Primary central nervous system lymphoma a report of nine cases.

Primary Central Nervous System Lymphoma (PCNSL) is a rare neoplasm of B cell origin and constitute less than 1% of Non-Hodgkin's lymphoma (NHL). Histology is mainly of high grade and intermediate type. Although NHL is known to be highly sensitive to both irradiation and cytotoxic drugs, being a curable malignancy, the therapeutic results remain disappointing. Clinical observations on nine cases of PCNSL seen in one of the major cancer centres in India is presented in this paper. Radiotherapy combined with Chemotherapy although yielded encouraging initial response in these patients, the long term response was unsatisfactory with median survival for these patients being only 19 months. This warrants an alternative therapeutic approach to improve the dismal prognosis of PCNSL.

Spinal cord compression by primary non-Hodgkin's lymphoma.

Epidural Cord Compression (ECC) by primary lymphomas is rare entity and constitutes less than 3% of total malignant lymphoma with Non-Hodgkin's Lymphoma (NHL), diffuse large cell type being the most common histological subtype. In this paper 16 cases of primary NHL with cord compression seen at the Department of Medical Oncology, during the period 1988-1990 are reviewed. At presentation all patients had undergone Laminectomy with decompression of epidural mass. The histological diagnosis of NHL was subclassified according to the International working formulation and was evaluated for disease process elsewhere in the body. All patients with ECC by lymphoma received high dose steroids with concurrent Radiotherapy (local) and combination Chemotherapy. These patients had longer duration of neurological deficit prior to treatment had poor response. After 6 courses of chemotherapy 50% of the patients had complete neurological recovery (CR), 31% had partial neurological recovery (PR) and in 19% there was no neurological recovery (NR).