Posterior reversible encephalopathy syndrome in pediatric acute leukemia: Case series and literature review.

Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic state coupled with a unique radio imaging appearance. We describe this rare, mostly reversible condition in five cases undergoing similar treatment under preset protocol (MCP-841) for acute lymphoblastic leukemia (ALL) at our centre. Hypertension is a well-known adverse effect of high-dose corticosteroid therapy primarily mediated by its effects on the mineralocorticoid receptor especially in pediatric population and we hypothesize that this may be the etiology of PRES in two of these patients.

Neurofibromatosis type I with breast cancer: not only for women!

The association of neurofibromatosis type I with invasive male breast cancer is a rare clinical entity with only one case in literature reported in 1953. Women with NF1 are at risk of developing breast cancer and men also may be at risk but there is scarce data on the risk and association of NF1 with male breast cancer due to its rarity. Established clinical trials in male breast cancer patients are lacking and the results are extrapolated from female breast cancer patients. The treatment of male breast cancer is followed as per the guidelines of premenopausal female breast cancer and tamoxifen is the hormone treatment in them. Mendes et al suggests that silencing of NF1 gene confers resistance to tamoxifen. Our conclusions are that since NF1 is mutated or deleted in one third of sporadic breast cancers, its role as a molecular driver for treatment has to be further explored.

Primary non-Hodgkin's lymphoma of bone: poly-ostotic versus mono-ostotic subtypes.

Primary non-Hodgkin's lymphoma of bone (PNHLB) accounts for less than 5% of all primary bone tumours and less than 1% of all non-Hodgkin's lymphoma. Due to its rarity, only a few retrospective studies have been published describing the prognosis and its treatment. We report our experience of 20 cases of PNHLB with their clinicopathologic correlation that were treated at our centre over a period of ten years. There were 16 cases of the mono-ostotic subtype and four cases of poly-ostotic subtype. All of these had a histological diagnosis of diffuse large B-cell lymphoma. The age of presentation was fifth to sixth decade. The mono-ostotic subtype commonly presented with the involvement of femur or humerus, while the poly-ostotic subtype commonly had paraparesis due to vertebral involvement. Cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP)-based chemotherapy was given to all patients, but definitive radiotherapy was used only in the mono-ostotic subtype. At median follow-up of 38 months (range 5-96 months), event-free survival was 81% and 25% with mono-ostotic and poly-ostotic subtypes, respectively. Thus poly-ostotic PNHLB is a distinctive entity with a poor prognosis, and larger studies are needed for better management of this subtype.

Systemic therapy in head and neck cancer: changing paradigm.

Head and neck cancers comprise a heterogenous group of cancers that require a multidisciplinary approach. Last few decades have seen an increasing role of chemotherapy with intent of treatment shifting from palliation to cure. We performed a thorough search online and offline for all relevant articles of chemotherapy in head and neck cancer. Cancers of nasopharynx and salivary glands were excluded.

L-asparginase induced pseudopancreatic cyst: a rare case report.

L-Asparginase is a frequently used drug in hematological malignancies. Various side effects associated with its use include hypersensitivity, hyperglycemia, hypercoaguability and acute pancreatitis. We describe a rare complication of pancreatic pseudocyst in a 12 year old boy of Acute lymphoblastic leukemia (ALL) treated with MCP-841 protocol (during re-induction phase). Nearing the end of induction i.e. day 25, patient complained of a painful swelling in the epigastrium and left hypochondrium. Investigations revealed a pancreatic pseudocyst for which he underwent cystojejunostomy. Post surgical period was uneventful.

Chronic myeloid leukemia in children and adolescents: results of treatment with imatinib mesylate.

Chronic myeloid leukemia (CML) is a rare disease in children, accounting for 2-3% of leukemias in this age group. Few studies have reported on efficacy of imatinib in childhood CML. The purpose of this retrospective study was to determine the efficacy of imatinib in children. A total of 43 patients from age 7 years to 20 years with newly diagnosed CML received imatinib daily at 260 mg/m(2). Response rates, survival and toxicity were evaluated. The median follow-up was 43 months. All patients achieved a complete hematological response. Twenty-five (58.1%) patients achieved a complete cytogenetic response and 18 (41.9%) achieved a major molecular response at any time during their follow-up period. Both overall survival and progression-free survival at 43 months' median follow-up were 100%. Event-free survival was 92.8%. Imatinib was well tolerated. We conclude that imatinib is effective in children and adolescents with CML.

Systemic therapy in soft tissue sarcomas: past, present and future.

Soft tissue sarcomas (STS) comprise 1% of all cancers diagnosed worldwide with more than 40 different histological subtypes each with distinct underlying biology, natural history and response to treatment. Due to the differential chemosensitivity it is imperative to have a correct histological diagnosis for optimal treatment of these patients. Even though surgery remains the primary modality of treatment there is increasing specialization of chemotherapy with respect to histological subtype. In general there is no place for "one size fits all strategy". To correctly define the role of chemotherapy, an extensive search was carried out online and offline for all relevant articles concerning chemotherapy in soft tissue sarcoma. This review aims to discuss the evolution of chemotherapy, its present role in neoadjuvant, adjuvant, metastatic settings and exciting trends with the advent of targeted therapies.