Castleman disease: Experience from a single institution.

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.

Acute myeloid leukemia patients with variant or unusual translocations involving chromosomes 8 and 21 - A comprehensive cytogenetic profiling of three cases with review of literature.

Background: t(8;21)(q22;q22) is the most frequent recurrent translocation in acute myeloid leukemia (AML) resulting in an in-frame fusion of RUNX1/RUNX1T1 that regulates various genes involved in the signaling pathways. This leukemogenic alteration is usually associated with a favorable clinical outcome. Variants of t(8;21) can be formed involving a third or fourth chromosome in ~3-4% of t(8;21)-AML. Due to the rarity of variant t(8;21), its clinicopathological features and prognostic significance are still unclear. Here we present three AML cases with cryptic rearrangements of chromosomes 8 and 21 without standard RUNX1/RUNX1T1. Materials and Methods: Conventional karyotyping and fluorescence in situ hybridization and/or spectral karyotyping of the pretreatment bone marrow aspirate of de novo AML patients were performed to delineate chromosomal abnormalities. Results: We identified three cases with novel variants of t(8;21); der(13)t(8;21;13), isodicentric derivative 8 with chromosome 21[,+idicder(8)(q11.1)t(8;21)(q22;q11.1)] and der(21)t(8;12;21)(q22;q?;q22). Conclusion: AML with t(8;21)(q22;q22);RUNX1-RUNX1T1 forms a distinct WHO subcategory and hence the identification of variants or unusual translocations associated with t(8;21) deserves more attention. Contribution to the variant/ unusual t(8;21) database will further refine the risk stratification and may help to significantly advance the current treatment regimen.

Regorafenib in gastrointestinal stromal tumors.

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the GI tract and constitute less than 1% of all digestive tract tumors--the stomach is the most common site. Regorafenib is a multi-tyrosine kinase inhibitor with regulatory approvals granted for colorectal cancers and GIST. The US FDA granted approval for the use of regorafenib in February 2013 in patients with advanced GIST for those who had failed on imatinib and sunitinib. This was based on a pivotal Phase III double-blind placebo controlled randomized trial that showed that there was a significant improvement in progression-free survival for patients on regorafenib.

Regorafenib: carving a niche in the crowded therapeutic landscape.

The oral multikinase inhibitor regorafenib targets both tumor cell proliferation and vasculature and is active in heavily pretreated patients with metastatic colorectal cancer, for which the US FDA granted its approval in September 2012. The benefit for regorafenib was seen in these patients in most prespecified subgroups. The drug is also being used in other tumor types where it has shown exciting potential especially in gastrointestinal stromal tumors. The drug is well tolerated but requires close monitoring during administration. Common side effects include asthenia/tiredness, loss of appetite, hand-foot skin syndrome, diarrhea, mucositis, weight loss, infections, hypertension and rash. Serious adverse events to look out for are liver toxicity, hemorrhage and gastrointestinal perforation. Biomarker data should help us to optimize the use of these drugs to select which patients are most likely to benefit.