Gastrointestinal Stromal Tumors: What Is the Best Sequence of TKIs?

OPINION STATEMENT: In our practice, we evaluate the mutation status of advanced unresectable disease to guide decisions on use of tyrosine kinase inhibitor (TKI) therapy. This review focuses on management of GIST with KIT and PDGFRA mutations. Imatinib is first-line treatment for unresectable gastrointestinal stromal tumors (GISTs) unless they harbor a PDGFRA D842V mutation; it is recommended to escalate imatinib to twice daily dosing for KIT exon 9 mutant tumors. When patients progress on first-line treatment, treatment is changed to sunitinib followed by regorafenib; while the spectrum of activity against resistance mutations varies with these agents, routine biopsies provide data on one area of disease and ctDNA has not been validated prospectively. For those with a PDGFRA D842V mutation, avapritinib is the first TKI to lead to tumor response and disease control. Ripretinib is approved in the 4th line setting, with limited data on its benefit for PDGFRA D842V GIST. Avapritinib can be considered for treatment beyond ripretinib for KIT mutant disease. The efficacy of other TKIs tested in GIST is reviewed. Ongoing therapy provides palliative benefit and should be continued given rapid decline observed off of treatment.

Interventions to improve quality of life (QOL) and/or mood in patients with head and neck cancer (HNC): a review of the evidence.

BACKGROUND: Patients with head and neck cancer (HNC) experience significant physical and psychological distress, which have a negative impact on their quality of life (QOL). Few strategies have been studied to help improve QOL in this patient population. RESULTS: In this article, we review the existing literature for intervention studies that focus on improving QOL and/or mood in HNC patients. Our review yielded 14 studies that met criteria. Types of interventions included educational, psychosocial, physical and psychological symptom management, mindfulness, pharmacologic, exercise, and telemedicine. Although the majority of the studies had small sample sizes or other methodological limitations, many showed preliminary feasibility and acceptability with some positive impacts on QOL and/or mood. CONCLUSIONS: Larger studies are warranted with more robust randomized designs to determine efficacy of interventions to improve QOL and/or mood in patients with HNC. Additionally, future studies must also consider strategies for implementation and dissemination of these interventions into the health care system to improve the physical and psychological burden of HNC as a population.

Brentuximab Vedotin Therapy for Cutaneous Lesions in T-Prolymphocytic Leukemia: A Case Report.

We present an 88-year-old male with simultaneous T-cell prolymphocytic leukemia and stable smoldering myeloma with excellent initial response to three months of alemtuzumab. The patient relapsed at twelve months with severe cutaneous disease. Biopsy of a representative plaque demonstrated CD30 positivity in rare malignant lymphocytes. The patient demonstrated no response to reintroduction with a full course of alemtuzumab. He was therefore treated with brentuximab vedotin, resulting in partial remission of skin involvement that persisted for three months.

Low-Grade Esthesioneuroblastoma Presenting as SIADH: A Review of Atypical Manifestations.

Esthesioneuroblastoma (ENB) is a neuroendocrine tumor that typically manifests as advanced stage malignancy in the superior nasal cavity. The hallmark symptoms include nasal obstruction and epistaxis, which result from local tissue invasion. Atypical clinical features can also arise and must be considered when diagnosing and treating ENB. These can include origin in an ectopic location, unusual presenting symptoms, and associated paraneoplastic syndromes. The case described here reports a nasal cavity ENB with atypical clinical features that occurred in a young female. Her tumor was low grade, appeared to arise primarily from the middle nasal cavity, and presented as syndrome of inappropriate antidiuretic hormone (SIADH). She also became pregnant shortly after diagnosis, which had implications on her surgical management. We review the atypical features that uncommonly occur with ENB and the clinical considerations that arise from these unusual characteristics.