Kevin O’Brien, Rahul Dave, Skand Shekhar, Fady Hannah-Shmouni, Leora E. Comis, Beth I. Solomon, Marcus Chen, William A. Gahl, Edmond FitzGibbon, Bernadette R. Gochuico, and Juvianee I. Estrada-Veras
Adult-onset histiocytoses (AOH), primarily Rosai-Dorfman disease (RDD), Erdheim-Chester Disease (ECD), and adult Langerhans cell histiocytosis (ALCH), are a group of related histiocytic neoplastic disorders featuring multisystemic manifestations. The disorders are largely incurable, and are essentially chronic neoplastic diseases with a variable prognosis. Prompt diagnosis and treatment is important to prevent debilitating and even life-threatening complications. Survivorship issues abound in AOH, due to their multisystemic manifestations and the sometimes recalcitrant chronic inflammation, which can lead to other debilitating complications such as fatigue, weakness, and pain. Because these disorders are rare, few healthcare professionals are proficient in their management; therefore the aim of these guidelines is to offer guidance on how to manage patients, and how to create survivorship care plans through the efforts of an interdisciplinary team.
Presenter: Thomas M. Habermann
Several important updates have emerged in the management of early-stage diffuse large B-cell lymphoma. Three trials resulted in the approval of rituximab + cyclophosphamide/doxorubicin/vincristine/prednisone (R-CHOP) for use in these patients internationally. Furthermore, studies have been initiated to determine whether 4 or 6 cycles of this regimen should be administered without radiation therapy (RT). Six cycles of R-CHOP plus central nervous system (CNS) prophylaxis and prophylactic testicular RT are recommended for patients with extranodal disease occupying the testicles. Although controversial, there is a reasonable consensus in the literature to consider 6 cycles of R-CHOP plus involved-site RT and CNS prophylaxis for patients with extranodal disease of the breast. Patients with primary bone and gastric extranodal disease do not seem to derive a significant survival benefit from RT. Molecular subtype evaluations may change treatment approaches.
Presenter: Alice S. Mims
For patients with newly diagnosed acute myeloid leukemia (AML) who are candidates for intensive induction regimens, all therapies include anthracycline- and cytarabine-based backbones. Core-binding factor AML is typically treated with gemtuzumab ozogamicin and 7 + 3 chemotherapy. Patients with FLT3-mutated (ITD or TKD) disease should have midostaurin + 7 + 3 and consolidation, and those with secondary or therapy-related AML should be considered for CPX-351. For patients ineligible for intensive induction regimens, venetoclax has changed the game and should be used in combination with hypomethylating agents or cytarabine. Glasdegib is also approved in combination with low-dose cytarabine. Patients with IDH1/2-mutated disease can be treated with ivosidenib and enasidenib, respectively. Although enasidenib has yet to secure its spot in the up-front setting, data support its use in newly diagnosed AML. An ongoing question in the field concerns how to treat patients with TP53-mutated AML, because most patients do not respond well to currently available therapies and continue to have poor overall outcomes.
Derek J. Erstad, Mariela Blum, Jeannelyn S. Estrella, Prajnan Das, Bruce D. Minsky, Jaffer A. Ajani, Paul F. Mansfield, Naruhiko Ikoma, and Brian D. Badgwell
Background: The optimal number of examined lymph nodes (ELNs) and the positive lymph node ratio (LNR) for potentially curable gastric cancer are not established. We sought to determine clinical benchmarks for these values using a large national database. Methods: Demographic, clinicopathologic, and treatment-related data from patients treated using an R0, curative-intent gastrectomy registered in the National Cancer Database during 2004 to 2016 were evaluated. Patients with node-positive (pTxN+M0) disease were considered for analysis. Results: A total of 22,018 patients met the inclusion criteria, with a median follow-up of 2.2 years. Mean age at diagnosis was 65.6 years, 66% were male, 68% were White, 33% of tumors were located near the gastroesophageal junction, and 29% of patients had undergone preoperative therapy. Most primary tumors (62%) were category pT3–4, 67% had a poor or anaplastic grade, and 19% had signet features. Clinical nodal staging was inaccurate compared with staging at final pathology. The mean [SD] number of nodes examined was 19 . On multivariable analysis, the pN category, ELNs, and LNR were independently associated with survival (all P<.0001). Using receiver operating characteristic (ROC) analysis, an optimal ELN threshold of ≥30 was established for patients with pN3b disease and was applied to the entire cohort. Node positivity and LNR had minimal change beyond 30 examined nodes. Stage-specific LNR thresholds calculated by ROC analysis were 11% for pN1, 28% for pN2, 58% for pN3a, 64% for pN3b, 30% for total combined. By using an ELN threshold of ≥30, prognostically advantageous stage-specific LNR values could be determined for 96% of evaluated patients. Conclusions: Using a large national cancer registry, we determined that an ELN threshold of ≥30 allowed for prognostically advantageous LNRs to be achieved in 96% of patients. Therefore, ≥30 examined nodes should be considered a clinical benchmark for practice in the United States.
Grace E. McKay, Anna L. Zakas, Fauzia Osman, and Amanda Parkes
Background: Given a link between sarcomas and hereditary cancer predisposition syndromes, including Li-Fraumeni syndrome, the consideration for genetic counseling is recommended for all adolescent and young adult (AYA) patients diagnosed with sarcoma. The aim of this study was to evaluate factors influencing genetic consultations in AYA patients with sarcoma at the University of Wisconsin (UW). Methods: A retrospective chart review was performed on AYA patients diagnosed with sarcoma between the ages of 15 and 39 years who were seen at least once between 2015 to 2019 at UW. Our chart review identified discussions regarding genetics, referrals to genetics, genetic consultations, and results of genetic testing. Variables hypothesized to affect patient referrals for genetic consultation were identified a priori. Descriptive statistical methods and a univariate analysis were used to identify patient characteristics associated with genetic counseling referral. Results: We identified 87 AYA patients with sarcoma. Only 19 (22%) of these patients had documentation of a discussion about genetics, 15 (17%) of whom were subsequently referred for genetic consultation. Of these 15 patients, 9 (60%) were seen in consultation. All 9 patients seen by genetics underwent genetic testing, with 4 (44%) of these patients having identified heritable cancer predisposition syndromes. Likelihood for genetics referral was linked most strongly to documented genetics discussion with an oncology provider (P<.001). Conclusions: Despite the recommendation for consideration for genetic counseling in AYA patients with sarcoma, <25% of such patients in our study had a documented discussion about genetics. Supporting this need, all referred patients met criteria for genetic testing, and 44% of tested patients were found to have a heritable cancer predisposition syndrome. These data support the initial conversation by a provider as critical to genetic referral and suggest the need for more specific national recommendations for the genetic evaluation of all AYA patients with sarcoma.
P. Connor Johnson, Netana H. Markovitz, Tamryn F. Gray, Sunil Bhatt, Ryan D. Nipp, Nneka Ufere, Julia Rice, Matthew J. Reynolds, Mitchell W. Lavoie, Carlisle E.W. Topping, Madison A. Clay, Charlotta Lindvall, and Areej El-Jawahri
Background: Social support plays a crucial role for patients with aggressive hematologic malignancies as they navigate their illness course. The aim of this study was to examine associations of social support with overall survival (OS) and healthcare utilization in this population. Methods: A cross-sectional secondary analysis was conducted using data from a prospective longitudinal cohort study of 251 hospitalized patients with aggressive hematologic malignancies at Massachusetts General Hospital from 2014 through 2017. Natural Language Processing (NLP) was used to identify the extent of patients’ social support (limited vs adequate as defined by NLP-aided chart review of the electronic health record). Multivariable regression models were used to examine associations of social support with (1) OS, (2) death or readmission within 90 days of discharge from index hospitalization, (3) time to readmission within 90 days, and (4) index hospitalization length of stay. Results: Patients had a median age of 64 years (range, 19–93 years), and most were White (89.6%), male (68.9%), and married (65.3%). A plurality of patients had leukemia (42.2%) followed by lymphoma (37.9%) and myelodysplastic syndrome/myeloproliferative neoplasm (19.9%). Using NLP, we identified that 8.8% (n=22) of patients had limited social support. In multivariable analyses, limited social support was associated with worse OS (hazard ratio, 2.00; P=.042) and a higher likelihood of death or readmission within 90 days of discharge (odds ratio, 3.11; P=.043), but not with time to readmission within 90 days or with index hospitalization length of stay. Conclusions: In this cohort of hospitalized patients with aggressive hematologic malignancies, we found associations of limited social support with lower OS and a higher likelihood of death or readmission within 90 days of hospital discharge. These findings underscore the utility of NLP for evaluating the extent of social support and the need for larger studies evaluating social support in patients with aggressive hematologic malignancies.
Michael B. Streiff, Bjorn Holmstrom, Dana Angelini, Aneel Ashrani, Amro Elshoury, John Fanikos, Kleber Yotsumoto Fertrin, Annemarie E. Fogerty, Shuwei Gao, Samuel Z. Goldhaber, Krishna Gundabolu, Ibrahim Ibrahim, Eric Kraut, Andrew D. Leavitt, Alfred Lee, Jason T. Lee, Ming Lim, Janelle Mann, Karlyn Martin, Brandon McMahon, John Moriarty, Colleen Morton, Thomas L. Ortel, Rita Paschal, Jordan Schaefer, Sanford Shattil, Tanya Siddiqi, Deepak Sudheendra, Eliot Williams, Liz Hollinger, and Mai Q. Nguyen
NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Cancer-Associated Venous Thromboembolic Disease focus on the prevention, diagnosis, and treatment of patients with cancer who have developed or who are at risk for developing venous thromboembolism (VTE). VTE is a significant concern among cancer patients, who are at heightened risks for developing as well as dying from the disease. The management of patients with cancer with VTE often requires multidisciplinary efforts at treating institutions. The NCCN panel comprises specialists from various fields: cardiology, hematology/hematologic oncology, internal medicine, interventional radiology, medical oncology, pharmacology/pharmacy, and surgery/surgical oncology. This article focuses on VTE prophylaxis for medical and surgical oncology inpatients and outpatients, and discusses risk factors for VTE development, risk assessment tools, as well as management methods, including pharmacological and mechanical prophylactics. Contraindications to therapeutic interventions and special dosing, when required, are also discussed.