Pleomorphic liposarcoma of the uterus (PLU) is an extremely rare disease with poor prognosis. Limited treatment options exist for these patients, and disease recurrence usually occurs rapidly within months of initial diagnosis. Few case reports of metastatic PLU are available in the literature. We describe a 70-year-old woman who presented with a large uterus and ovarian mass on imaging and negative serum tumor markers and endometrial biopsy. Staging revealed localized disease. Surgical resection revealed PLU on pathology. Immunohistochemistry was negative for smooth muscle actin (SMA), S100, and MDM2, and positive for CD10 and cyclin-D1. She was treated with adjuvant therapy and experienced disease recurrence in the liver at 15 months from surgery. Genetic testing of the metastasis showed IQGAP-NTRK3 gene fusion. She was given entrectinib but continued to show progression in the liver. Right partial hepatectomy was performed, showing positivity for CD10, BCL-1, MDM2, and SMA on tumor staining. Treatment was switched to pazopanib with disease progression in the neck. She was treated with larotrectinib last, showing no disease progression and adequate tolerance of therapy after 18 months of this treatment. This is the first case in the literature of metastatic PLU with NRTK3 fusion treated with sequential first-generation NRTK inhibitors. More case reports are needed to identify commonalities and therapeutic options. Genetic testing in all PLU cases is needed for targeted therapy approaches.
Anisley Valenciaga, O. Hans Iwenofu, and Gabriel Tinoco
S. Machele Donat
Since the initial report in 2003 of the Intergroup-0080 trial confirming benefit of combined neoadjuvant M-VAC (methotrexate, vinblastine, adriablastine, and cisplatin) chemotherapy and cystectomy in the treatment of muscle-invasive bladder cancer, debate has continued in the literature as to the relative risk/benefits of integrating perioperative chemotherapy into the care of patients, especially in those with organ-confined, muscle-invasive, node-negative disease in whom the benefit may be less. Because of the inaccuracies of clinical staging, the potential morbidity related to M-VAC chemotherapy, a 70% cure rate in pT2No disease with surgery alone, and only a modest (5%) improvement in absolute overall survival with combined therapy, many favor limiting chemotherapy to patients with a pathologic stage of pT3 or greater or node-positive disease. This philosophy was also reflected in the 2008 National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology: Bladder Cancer, in which neoadjuvant chemotherapy for clinical T2 disease versus adjuvant therapy based on pathologic risks is only “considered.” Additionally, a recent study looking at the perioperative integration of chemotherapy for stage III bladder cancer in the United States using the National Cancer Data Base showed that only 11.6% of patients underwent any perioperative chemotherapy, with most in the adjuvant setting. These findings indicate that despite randomized trial data showing survival benefit for perioperative chemotherapy, and the current guidelines for therapy supporting those findings, chemotherapy is not being integrated well into the care of patients with muscle-invasive bladder cancer, even in those who, experts agree, have the most potential for benefit.
Loretta Loftus, Christine Laronga, Karen Coyne, and Lynne Hildreth
Analysis of Moffitt Cancer Center data on time from breast biopsy to first definitive surgery showed an average of 6.9 weeks, which concerned the breast program faculty members. Delays in curative surgery may impact mortality, quality of life, and time to adjuvant therapy. The purpose of this study was to analyze steps from breast biopsy to definitive breast cancer surgery and to develop proposals and strategies for improvement. Data were collected from various sources, including the tumor registry, patient appointment system, tumor board lists, and the NCCN Oncology Outcomes Database for Breast Cancer. Three phases of the surgical process were identified with regard to lead time: biopsy to first consult (BX-FC); first consult to tumor board (FC-TB); and tumor board to surgery (TB-SU). Other factors, including operating room capacity and schedules, were also evaluated. The greatest percentage of total lead time occurred in the TB-SU phase (52% vs 35% in BX-FC, and 13% in FC-TB phases). The longest average lead time, 3.6 weeks, was also in the TB-SU phase. The TB-SU time was greatest when surgery was scheduled after tumor board and if surgery required breast reconstruction. Limitation of physician capacity was a major factor in treatment delay. The Opportunity for Improvement project enabled institutional analysis of the need for quality improvement in time for curative surgery for breast cancer. A significant factor that created time delay was physician capacity. As a result, additional faculty and staff have been recruited. A new expanded facility is currently in progress that will provide more physical space and services.
Philip Sobash and Nagla Abdel Karim
atypical disease and metastatic disease. Aim of the study is to explore the benefit of adjuvant therapy for thoracic carcinoid tumors. Methods: A literature review was conducted through PubMed in accordance with PRISMA guidelines to identify studies
of capecitabine + oxaliplatin was added as an option in adjuvant therapy with a category 2A designation. Footnote “j” was clarified by adding “exclusive of those cancers that are MSI-H” to grade 3-4. Surveillance Chest
Al B. Benson III, Tanios Bekaii-Saab, Emily Chan, Yi-Jen Chen, Michael A. Choti, Harry S. Cooper, Paul F. Engstrom, Peter C. Enzinger, Marwan G. Fakih, Moon J. Fenton, Charles S. Fuchs, Jean L. Grem, Steven Hunt, Ahmed Kamel, Lucille A. Leong, Edward Lin, Kilian Salerno May, Mary F. Mulcahy, Kate Murphy, Eric Rohren, David P. Ryan, Leonard Saltz, Sunil Sharma, David Shibata, John M. Skibber, William Small Jr, Constantinos T. Sofocleous, Alan P. Venook, Christopher G. Willett, Kristina M. Gregory, and Deborah A. Freedman-Cass
rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN®. Adjuvant Therapy for Stage II Disease The panel discussed the impact of adjuvant chemotherapy on
scientific peer-review process and are overseen by the ORP. An NCCN study funded through the grant mechanism is highlighted below. Randomized Phase II Study Comparing Concise (3 Months) Versus Prolonged (2 Years) Afatinib as Adjuvant Therapy for
Tiffany H. Svahn, Joyce C. Niland, Robert W. Carlson, Melissa E. Hughes, Rebecca A. Ottesen, Richard L. Theriault, Stephen B. Edge, Anne F. Schott, Michael A. Bookman, and Jane C. Weeks
. 5 Coates AS Keshaviah A Thurlimann B . Five years of letrozole compared with tamoxifen as initial adjuvant therapy for postmenopausal women with endocrine-responsive early breast cancer: update of study BIG 1-98 . J Clin Oncol 2007 ; 25 : 486
Nora M. Hansen, Sally Anne Scherer, and Seema Khan
of 60 cases, care was nonconcordant in 8. Among those cases, 1 patient was advised to have chemotherapy but refused; another patient’s record did not document a recommendation for adjuvant therapy; and 5 patients received treatment but not within the
margin,” she said. Dr. Tempero briefly reviewed some of the representative adjuvant therapy clinical trials conducted in pancreatic cancer, focusing primarily on the relatively recent phase III ESPAC-4 trial of gemcitabine/capecitabine 1 and the