Soft tissue sarcomas comprise multiple histologic subtypes, occur at a number of anatomic sites, and require individualized treatment. Over the past 5 years, 4 new drugs were approved for sarcoma, most of which are driven by histology or the anticipated response to treatment. Surgical resection remains the primary treatment for resectable tumors. For unresectable or metastatic disease, doxorubicin remains the backbone of chemotherapy, but other agents have improved on its single-agent efficacy. Chief among them is olaratumab, which, in combination with doxorubicin, is preferred over doxorubicin alone in the updated NCCN Clinical Practice Guidelines in Oncology for Soft Tissue Sarcoma.
Evolving Treatment of Soft Tissue Sarcoma
Developments in Systemic Therapy for Soft Tissue and Bone Sarcomas
The past year has led to significant changes in systemic therapies used to treat soft tissue sarcomas, mainly dominated by the removal of the recently approved drug olaratumab as part of combination therapy with doxorubicin from the NCCN Guidelines for Soft Tissue Sarcoma, according to Dr. Suzanne George. Several histology-specific drugs have entered the space, including pazopanib and pembrolizumab, the latter of which was approved as a category 2B recommendation for alveolar soft part sarcoma, highlighting the rather limited role of immunotherapy in sarcomas. Dr. George also discussed updated data for sorafenib in the treatment of desmoid tumors, as well as the importance of larotrectinib in TRK fusion–positive tumors.
Barriers to Treatment in Patients With Locally Advanced Breast Cancer
Laura Bourdeanu, Thehang Luu, Norma Baker, Suzanne Swain-Cabriales, Cathie T. Chung, Joanne Mortimer, Arti Hurria, Sandra Helton, David Smith, Betty Ferrell, Gloria Juarez, and George Somlo
Delays between presentation and treatment could have a significant effect on breast cancer mortality. The authors hypothesized that patient, physician, and system barriers are all responsible for treatment delays. Therefore, a study was conducted to define prevalent barriers to treatment from the patient’s perspective. A modified 43-item Likert-scale questionnaire was administered to patients with clinical stage III locally advanced breast cancer (LABC) who had experienced a delay in treatment of 3 months or more. Between October 2008 and January 2010, 153 patients presented with LABC; 43 patients (28.1%) met eligibility, and 40 completed the questionnaire. Among the patient barriers reported, 38% of patients delayed care for fear of losing their breast and 47% awaited previously scheduled routine appointments instead of seeking care. Among the physician barriers reported, 20% of physicians of initial contact did not believe the breast lump/symptom was related to cancer and 15% did not believe it needed a biopsy. Among the system barriers reported, the most prevalent were delays in performing diagnostic tests and obtaining insurance authorization for tests, treatment, or physician visits. Substantial delays were seen in 28.1% of patients from presentation to when they sought therapy at City of Hope Comprehensive Cancer Center. The high prevalence of patient barriers versus physician/system barriers suggests that increased educational efforts for patients and health care professionals are needed.
NCCN Guidelines Insights: Soft Tissue Sarcoma, Version 1.2021
Featured Updates to the NCCN Guidelines
Margaret von Mehren, John M. Kane III, Marilyn M. Bui, Edwin Choy, Mary Connelly, Sarah Dry, Kristen N. Ganjoo, Suzanne George, Ricardo J. Gonzalez, Martin J. Heslin, Jade Homsi, Vicki Keedy, Ciara M. Kelly, Edward Kim, David Liebner, Martin McCarter, Sean V. McGarry, Christian Meyer, Alberto S. Pappo, Amanda M. Parkes, I. Benjamin Paz, Ivy A. Petersen, Matthew Poppe, Richard F. Riedel, Brian Rubin, Scott Schuetze, Jacob Shabason, Jason K. Sicklick, Matthew B. Spraker, Melissa Zimel, Mary Anne Bergman, and Giby V. George
The NCCN Guidelines for Soft Tissue Sarcoma provide recommendations for the diagnosis, evaluation, treatment, and follow-up for patients with soft tissue sarcomas. These NCCN Guidelines Insights summarize the panel discussion behind recent important updates to the guidelines, including the development of a separate and distinct guideline for gastrointestinal stromal tumors (GISTs); reconception of the management of desmoid tumors; inclusion of further recommendations for the diagnosis and management of extremity/body wall, head/neck sarcomas, and retroperitoneal sarcomas; modification and addition of systemic therapy regimens for sarcoma subtypes; and revision of the principles of radiation therapy for soft tissue sarcomas.
Soft Tissue Sarcoma, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology
Margaret von Mehren, R. Lor Randall, Robert S. Benjamin, Sarah Boles, Marilyn M. Bui, Kristen N. Ganjoo, Suzanne George, Ricardo J. Gonzalez, Martin J. Heslin, John M. Kane III, Vicki Keedy, Edward Kim, Henry Koon, Joel Mayerson, Martin McCarter, Sean V. McGarry, Christian Meyer, Zachary S. Morris, Richard J. O'Donnell, Alberto S. Pappo, I. Benjamin Paz, Ivy A. Petersen, John D. Pfeifer, Richard F. Riedel, Bernice Ruo, Scott Schuetze, William D. Tap, Jeffrey D. Wayne, Mary Anne Bergman, and Jillian L. Scavone
Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for STS provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumors, desmoid tumors, and rhabdomyosarcoma. This portion of the NCCN Guidelines discusses general principles for the diagnosis, staging, and treatment of STS of the extremities, superficial trunk, or head and neck; outlines treatment recommendations by disease stage; and reviews the evidence to support the guidelines recommendations.
Uterine Neoplasms, Version 1.2014
Wui-Jin Koh, Benjamin E. Greer, Nadeem R. Abu-Rustum, Sachin M. Apte, Susana M. Campos, John Chan, Kathleen R. Cho, David Cohn, Marta Ann Crispens, Nefertiti DuPont, Patricia J. Eifel, Amanda Nickles Fader, Christine M. Fisher, David K. Gaffney, Suzanne George, Ernest Han, Warner K. Huh, John R. Lurain III, Lainie Martin, David Mutch, Steven W. Remmenga, R. Kevin Reynolds, William Small Jr, Nelson Teng, Todd Tillmanns, Fidel A. Valea, Nicole McMillian, and Miranda Hughes
Adenocarcinoma of the endometrium (also known as endometrial cancer or more broadly as uterine cancer or carcinoma of the uterine corpus) is the most common malignancy of the female genital tract in the United States. An estimated 49,560 new uterine cancer cases will occur in 2013, with 8190 deaths resulting from the disease. Uterine sarcomas (stromal/mesenchymal tumors) are uncommon malignancies, accounting for approximately 3% of all uterine cancers. The NCCN Guidelines for Uterine Neoplasms describe malignant epithelial carcinomas and uterine sarcomas; each of these major categories contains specific histologic groups that require different management. This excerpt of these guidelines focuses on early-stage disease.
Adolescent and Young Adult Oncology
Peter F. Coccia, Jessica Altman, Smita Bhatia, Scott C. Borinstein, Joseph Flynn, Suzanne George, Robert Goldsby, Robert Hayashi, Mary S. Huang, Rebecca H. Johnson, Lynda Kwon Beaupin, Michael P. Link, Kevin C. Oeffinger, Kathleen M. Orr, Alberto S. Pappo, Damon Reed, Holly L. Spraker, Deborah A. Thomas, Margaret von Mehren, Daniel S. Wechsler, Kimberly F. Whelan, Bradley J. Zebrack, Hema Sundar, and Dorothy A. Shead
Cancer is the leading cause of death among the adolescent and young adult (AYA) population, excluding homicide, suicide, or unintentional injury. AYA patients should be managed by a multidisciplinary team of health care professionals who are well-versed in the specific developmental issues relevant to this patient population. The recommendations for age-appropriate care outlined in these NCCN Guidelines include psychosocial assessment, a discussion of infertility risks associated with treatment and options for fertility preservation, genetic and familial risk assessment for all patients after diagnosis, screening and monitoring of late effects in AYA cancer survivors after successful completion of therapy, and palliative care and end-of-life considerations for patients for whom curative therapy fails.
Adolescent and Young Adult Oncology, Version 2.2014
Peter F. Coccia, Alberto S. Pappo, Jessica Altman, Smita Bhatia, Scott C. Borinstein, Joseph Flynn, A. Lindsay Frazier, Suzanne George, Robert Goldsby, Robert Hayashi, Mary S. Huang, Rebecca H. Johnson, Lynda Kwon Beaupin, Michael P. Link, Kevin C. Oeffinger, Kathleen M. Orr, Damon Reed, Holly L. Spraker, Deborah A. Thomas, Margaret von Mehren, Daniel S. Wechsler, Kimberly F. Whelan, Brad Zebrack, Dorothy A. Shead, and Hema Sundar
The NCCN Guidelines Insights on Adolescent and Young Adult (AYA) Oncology discuss the fertility and endocrine issues that are relevant to the management of AYA patients with cancer. Fertility preservation should be an essential part in the treatment of AYA patients with cancer. The NCCN Guidelines recommend discussion of fertility preservation and contraception before the start of treatment. Oophoropexy and embryo cryopreservation are the 2 established options for fertility preservation in women. Semen cryopreservation before the start of treatment is the most reliable and well-established method of preserving fertility in men. AYA women with cancer also have unique contraception needs, depending on the type of cancer, its treatment, and treatment-related complications. Management of cancer during pregnancy poses significant diagnostic and therapeutic challenges for both the patient and the physician. AYA women diagnosed with cancer during pregnancy require individualized treatment from a multidisciplinary team involving medical, surgical, radiation, and gynecologic oncologists; obstetricians; and perinatologists.
J. Sybil Biermann, Douglas R. Adkins, Robert S. Benjamin, Brian Brigman, Warren Chow, Ernest U. Conrad III, Deborah A. Frassica, Frank J. Frassica, Suzanne George, Kenneth R. Hande, Francis J. Hornicek, G. Douglas Letson, Joel Mayerson, Sean V. McGarry, Brian McGrath, Carol D. Morris, Richard J. O'Donnell, R. Lor Randall, Victor M. Santana, Robert L. Satcher, Herrick J. Siegel, Neeta Somaiah, and Alan W. Yasko
Soft Tissue Sarcoma, Version 2.2016, NCCN Clinical Practice Guidelines in Oncology
Margaret von Mehren, R. Lor Randall, Robert S. Benjamin, Sarah Boles, Marilyn M. Bui, Ernest U. Conrad III, Kristen N. Ganjoo, Suzanne George, Ricardo J. Gonzalez, Martin J. Heslin, John M. Kane III, Henry Koon, Joel Mayerson, Martin McCarter, Sean V. McGarry, Christian Meyer, Richard J. O'Donnell, Alberto S. Pappo, I. Benjamin Paz, Ivy A. Petersen, John D. Pfeifer, Richard F. Riedel, Scott Schuetze, Karen D. Schupak, Herbert S. Schwartz, William D. Tap, Jeffrey D. Wayne, Mary Anne Bergman, and Jillian Scavone
Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for Soft Tissue Sarcoma (available at NCCN.org) provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumor, desmoid tumors, and rhabdomyosarcoma. This manuscript discusses guiding principles for the diagnosis and staging of STS and evidence for treatment modalities that include surgery, radiation, chemoradiation, chemotherapy, and targeted therapy.