ABSTRACT
Mastocytosis is a heterogeneous group of disorders comprising cutaneous mastocytosis, systemic mastocytosis, and mast cell sarcoma. It is associated with a variety of symptoms related to the release of mast cell mediators and mast cell tissue infiltration. Referral to specialized centers with expertise in the management of mastocytosis and multidisciplinary collaboration with subspecialists (eg, allergists for the management of anaphylaxis and drug hypersensitivities, anesthesiologists for invasive procedures or surgery, high-risk obstetrician for pregnancy) is recommended. The NCCN Guidelines for Systemic Mastocytosis provide evidence- and consensus-based recommendations for the diagnosis and comprehensive care of patients with systemic mastocytosis. The multidisciplinary panel of experts convenes at least once a year to review requested changes to the guidelines from both internal and external entities as well as to discuss data on existing and new therapies. These NCCN Guidelines Insights focus on some of the recent updates to the guidelines.
Subject(s)
Mastocytosis, Systemic , Humans , Mastocytosis, Systemic/diagnosis , Mastocytosis, Systemic/therapy , Disease Management , Medical Oncology/standards , Medical Oncology/methodsABSTRACT
The NCCN Guidelines for Breast Cancer Screening and Diagnosis provide health care providers with a practical, consistent framework for screening and evaluating a spectrum of clinical presentations and breast lesions. The NCCN Breast Cancer Screening and Diagnosis Panel is composed of a multidisciplinary team of experts in the field, including representation from medical oncology, gynecologic oncology, surgical oncology, internal medicine, family practice, preventive medicine, pathology, diagnostic and interventional radiology, as well as patient advocacy. The NCCN Breast Cancer Screening and Diagnosis Panel meets at least annually to review emerging data and comments from reviewers within their institutions to guide updates to existing recommendations. These NCCN Guidelines Insights summarize the panel's decision-making and discussion surrounding the most recent updates to the guideline's screening recommendations.
Subject(s)
Breast Neoplasms , Early Detection of Cancer , Humans , Female , Breast Neoplasms/diagnosis , Family Practice , Health Personnel , Medical OncologyABSTRACT
The NCCN Guidelines for Central Nervous System (CNS) Cancers focus on management of the following adult CNS cancers: glioma (WHO grade 1, WHO grade 2-3 oligodendroglioma [1p19q codeleted, IDH-mutant], WHO grade 2-4 IDH-mutant astrocytoma, WHO grade 4 glioblastoma), intracranial and spinal ependymomas, medulloblastoma, limited and extensive brain metastases, leptomeningeal metastases, non-AIDS-related primary CNS lymphomas, metastatic spine tumors, meningiomas, and primary spinal cord tumors. The information contained in the algorithms and principles of management sections in the NCCN Guidelines for CNS Cancers are designed to help clinicians navigate through the complex management of patients with CNS tumors. Several important principles guide surgical management and treatment with radiotherapy and systemic therapy for adults with brain tumors. The NCCN CNS Cancers Panel meets at least annually to review comments from reviewers within their institutions, examine relevant new data from publications and abstracts, and reevaluate and update their recommendations. These NCCN Guidelines Insights summarize the panel's most recent recommendations regarding molecular profiling of gliomas.
Subject(s)
Brain Neoplasms , Central Nervous System Neoplasms , Adult , Humans , Central Nervous System Neoplasms/diagnosis , Central Nervous System Neoplasms/therapy , Brain Neoplasms/diagnosis , Brain Neoplasms/genetics , Brain Neoplasms/therapy , Central Nervous System , MutationABSTRACT
Gastrointestinal stromal tumors (GIST) are the most common type of soft tissue sarcoma that occur throughout the gastrointestinal tract. Most of these tumors are caused by oncogenic activating mutations in the KIT or PDGFRA genes. The NCCN Guidelines for GIST provide recommendations for the diagnosis, evaluation, treatment, and follow-up of patients with these tumors. These NCCN Guidelines Insights summarize the panel discussion behind recent important updates to the guidelines, including revised systemic therapy options for unresectable, progressive, or metastatic GIST based on mutational status, and updated recommendations for the management of GIST that develop resistance to specific tyrosine kinase inhibitors.
Subject(s)
Gastrointestinal Stromal Tumors , Humans , Gastrointestinal Stromal Tumors/diagnosis , Gastrointestinal Stromal Tumors/genetics , Gastrointestinal Stromal Tumors/therapy , Receptor, Platelet-Derived Growth Factor alpha/genetics , Proto-Oncogene Proteins c-kit/genetics , MutationABSTRACT
The classic Philadelphia chromosome-negative myeloproliferative neoplasms (MPN) consist of myelofibrosis, polycythemia vera, and essential thrombocythemia and are a heterogeneous group of clonal blood disorders characterized by an overproduction of blood cells. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for MPN were developed as a result of meetings convened by a multidisciplinary panel with expertise in MPN, with the goal of providing recommendations for the management of MPN in adults. The Guidelines include recommendations for the diagnostic workup, risk stratification, treatment, and supportive care strategies for the management of myelofibrosis, polycythemia vera, and essential thrombocythemia. Assessment of symptoms at baseline and monitoring of symptom status during the course of treatment is recommended for all patients. This article focuses on the recommendations as outlined in the NCCN Guidelines for the diagnosis of MPN and the risk stratification, management, and supportive care relevant to MF.
Subject(s)
Myeloproliferative Disorders , Polycythemia Vera , Primary Myelofibrosis , Thrombocythemia, Essential , Adult , Humans , Medical Oncology , Myeloproliferative Disorders/diagnosis , Myeloproliferative Disorders/therapy , Polycythemia Vera/diagnosis , Primary Myelofibrosis/diagnosis , Thrombocythemia, Essential/diagnosisABSTRACT
Soft tissue sarcomas (STS) are rare malignancies 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 Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Soft Tissue Sarcoma provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as retroperitoneal/intra-abdominal STS, desmoid tumors, and rhabdomyosarcoma. This portion of the NCCN Guidelines discusses general principles for the diagnosis and treatment of retroperitoneal/intra-abdominal STS, outlines treatment recommendations, and reviews the evidence to support the guidelines recommendations.
Subject(s)
Sarcoma , Soft Tissue Neoplasms , Extremities/pathology , Humans , Medical Oncology , Sarcoma/drug therapy , Sarcoma/therapy , Soft Tissue Neoplasms/diagnosis , Soft Tissue Neoplasms/pathology , Soft Tissue Neoplasms/therapyABSTRACT
Histiocytic neoplasms are rare hematologic disorders accounting for less than 1% of cancers of the soft tissue and lymph nodes. Clinical presentation and prognosis of these disorders can be highly variable, leading to challenges for diagnosis and optimal management of these patients. Treatment often consists of systemic therapy, and recent studies support use of targeted therapies for patients with these disorders. Observation ("watch and wait") may be sufficient for select patients with mild disease. These NCCN Guidelines for Histiocytic Neoplasms include recommendations for diagnosis and treatment of adults with the most common histiocytic disorders: Langerhans cell histiocytosis, Erdheim-Chester disease, and Rosai-Dorfman disease.
Subject(s)
Erdheim-Chester Disease , Hematologic Neoplasms , Histiocytosis, Langerhans-Cell , Histiocytosis, Sinus , Adult , Erdheim-Chester Disease/drug therapy , Histiocytosis, Langerhans-Cell/diagnosis , Histiocytosis, Langerhans-Cell/drug therapy , Histiocytosis, Langerhans-Cell/pathology , Histiocytosis, Sinus/diagnosis , Histiocytosis, Sinus/drug therapy , Histiocytosis, Sinus/pathology , Humans , PrognosisABSTRACT
Palliative care has evolved to be an integral part of comprehensive cancer care with the goal of early intervention to improve quality of life and patient outcomes. The NCCN Guidelines for Palliative Care provide recommendations to help the primary oncology team promote the best quality of life possible throughout the illness trajectory for each patient with cancer. The NCCN Palliative Care Panel meets annually to evaluate and update recommendations based on panel members' clinical expertise and emerging scientific data. These NCCN Guidelines Insights summarize the panel's recent discussions and highlights updates on the importance of fostering adaptive coping strategies for patients and families, and on the role of pharmacologic and nonpharmacologic interventions to optimize symptom management.
Subject(s)
Neoplasms , Palliative Care , Humans , Medical Oncology , Neoplasms/therapy , Quality of LifeABSTRACT
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.
Subject(s)
Sarcoma , Soft Tissue Neoplasms , Extremities , Gastrointestinal Stromal Tumors , Humans , Practice Guidelines as Topic , Retroperitoneal Neoplasms , Sarcoma/diagnosis , Sarcoma/therapy , Soft Tissue Neoplasms/diagnosis , Soft Tissue Neoplasms/therapyABSTRACT
The NCCN Guidelines for Central Nervous System (CNS) Cancers focus on management of adult CNS cancers ranging from noninvasive and surgically curable pilocytic astrocytomas to metastatic brain disease. The involvement of an interdisciplinary team, including neurosurgeons, radiation therapists, oncologists, neurologists, and neuroradiologists, is a key factor in the appropriate management of CNS cancers. Integrated histopathologic and molecular characterization of brain tumors such as gliomas should be standard practice. This article describes NCCN Guidelines recommendations for WHO grade I, II, III, and IV gliomas. Treatment of brain metastases, the most common intracranial tumors in adults, is also described.
Subject(s)
Astrocytoma , Brain Neoplasms , Central Nervous System Neoplasms , Glioma , Adult , Astrocytoma/diagnosis , Astrocytoma/therapy , Brain Neoplasms/diagnosis , Brain Neoplasms/therapy , Central Nervous System , Central Nervous System Neoplasms/diagnosis , Central Nervous System Neoplasms/therapy , Glioma/diagnosis , Glioma/therapy , Humans , Practice Guidelines as TopicABSTRACT
Eosinophilic disorders and related syndromes represent a heterogeneous group of neoplastic and nonneoplastic conditions, characterized by more eosinophils in the peripheral blood, and may involve eosinophil-induced organ damage. In the WHO classification of myeloid and lymphoid neoplasms, eosinophilic disorders characterized by dysregulated tyrosine kinase (TK) fusion genes are recognized as a new category termed, myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB or FGFR1 or with PCM1-JAK2. In addition to these aforementioned TK fusion genes, rearrangements involving FLT3 and ABL1 genes have also been described. These new NCCN Guidelines include recommendations for the diagnosis, staging, and treatment of any one of the myeloid/lymphoid neoplasms with eosinophilia (MLN-Eo) and a TK fusion gene included in the 2017 WHO Classification, as well as MLN-Eo and a FLT3 or ABL1 rearrangement.
Subject(s)
Eosinophilia , Myeloproliferative Disorders , Neoplasms , Eosinophilia/diagnosis , Eosinophilia/genetics , Humans , Myeloproliferative Disorders/diagnosis , Myeloproliferative Disorders/genetics , Myeloproliferative Disorders/therapy , Oncogene Proteins, Fusion/geneticsABSTRACT
Mastocytosis is a group of heterogeneous disorders resulting from the clonal proliferation of abnormal mast cells and their accumulation in the skin and/or in various extracutaneous organs. Systemic mastocytosis is the most common form of mastocytosis diagnosed in adults, characterized by mast cell infiltration of one or more extracutaneous organs (with or without skin involvement). The identification of KIT D816V mutation and the emergence of novel targeted therapies have significantly improved the diagnosis and treatment of systemic mastocytosis. However, certain aspects of clinical care, particularly the diagnosis, assessment, and management of mediator-related symptoms continue to present challenges. This manuscript discusses the recommendations outlined in the NCCN Guidelines for the diagnosis and management of patients with systemic mastocytosis.
Subject(s)
Anaphylaxis/therapy , Mastocytosis, Systemic/therapy , Medical Oncology/standards , Patient Care Team/standards , Anaphylaxis/diagnosis , Anaphylaxis/immunology , Antineoplastic Agents/pharmacology , Antineoplastic Agents/therapeutic use , Biomarkers, Tumor/blood , Biomarkers, Tumor/genetics , Biopsy , Bone Marrow/drug effects , Bone Marrow/pathology , Hematopoietic Stem Cell Transplantation/methods , Hematopoietic Stem Cell Transplantation/standards , Histamine Antagonists/pharmacology , Histamine Antagonists/therapeutic use , Humans , Immunophenotyping/methods , Immunophenotyping/standards , Mast Cells/drug effects , Mast Cells/immunology , Mast Cells/metabolism , Mastocytosis, Systemic/diagnosis , Mastocytosis, Systemic/genetics , Mastocytosis, Systemic/immunology , Medical Oncology/methods , Molecular Targeted Therapy/methods , Molecular Targeted Therapy/standards , Mutation , Oncogene Proteins, Fusion/genetics , Proto-Oncogene Proteins c-kit/genetics , Receptor, Platelet-Derived Growth Factor alpha/genetics , Societies, Medical/standards , Transplantation, Homologous/methods , Transplantation, Homologous/standards , Treatment Outcome , mRNA Cleavage and Polyadenylation Factors/geneticsABSTRACT
The NCCN Guidelines for Breast Cancer Screening and Diagnosis have been developed to facilitate clinical decision making. This manuscript discusses the diagnostic evaluation of individuals with suspected breast cancer due to either abnormal imaging and/or physical findings. For breast cancer screening recommendations, please see the full guidelines on NCCN.org.
Subject(s)
Breast Neoplasms/diagnosis , Early Detection of Cancer/standards , Mass Screening/standards , Medical Oncology/standards , Adult , Age Factors , Biopsy/methods , Biopsy/standards , Breast/diagnostic imaging , Breast/pathology , Breast Neoplasms/epidemiology , Breast Neoplasms/pathology , Breast Neoplasms/prevention & control , Clinical Decision-Making/methods , Early Detection of Cancer/methods , Female , Humans , Incidence , Mammography/methods , Mammography/standards , Mass Screening/methods , Medical Oncology/methods , Middle Aged , Societies, Medical/standards , United States/epidemiologyABSTRACT
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.
Subject(s)
Guidelines as Topic/standards , Medical Oncology/methods , Sarcoma/diagnosis , HumansABSTRACT
The NCCN Guidelines for Palliative Care provide interdisciplinary recommendations on palliative care for patients with cancer. These NCCN Guidelines Insights summarize and provide context for the updated guidelines recommendations regarding hospice and end-of-life (EOL) care. Updates for 2017 include revisions to and restructuring of the algorithms that address important EOL concerns. These recommendations were revised to provide clearer guidance for oncologists as they care for patients with cancer who are approaching the transition to EOL care. Recommendations for interventions and reassessment based on estimated life expectancy were streamlined and reprioritized to promote hospice referrals and improved EOL care.
Subject(s)
Neoplasms/therapy , Palliative Care , Hospice Care/methods , Humans , Palliative Care/methods , Terminal Care/methodsABSTRACT
Myeloproliferative neoplasms (MPNs) are a group of heterogeneous disorders of the hematopoietic system that include myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET). PV and ET are characterized by significant thrombohemorrhagic complications and a high risk of transformation to MF and acute myeloid leukemia. The diagnosis and management of PV and ET has evolved since the identification of mutations implicated in their pathogenesis. These NCCN Guideline Insights discuss the recommendations outlined in the NCCN Guidelines for the risk stratification, treatment, and special considerations for the management of PV and ET.
Subject(s)
Myeloproliferative Disorders/diagnosis , Myeloproliferative Disorders/therapy , Combined Modality Therapy/adverse effects , Combined Modality Therapy/methods , Humans , Risk Assessment , Treatment OutcomeABSTRACT
The NCCN Guidelines for Bone Cancer provide interdisciplinary recommendations for treating chordoma, chondrosarcoma, giant cell tumor of bone, Ewing sarcoma, and osteosarcoma. These NCCN Guidelines Insights summarize the NCCN Bone Cancer Panel's guideline recommendations for treating Ewing sarcoma. The data underlying these treatment recommendations are also discussed.
Subject(s)
Antineoplastic Agents/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Bone Neoplasms/therapy , Neoplasm Recurrence, Local/therapy , Sarcoma, Ewing/therapy , Amputation, Surgical , Biopsy , Bone Neoplasms/epidemiology , Bone Neoplasms/pathology , Chemoradiotherapy, Adjuvant/standards , Chemotherapy, Adjuvant/standards , Clinical Trials as Topic , Drug Resistance, Neoplasm , Humans , Incidence , Magnetic Resonance Imaging , Medical Oncology/standards , Neoplasm Recurrence, Local/diagnosis , Neoplasm Recurrence, Local/pathology , Neoplasm Staging , Positron Emission Tomography Computed Tomography , Practice Guidelines as Topic , Prognosis , Sarcoma, Ewing/epidemiology , Sarcoma, Ewing/pathology , Survival RateABSTRACT
For many years, the diagnosis and classification of gliomas have been based on histology. Although studies including large populations of patients demonstrated the prognostic value of histologic phenotype, variability in outcomes within histologic groups limited the utility of this system. Nonetheless, histology was the only proven and widely accessible tool available at the time, thus it was used for clinical trial entry criteria, and therefore determined the recommended treatment options. Research to identify molecular changes that underlie glioma progression has led to the discovery of molecular features that have greater diagnostic and prognostic value than histology. Analyses of these molecular markers across populations from randomized clinical trials have shown that some of these markers are also predictive of response to specific types of treatment, which has prompted significant changes to the recommended treatment options for grade III (anaplastic) gliomas.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biomarkers, Tumor/analysis , Central Nervous System Neoplasms/diagnosis , Glioma/diagnosis , Nervous System/pathology , Antineoplastic Combined Chemotherapy Protocols/standards , Central Nervous System Neoplasms/classification , Central Nervous System Neoplasms/pathology , Central Nervous System Neoplasms/therapy , Combined Modality Therapy/methods , Combined Modality Therapy/standards , Glioma/classification , Glioma/pathology , Glioma/therapy , Humans , Neoadjuvant Therapy/methods , Neoadjuvant Therapy/standards , Neoplasm Grading , Prognosis , Radiotherapy/methods , Radiotherapy/standardsABSTRACT
Myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET) are a group of heterogeneous disorders of the hematopoietic system collectively known as Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs). The diagnosis and the management of patients with MPNs have evolved since the identification of mutations that activate the JAK pathway (JAK2, CALR, and MPL mutations) and the development of targeted therapies has resulted in significant improvements in disease-related symptoms and quality of life. This manuscript discusses the recommendations outlined in the NCCN Guidelines for the diagnostic workup of MPN (MF, PV, and ET), risk stratification, treatment, and supportive care strategies for the management of MF.
Subject(s)
Medical Oncology/standards , Polycythemia Vera , Primary Myelofibrosis , Thrombocythemia, Essential , Calreticulin/genetics , Calreticulin/metabolism , Humans , Janus Kinase 2/genetics , Janus Kinase 2/metabolism , Mutation , Philadelphia Chromosome , Polycythemia Vera/diagnosis , Polycythemia Vera/epidemiology , Polycythemia Vera/genetics , Polycythemia Vera/therapy , Prevalence , Primary Myelofibrosis/diagnosis , Primary Myelofibrosis/epidemiology , Primary Myelofibrosis/genetics , Primary Myelofibrosis/therapy , Quality of Life , Receptors, Thrombopoietin/genetics , Receptors, Thrombopoietin/metabolism , Risk Assessment , Signal Transduction , Thrombocythemia, Essential/diagnosis , Thrombocythemia, Essential/epidemiology , Thrombocythemia, Essential/genetics , Thrombocythemia, Essential/therapyABSTRACT
The NCCN Guidelines for Palliative Care provide interdisciplinary recommendations on palliative care for patients with cancer. The NCCN Guidelines are intended to provide guidance to the primary oncology team on the integration of palliative care into oncology. The NCCN Palliative Care Panel's recommendations seek to ensure that each patient experiences the best quality of life possible throughout the illness trajectory. Accordingly, the NCCN Guidelines outline best practices for screening, assessment, palliative care interventions, reassessment, and after-death care.