NCCN Flash Updates: NCCN Guidelines^® and NCCN Compendium ^® Updated for Myeloid/Lymphoid Neoplasms with Eosinophilia and Tyrosine Kinase Fusion Genes and Small Cell Lung Cancer

NCCN has published updates to the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines^®) and the NCCN Drugs and Biologics Compendium (NCCN Compendium^®) for Myeloid/Lymphoid Neoplasms with Eosinophilia and Tyrosine Kinase Fusion Genes. These NCCN Guidelines^® are currently available as Version 2.2021.

• MLNE-3, MLNE-4, MLNE-8 and MLNE-9
  • The following footnote is new to the page, "The differential diagnosis of JAK2 and ABL1 fusions with a phenotype of ALL includes Ph-like ALL."
• Myeloid/lymphoid Neoplasms with Eosinophilia and the FLP1L1-PDGFRA Rearrangement (MLNE-5)
  • The following footnote corresponding to, if resistance mutation found, refer for clinical trial is new to this page. “Avapritinib is approved for adult patients with unresectable or metastatic gastrointestinal stromal tumors (GISTs) harboring a PDGFRA exon 18 mutation, including D842V mutations. This suggests a possible role for avapritinib in patients with FIP1L1-PDGFRA-positive myeloid/lymphoid neoplasms with eosinophilia harboring PDGFRA D842V mutation resistant to imatinib. If this mutation is identified, a clinical trial of avapritinib is preferred (if available), rather than off-label use.”
• Myeloid/lymphoid Neoplasms with Eosinophilia and FGFR1 Rearrangement (MLNE-7)
  • “Pemigatinib” has been added as a TKI with activity against FGFR1 under other recommended regimens.
    • The following footnote corresponding to, “clinical trial” and “pemigatinib” is new to this page. “Pemigatinib (FGFR1, 2, and 3 inhibitor) is approved for the treatment of adult patients with previously treated, unresectable, locally advanced or metastatic cholangiocarcinoma and a FGFR2 fusion or other rearrangement, as detected by an FDA-approved test. Pemigatinib has received orphan drug designation for the treatment of patients with myeloid/lymphoid neoplasms with eosinophilia and FGFR1 rearrangement and is currently being evaluated in a clinical trial for this indication. A clinical trial of pemigatinib is preferred (if available), rather than off-label use.”
• The Discussion has been updated to reflect the changes to the algorithm (MS-1).

Previous updates to the NCCN Guidelines for Myeloid/Lymphoid Neoplasms with Eosinophilia and Tyrosine Kinase Fusion Genes can be found in the UPDATES section of the current version.

NCCN has published updates to the NCCN Guidelines, the NCCN Compendium^®, the NCCN Radiation Therapy Compendium™ , and the NCCN Imaging Appropriate Use Criteria (NCCN Imaging AUC™) for Small Cell Lung Cancer. These NCCN Guidelines are currently available as Version 1.2021.

• Principles of Pathologic Review (SCL-B, 1 of 2)
  • Bullet was added under Pathologic Evaluation: SCLC is often associated with necrosis. However, necrosis, usually punctate, is also seen in atypical carcinoid tumors. Counting mitotic figures helps to distinguish these two entities.
• Principles of Surgical Resection (SCL-C)
  • Bullet was added: Surgery may be considered for selected patients with T3 (based on size), N0 SCLC, if invasive mediastinal lymph node staging is negative.
• Principles of Systemic Therapy (SCL-E, 1 of 5)
  • Primary or Adjuvant Therapy for Limited Stage SCLC
    • Dosing statement was revised: Maximum of 4-6 cycles Four cycles of systemic therapy are recommended.
  • Extensive-Stage chart heading was revised: Primary or Adjuvant Therapy for Extensive-Stage SCLC
    • Dosing statement was revised: Maximum of 4–6 cycles Four cycles of therapy are recommended, but some patients may receive up to 6 cycles based on response and tolerability after 4 cycles.
  • Footnote b was removed: Regimen not recommended for relapsed disease in patients on maintenance atezolizumab or durvalumab at time of relapse. For patients who relapse after >6 months of atezolizumab or durvalumab maintenance therapy, recommend re-treatment with carboplatin + etoposide alone or cisplatin + etoposide alone.
• Principles of Systemic Therapy (SCL-E, 2 of 5)
  • SCLC Subsequent Systemic Therapy
    • Regimen listed under Other Recommended Regimens was revised: Nivolumab ± ipilimumab
    • Footnote b was revised: Regimen not recommended for relapsed disease in patients on maintenance atezolizumab or durvalumab at time of relapse. For patients who relapse after >6 months of atezolizumab or durvalumab maintenance therapy, recommend re-treatment with carboplatin + etoposide alone or cisplatin + etoposide alone.
• Principles of Systemic Therapy (SCL-E, 3 of 5)
  • Bullet was added under Response Assessment: Response assessment after adjuvant therapy involves chest/abdomen/pelvic CT with contrast and brain MRI (preferred) with contrast or brain CT with contrast (see SCL-6).
• Principles of Systemic Therapy (SCL-E, 4 of 5)
  • Reference 18 was revised: Ready NE, Ott PA, Hellmann MD, et al. Nivolumab monotherapy and nivolumab plus ipilimumab in recurrent small cell lung cancer: results from the CheckMate 032 randomized cohort. J Thorac Oncol 2020;15:426-435.
• Principles of Radiation Therapy (SCL-F, 2 of 5)
  • Bullet was added under Extensive stage: Based on two randomized trials, immunotherapy during and after chemotherapy is a first-line approach, but these studies did not include consolidative thoracic RT. Nevertheless, consolidative thoracic RT after chemoimmunotherapy can be considered for selected patients as above, during or before maintenance immunotherapy (there are no data on optimal sequencing or safety).
• Principles of Radiation Therapy (SCL-F 3 of 5)
  • Two bullets were added under Prophylactic Cranial Irradiation:
    • Consider hippocampal-sparing PCI using IMRT.
    • Current randomized trials are evaluating whether MRI surveillance alone is non-inferior to MRI surveillance plus PCI on overall survival and whether hippocampal-sparing PCI reduces memory impairment compared to whole brain PCI in LS-SCLC and ES-SCLC.
  • Bullet was revised under Brain Metastases: For patients with a better prognosis (eg, ≥4 months), consider hippocampal-sparing WBRT brain RT using IMRT plus memantine is preferred because it produces less cognitive function failure than conventional brain RT plus memantine.

For the complete updated versions of the NCCN Guidelines, NCCN Guidelines with NCCN Evidence Blocks™, the NCCN Drugs & Biologics Compendium (NCCN Compendium^®), the NCCN Biomarkers Compendium^®, the NCCN Chemotherapy Order Templates (NCCN Templates^®), the NCCN Radiation Therapy Compendium™, and the NCCN Imaging Appropriate Use Criteria (NCCN Imaging AUC™), please visit NCCN.org.

To view the NCCN Guidelines for Patients^®, please visit NCCN.org/patientguidelines.

Free NCCN Guidelines apps for iPhone, iPad, and Android devices are now available! Visit NCCN.org/apps.

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