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Revised International Neuroblastoma Response Criteria (INRC)
Source: Neuroblastoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 28, 2025 · Captured 2026-09-09.
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Context: Treatment Option Overview for Neuroblastoma
INRC is used to assess response to treatment.[34-36] Overall response in the revised INRC integrates tumor response in three components: primary tumor, soft tissue and bone metastases, and bone marrow. Primary and metastatic soft tissue sites are assessed using Response Evaluation Criteria in Solid Tumors (RECIST) and iodine I 123 (123I) MIBG scans or fluorine F 18-fludeoxyglucose (18F-FDG) positron emission tomography (PET) scans if the tumor is MIBG nonavid. 123I-MIBG scans, or 18F-FDG PET scans for MIBG-nonavid disease, replaced Technetium Tc 99m (99mTc) diphosphonate bone scintigraphy for osteomedullary metastasis assessment. Bone marrow is assessed by histology with or without immunohistochemistry and cytology or immunocytology. Bone marrow with 5% or less tumor involvement is classified as minimal disease. Urinary catecholamine levels are not included in response assessment. Overall response is defined as complete response, partial response, minor response, stable disease, or progressive disease.[36]
Complete Response: No evidence of disease, including resolution of MIBG uptake (or PET scan positivity in MIBG non-avid disease) in any location of soft tissue or bone, with less than 10 mm remaining on 3-D imaging of primary tumor; target lymph nodes less than 15 mm in short dimension; and no histological tumor in two bone marrow biopsies and two bone marrow aspirates sampled at one time point.
Partial Response: 30% or more decrease in longest diameter of primary site and at least 30% decrease in sum of diameters of nonprimary soft tissue metastases (soft tissue sites may still be avid); and at least a 50% reduction in absolute MIBG bone score or a 50% or greater reduction in number of 18F-FDG PET-avid bone lesions; and bone marrow with 0% to 5% tumor; and no new lesions.
Minor Response: Partial response or complete response of at least one component of disease, but at least one other component with stable disease and no component with progressive disease.
Progressive Disease: Any new lesion; increase in longest diameter in any measurable soft tissue lesion by 20% with at least 5 mm absolute increase; previous negative bone marrow now positive for at least 5% tumor or previous positive bone marrow that increases twofold in tumor percentage and increases to more than 20% tumor; any new soft tissue lesion that is MIBG (or 18F-FDG PET) avid or positive by biopsy; a new avid bone site; or increase in relative MIBG score to at least 1.2.
Stable Disease: Stable disease in one component with no better than stable disease or no involvement at other two components. No components meet criteria for progressive disease or partial response.
Care should be taken in interpreting the development of metastatic disease in an infant who was initially considered to have stage 1 or 2 disease. If the pattern of metastases in such a patient is consistent with a 4S pattern of disease (involvement of skin, liver, and/or bone marrow, the latter less than 10% involved), these patients are not classified as having progressive/metastatic disease, which would typically be a criterion for removal from protocol therapy. Instead, these patients are managed as stage 4S patients.
Controversy exists regarding the necessity of measuring the primary tumor response in all three dimensions or whether the single longest dimension, as in RECIST tumor response determination, is equally useful.[37] The latter has been adopted for use in the INRC.
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