Summary Stage 2018: HemeRetic
Summary Stage 2018
Notes
**HemeRetic**
9724, 9727, 9740-9742, 9749, 9762-9809, 9811-9820, 9831-9920, 9931-9993
* C700-C729, C751-C753 for 9749, 9766 (2018-2022 only) (See Note 2)
C000-C440, C442-C689, C691-C694, C698-C809: 9591 and Schema Discriminator 1: 1, 2
C000-C699, C739-C750, C754-C809: 9751, 9755-9759
C000-C440, C442-C689, C691-C694, C698-C809: 9930
**Note 1:** The following sources were used in the development of this chapter
* SEER Extent of Disease 1988: Codes and Coding Instructions (3rd Edition, 1998) (https://seer.cancer.gov/archive/manuals/EOD10Dig.3rd.pdf)
* SEER Summary Staging Manual-2000: Codes and Coding Instructions (https://seer.cancer.gov/tools/ssm/ssm2000/)
* Collaborative Stage Data Collection System, version 02.05: https://cancerstaging.org/cstage/Pages/default.aspx
* Chapter 83 *Leukemia*, in the AJCC Cancer Staging Manual, Eighth Edition (2017) published by Springer International Publishing. Used with permission of the American College of Surgeons, Chicago, Illinois.
**Note 2:** For the histologies and primary sites listed below, these have moved to the Brain, CNS Other, and Intracranial Gland Summary Stage chapters starting with 2023 diagnoses.. If you have one of these cases for 2018-2022, then this is the appropriate chapter. If you have one of these cases diagnosed on January 1, 2023 forward, see the appropriate chapter
* 9749, 9766
* C700, C710-C719: *Brain*
* C701, C709, C720-C725, C728-C729: *CNS Other*
* C751-C753: *Intracranial Gland*
**Note 3:** See the following chapters for the listed histologies
* 9591 and Schema Discriminator 1: 3, 9 (C000-C440, C442-C689, C691-C694, C698-C809): *Lymphoma*
* 9751, 9755-9759 (C700, C710-C719): *Brain*
* 9751, 9755-9759 (C701, C709, C720-C725, C728-C729): *CNS Other*
* 9751, 9755-9759 (C751-C753): *Intracranial Gland*
* 9930 (C441, C690, C695-C696): *Lymphoma Ocular Adnexa*
**Note 4:** The following histologies can be localized (code 1), systemic (7) or unknown (9)
9740 Mast cell sarcoma
9749 Erdheim-Chester disease (2021+ only) (except C700-C729, C751-C753 for 1/1/2023+)
9751 Langerhans cell histiocytosis, disseminated (except C700-C729, C751-C753)
9755 Histiocytic sarcoma (except C700-C729, C751-C753)
9756 Langerhans cell sarcoma (except C700-C729, C751-C753)
9757 Interdigitating dendritic cell sarcoma (except C700-C729, C751-C753)
9758 Follicular dendritic cell sarcoma (except C700-C729, C751-C753)
9759 Fibroblastic reticular cell tumor (except C700-C729, C751-C753)
9766 Lymphomatoid granulomatosis, Grade 3 (2021+ only) (except C700-C729, C751-C753 for 1/1/2023+)
9930 Myeloid sarcoma (except C441, C690, C695-C696)
9971 Polymorphic PTLD (2018-2020 only, nonreportable as of 2021)
**Note 5:** For histologies listed in **Note 4**, it is possible to have lymph node involvement; however, at this time, lymph node involvement for these histologies is not collected.
**Note 6:** **The following histologies are systemic (code 7):**
9591 Splenic B-cell lymphoma/leukemia, unclassifiable (except C441, C690, C695-C696)
9724 Systemic EBV-positive T-cell lymphoma of childhood
9727 Blastic plasmacytoid dendritic cell neoplasm
9741 Systemic mastocytosis with an associated hematological neoplasm
9742 Mast cell leukemia
9762 Heavy chain diseases
9800 Leukemia, NOS
9801 Acute undifferentiated leukemia
9806 Mixed-phenotype acute leukemia with t(9;22)(q34.1;q11.2); *BCR-ABL1*
9807 Mixed-phenotype acute leukemia with t(v;11q23.3); *KMT2A*-rearranged
9808 Mixed-phenotype acute leukemia, B/myeloid, NOS
9809 Mixed-phenotype acute leukemia, T/myeloid, NOS
9811 B-lymphoblastic leukemia/lymphoma, NOS
9812 B-lymphoblastic leukemia/lymphoma with t(9;22)(q34.1;q11.2); *BCR-ABL1*
9813 B-lymphoblastic leukemia/lymphoma with t(v;11q23.3); *KMT2A*-rearranged
9814 B-lymphoblastic leukemia/lymphoma with t(12;21)(p13.2;q22.1); *ETV6-RUNX1*
9815 B-lymphoblastic/lymphoma with hyperdiploidy
9816 B-lymphoblastic/lymphoma with hypodiploidy (hypodiploid ALL)
9817 B-lymphoblastic/lymphoma with t(5;14)(q31.1;q32.1); IGH/*IL3*
9818 B-lymphoblastic/lymphoma with t(1;19)(q23;p13.3); *TCF3-PBX1*
9819 B-lymphoblastic/lymphoma, BCR-ABL1-like (2021+ only)
9820 Lymphoid leukemia, NOS
9831 T-cell large granular lymphocytic leukemia
9832 Prolymphocytic leukemia, NOS
9833 B-cell prolymphocytic leukemia
9834 T-cell prolymphocytic leukemia
9837 T-lymphoblastic leukemia/lymphoma
9840 Pure erythroid leukemia
9860 Myeloid leukemia, NOS
9861 Acute myeloid leukemia, NOS
9863 Chronic myeloid leukemia
9865 Acute myeloid leukemia with t(6;9)(p23;q34.1); *DEK-NUP214*
9866 Acute promyelocytic leukemia with *PML-RARA*
9867 Acute myelomonocytic leukemia
9869 Acute myeloid leukemia with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26;2); *RBM15-MKL1*
9870 Acute basophilic leukemia
9871 Acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); *CBFB-MYH11*
9872 Acute myeloid leukemia, minimal differentiation
9873 Acute myeloid leukemia without maturation
9874 Acute myeloid leukemia with maturation
9875 Chronic myeloid leukemia, *BCR-ABL1*-positive
9876 Atypical chronic myeloid leukemia *BCR-ABL1*-negative
9877 Acute myeloid leukemia with mutated NPM1 (2021+ only)
9878 Acute myeloid leukemia with biallelic mutation of CEBPA (2021+ only)
9879 Acute myeloid leukemia with mutated RUNX1 (2021+ only)
9891 Acute monoblastic and monocytic leukemia
9895 Acute myeloid leukemia with myelodysplasia-related changes
9896 Acute myeloid leukemia with t(8;21)(q22;q22.1), *RUNX1-RUNX1T1*
9897 Acute myeloid leukemia with t(9;11)(p21.3;q23.3); *KMT2A-MLLT3*
9898 Myeloid leukemia associated with Down Syndrome
9910 Acute megakaryoblastic leukemia
9911 Acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13.3;q13.1); *RBM15-MKL1*
9912 Acute myeloid leukemia with BCR-ABL1 (2021+ only)
9920 Therapy-related myeloid neoplasms
9931 Acute panmyelosis with myelofibrosis
9940 Hairy cell leukemia
9945 Chronic myelomonocytic leukemia, NOS
9946 Juvenile myelomonocytic leukemia
9948 Aggressive NK-cell leukemia
9950 Polycythemia vera
9961 Primary myelofibrosis
9962 Essential thrombocythemia
9963 Chronic neutrophilic leukemia
9964 Chronic eosinophilic leukemia, NOS
9965 Myeloid/lymphoid neoplasms with *PDGFRA* rearrangement
9966 Myeloid/lymphoid neoplasm with *PDGFRB* rearrangement
9967 Myeloid/lymphoid neoplasm with *FGFR1* rearrangement
9968 Myeloid/lymphoid neoplasm with PCM1-JAK2 (2021+ only)
9975 Myelodysplastic/myeloproliferative neoplasm, unclassifiable
9980 Myelodysplastic syndrome with single lineage dysplasia
9982 Myelodysplastic syndrome with ring sideroblasts and single lineage dysplasia
9983 Myelodysplastic syndrome with excess blasts
9985 Myelodysplastic syndrome with multilineage dysplasia
9986 Myelodysplastic syndrome with isolated del(5q)
9989 Myelodysplastic syndrome, unclassifiable
9991 Refractory neutropenia (2018-2020 only, see code 9980/3 for 2021+)
9992 Refractory thrombocytopenia (2018-2020 only, see code 9980/3 for 2021+)
9993 Myelodysplastic syndrome with ring sideroblasts and multilineage dysplasia (2021+ only)
**Note 7:** Summary Stage is the only applicable staging system for this site/histology/schema.
**Note 8:** Codes 0, 2, 3, 4, and 5 are not applicable for this chapter.
| SS2018 |
Description |
| 1 |
Localized only
- Localized disease
- (Single/solitary/unifocal/isolated)
- See Notes 2 and 3 |
| 7 |
Distant site(s)/lymph node(s) involved
- Systemic disease
- See Note 5 |
| 9 |
Unknown if extension or metastasis |
(1) Fritz AG, Ries LAG (eds). **SEER Extent of Disease 1988: Codes and Coding Instructions (3rd Edition, 1998)**, National Cancer Institute, NIH Pub. No. 98-2313, Bethesda, MD, 1998
(2) Young JL Jr, Roffers SD, Ries LAG, Fritz AG, Hurlbut AA (eds.). **SEER Summary Staging Manual-2000: Codes and Coding Instructions**, National Cancer Institute, NIH Pub. No. 01-4969, Bethesda, MD, 2001.
(3) Collaborative Stage Work Group of the American Joint Committee on Cancer. **Collaborative Stage Data Collection System User Documentation and Coding Instructions, version 02.05**. American Joint Committee on Cancer (Chicago, IL)
(4) Gress, D.M., Edge, S.B., Gershenwald, J.E., et al. **Principles of Cancer Staging**. In: Amin, M.B., Edge, S.B., Greene, F.L., et al. (Eds.) AJCC Cancer Staging Manual. 8th Ed. New York: Springer; 2017
(5) **Introduction to Hematologic Malignancies**. In: Amin, M.B., Edge, S.B., Greene, F.L., et al. (Eds.) AJCC Cancer Staging Manual. 8th Ed. New York: Springer; 2017
(6) Radich, J.P., Jaffe, E.S., Leonard, J.P., et al. **Leukemia**. In: Amin, M.B., Edge, S.B., Greene, F.L., et al. (Eds.) AJCC Cancer Staging Manual. 8th Ed. New York: Springer; 2017
