Summary Stage 2018: HemeRetic
Summary Stage 2018
Notes
**HemeRetic**
**The preferred histology terms listed in this chapter are from the 2024 WHO Classification of Tumours, Haematolymphoid tumours, 5th edition.**
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:** **Sources 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:** **Histologies moved to Brain, CNS, Intracranial Gland**
* 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**
* **Brain**: C700, C710-C719
* **CNS Other**: C701, C709, C720-C725, C728-C729
* **Intracranial Gland**: C751-C753
**Note 3:** **Other Summary Stage chapters with the listed histologies**
* **Brain**: 9751, 9755-9759, (9749, 9766 for 2023+) (C700, C710-C719)
* **CNS Other**: 9751, 9755-9759, (9749, 9766 for 2023+) (C701, C709, C720-C725, C728-C729) (9749, 9766 for 2023+)
* **Intracranial Gland**: 9751, 9755-9759, (9749, 9766 for 2023+) (C751-C753)
* **Lymphoma**: 9591 and Schema Discriminator 1: 3, 9 (C000-C440, C442-C689, C691-C694, C698-C809)
* **Lymphoma Ocular Adnexa**: 9930 (C441, C690, C695-C696
**Note 4:** **The following histologies can be localized (code 1), systemic (7) or unknown (9)**
* 9740: Mast cell sarcoma (MCS)
* 9749: Erdheim-Chester disease (ECD) *(2021+ only)*
* 9755: Histiocytic sarcoma
* 9756: Langerhans cell sarcoma (LCS)
* 9757: Interdigitating dendritic cell sarcoma (IDCS)
* 9758: Follicular dendritic cell sarcoma (FDCS)
* 9759: Fibroblastic reticular cell tumor
* 9766: Lymphomatoid granulomatosis, grade 3 *(2021+ only)*
* 9930: Myeloid sarcoma
* 9971: Polymorphic PTLD *(2018-2020, 2025+)*
* **Note:** 9971 is /1 and non-reportable *(EXCEPT for C700-C729, C751-C753)* for 2021-2024, moved back to /3 for 1/1/2025+
**Note 5:** **Lymph node involvement**
* 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
* 9724: Systemic EBV-positive T-cell lymphoma of childhood (SEBVTCL)
* 9727: Blastic plasmacytoid dendritic cell neoplasm (BPDC)
* 9741: Systemic mastocytosis with an associated hematological neoplasm (SM-AHN)
* 9742: Mast cell leukemia (MCL)
* 9750: ALK-positive histiocytosis
* 9751: Langerhans cell histiocytosis (LCH), disseminated
* 9762: Heavy chain diseases, NOS (HCD)
* 9800: Leukemia, NOS
* 9801: Acute undifferentiated leukemia
* 9805: Acute leukemia of ambiguous lineage with other defined genetic alterations
* 9806: Mixed-phenotype acute leukemia (MPAL) with *BCR::ABL1* fusion
* 9807: Mixed-phenotype acute leukemia (MPAL) with *KMT2A-rearranged*
* 9808: Mixed -phenotype acute leukemia, B/myeloid, NOS
* 9809: Mixed-phenotype acute leukemia (MPAL), T/myeloid, NOS
* 9811: B-lymphoblastic leukemia/lymphoma [B-ALL/LBL], NOS
* 9812: B-lymphoblastic leukemia/lymphoma [B-ALL/LBL] with *BCR::ABL1 fusion*
* 9813: B lymphoblastic leukemia/lymphoma [B-ALL/LBL] with *KMT2A rearrangement*
* 9814: B-lymphoblastic leukemia/lymphoma [B-ALL/LBL] with *ETV6::RUNX1 fusion*
* 9815: B-lymphoblastic leukemia/lymphoma with high hyperdiploidy (B-ALL/LBL with high hyperdiploidy)
* 9816: B-lymphoblastic leukemia/lymphoma [B-ALL/LBL] with hypodiploidy (Hypodiploid B-ALL/LBL)
* 9817: B-lymphoblastic leukemia/lymphoma [B-ALL/LBL] with *IGH::IL3 fusion*
* 9818: B-lymphoblastic leukemia/lymphoma [B-ALL/LBL] with *TCF3::PBX1*
* 9819: B-lymphoblastic leukemia/lymphoma [B-ALL/LBL] with *BCR::ABL1 like features* (BCR::ABL1-like B-ALL/LBL) *(2021+ only)*
* 9820: Lymphoid leukemia, NOS
* 9831: T-large granular lymphocytic leukemia (T-LGLL)
* 9832: Prolymphocytic leukemia (PPL), NOS
* 9833: Prolymphocytic leukemia, B-cell type (BLL)
* 9834: T-cell prolymphocytic leukemia (T-PLL)
* 9837: T-lymphoblastic leukemia/lymphoma (T-ALL/LBL)
* 9840: Acute erythroid leukemia (AEL)
* 9860: Myeloid leukemia, NOS
* 9861: Acute myeloid leukemia (AML), NOS
* 9863: Chronic myeloid leukemia (CML), NOS
* 9865: Acute myeloid leukemia with *DEK::NUP214 fusion*
* 9866: Acute promyelocytic leukemia with *PML::RARA* fusion (APL with *PML::RARA*)
* 9867: Acute myelomonocytic leukemia, NOS (AMML)
* 9869: Acute myeloid leukemia with *MECOM rearrangement*
* 9870: Acute basophilic leukemia
* 9871: Acute myeloid leukemia with *CBFB::MYH11 fusion*
* 9872: Acute myeloid leukemia, minimal differentiation
* 9873: Acute myeloid leukemia without maturation
* 9874: Acute myeloid leukemia with maturation
* 9875: Chronic myeloid leukemia (CML), *BCR::ABL1 positive*
* 9876: Myelodysplastic/myeloproliferative neoplasm with neutrophilia (MDS/MPN-N)
* 9877: Acute myeloid leukemia with mutated *NPM1* *(2021+ only)*
* 9878: Acute myeloid leukemia with *CEBPA mutation* *(2021+ only)*
* 9879: Acute myeloid leukemia with mutated *RUNX1* *(2021+ only)*
* 9891: Acute monocytic leukemia
* 9895: Myelodysplasia-related acute myeloid leukemia (AML-MR)
* 9896: Acute myeloid leukemia with *RUNX1::RUNX1T1*
* 9897: Acute myeloid leukemia with *KMT2a rearrangement*
* 9898: Myeloid leukemia associated with Down Syndrome (ML-DS)
* 9910: Acute megakaryoblastic leukemia (AMKL)
* 9911: Acute myeloid leukemia (megakaryoblastic) with *RBMI5::MRTFA*
* 9912: Acute myeloid leukemia with *BCR::ABL1 fusion* *(2021+ only)*
* 9920 Therapy-related myeloid neoplasms
* 9931: Acute panmyelosis with myelofibrosis (APMF)
* 9940: Hairy cell leukemia (HCL)
* 9945: Chronic myelomonocytic leukemia, NOS (CMML)
* 9946: Juvenile myelomonocytic leukemia (JMML)
* 9948: Aggressive NK-cell leukemia (ANKL)
* 9950: Polycythemia vera (PV)
* 9961: Primary myelofibrosis (PMF)
* 9962: Essential thrombocythemia (ET)
* 9963: Chronic neutrophilic leukemia (CNL)
* 9964: Chronic eosinophilic leukemia (CEL)
* 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 *JAK2* rearrangement *(2021+ only)*
* 9975: Myelodysplastic/myeloproliferative neoplasm, NOS, unclassifiable (MPN/MDS-U)
* 9980: Myelodysplastic neoplasm with low blasts and single-lineage dysplasia (MDS-LB-SLD)
* 9982 Myelodysplastic /myeloproliferative neoplasm with low blasts and SF3B1 mutation
* 9983: Myelodysplastic neoplasm with increased blasts (MDS-IB)
* 9985: Myelodysplastic neoplasm with low blasts, NOS (MDS-LB)
* 9986: Myelodysplastic neoplasm with low blasts and 5q deletion (MDS-5q)
* 9989: Myelodysplastic neoplasm, NOS
* 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+)*
**Note 7:** **Only staging system**
* Summary Stage is the only applicable staging system for this site/histology/schema.
**Note 8:** **Not applicable codes**
* Codes 0, 2, 3, and 4 are not applicable for this chapter.
| SS2018 |
Description |
| 1 |
**Localized only**
- Localized disease
- (Single/solitary/unifocal/isolated)
- See Notes 4 and 5 |
| 7 |
**Distant site(s)/lymph node(s) involved**
- Systemic disease
- See Note 6 |
| 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
