July 2019 Revision History for the Solid Tumor Rules

• Behavior requirement removed from the "same row rule" in all sites.

• Section renamed from "Coding Multiple Histologies" to "Coding Histology"
• Reorganized for clarity
• Notes added:
  • Note 2: Only use this section for one or more histologies within a single tumor.
  • Note 3: Do not use this section in place of the Histology Rules.

• "Biomarkers" removed from all sites except Non-malignant CNS and Malignant CNS and Peripheral Nerves
• "Treatment Plan" added to physician documentation list
• "Final Diagnosis" changed to "Final Diagnosis / synoptic report as required by CAP"

• Anticipated release date of Cutaneous Melanoma and Other Sites Rules has changed to 2021. Use the MPH 2007 General Instructions for melanoma and other sites cases diagnosed 1/1/2007-12/31/2020.
• Instruction added: "For instructions on coding grade, stage, SSDIs, and treatment, please refer to the appropriate manuals."
• Definition of "De novo" updated: "For colon cancer, de novo (formerly called frank) carcinoma originates in the mucosa of the colon rather than in a polyp."
• Definition of "Simultaneous" and "Synchronous" added: "This term is used in the Solid Tumor Rules to describe malignant tumors diagnosed at the same time or during initial workup (prior to first course of therapy)."

Terms and Definitions (Breast)

• Table 3: Specific Histologies, NOS/ NST, and Subtypes/Variants
  • Code for "Lymphangiosarcoma" (a subtype of sarcoma) corrected to 9170/3

Multiple Primary Rules (Breast)

• Rule M9: Abstract multiple primaries when the diagnosis is Paget disease with synchronous underlying tumor which is NOT duct.
  • Term "synchronous" removed (unnecessary for a multiple primary result)
• Rule M10: Abstract a single primary when synchronous multiple tumors are carcinoma NST/duct and lobular.
  • Term "synchronous" removed
• Rule M14: Abstract multiple primaries when separate/non-contiguous tumors are…
  • Second bullet added: A combination code in Table 2 and a code from Table 3

Histology Rules (Breast)

• Priority Order for Using Documentation to Identify Histology
  • The term "Hormone" added to Note 1: Histology changes do occur following immunotherapy, chemotherapy, hormone, and radiation therapy.
• Coding Histology:
  • Definition of "Different histologies" updated to include combination codes from Table 2
  • #2A Note Added: "This does not apply to invasive carcinoma NST/ductal and lobular carcinoma. Use the combination code 8522/3."
• Rule H10: Code mucinous carcinoma/adenocarcinoma 8480 ONLY when…
  • Note added: "When a tumor has both mucinous carcinoma and a different histology, and mucinous is less than or equal to 90% of the tumor (or the percentage is not documented), code the other histology."
• Rule H16: Code the histology that comprises the majority (greater than 50%) of tumor when two histologies are…
  • Bullet 3 added: A combination code from Table 2 and a code from Table 3

Terms and Definitions (Colon)

• Changes from 2007 Rules #5, bullet 3: Behavior code for Low-grade pseudomyxoma peritonei changed from /0 to /1
• Table 1: Specific Histologies, NOS, and Subtypes/Variants:
  • "Any carcinoid mixed with neuroendocrine carcinoma" removed as a synonym for Mixed adenoneuroendocrine carcinoma 8244

Multiple Primary Rules (Colon)

• Rule M8: Abstract a single primary when a subsequent tumor arises at the anastomotic site AND…
  • Note 1 added: "Bullet two does not apply to GIST. GISTs only start in the wall; never in the mucosa."

Histology Rules (Colon)

• Rule H2: Code the histology and ignore the polyp when a carcinoma originates in a polyp.
  • Term "Specific" removed from "Code the specific histology…" (causing confusion about NOS vs specific histologies)
• Rule H4: Code mixed mucinous and signet ring cell as follows:
  • Note added: "This rule is for mucinous carcinoma and signet ring cell carcinoma in a single tumor. For mucinous adenocarcinoma mixed with another histology OR signet ring cell carcinoma mixed with another histology, proceed through the rules."
• Rule H5 and H6 switched places (rules reordered)
• Rule H6: Code adenocarcinoma NOS 8140 when the final diagnosis is…
  • Note added: 'Adenocarcinoma in a polyp' removed from the rule due to registrar feedback. The situation is addressed in a previous rule.

Terms and Definitions (Head and Neck)

• NOT Equivalent Terms added:
  • "P16 positive is not equivalent to HPV positive"
  • "Phenotype is not equivalent to subtype/type/variant"
• Equivalent Terms removed:
  • "Squamous cell carcinoma with sarcomatoid features; sarcomatoid squamous cell carcinoma"
  • "Fibromyxosarcoma 8811, myxofibrosarcoma 8830"
• "Coding Primary Site" section renamed "Coding Primary Site When There is Conflicting Information"
  • Note added: "Record primary site based on the most definitive indication of primary site in the medical documentation and use the priority order when there is conflicting info without a definitive statement."
  • #6B added: "C058 Overlapping lesion of palate, junction of hard and soft palate"
• Table 1: Tumors of Nasal Cavity, Paranasal Sinuses and Skull base
  • "Transitional Cell Carcinoma" deleted as a synonym for Non-keratinizing squamous cell carcinoma 8072
  • Subtypes/variants of rhabdomyosarcoma indented beneath rhabdomyosarcoma
• Table 5: Tumors of the Oropharynx, Base of Tongue, Tonsils, Adenoids
  • Note added: "HPV-positive is not equivalent to HPV-mediated (p16+). According to the 2018 SEER Manual, HPV-type 16 refers to virus type and is different from p16 overexpression (p16+). HPV status is determined by tests designed to detect viral DNA or RNA. Tests based on ISH, PCR, RT-PCR technologies detect the viral DNA or RNA; whereas, the test for p16 expression, a surrogate marker for HPV, is IHC. HPV testing must be positive by viral detection tests in order to code histology as 8085."
• Table 7: Tumors of Odontogenic and Maxillofacial Bone (Mandible, Maxilla)
  • Synonyms added to Odontogenic carcinosarcoma 8989/3: "Ameloblastic carcinosarcoma" and "Mixed odontogenic carcinoma"
  • "Malignant ameloblastoma/classic ameloblastoma 9310/3" deleted as a subtype of Odontogenic carcinosarcoma 8989/3
• Table 10: Paired Sites
  • Skin of External Ear C442 added

Multiple Primary Rules (Head and Neck)

• Rule M3: Abstract multiple primaries when there are separate/non-contiguous tumors in any two of the following sites…
  • Site codes added:
    • Glottis C320 AND/OR supraglottis C321 AND/OR subglottis C322 AND/OR laryngeal cartilage C323
    • Maxilla C410 AND Mandible C411
    • Postcricoid C130 AND/OR hypopharyngeal aspect of aryepiglottic fold C131 AND/OR posterior wall of hypopharynx C132

Histology Rules (Head and Neck)

• Priority Order for Using Documentation to Code Histology:
  • "#2 Cytology of primary site (fine needle aspirate (FNA))" added

Terms and Definitions (Kidney)

• Table 1: NET row:
  • "Neuroendocrine tumor" code corrected to 8240
  • "Well differentiated neuroendocrine tumor" moved from the subtype to the synonym column
  • "Small cell neuroendocrine carcinoma 8041" added as subtype.

Terms and Definitions (Lung)

• Changes from 2007 Rules
  • The following codes added to "new adenocarcinoma terms and codes" list:
    • Adenocarcinoma, acinar predominant 8551
    • Adenocarcinoma, lepidic predominant 8250
    • Adenocarcinoma, micropapillary predominant 8265
    • Adenocarcinoma, papillary predominant 8260
    • Adenocarcinoma, solid predominant 8230
• Table 2: Combination/Mixed Histology Codes
  • New row added: SCC, keratinizing + SCC, non-keratinizing, is coded to SCC NOS
  • 8255 row: #1: Clarified that 8255/3 is used when percentages of adenocarcinoma subtypes are unknown
• Table 3: Specific Histologies, NOS, and Subtype/Variants
  • "Adenocarcinoma, non-mucinous, NOS" added as a synonym for adenocarcinoma 8140
  • Neuroendocrine tumor (NET) 8041 terminology changed to small cell carcinoma

Multiple Primary Rules (Lung)

• Rule M8: Abstract multiple primaries when separate/non-contiguous tumors are…
  • Second bullet added: A combination code in Table 2 and a code from Table 3

Histology Rules (Lung)

• Priority Order for Using Documentation for Coding Histology
  • #2 Cytology: Changed to "Fine Needle Biopsy from primary site, pleural fluid or pericardial fluid"
• Rule H1 and Rule H10: Code mucinous adenocarcinoma as follows (for lung only)
  • Note added: "When mucinous carcinoma is mixed with another histology, such as adenocarcinoma and mucinous carcinoma, code mucinous ONLY when mucinous is documented to be greater than 50% of the tumor."
• NEW RULE: Rule H7 Code the histology that comprises the greatest percentage of tumor when two or more of the following histologies are present: (See Solid Tumor Rules for notes and examples)
  • Acinar adenocarcinoma / Adenocarcinoma, acinar predominant 8551
  • Lepidic adenocarcinoma / Adenocarcinoma, lepidic predominant 8250
  • Micropapillary adenocarcinoma / Adenocarcinoma, micropapillary predominant 8265
  • Papillary adenocarcinoma / Adenocarcinoma, papillary predominant 8260
  • Solid adenocarcinoma / Adenocarcinoma, solid predominant 8230

Terms and Definitions (Malignant CNS)

• Introduction
  • Note added: See the Head and Neck Rules for coding paragangliomas.
• Terms that are NOT Equivalent or Equal
  • WHO Grade is not equivalent to tumor grade
• Table 1: WHO Grades of Select CNS Neoplasms
  • WHO Grade definitions added
  • Note added: "CNS WHO classifications use a grading scheme that is a 'malignancy scale' ranging across a wide variety of neoplasms rather than a strict histologic grading system that can be applied equally to all tumor types."
• Section 2: Reportable Primary Sites and Histologies
  • Note deleted: "Select peripheral nerve codes are included in the non-malignant CNS for nerve roots ONLY. Other than nerve roots, benign tumors of peripheral nerves and/or meninges of those nerves are not reportable." (Not appropriate for the malignant CNS rules)

Terms and Definitions (Non-malignant CNS)

• Introduction
  • Note added: "See the Head and Neck Rules for coding paragangliomas."
• Terms that are NOT Equivalent or Equal
  • Added: "WHO Grade is not equivalent to tumor grade"
• Changes from 2007 MPH Rules
  • Clarification of a clarification:
    • "Multiple cerebral meningiomas (same histology or NOS and subtype/variant) are a single primary"
    • Although NF1 and NF2 themselves are not reportable conditions, the tumors associated with them may be (see reportability section)
• Table 1: WHO Grades of Select CNS Neoplasms
  • WHO Grade definitions added
  • Note added: "CNS WHO classifications use a grading scheme that is a 'malignancy scale' ranging across a wide variety of neoplasms rather than a strict histologic grading system that can be applied equally to all tumor types."
• Table 6: Specific Histologies, NOS, and Subtypes/Variants
  • "Corticotroph" added as a synonym of Pituitary Adenoma 8272/0
  • "Cavernous hemangioma 9121/0" added as a subtype of Hemangioma 9120/0

Multiple Primary Rules (Non-malignant CNS)

• Rule M7: Abstract multiple primaries when multiple tumors are present in any of the following sites…
  • Bullet added: Cauda equina C721 AND any other part of CNS
  • Cranial nerve site code changed to C722
• Rule M9: Abstract a single primary when two or more separate/non-contiguous meningiomas arise in the cranial meninges
  • Note modified: This rule applies ONLY to meningiomas that are either a NOS and subtype/variant, OR they are the same histology.

Histology Rules (Non-malignant CNS)

• Priority Order for Using Documentation to Identify Histology
  • Biomarkers moved from #1A to #1D

Terms and Definitions (Urinary)

• Table 2: Specific Histologies, NOS, and Subtypes/Variants
  • "Plasmacytoid/signet ring cell/diffuse variant" added as a synonym of Lymphoepithelioma-like urothelial carcinoma 8082/3 (subtypes of urothelial carcinoma)

Multiple Primary Rules (Urinary)

• Rule M10: Abstract multiple primaries when the patient has a subsequent tumor after being clinically disease-free for greater than three years after the original diagnosis or last recurrence.
  • Note 1 modified to clarify that the rule does not apply when both/all tumors are urothelial carcinoma of the bladder
• Rule M15: Abstract a single primary when synchronous, separate/non-contiguous tumors are on the same row in Table 2 in the Equivalent Terms and Definitions
  • Example added

Histology Rules (Urinary)

• Rule H8: Code the subtype/variant when all synchronous multifocal/multicentric tumors are a NOS and a single subtype/variant of that NOS…
  • Term “synchronous” removed