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The ICD-O-3.2 Coding Table includes Mullerian mixed tumor as the preferred term for histology code 8950 (previously malignant mixed Mullerian tumor/MMMT). This table also includes carcinosarcoma, NOS as the preferred term for histology code 8980. Neither the ICD-O-3.2 Coding Table nor the Implementation Guidelines address the long-standing issue of coding histology for diagnoses of carcinosarcoma/malignant mixed Mullerian tumor.

These endometrial primaries are frequently diagnosed as both carcinosarcoma and MMMT. The questions regarding histology coding for carcinosarcoma and carcinosarcoma/MMMT of the endometrium date back to before the Multiple Primaries/Histology Rules, with at least three SINQ entries instructing registrars not to use code 8950/3 (MMMT) for diagnoses of MMMT. SINQ has instructed registrars that MMMT is a synonym for carcinosarcoma and these tumors should be coded to 8980 (carcinosarcoma), not to 8950 (MMMT). The most recent SINQ is partly inconsistent with the others, indicating 8950 can be used if the tumor is only described as MMMT. The other SINQ entries state carcinosarcoma should be used as it is the preferred term for MMMT. (See SINQ 20061008, 20100009, 20180071.)

The most recent SINQ (20180071) specifically indicates: According to the WHO Classification of Tumors of Female Reproductive Organs, 4th edition, MMMT (8950/3) is now a synonym for carcinosarcoma (8980/3) even though it has a separate ICD-O code. The ICD-O code for MMMT is no longer in the WHO book. However, MMMT is in the ICD-O-3.2 Coding Table and is not stated to be obsolete or a synonym. Which is correct, the clarification in the SINQ or the 2021 ICD-O-3.2 Coding Table?

For a 2021 diagnosis of carcinosarcoma/malignant mixed Mullerian tumor, how should registrars code the histology? Follow the previous SINQ entries and Rule H17 to code the histology to 8980 when the diagnosis includes both carcinosarcoma and MMMT? Do these previous SINQ entries still apply to cases diagnosed 2021 and later?

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

We received an edit error in SEER*DMS on the following site/histology (Orbit, NOS (C696)/9699/3) that involved an incorrect staging code being assigned to SS2018. The staging language is identical in AJCC, EOD and SS2018. SEER*RSA notes that SS2018 should be coded distant, but in the SS2018 manual, this language is noted Regional. Staging language is: Orbital adnexal lymphoma AND extraorbital lymphoma extending beyond the orbit to adjacent structures--Bone, Brain, Maxillofacial sinuses

Transurethral resection: Microscopic Diagnosis: Bladder, transurethral resection: Low-grade papillary urothelial carcinoma Gross Description: Received in formalin labeled with the patient's name and bladder tumor is a 3.0 x 2.0 1.0 cm aggregate of friable tan tissue biopsies. The specimen is submitted in toto, cassettes

This is all the information there is on this path report. Extent of Disease (EOD) instructions state inferred description of noninvasive: No statement of invasion (microscopic description present) SEER 2018 Appendix C Bladder Coding Guidelines state code behavior 3 if the only surgery performed is a transurethral resection of the bladder (TURB) documenting that depth of invasion cannot be measured because there is no muscle in the specimen OR the pathology report does not mention whether the submucosa is free of tumor or has been invaded by tumor.

Following the existing Solid Tumor Rules Histology Rules, it would seem this is a single primary with histology 8045 (Combined small cell carcinoma) because there is no indication there are multiple prostate tumors and Table 2 states combined adenocarcinoma and small cell carcinoma is Combined small cell carcinoma (8045).

Conversely, while not an exact match to this case, SINQ 20190083 implies small cell carcinoma and adenocarcinoma of the prostate are separate primaries. In that SINQ case, the patient was simultaneously diagnosed with metastatic small cell carcinoma of the prostate on a liver biopsy and prostate adenocarcinoma on a prostate biopsy. There is no indication that patient had separate tumors in the prostate, however the SINQ instructs to code as separate primaries.

Would the previous SINQ logic apply to synchronous diagnoses in the prostate as well? Or does code 8045 apply to this situation?

Per the 2018 Solid Tumor Rules instructions, Final Diagnosis and Staging Summary (synoptic report) have equal coding priority. However, it is unclear which takes priority, or if this should be a combination of components, when the histologies are two different specific histologic types per Table 3 of the Breast Solid Tumor Rules Manual.

Patient was diagnosed on 01/2020, and primary site on the pathology report is Ampulla of Vater (C241). Synoptic Report states histology as: Intra ampullary papillary-tubular neoplasm in association with microinvasion.

I have reviewed the ICD-O-3 coding table and found histology Intraductal tubulopapillary neoplasm (C25_) code 8503/2. Based on the Matrix principle (Rule F on the ICD-O-3), I will change the behavior to 3 and code as 8503/3. If I look in ICDO-3, Tubulopapillary adenocarcinoma is coded 8263/3.

Rule H6 states: Code 8742/3 (Lentigo maligna melanoma) when the diagnosis is lentigo maligna melanoma with no other histologic types. However, if the diagnosis was strictly lentigo maligna or lentigo maligna melanoma, the first rule that applies is Rule H1 because lentigo maligna melanoma is a single, specific histologic type and Rule H1 states, Code the histology when only one histologic type is identified. Following the current rules, one would never arrive at Rule H6. Should the H Rules be reordered? Or should an example of when one would use Rule H6 be added to clarify when to use this rule?

11/24/20 Adnexal mass, cyst fluid: Positive for malignant cells. Clusters of inhibin-positive, CK7-negative cells, consistent with adult granulosa cell tumor cells. Groups of inhibin-negative, CK7-positive epithelial cells consistent with serous borderline tumor cells. Peritoneal washing: Positive for malignant cells. Small groups of inhibin-positive, CK7-negative cells, consistent with adult granulosa cell tumor cells.

A. Left ovarian mass: Adult granulosa cell tumor (AGCT) of ovary (see note). pTNM Stage: pT1c3 pNX - Serous borderline tumor (SBT) of ovary (see note). pTNM Stage: pT1a pNX. Fallopian tube; unremarkable.

B. Right ovary: - Serous cystadenofibroma of ovary. Fallopian tube; unremarkable.

C. Left pelvic wall nodule: Fibro-calcified nodule, consistent with necrotic appendix epiploica.

D. Uterus (hysterectomy): Uterine leiomyomas. Endosalpingiosis of uterine serosa and paracervical tissue. Atrophic endometrium.

Note: The left ovarian mass is involved by a combined adult granulosa cell tumor and a serous borderline tumor. The AGCT mainly involves the thick-walled cystic area while the SBT the thin-walled cyst/s. The 2 neoplastic elements do, however, demonstrate areas of intimate and close intermingling. From the current literature, it appears that, based on FOXL2 mutation, the AGCT component of combined AGCT and ovarian epithelial tumors is either a true neoplastic processes or an AGCT- like proliferation morphologically indistinguishable from AGCT. To further evaluate the nature of the AGCT component, a FOXL2 analysis is in progress and an addendum will follow.

Neither the ICD-O-3.2 Implementation Guidelines nor the ICD-O-3.2 Coding Guidelines (including Tables 1-7) address reportability changes for chondrosarcoma grade 1. In the Solid Tumor Rules Manual, Head and Neck Equivalent Terms and Definitions, Table 7 (Tumors of Odontogenic and Maxillofacial Bone (Mandible, Maxilla)), Chrondrosarcoma grade 2/3 (9220/3) is included as a subtype/variant for sarcomas in these sites, but it does not address chrondrosarcoma, grade 1.

The ICD-O-3.2 Coding Table lists Chondrosarcoma, grade 1 as morphology code 9222/1. If Chondrosarcoma, grade 1 is no longer a reportable tumor for cases diagnosed 01/01/2021 and later, why wasn't this reportability change included in the ICD-O-3.2 Implementation Guidelines? If the standard setters chose not to include this reportability change, shouldn't Table 7 also indicate that all chondrosarcomas (NOS, grade 1, grade 2 or grade 3) are reportable for cases diagnosed 2018 and later?

How are registrars to make reportability and histology coding decisions for chondrosarcomas when neither source provides clear instructions regarding these tumors?