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Rule H8 of the Melanoma Solid Tumor Rules states that multiple variants of melanoma in one tumor are rare and a question must be submitted to Ask a SEER Registrar (AASR) for the correct histology code. However, our facility has seen a number of these cases in 2021 and would like to track the official answer and make it available to all in this format.

How should histology be coded for the following?

1. January 2021 diagnosis of left shoulder invasive malignant melanoma, histologic type: nodular and desmoplastic types per College of American Pathologists (CAP) summary of punch biopsy.

2. May 2021 shave biopsy of left arm invasive malignant melanoma, superficial spreading and nodular variant is listed in the CAP summary.

3. June 2021 diagnosis of right cheek invasive malignant melanoma, histologic subtype: superficial spreading and nodular seen on CAP summary of shave biopsy.

The 2022 SEER Manual indicates the p16 status (positive or negative) can be used to code more the specific histology for squamous cell carcinoma, human papilloma virus (HPV) positive (8085) and squamous cell carcinoma, HPV negative (8086). However, the histology coding instructions in the Other Sites schema have not been updated and the 2022 SEER Manual does not cover all situations commonly encountered in the registry. Does the clarification regarding p16 apply to these other situations?

1. How is histology coded when the final diagnosis of the most representative specimen is adenocarcinoma (NOS), but the immunohistochemistry is p16 negative? Is this adequate to code histology to 8484/3 (adenocarcinoma, HPV-independent, NOS)? The pathologist did not specifically indicate this was HPV-independent adenocarcinoma, and the clarification in the 2022 SEER Manual does not include this more specific adenocarcinoma histology.
2. How is histology coded when the Pap smear is positive for squamous cell carcinoma, p16 positive, but the most representative specimen from the primary tumor (the subsequent cervix biopsy) is only stated to be squamous cell carcinoma (NOS)? The p16 studies were not repeated on the most representative specimen, and the existing 2007 Multiple Primaries/Histology (MP/H) General Rules indicate to code the histology from the most representative specimen over a cytology report. Following the existing 2007 MPH General Rules, the histology should be 8070 (squamous cell carcinoma, NOS). However, this does not account for the p16 status of the tumor.
3. How is histology coded when a biopsy of a metastasis (e.g., a lymph node metastasis) proved squamous cell carcinoma, p16 negative, but a subsequent biopsy of the primary cervix tumor proved squamous cell carcinoma (NOS) without additional IHC studies? Again, the 2007 MP/H General Rules confirm the primary site specimen should be used to code the histology, resulting in a diagnosis of 8070 (squamous cell carcinoma, NOS), but this ignores the p16 status of the tumor.

Patient was diagnosed with invasive ductal carcinoma with mucinous features in February 2021, left breast biopsies at 2:00 (3 cm from nipple) and 3:00 (8 cm from nipple) positions. Intraductal component was absent in those specimens.

Subsequent left breast total mastectomy in March 2021, provided a final diagnosis of multifocal mixed mucinous carcinoma, grade 1, 27 and 8 mm and ductal carcinoma in situ (DCIS), low to intermediate grade, with focal mucinous features. The Staging Summary lists Histologic Type as mixed mucinous carcinomafor both foci of invasive carcinoma. DCIS is also noted as present negative for extensive intraductal component.

There does not appear to be a clear instruction or code for this histology. As a central registry, we are unable to follow-up with the pathologist regarding this diagnosis.

Just to be clear, there are 2 foci of invasive mixed mucinous carcinoma in this case measuring 27 mm and 8 mm. The DCIS component measured 56 mm.

A patient has a left breast cancer diagnosed in 2011; then has a "recurrence" in her lymph nodes in 2017. In 2018, she has a new left breast mass that is the same histology and behavior as the 2011 cancer. Based on the 2017 "recurrence" in the lymph nodes, this is not a new breast primary, is that correct?

While we recognize that paragangliomas originate in the parasympathetic or sympathetic nervous system, these are endocrine tumors and endocrine glands/structures are not included in ICD-O site code C479 (Autonomic nervous system). Endocrine tumors of the paraganglia have their own site codes (C75_) per the ICD-O. Additionally, the ICD-O-3.2 provides specific sites for most of the paragangliomas included in Table 9. Per the ICD-O-3.2, carotid body paraganglioma is C754, and middle ear paraganglioma, glomus jugulare tumor, jugulotympanic paraganglioma, and paraganglioma (NOS) are C755.

Why are paragangliomas not coded to the paraganglia sites (C75_) provided in the ICD-O? Should these sites be added to the Head & Neck schema for the specific paragangliomas arising in the head and neck? Obtaining consistency in coding primary site for these tumors will be difficult if registrars use the ICD-O provided site codes instead of the primary site statement preceding Table 9. Additionally, as most registrars may use the ICD-O provided site code, the Head and Neck schema in the Solid Tumor Rules would not apply, the Other Sites schema would apply to sites C754 and C755.

Should the updated Note 5 from the Non-malignant CNS regarding optic nerve glioma also be incorporated into Note 6 for Malignant CNS rules (the pilocytic astrocytoma note)?

This was one of the major issues identified in the SEER*Educate Workshop. Registrars have demonstrated they do not consistently think to look at the Non-malignant CNS schema when they see the term glioma and continue to misclassify optic nerve gliomas as malignant.

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

Patient has biopsy of prostate 12/28/2018 showing Gleason 5+5 adenocarcinoma. Liver biopsy on same date is metastatic small cell carcinoma consistent with prostate primary. Oncology consult states that liver biopsy is likely neuroendocrine conversion from prostate carcinoma. Patient also has bone metastasis and receives radiation, Lupron, Casodex, and chemotherapy of carboplatin and etopiside.

Per Solid Tumor Rules, we code histology from primary site over a metastatic site. Thus, the small cell carcinoma, which appears to be the focus of the chemotherapy is lost. Is it correct to code this as a single primary with an adenocarcinoma histology?

Both SINQ 20130221 and 20180088 instruct us to abstract multiple primaries when patient develops a metastatic small cell carcinoma of the prostate after being previously diagnosed with adenocarcinoma of the prostate.

The patient has a history of prostate adenocarcinoma with lymph node metastases, status post prostatectomy and treatment by Lupron in 2014. The most recent prostate serum antigen measurement (April 2018) was normal. CT scan of the abdomen and pelvis revealed new hypodense liver lesions, a slightly enlarging lung right lower lobe nodule, and enlarging lobular mass in the prostatectomy bed. The core liver biopsy contains areas of metastatic tumor with a differential diagnosis on pathology of high-grade neuroendocrine carcinoma of the prostate (small cell type), which may have been seen in association with prostate adenocarcinoma, or metastatic small cell carcinoma of a different site.

Clinically, the physician impression is that this represents metastatic castration-resistant prostate cancer. The Solid Tumor Rules note that the Multiple Primary Rules are not used for tumor(s) described as metastases. However, SINQ 20130221 indicates that, at least historically, these would have been accessioned as multiple primaries (histology 8140 & 8041 per Rule M10). Does the previous SINQ note still apply to these types of cases, and if so how would one know to move beyond the initial note indicating metastases are not new primaries?

11/18/2019 Left 1st Digit/Thumb Biopsy: Atypical Melanocytic Proliferation consistent with Early Acral Lentiginous Melanoma in situ. Margins Positive. (Not a reportable diagnosis for 2019.)

12/5/2019 Left 1st Digit Shave Biopsies: Malignant Melanoma in situ. Margins Positive.

1/15/2020 Started Aldara (treatment plan: use for ~3 months then Mohs/excision, but due to COVID could not get resection until 7/2020).

7/29/2020 Left Thumb Excision: Residual Melanoma in situ. Margins Positive. Treatment Plan: re-excision.

8/6/2020 Left Thumb Re-Excision: Atypical Lentiginous Melanocytic Proliferation at the 12-2 margin may represent the advancing edge of melanoma in situ. (8/19/2020 Plan to treat the 12-2 margin as positive with in situ; plan for re-excision).

8/20/2020 Left Thumb Re-Excision & Left Nail Plate Excision: Malignant Acral Lentiginous Melanoma with extensive melanoma in situ. Breslow 1.3mm. Margins Positive. Nail plate & bed epithelium with hemorrhage and a mild increase in melanocyte density likely represent melanoma in situ.

9/4/2020 Left thumb partial amputation & Left axillary Sentinel Lymph Node Excision: Residual Malignant Melanoma in situ. 0/3 sentinel nodes positive.