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Malignant Central Nervous System (CNS) Rule M5 instructs us to abstract a single primary (as malignant) when a single tumor is originally diagnosed as non-malignant, the “First course treatment was active surveillance (no tumor resection),” and the subsequent resection pathology is malignant.

This patient’s first course of treatment was not active surveillance. While the patient did not have first course tumor resection, the tumor was treated with Cabergoline.

Should Rule M5 apply because there was no tumor resection? If so, should Rule M5 clearly state no tumor resection is the criteria (not active surveillance)?

SINQ 20230023 does indicate a PitNET diagnosis following a diagnosis of pituitary adenoma does not fall into standard rules, but in the previous SINQ the first course treatment was a partial resection.

It is unclear whether other types of treatment could result in a new malignant PitNET, following a previously treated non-malignant pituitary tumor.

Example: Patient has a 04/2023 diagnosis of symptomatic anemia not responsive to Retacrit. Further testing includes diagnostic bone marrow biopsy 10/2023 proving MDS with low blasts and SF3B1 mutation, treated with Relozyl (Luspatercept).

There is no SEER*Rx listing for Reblozyl or Luspatercept. Per web search, Luspatercept, sold under the brand name Reblozyl, is a medication used for the treatment of anemia in beta thalassemia and myelodysplastic syndromes.

Is this non-cancer directed treatment since it is given to address the anemia rather than the MDS? If cancer-directed treatment, how should it be coded?

SEER Program Coding and Staging Manual indicates high grade dysplasia of esophagus, stomach, and small intestine are reportable. The ICD-O-3.2 does not include “high grade dysplasia” as equivalent to “high grade squamous dysplasia.”

If reportable, would high grade dysplasia (NOS) that originates in the stomach and small intestine default to 8148/2, while esophageal high grade dysplasia (NOS) default to 8077/2?

SINQ 20031110 addresses an overlapping lip lesion between skin and the vermillion border. We were instructed to go with area of greatest involvement. Case would be reportable if >50% of tumor was on the vermillion border and site would be coded to vermillion border (C00._). Often times percentage of involvement is not stated and all that is known is that the lesion overlaps skin and mucosa.

The CT findings identified a right kidney rim-enhancing centrally cystic lesion most suggestive of clear cell renal cell carcinoma measuring 3.2 cm.  The radiologist’s impression was “concerning for renal cell carcinoma.” The subsequent urologist’s consult states the right kidney lesion has a 75% chance of malignancy. The urologist discussed active surveillance, surgery, and ablation, and after discussion with the patient the plan was for active surveillance.

No further information is available, and we are unable to follow up with the physician regarding this case.

Should a lesion with a high percentage chance of malignancy (e.g., 75% chance) be considered a lesion “most likely” to be malignant?

The ICD-O-3.2 indicates histology 8051 only applies to diagnoses of condylomatous carcinoma and warty carcinoma made prior to 2018. For penis cases diagnosed 2018 and later, these neoplasms should be coded as 8054. This is consistent with SINQ 20200003.

However, a new Table was added to the Other Sites schema in the 2024 Solid Tumor Rules update. Table 23 lists “Verrucous carcinoma / carcinoma cuniculatum / Warty carcinoma” as histology 8051. While verrucous carcinoma is still listed under histology 8051 in the ICD-O-3.2, warty carcinoma is not.

Does Table 23 need to be updated? Or is this an error in both the ICD-O-3.2 and SINQ 20200003?

We would like to confirm that the correct histology code is 9431/1 per ICD-O-3.2, as the Non-Malignant CNS Solid Tumor Manual lists the histology code as both 9431/1 (pages 301 and 303 of the combined manual), but also shows it as a synonym for 9421/1 – Diffuse astrocytoma, MYB- or MYBL1 altered (page 304).

In the 2024 Solid Tumor Rules update, the small cell neuroendocrine carcinoma row in Table 2 was changed. The NOS histology became neuroendocrine carcinoma, NOS (8246) and both large cell and small cell neuroendocrine carcinomas (8013 and 8041, respectively) became the subtype/variants. This change impacts Rule H4 but Rule H4 was not updated. Rule H4 still refers to small cell neuroendocrine carcinoma as being the NOS histology.

In the prior STR versions, it was clear the tumor in question would be coded as 8045 per Rule H4 and Table 2. Considering Rule H4 was not updated according to the changes for Table 2, does histology 8045 still apply to this diagnosis?

There is currently no way to arrive at a histology for this case. Does Rule H4, bullet 3 need to be updated to indicate, “subtype/variant of neuroendocrine carcinoma mixed with any other carcinoma (does not apply to sarcoma)”?

Note 1 states that this histology is treatment-related neuroendocrine prostatic carcinoma demonstrating complete neuroendocrine differentiation or partial neuroendocrine differentiation with adenocarcinoma after androgen-deprivation therapy (ADT). Conversely, Note 2 says to code 8574/3 only when there is no history of previous prostate adenocarcinoma or history of androgen-deprivation therapy.

The WHO Blue Book does confirm this is a treatment-related histology, so it seems we would only use this for an adenocarcinoma with neuroendocrine differentiation (or even possibly a mixed histology tumor with adenocarcinoma and small cell carcinoma components) if the patient had previous treatment.

If this histology is treatment-related, why would we use this code for a patient without a history of prostate adenocarcinoma or androgen-deprivation therapy? Should Note 2 be corrected?

Does this histology apply to a post-treatment diagnosis of mixed adenocarcinoma and small cell carcinoma? If yes, should this clarification be added?

Laryngeal intraepithelial neoplasia II-III is not included in the ICD-O-3.2 and, while the SEER Program Coding and Staging Manual (SPCSM) confirms this is reportable, neither the SPCSM nor the Solid Tumor Rules Manual provide the specific histology to use for LIN II or LIN III. Should this be coded as 8077/2 since this is most like a high grade squamous dysplasia?