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Lymphangioleiomyomatosis (LAM) became reportable (9174/3) for diagnoses 2023 and later. While this neoplasm was only added to the Lung schema in the Solid Tumor Rules manual, this is a mesenchymal neoplasm which may arise outside of the lung and the reportability change was not limited to LAM of the lung.

How should primary site be coded when a left pelvic lymph node dissection for an unrelated high-grade serous carcinoma of the right fallopian tube incidentally proved LAM in the pelvic lymph nodes? The pelvic lymph nodes were the only site of involvement; there was no evidence of lung involvement. As this is a mesenchymal tumor, should the primary site default to C499 (Soft tissue, NOS) according to the default primary site rule for sarcomas described in the SEER Manual? Or should the primary site be coded to C775 (Pelvic lymph nodes) as this was the only proven site of involvement?

Lutathera is a radioconjugate consisting of the tyrosine-containing somatostatin analog Tyr3-octreotate (TATE) conjugated with the bifunctional, macrocyclic chelating agent tetra-azacyclododecanetetra-acetic acid (DOTA) and radiolabeled with the beta-emitting radioisotope lutetium Lu 177 with potential antineoplastic activities.

Lung Rule M14 appears to be the first rule that applies to this case and instructs the user to abstract a single primary. However, we were hoping for confirmation that a cancer (NOS) or malignancy (NOS) would not be a distinctly different histology that may qualify for Lung Rule M8. Currently, these histologic terms are not included in the Table 3 options or mentioned in the preceding notes.

Lung cancer, 2022

12/1/2022 PET/CT showed destructive hypermetabolic bone lesions in right frontal and left posterior calvarium. Left posterior calvarium lesion involves portions of left parietal and temporal bones w/invasion of mastoid air cells.

1/4/2023 MRI Brain showed large destructive mass involving left posterior temporal calvarium that extends into left mastoid region and may invade left distal transverse sinus.

2/8/2023 Radiation Oncology follow-up note: MD states there are extensive calvarium metastasis with the left parietal lesion invading the brain causing edema and MS-like changes.

2/13/23 Radiation Oncology Final Letter- Patient was treated with 1 EBRT fraction aimed at brain/skull before enrolling in hospice.

Lung abnormality on imaging prompted diagnosis on subsequent biopsy and clinical staging was documented as cT1b N0 M0. There was an attempt at resection, but the patient was found to have chest wall involvement and the procedure was aborted.

How would Reason for No Surgery of Primary Site be coded in these types of scenarios when the surgery is aborted and the treatment plan changes due to the extension seen during surgery?

Lumpectomy path shows two foci of invasive ductal carcinoma, 1.5 cm & 3 mm sizes, and CAP summary lists "DCIS: focally seen", no further description. The re-excision pathology specimen finds a 1.5 mm focus of residual invasive carcinoma, very close to the new inferior margin (so registrar assumed this was probably not part of the previously excised mass), and no mention of any more in situ.

Can we assume the DCIS was associated with/part of the invasive tumors because it was not measured or described separately? If we say there are 3 tumors (for the measured invasive foci), should Type of Multiple Tumors be coded 30 [In situ and invasive] or 40 [Multiple invasive]?

LUL lobectomy: 1.7cm apical tumor, DX=mod well diff subpleural SCC, with involvement of pleural surface. 3 peribronchial LN neg and 2 AP window LNs neg. Stage T2N0.

1. No lymph node dissection or sampling was stated to be done

2. The lobectomy specimen contained the LNs

3. Scope of regional LN surgery is coded

Would the surgery to primary site code 30 or 33?

LUL lobectomy: 1.7 cm apical tumor, diagnosis: moderately well differentiated subpleural squamous cell carcinoma, with involvement of pleural surface. 3 peribronchial LN neg and 2 AP window LNs neg. Stage T2N0.

1. No lymph node dissection or sampling was stated to be done

2. The lobectomy specimen contained the LNs

Low grade mucinous neoplasms can spread to the peritoneal cavity and in that sense are metastatic but histologically have bland/benign features (may be a benign cystadenoma that ruptured and spread by rupturing) are not a carcinoma. Thus, some have termed this group as DPAM (diseminated peritoneal adenomucinous) and not a true carcinoma. Others indicate that if you have metastasis the tumor is a carcinoma.

Lip has not been added to any of the site-specific histology tables, nor has any other instruction been provided for coding tumors in this site.

Coding histology for lip primaries is difficult because registrars do not know where to look first. The Solid Tumor Rules indicate one should use the tables first, but then do not inform registrars what table to use for a lip primary (i.e., a specific table, any table, no table).

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