Solid Tumor Rules/Histology--Esophagus: Are SMARCA4- deficient malignant neoplasms (8020/3) valid for esophagus or other sites besides lung? See Discussion.
SINQ 20200057 states to use SMARCA4-deficient malignant neoplasms newly identified to use 8020/3 in this example for lung. The annotated histology list shows this histology followed by (C34._) for 2023 forward. An esophagus pathology states the following, "The histologic features and immunohistochemical profile are those of a SMARCA2/SMARCA4-deficient malignant neoplasm." Is the 8020/3 histology valid for esophagus or other sites?
Assign 8020/3 for SMARCA4- deficient malignant neoplasms of the esophagus.
The WHO Classification of Digestive System Tumors, 5th edition, lists undifferentiated carcinoma as 8020/3. Undifferentiated carcinoma of the esophagus is characterized by the frequent loss of SMARCA4 or SMARCA2 by immunohistochemistry.
SINQ 20200057 was updated in August 2025 and assigns code 8044/3 for Thoracic SMARCA4-deficient undifferentiated tumor (SMARCA4-UT). The 2025 Solid Tumor Manual includes SMARCA4-deficient or SMARCB1-deficient tumors for thoracic and sinonasal sites (8044/3).
Assigning histology to other individual sites should be on a case-by-case basis.
2025 SEER Manual/Primary Site--Lymph Nodes: How is Primary Site coded when lymphangioleiomyomatosis is incidentally diagnosed in pelvic lymph nodes on a resection for an unrelated reason? See Discussion.
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?
Code the primary site to pelvic lymph nodes (C775) as it is the only site involved with this extrapulmonary lymphangioleiomyomatosis (E-LAM).
EOD 2018/EOD Regional Nodes--Oropharynx: Is code 550 missing “< equal to 6 cm” in the data item EOD Regional Nodes for Oropharynx HPV-Associated, Version 9? Otherwise, bilateral or contralateral lymph nodes with extranodal extension (ENE) that are >6 cm could fit into 550 OR 650.
Code 550 is missing “< equal to 6 cm.” In addition, code 650 should include ipsilateral lymph nodes as well.
Revised codes:
Code 550
CLINICAL ASSESSMENT only
Bilateral or contralateral lymph nodes, < equal to 6 cm WITH clinical evidence of ENE
Code 650
CLINICAL ASSESSMENT only
Ipsilateral, Bilateral or Contralateral lymph nodes > 6 cm WITH or WITHOUT clinical evidence of ENE
These changes will be implemented in Version 3.4 (October 2026). We apologize for the error.
First Course of Therapy/Hormone Therapy--Meningioma: Should Sandostatin be coded as treatment for a Grade 1 meningioma? Patient had surgery and was somatostatin receptor 2 (SSTR2) positive by immunohistochemistry.
Code Sandostatin (octreotide acetate) as hormonal therapy when given including:
· SSTR 2 positive meningioma (NCCN, 2025: smaller studies support the use of targeted therapy including somatostatin)
· Neuroendocrine tumor (NET) (NCCN, 2025: Tumor control: antitumor effect is supported by studies for well-differentiated G1/G2 gastro-entero-pancreatic NET. In lung/thymic NET, somatostatin analogues may be considered if metastatic or SSTR positive).
The SEER*Rx entry for Octreotide Acetate was updated as studies showed somatostatin analogs may shrink tumors or inhibit further growth.
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