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Do not use the NME size from magnetic resonance imaging (MRI) to code tumor size when both tumor size and NME size are stated or if NME is the only size available. The size of the solid tumor mass takes priority over the size of the NME when provided separately and the NME is larger.

The American College of Radiology, Breast Imaging Reporting and Data System (BI-RADS) defines NME as an area of enhancement on MRI that does not belong to a 3D mass or have distinct features of a mass. It is a separate descriptor from size that includes modifiers describing enhancement patterns with a specific MRI pattern.

Code the primary site to pelvic lymph nodes (C775) as it is the only site involved with this extrapulmonary lymphangioleiomyomatosis (E-LAM).

Update February 2026, note added:

Report this case as smoldering myeloma (9732/3) based on the plasma cell 10% threshold and favorable cytogenetics and lack of organ dysfunction (9732/3). According to the College of American Pathologists Plasma Cell Malignancies Protocol, in order to code smoldering multiple myeloma, both criteria must be met:

• Serum monoclonal protein (IgG or IgA) ≥3gm/dL, or urinary monoclonal protein ≥ 500 mg per 24h and/or clonal bone marrow plasma cells 10-60%

• Absence of myeloma defining events or amyloidosis.

Note: This case was answered by our expert pathologist and applies to this case only. Registrars should not use the plasma cell threshold to determine reportability or histology. The diagnosis must come from the pathologist or the managing physician.

Do not code as a treatment. Record the cessation of immunosuppressive drug treatment in text to explain the patient’s change in disease status.

Report severe dysplasia for selected sites. Not all high grade dysplasia and severe dysplasia are reportable. Refer to the list of examples in the SEER Manual Reportability Section and Appendix E, Reportable and Non-reportable Examples. Check also for other standard setters, state, and local reportability requirements.

High grade dysplasia, severe dysplasia, and carcinoma in situ are equivalent terms with behavior /2. Refer to ICD-O, WHO Classification of Tumors, and the SEER Solid Tumor Rules for preferred histology terms and codes. For example, WHO Classification of Head and Neck Tumors, 5th edition, states carcinoma in situ in the oral cavity is synonymous with severe dysplasia though it is not a recommended term.

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.

Report LR-M unless there is information to the contrary. The American College of Radiology defines LR-M as "probably or definitely malignant, not necessarily hepatocellular carcinoma (HCC)."

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.

Do not report superficial CD34-positive fibroblastic tumor (8810/1) of the thigh.

WHO Classification of Soft Tissue and Bone Tumors, 5th ed., defines superficial CD34-positive fibroblastic tumor as a distinctive low-grade neoplasm of the skin and subcutis, most frequently occurring in the lower extremities, especially thigh, followed by arm, buttock, shoulder, and rarely, vulva.

Report smoldering WM (9761/3) using the Hematopoietic and Lymphoid Neoplasms Manual and Database (Table B9). Smoldering WM is defined as a poorly described asymptomatic disorder with a high risk of progressing to symptomatic WM requiring treatment. The term “smoldering” refers to the process meaning it is progressing, perhaps slowly, or even at a slower pace than might be expected. Smoldering WM resembles smoldering MM.