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Assign the number of primaries following the Other Sites Solid Tumor Rules.  Based on sites, laterality and or timing there are 8 primaries.  This is similar to SINQ 20061112 that advised to follow the Multiple Primaries/Histology rules for sebaceous carcinoma.  According to the WHO Classification of Skin Tumors, 5th edition, there is a 30-40% risk of local tumor recurrence, and 20-25% risk of distant metastasis.  In only one instance did a physician refer this as a recurrence in the available notes. 

According to our dermopathology expert, code the histology to nodular melanoma 8721/3. There are numerous possible combinations of melanomas and the correct code depends on the types/variants present. We are currently working on a "Combined/Mixed Histology Code" Table for melanoma; however, it will likely not inlcude all possible combinations so continue submitting your questions to Ask A SEER Registrar. 

Abstract a single primary using 2018 Lung Solid Tumor Rule M9.

The 2014 and 2016 R lung tumors were pathologically confirmed; it is not stated if they were resected. Follow up after XRT noted stable disease but no indication of NED. Subsequent right lung tumor is also the same primary. The issue is the assumed left lung adenocarcinoma in situ. It is not clear how long the left lung nodules were present, but they appeared to be stable as well and only diagnosed as a malignancy based on treatment response. At this time M9 applies and the left lung AIS is not a separate primary. We have discussed at length with lung pathology experts the issue of determining multiple primaries. Identifying and diagnosing lung tumors has become easier with new technology and the result is patients are being diagnosed with multiple lung tumors. Some lung experts feel we are under-reporting lung primaries but all noted the many issues with creating rules for consistency.

In a situation like this with one area of metastatic involvement and an unknown primary, if there is no further information, we advise that the metastasis are "regional" until/unless proven otherwise. With this in mind, code the Mets at Diagnosis fields as 0, including the Mets at Diagnosis--Distant Lymph Node(s). This case should continue to be worked up to identify the primary site. If a primary site is identified later, update the abstract accordingly. In the meantime, use text fields to describe the situation.

Report this case as type A thymoma. We consulted an expert physician and his advice on this specific case is to interpret it as a malignancy and report.

Use text fields to record the details of this case.

Assign code 22 for LUL wedge resection followed by a lingular-sparing LUL lobectomy and mediastinal lymph node dissection.  Code the lymph node surgery in Scope of Regional Lymph Node Surgery. We obtained input from an expert who agrees with this code. He states a lingula-sparing lobectomy is best coded as a segmentectomy because it is the same as an apical trisegmentectomy.

Abstract this case as a single primary and code histology as carcinosarcoma (8980/3). The carcinosarcoma is intermixed with the EIN making this a single primary coded to the invasive histology. EIN is a precursor of endometrial carcinoma in the WHO Classification of Female Genital Tumors, 5th edition. Carcinosarcoma of the uterus is described in the literature as an aggressive variant of endometrial carcinoma characterized by unusual histologic features including discrete malignant epithelial and mesenchymal components (carcinoma and sarcoma).

Instructions for First Course of Therapy include using the documented first course of therapy (treatment plan) from the medical record. While a patient may weigh in on the treatment decision, the physician is responsible for developing and managing the treatment plan including closely watching a patient’s condition but not giving treatment unless symptoms appear or change.

We can add language to a future manual to clarify.

Use the NAACCR Data Dictionary Required Status Table or refer to standard setter requirements. Do not use SEER*RSA to determine which data items are required to be collected or transmitted. Though "All" in SEER*RSA generally refers to the standard setters including CoC, NPCR, CCCR, and SEER, some items in SEER*RSA need updating; this is planned for 2022.

We found that the term AML with recurrent genetic abnormalities, NOS"was incorrectly included as an alternate name with code 9896/3. We followed back with our expert hematopathologist and he stated that this should have been coded to 9861/3 (AML, NOS), for AML with recurrent genetic abnormalities, NOS. This alternate name has been added to 9861/3. (Note: The same alternate name has been removed from 9896/3).

IDH-2 is not listed as a genetic abnormality for any of the histologies listed in the database. It could be that this is a new genetic marker for one of the AML with recurrent genetic abnormalities that we are not aware of. Without further clarification on which histology the IDH-2 would indicate, you would have to default to 9861/3.

There are several histologies that are grouped as AML with recurrent genetic abnormalities."All of these have specific genetics listed as part of the ICD-O-3 histology name.

9865: Acute myeloid leukemia with t(6;9)(p23;q34.1) DEK-NUP214

9866: Acute promyelocytic leukemia with PML-RARA

9869: Acute myeloid leukemia with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2, MECOM

9871: Acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11

9877: Acute myeloid leukemia with mutated NPM1 (2021+)

9878: Acute myeloid leukemia with biallelic mutation of CEBPA (2021+)

9879: Acute myeloid leukemia with mutated RUNX1 (2021+)

9896: Acute myeloid leukemia with t(8;21)(q22;q22.1); RUNX1-RUNX1T1

9897: Acute myeloid leukemia with t(9;11)(p21.3;q23.3); KMT2A-MLLT3

9911: Acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13.3;q13.1); RBM15-MKL1

9912: Acute myeloid leukemia with BCR-ABL1 (2021)+

Of note, for the above histologies, since these are diagnosed solely based on genetics, diagnostic confirmation will always be 3. This instruction will be added to the Hematopoietic database for the 2022 update.