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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).

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.

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. 

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.

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.

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.

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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.

Serous tubal intraepithelial neoplasm (neoplasia) (STIN) is not equivalent to serous tubal intraepithelial carcinoma (STIC). Report STIN only when stated to be high grade. STIC is reportable. Do not report STIL.

According to our expert pathologist consultant, STIL and STIN are broad descriptive terms that reflect proliferation of epithelial cells with varying degrees of atypia, with the most developed, STIC, reflecting convincing neoplastic change.

Abstract as multiple primaries per rule M8. There are bilateral fallopian tube primaries. It sounds like the "primary" tumor was identified in the right fallopian tube with bilateral spread of disease. Incidental STIC was also identifed in the left fallopian tube. Do not record the STIC as another primary.

We consulted with our expert hematopathologist who advised this is a single primary, Hodgkin lymphoma (9659/3).

The diagnosis from 5/14/2021 states NLPHL. It also states there is T-cell histiocyte rich large B-cell lymphoma-like transformation. The WHO Classification of Hematopoietic and Lymphoid Tissues demonstrates six different patterns to NLPHL, which are: A) 'classical' nodular, B) serpiginous/interconnected nodular, C) nodular with prominent extra-nodular LP cells, D) T-cell-rich nodular, E) diffuse with a T-cell-rich background, and F) diffuse, B-cell-rich pattern.

In this case, they are describing a NLPHL type E (diffuse with a T-cell rich background). The term used is "T-cell histiocyte rich large B-cell lymphoma-LIKE transformation. "Like" as used here means that it is like a transformation; if it was NLPHL transforming to T-cell histiocyte rich large B-cell lymphoma, it would not have the word "like" in the diagnosis. This is a variant of NLPHL and not an actual transformation to another lymphoma. Even though NLPHL can transform to T-cell histiocyte rich large B-cell lymphoma, it is not the case here since the word "like" appears in the diagnosis.

We will update the histology in the Hematopoietic and Lymphoid Neoplasm Database to include these additional patterns.