Reportability/MP/H Rules/Histology: Is malignant perivascular epithelioid cell tumor (PEComa) reportable, and if so, what is the histology code?
Malignant perivascular epithelioid cell tumor (PEComa) is reportable because it is malignant. Assign 8005/3 to malignant PEComa.
We consulted an ICD-O-3 expert who explained that some PEComas such as angiomyolipoma and lymphangiomyomatosis have specific ICD-O codes and their malignant counterparts may be coded to 8860/3 and 9174/3 respectively. There are no separate ICD-O codes for other specific PEComas, e.g., clear cell “sugar” tumor of lung, clear cell myomelanocytic tumor of the falciform ligament and some “unusual” clear cell tumors occurring in other organs—or for PEComa, NOS. These PEComas may therefore be coded to 8005 as clear cell tumors NOS; in other words as clear cell tumors that are not clear cell variants of carcinomas, sarcomas, or other specific tumor type.
Please note, PEComa is non-specific as to behavior. Unless the pathologist states that it is malignant, (as was the case for this question), the default code is 8005/1 (non-reportable).
Reportability--Ovary: Is an "aggressive adult granulosa cell tumor with one of two lymph nodes positive for metastatic granulosa cell tumor" reportable?
Malignant granulosa cell tumor is reportable. The case described above is malignant as proven by metastasis to the lymph node.
Reportability--Skin: Is malignant proliferative trichilemmal tumor (PTT) reportable, and if so, do we apply the matrix rule and code it to 8103/3? A literature search reveals these do exist, but are extremely rare.
Malignant PTT (8103/3) of the skin is not reportable. A neoplasm originating in the skin with histology coded to 8103 is not reportable. See 1.b.i. on page 7 in the 2018 SEER manual for a complete list, https://seer.cancer.gov/manuals/2018/SPCSM_2018_maindoc.pdf
MP/H Rules/Multiple primaries--Colon: Does rule M7 apply here (A frank malignant or in situ adenocarcinoma and an in situ or malignant tumor in a polyp are a single primary)? Can the frank malignant adenocarcinoma be any specific type of adenocarcinoma for this rule to apply?
A patient has 2 synchronous tumors in the ascending colon. The first is grade 3 adenocarcinoma with signet ring differentiation and focal mucinous features (8255/3). The second is grade 2-3 adenocarcinoma in a tubulovillous adenoma (8263/3).
M7 applies to this case. The frank adenocarcinoma can be a specific type of adenocarcinoma.
Solid Tumor Rules/Histology--Breast: What is histology code of a breast tumor with ductal carcinoma, lymphoepithelioma-like carcinoma type? See Discussion.
Example: 12/2023 Breast lumpectomy final diagnosis is Invasive ductal carcinoma, lymphoepithelioma-like carcinoma type. This is a single tumor with no in situ carcinoma present.
Lymphoepithelioma-like carcinoma is not listed as a subtype/variant or synonym for breast carcinoma in the Solid Tumor Rules histology tables.
Lymphoepithelial carcinoma is a subtype of SCC usually seen in skin or H&N sites and often associated with EBV. CPC SME review determined 8082/3 invalid for breast but did not recommend a substitute code. There were only 45 cases coded 8082 2001 to 2019. For this case, it's possible the lesion originated in the breast skin and progressed to breast tissue. SCC is a subtype of metaplastic breast carcinoma so one could argue it code be coded either 8575 or 8070. For this case, we recommend assigning 8500/3. Use text fields to record the details.
Reportability--Brain: Is benign lymphangioma of the brain (9170/0) reportable? It is not on the list of non-malignant blood vessel tumors in the National Program of Cancer Registries Clarifications for Central Nervous System (CNS) tumors.
Lymphangioma of the brain or CNS is not reportable. Lymphangioma is a malformation of the lymphatic system. Even though it has an ICD-O-3 code, do not report it.
Lymph nodes/Melanoma: Is a single axillary lymph node regional or distant for a patient diagnosed in 2018 with metastatic melanoma to the brain found via imaging. The staging procedure was an single axillary lymph node excision that was positive for metastatic melanoma. The exact site of the primary was never determined; the primary site is coded to C449. See Discussion.
The patient was diagnosed in 2018 with met melanoma to the brain found via imaging. The staging procedure was a single axillary lymph node excision which was positive for metastatic melanoma. The exact site of the primary was never determined and the site code is C449. Is the axillary lymph node regional or distant? This affects how I code regional lymph nodes positive, regional lymph nodes examined, and scope of regional lymph node surgery or surgical procedure other site. Similar question was asked in the past (question # 20091101) but I have not found this question restated since the 2018 changes and just want to verify this is still what we are to do.
Lymph node mets from a melanoma of unknown primary site are presumed to be regional if the lymph node mets are confined to one area, as they are in this case. We are assuming there are no previous melanoma diagnoses for this patient. The workup should include examination of the skin areas that drain to the axillary area.
Reportability--Ambiguous Terminology: Should either of the terms, strongly characteristic of or most certainly, be used to accession a case as reportable when they are used to describe a malignancy and no other information is available? See Discussion.
SINQ 20130140 indicates a histologic diagnosis that is characteristic of a specified malignancy is reportable because this is equivalent to the term, diagnostic of. Does the same logic apply to a clinical diagnosis that is strongly characteristic of a malignancy on imaging?
SINQ 20180104 indicates the term, almost certainly, is not a reportable ambiguous term. If a radiologist notes a mass was most certainly malignant, is this adequate to accession this as reportable? Is a clinically certain diagnosis equivalent to diagnostic of? Or are the modifiers almost and most irrelevant because the terms certainly and certain are not on the ambiguous terminology list?
Look for more information. What is the plan for each of these patients? Consult with the physician and search for further information to assist with the decision. If no further information can be obtained, accession both of these cases based on the imaging reports. If more information becomes available later, review and revise as applicable.
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