Reportability--Breast: Is mammary fibromatosis reportable and if so, what histology code is assigned? See discussion.
The pathologist completed a CAP protocol using soft tissue. Pathology revealed a 2.5 cm tumor with invasion of skeletal muscle with deep margins positive for tumor.
Mammary fibromatosis is not reportable. The WHO classification for breast tumors assigns mammary fibromatosis a behavior code of /1. According to WHO, mammary fibromatosis "is a locally infiltrative lesion without metastatic potential…"
Primary site/MP/H Rules/Histology: What is the appropriate site and histology code for a tumor described as a "Large mass In suprasellar cistern encroaching into sphenoid & ethmoid sinuses", with the pathology described as "INI-1 deficient sinonasal undifferentiated carcinoma"? Of note, this patient has a history of a pituitary adenoma, resected overseas a few months prior to this diagnosis.
The primary site is unclear. The lesion is intracranial, but this may not be the primary site. In the absence of any additional information, assign C390, 8020/3. According to WHO, sinonasal undifferentiated carcinoma can involve the nasal cavity, maxillary antrum, and/or ethmoid sinus.
SMARCB1 (INI-1) is a tumor-suppressor gene located on chromosome 22q11.2. Tumors that showed loss of expression were SMARCB1-deficient tumors which are characterized by nests, sheets, and cords of cells without any histologic evidence of specific (eg, squamous or glandular) differentiation.
Grade--Kidney: Should WHO/ISUP grade for renal cell carcinoma be coded for cases diagnosed 2016 and later? See discussion.
The 2016 WHO Classification of Tumours of the Urinary System appears to be moving away from using Fuhrman grading toward using WHO/ISUP grade. These seem like similar 4 grade staging systems; however, the SEER Manual specifically states to not use the Special Grade System table for WHO/ISUP. We are seeing the WHO/ISUP grade being used on 2016 pathology reports.
Examples of new grading for renal cell carcinomas
Histologic type: Clear cell renal cell carcinoma
Histologic grade (WHO/ISUP 2016): Grade 3 in a background of 2 (of 4).
And
Histologic type: Clear cell renal cell carcinoma
Histologic grade (ISUP): Grade 2.
Do not record WHO/ISUP grade in the grade/differentiation field.
Designated fields for this grade system are being proposed for future implementation.
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.
MP/H Rules/Histology--Breast: What histology code(s) and MP/H rule applies for a breast resection final diagnosis of "undifferentiated sarcoma associated with a malignant phyllodes tumor and a tumor size of approximately 7 x 6.5 x 4 cm"? (The tumor is primarily sarcoma, with the phyllodes tumor measuring 2.8 cm)? See Discussion.
Patient has a diagnosis of undifferentiated sarcoma with an associated malignant phyllodes tumor in a single mass. Should this be abstracted as two primaries, one for an undifferentiated sarcoma and the other for a malignant phyllodes tumor? Which MP/H rule applies?
Abstract a single primary. Based on the information provided, this is a single tumor, and therefore a single primary, Rule M3. Code the histology to malignant phyllodes tumor.
According to our expert pathologist consultant, "The presence of a phyllodes tumor component identifies the whole thing as such. Stromal overgrowth/sarcoma is the usual identifier of malignancy in a phyllodes tumor. (If there were no phyllodes component we would be left with undifferentiated sarcoma, but that is not the case here. The diagnosis of malignancy in phyllodes tumor may be difficult/problematic when there is no overt stromal/sarcoma overgrowth as in this case.) As an aside, the behaviors of pure sarcoma and a phyllodes tumor such as we have here are similar, but we would lose the primary diagnosis if we just called this sarcoma."
Surgery of Primary Site--Breast: If the diagnosis is a single primary involving both breasts, do we code 41 Surgery Primary site with 1 in Surgery Other site, or code 76 Surgery Primary site with 0 in Surgery Other site? See discussion.
In Appendix C- Breast (SEER Manual 2015) it states under the codes for TOTAL MASTECTOMY (Codes 40-49, 75): For single primaries only, code removal of involved contralateral breast under the data item Surgical Procedure/Other Site (NAACCR Item # 1294). [SEER Note: Example of single primary with removal of involved contralateral breast--Inflammatory carcinoma involving both breasts. Bilateral simple mastectomies. Code Surgery of Primary Site 41 and code Surgical Procedure of Other Site 1.] HOWEVER, underneath that it states code 76 is used for: 76 Bilateral mastectomy for a single tumor involving both breasts, as for bilateral inflammatory carcinoma. So
Assign code 41 with 1 in surgery other site for simple mastectomy. Assign code 76 with 0 in surgery other site for a more extensive mastectomy.
Reportability--Skin: Is this a reportable skin cancer? See discussion.
Patient had a skin biopsy and this is the interpretation: NASAL SUPRATIP: INVASIVE BASAL CELL CARCINOMA OF SKIN WITH NEUROENDOCRINE DIFFERENTIATION
NOTE: The deep margin is positive for tumor; peripheral margins negative for tumor. The tumor has a basaloid appearance with focal areas appearing slightly squamoid, and it demonstrates myxoid/mucinous retraction from the stroma. It does not demonstrate peripheral palisading of cells within tumor nests and has nuclear chromatin which suggests neuroendocrine differentiation. Mitotic rate is more brisk than typical basal cell carcinoma as well. The differential diagnosis includes basal cell carcinoma with or without neuroendocrine differentiation, basal cell carcinoma with squamous differentiation, basaloid squamous cell carcinoma, Merkel cell carcinoma and metastatic small cell carcinoma. The tumor is further characterized per immunostains x 9 (controls work well). Tumor cells are positive for Ber EP4 and p63; focally positive for Chromagranin; while negative for EMA, CK20, CK7, TTF-1, CD56 and Synaptophysin. Overall, the staining pattern supports basal cell carcinoma with neuroendocrine differentiation.
Basal cell carcinoma with neuroendocrine differentiation of the skin is not reportable to SEER.
In this case, the pathologist discussed several possible options, and determined that the final diagnosis is basal cell ca with neuroendocrine diff based at least partially on the immunostains.
MP/H Rules/Histology--Appendix: What is the histology for an appendix resection diagnosis of "Malignant neoplasm of the appendix with the following features: Histologic type: Adenocarcinoma ex goblet cell carcinoid with mucin production (adenocarcinoma arising from goblet cell carcinoid)"? Is this histology best coded to a mixed adenocarcinoma/carcinoid tumor (8244/3)?
Code histology to combined carcinoid and adenocarcinoma (8244/3). The tumor is a mix of adenocarcinoma and carcinoid.
Diagnostic confirmation: When a CT guided Fine Needle Aspiration is performed and the pathology report indicates smears and cell block were prepared, if the diagnosis is positive for cancer, can you code diagnostic confirmation as 2 (positive cytology) because of the cell block?
Yes, assign diagnostic confirmation code 2 for diagnosis based on smears and cell block from CT guided FNA. This reply pertains to solid tumors.
MP/H Rules/Histology--Brain and CNS: What is the histology code for a tumor originating in the cerebellum and extending into the fourth venrticle described as a glioblastoma with primitive neuroectodermal tumor component (WHO Grade IV)?
The WHO Classification of CNS tumours lists glioblastoma with primitive neuroectodermal tumor component as a subtype of glioblastoma and assigns 9440/3. Also referred to as glioblastoma with a primitive neuronal component.