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.
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.
Grade/Sarcoma--Breast: Is the correct grade for high grade angiosarcoma of the breast a code 3 or 4? The breast usually uses a three grade system but sarcoma is not a typical histologic type of the breast.
Assign grade code 4 using the sarcoma table. Nottingham or Bloom-Richardson (BR) Score/Grade does not apply to angiosarcomas. This is a good question and points out needed clarification of the grade rules.
Reportability/MP/H Rules/Histology--Ovary: What is the histology code for an ovarian tumor described as a mucinous borderline tumor, intestinal type?
Mucinous borderline tumor, intestinal type, of the ovary is not reportable. The behavior is /1. There is no applicable histology code for this histology when it ocurs in the ovary.
Grade/Histology--Digestive System: What is the grade for neuroendocrine tumor (NET) or neuroendocrine carcinoma (NEC) of gastrointestinal morphologies described as: 1) NET G1 (M8240/3) and NET G2 (M8249/3) or 2) neuroendocrine carcinoma, low grade (M8240/3) and neuroendocrine carcinoma, well differentiation (M8240/3) and neuroendocrine carcinoma, moderate differentiation (M8249/3)? The SEER Instructions for Coding Grade for 2014+, Coding for Solid Tumors section, #3 state: Code the grade shown below (6th digit) for specific histologic terms that imply a grade. NET and NEC are not included in the specific terms.
You may code grade as follows.
Grade 1 – NET G1 (M8240/3)
Grade 2 – NET G2 (M8249/3)
Grade 1 – neuroendocrine carcinoma, low grade (M8240/3) or neuroendocrine carcinoma, well differentiation (M8240/3)
MP/H/Histology--Pituitary: Would you code Crooke cell adenoma as 8272/0 pituitary adenoma?
Yes, code Crooke cell adenoma to 8272/0 pituitary adenoma. According to the WHO classification, it is a variant of adrenocorticotropic hormone (ACTH) producing adenoma (8272/0).
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."
Reportability/Behavior--Small intestine: Is a carcinoid tumor, described as benign, reportable? See Discussion.
A segmental resection pathology report states "benign mucosal endocrine proliferation consistent with a 0.3 cm duodenal carcinoid tumor." The diagnosis comment further states, "the separate small endocrine lesion is histologically benign, consistent with a 3 mm carcinoid tumor." This seems to be an example of a description of a microcarcinoid tumor referenced in SINQ 20160011. However, in this new case the pathologist specifically states the tumor is benign.
The WHO definition of microcarcinoid indicates this is a precursor lesion, which seems to indicate it is not malignant. However, SEER's previous answer stated we should report these tumors because the ICD-O-3 definition of carcinoid is 8240/3. Do you think that the mention of the term "benign" in the pathology report is actually related to the size of this lesion? Is the reference to benign mucosal endocrine proliferation referring to the WHO classification (making the case reportable as stated in SINQ 20160011), or is this a situation in which we should apply the Matrix Rule and the case is nonreportable?
This carcinoid tumor, described as benign, is not reportable. According to our expert pathologist consultant, this case is not reportable because the pathologist uses "benign" to describe the mucosal endocrine proliferation and based on that, the neuroendocrine cell proliferation is hyperplasia/benign - not reportable.
Reportability/Histology--Pituitary Gland: How are Rathke cleft cyst and Rathke pouch tumor distinguished and are they both reportable?
Rathke cleft cyst is not reportable. Cysts are not neoplastic. However, Rathke pouch tumor (C751, 9350/1) is a reportable neoplasm for cases diagnosed 2004 and later. The Rathke pouch is coded to the pituitary gland. Benign and borderline pituitary tumors have been reportable since 2004.
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.
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