Report | Question ID | Question | Discussion | Answer | Year |
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20150046 | Reportability--Appendix: Is the appendix the primary site for a low grade mucinous appendiceal neoplasm (LAMN) with diffuse peritoneal dissemination? See discussion. |
Patient had an appendectomy revealing a low grade mucinous appendiceal neoplasm (LAMN) with diffuse peritoneal dissemination. Patient now with cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC), which revealed metastatic disease in the abdomen, omentum, pelvic peritoneum, peri-cecal, and gallbladder. |
For cases diagnosed prior to 1/1/2022 Low-grade appendiceal mucinous neoplasm (LAMN) is not reportable, even when it spreads within the peritoneal cavity, according to our expert pathologist consultant. Peritoneal spread of this /1 neoplasm does not indicate malignancy. It is still /1 when there is spread of LAMN in the peritoneal cavity. |
2015 |
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20150021 | MP/H Rules/Histology--Skin: How is histology coded for an "endocrine mucin-producing sweat gland carcinoma with transformation to mucinous carcinoma"? See Discussion. |
Endocrine mucin-producing sweat gland carcinoma (EMPSCG) is a rare type of low-grade sweat gland carcinoma. Some journal articles indicate that most patients with EMPSCG have coexisting mucinous carcinomas, suggesting that EMPSCG is a precursor to invasive mucinous carcinoma of the skin. Sweat gland carcinoma has its own histology code per the ICD-O-3 (8400/3); should an endocrine mucin-producing sweat gland carcinoma also be coded as 8400/3? If so, would the correct histology for the skin case above be mucinous carcinoma (8480/3) per Rule H17? Conversely, if the terms "mucin-producing" are referring to mucin-producing carcinoma, and not referring to the sweat gland carcinoma, would the histology be coded 8481/3 (mucin-producing carcinoma)? |
Assign 8480/3.
There is no mixed ICD-O-3 code for EMPSCG. Both histologies are in the mucinous family: mucinous adenocarcinoma (8480/3) and sweat gland carcinoma (8400/3). Apply Other sites rule H17 and code the numerically higher ICD-O-3 code (8480/3).
Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare low-grade sweat gland carcinoma with a strong predilection to the eyelid region. It is histologically analogous to endocrine ductal carcinoma/solid papillary carcinoma of the breast and is characterized by a multinodular solid cystic mucinous tumor with immunoreactivity to neuroendocrine markers. Only 20 cases of this unusual tumor have been reported. |
2015 |
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20150041 | MP/H Rules/Multiple primaries--Breast: Does rule M10 apply in this situation?
L breast biopsy = INVASIVE DUCTAL CARCINOMA
L breast simple mastectomy = 2.0 cm INVASIVE DUCTAL CARCINOMA with an incidental finding of separate 1.0 cm INVASIVE LOBULAR CARCINOMA; pathologist specifically states the tumors are morphologically different. The tumors are both pure Ductal/pure Lobular. |
Yes, Breast rule M10 applies. This case is a single primary.
Follow the MP/H rules even though the "pathologist specifically states the tumors are morphologically different" so that situations like this are reported consistenty accross cancer registries, regions, and states for consistent national reporting. |
2015 | |
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20150054 | Primary Site--Skin: Should cutaneous leiomyosarcoma be coded to primary skin of site (C44_) or soft tissue (C49_)? |
Code cutanteous leiomyosarcoma to skin. Leiomyosarcoma can originate in the smooth muscle of the dermis. The WHO classification designates this as cutaneous leiomyosarcoma. The major portion of the tumor is in the dermis, although subcutaneous extension is present in some cases. |
2015 | |
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20150059 | Primary Site--Liver: What is the topography code for combined hepatocellular carcinoma/cholangiocarcinoma (M-8180/3) especially when there is no documentation that intrahepatic bile duct is the tumor site? Reports usually just indicate a liver mass(es) but since the intrahepatic ducts are within the liver, is the code C221 due to the cholangiocarcinoma component, thus making the case stageable? |
If there is no further information about where the cancer originated, assign C220. Use ICD-O-3 as the source for coding topography. The topography code associated with combined hepatocellular and cholangiocarcinoma (8180/3) is C220 when there is no other information available, according to ICD-O-3. |
2015 | |
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20150033 | MP/H/Histology--Lung: Would you code a lung primary of "non-small cell carcinoma with neuroendocrine differentiation" to non-small cell carcinoma (8046/3) or carcinoma with neuroendocrine differentiation (8574/3)? See discussion. |
The pathology report states "Right mediastinal mass: poorly differentiated non-small cell carcinoma with neuroendocrine differentiation." This is the only histologic confirmation of this lung primary that is collected. |
Code carcinoma with neuroendocrine differentiation (8574/3). MP/H rule H7 applies: code the higher ICD-O-3 code. There is non-small cell lung carcinoma (8046/3) and a carcinoma, NOS with neuroendocrine differentiation present (8574/3). |
2015 |
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20150038 | 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). |
2015 | |
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20150036 | Reportability/MP/H--Kidney: "Multilocular clear cell renal cell carcinoma." Would this be coded 8310? See discussion. |
Multilocular clear cell renal cell carcinoma is a specifc histologic type listed in the CAP cancer protocol for kidney, but not in the ICD-O-3 and it is not on the list of specific types of renal cell carcinomas in Table 1 of the kidney equivalent terms and definitions in the MP/H manual. There is a malignant multilocular cystic nephroma 8959 in Table 1, but I can't tell if this the same histology as what is stated in this path report. |
Apply Kidney rule H5 and code the clear cell (8310/3) which is the specific type of renal cell. Multilocular is a variant of clear cell which is a variant of renal cell carcinoma. As of yet, no new ICD-O morphology code as been proposed for this specific histology. It will be addressed in the revised rules. |
2015 |
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20150057 | Reportability--Brain and CNS: Is this diagnosis reportable? If this neoplasm originated in the spinal cord, it is reportable, correct?
Specimen is described as a 'spinal cord mass.' The final diagnosis is 'fragments of adipose tissue demonstrating vascular proliferations consistent with angiolipoma. No histologic evidence of malignancy.' The microscopic description says: Sections of the spinal mass reveal bone, cartilage, fibrous tissue and adipose tissue. The adipose tissue demonstrates increased vascularity with thin walled blood vessels seen with islands of delicate fibrous stroma. The histologic findings are compatible with fragments of angiolipoma. |
The neoplasm is reportable if it originated in the spinal cord or is intradural (within the spinal dura; spinal nerve roots are intradural). If there is not enough information to determine the exact site of origin, do not report the case. |
2015 | |
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20150019 | Reportability/Histology--Pancreas: Is well-differentiated neuroendocrine tumor (M8240/3) as stated on a pathology report reportable or can the clinical information be used as an adjunct to the path report, which further states the specific type of neuroendocrine tumor is an Insulinoma, therefore, NOT reportable (M8151/0)? See discussion. |
The diagnosis date is 2/24/14. The pathology report of the pancreas shows: Well-differentiated neuroendocrine tumor (NET), low grade (WHO G1 of 3). Addendum: Ki-67 confirms low grade of pancreatic endocrine tumor (less than 2% Ki-67/MIB-1 index). Chromogranin confirms the endocrine nature of the tumor. The Pre and Post Op Diagnosis is pancreatic neuroendocrine tumor consistent with insulinoma. AJCC Stage as noted on path report: pT1, pNX, pM.
The physician states: The patient has a well-documented insulinoma. Biochemistries confirmed the disease and it is localized in the tail of the pancreas.
The issue with NETs is that pathology report reflects what is seen or what is quantified under the microscope; often, there is a specimen without the accompanying medical history and clinical signs. Many of these NETs are specified on the basis of the hormone, as usually measured in the blood, that is overproduced, something not seen microscopically. All of the islet cell tumors are NETs. The insulinoma in the example above is a well-differentiated NET that is causing insulin to be over-produced. Thus, the diagnoses are not discordant; insulinoma is a more specific NET. |
When the pathology diagnosis is a neuroendocrine tumor (/3) and the clinical diagnosis is an insulinoma (/0), report the case. Although ICD-O-3 classifies insulinoma as /0, the most recent WHO classification lists it as /3. The pathologist and physicians for this case are likely guided by the WHO classification and as a result, would view both the NET diagnosis and the insulinoma diagnosis as malignant. You could report this case as 8240/3 or 8151/3. |
2015 |