| Report | Question ID | Question | Discussion | Answer | Year |
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20160076 | 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. |
2016 | |
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20160028 | MP/H/Histology--Sarcoma: How should Ewing Sarcoma/primitive neuroectodermal tumor (PNET) be coded for a 2012 case? See Discussion. |
SEER SINQ 20031051 applies to cases diagnosed before 2007 and advises: Code histology as 9260/3, Ewing sarcoma. Ewing sarcoma is a specific histology on the continuum of primitive neuroectodermal tumors. Code Ewing sarcoma as it is more specific than PNET, NOS.
For tumors diagnosed 2007 or later, refer to the MP/H rules. |
Apply 2007 MP/H rule H6 and assign the numerically higher ICD-O-3 code that reflects PNET (9364/3). According to the WHO Tumors of Soft Tissue and Bone, though Ewing sarcoma ICD-O-3 code is 9260/3, Ewing sarcoma with a higher degree of neuroectodermal differentiation present is classically termed peripheral neuroectodermal tumors (PNET). WHO does not offer guidance how to classify tumors stated to be Ewing sarcoma PNET.
Histology code 9364/3 is assigned for a Ewing/PNET that arises outside of the brain/CNS. Peripheral neuroectodermal tumor (PNET) and peripheral primitive neuroectodermal tumor (PPNET) are Ewing family tumors.
Histology code 9473/3 (PNET, primitive neuroectodermal tumor, central primitive neuroectodermal tumor, or supratentorial PNET) is only used for tumors arising inside the brain/CNS. |
2016 |
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20150037 | Reportablility--Breast: Is lobular neoplasia reportable as lobular carcinoma in situ? See Discussion. |
According to College of American Pathologists (CAP), lobular neoplasia is also known as lobular carcinoma in situ. In a previous SEER question 20041089, it was stated that they were not the same and should not be reported unless it was a Grade 3. I assume this has changed and we are to report lobular neoplasia as lobular carcinoma in situ, is this correct? |
For cases diagnosed 2021 or later Lobular neoplasia (LN II and LN III) and lobular intraepithelial neoplasia (LIN II and LIN III) are reportable and coded 8520/2. |
2015 |
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20150045 | MP/H/Histology--Thyroid: What is the histology code for primary site of thyroid cancer with the histology of papillary thyroid carcinoma, classical and oncocytic type? |
Code the histology to 8342/3, thyroid oncocytic (oxyphillic) papillary carcinoma. |
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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20150014 | Reportability--Brain and CNS: Is "Lhermitte-Duclos disease" is reportable? See discussion. |
The MRI states "Lhermitte-Duclos disease" but does not include "dysplastic gangliocytoma of cerebellum"; is this the same as "Lhermitte-Duclos dysplastic gangliocytoma of cerebellum (C716)"? |
"Lhermitte-Duclos disease" alone can be interpreted as "Lhermitte-Duclos dysplastic gangliocytoma of cerebellum (C716)" and reportable. The WHO classification for CNS tumors lists this entity as "Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease)" signifying that the terms are used synonymously. |
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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20150016 | Reportability--Stomach: Is a well-differentiated neuroendocrine tumor of the stomach reportable? |
Well-differentiated neuroendocrine tumor (NET) of the stomach is reportable. The WHO classification of digestive system tumors uses the term NET G1 (grade 1) as a synonym for carcinoid and well-differentiated NET, 8240/3. |
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 |
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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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