Histology--Brain and CNS: What morphology code should be assigned to a low-grade glial/glioneuronal neoplasm? See Discussion.
Pathology Diagnosis: Left temporal lesion - Low grade glial/glioneuronal neoplasm BRAF mutant. Pathologist Comment: The histopathological appearance of this lesion does not allow for a definitive diagnosis. However, the low-grade appearance, fibrillary nature, immunohistochemical profile, and the presence of a BRAF V600E mutation allow this to be categorized as a low-grade glial or possibly glioneuronal tumor. Despite the lack of exact classification this neoplasm can be expected to behave in a very indolent manner consistent with a WHO grade I classification.
Assign 9413/0 for glioneuronal neoplasm.
We consulted with our expert neuropathologist about the histology "glioneuronal neoplasm." This term is relatively new and has not yet been recognized by WHO or assigned an ICD-O code. Until such time that WHO determines a code for this neoplasm, our expert instructed us to use 9413/0. Since this is not a recognized neoplasm it is not included in the solid tumor rules.
Solid Tumor Rules/Histology--Brain and CNS: What is the histology code for low grade glioma? See Discussion.
Patient has a 3 cm tumor in the temporal lobe of the brain. This was noted on MRI 12/2022. The radiologist states this is a low-grade glioma and recommends following with routine scans. No pathology or resection performed or planned. Patient has been followed with imaging every six months with stable disease. Low grade glioma is not currently listed in ICD-O-3.2 or the current Solid Tumor Rules. What histology should be assigned to the case?
Assign 9380/1 for low grade glioma diagnosed 1/1/2018 forward and for low grade glioma diagnosed prior to 1/1/2018 assign code 8000/1 on the advice of our expert neuropathologists. The site/type combination of C71 _ and 9380/1 will flag histology/site/behavior edits which should be overridden.
Low grade glioma is an umbrella term or non-specific diagnosis, primarily seen on radiologic reports such as CT scans and MRIs. Often, the patient is actively followed with scans and surgical intervention delayed or not recommended. WHO Classification of Central Nervous System Tumors, 5th edition, does not recognize this term and indicates that tissue diagnosis (including genetic testing) is needed to provide a specific diagnosis. Since biopsy of these “neoplasms” is not routinely done, a definitive diagnosis is not available. Literature searches yielded conflicting information with some stating low grade gliomas are malignant with an indolent clinical course while others felt they were benign. Until such time as WHO proposes a code for this neoplasm, our expert neuropathologists recommend coding glioma, NOS with borderline behavior 9380/1.
Histology/Behavior--Brain and CNS: How are histology and behavior coded for a diagnosis of pineal anlage tumor in an infant? See Discussion.
Patient is an 11 month old with brain biopsy showing final diagnosis of pineal anlage tumor. How are behavior and histology coded for this rare tumor?
Assign 9362/3 for pineal anlage tumors. According to the WHO Classification of Tumors of the Central Nervous System, 4th edition, pineal anlage tumors, while extremely rare, share features with pineoblastoma. Although they have a distinct morphology, there is no other ICD-O-3 code for pineal anlage tumors.
MP/H Rules/Histology--Testis: How should histology be coded for a testicular primary with a combination of teratoma, yolk sac tumor and embryonal carcinoma? See discussion.
Patient had a radical orchiectomy with the final diagnosis of "Mixed germ cell tumor with the following features -- histologic type: Mixed germ cell tumor (teratoma 50%, yolk sac tumor 25%, and embryonal 25%)."
Assign 9085/3. Code this combination of teratoma, yolk sac tumor, and embryonal tumor in the testis to mixed germ cell tumor (9085/3) based on the WHO Classification of Tumors of the Male Genital Organs.
MP/H Rules/Histology--Sarcoma: What is the appropriate histology code for a final diagnosis of undifferentiated pleomorphic sarcoma and/or pleomorphic sarcoma, undifferentiated? See Discussion.
Does the Other Sites MP/H Rule H17 apply in this case, which results in coding the higher histology 8805/3 (undifferentiated sarcoma)? Or does the "undifferentiated" statement only refer to grade, which results in coding histology to 8802/3 (pleomorphic sarcoma)?
Assign 8802/34 to pleomorphic cell sarcoma/undifferentiated pleomorphic sarcoma. Pleomorphic is more important than undifferentiated when choosing the histology code in this case. Undifferentiated can be captured in the grade code.
MP/H Rules/Histology--Skin: What histology code and MP/H Rule apply to a skin primary with the final diagnosis, ? See Discussion.
The patient had an upper arm shave biopsy with final diagnosis of basaloid carcinoma with squamous and neuroendocrine differentiation. The pathologist also comments: Further resection was negative for residual malignancy.
Would SINQ 20150033 apply, thus resulting in final histology of carcinoma with neuroendocrine carcinoma (8574/3)?
Assign 8574/3 according to Other Sites rule H17 for basaloid carcinoma with squamous and neuroendocrine differentiation.
There is no combination code that includes basal cell, squamous, and neuroendocrine. We can combine basal cell with squamous, 8094/3, or carcinoma with neuoendocrine differentiation, 8574/3. Rule H17 directs us to assign the higher code, 8574/3.
MP/H Rules/Histology--Endometrium: What is the correct histology code for an endometrial cancer described as "Adenocarcinoma with areas of squamous differentiation?"
Assign 8570/3 to adenocarcinoma with squamous differentiation of the endometrium. The most recent WHO classification does not list "adenocarcinoma" for tumors of the uterine corpus. WHO does state that "endometroid carcinoma of the usual type is a glandular neoplasm..." Further, WHO states "Endometroid carcinoma typically displays a glandular or villoglandular architecture..." Based on the WHO classification, the use of the term "adenocarcinoma" in this context can be interpreted as endometroid carcinoma.
MP/H Rules/Histology--Prostate: What is the histology code for a prostate case whose histology reads “adenoca with mixed ductal and acinar variants?
Assign 8523/3.
The 2013 revision to ICD-O-3 has a new code for mixed acinar ductal carcinoma; however, this new code will not be implemented in the U.S. until 2018 or later. Page 7 of the Guidelines for ICD-O-3 Update Implementation document released by NAACCR 1/1/2014 instructs us to use 8523/3 in the meantime.
MP/H Rules/Histology--Pancreas: What is the histology code of invasive adenocarcinoma, non-mucinous with intraductal tubulopapillary features, moderately differentiated, from the pathology report final diagnosis of the pancreas? Does 'intraductal" refer to a non-invasive/in-situ component or describe the pattern of growth?
Assign 8503/3, intraductal papillary adenocarcinoma with invasion, to capture the more specific features of the adenocarcinoma. Histology Rule H13 for Other Sites states to code the most specific histologic term. Examples include Adenocarcinoma and a more specific adenocarcinoma. Note: The specific histology may be identified as type, subtype, predominantly, with features of, major, or with ___ differentiation.
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