Reportability--Brain and CNS: Is a thalamic amyloidoma reportable if so what histology code is used?
Thalamic amyloidoma is not reportable. Amyloidoma (tumoral amyloidosis, amyloid tumor) is a tumor-like deposit of amyloid. It is not neoplastic. Amyloid is a protein derived substance deposited in various clinical settings.
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.
Summary Stage 2000--Lymphoma: How is SEER SS2000 coded for an ocular adnexal lymphoma when it extends from the primary site to adjacent sites that are still orbital structures? See Discussion.
In this case, the lymphoma arose in the posterior orbit and the primary site was coded as C696 (orbit, NOS). The mass directly extended to at least one "adjacent" site, the lacrimal gland. Should SS2000 be coded to 1 (localized) or 5 (regional, NOS) when an ocular adnexal lymphoma arises in the posterior orbit and extends to involve the lacrimal gland? Although both the posterior orbit and the lacrimal gland are parts of the orbit, they have separate ICD-O-3 topography codes. Should extension to multiple sites within the orbit be classified as localized disease?
The issue is what constitutes "adjacent" structures for a tumor that arises in the orbit. In an article published by the Indian Journal of Opthamology it states, "According to the Ann-Arbor staging system, lymphoma confined to the orbit is designated as Stage I, involvement of adjacent structures (sinuses, tonsil and nose) makes it Stage II." Does SEER agree with this definition of "adjacent" structures? Or are the lacrimal gland, ciliary body, retina, conjunctiva and/or choroid "adjacent" structures for a lymphoma stated to arise in the posterior orbit?
Assign SEER SS2000 code 5 (Regional, NOS) for a lymphoma of orbit extending to lacrimal gland. In SEER SS2000, this is Stage IIE: Direct extension to adjacent organs or tissues.
Reportability--Thyroid: Is a final diagnosis of "non-invasive follicular thyroid neoplasm with papillary-like nuclear features" (NIFTP) reportable when the diagnosis comment states this tumor was historically classified as encapsulated follicular variant of papillary thyroid carcinoma? See Discussion.
The term "non-invasive follicular thyroid neoplasm with papillary-like nuclear features" is now being used, instead of the previous classification of an encapsulated malignant thyroid tumor. Recent evidence supports a very minimal risk of aggressive behavior for these tumors, and pathologists in our area are no longer classifying these as malignant in the final diagnosis.
As of January 1, 2021
Non-invasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP) C739 is no longer reportable for cases diagnosed 1/1/2021 forward. See the ICD-O-3.2 material on the NAACCR website,https://www.naaccr.org/icdo3/#1582820761121-27c484fc-46a7
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Answer for cases diagnosed 1-1-2017 to 12/31/2020
Report NIFTP and assign ICD-O-3 morphology code 8343/2. See the NAACCR document, page 3, https://20tqtx36s1la18rvn82wcmpn-wpengine.netdna-ssl.com/wp-content/uploads/2017/01/What-You-Need-to-Know-for-2017.pdf
Reportability--Carcinoid: Is a diagnosis of carcinoid heart disease, based solely on clinical information and no pathology, reportable?
Carcinoid heart disease is not reportable but this diagnosis indicates that the patient likely has a carcinoid tumor which may be reportable. Obtain further information.
SEER Summary Stage 2000--Melanoma: Can Clark's level classification still used to Summary Stage melanoma? It was previously used by AJCC TNM, but was not included in the 7th edition. I see it is still listed in the CAP protocols for melanoma.
Clark's level can be used to assign in situ, localized or regional summary stage.
If there is a discrepancy between the Clark’s level and the pathologic description of extent, use the higher Summary Stage code.
Grade--Head & Neck: How should grade be coded for a tonsillar primary (or other solid tumor) with resection pathology final diagnosis of poorly differentiated SCC with histologic grade: G2-3 of 3. See discussion.
We are seeing multiple head and neck cases with unclear or multiple grade assignments. Another example is alveolar mucosa SCC with histologic grade stated as: Moderately differentiated (G2 of 3). Grade Coding for Solid Tumor instruction 5.b. is not clear regarding this situation. Does a statement of differentiation take priority? Should we disregard the differentiation statement and code using the 3-grade systems?
Use the three-grade system table in instruction #7.b to code grade for the situations you describe. Use the Grade Coding Instructions in order. Instruction #7.b (three-grade system) comes before instruction #8 (terminology).
MP/H Rules/Histology: What is the correct histology code for a NUT midline carcinoma?
Code histology to 8010/3.
NUT carcinoma is identified by the NUTM1 gene rearrangement.
NUT midline carcinomas (NMC) are lethal and morphologically indistinguishable from other poorly diff carcinomas. They are epithelial tumors which can range from undifferentiated carcinomas to carcinomas with prominent squamous differentiation.
A new proposed ICD-O-3 code has been suggested for NUT tumors but it is not yet approved for implementation. Do not use the new code until it is approved for use in the United States.
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.
Reportability/Histology--Head and Neck: Is mammary analogue secretory carcinoma (MASC) of the left submandibular gland reportable and how is it coded? See Discussion.
The physician is calling it an indolent tumor, pT3/NX/M0 stage 3 with positive margins. Is the correct code C509, 8502/3?
Mammary analogue secretory carcinoma (MASC) is reportable. MASC is a recently described tumor that predominantly arises in the parotid gland. In this case, if the primary site is submandibular gland, assign C080. We contacted our expert pathologist and he stated that the best code to use for MASC is 8502/3. Override any edits triggered by the combination of C080 and 8502/3.
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