SEER Inquiry System - Search

Should the updated Note 5 from the Non-malignant CNS regarding optic nerve glioma also be incorporated into Note 6 for Malignant CNS rules (the pilocytic astrocytoma note)?

This was one of the major issues identified in the SEER*Educate Workshop. Registrars have demonstrated they do not consistently think to look at the Non-malignant CNS schema when they see the term glioma and continue to misclassify optic nerve gliomas as malignant.

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

Should histology be coded as paraganglioma, NOS (8680/3) or as extra-adrenal paraganglioma, NOS (8693/3) for a diagnosis of paraganglioma in the bladder? Does the pathologist have to specifically diagnose the tumor as extra-adrenal paraganglioma, NOS to use histology code 8693/3? Or, does any diagnosis of paraganglioma (NOS) arising outside of the adrenal gland, carotid body, middle ear, or aortic body (the specified sites for other types of paragangliomas) qualify as an extra-adrenal paraganglioma, NOS?

The ICD-O-3.2 Implementation Guidelines (Tables 6 and 7) provide an associated site of C755 for histology 8680/3 (paraganglioma, NOS), but no associated site code is provided for histology 8693/3 (extra-adrenal paraganglioma, NOS). If the preferred site for paraganglioma, NOS is the paraganglia, would a paraganglioma in the bladder be an extra-adrenal paraganglioma?

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

We received an edit error in SEER*DMS on the following site/histology (Orbit, NOS (C696)/9699/3) that involved an incorrect staging code being assigned to SS2018. The staging language is identical in AJCC, EOD and SS2018. SEER*RSA notes that SS2018 should be coded distant, but in the SS2018 manual, this language is noted Regional. Staging language is: Orbital adnexal lymphoma AND extraorbital lymphoma extending beyond the orbit to adjacent structures--Bone, Brain, Maxillofacial sinuses

The patient was clinically diagnosed with a pituitary adenoma on MRI in June 2009. The MRI noted an unusual contour involving the superior margin of the pituitary gland and the clinical interpretation was a small pituitary adenoma. The patient did not follow-up with the recommended repeat imaging and never received treatment for the pituitary adenoma.

The patient was eventually seen again in January 2020 and the MRI showed no adenoma in the pituitary gland. Since pituitary adenomas are known to spontaneously regress, should the 2009 diagnosis of pituitary adenoma be accessioned as a SEER reportable benign central nervous system (CNS) tumor?

According to the literature, PMH is a low-grade malignant vascular neoplasm of different tissue planes including skin and soft tissue. However, the references also state: PMH is a cutaneous tumor that behaves in an indolent fashion. There is no indication that this was a malignant diagnosis.

12/3/18 Foot, left skin lesion, punch biopsy: Superficial squamous epithelium demonstrating hyperkeratosis and fragments of keratin debris, no tumor seen.

Foot, left skin lesion, punch biopsy: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma, see note.

NOTE: The submitted immunohistochemical slides were reviewed. Positive and negative controls reacted appropriately. The tumor cells demonstrate immunoreactivity to CK AE1/AE3 and CK7. The CD31 immunoreactivity described in the report cannot be confirmed as only the positive control is submitted for review. The tumor cells are negative for desmin, CD45, CD68, S-100, CD34, SMA, CD20, and HHV8. The proliferative index via Ki-67 is approximately 10%. The morphology (described below) and immunohistochemistry performed are compatible with a pseudomyogenic hemangioendothelioma.

12/4/18 Final Pathologic Diagnosis: Foot, left bone lesion, biopsy: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma, see note.

Note: The patient's imaging findings were reviewed in conjunction with this case, revealing numerous lytic lesions of the tibia, fibula, talus, tarsal, metatarsal, and phalangeal bones. Additionally, as per the medical record, also reviewed in conjunction with this case, there are lesions of the skin. Thus, an extensive immunohistochemical panel was performed in an attempt to support the morphologic findings in this case, which were morphologically similar to the patient's skin biopsy. The tumor cells demonstrate strong immunoreactivity to pancytokeratin (CK AE1/AE3) and vimentin with moderate immunoreactivity to Fli-1. The tumor cells demonstrate weak immunoreactivity to epithelial membrane antigen. INI-1 is retained. There is focal immunoreactivity to CD31 although this is limited to the edges of the tissue fragments. The tumor cells are negative for HHV-8, CD34, smooth muscle actin, CK8/18, desmin, CD99, and Bcl-2. The combination of morphologic (see below for microscopic description) and immunohistochemical findings are consistent with pseudomyogenic hemangioendothelioma. Fresh tissue was submitted for karyotype analysis at the time of intraoperative consultation; however, it revealed only a normal appearing male karyotype. Thus, molecular confirmation was sought. The original slides and a paraffin block were submitted for FOSB rearrangement analysis, as pseudomyogenic hemangioendothelioma is known to have recurrent rearrangements with FOSB. Additional immunohistochemistry performed at (FACILITY) demonstrating immunoreactivity for ERG, supporting a vascular origin for this neoplasm. Fluorescence in situ hybridization demonstrated that 13% of the cells examined show FOSB rearrangement. While this FISH probe is for investigational purposes, the above findings support the diagnosis of pseudomyogenic hemangioendothelioma.

Rule M4 does not seem to apply because Note 1 defines clinically disease free to mean no evidence of recurrence in the same lung on follow-up. Patient had been disease free in the left lung after 09/2014 diagnosis. The 04/2019 diagnosis was in a different lung than the 4/2016 diagnosis.

The next applicable rule is either M11 or M14 depending on how we should compare the new 2019 tumor: to the most recent prior tumor in 2016 or to both prior tumors.

In the 2018 ICD-O-3 Update Table, undifferentiated high-grade pleomorphic sarcoma and undifferentiated high-grade pleomorphic sarcoma of bone (C40_) were both listed as a New Term for histology 8830/3. There was no site restriction for a diagnosis of undifferentiated high-grade pleomorphic sarcoma. Therefore, it appears the diagnosis could easily be applied to a soft tissue tumor. This histology is used by pathologists in our region for soft tissue tumors as well as bone tumors. However, in the ICD-O-3.2 Table an entry (or synonym) was not provided for a tumor outside the bone.

The ICD-O-3.2 Table only lists undifferentiated high-grade pleomorphic sarcoma of bone for site codes C40_ and C41_ as a synonym for histology 8830/3. This also is not listed in the ICD-O-3.2 Implementation Guidelines. As a result, it is unclear whether a diagnosis of undifferentiated high-grade pleomorphic sarcoma of the soft tissue can be coded to 8830/3 and/or can be a synonym for the preferred term (8830/3, Malignant fibrous histiocytoma). Can a diagnosis of undifferentiated high-grade pleomorphic sarcoma of the soft tissue be coded to 8830/3, C49_ as it was per the 2018 ICD-O-3 Update Table?

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

Race information is provided in the Centers for Medicare and Medicaid Services (CMS) linkage results. Oftentimes it matches what is coded in the database, but other times it does not.

In situations where the CMS (or other) linkage provides a race value that differs from the coded Patient set, are we to ignore the CMS stated race given the SEER Manual instructions indicating self-reported race has priority or should we add the different Race values from linkages as an additional race (ex. Race 02)?