SEER Inquiry System - Search

Per the site validation list: https://seer.cancer.gov/icd-o-3/sitetype.icdo3.20190618.pdf#search=site%20validation, ill-defined sites (ILL-DEFINED C760-C768) does not include 8070- Squamous cell carcinoma as a valid histology. Therefore when a Cervical Lymph Node and Unknown Primary Tumor of the Head and Neck is submitted with a C760 and 8070/3, it requires an override be set.

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.

The ICD-O-3.2 Coding Table includes hybrid oncocytic chromophobe tumor as a related term for histology code 8317 (Renal cell carcinoma, chromophobe type). However, this related term is not discussed in the implementation guidelines as being a new term/reportable tumor. The Solid Tumor Rules do not indicate a hybrid oncocytic chromophobe tumor is reportable; however, if a registrar only looked at the ICD-O-3.2 Coding Table, it may seem as though this histology should be collected.

The term hybrid oncocytic chromophobe tumor was not included in the Solid Tumor Rules as a subtype/variant of RCC, or as an equivalent term for chromophobe RCC. There is a SINQ (20180047) that states not to report renal hybrid oncocytic tumor, despite the fact these tumors exhibit mixed features of both oncocytoma and chromophobe RCC. For cases diagnosed 2021 and later, should the clarification in the SINQ apply? Or should the ICD-O-3.2 Coding Table be used which indicates this is a reportable diagnosis? If the standard setters decided not to implement use of hybrid oncocytic chromophobe tumor for 2021, can clarification be added to the Solid Tumor Rules or Implementation Guidelines?

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

Is an M rule needed to address multiple tumors and Note 2 in Table 3? Does Note 2 in Table 3 apply when multiple tumors exist and one tumor contains only ductal carcinoma?

The M Rules currently confirm that a metaplastic carcinoma (whether it is involved with ductal or lobular) and a separate ductal carcinoma are separate primaries because these histologies are on different rows in Table 3 (separate primaries per M14). There is no specific rule regarding metaplastic carcinomas in the Multiple Tumors (M Rules) module, so presumably, the presence of a separate ductal carcinoma is not lumped into Note 2 in Table 3 for metaplastic carcinoma.

However, the note is confusing when there are multiple tumors involved because it appears to the registrars there are two options for coding the histology. To some registrars, the rules indicate it does not matter if the tumor is predominantly ductal carcinoma as long as some percentage of metaplastic carcinoma is present, code histology to metaplastic carcinoma. For other registrars, the presence of solely a ductal carcinoma in a second tumor is a separate primary from the separate metaplastic carcinoma.

The M rules and Note 2 need to clarify this issue to promote consistency.

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

If the term discontinuous foci means separate tumors, then rule M14 would apply making these multiple reportable tumors.

The Terms and Definitions Introduction discusses how these are older terms, but pathologists may still use them. In our region, pathologists do, in fact, still use these terms. Can these terms be added to Table 1? For registrars who do not reference the Introduction every time they code histology but go directly to Table 1, coding consistency would likely improve if such terms were added in the Table.

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

Per Mayo consulting pathologist, the final diagnosis on this right lung biopsy is: SMARCA4-deficient malignant neoplasm (see Comment). Comment: Sections show a poorly-differentiated malignant neoplasm without any apparent glandular, squamous, or stromal differentiation. The tumor near totally replaces the underlying lung tissue without recognizable underlying alveolar parenchyma. Immunohistochemical stains performed at Mayo Clinic (Oscar keratin, INSM1, NUT, S100, desmin and BRG1 protein encoded by SMARCA4 gene) demonstrate that the malignant cells are positive for Oscar keratin (rare cells only), synaptophysin (weak/patchy) and p63 (focal) while negative for the remaining antibodies tested. Of note, SMARCA4 stain is negative in the tumor cells. Thus, this tumor can be categorized as a SMARCA4-deficient malignant neoplasm, which is known to be an aggressive malignancy, likely represent a SMARCA4-deficient thoracic sarcoma, a recently described entity. SMARCA4-deficient carcinomas in the lung have been reported to be mostly adenocarcinomas or squamous cell carcinomas, which would not fit for this case. Please refer to a paper published by our group (Sauter JL et al. Mod Pathol 2017;30:1422-32.

Clinically, the patient was felt to have a pineocytoma (borderline tumor) on imaging, but the subsequent biopsy proved a pineal germinoma (malignant tumor). The Date of Diagnosis instructions state to code the month, day and year the tumor was first diagnosed, clinically or microscopically, by a recognized medical practitioner, but it does not indicate whether differences in behavior alter the diagnosis date.

For brain and central nervous system tumors, should the diagnosis date be the first date a tumor is SEER reportable? Or should the diagnosis date for those tumors ultimately proven to be malignant, be the date the malignancy was diagnosed?

CT a/p read by radiologist shows: "Bosniak 4 cystic lesion of right kidney." Follow-up MRI a month later reads "right kidney cystic lesion with enhancing mural nodule concerning for cystic renal cell carcinoma (RCC)." Urologist consult used the same wording of "Bosniak 4 cystic lesion" and "concerning for renal cell carcinoma." Treatment discussed but due to patient health status recommended repeat imaging. Repeat CT few months later reads: "cystic right renal lesion with enhancing nodule similar to most recent prior and suspicious for cystic RCC."

Though "suspicious for cystic RCC" per latest imaging is reportable, Bosniak 4 is "clearly malignancy, ~100% malignant" by definition, so is the case actually reportable with the first CT a/p date as date of diagnosis?

Example: Posterior fossa tumor resection final diagnosis was medulloblastoma, WHO Grade IV. The diagnosis comment notes the current tumor resection reveals large irregular reticulin-free nodules with streams of neoplastic cells in a fibrillary background in association with narrow reticulin-rich internodular strands of poorly differentiated neoplastic cells. Taken together, these findings are indicative of medulloblastoma with extensive nodularity. The diagnosis comment provided only one histology.

Per the 2018 Solid Tumor Manual, Malignant CNS, Priority Order for Using Documentation to Identify Histology instructions, an addendum or comment has priority over the final diagnosis. Although indicative is not listed on any ambiguous terminology list, is this an ambiguous diagnosis that must be ignored? Or does the diagnosis comment in this case provide a single, specific diagnosis of medulloblastoma with extensive nodularity?