SEER Inquiry System - Search

Patient had a vulvar biopsy with final diagnosis of Extramammary Paget neoplasm (intraepithelial glandular neoplasm). No invasion identified. We are unable to contact the pathologist or physician for clarification.

Although this terminology is not listed in the ICD-O-3, web search results refer to this as a possible synonym for Paget disease with associated VIN III, which is reportable.

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.

Bone marrow, right iliac crest (aspirate smear, touch preparation, clot section and core biopsy): Hypercellular marrow (40-50%) with plasma cell neoplasm (see Comment): " No evidence of metastatic carcinoma. " Adequate iron storage.

Comment: CBC data shows normocytic anemia. Flow cytometric analysis of bone marrow detects a kappa restricted plasma cell population that expresses CD138 and CD38. CD56 is positive. CD19 and CD20 are negative. T lymphocytes are immunophenotypically unremarkable. Polyclonal B lymphocytes are detected. Blast gate is not significantly increased. Immunohistochemical stains are performed on the biopsy core and clot section for greater sensitivity and further architectural assessment with adequate controls. CD138 positive plasma cells comprise > 70% of the total cellularity. AE1/AE3 is negative. Taken together, the morphologic and immunophenotypic findings are consistent with a diagnosis of plasma cell neoplasm. Trilineage hematopoietic activity as are seen.

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?

Table 1 of the 2018 Kidney Solid Tumor Rules (STR) lists succinate dehydrogenase-deficient renal cell carcinoma as histology code 8312, but in the ICD-O-3.2 Coding Table it is listed as histology code 8311.

No changes were made in the Kidney STR. As a result, the histology change described in the ICD-O-3.2 Coding Table conflicts with Table 1. Succinate dehydrogenase-deficient renal cell carcinoma (SDHD) is listed in Table 1 as a synonym for renal cell carcinoma, NOS (8312). However, the ICD-O-3.2 Coding Table lists this as a related term for histology code 8311/3. This related term was not discussed in the Implementation Guidelines, and no change was noted in the STR.

While it seems we should continue to follow the STR, without clarification as to why this histology change was not implemented in STR, achieving consistency will be problematic if registrars jump straight to the ICD-O-3.2 Coding Table to code histology for cases diagnosed 2021 and later. If this code cannot be used for cases diagnosed prior to 2021, should that clarification be included in the STR?

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

For example, a patient has a 2.6 cm breast tumor on MRI; a core biopsy measuring 0.7 cm is positive for infiltrating duct carcinoma. Rule #1 states "Use the largest measurement of the primary tumor from physical exam, imaging, or other diagnostic procedures before any form of treatment." However, Rule #9 seems to imply that size from an "incisional biopsy" takes precedence over imaging, even though it is known to be less than the entire tumor in size.

Pituitary blastoma was not added to Table 3 (Specific Histologies, NOS, and Subtypes/Variants) of the 2018 Malignant CNS and Peripheral Nerves Solid Tumor Rules as part of the December 2020 update. This is a new malignant CNS histology for 2021 and later. Not including this histology in Table 3 results in the registrars being required to check another source to correctly code this histology. If this histology cannot be used for cases diagnosed prior to 2021, should that diagnosis year clarification be included in the STR?

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

The bone marrow biopsy proved bone marrow with blasts comprising 15-19%. Neither the pathologist nor the physician specifically diagnosed this as AML, calling this only high risk disease or early evolving AML prior to starting the patient on Vidaza.

No further information can be obtained from the pathologist or the physician for this case. Should this early evolving AML be accessioned as an additional primary per Rule M10, or is this the same MDS that is now high risk as the blast count is up to 19%, but has not yet reached the threshold of 20% blasts usually required for AML per the Hematopoietic and Lymphoid Neoplasm Database?

Patient had a radical pharyngectomy showing a glossotonsillar sulcus tumor with high grade squamous cell carcinoma and adjacent high grade mucoepidermoid carcinoma. The pathologist commented, the tumor is composed of high grade mucoepidermoid carcinoma and high grade conventional-type squamous cell carcinoma that are immediately adjacent to one another. Given that the tumors are arising so close together and could represent a single neoplastic process with divergent morphologies, they are staged together.

Employing Solid Tumor Manual Rule M1 (single primary if unable to determine if there is a single or multiple tumors), it was determined that this should be reported as a single tumor because the pathologist referred to the case as both a tumor singular and tumors pleural. However, the Solid Tumor Manual Histology Rules for a Single Tumor do not appear to have an instruction for coding this histology combination.

Penile mass excision shows final diagnosis of squamous cell carcinoma, verrucous type. Subsequent partial penectomy has a final diagnosis of squamous cell carcinoma, verrucous type and the summary cancer data lists

Both the final diagnosis and summary cancer data indicate a histology code of 8051/3 (squamous cell carcinoma, verrucous type / verrucous carcinoma). However, this site and histology combination triggers edit IFN4911. Edit documentation indicates that for sites C600-C609 (all penile sites) use histology code 8051 and do not use 8054.

Review of the 2018 ICD-O-3 Histology Updates table does not indicate these terms are synonymous.