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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.

This direction is also in SEER*RSA for Ovary. Researching a possible explanation for this, we found that LVI is an independent predictor of progression and survival in patients with primary epithelial ovarian cancer at early stage but not at advanced stage. However, studies also recommend that routine evaluation of LVI in ovarian cancer is highly recommended in daily practice.

The ICD-O-3.2 Implementation Guidelines and ICD-O-3.2 Coding Table indicate that insulinoma, NOS has changed behavior from /0 to /3 for cases diagnosed 2021 and later. However, the ICD-O-3.2 Implementation Guidelines do not indicate whether this change applies to tumors described as above. Insulinomas are generally neuroendocrine tumors/neoplasms, so it seems any neuroendocrine tumor described as an insulinoma should be collected as 8151/3, but does that apply to an epithelioid tumor/neoplasm also described as insulinoma?

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

It is unclear if code 300 is appropriate, since technically the fissure is comprised of pleura, involvement of the fissure appears to imply a tumor that is no longer localized.

An argument could be made for code 400, since the term traverses could be interpreted as crossing into adjacent lobe, however the lower lobe is not mentioned in this scan.

Example: Diagnosis is made on liver core biopsy path showing Metastatic carcinoma, poorly-differentiated, consistent with lung primary. Diagnosis Comment notes: Carcinoma cells are positive for CK7 and TTF-1, negative for CK20.

Subsequent immunohistochemistry report for PD-L1 testing states Liver: Metastatic adenocarcinoma consistent with lung primary. Interpretation: no PD-L1 expression.

IHC/Biomarker testing is often performed to determine treatment type, but it seems like some of the biomarkers for treatment planning are also histology specific. The Solid Tumor Rules do not address the use of biomarkers reports in the histology coding instructions.

Table 3 (Specific Histologies, NOS, and Subtypes/Variants) lists Ewing sarcoma as a synonym for Peripheral primitive neuroectodermal tumor 9364. Presumably, this is to be used for the reportable malignant peripheral nerve tumors when diagnosed as pPNET or Ewing sarcoma. However, this patient has a type of central (or CNS) primitive neuroectodermal tumor (histology 9473). Table 3 does not list central primitive neuroectodermal tumor (PNET or CPNET) as a valid histology for CNS tumors.

While Table 3 does not list all the possible histologies for the CNS, it currently is not clear how one would arrive at the histology code for a CNS Ewing sarcoma family tumor with CIC alteration, as this is recognized as a new entity for primitive neuroectodermal tumors of the CNS (i.e., PNET, histology 9473) per multiple journal articles. Ewing sarcoma family tumors include both peripheral PNET and central PNET tumors, but to code this histology as a peripheral PNET (9364) in this case seems incorrect when the primary tumor is stated to be of central nervous system origin, not peripheral nervous system origin.

Patient was diagnosed with Stage IV colon cancer (liver metstasiss) and started on Folfox with Avastin. The medical oncologist decided to continue maintenance treatment with Xeloda and Avastin.

Per Colon NCCN Guidelines Version 3.2019, interest in the use of maintenance therapy approach after first-line treatment of unresectable, metastatic colorectal cancer is growing. In general, this approach involves intensive first-line therapy, followed by less intensive therapy until progression in patients with good response to initial treatment.

Colon Therapy

5/1/18 Colonoscopy biopsy: mod diff colon adenoca, MMR proficient, BRAF wild type

5/5/18 Liver biopsy: mets from colon cancer

6/18/18 " 11/20/2018 Med Onc: started 12 cycles Chemo - Folfox (Fluorouracil, leucovorin, Oxaliplatin) with Avastin

11/28/18 CT Pelvis: continued improvement in the liver mets; no residual tumor involving colon; no new mas or adenopathy in the chest, abdomen or pelvis

12/02/18 Med Onc follow up: Pt had tremendous response to chemotherapy and Avastin, cancer is not curable. Is amenable to maintenance therapy with Xeloda and Avastin; also amenable to descending colectomy in the future

1/7/19 Med Onc: starting maintenance treatment Xeloda + Avastin.

Head and Neck Multiple Primary Rule M8 appears to be the first rule that applies to this case and instructs the user to abstract multiple primaries when separate/non-contiguous tumors are on different rows in the appropriate site table (Tables 1-9) in the Equivalent Terms and Definitions. Table 1 (tumors of the nasal cavity) shows Non-keratinizing squamous cell carcinoma and squamous cell carcinoma on different rows making the 2019 case a new primary. Is this correct?

The Extent of Disease (EOD) Manual states: Neoadjuvant (preoperative) therapy: If the patient receives neoadjuvant (preoperative) systemic therapy (chemotherapy, immunotherapy) or radiation therapy, code the clinical information if that is the farthest extension documented. If the post-neoadjuvant surgery shows more extensive disease, code the extension based on the post-neoadjuvant information.

The patient is a 34 year old who presented with testicular pain radiating into the abdomen approximately 1 month before orchiectomy in 2018. CT abdomen/pelvis: Multiple focal sclerotic bone lesions. Given the lack of change from July 2014, these are likely benign bone islands. No adenopathy mentioned. He has no prior history of germ cell tumor nor any surgery for any tumor/cancer before this. Pathology: Testis, left, radical orchiectomy: - Demarcated scar tissue (1.3 cm), with atrophic seminiferous tubules and cortical bone consistent with burnt-out germ cell tumor. No evidence of germ cell neoplasia in situ (GCNIS). - Margins are unremarkable.