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20240061 | Heme & Lymphoid Neoplasms/Histology--Myelodysplastic Neoplasm: What is the histology code for myelodysplastic neoplasm with low blasts and SF3B1 mutation with primary site bone marrow (C421) diagnosed in 2023? |
Assign histology as 9982/3 (myelodysplastic neoplasm with low blasts and SF3B1 mutation). This is a new term for 9982/3. WHO Classification of Hematolymphoid Tumors, 5th edition, defines myelodysplastic neoplasm (MDS) with low blasts and SF3B1 mutation (MDS-SF3B1) as a myeloid neoplasm with cytopenia and dysplasia characterized by SF3B1 mutation and often ring sideroblasts. |
2024 | |
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20230016 | Solid Tumor Rules/Histology--Brain: How is histology coded for an anaplastic glioneuronal tumor, BRAF p.V600E mutant, WHO Grade III, diagnosed following a right temporal lobe resection in 2021? See Discussion. |
The patient has a history of ganglioglioma, WHO grade I, involving the deep right parietal lobe diagnosed on resection in 07/2012. Tumor recurrence in 2017 was treated with radiation. The patient then had right temporal tumor biopsy and resection 06/2021 with final diagnosis of anaplastic glioneuronal tumor, BRAF p.V600E mutant, WHO Grade III. Pathologist notes that the tumor demonstrates a ganglioglioma with frequent mitoses and possible vascular proliferation. Subsequent consult findings support an anaplastic glioneuronal tumor, compatible with progression of the patient's ganglioglioma that is post-irradiation. However, the pleomorphic and epithelioid areas are also reminiscent of pleomorphic xanthoastrocytoma, which may occur in combination with ganglion cell components. There is no related SINQ to code this histology. |
Assign histology as 9505/3. WHO Classification of Central Nervous System (CNS) Tumors describe ganglioglioma as a well-diffferentiated and slow-growing glioneuronal neoplasm. While WHO does not recognize the histology/behavior combination 9505/3, the 2021 CNS Solid Tumor Rules identify non-malignant tumors that have the potential of transforming to a malignant tumor (new primary). Ganglioglioma (9505/1) is listed with the transformed histology and instructs us to code as anaplastic ganglioglioma (9505/3). |
2023 |
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20240074 | Solid Tumor Rules/Histology--Head & Neck: How is histology coded for nasopharyngeal non-keratinizing squamous cell carcinoma, undifferentiated type? See Discussion. |
Example: Patient had a 2023 nasopharyngeal mass biopsy showing “Nasopharyngeal non-keratinizing squamous cell carcinoma, undifferentiated type.” The Head and Neck Solid Tumor Rules (STRs) do not include an H Rule that instructs us how to code histology when there are two subtypes/variants present for a head and neck primary, nor does the STR define undifferentiated carcinoma as a subtype/variant for 8072. The WHO Classification of Head and Neck Tumors states non-keratinizing nasopharyngeal carcinoma (non-keratinizing squamous cell carcinoma (SCC) is the most common subtype for nasopharyngeal ca, but that non-keratinizing can be subdivided into undifferentiated and differentiated subtypes. Should histology be 8020 (undifferentiated carcinoma) or 8072 (non-keratinizing SCC)? |
Assign histology as 8072 for non-keratinizing SCC, undifferentiated subtype. WHO Classification of Head and Neck Tumors, 5th edition assigns 8072/3 to squamous cell carcinoma, non-keratinizing, NOS in the nasopharynx. As the tumor exhibits a variety of architectural patterns and appearances histologically, they can be further classified as undifferentiated or differentiated subtypes. These subtypes do not change the histology code. |
2024 |
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20160049 | Grade/Sarcoma--Breast: Is the correct grade for high grade angiosarcoma of the breast a code 3 or 4? The breast usually uses a three grade system but sarcoma is not a typical histologic type of the breast. |
Assign grade code 4 using the sarcoma table. Nottingham or Bloom-Richardson (BR) Score/Grade does not apply to angiosarcomas. This is a good question and points out needed clarification of the grade rules. |
2016 | |
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20180103 | Histology/Grade--Small intestine: For a 2017 diagnosis, is the grade/differentiation field coded 1 or 9 when the diagnosis is well-differentiated neuroendocrine tumor (NET) (carcinoid)? It seems as though the term well-differentiated defines type of neuroendocrine tumor so they can diagnosis the carcinoid. See Discussion. |
5/15/17 Duodenal bulb, biopsy: Fragments of duodenal mucosa with well differentiated neuroendocrine tumor (carcinoid), extending to the edge of specimen and peptic duodenitis in the submitted tissue. No significant intraepithelial lymphocytosis. |
Assign grade code 1 for well-differentiated NET (8240/3). Well-differentiated is synonymous with NET, grade 1, according to WHO Classification of Tumors of the Digestive System. |
2018 |
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20180010 | Diagnostic confirmation--Heme & Lymphoid Neoplasms: Is Diagnostic Confirmation coded as 5 (positive laboratory test/ marker study) or code 8 (clinical diagnosis only) for a case that has a positive JAK2 mutation, and based on the results of the JAK2, the physician diagnosed the patient with polycythemia vera? There were no blood smears or bone marrow biopsies done. |
Assign diagnostic confirmation code 5 for a positive laboratory test/marker study. A note was added to the Hematopoietic manual to state that code 5 now includes cases with no histological confirmation but there is positive immunophenotyping or genetic studies. |
2018 | |
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20140082 | MP/H Rules/Histology--Testis: How should histology be coded for a testicular teratoma with somatic type malignancy (adenocarcinoma)? See discussion. |
11/8/2013 Rt orchiectomy: teratoma with somatic type malignancy (adenocarcinoma). 5/2/2014 Abdominal mass excision: metastatic teratoma involving matted lymph nodes. Patient age at diagnosis is 31.
Per web search, a teratoma with somatic type malignancy is a rare type of tumor. Should the histology be coded to 8140/3? This seems to conflict with SINQ 20120085, which indicates a testicular mature teratoma in an adult is malignant, and in this example, it was also the portion of tumor that metastasized. |
Assign code 9084/3, listed in ICDO as teratoma with malignant transformation.
Our expert pathologist consultant states that this is a very rare situation. The non-germ cell components are believed to arise out of the teratoma portions, and are seen in only of few percent of teratomas. They are given the designation "teratoma with somatic type malignancies" (WHO). |
2014 |
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20160038 | Birthplace/Place of birth, country: For patients originally born in a country that is currently listed as "historic only", where the original birth country now has a one-to-many relationship with the current country, how should the reported original birthplace be coded? (Example: Yugoslavia) |
Assign code for Europe, NOS (ZZE) for Yugoslavia, NOS, without further information. |
2016 | |
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20091006 | Primary site--Lung: What primary site code is used for bronchus intermedius? |
Assign code C340 [main bronchus].
The bronchus intermedius is the lower part of the main bronchus on the right side. The bronchus intermedius begins just below the point where the upper lobe bronchus branches off from the main bronchus. The bronchus intermedius branches into the middle lobe bronchus and the lower lobe bronchus.
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2009 | |
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20210050 | EOD 2018/Extension--Testis: How is Extent of Disease (EOD) Primary Tumor coded if it appears limited to testis on scrotal ultrasound and is treated with neoadjuvant chemotherapy prior to the orchiectomy when there is no residual tumor (staged as ypT0 disease) and in cases where there is residual tumor? See Discussion. |
Unless there is a biopsy that proves in situ tumor (EOD code 000, Tis) or extratesticular invasion into the scrotum, penis, or further contiguous extension (EOD code 700, T4), EOD Primary Tumor must be coded based on the PATHOLOGICAL assessment (orchiectomy). There are no other CLINICAL codes because the AJCC indicates imaging is not used for local T-categorization, and the EOD derives the AJCC TNM staging. If the case can not be coded to either EOD Primary Tumor codes 000 or 700 clinically, the only clinical code that seems to apply is 999 (Unknown). We are seeing more cases treated with neoadjuvant chemotherapy prior to orchiectomy, especially in patients with distant metastatic disease. The EOD Manual indicates that clinical evidence takes priority over pathological evidencewhen neoadjuvant treatment is given, unless the extent of disease following neoadjuvant treatment is greater than pre-treatment clinical findings. If the clinical and pathological information are the same, code the extension based on the clinical information. Do these general rules also apply to testis even though we cannot code CLINICAL findings for these tumors? If so, will EOD Primary Tumor be coded to 999 (Unknown) for any testis primary that is not in situ or invasive into the scrotum, etc., that is treated with neoadjuvant therapy? Or should the post-neoadjuvant PATHOLOGICAL assessment be coded for these tumors because the CLINICAL assessment would otherwise be unknown? How is the EOD Primary Tumor coded for the following two cases? 1. Left testicular mixed germ cell tumor, biopsy-proven metastasis to a supraclavicular lymph node. The left testis contained a small mass on scrotal ultrasound. The patient underwent neoadjuvant chemotherapy, and the post-treatment orchiectomy proved no residual primary tumor (ypT0). Is EOD Primary Tumor 999 because it is clinically unknown (even though it was clinically limited) or 800 (No evidence of primary tumor) because there was no pathological evidence of tumor following neoadjuvant treatment? 2. Right testicular mixed germ cell tumor with biopsy-proven inguinal lymph node metastasis. There was a palpable mass in right testis on physical exam (not described as fixed or involving scrotum). The patient underwent neoadjuvant chemotherapy, and the post-treatment orchiectomy proved a residual 2 cm tumor limited to the testis without lymphovascular invasion (LVI). Is EOD Primary Tumor 999 because it is clinicallyunknown or 200 (PATHOLOGICAL assessment only - Limited to testis WITHOUT LVI)? |
Assign code 999 to EOD Primary Tumor for testis when neoadjuvant therapy is given and clinical assignment is unknown and the extent of the primary tumor is not fully assessed due to post neoadjuvant treatment effect as with the two case scenarios. Both clinical examination and histologic (pathologic) confirmation are required by AJCC for clinical assessment and was not met in these scenarios. While EOD Primary Tumor is based on pathologic assessment, the EOD general instructions are to code the clinical information if that is the farthest extension when the patient received neoadjuvant systemic therapy unless the post-neoadjuvant surgery shows more extensive disease. As there is neoadjuvant treatment effect and there is no clinical assessment, the primary tumor cannot be fully assessed. |
2021 |
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