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Multilocular clear cell renal cell carcinoma is a specifc histologic type listed in the CAP cancer protocol for kidney, but not in the ICD-O-3 and it is not on the list of specific types of renal cell carcinomas in Table 1 of the kidney equivalent terms and definitions in the MP/H manual. There is a malignant multilocular cystic nephroma 8959 in Table 1, but I can't tell if this the same histology as what is stated in this path report.

Example: Right breast core biopsy found ductal carcinoma in situ in the upper outer quadrant. Subsequent resection has a final diagnosis of invasive mucinous carcinoma, grade 1, measuring approximately 7 mm, with close margins. See staging summary. Gross description mentions only the primary tumor with associated marker clip from previous biopsy.

Breast Cancer Staging Summary lists (testing and margins removed for brevity):

Procedure type: Lumpectomy.

Specimen laterality: Right.

Tumor size: 7mm.

Histologic type: Invasive mucinous carcinoma.

Histologic grade (Nottingham histologic score): Grade 1, (score 5/9).

Tumor focality: Single focus.

Lymph-vascular invasion: Not identified.

Treatment effect: No known therapy.

Ductal carcinoma in situ (DCIS): Present.

Architectural pattern: Cribriform.

Nuclear grade: Grade 1.

Necrosis: Not identified.

Calcifications: Not identified.

Estimated size/extent of DCIS: Spanning an area measuring 15mm.

Pathologic stage: pT1b, pNx. (AJCC 8th ed).

Distant metastasis: Not applicable.

Additional findings: Background lobular carcinoma in situ (LCIS), flat epithelial atypia (FEA), and atypical ductal hyperplasia (ADH).

The patient has two, separate, non-contiguous tumors. One tumor is a benign meningioma and the other is a malignant oligodendroglioma.

The original plan was not to treat the asymptomatic meningioma. However, after worsening symptoms, imaging and resection proved a separate left frontal lobe malignant tumor.

Rule M5 is the only M Rule in the Malignant CNS Multiple Primary Rules, Multiple Tumors module that addresses separate non-malignant and malignant tumors. This rule provides only two criteria to follow when a malignant tumor follows a non-malignant tumor. The first criteria (for non-malignant tumor followed by malignant tumor) states:

--Patient had a resection of the non-malignant tumor (not the same tumor) OR

--It is unknown/not documented if the patient had a resection.

This patient did not have a resection of the original, separate, non-malignant tumor, but the treatment plan was known to not include a resection. Should Rule M5 also apply to cases where the patient never had treatment planned for the separate non-malignant tumor?

SEER SINQ 20031051 applies to cases diagnosed before 2007 and advises:  Code histology as 9260/3, Ewing sarcoma. Ewing sarcoma is a specific histology on the continuum of primitive neuroectodermal tumors. Code Ewing sarcoma as it is more specific than PNET, NOS.

For tumors diagnosed 2007 or later, refer to the MP/H rules.

While we recognize that paragangliomas originate in the parasympathetic or sympathetic nervous system, these are endocrine tumors and endocrine glands/structures are not included in ICD-O site code C479 (Autonomic nervous system). Endocrine tumors of the paraganglia have their own site codes (C75_) per the ICD-O. Additionally, the ICD-O-3.2 provides specific sites for most of the paragangliomas included in Table 9. Per the ICD-O-3.2, carotid body paraganglioma is C754, and middle ear paraganglioma, glomus jugulare tumor, jugulotympanic paraganglioma, and paraganglioma (NOS) are C755.

Why are paragangliomas not coded to the paraganglia sites (C75_) provided in the ICD-O? Should these sites be added to the Head & Neck schema for the specific paragangliomas arising in the head and neck? Obtaining consistency in coding primary site for these tumors will be difficult if registrars use the ICD-O provided site codes instead of the primary site statement preceding Table 9. Additionally, as most registrars may use the ICD-O provided site code, the Head and Neck schema in the Solid Tumor Rules would not apply, the Other Sites schema would apply to sites C754 and C755.