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SEER Inquiry System - Report
Produced: 08/31/2025 8:40 AM
Question 20210003
Inquiry Details
References:
#1: Solid Tumor Rules. Head and Neck; December 2020 Update
#2: WHO Class H & N Tumors. 4th ed.; online version
Question:
Solid Tumor Rules (2018)/Primary Site--Head & Neck: The instructions for Table 9 of the Head and Neck Solid Tumor Rules instruct registrars to code the primary site to C479 (Autonomic nervous system) for paragangliomas that arise in the head and neck region, but the ICD-O-3.2 provides a site-associated code for most of these tumors (C754, Carotid body and C755, Paraganglion). Which primary site is correct? See Discussion.
Discussion:
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.
Answer:
Always code primary site to the site of origin. Look for information about where the neoplasm originated. Primary site should always be coded to reflect the site of origin according to the medical opinion on the case. Always code the primary site based on where the tumor arose / site of origin. Site of origin may be indicated by terms such as "tumor arose from," "tumor originated in," or similar statements.
Refer to ICD-O-3.2 and ICD-O-3 for topographty codes that are associated with specific histologies whenthe medical documentation does not specify the primary site.
