Neoplasm of uncertain behavior of paraganglia

This code represents a tumor originating from paraganglionic tissue (e.g., carotid body, aortic body, glomus jugulare) where the pathologist cannot definitively classify it as benign or malignant based on microscopic examination. These neoplasms are characterized by their potential for aggressive behavior, but without clear evidence of malignancy at the time of diagnosis.

Use this code when pathology reports describe a paraganglioma or chemodectoma as having "uncertain" or "unknown" malignant potential. This typically occurs when the tumor exhibits some atypical features but lacks definitive criteria for malignancy (e.g., clear invasion, metastasis). Documentation must explicitly state the uncertain behavior of the paraganglionic neoplasm.

• Do not use this code if the pathology report definitively states the paraganglioma is benign (e.g., D35.6) or malignant (e.g., C75.5).
• Ensure the documentation specifies the paraganglionic origin; otherwise, consider codes for neoplasms of uncertain behavior of other endocrine glands.
• This code is for the primary diagnosis of the neoplasm itself, not for symptoms it may cause.