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. 2021 Apr 6;36(2):322–338. doi: 10.3803/EnM.2020.908

Table 3.

The AJCC Staging System of Pheochromocytoma and Paraganglioma

TNM staging
Primary tumor (pT)a
 pTX: primary tumor cannot be assessed
 pT1: pheochromocytoma <5 cm in greatest dimension
 pT2: pheochromocytoma ≥5 cm or sympathetic paraganglioma
 pT3: invasion into surrounding tissues (including extra-adrenal adipose)

Regional lymph nodes (pN)b
 pNX: regional lymph nodes cannot be assessed
 pN0: no regional lymph node metastasis
 pN1: regional lymph node metastasis

Distant metastases (pM)
 M0: no distant metastasis
 pM1a: metastasis to bone
 pM1b: metastasis to non-regional lymph node, liver or lung
 pM1c: metastasis to bone and multiple other sites

AJCC prognostic stage groups
 Stage I: T1 N0 M0
 Stage II: T2 N0 M0
 Stage III: T1–2 N1 M0 or T3 any N M0
 Stage IV: any T any N M1

AJCC, American Joint Committee on Cancer; TNM, tumor-node-metastasis.

a

Nonfunctional parasympathetic paragangliomas (arising from the head and neck) are excluded in this staging;

b

Regional lymph nodes includes aortic and retroperitoneal nodes for abdominal and pelvic tumors.