Table 1.

AJCC TNM and staging definitions for PPGLa

Primary tumor (T)
ȃ• TX: primary tumor cannot be assessed
ȃ• T1: PHEO < 5 cm in greatest dimension, no extra-adrenal invasion
ȃ• T2: PHEO ≥ 5 cm OR sympathetic PGL of any size, no extra-adrenal invasion
ȃ• T3: Tumor of any size with invasion into surrounding tissuesb
Regional lymph nodes (N)
ȃ• NX: regional lymph nodes cannot be assessed
ȃ• N0: no regional lymph node metastasis
ȃ• N1: regional lymph node metastasis
Distant metastasis (M)
ȃ• M0: no distant metastasis
ȃ• M1: distant metastasis
ȃȃ• M1a: metastasis to bone only
ȃȃ• M1b: metastasis to non-regional lymph node(s), liver, and/or lung; no skeletal metastases
ȃȃ• M1c: metastasis to bone and multiple other sites
AJCC prognostic stage groups
ȃ• Stage I: T1 N0 M0
ȃ• Stage II: T2 N0 M0
ȃ• Stage III: T1-2 N1 M0 or T3 N0-1 M0
ȃ• Stage IV: T1-3 N0-1 M1
Primary tumor (T)
ȃ• TX: primary tumor cannot be assessed
ȃ• T1: PHEO < 5 cm in greatest dimension, no extra-adrenal invasion
ȃ• T2: PHEO ≥ 5 cm OR sympathetic PGL of any size, no extra-adrenal invasion
ȃ• T3: Tumor of any size with invasion into surrounding tissuesb
Regional lymph nodes (N)
ȃ• NX: regional lymph nodes cannot be assessed
ȃ• N0: no regional lymph node metastasis
ȃ• N1: regional lymph node metastasis
Distant metastasis (M)
ȃ• M0: no distant metastasis
ȃ• M1: distant metastasis
ȃȃ• M1a: metastasis to bone only
ȃȃ• M1b: metastasis to non-regional lymph node(s), liver, and/or lung; no skeletal metastases
ȃȃ• M1c: metastasis to bone and multiple other sites
AJCC prognostic stage groups
ȃ• Stage I: T1 N0 M0
ȃ• Stage II: T2 N0 M0
ȃ• Stage III: T1-2 N1 M0 or T3 N0-1 M0
ȃ• Stage IV: T1-3 N0-1 M1

Abbreviations: PGL, paraganglioma; PHEO, pheochromocytoma; PPGL, pheochromocytoma/paraganglioma.

Jimenez et al (11).

As per AJCC definitions, invasion into surrounding tissues includes any involvement outside the wall/capsule of any organ. For adrenal tumors, this may include infiltration of extra-adrenal adipose tissue and other soft tissues and/or adjacent organs such as the kidneys, pancreas, liver, and spleen.

Table 1.

AJCC TNM and staging definitions for PPGLa

Primary tumor (T)
ȃ• TX: primary tumor cannot be assessed
ȃ• T1: PHEO < 5 cm in greatest dimension, no extra-adrenal invasion
ȃ• T2: PHEO ≥ 5 cm OR sympathetic PGL of any size, no extra-adrenal invasion
ȃ• T3: Tumor of any size with invasion into surrounding tissuesb
Regional lymph nodes (N)
ȃ• NX: regional lymph nodes cannot be assessed
ȃ• N0: no regional lymph node metastasis
ȃ• N1: regional lymph node metastasis
Distant metastasis (M)
ȃ• M0: no distant metastasis
ȃ• M1: distant metastasis
ȃȃ• M1a: metastasis to bone only
ȃȃ• M1b: metastasis to non-regional lymph node(s), liver, and/or lung; no skeletal metastases
ȃȃ• M1c: metastasis to bone and multiple other sites
AJCC prognostic stage groups
ȃ• Stage I: T1 N0 M0
ȃ• Stage II: T2 N0 M0
ȃ• Stage III: T1-2 N1 M0 or T3 N0-1 M0
ȃ• Stage IV: T1-3 N0-1 M1
Primary tumor (T)
ȃ• TX: primary tumor cannot be assessed
ȃ• T1: PHEO < 5 cm in greatest dimension, no extra-adrenal invasion
ȃ• T2: PHEO ≥ 5 cm OR sympathetic PGL of any size, no extra-adrenal invasion
ȃ• T3: Tumor of any size with invasion into surrounding tissuesb
Regional lymph nodes (N)
ȃ• NX: regional lymph nodes cannot be assessed
ȃ• N0: no regional lymph node metastasis
ȃ• N1: regional lymph node metastasis
Distant metastasis (M)
ȃ• M0: no distant metastasis
ȃ• M1: distant metastasis
ȃȃ• M1a: metastasis to bone only
ȃȃ• M1b: metastasis to non-regional lymph node(s), liver, and/or lung; no skeletal metastases
ȃȃ• M1c: metastasis to bone and multiple other sites
AJCC prognostic stage groups
ȃ• Stage I: T1 N0 M0
ȃ• Stage II: T2 N0 M0
ȃ• Stage III: T1-2 N1 M0 or T3 N0-1 M0
ȃ• Stage IV: T1-3 N0-1 M1

Abbreviations: PGL, paraganglioma; PHEO, pheochromocytoma; PPGL, pheochromocytoma/paraganglioma.

Jimenez et al (11).

As per AJCC definitions, invasion into surrounding tissues includes any involvement outside the wall/capsule of any organ. For adrenal tumors, this may include infiltration of extra-adrenal adipose tissue and other soft tissues and/or adjacent organs such as the kidneys, pancreas, liver, and spleen.

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