Adrenal gland & paraganglia


Staging-pheochromocytoma and paraganglioma

Deputy Editor-in-Chief: Maria Tretiakova, M.D., Ph.D.
Debra L. Zynger, M.D.

Topic Completed: 23 August 2021

Minor changes: 23 August 2021

Copyright: 2017-2021,, Inc.

PubMed search: Staging[TIAB] pheochromocytoma paraganglioma

Debra L. Zynger, M.D.
Page views in 2020: 1,827
Page views in 2021 to date: 1,568
Cite this page: Zynger DL. Staging-pheochromocytoma and paraganglioma. website. Accessed October 15th, 2021.
Definition / general
  • All pheochromocytoma and sympathetic (functional) paraganglioma are covered by this staging system
  • These topics are not covered: parasympathetic (nonfunctional) paraganglioma of the head and neck, adrenal cortical carcinoma, neuroblastic tumors
Essential features
  • AJCC 7th edition staging was sunset on December 31, 2017; as of January 1, 2018, use of the 8th edition is mandatory
ICD coding
  • ICD-O-3:
    • C74.1 - adrenal medulla
    • C75.5 - aortic body and other paraganglioma
Primary tumor (pT)
  • pTX: primary tumor cannot be assessed
  • pT1: pheochromocytoma < 5 cm
  • pT2: pheochromocytoma 5 cm or larger OR sympathetic paraganglioma
  • pT3: invasion into surrounding tissues (including extra-adrenal adipose)

  • Parasympathetic (nonfunctional) paraganglioma, usually arising in the head and neck, are typically benign and do not require staging
Regional lymph nodes (pN)
  • pNX: regional lymph nodes cannot be assessed
  • pN0: no regional lymph node metastasis
  • pN1: regional lymph node metastasis

  • Includes aortic and retroperitoneal nodes for abdominal and pelvic tumors
Distant metastasis (pM)
  • pM1a: metastasis to bone
  • pM1b: metastasis to nonregional lymph node, liver or lung
  • pM1c: metastasis to bone and multiple other sites
AJCC prognostic stage groups
Stage group I:  T1  N0  M0
Stage group II:  T2  N0  M0
Stage group III:  T1 - 2  N1  M0
 T3  N0 - 1  M0
Stage group IV:  T1 - 3  N0 - 1  M1
Registry data collection variables
  • Size (cm)
  • Location
  • Reginal lymph node metastases
  • Location distant metastases
  • 24 hr urinary fractional metanephrines and plasma metanephrines
  • Chromogranin A
  • Mitotic count
  • Germline mutation status
  • Plasma methoxytyramine
Histologic grade (G)
  • There is currently no recommended grading system
Gross images

Contributed by Debra L. Zynger, M.D.




pT2 sympathetic

Microscopic (histologic) images

Contributed by Debra L. Zynger, M.D.

pT2 sympathetic

with extra-adrenal
adipose invasion

with invasion
of the kidney

Board review style question #1

What is the pT classification for a pheochromocytoma that is 1.8 cm and is confined to the adrenal gland?

  1. pT1
  2. pT2
  3. pT3
  4. pT4
Board review style answer #1
A. pT1. Pheochromocytoma < 5 cm that is confined to the adrenal is pT1.

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Reference: Staging-pheochromocytoma and paraganglioma
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