Adrenal gland and paraganglia
Pheochromocytoma / paraganglioma
Pathologic TNM staging of Pheochromocytoma and paraganglioma (AJCC 8th edition)


Topic Completed: 1 August 2017

Revised: 25 June 2019

Copyright: 2017-2019, PathologyOutlines.com, Inc.

PubMed search: Staging[TIAB] pheochromocytoma paraganglioma

Debra Zynger, M.D.
Page views in 2018: 1,063
Page views in 2019 to date: 1,021
Cite this page: Zynger D. Pathologic TNM staging of Pheochromocytoma and paraganglioma (AJCC 8th edition). PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/adrenalpheostaging.html. Accessed August 24th, 2019.
Definition / general
  • Pheochromocytoma and sympathetic (functional) paraganglioma are covered by this staging system
Essential features
  • This AJCC staging system is new as of January 1, 2018 and use of theĀ 8th editionĀ 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)

Notes:
  • 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

Notes:
  • 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 I: T1 N0 M0
  • Stage II: T2 N0 M0
  • Stage III: T1-2 N1 M0 or T3 N0-1 M0
  • Stage 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 Zynger, M.D.

pT1 pheochromocytoma

pT2 pheochromocytoma

pT2 sympathetic
paraganglioma
Microscopic (histologic) images

Contributed by Debra Zynger, M.D.

pT2 sympathetic
paraganglioma

pT3 pheochromocytoma
with extra-adrenal
adipose invasion

pT3 pheochromocytoma
with invasion
of the kidney
Board review question #1
    What is the pT classification for a pheochromocytoma that is 3 cm and is confined to the adrenal gland?

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

Reference: Pathologic TNM staging of Pheochromocytoma and paraganglioma (AJCC 8th edition)

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