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Staging-pheochromocytoma & paraganglioma


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

Last author update: 4 January 2023
Last staff update: 4 January 2023

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PubMed search: Staging pheochromocytoma paraganglioma

Debra L. Zynger, M.D.
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Page views in 2023 to date: 1,851
Table of Contents
Definition / general | Essential features | ICD coding | Primary tumor (pT) | Regional lymph nodes (pN) | Distant metastasis (pM) | AJCC prognostic stage groups | Registry data collection variables | Histologic grade (G) | Gross images | Microscopic (histologic) images | Board review style question #1 | Board review style answer #1
Cite this page: Zynger DL. Staging-pheochromocytoma & paraganglioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/adrenalpheostaging.html. Accessed September 21st, 2023.
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:
    • C74.1 - medulla of adrenal gland
    • 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)

Note:
  • 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

Note:
  • 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 hour 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.

pT1
pheochromocytoma

pT2
pheochromocytoma

pT3 pheochromocytoma  

pT3 pheochromocytoma

pT2 sympathetic
paraganglioma

Microscopic (histologic) images

Contributed by Debra L. Zynger, M.D., Brian Werstein, M.D. and Nicole K. Andeen, M.D. (Case #489)

pT2 sympathetic
paraganglioma

pT3
pheochromocytoma
with extra-adrenal
adipose invasion

pT3
pheochromocytoma
with invasion
of the kidney

pN1
pheochromocytoma

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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