Adrenal gland & paraganglia
General
Staging-pheochromocytoma and paraganglioma
Topic Completed: 23 August 2021
Minor changes: 23 August 2021
Copyright: 2017-2021, PathologyOutlines.com, Inc.
PubMed search:
Staging[TIAB] pheochromocytoma paraganglioma
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Cite this page: Zynger DL. Staging-pheochromocytoma and paraganglioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/adrenalpheostaging.html. 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)
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
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.
pT1
pheochromocytoma
pT2
pheochromocytoma
pT3
pheochromocytoma
pT2 sympathetic
paraganglioma
Microscopic (histologic) images
Contributed by Debra L. Zynger, M.D.
pT2 sympathetic
paraganglioma
pT3
pheochromocytoma
with extra-adrenal
adipose invasion
pT3
pheochromocytoma
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?
- pT1
- pT2
- pT3
- pT4
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