Adrenal gland & paraganglia

General

Staging-adrenal cortical carcinoma



Topic Completed: 9 June 2020

Minor changes: 1 October 2021

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PubMed Search: Staging of adrenocortical carcinoma[TI]

Debra L. Zynger, M.D.
Page views in 2020: 992
Page views in 2021 to date: 897
Cite this page: Zynger DL. Staging-adrenal cortical carcinoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/adrenalstaging.html. Accessed November 29th, 2021.
Definition / general
  • All carcinomas of the adrenal gland are covered by this staging system
  • These topics are not covered: pheochromocytoma, 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: C74.0 - cortex of the adrenal gland
Primary tumor (pT)
  • pTX: cannot be assessed
  • pT0: no evidence of primary tumor
  • pT1: tumor ≤ 5 cm, no extra-adrenal invasion
  • pT2: tumor > 5 cm, no extra-adrenal invasion
  • pT3: extra-adrenal invasion but no invasion of adjacent organs
  • pT4: invasion of kidney, diaphragm, pancreas, spleen, liver, renal vein or vena cava
Regional lymph nodes (pN)
  • pNX: cannot be assessed
  • pN0: no regional lymph node metastasis
  • pN1: regional lymph node metastasis (ex: paraaortic, periaortic, retroperitoneal)
Distant metastasis (pM)
  • pM1: distant metastasis
AJCC prognostic stage groups
Stage group I: T1 N0 M0

Stage group II: T2 N0 M0
Stage group III: T1 - 2 N1 M0
T3 - 4 N0 - 1, X M0
Stage group IV: T1 - 4, X   N0 - 1, X   M1  
Registry data collection variables
  • Tumor weight
  • Vascular invasion
  • Mitotic count
  • Ki67 proliferation index
  • Weiss score
Histologic grade (G)
  • Low grade: ≤ 20 mitoses per high powered field
  • High grade: > 20 mitoses per high powered field; TP53 or CTNNB mutation
Histopathologic type
  • Oncocytic
  • Myxoid
  • Sarcomatoid
Gross images

Contributed by Debra Zynger, M.D.

Organ confined, ≤ 5 cm (pT1)

Organ confined, > 5 cm (pT2)

Extra-adrenal invasion (pT3)

Liver invasion (pT4)

Microscopic (histologic) images

Contributed by Debra Zynger, M.D.

Extra-adrenal adipose invasion (pT3)

Liver involvement (pT4)

Board review style question #1

    The adrenal cortical carcinoma shown above has invasion of the extra-adrenal adipose tissue but does not invade adjacent organs. Which is the correct pT for this tumor?

  1. pT1
  2. pT2
  3. pT3
  4. pT4
Board review style answer #1
C. pT3. Adrenal cortical carcinoma with extra-adrenal adipose invasion is pT3. pT1 tumors are organ confined and ≤ 5 cm. pT2 tumors are organ confined and > 5 cm. pT4 tumors show direct invasion of adjacent structures including the kidney, diaphragm, pancreas, spleen, liver, renal vein or vena cava.

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