Adrenal gland & paraganglia

General

Features to report-pheochromocytoma / paraganglioma


Editorial Board Member: Bonnie Choy, M.D.
Deputy Editor-in-Chief: Maria Tretiakova, M.D., Ph.D.
Debra L. Zynger, M.D.

Last author update: 25 May 2023
Last staff update: 25 May 2023

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PubMed Search: Pheochromocytoma paraganglioma features to report

Debra L. Zynger, M.D.
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Cite this page: Zynger DL. Features to report-pheochromocytoma / paraganglioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/adrenalparagangliomareport.html. Accessed March 19th, 2024.
Definition / general
Required features to report
  • The following items are recommended to be included in the pathology report (there is currently no College of American Pathologists cancer protocol)
    • Size (greatest dimension)
    • Composite features (ganglioneuroma, ganglioneuroblastoma, neuroblastoma or peripheral nerve sheath tumors)
    • Lymphovascular invasion
    • Tumor extent (into capsule, extra-adrenal tissue or adjacent organs)
    • Margins
    • Regional lymph node status
    • Distant metastasis (if applicable)
    • pTNM stage
  • The following items are necessary to establish the 8th edition AJCC pTNM stage (note that parasympathetic paragangliomas are not staged)
    • Size (< 5 cm or ≥ 5 cm)
    • Invasion into surrounding tissue (ex: extra-adrenal adipose, liver, pancreas, spleen, kidney)
    • Regional lymph node status
    • Distant metastasis (if applicable)
  • The following items are 8th edition AJCC registry data collection variables
    • Primary tumor size
    • Primary tumor location
    • Regional lymph node metastases
    • Location of distant metastases
    • Hormone function
    • Chromogranin A
    • Mitotic count
    • Germline mutation status
    • Plasma methoxytyramine
  • Reference: Amin: AJCC Cancer Staging Manual, 8th Edition, 2017
Pheochromocytoma of the adrenal gland scaled score (PASS)
  • The PASS score can be evaluated to determine malignant potential with a score of ≥ 4 concerning for malignancy (it is not required to report each criteria but may be helpful to assess and report if concern for malignancy is identified) (Am J Surg Pathol 2002;26:551)
    • Periadrenal adipose invasion (+2)
    • > 3 mitosis per 10 high powered fields (+2)
    • Atypical mitoses (+2)
    • Necrosis (+2)
    • Cellular spindling (+2)
    • Cellular monotony (+2)
    • Large nests or diffuse growth (+2)
    • High cellularity (+2)
    • Marked nuclear pleomorphism (+1)
    • Capsular invasion (+1)
    • Vascular invasion (+1)
    • Hyperchromasia (+1)
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.
Capsular invasion

Capsular invasion

Vascular, capsular and adipose invasion

Vascular, capsular and adipose invasion

Cellular spindling

Cellular spindling

Nuclear pleomorphism

Nuclear pleomorphism

Mitosis

Mitosis


pT2 sympathetic paraganglioma

pT2 sympathetic
paraganglioma

pT3 pheochromocytoma with extra-adrenal adipose invasion

pT3 pheochromocytoma
with extra-adrenal
adipose invasion

pT3 pheochromocytoma with invasion of the kidney

pT3 pheochromocytoma
with invasion
of the kidney

Board review style question #1

For a pheochromocytoma, which of the following tumor characteristics is needed in order to establish the pT category of the tumor?

  1. Cellularity
  2. Mitotic rate
  3. Presence of necrosis
  4. Size
  5. Weight
Board review style answer #1
D. Size. Tumor parameters that determine the pT category for pheochromocytoma are tumor size (< 5 cm versus ≥ 5 cm) and tumor extent (organ confined versus extra-adrenal invasion). Other parameters can be associated with risk of aggressive behavior but are not used to determine the pT category.

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Reference: Features to report-pheochromocytoma / paraganglioma
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