Ppgl Partial Adrenalectomy Indication

---
name: ppgl-partial-adrenalectomy-indication
description: This skill assesses whether partial adrenalectomy is indicated for pheochromocytoma and paraganglioma (PPGL) to preserve adrenal function in patients with familial PPGL syndrome, imaging showing bilateral adrenal tumors or high-risk genetics, or young PPGL patients desiring adrenal function preservation. Indication is considered for familial PCC, tumors ≤5 cm, or high risk of future contralateral adrenal lesions, requiring surgical expertise and informed consent regarding recurrence risk.
---

# Partial Adrenalectomy Indication Assessment for PPGL

## STEP 1 — Gather Information
Collect: family history of PPGL syndrome (VHL, MEN2, RET, SDHx), genetic testing results, imaging (CT/MRI) showing bilateral adrenal tumors or unilateral lesion with high-risk contralateral genetics, tumor size (≤5 cm vs >5 cm), patient age, patient preference for adrenal preservation, surgeon expertise in partial adrenalectomy, and documentation of informed consent discussion regarding recurrence and steroid dependence risk.

## STEP 2 — Rule In / Rule Out
Is there a familial PPGL syndrome, bilateral adrenal tumors/high-risk genetics, tumor ≤5 cm, or a young patient desiring adrenal preservation? If yes, proceed to Step 3; if no, partial adrenalectomy is not indicated.

## STEP 3 — Classify or Stratify
Classify as suitable for partial adrenalectomy if tumor ≤5 cm, surgeon has expertise in partial adrenalectomy, and informed consent obtained; otherwise classify as unsuitable (tumor >5 cm, insufficient expertise, or lack of consent).

## STEP 4 — Decide
If suitable, proceed with partial adrenalectomy; if unsuitable, perform total adrenalectomy or consider alternative surgical approach.

## Clinical Guardrails / Mimics / Pitfalls
Do not perform partial adrenalectomy in tumors >5 cm, in surgeons without specific expertise, without documented informed consent, or in sporadic PPGL lacking high-risk genetics/bilateral disease; avoid ignoring recurrence risk (ipsilateral tumor recurrence ~13%) and steroid dependence (23.5% postoperative).

## Concrete Clinical Example
A 28‑year‑old patient with a known VHL pathogenic variant presents with a 4 cm left adrenal tumor and normal right adrenal gland; genetic counseling confirms high risk of contralateral lesion, the patient desires adrenal preservation, and an experienced endocrine surgeon is available. After informed consent discussing recurrence and steroid dependence risks, a left partial adrenalectomy is performed.

**Source:** Japan Endocrine Society Clinical Practice Guideline for the Diagnosis and Management of Pheochromocytoma and Paraganglioma 2025, Japan Endocrine Society, 2025, DOI:10.1507/endocrj.EJ25-0165