What are the stages of adrenal carcinoma?

Stages of Adrenal Carcinoma

Adrenocortical carcinoma (ACC) should be staged using the ENSAT (European Network for the Study of Adrenal Tumors) TNM classification system, which is superior to older staging systems and directly impacts surgical approach and prognosis. 1

ENSAT Staging System for ACC

The ENSAT classification divides ACC into four distinct stages based on tumor size, local invasion, lymph node involvement, and distant metastases 1:

Stage I

• Tumor ≤5 cm 1
• No infiltration of surrounding tissues 1
• No lymph node involvement (N0) 1
• No distant metastases (M0) 1
• Treatment approach: Open surgery with transperitoneal access for complete R0 resection 1

Stage II

• Tumor >5 cm 1
• No infiltration of surrounding tissues 1
• No lymph node involvement (N0) 1
• No distant metastases (M0) 1
• Treatment approach: Open surgery with transperitoneal access for complete R0 resection 1

Stage III

• Any tumor size WITH one of the following: 1
  • Positive lymph nodes (N1) 1
  • Infiltration of surrounding tissue 1
  • Venous tumor thrombus 1
• No distant metastases (M0) 1
• Treatment approach: Open surgery often requiring resection of adjacent organs (vena cava wall, liver, spleen, colon, pancreas, stomach) to achieve R0 resection 1
• Adjuvant therapy: Systemic mitotane recommended for incomplete resection (R1, Rx) or high-risk features (Ki67>10%) 1

Stage IV

• Presence of distant metastases 1
• Regardless of tumor size or local invasion 1
• Treatment approach: Combination cytotoxic chemotherapy plus mitotane (EDP-M) for fit patients with high tumor volume and rapid progression 1

Critical Prognostic Factors Beyond Stage

Disease stage and margin-free resection (R0) are the most important prognostic factors in ACC 1:

• R0 resection provides the only means to achieve long-term survival 1
• Additional prognostic parameters include: age, mitotic count, Ki67 proliferation marker, and glucocorticoid excess 1
• Locoregional lymphadenectomy improves tumor staging and appears to lead to favorable oncologic outcomes 1

Important Clinical Caveats

The ENSAT system is superior to the 2004 UICC staging system because it better differentiates prognosis between stages, particularly separating Stage II and III patients who had similar outcomes under the old system 1, 2. The key improvement is restricting Stage IV exclusively to patients with distant metastases 2.

Laparoscopic surgery should only be considered for small ACCs (<8 cm) without preoperative evidence of invasiveness, and only in centers with extensive laparoscopic adrenal surgery experience 1. For all other cases, open surgery is mandatory to achieve R0 resection 1.

Fine needle biopsy of suspected ACC is almost never justified due to risk of tumor spill and is contraindicated in suspected pheochromocytoma 1, 3.