Endocrine: Adrenocortical Carcinoma

Adrenocortical Carcinoma

Basics

  • More Common in Women (1.5-2.5:1)
  • Bimodal Age Distribution: Age < 5 Years or 40-50’s
  • Genetic Associations:
    • *Most are Sporadic
    • Li-Fraumeni Syndrome
    • Beckwith-Wiedemann Syndrome
    • MEN1
  • 60% Are Functional

Presentation

  • Mass/Malignant Effects
    • Abdominal/Flank Pain
    • Weight Loss
    • Anorexia
  • Glucocorticoid Excess (Cushing Syndrome) – Most Common Functional Presentation
    • Weight Gain
    • Skin Changes
    • Weakness
  • Androgen Excess – Often Seen Along with Cushing Syndrome Mixed
    • Virilization (Development of Male Physical Characteristics)
  • Mineralocorticoid Excess – Rare
    • Hypertension
    • Hypokalemia

Prognosis

  • Generally Poor Prognosis
  • 5-Year Survival:
    • Stage I: 66-82%
    • Stage II: 58-64%
    • Stage III: 24-50%
    • Stage IV: 0-17%
  • Most Common Sites of Metastases:
    • Liver (48-85%)
    • Lungs (30-60%)
    • Lymph Nodes (7-20%)
    • Bone (7-13%)

Diagnosis

Adrenocortical Carcinoma (Arrow); 7.8×4.8 cm, Heterogenous 1

TNM Staging

  • TNM Staging
T N M
1 ≤ 5.0 cm & No Invasion Regional LN Mets
2 > 5.0 cm & No Invasion
3 Invades Surrounding Fat
4 Invades Surrounding Organs
  • Staging
  T N M
I T1 N0 M0
II T2 N0 M0
III T3-4 N0 M0
Any T N1 M0
IV Any T Any N M1

Treatment

  • Primary Treatment: Open Adrenalectomy
    • *See Endocrine: Adrenalectomy
    • Open Approach is Preferred
    • Laparoscopic Adrenalectomy is Evolving but Not Yet Widely Accepted
      • Minimally Invasive Approaches Generally Have Higher Risk of Recurrence, Seeding & Peritoneal Dissemination
    • No Benefit to Routine Lymphadenectomy Yet Established
  • Adjuvant Therapy:
    • Adjuvant Mitotane (Adrenal Lytic)
      • General Indications:
        • Unresectable Tumors
        • High Proliferation Rate (Mitotic Rate or Ki67 Expression)
        • Intraoperative Tumor Spillage
        • Residual or Recurrent Tumors
        • Metastatic
      • May Be Considered for All Patients with Localized Disease Regardless of Stage or Size
    • Adjuvant Chemotherapy & Radiation Therapy is Evolving
  • Postop Surveillance:
    • Initially: CT Every 3 Months for 2 Years
    • After 2 Years: CT Every 6 Months for 5 Years
    • After 5 Years: CT Every 1-2 Years

References

  1. Huang CJ, Wang TH, Lo YH, Hou KT, Won JG, Jap TS, Kuo CS. Adrenocortical carcinoma initially presenting with hypokalemia and hypertension mimicking hyperaldosteronism: a case report. BMC Res Notes. 2013 Oct 8;6:405. (License: CC BY-2.0)