Makindo Medical Notes.com

Related Subjects: |Adrenal Physiology |Addisons Disease |Phaeochromocytoma |Adrenal Adenomas |Adrenal Cancer |Cushing Syndrome |Cushing Disease |Congenital Adrenal hyperplasia |Primary hyperaldosteronism (Conn's syndrome) |ACTH |McCune Albright syndrome

Adrenocortical carcinomas (ACC) are very uncommon and some may cause hormonal excess with hypercortisolism, hyperandrogenism or both

About

• Seen in children younger than 5 and adults in their 40s and 50s
• Incidence about 1 in 1,000,000
• Usually very aggressive lesions
• Some may be functional and release cortisol or other hormones

Associations

• Beckwith-Wiedemann syndrome
• Carney complex
• Li-Fraumeni syndrome
• Lynch syndrome
• Multiple endocrine neoplasia, type 1 (MEN 1)

Causes of Adrenal Cancer

• Genetic Factors:
  • Hereditary syndromes such as Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, and familial adenomatous polyposis increase the risk.
  • Mutations in specific genes (e.g., TP53, IGF2) are associated with adrenal cancer.
• Environmental Factors:
  • Exposure to certain chemicals and prolonged use of synthetic steroids may contribute to risk.
• Unknown Causes:
  • In many cases, the exact cause remains unknown.

Symptoms of Adrenal Cancer

• Hormonal Imbalance Symptoms:
  • Weight gain, particularly around the abdomen and face (Cushing's syndrome).
  • High blood pressure.
  • Excessive hair growth or hair loss.
  • Muscle weakness.
  • High blood sugar levels (diabetes).
• Non-Hormonal Symptoms:
  • Abdominal pain or discomfort.
  • A palpable mass in the abdomen.
  • Unexplained weight loss.
  • Loss of appetite.
  • Fatigue.

Investigations

• FBC, U&E, CRP. Anaemia. Raised CRP.
• Exclude Cushing syndrome:Urinary free cortisol testing. Dexamethasone suppression tests, Cortisol, Aldosterone, androgens, and estrogens
• Urinary or plasma Catecholamines (metanephrine and normetanephrine)
• CT Chest and Abdomen: Adrenal mass may be found on imaging with metastases. Usually more than 6 cm diameter. Size of the tumour increases the risk of the mass being malignant. May be heterogeneous enhancement because of internal haemorrhage, necrosis, and calcification. May see metastases to the lung, lymph nodes, or liver. Also see adrenal adenoma
• MRI: the presence of isointense to hypointense signal on T1-weighted images, a hyperintense signal on T2-weighted images and a heterogeneous signal drop on chemical shift
• Adrenal vein sampling if high cortisol and low ACTH.
• Markers for adrenal cortical carcinoma (e.g., DHEA-S, cortisol, aldosterone).
• FDG PET the vast majority of adenomas demonstrate background FDG avidity. An FDG-avid adrenal mass is suspicious for malignancy until proven otherwise.
• Fine needle aspiration (FNA) or surgical biopsy to confirm diagnosis.

Pathology

• The hallmarks of adrenocortical carcinoma include the large size with irregular margins, invasion of surrounding structures, and the presence of distant metastases.
• The surface of the tumour ranges in color from brown to orange to yellow depending on the lipid content of their cells.
• Necrosis is almost always present. Importantly, tumour invasion of the capsule, the extra-adrenal soft tissue or direct invasion of lymphatic channels/blood vessels is a common feature of ACC

Adrenal cancer is a rare but aggressive form of cancer originating in the adrenal glands. It can cause significant hormonal imbalances and a range of symptoms. Early diagnosis and treatment are crucial for improving prognosis. Treatment options include surgery, radiation therapy, chaemotherapy, targeted therapy, and hormone therapy. Regular follow-up and monitoring are essential for managing this complex condition.

Differentials

• Adrenal pseudocyst
• Phaeochromocytoma
• Adrenocortical carcinoma

Staging

• Stage I with a tumour size of smaller than 5 cm.
• Stage II is a tumour with a size of over 5 cm; strictly localized both stages.
• Stage III is characterized by the positive regional lymph nodes, infiltration of the tumour in the surrounding tissue, or a tumour thrombus in the vena cava and/or renal vein.
• Stage IV is defined by distant metastasis

Management

• Prognosis previously quite poor. Average overall survival in patients with adrenocortical carcinoma was 14.5 months with a 5-year mortality rate of about 75 to 90%
• Referral to Oncology MDT for assessment and planning and risk assessing
• Tumour resection is the aim. Local recurrence is seen. Chemotherapy and Radiation may be added.
• Spironolactone 25-100 mg OD or Eplerenone for Aldosterone secreting adenomas or hyperplasia
• Ketoconazole, metyrapone, and mifepristone can help reduce cortisol
• Radiotherapy & adrenolytic drugs (mitotane) follow if carcinoma.

References

• Adrenal cancer review
• Colection of articles