|Congenital Adrenal hyperplasia
|Primary hyperaldosteronism (Conn's syndrome)
|McCune Albright syndrome
Adrenocortical carcinomas (ACC) are very uncommon and some may cause hormonal excess with hypercortisolism, hyperandrogenism or both
- 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
- Beckwith-Wiedemann syndrome
- Carney complex
- Li-Fraumeni syndrome
- Lynch syndrome
- Multiple endocrine neoplasia, type 1 (MEN 1)
- Asymptomatic and found during imaging
- Asymptomatic + symptoms of malignancy
- Produce Cortisol: Cushing's Syndrome
- Produce Aldosterone: Hypertension
- Produce Sex hormone: virilization/feminization.
- Back pain, Fever, Weight loss, Anorexia
- Testicular pain and varicocele
- FBC, U&E, CRP. Anaemia. Raised CRP. Urinary free cortisol testing.
- Exclude Cushing syndrome - 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 tumor increases the risk of the mass being malignant. May be heterogeneous enhancement because of internal hemorrhage, 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.
- FDG PET the vast majority of adenomas demonstrate background FDG avidity. An FDG-avid adrenal mass is suspicious for malignancy until proven otherwise.
- 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, tumor invasion of the capsule, the extra-adrenal soft tissue or direct invasion of lymphatic channels/blood vessels is a common feature of ACC
- Adrenal pseudocyst
- Adrenocortical carcinoma
- Stage I with a tumor size of smaller than 5 cm.
- Stage II is a tumor with a size of over 5 cm; strictly localized both stages.
- Stage III is characterized by the positive regional lymph nodes, infiltration of the tumor in the surrounding tissue, or a tumor thrombus in the vena cava and/or renal vein.
- Stage IV is defined by distant metastasis
- 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.