☄️Adrenocortical Carcinoma (ACC) Flashcards
(9 cards)
What is Adrenocortical Carcinoma?
- Very rare tumor, responsible for 10% Cushing’s syndrome & 1% of all adrenal tumor.
- 80% functioning, 20% non-functioning.
- Size usually > 5cm, rarely palpable
Epidemiology of ACC
- 0.1/100,000.
- F: M = 1:1.
- Before 5 years and 40 – 50 years old.
- May be associated with Li-Fraumani Syndrome, Beckwith-Wiedemann Syndrome, MEN 1.
- 20% confined to adrenal, 20% regional disease, 40% metastatic at presentation.
Presentation of ACC patient
Incidentaloma on imaging
Clinical: palpable mass, LOW, LOA, abdominal pain, tumor bleed (Acute pain, fever, shock), hirsutism, deepening of voice, acne, male pubic hair distribution, HTN.
- Weakness due to hypokalemia.
- Functional symptoms
- Pheochromocytoma
- Cushing’s Syndrome
- Primary hyperaldosteronism
- Male pattern hair growth, abnormal menses, Virilization.
- Metastatic symptoms - Lung, Liver, Bones.
Differential Diagnosis: Adrenal metastases, Adrenal adenoma, Adrenal hemorrhage, Pheochromocytoma.
Investigation of ACC
- Rule out other Functional tumour ( Cushing, Conn, Pheochromocytoma)
CT Scan
- CT findings - (> 4cm, >20HU, <50% washout at 10 mins), Unilateral lesion, irregularly shaped, intra-lesional calcification, central areas of necrosis and hemorrhage, Invasion to surrounding structure.
- Dedicated adrenal washout CT protocol consists of a non-contrast, a contrast -enhanced scan with a delay of 60-90 sec and a delayed scan at 15 minutes.
MRI
- Intermediate to high density on T2
- Rapid and intense enhancement with sluggish washout on gadolinium-diethylenetriamine penta acetic acid (DTPA) contrast enhanced
FDG-PET-CT
- In suspicious lesion in the diagnosis and follow-up evaluations.
FNAC
- Biopsies of suspected ACC are usually not informative and should be avoided.
- FNA cytology cannot reliably differentiate a benign adrenal adenoma from adrenal cortical carcinoma (ACC).
- Finally, adrenal biopsy is associated with multiple risks including malignant seeding of the FNA tract, adrenal hemorrhage that may complicate subsequent adrenalectomy, damage to surrounding organs and vessels, and hypertensive crisis in patients with undiagnosed pheochromocytoma.
Staging for ACC
Based on Macfarlane staging or TNM
Macfarlane Staging
- Stage I : Confined to gland, < 5cm
- Stage II : Confined to gland, > 5cm
- Stage III: Extends outside gland without involving adjacent organs
- Stage IV: Distant metastasis or involvement of adjacent organs.
Management of ACC
Operative
- En bloc resection to achieve R0 margin.
- Non functioning mass > 6cm (4 cm) should be removed.
- Non functioning mass < 3 cm can be observed, risk of metastasis very low.
- IVC involvement may need bypass
Chemotherapy
- For un-resectable, recurrent, metastatic disease.
- Mitotane (adrenolytic agent) effective in functioning tumor (80% response).
- Cisplatin 2nd line.
- Functioning tumor - Ketoconazole, metyrapone, amino glutethimide, RU 486 (mifepristone), IV Etomidate - given to control symptoms from excess hormones.
- Given with cortisol & mineralocorticoid replacement.
RADIO-RESISTANT,
EBRT - limited to palliative control of local disease or bone metastasis.
What is Mitotane?
Mitotane, sold under the brand name Lysodren, is a steroidogenesis inhibitor and cytostatic antineoplastic medication which is used in the treatment of adrenocortical carcinoma and Cushing’s syndrome.
HPE of ACC
Have a nodular appearance with individual nodules varying from pink to yellow-tan, depending on their lipid content. Foci of necrosis, hemorrhage, and calcification are common, particularly in large tumors.
Carcinomas associated with:
- Feminization or virilization tend to be red-brown,
- Cushing syndrome are more often yellow-tan.
Prognosis for ACC
5-year survival:
- Overall : 16%
- Localized disease (stage I and II) : 42%
- Locally advance (stage III) : 30%
- Metastases (Stage IV) : 0%
