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Adrenocortical carcinoma --short review


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Short review on Adrenocortical carcinoma

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Adrenocortical carcinoma --short review

  1. 1. Adrenocortical carcinoma Short review Dr.Ravi Roshan Khadka Resident MS--Urology
  2. 2. Introduction and Epidemiology • Rare malignancy with an incidence of 0.5 to 2 per million. • Bimodal age distribution that peaks in children in the first decade of life and adults in the fourth to fifth decdes of life. • Slight female predominance of 1.5 to 2.1 • Majority of ACCs are sporadic and unilateral • loss of TP53 function and increased IGF expression represent late events in the tumorigenesis of sporadic ACC
  3. 3. Clinical Characteristics • Incidental detection. • Tumor-related symptoms – Local symptoms: abdominal fullness, back pain, nausea and vomiting. – Systemic / metastatic futures: Anorexia, anaemia, asthenia and weight loss and features of metastasis. – Hormone secreting ACC (50% to 79% of adult and 90% of pediatric ACCs)
  4. 4. Clinical Characteristics most common hormone secreted by adrenal cortical carcinoma is cortisol
  5. 5. Investigations
  6. 6. Imaging- CT scan/ MRI • Size – Relative indicator of malignancy, with – 4% to 5% of tumors less than 4 cm, 10% of tumors larger than 4 cm, and 25% of tumors greater than 6cm. • Common radiographic characteristic of ACC on CT imaging include – the presence of irregular borders, – irregular enhancement, – calcifications – Necrotic areas with cystic degeneration. • Evaluation of spread/mets – IVC, Adjacent organs, Liver ,lungs, Bones
  7. 7. Role of biopsy • For diagnosing adrenal cortical carcinomas, percutaneous needle biopsy is generally not performed prior to surgical excision – due to a clinically unacceptable risk of needle- tract seeding (Fassnacht et al, 2004; Schteingart et al, 2005). • The primary indication for needle biopsy is – in cases of unresectable, locally advanced, or metastatic disease, to confirm the diagnosis prior to systemic medical therapy.
  8. 8. Pathologic Evaluation.
  9. 9. •Stage I and II tumors are confined to the adrenal gland and are distinguished by a size cutoff of 5 cm. •Stage III disease includes tumor extension into adjacent adipose tissue or having regional lymph node involvement. •Stage IV disease includes tumors invading adjacent organs and the presence of distant metastatic disease.
  10. 10. Management • Unfortunately, the majority of patients with adrenal cortical carcinomas present with advanced disease those who do have localized disease are at a high risk of local recurrence and metastatic progression. • Treatment of ACC often includes multimodal therapy directed by a team of surgeons, medical oncologists, endocrinologists, and radiation oncologists. • Despite aggressive surgical resection, adrenal
  11. 11. Surgery • Complete surgical excision is essential in the management of adrenal cortical carcinomas • En bloc resection of surrounding organs involved with locally advanced disease should be performed whenever possible. • Cases of venous tumor thrombus involving the IVC may require vascular bypass techniques, IVC replacement, and/or IVC interruption. • In cases of metastatic adrenal carcinoma, – Cytoreductive removal of the primary tumor and debulking metastatectomy should be considered if greater than 90% of the disease burden can be removed. – Although debulking surgery may not improve survival, it may alleviate tumor- related side effects and facilitate additional therapies . • Local or distant disease recurrences, following initial resection, should be considered for surgical excision and have been associated with improved survival in retrospective series.
  12. 12. RADIATION • Currently there is a limited role for radiation for therapy in the treatment of primary adrenal cortical carcinomas; • Radiation therapy remains the treatment of choice in – Management of bone and CNS metastasis – To decrease local recurrence rates following complete tumor resection, (with reported local recurrence rates of 14% and 79%, with and without adjuvant radiation therapy, respectively ) • Unfortunately, a significant improvement in
  13. 13. MEDICAL THERAPY • Mitotane is most commonly used chemotherapeutic agent in the treatment of adrenal cortical carcinoma. • Benefit in the adjuvant setting following surgical resection and in patients with metastatic disease • A significant increase in recurrence-free survival and overall survival was noted in patients receiving mitotane compared with controls
  14. 14. Follow up • Follow-up should include CT examination of the chest, abdomen, and pelvis every 3 months for the first 2 years. • In patients with evidence of functional tumors, measurement of the initially elevated hormones postoperatively may help to reveal early disease recurrence despite negative radiographic studies. • After a disease-free interval of 2 years, surveillance should continue, but the frequency of imaging may decrease.
  15. 15. Prognosis • Overall 5-year survival in adrenal cortical carcinomas is poor, ranging from 20% to 47%.