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. 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)
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. 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.
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. 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. 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. 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. 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. 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.
In cases of suspicion for venous tumor thrombus,
MRI can be an essential tool in detecting the presence of a
tumor clot and delineating its extent.
Approach
Smaller radiographically organ- confined disease can be approached laparoscopically, but should be performed with caution due to the risk of tumor spillage
