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
Adrenocortical carcinoma --short review
Dr.Ravi Roshan Khadka
Introduction and Epidemiology
• Rare malignancy with an incidence of 0.5 to 2 per
• 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
• 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
– Hormone secreting ACC (50% to 79% of adult and
90% of pediatric ACCs)
most common hormone secreted by adrenal cortical carcinoma
Imaging- CT scan/ MRI
– 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
– the presence of irregular borders,
– irregular enhancement,
– Necrotic areas with cystic degeneration.
• Evaluation of spread/mets
– IVC, Adjacent organs, Liver ,lungs, Bones
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
• 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.
•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.
• 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
• Despite aggressive surgical resection, adrenal
• Complete surgical excision is essential in the management of adrenal
• 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
– 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.
• Currently there is a limited role for radiation
for therapy in the treatment of primary
adrenal cortical carcinomas;
• Radiation therapy remains the treatment of
– 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
• 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
• A significant increase in recurrence-free
survival and overall survival was noted in
patients receiving mitotane compared with
• 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
• After a disease-free interval of 2 years,
surveillance should continue, but the
frequency of imaging may decrease.
• Overall 5-year survival in adrenal cortical
carcinomas is poor, ranging from 20% to 47%.