Tips on using my ppt.
1. You can freely download, edit, modify and put your
2. Don’t be concerned about number of slides. Half the
slides are blanks except for the title.
3. First show the blank slides (eg. Aetiology ) > Ask
students what they already know about ethology of
today's topic. > Then show next slide which enumerates
4. At the end rerun the show – show blank> ask questions >
show next slide.
5. This will be an ACTIVE LEARNING SESSION x
6. Good for self study also.
7. See notes for bibliography.
• Adrenocortical carcinomas (ACs) are
uncommon malignancies that can have
protean clinical manifestations.
• A majority of cases are metastatic at the
time of diagnosis, with the most common
sites of spread being the local periadrenal
tissue, lymph nodes, lungs, liver, and bone.
• AC is relatively rare, however, accounting
for just 0.02-0.2% of all cancer-related
• mutations of theTP53 and TP57 genes (both
• increased production of insulinlike growth
factor 2 (IGF-2).
• Incidence 0.6-1.67 cases per million persons per
• In southern Brazil, however, the incidence of
adrenal tumors is 10-15 times that of the general
population, a difference that has been associated
with a mutation in the P53 gene.
• The female-to-male ratio for ACs is approximately
• AC occurs in 2 major peaks: in the first decade of
life and again in the fourth to fifth decades.
Clinical Features: hormonally active AC
• Physical examination findings in patients
with hormonally active AC include the
• Cushing syndrome
• Virilization -Hirsutism, facial acne,
oligo/amenorrhea, and increased libido
• Feminization (rare)
• profound weakness, hypertension, and/or
ileus from hypokalemia related to
hyperaldosteronism and hypoglycemia.
• palpable mass in the abdomen.
• round face
• double chin
• buffalo-hump fat distribution
• generalized obesity
• failure of growth velocity
• Findings in males --premature puberty with
enlargement of the penis and scrotum,
pubic hair, acne, and deepening voice.
• Findings in females- premature appearance
of pubic and axillary hair, clitoral
hypertrophy, acne, deepening voice,
premature increase in growth velocity, lack
of appropriate breast development, and lack
• Male -gynecomastia and hypertension;
• Female –
– precocious sexual development
Diagnostic Studies Imaging studies
• The typical case is characterized by a large
unilateral adrenal mass with irregular edges.
The presence of contiguous adenopathy
serves as corroborating evidence.
• Adjunct to surgery.
• Treatment of endocrine excess syndromes
• Use of mitotane or several multiagent
• Treatment and prevention of potential
• Strategies for palliative and terminal care
issues, including symptom relief and
• Mitotane plus etoposide
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