JAFES-Booklet (English - pdf - 1103 Kb) - MEMS

A Series Of Five Cases Of Adrenocortical Carcinoma
Norasyikin AW, Nor Shafinas NA, Norhaliza MA, Wong Ming,
Foo, Suezhazlyn Z, Norlaila M, Rohaizak M, Nor Azmi K
Departments of Medicine and Surgery, UKM Medical Center, Kuala Lumpur.
Abstract
Adrenocortical carcinoma (ACC) is an extremely rare tumor. Although it is potentially curable in
the early stages, only a third is confined to the adrenal gland at the time of diagnosis. Five cases
of ACC with various presentations, managed in our institution within the last 2 years are discussed
in the light of associated issues in their management.
Of the three ACC cases that presented with
Cushing’s syndrome, two were complicated by
uncontrolled hypertension and psychosis as a result
of excessive corticosteroid levels. Three of the
patients had large tumours, two of whom had left
adrenal tumor measuring greater than 6 cm in diameter
(fig 1) whilst the third had a right adrenal tumour
with the same dimension (fig 2).
Figure 1 Figure 2
Of the three functioning tumours, surgical intervention was not performed in two as they had
succumbed to overwhelming sepsis despite being on adrenalytic therapy. The remaining patient
underwent a successful debulking surgery followed by mitotane therapy.
The 4th case was a middle-aged Chinese man who presented with a back pain of two year duration.
The abdominal CT scan revealed an inhomogeneous right adrenal mass measuring 3x4x6
cm. He was subjected to a successful adrenalectomy. Unfortunately, he declined any further
treatment after the surgery.
The last case was that of a young Malay school girl who presented with
pulmonary embolism. Abdominal and thoracic CT scans revealed a huge
ACC measuring 10x12x13cm with inferior vena cava and right atrial
extensions (fig 3). She underwent an uneventful radical thoracoabdominal
surgery with the aid of a cardio-pulmonary bypass procedure.
Mitotane therapy was started before the operation and the dose was
titrated as tolerated (This case is described separately in detail elsewhere
in this proceeding).
Figure 3
Conclusion: Surgery seems to offer the only satisfactory mode of treatment in ACC regardless of
the stages while adrenocorticolytic therapy should be commenced as soon as possible. Efforts to
control excessive corticosteroid levels in functioning cases should be an immediate primary
concern as overwhelming sepis seems to carry the day.
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