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Adrenocortical carcinoma (ACC) is
a rare malignancy with incompletely understood pathogenesis
and poor prognosis. Patients present with hormone excess
(e.g. virilization, Cushing's syndrome) or a local mass
effect (median tumour size at diagnosis > 10cm).
Tumours typically appear inhomogeneous in both computerised
tomography and magnetic resonance imaging with irregular
borders, and differ from benign adrenal tumours by their
low fat content. Hormonal analysis reveals evidence
of steroid hormone secretion by the tumour in the majority
of cases, even in seemingly hormonally inactive lesions.
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Histopathology is crucial for the
diagnosis of malignancy and may also provide important
prognostic information. In stages I -III open surgery
by an expert surgeon aiming at complete resection is
the treatment of choice. Local recurrence is frequent,
particularly after violation of the tumour capsule.
Surgery plays also a role in local tumour recurrence
and metastatic disease.
In patients not amenable to surgery, mitotane (a drug
specifically killing cells of adrenocortical origin)
remains the treatment of choice. Monitoring of drug
levels (therapeutic range 14 - 20 mg/l) is mandatory
for optimum results. In advanced disease, the most promising
therapeutic options (etoposide, doxorubicin, cisplatin
plus mitotane and streptozotocin plus mitotane) are
currently compared in an international phase III trial.
Adjuvant treatment options after complete tumour removal
(e.g. mitotane, radiotherapy) are urgently needed, as
postoperative disease free survival at five years is
below 50%.
International cooperations like ENSAT and trials
provide important new structures for patients, but also
for researchers aiming at systematic and continuous
progress in ACC. Future advances in the management of
ACC will depend on a better understanding of the molecular
pathogenesis facilitating the use of modern cancer treatments
(e.g. tyrosine kinase inhibitiors).
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