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Adrenocortical
Carcinoma
Adrenocortical carcinoma is a rare tumor that affects only 1 to 2
persons per one million population. It usually occurs in adults, and the median
age at diagnosis is 44 years. Although adrenal carcinoma is potentially curable
at early stages, only 30% of these malignancies are confined to the adrenal
gland at the time of diagnosis.[1]
Radical surgical excision is the treatment of choice for patients with localized
malignancies and remains the only method by which long-term disease-free
survival may be achieved. Overall 5-year survival for tumors resected for cure
is approximately 40%.
Retrospective studies have identified 2 important prognostic
factors: completeness of resection and stage of disease. Patients without
evidence of invasion into local tissues or spread to lymph nodes have an
improved prognosis.[2]
The role of DNA ploidy as a prognostic indicator is controversial, with some [3]
studies showing correlation between aneuploidy and prognosis, and other studies
[2,4]
showing no correlation.
Approximately 60% of patients present with symptoms related to
excessive hormone secretion, but hormone testing reveals that 60% to 80% of
tumors are functioning.[5,6]
Nonfunctioning carcinomas may be heralded by symptoms of local invasion by tumor
or by metastases. Initial evaluation should include, in addition to appropriate
endocrine studies, computed tomography and/or magnetic resonance imaging of the
abdomen. Selective angiography and adrenal venography may be helpful in
identifying smaller lesions and for distinguishing tumors of the adrenal gland
from tumors of the upper pole of the kidney. Although the use of positron
emission tomography may be effective in identifying unsuspected sites of
metastases, its role as a staging tool is unclear.[7]
The detection of metastatic lesions may allow effective palliation of both
functioning and nonfunctioning lesions.
The most common sites of metastases are the peritoneum, lung,
liver, and bone. Palliation of metastatic functioning tumors may be achieved by
resection of both the primary tumor and metastatic lesions. Unresectable or
widely disseminated tumors may be palliated by antihormonal therapy with
mitotane, systemic chemotherapy, or (for localized lesions) radiation therapy.
However, survival for patients with stage IV tumors is usually less than 9
months unless a complete remission is achieved.[6,8-10]
There is no convincing evidence to date that systemic therapy will improve the
survival duration of patients with adrenal cancer.
From the National Cancer Instute
8/16/2006
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