Management of Adrenal Cancer

Schteingart, D.E.; Homan, Dominic

doi:10.1007/978-1-59259-101-5_17

Cited by 6 publications

(7 citation statements)

References 49 publications

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“…Prognosis (Bradley 1975, King & Lack 1979, Hogan 1980, Didolkar et al 1981, Thompson 1983, Bodie et al 1989, Venkatesh et al 1989, Luton et al 1990, Kasperlik-Zaluska 1994, Wajchenberg et al 2000, Schteingart & Homan 2001 Moderators: Gerard Doherty, Thomas Giordano Lead Discussants: Xavier Bertagna, Luisa Barzon, Andrezj Kudelka…”

Section: Diagnosismentioning

confidence: 99%

Management of patients with adrenal cancer: recommendations of an international consensus conference

Schteingart¹,

Doherty²,

Gauger³

et al. 2005

Endocr Relat Cancer

382

280

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Adrenocortical carcinomas are rare, highly malignant tumors that account for only 0.2% of deaths due to cancer. Given the limited number of patients seen in most medical centers with this diagnosis, series usually reported are small and clinical trials not randomized or blinded. In an attempt to answer important questions concerning the management of patients with adrenal cancer, a consensus conference was organized and held at the University of Michigan in Ann Arbor, MI, 11–13 September 2003, with the participation of an international group of physicians who had reported on the largest series of patients with this disease and who had recognized basic and clinical research expertise in adrenal cortical cancer. Totally 43 questions were addressed by the presenters and recommendations discussed in plenary and breakout sessions. Evidence for the recommendations of this conference was at the 2–4+ level and based on available literature and participants’ experience. In addition to setting up guidelines in specific areas of the diagnosis and treatment of adrenal cancer, the conference recommended and initiated the planning of an international prospective trial for treatment of patients with adrenal cancer in stages III and IV. In terms of new therapies, first trials of dendritic cell therapy in human subjects with adrenal cancer have been started, but it is too early to comment on efficacy. Different strategies of immunotherapy, including DNA vaccination are currently being tried in animal models. There are no clinical gene therapy trials for human adrenal cortical cancer. The adrenals are a preferred target for adenovirus and the results of gene therapy in preclinical studies are promising. In addition, there is evidence that histone deacetylase inhibitors can further enhance the rate of adenoviral infectivity in human adrenal cancer cells. Testing of retroviral vectors, non-viral vectors, small interfering RNA technology, and combined approaches could be performed in various laboratories. Anti-angiogenic substances have only been applied in preclinical studies. The use of these and other agents in the treatment of adrenal cancer should be hypothesis-driven and based on a thorough analysis of tumor biology.

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Section: Diagnosismentioning

confidence: 99%

Management of patients with adrenal cancer: recommendations of an international consensus conference

Schteingart¹,

Doherty²,

Gauger³

et al. 2005

Endocr Relat Cancer

382

280

View full text Add to dashboard Cite

show abstract

“…The anabolic action of concomitantly secreted androgens may counteract the glucocorticoid-induced muscle atrophy. Oestrogen-secreting ACCs in males usually present with gynaecomastia and testicular atrophy, in women breast tenderness and irregular menstrual bleeding may occur (Schteingart & Homan, 2001). Aldosterone hypersecretion in ACC is rare and may lead to hypokalaemia and hypertension which may also occur in severe adrenal CS with massive hypercortisolaemia, leading to incomplete renal inactivation by 11 β -dehydrogenase type II and hence mineralocorticoid excess (Stewart et al ., 1995).…”

Section: Clinical Presentationmentioning

confidence: 99%

Management of adrenocortical carcinoma

Allolio

Hahner

Weismann

et al. 2003

Clinical Endocrinology

193

173

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Adrenocortical carcinoma (ACC) is a rare neoplasm with poor prognosis. Patients present with signs of steroid hormone excess (e.g. Cushing's syndrome, virilization) or an abdominal mass. Tumour size at presentation (mean diameter at diagnosis > 10 cm) is the most important indicator of malignancy. In addition, computed tomography (CT) typically demonstrates an inhomogeneous adrenal lesion with irregular margins and variable enhancement of solid components after intravenous contrast media. Magnetic resonance imaging (MRI) is equally effective as CT and is particularly helpful to visualize invasion into large vessels. Complete tumour removal (R0 resection) offers by far the best chance for long-term survival and therefore surgery is the treatment of choice in stage I-III ACC. Despite tumour resection for cure most patients will eventually develop local recurrence or distant metastases. Thus adjuvant treatment options need to be evaluated in high-risk patients (e.g. radiation therapy of the tumour bed and/or chemotherapy). In tumour recurrence re-operation should always be considered. In metastatic disease (stage IV ACC) not amenable to surgery mitotane (o,p'DDD) remains the first-line therapy. Drug monitoring is needed for effective treatment aiming at concentrations between 14 and 20 mg/l. Patients not responding to mitotane may benefit from cytotoxic chemotherapy (23% partial remissions, 4% complete remissions). Only large prospective multicentre trials comparing different treatment options will allow to make systematic progress in the management of ACC.

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“…Einwohner. Dies entspricht etwa 0,2% aller Krebstodesfälle [1]. Die Altersverteilung ist bimodal mit einem ersten Gipfel im Kindesalter und einem zweiten Gipfel in der 5.…”

Section: Epidemiologie Und Pathogeneseunclassified

“…zur Diagnosestellung. Etwa die Hälfte der NNR-Karzinome verursachen über eine gesteigerte Hormonsekretion klinische Symptome, genaue endokrinologische Funktionsdiagnostik zeigt jedoch, dass der Prozentsatz hormonproduzierender Tumoren noch deutlich höher liegt [1]. Die Steroidbiosynthese in der NNR-Karzinomzelle ist allerdings relativ ineffektiv, sodass bei klinischer Manifestation oft bereits eine große Tumormasse vorliegt.…”

Section: Klinikunclassified