Resektionsstrategie bei Nebennierenrindenkarzinomen

Abstract

Adrenocortical carcinomas (ACC) are rare and aggressive neoplasms. Due to their high rate of local recurrence and distant metastases (up to 85%) they are associated with a poor survival. The 5‑year survival in ACC patients with lymph node metastasis or local infiltration is 50% and with distant metastasis less than 15%. An R0 resection with locoregional and para-aortic/paracaval lymphadenectomy is the only curative option and reasonable treatment possibility. The treatment of these patients should therefore be planned and carried out in centers. Local recurrences and distant metastases should also be treated with R0 resection when feasible, combined with neoadjuvant/adjuvant chemotherapy and/or radiation. In the case of an asymptomatic non-resectable ACC, debulking operations cannot be recommended. The primary operation can also be done in a minimally invasive procedure if principles of oncological surgery are followed (radical resection, no damage of the tumor capsule, lymphadenectomy), since survival after open and minimally invasive laparoscopic resection was comparable. Palliative resections are only indicated in symptomatic patients.

Multidisciplinary team therapy for left giant adrenocortical carcinoma: A case report

“…Its incidence in the population is 1-2 per million cases per year in North America and 4-12 cases per million cases per year worldwide[ 2 ]. The 5-year survival rate and median survival time reported by various medical centers vary to some extent[ 3 - 5 ], with 5-year survival ranging from 16%-47%[ 6 ]. The molecular mechanism of ACC is not completely clear at present and may be related to the inactivation of tumor suppressor genes (TP53, Men-1, p57Kip2, and H19), abnormal activation of proto-oncogenes (deletion of GAS, RAS, ACTH receptors), overexpression of growth factor IGF-2, and abnormal activation of the B-catenin gene[ 7 ].…”