Abstract
Amaç: Adrenokortikal kanserler nadir görülen bir kanser türüdür. Prognozları sitotoksik kemoterapilere rağmen oldukça kötüdür. Primer tedavi cerrahi olup, cerrahi sonrası nüks sıktır. Nüks eden olgularda daha kısa sağkalım izlenmektedir. Cerrahi sonrası adjuvan tedavi yüksek riskli değerlendirilen vakalarda uygulanmaktadır. İnoperabl ya da metastatik hastalarda sitotoksik kemoterapi yapılabilir. Çalışmamızın amacı, nadir görülen bu hastalık grubunda merkez deneyimlerimizi paylaşmaktır. Gereç ve Yöntem: Ege Üniversitesi Tülay Aktaş Onkoloji Hastanesine 2010-2018 yılları arasında adrenokortikal kanser (AKK) tanısı ile başvuran hastaların dosyaları retrospektif olarak incelendi. Hastaların demografik verileri, adjuvan ve metastatik tedavi seçenekleri ve sağkalım süreleri değerlendirildi. Bulgular: AKK tanısı ile bölümümüze başvuran 11 hasta mevcuttu. Hastalarımızın medyan yaşı 44 (31-69) idi. Tanı esnasında 3 hastada metastaz mevcuttu. Metastazı olmayan 8 hastanın 5’inde adjuvan kemoterapi tedavisi uygulanmıştı. Tüm grupta medyan genel sağkalım (OS) 30,2 ay (%95 CI 7,9-52,4) idi. Lokal hastalıkta metastatik hastalığa göre medyan OS istatistiksel olarak anlamlı yüksek bulundu (sırasıyla lokal hastalık medyan OS 48,5, metastatik hastalık medyan OS 11,3; p=0,02). Adjuvan kemoterapi alan ve almayan hastalarda sırasıyla medyan OS 48,5 ve 9 ay olmakla birlikte, hasta kollarının sayısının çok az olması nedeniyle istatistiksel açıdan anlamlı olarak değerlendirilemedi. Sonuç: AKK tanısı ile izlenen hastalarımızda adjuvan tedavi alan hastalarda almayanlara göre daha iyi OS eğilimi görüldü. Mevcut bulgular prospektif çok merkezli çalışmalar ile doğrulanmadıkça tedavi yaklaşımlarını değiştirmeyecektir.
Keywords
References
- Ng L, Libertino JM. Adrenocortical carcinoma: Diagnosis, evaluation and treatment. J Urol 2003;169(1):5-11.
- Crucitti F, Bellantone R, Ferrante A, Boscherini M, Crucitti P. The Italian Registry for Adrenal Cortical Carcinoma: Analysis of a multiinstitutional series of 129 patients. The ACC Italian Registry Study Group. Surgery 1996;119(2):161-70.
- Koch CA, Pacak K, Chrousos GP. The molecular pathogenesis of hereditary and sporadic adrenocortical and adrenomedullary tumors. J Clin Endocrinol Metab 2002;87(12):5367-84.
- Schultz CL, Haaga JR, Fletcher BD, Alfidi RJ, Schultz MA. Magnetic resonance imaging of the adrenal glands: A comparison with computed tomography. Am J Roentgenol 1984;143(6):1235-40.
- Saunders BD, Doherty GM. Laparoscopic adrenalectomy for malignant disease. Lancet Oncol 2004;5(12):718-26.
- Porpiglia F, Fiori C, Daffara F, et al. Retrospective evaluation of the outcome of open versus laparoscopic adrenalectomy for stage I and II adrenocortical cancer. Eur Urol 2010;57(5):873-8.
- Dickstein G, Shechner C, Arad E, Best LA, Nativ O. Is there a role for low doses of mitotane (o,p'-DDD) as adjuvant therapy in adrenocortical carcinoma? J Clin Endocrinol Metab 1998;83(9):3100-3.
- Vassilopoulou-Sellin R, Guinee VF, Klein MJ, et al. Impact of adjuvant mitotane on the clinical course of patients with adrenocortical cancer. Cancer 1993;71(10):3119-23.
- Fassnacht M, Terzolo M, Allolio B, et al. Combination chemotherapy in advanced adrenocortical carcinoma. N Engl J Med 2012;366(23):2189-97.
- Allolio B, Fassnacht M. Clinical review: Adrenocortical carcinoma: Clinical update. J Clin Endocrinol Metab 2006;91(6):2027-37.
- U.S National Library of Medicine. Clinicaltrials.gov. Available online at: https://clinicaltrials.gov/ct2/show/NCT00777244
- Fassnacht M, Allolio B. What is the best approach to an apparently nonmetastatic adrenocortical carcinoma? Clin Endocrinol 2010;73(5):561-5.
- Polat B, Fassnacht M, Pfreundner L, et al. Radiotherapy in adrenocortical carcinoma. Cancer 2009;115(13):2816-23.
- Bertherat J, Bertagna X. Pathogenesis of adrenocortical cancer. Best Pract Res Clin Endocrinol Metab 2009;23(2):261-71.
- Barlaskar FM, Spalding AC, Heaton JH, et al. Preclinical targeting of the type I insulin-like growth factor receptor in adrenocortical carcinoma. J Clin Endocrinol Metab 2009;94(1):204-12.
Abstract
Aim: Adrenocortical cancer is a rare type of disease. Prognosis is poor despite cytotoxic chemotherapies. The primary treatment is surgery however postoperative disease relapse is common. The survival is poorer in patients who develop recurrence. Adjuvan treatment may be performed in patients carrying high risk. Cytotoxic chemotherapy is the only treatment option for inoperable and metastatic patients. In our study, we aimed to evaluate our patient outcome in this rarely seen cancer type. Materials and Methods: We retrospectively reviewed medical records of patients with adrenocortical cancer admitted to Ege University Medical Oncology Clinic between 2010 and 2018. Patient demographics, treatment types for adjuvant and metastatic setting and survival data were collected. Results: We identified 11 adrenocortical cancer patients. Median age was 44 (31-69). Three patients were metastatic at initial diagnosis. Five of 8 patients with nonmetastatic disease received adjuvant chemotherapy . The median overall survival (OS) was 30.2 months (%95 CI 7.9-52.4) in whole patient group. The median OS was found to be better in patients who presented with local disease than de novo metastatic setting (median OS 48.5 and 11.3 months respectively, p=0.02). The OS was 48.5 and 9 months respectively in patients who did and did not receive adjuvant treatment, however this finding was not found to be statistically significant. Conclusion: Nonmetastatic adrenocortical tumor patients who received adjuvant chemotherapy was found to have a slightly favorable OS. However present findings may not enlighten the management of this disease without evaluating treatment efficacy of these patients on multicenter, prospective trial study results.
Keywords
References
- Ng L, Libertino JM. Adrenocortical carcinoma: Diagnosis, evaluation and treatment. J Urol 2003;169(1):5-11.
- Crucitti F, Bellantone R, Ferrante A, Boscherini M, Crucitti P. The Italian Registry for Adrenal Cortical Carcinoma: Analysis of a multiinstitutional series of 129 patients. The ACC Italian Registry Study Group. Surgery 1996;119(2):161-70.
- Koch CA, Pacak K, Chrousos GP. The molecular pathogenesis of hereditary and sporadic adrenocortical and adrenomedullary tumors. J Clin Endocrinol Metab 2002;87(12):5367-84.
- Schultz CL, Haaga JR, Fletcher BD, Alfidi RJ, Schultz MA. Magnetic resonance imaging of the adrenal glands: A comparison with computed tomography. Am J Roentgenol 1984;143(6):1235-40.
- Saunders BD, Doherty GM. Laparoscopic adrenalectomy for malignant disease. Lancet Oncol 2004;5(12):718-26.
- Porpiglia F, Fiori C, Daffara F, et al. Retrospective evaluation of the outcome of open versus laparoscopic adrenalectomy for stage I and II adrenocortical cancer. Eur Urol 2010;57(5):873-8.
- Dickstein G, Shechner C, Arad E, Best LA, Nativ O. Is there a role for low doses of mitotane (o,p'-DDD) as adjuvant therapy in adrenocortical carcinoma? J Clin Endocrinol Metab 1998;83(9):3100-3.
- Vassilopoulou-Sellin R, Guinee VF, Klein MJ, et al. Impact of adjuvant mitotane on the clinical course of patients with adrenocortical cancer. Cancer 1993;71(10):3119-23.
- Fassnacht M, Terzolo M, Allolio B, et al. Combination chemotherapy in advanced adrenocortical carcinoma. N Engl J Med 2012;366(23):2189-97.
- Allolio B, Fassnacht M. Clinical review: Adrenocortical carcinoma: Clinical update. J Clin Endocrinol Metab 2006;91(6):2027-37.
- U.S National Library of Medicine. Clinicaltrials.gov. Available online at: https://clinicaltrials.gov/ct2/show/NCT00777244
- Fassnacht M, Allolio B. What is the best approach to an apparently nonmetastatic adrenocortical carcinoma? Clin Endocrinol 2010;73(5):561-5.
- Polat B, Fassnacht M, Pfreundner L, et al. Radiotherapy in adrenocortical carcinoma. Cancer 2009;115(13):2816-23.
- Bertherat J, Bertagna X. Pathogenesis of adrenocortical cancer. Best Pract Res Clin Endocrinol Metab 2009;23(2):261-71.
- Barlaskar FM, Spalding AC, Heaton JH, et al. Preclinical targeting of the type I insulin-like growth factor receptor in adrenocortical carcinoma. J Clin Endocrinol Metab 2009;94(1):204-12.
Details
| Primary Language | English |
|---|---|
| Subjects | Health Care Administration |
| Journal Section | Research Articles |
| Authors | |
| Publication Date | March 14, 2019 |
| Submission Date | December 5, 2018 |
| Published in Issue | Year 2019Volume: 58 Issue: 1 |
