Primer Adrenal Malignitelere Yaklaşım
Özet
Adrenal bezler, retroperitonda yer alan, vücut homeostazisinin merkezi kabul edilen ve hormon salgılayan hayati organlardır. Makalede, adrenal patolojilerin tanı ve cerrahi yönetimindeki güncel yaklaşımlar ele alınmaktadır. En sık rastlanan benign kitlelerden olan adrenal adenomların tanısında kontrastsız BT ve metabolik değerlendirmeler kritik rol oynarken, 4 cm’den büyük veya fonksiyon gösteren lezyonlar için cerrahi rezeksiyon önerilmektedir. Nadir görülen onkositom, myelolipom ve gangliyonörom gibi tümörlerin yanında, psödokistlerin malignite riski barındırabileceği belirtilmektedir. Oldukça nadir ve agresif bir kanser türü olan adrenal karsinom, genellikle ileri evrelerde ve Cushing Sendromu bulgularıyla teşhis edilmekte, tedavisinde geniş cerrahi eksizyon ve adjuvan mitotan kullanılmaktadır. Çocukluk çağında görülen pediyatrik adrenal karsinom ve prognozu kötü olan nöroblastomun yanı sıra, yoğun katekolamin salgısı ve epizodik hipertansiyon ataklarıyla karakterize feokromasitoma da cerrahi olarak tedavi edilen önemli medulla tümörlerindendir. Tanı süreçlerinde biyopsinin kullanımı komplikasyon riskleri nedeniyle kısıtlı olup, görüntüleme yöntemleri ve fonksiyonel testler ön plandadır. Fonksiyonel kitleler, malignite bulguları taşıyan veya takipte büyüme gösteren tüm lezyonlar adrenelektomi endikasyonudur.
Adrenal glands are vital, hormone-secreting organs located in the retroperitoneum, considered the center of body homeostasis. The article discusses current approaches in the diagnosis and surgical management of adrenal pathologies. While unenhanced CT and metabolic evaluations play a critical role in the diagnosis of adrenal adenomas, which are among the most common benign masses, surgical resection is recommended for lesions larger than 4 cm or those showing function. Alongside rare tumors such as oncocytoma, myelolipom, and ganglioneuroma, it is noted that pseudocysts may carry a risk of malignancy. Adrenocortical carcinoma, a very rare and aggressive type of cancer, is usually diagnosed at advanced stages with symptoms of Cushing's Syndrome, and its treatment utilizes wide surgical excision and adjuvant mitotane. In addition to pediatric adrenocortical carcinoma and neuroblastoma, which has a poor prognosis in childhood, pheochromocytoma, characterized by intense catecholamine secretion and episodic hypertension attacks, is another important medulla tumor treated surgically. The use of biopsy in diagnostic processes is limited due to the risk of complications, leaving imaging methods and functional tests at the forefront. Functional masses, all lesions carrying signs of malignancy, or those demonstrating growth during follow-up represent indications for adrenalectomy.
Referanslar
Kutikov A, Crispen P, Uzzo R. (2020). Pathophysiology, Evaluation, and Medical Management of Adrenal Disorders. In Partin AW (Ed), Campbell-Walsh-Wein Urology. (12th ed., pp. 2354-2404). PA USA: Elsevier.
Avisse C, Marcus C, Patey M, et al. Surgical anatomy and embryology of the adrenal glands. Surg Clin North Am. 2000;80(1):403-415. Doi: 10.1016/s0039-6109(05)70412-6
Putz, R. (2001). Sobotta İnsan Anatomisi Atlası. (Kaplan ARINCI, Çev. Ed.). Münih: Beta Basım Yayım Dağıtım
Barwick TD, Malhotra A, Webb JAW, et al. Embryology of the adrenal glands and its relevance to diagnostic imaging. Clin Radiol. 2005;60(9):953-959. Doi: 10.1016/j.crad.2005.04.006
Young WF Jr. Management approaches to adrenal incidentalomas. A view from Rochester, Minnesota. Endocrinol Metab Clin North Am. 2000;29(1):159-185. Doi:10.1016/s08889-8529(05)70122-5
Barzon L, Sonino N, Fallo F, et al. Prevalence and natural history of adrenal incidentalomas. Eur J Endocrinol. 2003;149(4):273-285. Doi:10.1530/eje.0.1490273
Grumbach MM, Biller BMK, Braunstein GD, et al. Management of the clinically inapparent adrenal mass (“incidentaloma”). Ann Intern Med. 2003;138:424-429. Doi:10.7326/0003-4819-138-5-200303040-00013
Szolar DH, Korobkin M, Reittner P, et al. Adrenocortical carcinomas and adrenal pheochromocytomas: mass and enhancement loss evaluation at delayed contrast-enhanced CT. Radiology. 2005;234(2):479-485. Doi:10.11487radiol.2342031876
Fassnacht M, Arlt W, Bancos I, et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with th European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2016;175(2):G1-G34. Doi:10.1530/EJE-16-0467
Juliano JJ, Cody RL, Suh JH. Metastatic adrenocortical oncocytoma: a case report. Urol Oncol. 2008;26(2):198-201. Doi:10.1016/j.urolonc.2007.02.2008
Bishop E, Eble JN, Cheng L, et al. Adrenal myelolipomas show nonrandom X-chromosome inactivation in hematopoietic elements and fat: support for a clonal origin of myelolipomas. Am J Surg Pathol. 2006;30(7):838-843. Doi:10.1097/01.pas.0000202044.05333.17
Radin R, David CL, Goldfarb H, et al. Adrenal and extra-adrenal retroperitoneal ganglioneuroma: imaging findings in 13 adults. Radiology. 1997;202(3):703-707. Doi:10.1148/radiology.202.3.9051020
Neri LM, Nance FC. Management of adrenal cysts. Am Surg. 1999;65(2):151-163.
Else T, Williams AR, Sabolch A, et al. Adjuvant therapies and patient and tumor characteristics associated with survival of adult patients with adrenocortical carcinoma. J Clin Endocrinol Metab. 2014;99(2):455-461. Doi:10.1210/jc.2013-2856
Fassnacht M, Allolio B. Clinical management of adrenocortical carcinoma. Best Pract Res Clin Endocrinol Metab. 2009;23(2):273-289. Doi:10.1016/j.beem.2008.10.008
Sundin A. Imaging of adrenal masses with emphasis on adrenocortical tumors. Theranostics. 2012;2(5):516-522. Doi:10.7150/thno.3613
Aubert S, Wacrenier A, Leroy X, et al. Weiss system revisited: a clinicopathologic and immunohistochemical study of 49 adrenocortical tumors. Am J Surg Pathol. 2002;26(12):1612-1619. Doi:10.1097/00000478-200212000-00009
Meyer A, Niemann U, Behrend M. Experience with the surgical treatment of adrenal cortical carcinoma. Eur J Surg Oncol. 2004;30(4):444-449. Doi:10.1016/j.ejso.2004.01.021
Schteingart DE, Doherty GM, Gauger PG, et al. Management of patients with adrenal cancer: recommendations of an international consensus conference. Endocr Relat Cancer. 2005;12(3):667-680. Doi:10.1677/erc.1.01029
Berruti A, Grisanti S, Pulzer A, et al. Long-Term Outcomes of Adjuvant Mitotane Therapy in Patients With Radically Resected Adrenocortical Carcinoma. J Clin Endocrinol Metab. 2017;102(4):1358-1365. Doi:10.1210/jc.2016-2894
Ayala-Ramirez M, Jasim S, Feng L, et al. Adrenocortical carcinoma: clinical outcomes and prognosis of 330 patients at a tertiary care center. Eur J Endocrinol. 2013;169(6):891-899. Doi:10.1530/EJE-13-0519
Cecchetto G, Ganarin A, Bien E, et al. Outcome and prognostic factors in high-risk childhood adrenocortical carcinomas: A report from the European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT). Pediatr Blood Cancer. 2017;64(6). Doi:10.1002/pbc.26368
Park JR, Eggert A, Caron H. Neuroblastoma: biology, prognosis, and treatment. Pediatr Clin North Am. 2008;55(1):97-120. Doi:10.1016/j.pcl.2007.10.014
Lenders JWM, Eisenhofer G, Mannelli M, et al. Phaeochromocytoma. Lancet. 2005;366(9486):665-675. Doi:10.1016/S0140-6736(05)67139-5
Boland GW, Lee MJ, Gazelle GS, et al. Characterization of adrenal masses using unenhanced CT: an analysis of the CT literature. AJR Am J Roentgenol. 1998;171(1):201-204. Doi:10.2214/ajr.171.1.9648789
Welch TJ, Sheedy PF, Stephens DH, et al. Percutaneous adrenal biopsy: review of a 10-year experience. Radiology. 1994;193(2):341-344. Doi:10.1148/radiology.193.2.7972740.
