CUSHING’S SYNDROME PRESENTING WITH METASTATIC GIANT ADRENOCORTICAL CANCER: A CASE REPORT

Year 2018, , 38 - 41, 15.01.2018

Ferhat Gökay , Ayşe Arduç , Gülhan Akbaba , Dilek Berker Serdar Güler

https://doi.org/10.18229/kocatepetip.411214

Abstract

Adrenocortical carcinoma is a rare tumor with poor prognosis. Cushing’s syndrome is the most common clinical presentation but adrenocortical carcinoma is one of the rare causes of Cushing’s syndrome. Because of the rarity, we report a patient who had cortisol-secreting metastatic giant adrenocortical carcinoma in the article. 46-year-old female with a history of malignant melanoma presented with weight gain, hirsutism, menstrual irregularities. She had abdominal obesity, hirsutism, and acneiform eruptions. Her midnight serum cortisol and 24-hour urinary cortisol were high; adrenocorticotropic hormone was low. 2mg and 8mg dexamethasone suppression tests were not suppressed. Adrenal magnetic resonance imaging showed 7x8x9cm right adrenal mass. The patient underwent adrenalectomy. Adrenocortical carcinoma was detected on pathological examination. Despite the chemotherapy (cisplatin+etoposide+mitotane) was given, she died 36 months later because of disseminated adrenocortical carcinoma.

Keywords

Adrenocortical carcinoma, malignant melanoma, , cushing’s syndrome

CUSHING SENDROMU İLE ORTAYA ÇIKAN METASTATİK DEV ADRENOKORTİKAL KARSİNOM OLGUSU

Abstract

Adrenokortikal karsinom nadir görülen kötü prognozlu bir tümördür. En sık klinik prezentasyonu Cushing Sendromu’dur ama bunun tersine Cushing sendromunun nadir sebeplerindendir. Biz bu makalede nadir görülmesi nedeniyle kortizol salgılayan metastatik dev adrenokortikal karsinom tanıları olan bir vakayı sunduk. Kilo alımı, kıllanma artışı, adet düzensizliği ile başvuran 46 yaşındaki kadın hasta 15 yıl önce malign melanom tanısı almış, cerrahi ve kemoterapi sonrası kür kabul edilmişti. Hastada abdominal obezite, hirsutismus ve akneiform döküntüler mevcuttu. Gece serum kortizolü, 24 saatlik idrar kortizolü artmış, adrenokortikotropik hormon baskılı idi. Deksametazon 2mg ve 8mg supresyon testlerinde baskılanma olmadı. Adrenal Cushing sendromu tanısı ile çekilen surrenal magnetik rezonans görüntüleme sağ sürrenalde 7x8x9cm kitle saptandı. Sürrenalektomi patolojik incelemesinde adrenokortikal karsinom saptandı. Sisplatin, etoposid, mitotan kemoterapisi verilen hasta 36 ay sonra yaygın metastazlar nedeni ile öldü.

Keywords

Adrenokortikal karsinom, malign melanom, , cushing sendromu

References

• Allolio B, Fassnacht M. Clinical review: Adrenocorticalcarcinoma: clinical update. J Clin Endocrinol Metab 2006;91(6):2027-37.
• Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis,evaluation and treatment. J Urol 2003;169(1):5-11.
• Wajchenberg BL, Albergaria Pereira MA, Medonca BB,Latronico AC, Campos Carneiro P, Alves VA, et al. Adrenocortical carcinoma: clinical and laboratory observations. Cancer 2000;88(4):711-36.
• Luton JP, Cerdas S, Billaud L. Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy. N Engl J Med 1990; 322 (17): 1195-201.
• Xiao XR, Ye LY, Shi LX, Cheng GF, Li YT, Zhou BM. Diagnosis and treatment of adrenal tumours: a review of 35 years’ experience. Br J Urol 1998;82(2):199-205.
• Koch CA, Pacak K, Chrousos GP. The molecular pathogenesisof hereditary and sporadic adrenocortical and adrenomedullary tumors. J Clin Endocrinol Metab 2002;87(12):5367-84.
• Fassnacht M, Allolio B. Clinical management of adrenocortical carcinoma. Best Pract Res Clin Endocrinol Metab 2009;23(2):273-89.
• Young WF Jr. Clinical practice. The incidentally discoveredadrenal mass. N Engl J Med 2007;356(6):601-10.
• Maurea S, Klain M, Mainolfi C, Ziviello M, Salvatore M. Thediagnostic role of radionuclide imaging in evaluation of patients with nonhypersecreting adrenal masses. J Nucl Med 2001;42(6):884.
• Abiven G, Coste J, Groussin L, Anract P, Tissier F, LegmannP, et al. Clinical and biological features in the prognosis of adrenocortical cancer: poor outcome of cortisol-secreting tumors in a series of 202 consecutive patients. J Clin Endocrinol Metab 2006;91(7):2650-5.
• Straka M, Soumarova R, Bulejcik J, Banik M, Pura M, Skrovina M. Giant adrenocortical carcinoma with 27-month disease-free survival by surgical resection alone: a case report. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2014;158(3):474-8.
• Terzolo M, Angeli A, Fassnacht M, Daffara F, Tauchmanova L, Conton PA, et al. Adjuvant mitotane treatment for adrenocortical carcinoma. N Engl J Med 2007;356(23):2372-80.
• Haluska FG, Hodi FS. Molecular genetics of familialcutaneous melanoma. J Clin Oncol 1998;16(2):670-82.