Adrenokortikal Karsinom: Olgu Sunumu

Abstract

Adrenokortikal karsinom (AKK) çocukluk çağının nadir görülen malign tümörlerindendir. Klinik ve laboratuvar bulgularıyla konjenital adrenal hiperplaziye (KAH) benzerlik gösterebilir. Burada başlangıçta KAH düşünülen, ancak hemen sonrasında adrenokortikal karsinom tanısı alan 3 yaşında bir erkek hasta sunulmuştur. Hasta, altı aydır vücudunda kıllanma ve penis büyümesi şikayeti ile başvurdu. Fizik muayenesin de ciddi hipertansiyon saptanan hastanın penis boyu 90 persentilin üzerinde, testis volümleri bilateral 5 ml ve evre-2 pubarşı vardı. Laboratuvar değerlendirmede, ACTH, 0.8 pg/ml, bazal kortizol düzeyi 19, pik kortizol düzeyi 19.2 mcg/dl, pik 17OH progesteron 60.1 ng/ml, bazal 11- deoksikortizol 16.3 ng/ml (normal düzey: 0–8), serum testesteron; 27.9 ng/dl, Serum Na138mmol/L, K3.3 mmol/L bulunması üzerine 11 ß hidroksilaz eksikliğine bağlı KAH düşünüldü. Günlük 20 mg/m2 hidrokortizon başlanan hastaya bir haftalık izlem süresince antihipertansif tedaviye yanıt alınamaması ve trunkal obezitesi nedeniyle yapılan abdominal USG’de sağ adrenal yerleşimde 5x6x5 cm’lik kitle saptandı. Hasta preoperatif ve postoperatif adrenalyetmezlik riski nedeniyle steroid profilaksisi ile opere edildi. Total cerrahi rezeksiyon sonrası AKK tanısı alan hastanın, klinik ve laboratuar bulguları bir hafta içerisinde normale döndü.

Keywords

• Adrenokortikal Karsinom, Konjenital Adrenal hiperplazi

ADRENOCORTİCAL CARCİNOMA: A CASE REPORT

Abstract

Adrenocortical carcinoma (AKC) is an uncommon malignancy with poor prognosis in childhood. Because of the similar clinical and laboratary findings, it can be misdiagnosed as congenital adrenal hyperplasia (CAH). Here, we present a three year old male who was firstly thought to be CAH, but later diagnosed with AKC. He was admitted to our hospital with the complaints of increase in body hair and penile enlargement six month. His physical examination revealed high blood pressure over 95 percentile for his age, streched penis length was above 90 percentile for age, his testes volumes were 5 mL/5mL and he had Taner stage 2 pubic hair. Other vital sign and physical examination findings were normal. His plasma ACTH level ( 0.8 pg/mL), basal cortisol level (19.0 mcg/dl), peak cortisol level (19.2 mcg/dl), peak 17-OH progesteron level ( 60.1 ng/mL), basal 11- deoxycortisol (16.3 ng/mL) and testosteron level (27.9 ng/dl).Were high for his age. His plasma Na and K levels were within normal limits. With these clinical and laboratory findings he was firstly diagnosed as congenital adrenal hyperplasia due to 11 ß hydroxylase deficiency. He was put on hydrocortisone tretment at a dose of 20 mg/m2 /day but after one week of treatment his blood pressure levels were still high and he had prominent truncal obesity. Abdominal ultrasonography showed a 5x6x5 cm sized solid mass in the right surrenal region. The patient was operated in pediatric Surgery Department of our hospital. while on prophylaxis with steroid treatment for adrenal insufficiency. The clinical and laboratory findings of the patient ameliorated in a week after the total surgical resection of the tumor which was pathologically proven to be ACC

Keywords

• Adrenocortical carcinoma, Congenital adrenal hyperplasia

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