Şenay ERDEVE SAVAŞ, Mehmet Onur CANDİR, Ayşe Ceyda ÖREN, Sibel TEKGÜNDÜZ AKPINAR, Zehra AYCAN, Semra ÇETİNKAYA, Şule YEŞİL

Childhood adrenocortical tumors: A single-center experience

Amaç: Çocukluk çağı adrenokortikal tümörleri, etyopatogenezi tam olarak bilinmeyen ve standart bir tedavi yaklaşımı olmayan oldukça nadir tümörlerdir. Bu yazımızda bölümümüzde adrenokortikal tümör tanısı almış çocukların tedavi, takip ve sonuçlarını yayınlamayı amaçladık. Yöntemler: Bölümümüzde son 10 yılda tanı alan 15 yaş ve altı 7 tane adrenokortikal tümörlü hastanın kayıtları geriye dönük olarak incelendi. Hastaların yaş, cinsiyet, başvuru belirtileri, hormonal durumu, patolojik bulguları, tümörün evresi, tedavisi ve sonuçları değerlendirildi. Bulgular: Son 10 yılda 4 kız ve 3 erkek hasta adrenokortikal tümör tanısı alarak tedavi edildi. Hastaların beşi adrenokortikal karsinom, ikisi adrenokortikal adenom tanısı aldı. Bütün hastalarda laparotomi yapılarak komplet tümör eksizyonu gerçekleştirildi. Adrenokortikal karsinomlu hastaların ikisi evre I, biri evre III ve kalan ikisi evre IV olarak değerlendirildi. Adrenokortikal adenomlu hastalar sadece cerrahi ile başarılı şekilde tedavi edilirken, adrenokortikal karsinomlu hastalara mitotan ve adjuvan kemoterapi uygulandı. Ortalama takip süresi 5 yıl, 8 ay olan hastalardan altısı halen remisyonda izlenmektedir. Bir hastamız cerrahiden 20 ay sonra, remisyonda iken takipten çıkmıştır. Sonuç: Literatürde adrenokortikal tümörlü hasta sayısının sınırlı ve takip sürelerinin kısa olması, prognozu ve tedavi yaklaşımını belirlemeyi güçleştirmektedir. Sonuç olarak bu hastalar çok merkezli çalışmalara dahil edilerek prognostik faktörler ve tedavide sitotoksik ilaçların rolü incelenmelidir.

Çocukluk çağı adrenokortikal tümörleri: Tek merkez deneyimi

Objective: Childhood adrenocortical tumors (ACTs) are rare neoplasms, about which etiopathogenesis and disease management are not yet clearly understood. We aimed to review the management of ACTs in our single medical center. Methods: We retrospectively reviewed findings in seven children, who were 15 years old or younger, and were diagnosed with ACTs in our institution over the past 10 years. Information recorded for each patient included age, sex, presenting symptoms, hormonal status, pathological findings, stage of disease, treatment and outcome. Results: Four girls and three boys were treated for ACTs. Five had adrenocortical carcinoma (ACC) and two had adrenocortical adenoma (ACA). All patients underwent laparotomy and complete excision. Two of ACCs had a stage I disease, one had a stage III, and the latter two ACCs had a stage IV disease. ACAs were treated successfully by total excision without any concomitant therapy. Adjuvant chemotherapy and mitotane were commenced postoperatively in ACC patients. Six patients are alive and doing well at a mean follow-up of 5 years and 8 months. Conclusion: The small number of ACTs and the short follow-up period limit the assessments of prognosis and management. As a result, patients with ACTs should be studied in multi-institutional trials to address the role of prognostic factors and cytotoxic drugs in the disease management.

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1.Rowe MI, ONeill JA, Grosfeld JL, et al. Endocrine tumors. In: Rowe MI, et al. (eds). Essentials of Pediatric Surgery, Philadelphia, PA, Mosby 1995:311-8.
2.Lack EE, Mulvihill JJ, Travis WD, et al. Adrenal cortical neoplasms in the pediatric and adolescent age group: Clinicopathologic study with emphasis on epidemiological and prognostic factors. Pathol Annual 1992;27:1-53.
3.Ribeiro RC, Sandrini Neto RS, Schell MJ, et al. Adrenocortical carcinoma in children: A study of 40 cases. Journal of Clinical Oncology 1990;8:67-74.
4.Chudler RM, Kay R. Adrenocortical carcinoma in children. Urol Clin North Am 1989;16:469-79.
5.Ribeiro RC, Figueiredo B. Childhood adrenocortical tumours. Eur J Cancer 2004;40:1117-26.
6.Ribeiro RC, Michalkiewicz EL. Adrenocortical tumors in children. In: Raghavan D, Brecher ML, Johnson DH, et al (eds). Textbook of Uncommon Cancer. 2. ed. New York, John Wiley & Sons, 1999:611-20.
7.Sandrini R, Riberio RC, DeLacerda L. Childhood adrenocortical tumors. J Clin Endocrinol Metab 1997;82:2027-31.
8.Michalkiewicz EL, Sandrini R, Figueiredo B. Clinical and outcome characteristics of children with adrenocortical tumors: A report from the International Pediatric Adrenocortical Tumor Registry. Journal of Clinical Oncology 2004;22:838-45.
9.Onder A, Kendirci HN, Aycan Z, et al. A pediatric Conn syndrome case. J Pediatr Endocrinol Metab 2012;25:203-6.
10.Riberio RC, Michalkiewicz EL, Figueiredo B. Adrenocortical tumors in children. Brasilian J Medical and Biol Research 2000;33:1225-34.
11.Sabbage CC, Avilla SG, Schultz C, et al. Adrenocortical carsinoma in children: Clinical aspects and prognosis. J Pediatr Surg 1993;28:841-3.
12.Ciftci AO, Senocak ME, Tanyel CF, et al. Adrenocortical tumors in children. J Pediatr Surg 2001;36:549-54.
13.Lee PD, Winter RJ, Green OC. Virilizing adrenocortical tumors in childhood. Eight cases and a review of the literature. Pediatrics 1985;76:437-44.
14.Lefevre M, Gerard-Marchant R, Gubler JP, et al. Adrenal cortical carsinoma in children: 42 patients treated from 1958 to 1980 at Villejuif. In: Humphrey GB, et al. (eds). Adrenal and Endocrine Tumors in Children, Boston, MA, Martinus Nijhoff Publisher 1983;11:265-76.
15.Daneman A, Chan HS, Martin J. Adrenal carcinoma and adenoma in children: A review of 17 patients. Pediatr Radiol 1983;13:11-8.
16.Mc Ateer JP, Huaco JA, Gow KW. Predictors of survival in pediatric adrenocortical carcinoma: a Surveillance, Epidemiology, and Results (SEER) program study. J Pediatr Surg 2013;48:1025-31.
17.Johnson DH, Greco FA. Treatment of metastatic adrenal cortical carcinoma with cisplatin and etoposide. Cancer 1986;58:2198-202.
18.Marques-Pereira R, DeLacerda L, Sandrini R. Childhood adrenocortical tumors: a Review. Hereditary Cancer in Clin Practice 2006;4:81-9.
19.Zancanella P, Pianovski MAD, Figueiredo B, et al. Mitotane associated with cisplatin, etoposide, and doxorubicin in advanced childhood adrenocortical carcinoma:Mitotane monitoring and tumor regression. J Pediatr Hematol Oncol 2006:28;513-24.
20.Berruti A, Terzolo M, Pia A, et al. Mitotane associated with etoposide, doxorubicin and cisplatin in the treatment of advanced adrenocortical carcinoma. Italian Group for the Study of Adrenal Cancer. Cancer 1998;83:2194-200.