Gigantic ovarian and suprarenal cysts ten years after enucleation of unilateral retinoblastoma

Abstract

INTRODUCTION Retinoblastoma (RB) is the most common eye tumour in children and is curable. Patients with hereditary RB have increased risk of developing additional tumours, predominantly sarcomas. The published results on the treatment of bilateral RB have shown that 10-15% of patients develop second primary tumours (SPT). Average latent period, e.g. the period from the diagnosis of bilateral RB to the appearance of SPT is 10.4 years. In the literature, the appearance of second benign tumours in patients suffering from unilateral and bilateral RB has not been analyzed separately. However, the size and the location of benign tumours can cause serious complications. CASE OUTLINE We present a 14-year-old female patient. At age 4 years, her left eye was enucleated owing to unilateral RB. In the postoperative period, she did not undergo irradiation, nor did she receive chemotherapy. On her regular ophthalmologic check-up, the patient informed us that her belly was 'swelling' over the past four months. She had no pains or other complaints. The abdomen was significantly above the level of the chest, tense, painless on palpation, with obvious fluctuations. Abdominal echosonography confirmed the presence of a huge multilocular cystic formation, 19?18 cm in diameter, spreading from the pelvis to the epigastrium, and pressing the liver and spleen. A cyst of 4.7 cm in diameter was observed in the upper pole of the left kidney. Both the gigantic cyst of the left ovary (weighing 10300 g) and the left suprarenal cyst (weighing 30g) were removed. CONCLUSION We report a patient who developed second nonocular tumors (ovarian and suprarenal cysts) after successful treatment of unilateral RB. Patients treated for RB (hereditary and non-hereditary) should be checked regularly and meticulously. Early recognition of tumours, treatment can prevent possible complications.Uvod. Retinoblastom je najcesci maligni tumor oka kod dece. S povecanjem stope i perioda prezivljavanja kod bolesnika s naslednim retinoblastomom javlja se povecan rizik od razvoja rekurentnih i drugih primarnih tumora (DPT), prvenstveno sarkoma. Rizik za nastanak DPT je veci ukoliko su u lecenju retinoblastoma primenjene spoljna zracna terapija i hemioterapija. Prosecan latentni period od pocetne dijagnoze oboljenja do nastanka DPT je 10,4 godine. Kod nenaslednih retinoblastoma DPT se retko opisuju. Pojava benignih DPT kod bolesnika s naslednim i nenaslednim retinoblastomom u literaturi nije posebno analizirana. Prikaz bolesnika. Prikazana je bolesnica uzrasta 14 godina kod koje je u cetvrtoj godini uradjena enukleacija levog oka zbog unilateralnog retinoblastoma. Posle operacije nije zracena, niti je primala hemioterapiju. Na redovnom kontrolnom pregledu kod oftalmologa bolesnica je kazala da joj se u poslednja cetiri meseca 'naduvava' trbuh. Drugih tegoba nije imala. Trbuh je bio znatno iznad ravni grudnog kosa, napet, palpatorno bezbolan, s jasnom fluktuacijom. Ehosonografskim pregledom trbuha utvrdjena je ogromna multilokularna cisticna formacija velicine 19?18 cm, koja se prostirala od male karlice do epigastrijuma, potiskujuci jetru i slezinu. Na gornjem polu levog bubrega uocena je cista precnika 4,7 cm. Kroz malu medijalnu laparotomiju izvadjene su gigantska cista levog jajnika mase 10,3 kg i cista levog nadbubrega mase 30 g. Patohistoloski nalaz je potvrdio benignu prirodu obe promene. Zakljucak. U ovom radu je istaknut znacaj pazljivog, redovnog (sestomesecnog), dugotrajnog i ne samo oftalmoloskog kontrolisanja bolesnika s retinoblastomom. S obzirom na cinjenicu da je kod osoba izlecenih od retinoblastoma moguce ocekivati nastanak malignih ili benignih DPT, njihovo rano otkrivanje i lecenje bi smanjili rizik od mogucih komplikacija i loseg ishoda i doprineli poboljsanju kvaliteta zivota bolesnika.