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Background: Carney triad (gastric leiomyosarcoma, pulmonary chondroma and extraadrenal paraganglioma) is a rare syndrome comprising of multiple tumors of unknown etiology. Long-term follow-up shows that the syndrome is chronic and generally indolent and persistent; outcome is largely dependent on the behaviour of the metastases of the gastric sarcoma. Case Report: We report a case of 16-year-old girl who presented with full triad (multiple bilateral pulmonary chondroma and adrenal tumor) and was treated with surgery for gastric GIST and adjuvant imatinib. Conclusion: This report will sensitize the physicians about the possibility of Carneys triad, especially in young females presenting with one of the three components at unusual locations.

An extra-adrenal paraganglioma is a rare tumour derived from chromaffin cells of sympathetic ganglia. This report documents a rare case of a non-functional aortocaval paraganglioma in a 24-year-old woman with persistent abdominal pain. Computed tomography revealed a solid mass, measuring 2.5x3cms, localized between the celiac trunk and superior mesenteric artery in aortocaval location along with right ovarian cystic mass. A clinical diagnosis of malignant ovarian tumour with celiac nodal metastasis was made. Excision and pathological analysis of both revealed an aortocaval extra-adrenal paraganglioma and benign ovarian cyst. On serial follow-up the patient was in a good health, asymptomatic and without evidence of tumour recurrence. This case emphasizes the necessity of including extra-adrenal paraganglioma in the differential diagnosis and management of retroperitoneal tumours, despite its rarity.Key Words: Aorta, venacava, lymph node, paraganglioma, surgery ÖzetEkstra-adrenal paraganglioma sempatik ganglionların kromafin hüc-relerinden gelişmiş nadir bir tümördür. Bu rapor; 24 yaşındaki sürekli karın ağrıları olan, nonfonksiyonel aortokaval bulunan nadir bir olgunun sunumudur. Tomografisinde sağ over kistik kitle ile birlikte aortokaval konumda çölyak gövde ve superior mezenterik arter arasında lokalize 2.5x3 cm boyutlarında ölçülen, solid bir kitle saptandı. Çölyak nodal metastazı olan malign over tümörün klinik tanısı konulmuştu. Eksizyon ve patolojik incelemenin her ikisi aortokaval ekstra-adrenal paraganglioma ve benign over kistinin olduğunu ortaya koydu. Seri takiple hasta asemptomatik ve tümör nüksü olmadan sağlığına kavuştu. Bu olgu, nadir olmasına rağmen, retroperitoneal tümörlerin ayırıcı tanısında ve tedavisinde ekstra-adrenal paragangliomanın dahil edilme gerekliliğini vurgulamaktadır.

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