Feokromositomada İdrar Vanilmandelik Asidinin Yeniden Gözden Geçirilmesi: 16 Yıllık Tersiyer Merkez Kohort Deneyimi

Yıl 2026, Cilt: 7 Sayı: 1, 189 - 197, 31.01.2026

Özge Baş Aksu , Aderin Arık , Asude Samancı , Atakan Süsoy , Emir Ceyhan , Hilal Aycan Çakmakçı , Şehri Sude Kahraman , Yağmur Karaca , Rifat Emral , Bilge Ayça Kırmızı , Beyza Doğanay Erdoğan , Sevim Gullu

https://doi.org/10.47482/acmr.1718143

Öz

Amaç: Bu çalışma, Türkiye'de Ankara Üniversitesi Tıp Fakültesi Hastanelerinde tanı konulan feokromositoma olgularının klinik, biyokimyasal, patolojik ve genetik özelliklerini değerlendirmeyi amaçlamıştır. Ayrıca, katekolaminlerin tümör boyutunu öngörmedeki tanısal faydasını değerlendirmiştir.
Yöntemler: Ocak 2008 ile Nisan 2024 tarihleri arasında Ankara Üniversitesi Tıp Fakültesinde histopatolojik olarak doğrulanmış feokromositoma tanısı alan 68 yetişkin hasta retrospektif olarak analiz edilmiştir. Tümör boyutu, genetik mutasyonlar, semptomlar, komorbid durumlar, demografik özellikler ve 24 saatlik idrar katekolamin düzeyleri hakkında bilgiler toplanmıştır. ROC eğrilerinin değerlendirilmesi dahil olmak üzere istatistiksel analizler SPSS versiyon 20 kullanılarak gerçekleştirilmiştir.
Bulgular: Ortanca yaş 44 yıl olup, hastaların %61,8'i kadındı. Hipertansiyon (%70,2), çarpıntı (%45,1) ve baş ağrısı (%41,9) en sık görülen semptomlar olmuştur. Tek odaklı tümörü olan hastalar arasında, ≥4 cm tümörü olanların VMA düzeyleri anlamlı olarak yüksek bulunmuştur (p=0,020). 6,35 mg/24 saat VMA kesim değeri, büyük tümörlerin tanınmasında %84 duyarlılık ve %62,5 özgüllük sağlamıştır (AUC=0,717). Genetik testler, test edilen bireylerin %50'sinde RET mutasyonları ve %15,4'ünde SDH mutasyonları ortaya çıkarmıştır. Kohortun %17,6'sında metastatik hastalık gelişmiştir.
Sonuçlar: İdrar VMA'sı, özellikle plazma testinin mümkün olmadığı durumlarda, feokromositomada tümör boyutunu tahmin etmek için yararlı bir biyokimyasal belirteç olabilir. Çalışma ayrıca özellikle genç hastalarda veya ilişkili endokrin neoplazmlı hastalarda erken genetik testlerin önemini göstermektedir. Bu nadir ancak potansiyel olarak yaşamı tehdit eden durumu olan hastalarda gelişmiş sonuçlar elde etmek için yapılandırılmış bir tanı ve takip planı gereklidir.

Anahtar Kelimeler

feokromositoma , katekolaminler , vanilmandelic asit , tümör boyutu , genetik test

Revisiting Urinary Vanillylmandelic Acid in Pheochromocytoma: Insights from a 16-Year Tertiary Center Cohort

Öz

Background: This study aimed to evaluate the clinical, biochemical, and pathological characteristics of pheochromocytoma cases diagnosed at a tertiary center. Additionally, it investigated the diagnostic value of urinary catecholamines in predicting tumor size.
Methods: Sixty-six adult patients with histopathologically confirmed pheochromocytoma diagnosed between January 2008 and April 2024 were retrospectively analyzed. Demographic data, clinical symptoms, tumor size, and 24-hour urinary catecholamine levels were examined. Statistical analyses were performed using SPSS version 25.
Results: A total of 66 patients were included in the study; the mean age was 46.10 ± 15.84 years, and 62.1% were female. The most frequently reported symptoms were hypertension (71.4%), palpitations (46.0%), and headache (42.9%). Among patients with unifocal tumors, both VMA (p=0.001) and normetanephrine (p=0.039) levels were significantly higher in large tumors ≥4 cm compared to small tumors <4 cm. A VMA cutoff value of 6.61 mg/24h achieved 85.7% sensitivity and 70.6% specificity (AUC=0.807) in identifying large tumors.
Conclusions: Urinary VMA levels may serve as a useful biochemical marker for predicting tumor size in pheochromocytoma patients, particularly when plasma testing is not available. A structured diagnostic and follow-up process is necessary to achieve better outcomes in this rare but potentially life-threatening condition.

Anahtar Kelimeler

pheochromocytoma , catecholamines , vanillylmandelic acid , tumor size , genetic testing

Etik Beyan

Ethical approval was obtained from the Human Research Ethics Committee of Ankara University Faculty of Medicine (Decision No: i04-333- 24, dated 13.05.2024).

Destekleyen Kurum

The authors received no financial support for the research and/or authorship of this article. No funding was received for this study.

Teşekkür

The authors acknowledge the use of artificial intelligence for language editing and improving the fluency and grammar of this manuscript. All scientific content, data analysis, interpretation, and conclusions remain entirely the work and responsibility of the authors.

Kaynakça

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