Deniz TUNCEL, Banu YILMAZ ÖZGÜVEN, Ayşe Ayşim ÖZAGARİ, Muharrem BATTAL, Ahmet UÇAR, Elif DEVECİOĞLU, Fevziye KABUKCUOĞLU

Distal pankreatektomide solid psödopapiller tümör ve insulinoma birlikteliği

Multipl Endokrin Neoplazi Tip 1 otozomal dominant geçişli nadir bir genetik hastalık olup pankreas, paratiroid ve anterior pitüiter tümörleri ile karakterizedir. Pankreasın adacık hücre tümörleri daha az sıklıkta görülmekte olup bu tümörler içerisinde gastrinoma ve insulinoma daha çok ortaya çıkan tümörlerdir. Solid Psödopapiller Tümör pankreasın diğer bir nadir tümörü olup erken yaşta kadın hastalarda ortaya çıkan sıklıkla benign bir neoplazidir. Distal pankreatektomili, MEN 1 sendromu olan 16 yaşında erkek hastada eş zamanlı görülen solid psödopapiller neoplazi ve insulinoma olgusunu sunuyoruz. Hastanın nöbet şikayetleri mevcut olup acil servise başvurduğunda yüksek insülin düzeyine bağlı olarak hipoglisemi saptandı. Tanısal çalışmalar sırasında pankreas korpusunda multipl lezyonlar saptandı. Ailede, erkek kardeşinde asemptomatik paratiroid adenomu görüldü. Hastaya distal pankreatektomi uygulandı. Makroskopik incelemede bir neoplazi proksimal cerrahi sınıra yakın diğeri distal pankreas yerleşimli iki adet neoplazi bulundu. Histomorfolojik ve immünohistokimyasal değerlendirmede proksimaldeki lezyonun solid psödopapiller neoplazi, distaldeki lezyonun fonksiyonel nöroendokrin tümör (insulinoma) olduğu dikkati çekti.

Solid pseudopapillary tumor and insulinoma in distal pancreatectomy

Multiple endocrine neoplasia type 1 (MEN 1) is a rare autosomal dominant inherited endocrine disease characterized by pancreatic, parathyroid, and anterior pituitary tumours. Pancreatic islet tumours occur less frequently, among them gastrinomas and insulinomas are the most prevalent. A solid pseudopapillary neoplasm (SPN) is another extremely rare tumour of the pancreas that frequently occurs in young females and is mostly benign. We report a case of a 16-year-old male MEN 1 syndrome with insulinoma and solid pseudopapillary neoplasm in distal pancreatectomy. The patient presented with seizures. When admitted to the emergency room, hypoglisemia has been detected due to the high levels of insulin. Multiple lesions have been observed in the corpus of the pancreas during the diagnostic work-up. According to the parathyroid scintigraphy, an adenoma has been detected. In the family, an asymptomatic parathyroid adenoma has also been seen in his brother. Distal pancreatectomy has been performed. In the gross examination, one neoplasm close to the proximal margin, one in the distal part of the pancreas have been found. The histomorphological and immunohistochemical evaluation revealed the proximal neoplasm as solid pseudopapillary neoplasm and the distal one as functioning neuroendocrine tumor (insulinoma).

Keywords:

insulinoma, pancreas, Solid pseudopapillary neoplasm, MEN 1, pancreas,

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