Solid Pseudopapillary Tumor and Insulinoma in Distal Pancreatectomy: A Case Report

2016-12-30T05-45-32Z
Source: Cukurova Medical Journal
Deniz Tuncel, Banu Yilmaz Ozguven, Ayşe Ayşim Ozagari, Muharrem Battal, Ahmet Ucar, Elif Devecioglu, Fevziye Kabukcuoglu.
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).


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