Mesenteric paraganglioma's: an important differential diagnosis in intra-abdominal tumours.
نویسندگان
چکیده
1 of 2 DESCRIPTION We present the case of a 63-year-old lady who complained of feeling constantly tired and lethargic, and was found to have iron defi ciency anaemia. There was no history of per vaginal bleeding. Examination was normal apart from a large, mobile, fi rm, well-defi ned abdominal mass in the upper abdomen approximately 10cm in diameter. The abdominal CT scan revealed a large intra-abdominal tumour (fi gure 1). Histology of the specimen obtained by surgical resection revealed this to be a mesenteric paraganglioma (fi gure 2). To the best of our knowledge, there are eight other reported cases of mesenteric paraganglioma’s. Ninety per cent of these sympathetic tissue tumours arise from the adrenals and are called phaeochromocytomas. Paraganglioma’s are extra-adrenal phaeochromocytoma’s, of which 85% arise in the abdomen, 12% in the thorax and the remaining 3% in the head and neck. 1 Paraganglioma’s that hypersecrete catecholamines may cause signs and symptoms identical to those in patients with hyperfunctioning adrenal pheochromocytoma. Recent studies implicate genetic factors in up to 25% of cases. The most frequent causes of phaeochromocytoma susceptibility are von Hippel–Lindau disease, multiple endocrine neoplasia type 2, and the newly delineated phaeochromocytoma–paraganglioma syndrome. Germline mutations in three of the succinate dehydrogenase subunits (SDHD, SDHB and SDHC) increase susceptibility to head and neck paragangliomas, and familial and isolated phaeochromocytomas. 2 Differentiation between benign and malignant forms is diffi cult by imaging alone. There are no defi nite documented criteria for classifi cation as a malignant form apart from the presence of distant metastases, which must be proven with biopsy, because paragangliomas may exhibit multicentricity. 3
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عنوان ژورنال:
- BMJ case reports
دوره 2012 شماره
صفحات -
تاریخ انتشار 2012