Endocrine Abstracts

Gastrointestinal stromal tumour (GIST) is now defined as a specific, KIT-expressing and KIT-signalling driven mesenchymal tumour of the gastrointestinal (GI) tract. We report a case of stromal tumour revealed by prolonged fever and skin lesions associated with primary hyperparathyroidism and pituitary adenoma.

Observation: We present the case of a 59-year-old patient admitted for prolonged fever with inflammatory syndrome and latte tasks cutaneous lesions. Abdominal ultrasound showed two intra abdominal masses of the left hypochondrium and inter-spleno kidney. Their scanno guided biopsy concluded to a double localisation of a gastro intestinal stromal tumour CKITT⁺. The dermatological examination found a paraneoplastic dermatosis. The patient was put under Glivec 400 mg/day. A cervicothoracic CT discovered lung nodules. The colonoscopy found a juxta appendiceal tumour with multiple layered flat lesions which the biobsy concludes to a tubulo-villous adenoma with high-grade dysplasia. Hypercalcaemia 2.9 mmol/l is discovered. The diagnosis of primary hyperparathyroidism is confirmed by high PTH 217 pg/ml. A type 1 multiple endocrine neoplasia is evoked. A pituitary MRI found a micro pituitary adenoma. Hormonal exploration has concluded that it was a non-secreting adenoma. An abdominal CT scan finds a nodular hypertrophy of the left adrenal. The dosage of urinary Metanephrine was normal. This case illustrates the possible association of a stromal tumour with MEN1.