Composite pheochromocytoma: A rare adrenal tumor

被引:1
作者
Robinet, Gwladys [1 ]
Rioux-Leclercq, Nathalie [1 ]
Manunta, Andrea [2 ]
Mathieu, Romain [2 ]
Tissier, Frederique [3 ]
Peyronnet, Benoit [2 ]
Kammerer-Jacquet, Solene-Florence [1 ]
机构
[1] CHU Pontchaillou, Serv Anat & Cytol Pathol, 2 Rue Henri le Guilloux, F-35033 Rennes 9, France
[2] CHU Pontchaillou, Serv Urol, 2 Rue Henri le Guilloux, F-35033 Rennes 9, France
[3] Hop Univ La Pitie Salpetriere, Serv Anat & Cytol Pathol, 47-83 Blvd Hop, F-75651 Paris 13, France
关键词
Composite pheochromocytome; Gangtioneuroma; Neurofibromatosis type 1; Adrenal gland; CASE SERIES; GANGLIONEUROMA; FEATURES; GLAND;
D O I
10.1016/j.annpat.2016.10.001
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
Introduction. - Composite pheochromocytoma is a rare tumor of the adrenal medulla composed of pheochromocytoma and neuroblastic tumor. We report the case of a composite pheochromocytoma detected in a patient with neurofibromatosis type 1. Case report. - A 61-year-old male patient presented occasional sweats with palpitation and moderate high blood pressure. Urinary catecholamine level was increased. CT scan showed a heterogeneous tumor limited to the adrenal gland. Histologically, the tumor showed two components: pheochromocytoma and ganglioneuroma and was diagnosed as a composite pheochromocytoma. This tumor is particularly associated with neurofibromatosis type 1, the NF1 germline gene mutation may be involved in its physiopathology. Conclusion. - Composite pheochromocytoma is a rare tumor whose pheochromocytoma component is suspected clinically but the final diagnosis is assessed by pathological examination. Prognosis is still difficult to establish due to the rarity of these tumors. (C) 2016 Elsevier Masson SAS. All rights reserved.
引用
收藏
页码:158 / 161
页数:4
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