Muriel D Brada1,2, Elisabeth J Rushing3,4, David Bächinger5,4, Loris Zoller5,4, Irene A Burger6,7, Martin W Hüllner7, Holger Moch8,4, Alexander Huber5,4, Andreas H Eckhard5,4, Niels J Rupp8,4. 1. Department of Pathology and Molecular Pathology, University Hospital Zurich, Schmelzbergstrasse 12, 8091, Zurich, Switzerland. murieldiana.brada@usz.ch. 2. Faculty of Medicine, University of Zurich, Zurich, Switzerland. murieldiana.brada@usz.ch. 3. Department of Neuropathology, University Hospital Zurich, Zurich, Switzerland. 4. Faculty of Medicine, University of Zurich, Zurich, Switzerland. 5. Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital Zurich, Zurich, Switzerland. 6. Department of Nuclear Medicine, Baden Cantonal Hospital, Baden, Switzerland. 7. Department of Nuclear Medicine, University Hospital Zurich, Zurich, Switzerland. 8. Department of Pathology and Molecular Pathology, University Hospital Zurich, Schmelzbergstrasse 12, 8091, Zurich, Switzerland.
Abstract
BACKGROUND: Endolymphatic sac tumors are rare neoplasia characterized by slow growth. However, their clinical impact should not be underestimated, considering their potential for local aggressive behavior and strong association with von Hippel-Lindau syndrome. Therefore, early detection with emerging theragnostic examinations such as 68Ga-DOTATATE-PET/CT might improve patient management and reduce morbidity. METHODS: We report the clinicopathological features of seven endolymphatic sac tumors. In this cohort, we performed immunohistochemical analysis of somatostatin receptor 2A (SSTR2A) and prostate specific membrane antigen (PSMA) protein expression patterns; two targets providing rationale for novel imaging modalities such as PSMA- or SSTR-targeted PET. RESULTS: The tumor cells of all cases were negative for prostate specific membrane antigen and somatostatin receptor 2A, however immunolabeling was consistently detected in intratumoral endothelial cells of endolymphatic sac tumors for PSMA (7/7 cases, 100%), and for SSTR2A (5/7 cases, 71%). CONCLUSIONS: Our results show a high rate of PSMA and SSTR2A expression in the tumor vasculature of endolymphatic sac tumors. PSMA and SSTR2A can be targeted with appropriate radioligands for diagnostic and therapeutic purposes. This finding provides a rationale for prospective clinical studies to test this approach as a sensitive screening tool for patients with suspected endolymphatic sac tumors including an improved management of von Hippel-Lindau syndrome.
BACKGROUND: Endolymphatic sac tumors are rare neoplasia characterized by slow growth. However, their clinical impact should not be underestimated, considering their potential for local aggressive behavior and strong association with von Hippel-Lindau syndrome. Therefore, early detection with emerging theragnostic examinations such as 68Ga-DOTATATE-PET/CT might improve patient management and reduce morbidity. METHODS: We report the clinicopathological features of seven endolymphatic sac tumors. In this cohort, we performed immunohistochemical analysis of somatostatin receptor 2A (SSTR2A) and prostate specific membrane antigen (PSMA) protein expression patterns; two targets providing rationale for novel imaging modalities such as PSMA- or SSTR-targeted PET. RESULTS: The tumor cells of all cases were negative for prostate specific membrane antigen and somatostatin receptor 2A, however immunolabeling was consistently detected in intratumoral endothelial cells of endolymphatic sac tumors for PSMA (7/7 cases, 100%), and for SSTR2A (5/7 cases, 71%). CONCLUSIONS: Our results show a high rate of PSMA and SSTR2A expression in the tumor vasculature of endolymphatic sac tumors. PSMA and SSTR2A can be targeted with appropriate radioligands for diagnostic and therapeutic purposes. This finding provides a rationale for prospective clinical studies to test this approach as a sensitive screening tool for patients with suspected endolymphatic sac tumors including an improved management of von Hippel-Lindau syndrome.
Authors: A O Vortmeyer; S C Huang; C A Koch; L Governale; R D Dickerman; P E McKeever; E H Oldfield; Z Zhuang Journal: Cancer Res Date: 2000-11-01 Impact factor: 12.701
Authors: Joana Lima Ferreira; Bernardo Marques; C Willemien Menke-van der Houven van Oordt; Wouter W de Herder; Tessa Brabander; Johannes Hofland Journal: Endocrinol Diabetes Metab Case Rep Date: 2021-05-01