Ahmad Saya, Muhlebner Angelika, Snijders Tom J, de Leng Wendy W, Seute Tatjana, van Leeuwaarde Rachel S
Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, the Netherlands.
Department of Pathology, University Medical Center Utrecht, Utrecht, the Netherlands.
Cancer. 2024 Oct 15;130(20):3473-3479. doi: 10.1002/cncr.35418. Epub 2024 Jun 2.
Central nervous system hemangioblastomas are the most prevalent manifestation of von Hippel-Lindau (VHL) disease and remain the main cause of mortality. Surgical resection is the primary treatment strategy, but is not always possible, and should be used as restrictively as possible. There is an unmet need for less invasive treatment strategies, such as targeted therapy. Expression of somatostatin receptor 2A (SSTR2A) in VHL-related hemangioblastomas has been described earlier, but the extent of expression in a larger population has yet to be determined. The authors hypothesize that a substantial subset of VHL-related hemangioblastomas show SSTR2A expression, which may serve as a potential new treatment target.
Patients who were surgically treated for a VHL-related hemangioblastoma from 1990 until 2021 at the UMC Utrecht were included. Clinical data was derived from a clinical database. Tissue samples were histopathologically examined with use of hematoxylin and eosin staining, and immunohistochemical analysis of SSTR2A expression was performed.
Forty-three tissue samples were obtained from 26 patients. Nine showed strong positivity for SSTR2A expression, whereas 13 showed moderate and 15 sparse expression. Three samples showed no expression of SSTR2A. The distribution showed right-skewedness favoring a strong expression. SSTR2A expression colocalized with endothelial markers and not with stromal cells. Additionally, within-patient variability for SSTR2A expression was described in 14 patients.
SSTR2A is expressed in varying degrees in the majority of VHL-related hemangioblastomas. Future treatment with somatostatin analogues or even peptide receptor radionuclide treatment may be considered for SSTR2A-positive cases.
中枢神经系统血管母细胞瘤是冯·希佩尔-林道（VHL）病最常见的表现形式，仍是主要的致死原因。手术切除是主要的治疗策略，但并非总是可行，应尽可能严格使用。对于侵入性较小的治疗策略，如靶向治疗，仍有未满足的需求。先前已描述过生长抑素受体2A（SSTR2A）在VHL相关血管母细胞瘤中的表达，但在更大人群中的表达程度尚未确定。作者推测，相当一部分VHL相关血管母细胞瘤显示SSTR2A表达，这可能成为潜在的新治疗靶点。
纳入1990年至2021年在乌得勒支大学医学中心接受手术治疗的VHL相关血管母细胞瘤患者。临床数据来自临床数据库。组织样本采用苏木精和伊红染色进行组织病理学检查，并进行SSTR2A表达的免疫组织化学分析。
从26例患者中获取了43个组织样本。9个样本显示SSTR2A表达强阳性，13个样本显示中度表达，15个样本显示弱阳性。3个样本未显示SSTR2A表达。分布呈右偏态，倾向于强表达。SSTR2A表达与内皮标志物共定位，而非与基质细胞共定位。此外，描述了14例患者中SSTR2A表达的患者内变异性。
大多数VHL相关血管母细胞瘤中SSTR2A呈不同程度表达。对于SSTR2A阳性病例，未来可考虑使用生长抑素类似物甚至肽受体放射性核素治疗。
