Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
Histopathology. 2024 Feb;84(3):463-472. doi: 10.1111/his.15085. Epub 2023 Nov 7.
Anastomosing haemangiomas are benign tumours with anastomosing vascular channels that may mimic angiosarcoma. While anastomosing haemangiomas have been described in diverse locations, particularly the abdominal/paraspinal region, data on anastomosing haemangiomas in the mediastinum remain limited. We report the clinicopathological, radiological and molecular characteristics of the largest single-institutional series of mediastinal anastomosing haemangiomas.
In our pathology archives in 2011-23, we reviewed all vascular lesions involving the mediastinum and identified seven anastomosing haemangiomas. Clinical information was abstracted from medical charts; available radiological imaging was reviewed. Targeted DNA-based next-generation sequencing (447 genes, including GNAQ and GNA11) was performed on five cases. The seven patients included five women and two men, with an age range of 55-77 (median = 72) years. Of the six tumours with available radiology, two each were in the prevascular, visceral and paravertebral mediastinum, with lobulated peripheral enhancement in all tumours examined with contrast enhancement. Six patients underwent tumour resection; one patient received proton radiotherapy. Microscopically, each tumour was solitary and characterised by anastomosing capillary-sized vessels lined by hobnail endothelial cells. Fibrin microthrombi, hyaline globules and extramedullary haematopoiesis were common. In the five tumours analysed by next-generation sequencing, GNAQ p.Q209P was identified in one tumour; no additional reportable alterations were identified in the remaining cases. No recurrence was noted in the four patients with available follow-up of 3-58 (median = 9.5) months after resection.
While mediastinal anastomosing haemangiomas can microscopically mimic angiosarcoma, awareness of this entity and radiological correlation may help to circumvent this diagnostic pitfall.
吻合性血管瘤是一种良性肿瘤，具有吻合的血管通道，可能类似于血管肉瘤。虽然吻合性血管瘤已在不同部位（特别是腹部/脊柱旁区域）描述过，但有关纵隔吻合性血管瘤的数据仍然有限。我们报告了最大的单一机构系列纵隔吻合性血管瘤的临床病理、放射学和分子特征。
在我们 2011 年至 2023 年的病理档案中，我们回顾了所有涉及纵隔的血管病变，并确定了 7 例吻合性血管瘤。从病历中提取临床信息；回顾了可用的影像学检查。对 5 例进行了靶向基于 DNA 的下一代测序（447 个基因，包括 GNAQ 和 GNA11）。7 名患者包括 5 名女性和 2 名男性，年龄范围为 55-77 岁（中位数=72 岁）。在 6 例有可用影像学检查的肿瘤中，2 例位于前纵隔、内脏和椎旁纵隔，所有检查的肿瘤均有结节状周边增强，增强后增强。6 例患者行肿瘤切除术；1 例患者接受质子放疗。显微镜下，每个肿瘤都是孤立的，特征是吻合的毛细血管大小的血管，由钉突状内皮细胞排列。纤维蛋白微血栓、透明小体和髓外造血常见。在 5 例通过下一代测序分析的肿瘤中，1 例肿瘤中发现 GNAQ p.Q209P；在其余病例中未发现其他可报告的改变。在 4 例有随访的患者中，4 例有随访，随访时间为 3-58 个月（中位数=9.5 个月），无复发。
虽然纵隔吻合性血管瘤在显微镜下可能类似于血管肉瘤，但对这种实体的认识和放射学相关性可能有助于避免这种诊断陷阱。
