Division of Vascular Surgery, Dept. of General, Abdominal, Vascular and Transplant Surgery, University Hospital, Otto-von-Guericke University at Magdeburg, Germany.
Institute of Pathology, University Hospital, Otto-von-Guericke University at Magdeburg, Germany.
Pathol Res Pract. 2019 Jun;215(6):152359. doi: 10.1016/j.prp.2019.02.009. Epub 2019 Feb 28.
Intravascular leiomyoma is a rare type of myoma. It was firstly described by Birch-Hirschfeld in 1896, however, its intracardiac subtype was firstly reported by Durck in 1907. Most patients are asymptomatic. The tumor invades mostly the tributaries of the inferior vena cava (IVC) with upward extension that may approach the intracardiac space.
By means of a scientific case report, a patient with the very rare diagnosis of an endocaval leiomyoma thrombus post-hysterectomy is described based on the clinical experiences obtained in the specific case management and selective references from the literature.
A 48-years old female was diagnosed with intravascular tumor growth within the IVC with intracardiac extension using chest and abdominal CT scan, ECG and echocardiography which was approached by an interdisciplinary (vascular and cardiothoracic) surgical intervention (278 min) including heart-lung machine (99 min) with favorable postoperative result (R0 resection status with mid-term outcome, no recurrent tumor growth). Histopathological investigation diagnosed leiomyoma origin already from ovarian vein most likely in context to the former hysterectomy (3 years ago).
Intravascular leiomyoma is a benign tumor with invasive tendency, which can be considered a diagnostic and therapeutic challenge. It should be thoroughly investigated to be planned for a radical surgical removal. By possible adherence to the intraabdominal or -thoracic organs, an interdisciplinary and eventually step-wise surgical approach (combining vascular, abdominal, thoracic and heart surgery as well as gynecology and urology), which can be demanding, is recommended to be seriously considered to i) reliably achieve R0 resection status and, thus, ii) provide best outcome, quality of life and prognosis.
血管内平滑肌瘤是一种罕见的肌瘤类型。它于 1896 年由 Birch-Hirschfeld 首次描述，然而，其心内型是由 Durck 于 1907 年首次报道的。大多数患者无症状。肿瘤主要侵犯下腔静脉（IVC）的支流，向上延伸，可能接近心脏内空间。
通过科学的病例报告，描述了一例非常罕见的子宫切除术后下腔静脉内平滑肌瘤血栓形成的病例，该病例基于对特定病例管理的临床经验和对文献的选择性参考。
一名 48 岁女性因胸腔和腹部 CT 扫描、心电图和超声心动图诊断为 IVC 内血管内肿瘤生长伴心内延伸，采用多学科（血管和心胸）手术干预（278 分钟），包括心肺机（99 分钟），术后结果良好（R0 切除状态，中期结果，无肿瘤复发）。组织病理学检查诊断为平滑肌瘤起源于卵巢静脉，很可能与 3 年前的子宫切除术有关。
血管内平滑肌瘤是一种具有侵袭性的良性肿瘤，可能是诊断和治疗的挑战。应彻底调查，以计划彻底切除。由于可能与腹部或胸部器官粘连，建议认真考虑多学科、逐步的手术方法（结合血管、腹部、胸部和心脏手术以及妇科和泌尿科），该方法可能要求较高，以 i）可靠地实现 R0 切除状态，从而 ii）提供最佳的结果、生活质量和预后。
