Intravascular Leiomyomatosis as a Rare Cause of Nonthrombotic Pulmonary Embolism

Abstract

Intravascular leiomyomatosis (IVL) is a very rare condition. It is characterized by the proliferation of benign smooth muscle cells within vascular structures without invasion of these tissues. Symptoms depend on the site of origin and the extent of invasion. Rarely, this neoplasm is located in the inferior vena cava or in the pulmonary vasculature potentially causing symptoms of dyspnea, chest pain, or syncope. We report the case of a 53-year-old woman who was referred to our hospital with extensive pulmonary embolism comprising of a subtotal occlusion of the right pulmonary artery with extension into the left pulmonary artery. Due to persistent dyspnea (New York Heart Association class II) despite anticoagulation, after a six-week period, imaging was repeated and showed stable findings. As she was not responding to adequate anticoagulant therapy, intima sarcoma of the pulmonary artery was suspected, and a pulmonary endarterectomy (PEA) was performed. A smooth, white, intravascular mass was easily and completely removed. Analysis demonstrated a lesion consisting of cells without atypia, showing expression of alpha-smooth muscle actin (alpha SMA) and desmin with partial expression of estrogen receptor (ER) and progesterone receptor (PR), leading to the diagnosis of intravascular leiomyomatosis. The patient fully recovered. Complete surgical removal of the intravascular tumor is recommended to relieve symptoms and prevent possible complications. Clinicians have to be aware that in unresolved pulmonary embolism, nonthrombotic and rare causes, like an intima sarcoma or intravascular leiomyomatosis, should be considered.

“Evolution” of intravascular leiomyomatosis

“…Chen et al analyzed the clinical data of 361 IVL patients(38 patients from Qilu Hospital of Shandong University and 323 patients from the published literature) and confirmed the hypothesis that IVL originates from the uterus to a certain extent [ 8 ]. Similarly, Van et al found that the estrogen and progesterone receptor in IVL cells were weakly positive to strongly positive, while normal vascular smooth muscle cells were often negative or weakly positive, also confirming that IVL originated in the uterus [ 9 ]. The patient we report here obtained IVL after hysteroscopic myomatectomy.…”

Right atrial tumor revealing intravascular leiomyomatosis: about a case and literature review

“…Clinical symptoms may include cardiac signs such as syncope, cardiac myxoma-like symptoms, right heart failure, Budd-Chiari syndrome, systemic embolism, and pulmonary embolism [ 7 ]. Recurrent ascites and sudden death are also potential clinical manifestations [ 3 ].…”

Intravenous Leiomyomatosis: An Uncommon Cause of Pulmonary Embolism

“…They are formed by a variety of cell types, including adipocytes, amniotic, trophoblastic, and neoplastic cells, as well as foreign or infectious material [ 6 ]. IVL as a cause of NTPE is exceptionally rare, the diagnosis difficult, and based on comprehensive clinical history and imaging including CT scan with contrast, ECHO and often MRI [ 7 ]. Unfortunately, due to the diagnostic challenges, the final diagnosis is often made at the time of surgery or when repeated imaging reveals persistent pulmonary embolism not responsive to anticoagulation therapy [ 5 , 7 ].…”

“…IVL as a cause of NTPE is exceptionally rare, the diagnosis difficult, and based on comprehensive clinical history and imaging including CT scan with contrast, ECHO and often MRI [ 7 ]. Unfortunately, due to the diagnostic challenges, the final diagnosis is often made at the time of surgery or when repeated imaging reveals persistent pulmonary embolism not responsive to anticoagulation therapy [ 5 , 7 ]. Our patient presented with symptoms and findings suggestive of PE with a large right atrial mass.…”