A 52-year-old female with no significant past medical history presented
to her primary care physician with bilateral lower extremity swelling and lower
extremity venous varicosities, both of which had worsened during the previous 2
She underwent a partial hysterectomy for uterine fibroids 8 years before
the current presentation. Review of systems included worsening abdominal
discomfort from enlarging uterine fibroids for 6 to 12 months before
presentation. Physical exam revealed a lower abdominal mass originating from
the pelvis and bilateral lower extremity edema along with dilated veins.
Preliminary laboratory tests with complete blood counts and
comprehensive metabolic panel was unremarkable. An MRI of the abdomen and
pelvis demonstrated a 24 cm × 11 cm × 21 cm heterogeneous mass
arising from the uterine myometrium with areas of hemorrhage and necrosis
(Figure 1). The mass extended into the extrauterine pelvic veins, inferior vena
cava, and the right atrium and right ventricle.
Figure 1. Panel 1a shows a contrast-enhanced axial T1-weighted
image of the abdomen with a filling defect in the inferior vena cava.
Images courtesy of M. Ghesani, MD
shows an axial T1W post-contrast image through the pelvis demonstrating a
heterogeneous necrotic mass.
Panel 1c is a sagittal T1-weighted post-contrast image
demonstrating the mass arising from the uterine myometrium.
Differential diagnosis at the time included both malignant and benign
etiologies, such as leiomyosarcoma and IV leiomyomatosis. A PET/CT was done to
assess malignant potential. It revealed a low-grade hypermetabolism of the
uterine mass with a maximum standard uptake value of 2.7 and extensive tumor
thrombus involving the extrauterine pelvic veins extending into the right
ventricle with low-grade hypermetabolism (Figure 2).
Figure 2. PET/CT images of the pelvic mass show low-grade
fluorodeoxyglucose uptake in the mass.
This could be suggestive of benign etiology such as IV leiomyomatosis
(IVL), but a literature search did not reveal any reported cases of PET/CT
findings in IVL for comparison. CT angiogram of the chest, abdomen and pelvis
performed for surgical planning confirmed the above findings
Figure 3. Coronal and sagittal images from CT angiogram
demonstrate an extensive heterogeneously enhancing tumor originating in the
uterus and extending into the inferior vena cava, right atrium and right
The patient underwent a single-stage resection of the pelvic tumor,
thrombectomy and thoracotomy for removal of the tumor in its entirety,
including the right atrial and ventricular components. Her postoperative course
was uneventful. Pathology of the resected tumor revealed benign proliferation
of smooth muscle with intravascular extension consistent with IVL (Figure 4).
Figure 4. Gross specimen shows pink-tan benign smooth muscle
neoplasm consistent with intravenous leiomyomatosis.
IVL is a rare uterine smooth muscle tumor characterized by IV growth and
extension of benign smooth muscle. The tumor originates in the pelvic veins and
extends upward to varying distances, including the inferior vena cava, adrenal
and renal veins, right heart (intracardiac leiomyomatosis) and pulmonary
Pathogenesis is unclear, although it is postulated to be arising from
uterine leiomyoma invading into the uterine vein and extending along the lumen
of the major veins. The incidence of IVL is thought to be underestimated
because, in the early stages, the tumor extension remains in the vessels of the
myometrium and cannot be detected on imaging. The correct diagnosis relies on a
high index of suspicion and should be suspected whenever an intravascular tumor
is noted during surgery or microscopic analysis.
Lam and colleagues reported summary of 62 cases. In their series, the
median age of presentation was 42 years. The most common presenting features
included congestive heart failure, features of venous obstruction and abdominal
distention. Other clinical features such as pelvic discomfort, low abdominal
pain and irregular vaginal bleeding due to pelvic mass, uterine myoma, lower
extremity edema due to inferior vena cava occlusion, dyspnea, chest pain and
even sudden death due to mechanical obstruction of the heart have been reported
by other authors.
More than 50% of the patients in the series had history of hysterectomy
before the presentation as IVL. In these cases, there is a possibility that the
IVL could have been missed at an earlier stage in the pelvic veins during the
Preoperative imaging is important to define the extent of tumor, as the
presence of intrathoracic disease affects the surgical approach. CT angiogram
with 3-D post-processing can show the path and extent of disease. Some of the
features on the CT could be suggestive of IVL. These include pelvic masses that
are continuous with the mass in the veins, inhomogeneous enhancement and
Enhancement of the tumor thrombosis is similar to that of renal
carcinoma, hepatocellular carcinoma and adrenal cortical carcinoma, but the
lesion is more extensive in IVL. Differential diagnosis of IVL includes IV
thrombus, leiomyosarcoma, right atrial myxoma and tumor thrombosis with
malignant carcinoma. The history of past or present uterine myoma with the
above-mentioned CT finding can help with the diagnosis.
Surgery remains the treatment of choice. Surgical management includes a
total abdominal hysterectomy and bilateral salpingo-oophorectomy. The ovaries
usually are removed, as these tumors are estrogen sensitive. Complete resection
is mandatory. This may involve thoracotomy and cardiac surgery, including
cardiopulmonary bypass depending on the extent of spread. Gross examination of
the intravascular mass could show worm-like plugs of tumor, and histology
usually demonstrates benign proliferation of smooth muscle with intravascular
extension. Prognosis after surgery usually remains very good.
Munir Ghesani, MD, is an attending radiologist at St.
Lukes-Roosevelt Hospital Center and Beth Israel Medical Center, an
associate clinical professor of radiology at Columbia University College of
Physicians and Surgeons, and a HemOnc Today section editor. Nasima
Jafferjee, MD, is a radiology resident at St. Lukes-Roosevelt Hospital
Chintapatla, MD, is a fellow in hematology and oncology at St.
Lukes-Roosevelt Hospital Center. Peerapod Chiowanich, MD, is an assistant
attending radiologist at St. Lukes-Roosevelt Hospital Center. Darren
Buonocore, MD, is a post-doctoral residency fellow in pathology at St.
Lukes-Roosevelt Hospital Center. Alan Benvenisty, MD, is chief of the
division of transplant surgery at St. Lukes-Roosevelt Hospital Center.
Disclosure: Drs. Ghesani, Jafferjee, Chintapatla, Chiowanich, Buonocore
and Benvenisty report no relevant financial disclosures.
For more information:
- Bertrand P. Arch Surg. 1998;133:460-462.
- Clement PB. Am J Surg Pathol. 1988;12:932-945.
- Gawne-Cain ML. Clin Radiol. 1995;50:123-125.
- Lam PM. J Vasc Surg. 2004;39:465-469.
- Moorjani N. J Card Surg. 2005;20:382-385.
- Peng HJ. Abdom Imaging.