Intravenous leiomyomatosis with intracardiac extension – A review of
diagnosis and management with a case
Abstract
Intravenous leiomyomatosis with intracardiac extension is an uncommon pathologic
progression of uterine leiomyomata. It is a histologically benign condition,but own to
interfence with right sided cardiac function patients may present with marked
cardiovascular compromise and present a diagnostic dilemma to clinicians who are
unfamiliar with this condition. We present an case and provide the clinical presentation,
preoperative assessment, operative approach, pathology and postoperative issues. The
importance of a multidisciplinary approach to diagnosis and management ishighlighted.
Operative management aims to completely resect all tumour in the safest manner for the
patient, most commonly via single or two stage operation.
Key words:tumor, Intravenous leiomyomatosis,surgey
introduction
Intravenous leiomyomatosis (IVL) is a rare type of uterine leiomyoma. Although it
consists of benign smooth muscle cells, but it proliferates like malignant neoplasm, often
growing outside of the uterine and protruding into the RA through CIV and IVC[1]. IVL
can lead to sudden death by causing right heart failure.Surgery is the only way to cure
IVL, including one-stage radical surgery and staged surgery.We present an case and
provide the clinical presentation, preoperative assessment, operative approach, pathology
and postoperative issues.
Case report
A 50-year-old women presented to the hospital with a 5-month history of consistent
right lower abdominal pain. Hertemperature 37.2°C, respiratory rate 20/min, pulse
85/min, blood pressure 132/76 mm Hg (1mm Hg=0.133kPa). No cyanosis on lips, no
jugular vein distension. Normal shape of the ribcage and clear breath sounds. Heart rate is
80/min with normal rhythm. A 3cm×4cm sized mass was palpated in the right lower
abdomen with tenderness. The patient underwent hysterectomy and right oophorectomy
because of leiomyomata and right oophoritic cyst 5 months ago. CT showed the
intraluminal structure of right internal iliac vein (IIV)was unclear, lesions connected with
right pelvic mass seemed to protrude upwards into the common iliac vein (CIV).Soft
tissues were found predominantly in the lumen of the right CIV and inferior vena cava
(IVC)
and
protruded
into
the
right
atrium
(RA).
The
IVC
wasenlarged(Fig1A,1B,1C).There were light regurgitation*2.4cm of the aortic valve and
mitral valve. A consistent strip-type enhanced echo shadow was found in the whole
length of the IVC, with a coronary-section area of 1.6cm×17cm. The left ventricle had a
decreased compliance.The diameter of the IVC was 2.3cm, the diameter of the CIV was
1.9cm. Enhanced echo shadow was seen in the lumen of the CIV.Thethe colorful
bloodstream signals in the IVC and right CIV diminished. Pulsed Doppler spectrum was
periodical.Transesophagealultrasound
showed
a
30×26mm
solid
heterogeneous
echocontaining small bloodstream signals was observedat the entrance of IVC inside RA.
No obvious stem echo was observed between this solid echo and the RA wall. The
bloodstream from the IVC entrance of RA accelerated when it detoured the solid
echo.Giventhese
findings,
the
diagnosis
was
considered
as
IVCintravenous
leiomyomatosis, originating from the right IIV and protruding into the RA. The patient
underwent a RA and IVC mass resection. A midline sternotomy was performed and a
deep-hypothermic low-volume extracorporeal circulation (23°C、10ml·kg-1·min-1) was
established. In the meantime, surgeons exposed the IVC and ECV from a midline
abdominal incision and removed the mass by cutting off the stem(Fig1D). The length of
the mass was 35cm, the diameter of the venous end was 1.3cm, and the maximum
diameter of the atrium end was 3cm(Fig1E). Pathological findings: the mass consisted of
smooth muscle cells without abnormalcaryokinesis(Fig1F). The pathological diagnosis
was IVC leiomyomatosis. The patient recovered well after surgery. During 2-week
postoperative follow-up examinations, there was no abnormal findings in abdominal
duplexultrasonographyand gynecological physical examination. The cardiac and
pulmonary functions are stable, without any complications after extracorporeal
circulation. The incision got a I classification healing. The patient was discharged home
on postoperative day 21. Then GnRHa therapy was administered after discharge. No
signs of recurrence were observed during follow-up.
Discussion
Intravenous leiomyomatosis (IVL) is a rare type of uterine leiomyoma. Although it
consists of benign smooth muscle cells, but it proliferates like malignant neoplasm, often
growing outside of the uterine and protruding into the RA through CIV and IVC[1]. IVL
can lead to sudden death bycausing right heart failure. Currently, reported IVL cases
which involved heartleasions are less than 200[2], thus IVL still remains unclear in
clinical practice.A combination of imaging and pathological tests is the main diagnostic
method of IVL[3]. Clinicians can rule out the space-occupying lesions and abnormal flow
signals in the veins and heart by abdominal B-mode ultrasound, Doppler ultrasound and
transesophageal ultrasound. CT, CTA and MRI can help to obtain first impression of the
nature, shape as well as adhesions of the mass. The pathological pattern of IVL is
relatively typical, the mass is covered by a layer of single squamous mesothelium under
the microscope; the substance consists of leiomyoma cells without abnormal caryokinesis;
thick-wall microvessels can be observed in the substance, part of tissues underwent
hydropic degeneration. It is not hard to confirm the diagnosis with pathological findings.
Surgery is the only way to cure IVL, including one-stage radical surgery and staged
surgery[4]. One-stage radical surgery adopts the midline sternotomy and relative
laparotomy. Surgeons firstly remove the mass inside the vein and heart with the help of
extracorporeal circulation, then perform hysterectomy and bilateral oophorectomy. The
drawbacks of this type of procedure are long-standing operation time, large trauma and
multiple postoperative complications. On the other hand, staged surgery usually includes
two stages of operations. First-stage surgery removes the part of mass inside the IVC and
heart under extracorporeal circulation through midline sternotomy. Then second-stage
surgery is performed 10 days later and the remaining mass, uterine and bilateral adnexa
uteri are resected. Staged surgery can offset the shortages of the one-stage surgery,
however, the remaining mass and mural thrombus are risks of pulmonary embolism. We
improved the procedures according to our experience: first-stage surgery adopted
sternotomy and minimal laparotomy and removed all the mass under deep hypothermic
extracorporeal circulation[5]; second-stage surgery was performed 10 days later to
remove the uterine and bilateral appendages. As our patient already had her uterine
removed 5 months ago, we only performed the first-stage surgery in this case.
Most patients with IVL have relatively good prognosis, with the only concern of
recurrence. Thus, follow-up is essential after surgery, especially in patients who preserve
their ovaries. The patient in this case had no signs of recurrence for 3 years, which
indicated the effectiveness of our improved procedures.
Declarations :
Consent for publication： The authors declare this article for publication
Availability of data and material： The authors declare this article availability of data
and material
Competing interests: The authors declare that they have no competing interests
Funding: Not applicable
Acknowledgements: Not applicable
All authors read and approved the final manuscript
References
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Fig 1
A inferior vena cava tumor
B hepatorenal plane, inferior vena cava tumor, irregular surface
C tumor over inferior vena cava, and part of the venous wall have adhesion
D the chest and abdomen incision after extracorporeal circulation
E resection of tumor surface was smooth
F pathological diagnosis, tumor essence consists of smooth muscle cells, smooth muscle cells without
pathological fission