DOI:10.2214/AJR.06.0112
AJR 2006; 187:W546-W547
© American Roentgen Ray Society
Leiomyomatosis with Extension to the Left Gluteal Muscle, Inferior Vena Cava, and Right Atrium
Bang-Bin Chen,
Chi-An Chen and
Kao-Lang Liu
National Taiwan University Hospital and National Taiwan University
College of Medicine Taipei 100, Taiwan
WEB—This is a Web exclusive article.
Keywords: CT • genitourinary tract imaging • leiomyomatosis • soft-tissue neoplasms • venography • women's imaging
A 49-year-old woman who had a 1-month history of abdominal pain,
discomfort, and bilateral leg edema and had undergone hysterectomy for a
uterine leiomyoma 10 years earlier presented with a large abdominal mass that
was palpated in the lower abdomen on physical examination. No cardiac murmur
was noted, and laboratory findings were unremarkable.
Contrast-enhanced CT of the abdomen and pelvis showed a large heterogeneous
pelvic mass between the urinary bladder and rectum with anterior displacement
of the urinary bladder and with local invasion of the left sciatic foramen and
gluteal muscles (Fig. 1A). The
tumor directly invaded the veins along the left iliac vein and inferior vena
cava to the right atrium (Figs.
1B and
1C). Bilateral hydronephrosis
and hydroureter were observed. No pulmonary metastasis was found. Neither
ovary was identified.
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Fig. 1A —49-year-old woman with abdominal discomfort for 1 month.
Axial contrast-enhanced CT image of pelvis at level of urinary bladder shows
heterogeneously enhancing lobulated mass in midline pelvis with anterior
displacement of urinary bladder (asterisk). Tumor extends to left
gluteal muscles (arrows) through greater sciatic foramen.
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CT-guided biopsy of the tumor was performed with a left gluteal approach,
and pathology revealed a leiomyoma. Given the patient's clinical history and
previous uterine leiomyoma, intravenous (IV) leiomyomatosis with extension to
the left gluteal muscle, inferior vena cava, and right atrium was diagnosed.
Surgery was not performed because the patient would not undergo surgery given
the risks of morbidity; instead, she underwent follow-up at the gynecologic
clinics, even though we explained the progressive nature of tumor emboli and
possibility of sudden death.
IV leiomyomatosis is a rare mesenchymal tumor of the uterus characterized
by microscopic intravascular proliferations of benign smooth muscle that
extend beyond the boundaries of the uterine leiomyomata
[1]. Although histologically
benign, IV leiomyomatosis may grow into the venous system and extend along the
inferior vena cava, reaching the right cardiac chamber and eventually the
pulmonary artery, resulting in death. Pulmonary or other distant metastases
are not typical. Most tumors involve the venous system on one side, and the
uterine veins are more commonly involved than the ovarian veins
[2]. The exact histogenesis of
this rare neoplasm is unclear. Recent immunohistochemical data do not support
the hypothesis of a vessel wall origin for IV leiomyomatosis
[3].
In previous reports, all patients with a history of uterine leiomyoma were
women who ranged in age from 26 to 76 years (median, 47 years). Thirty-eight
(56%) of 68 patients had undergone hysterectomy, and the median interval
between hysterectomy and the diagnosis of IV leiomyomatosis was 4 years
(range, 0.25-47 years) [2].
Tumor left in the pelvic veins at the time of hysterectomy is presumed to have
extended up the major vessels to the cardiac chambers.
The most common clinical presentations are congestive heart failure
(66.1%), abdominal distention (14.7%), and venous obstruction (5.9%)
[2]. The diagnosis is easily
missed in the early stage when tumoral extension remains inside the small
myometrial vessels.
Treatment is total surgical excision, including hysterectomy with bilateral
salpingo-oophorectomy and removal of the tumors in the heart and inferior vena
cava under deep hypothermia and circulatory arrest. In the previous
literature, the attachment site of IV leiomyomatosis was nearly always
localized, and intracaval and intracardiac leiomyomatosis was removed without
difficulty [4]. Incomplete
resection causes recurrences as long as 15 years after the primary occurrence,
leading to further surgery or death. Bilateral oophorectomy should also be
performed because the tumor is estrogen receptor sensitive.
Our patient's diagnosis was made after her abdominal pain was investigated.
Her bilateral leg edema may have resulted from poor venous return because of
obstruction of the inferior vena cava by the tumor. The tumor involved the
left gluteal muscle due to tumor extension through the left superior gluteal
vein via the sciatic foramen. Her tumor invaded the left sciatic foramen and
gluteal muscles, making complete surgical removal difficult.
References
- Norris HJ, Parmley T. Mesenchymal tumors of the uterus. V.
Intravenous leiomyomatosis: a clinical and pathologic study of 14 cases.
Cancer 1975; 36:2164
-2178[Medline]
- Lam PM, Lo KW, Yu MY, et al. Intravenous leiomyomatosis: two cases
with different routes of tumor extension. J Vasc Surg2004; 39:465
-469[CrossRef][Medline]
- Kir G, Kir M, Gurbuz A, Karateke A, Aker F. Estrogen and
progesterone expression of vessel walls with intravascular leiomyomatosis:
discussion of histogenesis. Eur J Gynaecol Oncol2004; 25:362
-366[Medline]
- Topcuoglu MS, Yaliniz H, Poyrazoglu H, et al. Intravenous
leiomyomatosis extending into the right ventricle after subtotal hysterectomy.
Ann Thorac Surg 2004;78
: 330-332[Abstract/Free Full Text]
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