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Year : 2011  |  Volume : 54  |  Issue : 1  |  Page : 158-160
Intracardiac leiomyomatosis: Report of two cases

1 Department of Pathology (Cardiovascular and Thoracic Division), Seth G.S. Medical College and KEM Hospital, Parel, Mumbai - 400 012, India
2 Dr. P. K. Sen Department of Cardiovascular & Thoracic Surgery, Seth G.S. Medical College and KEM Hospital, Parel, Mumbai - 400 012, India
3 Department of Radiology, Seth G.S. Medical College and KEM Hospital, Parel, Mumbai - 400 012, India

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Date of Web Publication7-Mar-2011


Intravenous leiomyomatosis is an example of unusual growth patterns that is sometimes exhibited by uterine leiomyomas, characterized by smooth muscle proliferations within the uterine or pelvic veins. Extension into the heart via the inferior vena cava is even rarer and is designated as intracardiac leiomyomatosis. This is a report of two such cases that had preoperative diagnoses of a thrombus and right atrial myxoma, respectively.

Keywords: Benign tumors, heart, inferior vena cava, leiomyoma, uterus

How to cite this article:
Vaideeswar P, Kulkarni DV, Karunamurthy A, Hira P. Intracardiac leiomyomatosis: Report of two cases. Indian J Pathol Microbiol 2011;54:158-60

How to cite this URL:
Vaideeswar P, Kulkarni DV, Karunamurthy A, Hira P. Intracardiac leiomyomatosis: Report of two cases. Indian J Pathol Microbiol [serial online] 2011 [cited 2022 May 20];54:158-60. Available from: https://www.ijpmonline.org/text.asp?2011/54/1/158/77388

   Introduction Top

Benign smooth muscle neoplasms or leiomyomas are extremely common uterine tumors occurring in 30-40% of women during reproductive years. These may remain asymptomatic or produce symptoms referable to size and location within the uterus. On rare occasions, the neoplasms exhibit unusual growth patterns whose management may be beyond the domain of the gynecologists. [1] One such pattern is intravenous leiomyomatosis (IVL), characterized by growth of smooth muscle within the uterine or pelvic veins. [2] Even rarer is their extension into the inferior vena cava (IVC) and heart, the therapeutic options of which fall into the realms of the cardiovascular surgeon. This is a report of two such cases that had preoperative diagnoses of a thrombus and right atrial myxoma, respectively.

   Case Reports Top

Case 1

A 32-year-old woman was admitted in a tertiary cancer hospital with history of exertional dyspnea for the past 2 months. Her past medical history had included a subtotal hysterectomy performed 6 months ago for uterine leiomyoma in a private hospital. An echocardiographic examination revealed an echogenic mass extending from the IVC into the right atrium (RA) with prolapse into right ventricle (RV). Left and right ventricular systolic functions were normal. Computed tomographic scan with contrast revealed a large filling defect within IVC, with an intracardiac extension [Figure 1]a and b. A diagnosis of an intracaval thrombus was entertained and the patient received low molecular weight heparin and oral anticoagulants.
Figure 1: Case 1: (a and b) Axial and reconstructed sagittal images of abdomen in venous phase reveal a filling defect (arrows) in the IVC extending from infra-renal portion up to RA (note dilatation of the vein); (c) smooth-surfaced serpentine tumor; (d) cut surface is gray-white, glistening with few cystic areas (arrows point to the IVC end)

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The patient was then referred to Department of Cardiovascular and Thoracic Surgery at our center for the surgical excision of the IVC thrombus. A target color doppler demonstrated a large, free floating, loose mass moving across the tricuspid valve without significant obstruction and without encroachment of RV outflow tract. The operation was performed with median sternotomy, and cardiopulmonary bypass was established. A right atriotomy from mid-right atrium toward IVC was performed and a capsulated tumor was found extending from RA into RV. The tumor did not show attachment to the wall of the infrahepatic IVC but was attached to its tributaries below renal veins. The tumor was extracted with a gentle pressure. The patient was weaned off bypass without support and had an uneventful postoperative recovery.

The excised mass [Figure 1]c and d, sent for histopathology, measured 28 cm in length with a maximum diameter of 3.8 cm. It was smooth-surfaced and soft to firm in consistency; at places, it felt distinctly cystic. The cardiac end was smooth and rounded while the caval end had short blunt finger-like projections. The mass, cut along its long axis, revealed gray-white, slightly fasciculated areas with interspersed cystic areas containing greenish-yellow mucinous material. A haphazard proliferation of benign smooth muscle cells alternating with areas of hyalinization and abundant myxoid change were seen on histology. The striking feature was the presence of large numbers of vascular channels ranging from dilated capillaries to large artery-like vessels [Figure 2]. There was no evidence of mitosis or tumor necrosis. The diagnosis of intracaval leiomyomatosis was made. In view of this diagnosis, the surgical data of past hysterectomy were also reviewed. The microscopy was suggestive of angioleiomyoma; tumor cells had been positive for smooth muscle actin and negative for C-Kit. Completion hysterectomy and excision of pelvic leiomyoma was performed 7 months later. There was no recurrence of tumor on 1 year 7 months of follow-up.
Figure 2: (a) Bundles of benign spindle-shaped cells in a collagenous and vascularized stroma; (b and c) presence of thick-walled blood vessels (hematoxylin and eosin, ×250); (d) plump spindle cells with classic cigar-shaped nuclei. There is no nuclear pleomorphism or mitosis (hematoxylin and eosin, ×400). Inset shows smooth muscle actin positivity in the spindle-shaped cells (×400)

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Case 2

A 53-year-old lady was admitted to the cardiovascular and thoracic surgical center with a 6-month history of progressive breathlessness, palpitation and episodic chest pain. On clinical examination, her jugular venous pressure was 7 cm and she had a loud first heart sound with grade III/IV mid-diastolic murmur at left lower sternal border. Chest radiography revealed a cardiothoracic ratio of 0.65 with enlargement of RA. Her echocardiogram revealed a large, mobile mass attached to the inferior cavo-atrial junction, extending into the IVC little beyond the hepatic veins. There was also mild tricuspid regurgitation. Ventricular function and coronary angiogram were normal. With a clinical diagnosis of RA myxoma, she underwent tumor excision via a vertical right atriotomy under hypothermic cardiopulmonary bypass and multidose cold hyperkalemic sanguineous cardioplegia. She was discharged from the hospital after an uneventful postoperative course.

The surgical specimen [Figure 3] revealed an elongated, 12 cm × 2.5 cm × 2.5 cm, cylindrical sausage-like mass. It had a smooth, glistening, undulant surface and firm consistency. The cut surface showed a glistening gray-white appearance with few foci of hemorrhage. The histomorphology was almost identical to the first case. On subsequent follow-up, a pelvic ultrasonography revealed multiple leiomyomas of the uterus, but the patient refused further investigations and treatment.
Figure 3: Case 2: (a) External surface and (b) cut surface of a sausage-shaped mass. Arrow points to the site of attachment to the inferior cavo-atrial junction

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   Discussion Top

In this presentation, we have reported intracardiac extension of IVL in two women who had multiple uterine fibroids. This non-invasive extension of IVL into the right side of the heart is designated as intracardiac leiomyomatosis (ICL). It is exceedingly rare and is said to affect 10% cases of IVL. [3] One of the earliest cases was documented in 1903 as cited by Grella et al., [4] and till 2006, only about 100 of these cases had been reported. [5] Most patients are usually middle-aged and/or premenopausal (as seen in our cases) with a median age of 44 years, though the age range is wide ranging from 28 to 80 years. [3]

One of our patients had undergone hysterectomy 6 months before the onset of cardiac symptoms. A review of literature reveals that over half of the women with ICL had a history of hysterectomy; the median time between the two was 4 years. [6] It is presumed that in such cases that the tumoral extension into uterine or pelvic veins escapes detection on preoperative imaging, or at operation or even on pathological examination of hysterectomy specimens. [6] This leaves sufficient time for the proliferating smooth muscle to not only extend into the major vessels, but also spread their tentacles into the cardiac chambers. In majority of the cases, since involvement of the IVC is fairly constant, symptoms of venous obstruction are attributable to mass lesion in the vena cava and the heart; the severity often depends on the length and width of the lesion. The widest diameter usually falls between 2 and 3 cm, though the length may vary. [7]

The intracardiac extension causes right-sided valvular obstruction or cavitary compromise that produces features of dyspnea (seen in our patients), syncope, murmurs or clicks. [3] Some patients remain asymptomatic, while few others have high-output state, pulmonary or even systemic embolism and sudden death. [3] Attachment to the IVC was seen in the first patient and the presence of blunt projections at one end reiterates the tumor origin from the pelvic veins. However, in the second patient, the tumor was attached to the inferior cavo-atrial junction with a "retrograde" prolapse (not reported before to the best of our knowledge), which made us wonder if this tumor truly arose from the vascular smooth muscle. But this school of thought is not supported by immmunohistochemical studies. [8]

Imaging studies such as transthoracic or transesophageal echocardiography, computed tomography and magnetic resonance imaging are important investigations, in general, that have proved to be useful in identifying and delineating mass lesions within the heart and vessels. Because of its non-specific clinical presentation and rarity, ICL continues to be misdiagnosed as either thrombus or myxoma as was the case in our patients. [9] More often than not, the final diagnosis is made after surgical resection and histological examination. [5] It is interesting to note that there is growing awareness of this condition and hence more than 50% of ICL have been reported in the past 10 years. [6]

Complete removal of the tumor is mandatory as this prevents recurrences, damage to the right-sided valvular apparatus and major complications like pulmonary tumor embolism or sudden death. [6],[9] The procedure involves not only removal of the atrial, caval and other venular extensions, but also total hysterectomy with bilateral salpingo-oophorectomy (as the tumor is said to estrogen-dependent). [7] An adequate preoperative diagnosis should provide detailed information about the tumor localization, extravascular and intravascular diameters, site of vascular entry, and patency of iliac and femoral veins. [10] If this is available, a single-stage procedure with combined thoracic and abdominal approach is performed. But on most occasions, a two-stage procedure has to be adopted. [6],[9],[10] In the first patient, a subtotal hysterectomy had been performed initially. Only the cavo-atrial tumor had been excised and the caval extension was gently extracted. This may not be case in all patients as the leiomyoma may be firmly attached to the IVC and a hard pull may cause retroperitoneal hemorrhage. [6] The second patient refused further treatment even though the anticipated problems were explained to her.

IVL with an intracardiac extension should be considered as one of the differential diagnosis in middle-aged patients with past history of hysterectomy or with uterine tumors. Surgical resection by a multidisciplinary surgical team is the treatment of choice.

   References Top

1.Fasih N, Prasad Shanbhogue AK, Macdonald DB, Fraser-Hill MA, Papadatos D, Kielar AZ, et al. Leiomyomas beyond the uterus: Unusual locations, rare manifestations. Radiographics 2008;28:1931-48.  Back to cited text no. 1
2.Norris HJ, Parmley T. Mesenchymal tumors of the uterus. V. Intravenous leiomyomatosis. A clinical and pathologic study of 14 cases. Cancer 1975;36:2164-78.   Back to cited text no. 2
3.Kullo II, Oh JK, Keeney CL, Khandheria BK, Seward JB. Intracardiac leiomyomatosis. Echocardiographic features. Chest 1999;115:587-91.   Back to cited text no. 3
4.Grella L, Arnold TE, Kvilekval KH, Giron F. Intravenous leiomyomatosis. J Vasc Surg 1994;20:987-94.   Back to cited text no. 4
5.Castelli P, Caronno R, Piffaretti G, Tozzi M. Intravenous uterine leiomyomatosis with right heart extension: Successful two-stage surgical removal. Ann Vasc Surg 2006;20:405-7.   Back to cited text no. 5
6.Lam PO, Lo KW, Yu MY, Wong WS, Lau JY, Arifi AA, et al. Intravenous leiomyomatosis: Two cases with different routes of tumor extension. J Vasc Surg 2004;39:465-9.   Back to cited text no. 6
7.Ling FT, David TE, Merchant N, Yu E, Butany JW. Intracardiac extension of intravenous leiomyomatosis in a pregnant woman: A case report and review of the literature. Can J Cardiol 2000;16:73-9.  Back to cited text no. 7
8.Kir G, Kir M, Gurbuz A, Aker F. Estrogen and progesterone expression of vessel walls with intravascular leiomyomatosis: Discussion of histogenesis. Eur J Gynaecol Oncol 2004;25:362-6.  Back to cited text no. 8
9.Kutay V, Tuncer M, Harman M, Ekim H, Yakut C. Intracardiac extension of intravenous leiomyoma. Tex Heart J 2005;32:232-4.   Back to cited text no. 9
10.Roques F, Sanchez B, Bucher B, Lariviere J. Role of pre-operative assessment in the surgical management of leiomyoma extended to the right heart chambers: A compendium of information from isolated reports. Eur J Cardiothorac Surg 2001;19:522-4.  Back to cited text no. 10

Correspondence Address:
Pradeep Vaideeswar
Department of Pathology (Cardiovascular and Thoracic Division), Seth G.S. Medical College and KEM Hospital, Parel, Mumbai - 400 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.77388

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  [Figure 1], [Figure 2], [Figure 3]

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