Correspondence

Extension of a Pelvic Tumor into the Right Atrium

N Engl J Med 1995; 333:1013-1014October 12, 1995

Article

To the Editor:

Secondary localization of intrathoracic tumors in the heart is often due to metastatic spread or neoplastic infiltration of veins. Renal, adrenal, and hepatic carcinomas as well as leiomyomas of the uterus can also reach the heart through the inferior vena cava. Fewer than 100 cases of intraluminal extension of benign smooth-muscle tumors into uterine and pelvic veins have been reported,1 and fewer than 30 cases of intracardiac leiomyomatosis have appeared in the literature.2

We describe a 39-year-old woman without any previous disease, who was admitted for positional syncope that occurred when she bent forward. The same symptoms appeared during deep breathing, while she was seated. Echographic scan of the heart and abdomen showed an echogenic mass extending from the inferior vena cava and occupying about 70 percent of the right atrium, with diastolic protrusion into the right ventricle. At the pelvic level, on the left side of the uterus, a mass with a maximal diameter of 9 cm was also detected. These findings were confirmed by computed tomography. The patient underwent emergency cardiac surgery, with partial transatrial leiomyomectomy (Figure 1AFigure 1Tumor Removed during Emergency Cardiac Surgery. and Figure 1B). The histologic pattern of the excised mass was that of a lymphangioleiomyoma. Three months later the patient underwent total abdominal hysterectomy, with removal of the tumor, which extended from the left common iliac vein through the inferior vena cava into the right atrium. One year after hysterectomy the patient has no symptoms.

Intravenous leiomyomatosis is a rare condition in which macronodules of benign smooth-muscle tissue grow within myometrial veins and can extend into extrauterine vascular structures, so that the tumor can reach the heart through the inferior vena cava,3 by means of wormlike projections. To be deemed intravenous leiomyomatosis, any extrauterine benign smooth muscle must remain within the vascular lumen or cardiac chambers.4 There are two principal theories about the origin of intravenous leiomyomatosis: it may arise from the smooth-muscle wall of a myometrial vein, or it may result from unusual vascular invasion by a uterine leiomyoma. The longest survival is reported to be 16 years, but the outcome may be poor if the extension of the tumor is not recognized at the time of surgery.5

Benedetta Stancanelli, M.D.
Giuseppe Seminara, M.D.
Alfonsina Vita, M.D.
Aurelio Pantò, M.D.
Marcello Romano, M.D.
Università di Catania, 95126 Catania, Italy

5 References

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   Suginami H, Kaura R, Ochi H, Matsuura S. Intravenous leiomyomatosis with cardiac extension. Obstet Gynecol 1990;76:527-529
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   Rotter AJ, Lundell CJ. MR of intravenous leiomyomatosis of the uterus extending into the inferior vena cava. J Comput Assist Tomogr 1991;15:690-693
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   Sternberg SS, ed. Diagnostic surgical pathology. New York: Raven Press, 1989:1642-3.
   

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   Harper RS, Scully RE. Intravenous leiomyomatosis of the uterus: a report of four cases. Obstet Gynecol 1961;18:519-529
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Citing Articles (4)

Citing Articles

1. 1

   M. Leitman, R. Kuperstein, B. Medalion, A. Stamler, E. Porat, S. Rosenblatt, E. Konen, R. Krakover, Z. Vered. (2008) A highly unusual right atrial mass presented in two women. European Journal of Echocardiography 9:6, 833-834
   CrossRef

2. 2

   F Roques. (2001) Role of pre-operative assessment in the surgical management of leiomyoma extended to the right heart chambers: a compendium of information from isolated reports. European Journal of Cardio-Thoracic Surgery 19:4, 522-524
   CrossRef

3. 3

   Yoshihiko Tsuji, Chojiro Yamashita, Hidetaka Wakiyama, Yoshiya Toyoda, Masato Yoshida, Takaki Sugimoto, Keiji Ataka, Noboru Ishii, Tsutomu Shida, Masayoshi Okada. (1998) Surgical treatment for transvenous tumor extension into the heart: Four cases. Journal of Vascular Surgery 27:4, 740-744
   CrossRef

4. 4

   Ian Boiskin, Richard E. Marsan, Mark Boles, William R. Jacobs, Terrence C. Demos. (1997) Hepatocellular carcinoma with intraatrial extension. Cardiovascular and Interventional Radiology 20:4, 324-327
   CrossRef