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Death due to intravenous leiomyomatosis extending to the right pulmonary artery
202
Pathology (2004), 36(2), April
CORRESPONDENCE
Death due to intravenous leiomyomatosis extending to the right pulmonary artery Sir, Intravenous leiomyomatosis is a rare benign uterine proliferation of smooth muscle involving myometrial veins that may extend into the inferior vena cava and right side of the heart.1 It may be a cause of significant morbidity and even mortality. A recent review of the literature revealed a total of 16 cases with intracaval and intracardiac extension.2 We present the case of a 47-yearold woman who presented with collapse, was considered clinically to have a pulmonary thromboembolus, for which she was anticoagulated, and who subsequently had a cardiac arrest and died. Intravenous leiomyomatosis extending into the right pulmonary artery was found at autopsy. The clinical and pathological features of intravenous leiomyomatosis are discussed. A 47-year-old woman was seen at a major teaching hospital with an episode of collapse in which she lost consciousness for approximately 5 min. The episode was diagnosed as a vasovagal incident and she was discharged. She re-presented to a different teaching hospital some 7 days later with a history of daily collapses associated with palpitations, tightness in the chest and throat, and acute shortness of breath. A ventilation perfusion scan suggested a pulmonary thromboembolus. The patient suffered a cardiac arrest with electromechanical dissociation whilst being prepared for an echocardiogram. Resuscitative measures were successful, and she was treated with anticoagulation therapy and thrombolysis and had an inferior vena caval filter inserted. An echocardiogram showed extensive thrombus within the inferior vena cava, right atrium and ventricle, and right ventricular outflow tract. A second cardiac arrest occurred from which she could not be resuscitated. The post-mortem examination revealed an encapsulated, cylindrical mass within the left common iliac vein extending through the inferior vena cava, right atrium, right ventricle and into the right pulmonary artery (Fig. 1, 2). No thrombus was seen within the deep calf veins. Within the uterus was a circumscribed mucoid tumour measuring 10 cm in maximum extent with a whorled
Fig. 1 Overall view of organ block showing intravenous leiomyomatosis within inferior vena cava (arrow).
Fig. 2
Intravenous leiomyomatosis within right pulmonary artery.
trabecular cut surface. No necrosis or haemorrhage was seen in the tumour. The mass within the iliac vein was tracked back to myometrial veins in the region of the uterine tumour. The rest of the autopsy was normal. The provisional autopsy differential diagnosis was of a uterine leiomyosarcoma with extension into the myometrial and pelvic veins, inferior vena cava, right heart and pulmonary artery, or uterine leiomyosarcoma, with associated thrombosis of myometrial veins extending to pelvic veins and right pulmonary artery. Histological examination of the intravascular mass and the uterine tumour showed fasicles of bland spindle cells with features of smooth muscle and no evidence of atypia, mitoses or necrosis (Fig. 3). The spindle cells of both the intravascular mass and the uterine tumour stained strongly and diffusely for desmin and actin (Fig. 4). The features were consistent with intravenous leiomyomatosis.3 True intravenous leiomyomatosis, by definition, is seen outside the borders of a leiomyoma. Vascular invasion within a leiomyoma (leiomyoma with vascular intrusion)
Fig. 3 Intravenous tumour composed of bland spindle cells with features of smooth muscle (H&E, original magnification, 6200).
CORRESPONDENCE
203
Contact Dr Michael Burke on E-mail: michaelb@vifp. monash.edu.au ACKNOWLEDGEMENT We thank the mortuary and histology staff, Kerry Johannes, Caroline Rosenberg and the State Coroner for making available his files.
Fig. 4 Intravenous tumour showing cells staining strongly and diffusely for desmin (Desmin immunoperoxidase stain, original magnification, 6200).
does not warrant the diagnosis of intravenous leiomyomatosis. This latter situation has not been found to be associated with the extension of tumour beyond the uterus or of other evidence of aggressive behaviour. Microscopically, the tumour may be found only within venous lumina, continuous with the venous wall, or be confined to the muscularis of the venous wall. In the first situation, it appears a uterine leiomyoma extends into uterine veins, as applied with our case. In the other situations, it is thought the tumour arises from venous smooth muscle.4 Histologically, the appearance of the intravascular tumour may resemble that of a leiomyoma, as applied with our case, but it may have a lobulated or clefted outline and be hyalinised and/or hydropic. Intravenous leiomyomatosis may demonstrate the same histological variations seen in leiomyomas, including cellular, bizarre, epithelioid, myxoid and angiomatoid variants. Clinically, intravenous leiomyomatosis may present as palpitations, syncope, exertional dyspnoea and very rarely as sudden death. Tumour extending to the tricuspid valve is particularly associated with a heightened risk of sudden death.5 Imaging techniques—including computed tomography, magnetic resonance imaging, echocardiography and angiography—are important for diagnosis, demonstrating the extent of the disease and planning surgical intervention.6 Cases of successful surgical resection with cardiopulmonary bypass have been described.7,8 In summary, intravenous leiomyomatosis is a rare tumour that may extend to the right side of the heart and be a significant cause of morbidity and even mortality. Our case adds to the literature on the exceedingly rare occurrence of death due to intravenous leiomyomatosis. It is only the 16th case of intracaval and intracardiac intravenous leiomyomatosis described. Michael Burke* Kenneth Opeskin { *Victorian Institute of Forensic Medicine, Department of Forensic Medicine, Monash University, Southbank, Victoria and {Department of Anatomical Pathology, St Vincent’s Hospital, Fitzroy, Victoria, Australia
1. Quade BJ, Dal Cin P, Neskey DM, et al. Intravenous leiomyomatosis: molecular and cytogenetic analysis of a case. Mod Pathol 2002; 15: 351±6. 2. Nam MS, Jeon MJ, Kim YT, Kim JW, Park KH, Hong YS. Pelvic leiomyomatosis with intracaval and intracardiac extension: a case report and review of the literature. Gynecol Oncol 1997; 64: 547±50. 3. Silverberg SG, Kurman RJ. Smooth muscle and other mesenchymal tumours. In: Rosai J, editor. Tumors of the Uterine Corpus and Gestational Trophoblastic Disease. 3rd ed. Washington: Armed Forces Institute of Pathology, 1992; 113±52. 4. Clement PB. Intravenous leiomyomatosis of the uterus. Pathol Ann 1998; 23: 153±83. 5. Roman DA, Mirchandani H. Intravenous leiomyoma with intracardiac extension causing sudden death. Arch Path Lab Med 1987; 111: 1176±8. 6. Stolf NA, dos Santos GG, Haddad VL. Unusual abdominal tumours with intracardiac extension. Two cases with successful surgical resection. Rev Hos Clin Fac Med Sao Paulo 1999; 54: 195±64. 7. Ricci MA, Cloutier LM, Mount S, et al. Intravenous leiomyomatosis with intracardiac extension. Cardiovasc Surg 1995; 3: 693±6. 8. Stolf NA, Santos GG, Hahhard VLS, et al. Successful one-stage resection of intravenous leiomyomatosis of the uterus with extension into the heart. Cardiovasc Surg 1999; 7: 661±4.
Pathology (2004), 36(2), April
CORRESPONDENCE
Death due to intravenous leiomyomatosis extending to the right pulmonary artery Sir, Intravenous leiomyomatosis is a rare benign uterine proliferation of smooth muscle involving myometrial veins that may extend into the inferior vena cava and right side of the heart.1 It may be a cause of significant morbidity and even mortality. A recent review of the literature revealed a total of 16 cases with intracaval and intracardiac extension.2 We present the case of a 47-yearold woman who presented with collapse, was considered clinically to have a pulmonary thromboembolus, for which she was anticoagulated, and who subsequently had a cardiac arrest and died. Intravenous leiomyomatosis extending into the right pulmonary artery was found at autopsy. The clinical and pathological features of intravenous leiomyomatosis are discussed. A 47-year-old woman was seen at a major teaching hospital with an episode of collapse in which she lost consciousness for approximately 5 min. The episode was diagnosed as a vasovagal incident and she was discharged. She re-presented to a different teaching hospital some 7 days later with a history of daily collapses associated with palpitations, tightness in the chest and throat, and acute shortness of breath. A ventilation perfusion scan suggested a pulmonary thromboembolus. The patient suffered a cardiac arrest with electromechanical dissociation whilst being prepared for an echocardiogram. Resuscitative measures were successful, and she was treated with anticoagulation therapy and thrombolysis and had an inferior vena caval filter inserted. An echocardiogram showed extensive thrombus within the inferior vena cava, right atrium and ventricle, and right ventricular outflow tract. A second cardiac arrest occurred from which she could not be resuscitated. The post-mortem examination revealed an encapsulated, cylindrical mass within the left common iliac vein extending through the inferior vena cava, right atrium, right ventricle and into the right pulmonary artery (Fig. 1, 2). No thrombus was seen within the deep calf veins. Within the uterus was a circumscribed mucoid tumour measuring 10 cm in maximum extent with a whorled
Fig. 1 Overall view of organ block showing intravenous leiomyomatosis within inferior vena cava (arrow).
Fig. 2
Intravenous leiomyomatosis within right pulmonary artery.
trabecular cut surface. No necrosis or haemorrhage was seen in the tumour. The mass within the iliac vein was tracked back to myometrial veins in the region of the uterine tumour. The rest of the autopsy was normal. The provisional autopsy differential diagnosis was of a uterine leiomyosarcoma with extension into the myometrial and pelvic veins, inferior vena cava, right heart and pulmonary artery, or uterine leiomyosarcoma, with associated thrombosis of myometrial veins extending to pelvic veins and right pulmonary artery. Histological examination of the intravascular mass and the uterine tumour showed fasicles of bland spindle cells with features of smooth muscle and no evidence of atypia, mitoses or necrosis (Fig. 3). The spindle cells of both the intravascular mass and the uterine tumour stained strongly and diffusely for desmin and actin (Fig. 4). The features were consistent with intravenous leiomyomatosis.3 True intravenous leiomyomatosis, by definition, is seen outside the borders of a leiomyoma. Vascular invasion within a leiomyoma (leiomyoma with vascular intrusion)
Fig. 3 Intravenous tumour composed of bland spindle cells with features of smooth muscle (H&E, original magnification, 6200).
CORRESPONDENCE
203
Contact Dr Michael Burke on E-mail: michaelb@vifp. monash.edu.au ACKNOWLEDGEMENT We thank the mortuary and histology staff, Kerry Johannes, Caroline Rosenberg and the State Coroner for making available his files.
Fig. 4 Intravenous tumour showing cells staining strongly and diffusely for desmin (Desmin immunoperoxidase stain, original magnification, 6200).
does not warrant the diagnosis of intravenous leiomyomatosis. This latter situation has not been found to be associated with the extension of tumour beyond the uterus or of other evidence of aggressive behaviour. Microscopically, the tumour may be found only within venous lumina, continuous with the venous wall, or be confined to the muscularis of the venous wall. In the first situation, it appears a uterine leiomyoma extends into uterine veins, as applied with our case. In the other situations, it is thought the tumour arises from venous smooth muscle.4 Histologically, the appearance of the intravascular tumour may resemble that of a leiomyoma, as applied with our case, but it may have a lobulated or clefted outline and be hyalinised and/or hydropic. Intravenous leiomyomatosis may demonstrate the same histological variations seen in leiomyomas, including cellular, bizarre, epithelioid, myxoid and angiomatoid variants. Clinically, intravenous leiomyomatosis may present as palpitations, syncope, exertional dyspnoea and very rarely as sudden death. Tumour extending to the tricuspid valve is particularly associated with a heightened risk of sudden death.5 Imaging techniques—including computed tomography, magnetic resonance imaging, echocardiography and angiography—are important for diagnosis, demonstrating the extent of the disease and planning surgical intervention.6 Cases of successful surgical resection with cardiopulmonary bypass have been described.7,8 In summary, intravenous leiomyomatosis is a rare tumour that may extend to the right side of the heart and be a significant cause of morbidity and even mortality. Our case adds to the literature on the exceedingly rare occurrence of death due to intravenous leiomyomatosis. It is only the 16th case of intracaval and intracardiac intravenous leiomyomatosis described. Michael Burke* Kenneth Opeskin { *Victorian Institute of Forensic Medicine, Department of Forensic Medicine, Monash University, Southbank, Victoria and {Department of Anatomical Pathology, St Vincent’s Hospital, Fitzroy, Victoria, Australia
1. Quade BJ, Dal Cin P, Neskey DM, et al. Intravenous leiomyomatosis: molecular and cytogenetic analysis of a case. Mod Pathol 2002; 15: 351±6. 2. Nam MS, Jeon MJ, Kim YT, Kim JW, Park KH, Hong YS. Pelvic leiomyomatosis with intracaval and intracardiac extension: a case report and review of the literature. Gynecol Oncol 1997; 64: 547±50. 3. Silverberg SG, Kurman RJ. Smooth muscle and other mesenchymal tumours. In: Rosai J, editor. Tumors of the Uterine Corpus and Gestational Trophoblastic Disease. 3rd ed. Washington: Armed Forces Institute of Pathology, 1992; 113±52. 4. Clement PB. Intravenous leiomyomatosis of the uterus. Pathol Ann 1998; 23: 153±83. 5. Roman DA, Mirchandani H. Intravenous leiomyoma with intracardiac extension causing sudden death. Arch Path Lab Med 1987; 111: 1176±8. 6. Stolf NA, dos Santos GG, Haddad VL. Unusual abdominal tumours with intracardiac extension. Two cases with successful surgical resection. Rev Hos Clin Fac Med Sao Paulo 1999; 54: 195±64. 7. Ricci MA, Cloutier LM, Mount S, et al. Intravenous leiomyomatosis with intracardiac extension. Cardiovasc Surg 1995; 3: 693±6. 8. Stolf NA, Santos GG, Hahhard VLS, et al. Successful one-stage resection of intravenous leiomyomatosis of the uterus with extension into the heart. Cardiovasc Surg 1999; 7: 661±4.