27 March 2008 - Case #114
All cases are archived on our website. To view them sorted by case number, diagnosis or category, visit our main Case of the Month page. To subscribe or unsubscribe to Case of the Month or our other email lists, click here.
This case was contributed by Dr. Jerad Gardner, The Methodist Hospital, Houston, Texas (USA).
Case #114
Clinical history:
A 47 year old woman had a left ventricular mass, which was excised. There was no prior history of malignancy.
Gross image:
Microscopic images:
What is your diagnosis?
Diagnosis: Leiomyosarcoma, arising in left ventricle
Immunostains:
Discussion:
The diagnosis was confirmed by desmin and smooth muscle actin immunostains, which were positive.
Leiomyosarcomas account for 8% of cardiac sarcomas and often occur in patients in their 30s. They may arise from smooth muscle of pulmonary veins and arteries and spread into the heart, which can often be documented (Heart Lung Circ 2005;14:121). They are also associated with Epstein Barr virus infection in cardiac transplant recipients or AIDS patients (J Heart Lung Transplant 2007;26:944).
Grossly, as in this case, these tumors often resemble a myxoma (Can J Cardiol 2001;17:331). Most (75%) occur in the left atrium, but may appear to arise from the pulmonary vein. 30% are multiple. Microscopically, they resemble classic leiomyosarcoma with compact bundles or fascicles of spindled or epithelioid smooth muscle type cells, intracytoplasmic glycogen, blunted nuclei and moderate to marked pleomorphism. There is usually necrosis and mitotic activity. Occasional tumors are low grade with low mitotic activity. PAS highlights the intracytoplasmic glycogen and trichrome staining highlights the longitudinal fibers. Actin and desmin are usually positive.
The differential diagnosis includes fibrosarcoma, which has tapered not blunted nuclei, lacks intracytoplasmic glycogen and pleomorphism and does not stain for actin or desmin. One must also consider metastatic leiomyosarcoma, particularly from the uterus or even intravenous extension of tumor (Clin Transl Oncol 2006;8:375, Ann Thorac Surg 2007;84:644).
Treatment is excision or transplantation (J Heart Lung Transplant 1998;17:1133). Prognosis is usually poor, unless the tumor is low grade (Histopathology 1999;34:295).
All cases are archived on our website. To view them sorted by case number, diagnosis or category, visit our main Case of the Month page. To subscribe or unsubscribe to Case of the Month or our other email lists, click here.
This case was contributed by Dr. Jerad Gardner, The Methodist Hospital, Houston, Texas (USA).
Case #114
Clinical history:
A 47 year old woman had a left ventricular mass, which was excised. There was no prior history of malignancy.
Gross image:
Microscopic images:
What is your diagnosis?
Click here for diagnosis and discussion:
Diagnosis: Leiomyosarcoma, arising in left ventricle
Immunostains:
Desmin
Smooth muscle actin
Discussion:
The diagnosis was confirmed by desmin and smooth muscle actin immunostains, which were positive.
Leiomyosarcomas account for 8% of cardiac sarcomas and often occur in patients in their 30s. They may arise from smooth muscle of pulmonary veins and arteries and spread into the heart, which can often be documented (Heart Lung Circ 2005;14:121). They are also associated with Epstein Barr virus infection in cardiac transplant recipients or AIDS patients (J Heart Lung Transplant 2007;26:944).
Grossly, as in this case, these tumors often resemble a myxoma (Can J Cardiol 2001;17:331). Most (75%) occur in the left atrium, but may appear to arise from the pulmonary vein. 30% are multiple. Microscopically, they resemble classic leiomyosarcoma with compact bundles or fascicles of spindled or epithelioid smooth muscle type cells, intracytoplasmic glycogen, blunted nuclei and moderate to marked pleomorphism. There is usually necrosis and mitotic activity. Occasional tumors are low grade with low mitotic activity. PAS highlights the intracytoplasmic glycogen and trichrome staining highlights the longitudinal fibers. Actin and desmin are usually positive.
The differential diagnosis includes fibrosarcoma, which has tapered not blunted nuclei, lacks intracytoplasmic glycogen and pleomorphism and does not stain for actin or desmin. One must also consider metastatic leiomyosarcoma, particularly from the uterus or even intravenous extension of tumor (Clin Transl Oncol 2006;8:375, Ann Thorac Surg 2007;84:644).
Treatment is excision or transplantation (J Heart Lung Transplant 1998;17:1133). Prognosis is usually poor, unless the tumor is low grade (Histopathology 1999;34:295).
