ARTICLE IN PRESS doi:10.1510/icvts.2010.240200
Interactive CardioVascular and Thoracic Surgery 11 (2010) 490–492 www.icvts.org
Case report - Cardiac general
Primary cardiac synovial sarcoma Muhammad Talukdera,*, Lyle Joycea, Randolph Marksb, Keith Kaplanc Division of Cardiovascular Surgery, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA b Department of Medical Oncology, Mayo Clinic, Rochester, MN 55905, USA c Department of Clinical Pathology, Mayo Clinic, Rochester, MN 55905, USA
a
Received 9 April 2010; received in revised form 17 June 2010; accepted 22 June 2010
Abstract Synovial sarcoma is a rare entity and accounts for -1% in all primary cardiac tumors. This is typically a highly aggressive tumor and survival is usually less than nine months in this location, even with surgery and adjuvant chemoradiation. Primary cardiac synovial sarcoma has rarely been reported in the literature. We report a recent case from the Mayo Clinic, Rochester, Minnesota. The patient is still alive and with some recurrence of tumor but without significant symptoms 22 months after primary surgery. 䊚 2010 Published by European Association for Cardio-Thoracic Surgery. All rights reserved. Keywords: Cardiac; Synovial sarcoma; Monophasic type
1. Introduction Primary cardiac synovial sarcoma is a rare malignancy, comprising approximately 5% of cardiac sarcomas and -1% of all primary cardiac tumors w1x. In comparison, metastatic involvement of the heart is over 20 times more common. About 75% of primary heart tumors are benign, and 75% of those are atrial myxomas, the remaining 25% of cardiac tumors are malignant, 75% of them being sarcoma w2x. Fewer than 20 cases have been reported in the medical literature so far w1x. Our case is a young woman who is still alive with some recurrence of tumor but without significant symptoms 22 months after primary surgery. 2. Case report A 38-year-old Caucasian female patient was evaluated for suspected cholecystitis in another hospital with progressive abdominal distension and pain for one month and shortness of breath for five days. Evaluation for cholecystitis was negative, but an echocardiogram showed a large pericardial mass. On admission to our hospital, we found she had ascites and hepatomegaly. A chest X-ray showed cardiomegaly and pleural effusion. Subsequent computed tomography (CT) and magnetic resonance imaging (MRI) scans revealed a large mass suggestive of tumor in the pericardial cavity (Fig. 1a). It was located in the left atrioventricular groove and appeared to exert some pressure effect on the left ventricular wall. The decision was made for resection of the mass for definitive diagnosis and restoration of hemodynamic stability. A conventional median sternotomy was performed to *Corresponding author. Tel.: q1 507-3964502; fax: q1 507-2557378. E-mail address:
[email protected] (M. Talukder). 䊚 2010 Published by European Association for Cardio-Thoracic Surgery
access the pericardial cavity. Approximately 300 ml of hemorrhagic fluid was drained and a large, highly vascular, lobulated mass was found occupying almost all the pericardial cavity with adhesions with left atrioventricular groove, left ventricle and pericardium (Fig. 1b). It was three to four times the size of heart itself. Approximately 98% of the tumor was removed without resorting to bypass (Fig. 1c). Frozen section confirmed sarcoma. Her chest was closed after thorough washing of the pericardial cavity. Histological features revealed a monophasic synovial sarcoma, with malignant spindle cells in loose fascicles (Fig. 2a). The tumor cells were diffusely and strongly positive for a cluster of differentiation antigen (CD99) (Fig. 2b) and B-cell CLLylymphoma 2 (BCL2) (Fig. 2c) and focally positive for epithelial membrane antigen (EMA) histochemical stain (Fig. 2d). The characteristic t(X; 18) (p11.2; q11.2) translocation was present. Her postoperative period was uneventful. She was discharged on postoperative day 6 with arrangement of further therapy under supervision of an oncologist. She underwent four cycles of chemotherapy with doxorubicin and ifosfamide followed by radiation therapy. She received 6650 cGy in 35 fractions to the tumor bed. She tolerated this treatment well. At one year postoperatively, an MRI showed recurrence of tumor although she was asymptomatic except for a few episodes of tachyarrhythmia. She received a course of chemotherapy with gemcitabine and docetaxel to shrink the mass and then underwent a second operation 15 months after the primary operation without any complication. She recovered well postoperatively and received a third cycle of chemotherapy using ifosfamide and etoposide. Repeat MRI three months following redo surgery showed a small residual tumor in the inferior wall adherent to the pericar-
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cents w3x. Its occurrence in the myocardium as a primary tumor is a rare entity. There is a male preponderance of 2.5:1. Most cardiac synovial sarcomas are located in the right side of the heart, but it can occur in the left heart and in the pericardium w4x. The synovial sarcoma in this case report was located in the pericardium in the left side with adhesion with left atrium, atrioventricular groove and left ventricular wall. However, the exact origin remained unclear. Synovial sarcoma displays two histological patterns, monophasic and biphasic. The biphasic tumor was composed of two types of cells, epithelial cells resembling those of carcinoma and fibrosarcoma like spindle cells, while monophasic shows only one type of cells pattern. The availability of a frozen section during surgery allows the diagnosis of sarcoma while for more specific diagnosis, immunohistochemical studies (i.e. EMA, CD99, BCL2) are required. The hallmark of diagnosis of synovial sarcomas is the detection of the translocated chromosome t(X; 18) (p11.2; q11.2), which is present in more than 90% of the synovial sarcomas w5x. Molecular confirmation of synovial sarcoma by cytogenetics or reverse transcriptase–polymerase chain reaction is a valuable adjunctive diagnostic method when it is suggested in an uncommon location, such as in the heart w6x. The patients with primary synovial sarcoma usually present with dyspnea, chest pain and features of congestive heart failure. These tumors proliferate rapidly and often cause death through widespread infiltration of the myocardium or obstruction of flow within the heart w7x. Complete resection is seldom possible and if so, frequently followed by recurrence within two years. Treatment is usually palliative resection followed by chemotherapy with or without radiation. Adjuvant chemoradiation is known to be of value to reduce recurrence but survival of more than 12 months is rare. A possible exception to this poor prognosis has been reported by Sassi et al. w8x. They described the case of a
Best Evidence Topic Nomenclature
3. Discussion Synovial sarcomas predominantly occur in para-articular soft tissues of the extremities of young adults and adoles-
Fig. 2. (a) Histological slide showing spindle malignant cells in loose fascicle (hematoxylin and eosin, 10=). (b) Immunostain strongly positive for CD99 antigen (20=). (c) Immunostain strongly positive for BCL2 antigen (20=). (d) Immunostain focally positive for EMA stain (20=). CD99, cluster of differentiation antigen; BCL2, B-cell CLL/lymphoma 2; EMA, epithelial membrane antigen.
Brief Case Report Communication
dium. To date she is alive without significant clinical issues 22 months after her primary surgery.
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Fig. 1. (a) MRI chest; tumor (T), pericardial effusion (E), heart (H). (b) Per operative picture; large tumor (T) in the pericardial cavity, heart (H). (c) Excised tumor; about 15 cm=10 cm size, highly vascular with fungating surface.
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45-year-old man with a pedunculated right atrial mass who had complete surgical resection. The tumor was a biphasic synovial sarcoma. No adjuvant chemotherapy was given and at five years the patient was reported to be alive w8x. There is a hope that in the future, there will be targeted therapies on the basis of cytogenetic analysis of the tumor cells to improve survival. References w1x Miller DV, Deb A, Edwards WD, Zehr KJ, Oliveira AM. Primary synovial sarcoma of the mitral valve. Cardiovasc Pathol 2005;14:331–333. w2x Vander Salm TJ. Unusual primary tumors of the heart. Semin Thorac Cardiovasc Surg 2000;12:89–100. w3x Weiss SW, Goldblum JR. Malignant soft tissue tumors of uncertain types. In: Enziger and Weiss’s soft tissue tumors, 4th edition. St. Louis, MO: Mosby, 2001:1483–1509. w4x de Zwaan C, Beckkers SCAM, van Garsse L, Jansen R, van Suylen RJ. Primary monophasic mediastinal, cardiac and pericardial synovial sarcoma: a young man in distress. Neth Heart J 2007;15:226–228. w5x Sand berg AA, Birdie JA. Updates on the cytogenetics and molecular genetics of bone and soft tissue tumors. Synovial sarcoma. Cancer Genet Cytogenet 2002;133:1–23. w6x Iyengar V, Lineberger AS, Kerman S, Burton NA. Synovial sarcoma of the heart-correlation with cytogenetic findings. Arch Pathol Lab Med 1995;119:1080–1082. w7x McGilbray TT, Schulz TK. Primary cardiac synovial sarcoma. Lancet Oncol 2003;4:283. w8x Sassi SH, Zargonoui N, Dakhlia MS, Mard K, Cammoun M, Romdhane BK. Primary synovial sarcoma of the heart. A clinicopathological study of one case and review of the literature. Pathologica 2004;96:29–34.
eComment: Re: Primary cardiac synovial sarcoma Author: Ji-Gang Wang, Department of Pathology, The Affiliated Hospital of Medical College, 266003 Qingdao, China doi:10.1510/icvts.2010.240200A We have read the report on synovial sarcoma located in the pericardium with adhesion to the neighboring structure w1x with great interest. The tumor mass was extremely large (15=10 cm in size). Immunohistochemistry and reverse transcriptase-polymerase chain reaction were carried out to detect immunophenotype and chromosome translocation. In this case, the spinal cells bear wide expression for CD99 and BCL-2, and focal expression for epithelial membrane antigen (EMA). The characteristic t(X;18) (p11.2; q11.2) translocation was present. Synovial sarcoma is a mesenchymal spindle cell tumor which displays variable epithelial differentiation including glandular formation and has a specific chromosomal translocation t(X;18) (p11;q11). Over 80% of the synovial sarcomas arise in deep soft tissue of the extremities, especially around the knee and the tumor frequently arises adjacent to joints or tendon sheaths. Around 5% occur in the head and neck region; however, any site can be affected w2x. Synovial sarcoma is biphasic or monophasic (the tumor of this case was monophasic and composed of spinal cells). In our practice, we usually use EMA along with cytokeratins (CK). CK is expressed in 90% of all synovial sarcoma. EMA is expressed more often and more widely than CK, which could outline glandular lumina. The synovial sarcomas often co-express both. However, some cases were positive for CK and negative for EMA, and vice versa. References w1x Talukder MQ, Joyce LD, Marks RS, Kaplan KJ. Primary cardiac synovial sarcoma. Interact CardioVasc Thorac Surg 2010;11:490–492. w2x Fletcher CDM, Unni KK, Mertens F, editors. World Health Organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone. Lyon: IARC Press, 2002:200–204.
