Complete resection of giant pericardial synovial sarcoma in a 7-year-old boy: a case report
Qingyun Liu1; Zhenhua Huang1; Sihai Gao1
1Divison of Cardiothoracic and Vascular Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, China.
Correspondence:
Sihai Gao, Divison of Cardiothoracic and Vascular Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, China.
Email: sihaigao73@163.com
Abstract
Background: Synovial sarcoma is a rare soft-tissue malignant tumor most commonly occurring in the extremities and head and neck region, and rarely occurring in the pericardium.
Case presentation: We report a 7-year-old boy was admitted to the hospital with recurrent fever and chest pain over the past four months. A cardiac magnetic resonance imaging (MRI) revealed a tumor beneath the heart in the pericardial, and we surgical resection it completely. The postoperative histopathological examination resulted in a diagnosis of monophasic spindle cell type synovial sarcoma. After two weeks of hospitalization, the patient was discharged. Three months after discharge, the positron emission tomography (PET/CT) scans did not show any signs of recurrence.
Conclusion: Pericardial synovial sarcoma is a rare disease that is detected early, and complete resection improves patient survival. We recommend CT be performed in patients with recurrent fever and sizeable pericardial effusion to rule out possible pericardial synovial sarcoma considering the echocardiography limitations.
Keywords
Pericardial; synovial sarcoma; heart neoplasms; case report
Background
Synovial sarcoma is a rare soft-tissue malignant tumor. Pericardial synovial sarcoma (PSS) is a sporadic disease with a dismal prognosis (1). Most patients present with dyspnea, cough, and chest pain caused by heart failure (2). According to JOSE Duran-Moreno’s analysis from 37 cases reported in English during the past 20 years, PSS was found to occur in the 13-67 age range (3). To our knowledge, our case is younger than previously reported. In this letter, we report a case of the enormous pericardial synovial sarcoma in a 7-year-old boy.
Case presentation
A 7-year-old boy was weighing 22 kilograms, with no meaningful medical history, presented with fever and chest pain four months ago. A small amount of pericardial effusion was found in the local hospital, and the symptoms were relieved after treatment. Two months later, however, these symptoms reappeared. They went to another hospital, where their symptoms were reduced after treatment. After echocardiography and Computed Tomography (CT) revealed a large mass in the pericardial cavity the patient was referred to our institution. The patient had no additional significant medical, surgical, or family history. An echocardiogram revealed a large pericardial mass with pericardial effusion and right heart enlargement. Fortunately, the ejection fraction was in the normal range of 69%. Doppler echocardiography revealed a huge irregular hypoechogenic area seen in the pericardial cavity, ranging from about 144 × 95mm, and irregular anechoic area could be seen(Figure 1.A). A cardiac Magnetic Resonance Imaging (MRI) demonstrated a 142×94×81-mm mass below the heart in the pericardial cavity and close to left and right ventricles and inferior wall of left and right atria (Figure 1.B), partial enclosure of inferior vena cava (IVC).
We perform surgical resection of the mass by median sternotomy. The histopathology showed a monophasic tumor of spindle cells (Figure 2). Immunohistochemistry: EMA (partial +), VIM (+), tle-1 (+), INI1 (+), PCK (-), CK8 / 18 (-), CD117 (c-kit9.7) (-), CD117 (positive control) (+), CD34 (vascular +), dog1 (-), SMA (-), DES (-), MyoD1 (-), myogenin (-), caldesmon (-), S-100 (-), Sox10 (-), SDHB (-), STAT6 (-), Ki-67 (hot spot Li~ 40%). Molecular testing: ss18 FISH (+); ETV6 (-). Two weeks from the resection, the patient was discharged and declined adjuvant therapy in our hospital. Three months later, PET/CT has not shown any evidence of recurrence or metastasis.
Discussion and conclusions
Primary cardiac and pericardial tumors are extremely rare entities at an incidence of 0.001% to 0.03% in autopsy series, and most of them are benign tumors (4, 5). From our center’s retrospective data, malignant tumors accounted for about 10% of cardiac and pericardial tumors in the past ten years. Primary pericardial tumors are even less common, accounting for only 6.7% to 12.8% of primary cardiac tumors (4, 6). The most frequent are synovial sarcoma (approximately 5% of cardiac sarcoma),angiosarcoma, and undifferentiated pleomorphic sarcoma (1). According to our center data of ten years, six cases were diagnosed as pericardial malignancies. Three were synovial sarcomas, including two males (7 years old and 31 years old) and one female (50 years old). It coincides with a previously reported trend that pericardial synovial sarcomas are more common in young men (3, 7).
Most of the patients were hospitalized because of dyspnea, cough, chest pain, edema of lower limbs, and other symptoms caused by heart failure caused by pericardial effusion or tumor oppressing the heart (8). Echocardiography and chest CT are often used in diagnosis; however, a cardiac MRI may help for the surgery approach (3). More than 90% of synovial sarcomas contain a chromosomal translocation in t(X;18)(p11.2;q11.2), and it would be helpful in diagnosis (9-11). Due to these diseases’ rare occurrence, this tumor’s treatment is unidentified, but most patients get surgical resection. Although pooled analysis performed that surgical treatment was no impact on overall survival,two—thirds of patients who do not get surgery died within the first six months. The researcher believed it was due to the frequent failure of complete resection, complete resection is recommended as it was the only independent prognostic factor associated with survival. (3) Postoperative adjuvant therapy is also controversial; consider incomplete resection and the risk of metastases, Ganesh Shanmugam recommended using adjuvant chemotherapy and radiotherapy (12). But radiotherapy is not the preferred method because of cardiac complications in a long time follow-up (13, 14). The prognosis is recognized to be miserable despite the treatment, and the 5-year survival rate of pericardial synovial sarcoma was 22.2% (15), the median survival was 27 months (3), maximum survival is 14 years (16).
For the safety of our patient, we used the following methods in the operation: 1. To control the operation process and avoid massive bleeding and heart damage, we used the segmented resection method; 2. Prepare extracorporeal circulation to deal with emergencies.
In our case, we noticed that the patient began to have symptoms four months before admission and had been examined in two other hospitals with echocardiography. Still, only pericardial effusion was found, but no tumor. The tumor wasn’t discovered until four months after the onset of the symptoms. In the literature, we found the same cases with pericardial effusion but no tumor on echocardiography. And like our case, they had a fever as the first symptoms (17, 18). We recommend CT be performed in patients with recurrent fever and sizeable pericardial effusion to rule out possible pericardial synovial sarcoma considering the echocardiography limitations.
List of abbreviations:
CT: Computed tomography
MRI: magnetic resonance imaging
PSS: Pericardial synovial sarcoma
PET/CT: positron emission tomography
Authors’ contributions
QL and ZH collected data; QL analysed the patient data and wrote the first draft of this manuscript. ZH and SG helped to draft the manuscript and revised it critically for important intellectual content. All authors read and approved the final manuscript.
References:
1. Burke A, Tavora F. The 2015 WHO Classification of Tumors of the Heart and Pericardium. J THORAC ONCOL. 2016;11(4):441-52.
2. Maleszewski JJ, Anavekar NS. Neoplastic Pericardial Disease. CARDIOL CLIN. [Journal Article; Review]. 2017 2017-11-01;35(4):589-600.
3. Duran-Moreno J, Kampoli K, Kapetanakis EI, Mademli M, Koufopoulos N, Foukas PG, Kostopanagiotou K, Tomos P, Koumarianou A. Pericardial Synovial Sarcoma: Case Report, Literature Review and Pooled Analysis. IN VIVO. 2019;33(5):1531-8.
4. Patel J, Sheppard MN. Pathological study of primary cardiac and pericardial tumours in a specialist UK Centre: surgical and autopsy series. CARDIOVASC PATHOL. [Journal Article; Research Support, Non-U.S. Gov’t]. 2010 2010-11-01;19(6):343-52.
5. Reynen K. Frequency of primary tumors of the heart. AM J CARDIOL. [Journal Article; Meta-Analysis]. 1996 1996-01-01;77(1):107.
6. Meng Q, Lai H, Lima J, Tong W, Qian Y, Lai S. Echocardiographic and pathologic characteristics of primary cardiac tumors: a study of 149 cases. INT J CARDIOL. [Journal Article]. 2002 2002-07-01;84(1):69-75.
7. Vega HB, Bangueses QR, Diaz MR, Lozano MI, Folgueras SM, Silva GJ. Primary Pericardial Synovial Sarcoma. A Clinical Challenge. Rev Esp Cardiol (Engl Ed). [Case Reports; Letter]. 2018 2018-08-01;71(8):673-4.
8. Avondo S, Andreis A, Casula M, Imazio M. Update on diagnosis and management of neoplastic pericardial disease. Expert Rev Cardiovasc Ther. [Journal Article]. 2020 2020-09-01;18(9):615-23.
9. Turc-Carel C, Dal Cin P, Limon J, Rao U, Li FP, Corson JM, Zimmerman R, Parry DM, Cowan JM, Sandberg AA. Involvement of chromosome X in primary cytogenetic change in human neoplasia: nonrandom translocation in synovial sarcoma. Proc Natl Acad Sci U S A. [Case Reports; Journal Article; Research Support, Non-U.S. Gov’t; Research Support, U.S. Gov’t, P.H.S.]. 1987 1987-04-01;84(7):1981-5.
10. Clark J, Rocques PJ, Crew AJ, Gill S, Shipley J, Chan AM, Gusterson BA, Cooper CS. Identification of novel genes, SYT and SSX, involved in the t(X;18)(p11.2;q11.2) translocation found in human synovial sarcoma. NAT GENET. [Journal Article; Research Support, Non-U.S. Gov’t]. 1994 1994-08-01;7(4):502-8.
11. Fligman I, Lonardo F, Jhanwar SC, Gerald WL, Woodruff J, Ladanyi M. Molecular diagnosis of synovial sarcoma and characterization of a variant SYT-SSX2 fusion transcript. AM J PATHOL. [Journal Article; Research Support, Non-U.S. Gov’t]. 1995 1995-12-01;147(6):1592-9.
12. Shanmugam G. Primary cardiac sarcoma. Eur J Cardiothorac Surg. [Journal Article; Review]. 2006 2006-06-01;29(6):925-32.
13. Al-Rajhi N, Husain S, Coupland R, McNamee C, Jha N. Primary pericardial synovial sarcoma: a case report and literature review. J SURG ONCOL. [Case Reports; Journal Article; Review]. 1999 1999-03-01;70(3):194-8.
14. Ozmen E, Kayadibi Y, Samanci C, Ustundag N, Ozdemir G, Adaletli I, Kurugoglu S. Primary pericardial synovial sarcoma in an adolescent patient: magnetic resonance and diffusion-weighted imaging features. Journal of pediatric hematology/oncology. 2015 2015-01-01;37(4):e230-3.
15. Yoshino M, Sekine Y, Koh E, Kume Y, Saito H, Kimura S, Hamada H, Wu D, Hiroshima K. Pericardial synovial sarcoma: a case report and review of the literature. SURG TODAY. 2014;44(11):2167-73.
16. Van der Mieren G, Willems S, Sciot R, Dumez H, Van Oosterom A, Flameng W, Herijgers P. Pericardial synovial sarcoma: 14-year survival with multimodality therapy. ANN THORAC SURG. [Case Reports; Journal Article]. 2004 2004-09-01;78(3):e41-2.
17. Akerstroem F, Santos B, Alguacil AM, Orradre JL, Lima P, Zapardiel S. Pericardial Synovial Sarcoma. THORAC CARDIOV SURG. 2011;59(3).
18. Youn HC, Lee Y, Kim S. Pericardial synovial sarcoma presenting with large recurrent pericardial effusion. J THORAC DIS. 2016;8(6).
Figure Legends