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Synovial Sarcoma of The Heart
CASE REPORT
Case Report
Synovial Sarcoma of The Heart Sylvio Carvalho Provenzano, MD, MSc ∗ , Richard Con, FRACP, Owen D Jones, FRACP and Peter W Grant, FRACS, MBBS Department of Cardiothoracic Surgery, Royal Perth Hospital, Wellington Street, Perth, WA 6000, Australia
We report a case of synovial sarcoma of the heart in a 14-year-old boy, who presented with signs of mild generalized illness and rapidly progressed to haemodynamic collapse. On operation the tumour was attached to the tricuspid valve, away from the atrioventricular node. He was considered to have incomplete resection due to extensive adhesions to the atrioventricular junction, and was offered chemotherapy. On 18 months follow-up, he was asymptomatic. (Heart, Lung and Circulation 2006;15:278–279) © 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved. Keywords. Cancer; Heart pathology; Sarcoma; Tricuspid valve
A
14-year-old boy, with no past history of disease, presented to a local hospital with a non-specific illness consisting of fever, vomiting, headache, tiredness, abdominal pain, and nocturnal dyspnoea progressing over two weeks. He was treated for gastritis, however tachycardia persisted, and a cardiac murmur was noted. His electrocardiogram (ECG) showed inverted T waves and a right bundle branch block. An echocardiogram (echo) revealed a fluid-filled mass arising from either the ventricular side of the tricuspid valve or the right ventricular wall, causing inflow obstruction. He was transferred to our hospital for further investigation. On arrival, he showed signs of cardiovascular collapse and was admitted to the ICU. Fluid resuscitation was undertaken with acceptable response, and surgery for mass removal was performed early next day. Blood cultures were taken. There was also evidence of disseminated intravascular coagulation with platelet count of 70.000 mm−3 and INR of 1.6. The patient was operated on using cardiopulmonary bypass (CPB) with cannulation of both venae cava and return to the ascending aorta and antegrade cardioplegia. On atriotomy, the mass was occupying most of the right atrium and obstructing the inflow to the right ventricle. It was heterogenous and had atrial and ventricular elements. It was attached to the tricuspid annulus at the level of the posterior leaflet, destroying it entirely. There was a short and 15 mm wide stalk located away from the atrioventricular (AV) node, which was partially calcified and deeply attached to the AV junction: for this reason it was not considered completely resectable. The atrial component was 80 mm × 65 mm cyst with a wall of 15 mm, filled
Received 13 December 2004; received in revised form 23 January 2006; accepted 27 January 2006; available online 4 April 2006 ∗
Corresponding author. Tel.: +61 411210658; fax: +61 293820493. E-mail address: [email protected] (S.C. Provenzano).
with approximately 70 mL of haemorrhagic fluid. The ventricular component was lobulated myxoid tissue measuring about 20 mm × 40 mm × 10 mm. The gap of posterior tricuspid leaflet was repaired with a gusset of autologous pericardium, and an annuloplasty was performed with a malleable Dacron band (Duran An CoreTM ring 620R, Medtronic Inc., Minneapolis, United States). The patient was weaned from CPB with minimal support and transferred to ICU. On the second postoperative day he presented transient junctional rhythm that recovered to sinus without intervention. He was discharged on warfarin on the seventh post-operative day. The histological features of this tumour were those of a biphasic synovial sarcoma. The mass was focally biphasic, with epithelial and sarcomatous elements. The latter varied in appearance: it was partially composed of small, fairly uniform spindle cells in a background myxoid stroma. Elsewhere it was more cellular with interlacing bundles of more elongated spindle cells with a focally collagenous stroma. In some areas the stroma was hyalinized. The epithelial component was composed of cuboidal to columnar cells arranged in cords, nests and forming glandular structures, with PAS-positive diastase-resistant mucinous material. The cystic component was free of malignant cells. A CT scan of the chest, abdomen and pelvis, and a gallium scan performed 10 days after surgery showed no metastatic disease. He was considered to have incomplete surgical resection, and this was confirmed on a postoperative MRI, which showed residual calcification of the tricuspid annulus. Chemotherapy was commenced on an Ewing’s tumour protocol derived from the Australian and New Zealand Children’s Cancer Group Ewing’s Tumour Study. After 12 months follow-up, he was asymptomatic and chemotherapy was discontinued. An echo showed a peak and mean gradient across the tricuspid valve of 10 and 4 mmHg, respectively. An MRI of the heart was performed
© 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved.
1443-9506/04/$30.00 doi:10.1016/j.hlc.2006.01.009
and showed no evidence of tumour. After 18 months follow-up, he is still asymptomatic, writing his Year 11 examination, and part of a dancing group.
Discussion An excellent review by Salm found that about 75% of primary cardiac tumours are benign (75% of these are atrial myxomas) and 25% malignant. These were sarcomas, including the rare synovial sarcoma.1 Primary synovial sarcoma of the heart (SSH) is mentioned in the literature mainly as case reports. It is an extremely rare tumour with very poor prognosis: in most cases patients succumbed within 12 months from diagnosis, irrespective of the therapy employed.2,3 The longest survival was reported at five years follow-up, but it is a fortunate exception. Attempt to classify SSH by its proximity to the AV node as a prognostic indicator by some authors seems at least arbitrary, and it lacks solid evidence due to the small number of cases. In two reports of SSH, when its stalk was close to the AV node, survival was 3 and 6 months.4,5 Nicholson et al.2 reported a case with similarities to the present: stalk on AV junction away from the AV node and no distant metastasis. These patients survived at least 10 months follow-up with surgery and chemotherapy. In our case, tumour resection was considered incomplete;
Provenzano et al. Synovial Sarcoma of The Heart
279
nevertheless, he is alive and well after 18 months followup. The anatomical location of SSH might not be as important as prognostic factor as the advent of new chemotherapy agents. In conclusion, the ability to identify the disease early in its clinical course before distant metastasis occurs and current chemotherapy agents must play an important role in the natural history of the SSH. Longer follow-up and alertness to diagnose this rare tumour are imperative to determine an optimal clinical management.
References 1. Salm VTJ. Unusual primary tumours of the heart. Semin Thorac Cardiovasc Surg 2000;12(2 (April)):89–100. 2. Nicholson AG, Rigby M, Lincoln C, Meller S, Fisher C. Synovial sarcoma of the heart. Histopathotology 1997;30:349– 52. 3. Hannachi Sassi S, Zargouni N, Saadi Dakhlia M, Mrad K, Cammoun M, Ben Romdhane K. Primary synovial sarcoma of the heart: a clinicopathologic study of one case and review of the literature. Pathologica 2004;96(1 (February)):29–34. 4. Stebenmann R, Jenni R, Makek M, Oelz O, Turtna M. Primary synovial sarcoma of the heart treated by heart transplantation. J Thoracic Cardiovasc Surg 1990;99:567–8. 5. Shefield EA, Corrin B, Addis BJ, Gelder C. Synovial sarcoma of the heart arising from a so-called mesothelioma of the atrioventricular node. Histopathology 1988;12:191–202.
CASE REPORT
Heart, Lung and Circulation 2006;15:278–279
Case Report
Synovial Sarcoma of The Heart Sylvio Carvalho Provenzano, MD, MSc ∗ , Richard Con, FRACP, Owen D Jones, FRACP and Peter W Grant, FRACS, MBBS Department of Cardiothoracic Surgery, Royal Perth Hospital, Wellington Street, Perth, WA 6000, Australia
We report a case of synovial sarcoma of the heart in a 14-year-old boy, who presented with signs of mild generalized illness and rapidly progressed to haemodynamic collapse. On operation the tumour was attached to the tricuspid valve, away from the atrioventricular node. He was considered to have incomplete resection due to extensive adhesions to the atrioventricular junction, and was offered chemotherapy. On 18 months follow-up, he was asymptomatic. (Heart, Lung and Circulation 2006;15:278–279) © 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved. Keywords. Cancer; Heart pathology; Sarcoma; Tricuspid valve
A
14-year-old boy, with no past history of disease, presented to a local hospital with a non-specific illness consisting of fever, vomiting, headache, tiredness, abdominal pain, and nocturnal dyspnoea progressing over two weeks. He was treated for gastritis, however tachycardia persisted, and a cardiac murmur was noted. His electrocardiogram (ECG) showed inverted T waves and a right bundle branch block. An echocardiogram (echo) revealed a fluid-filled mass arising from either the ventricular side of the tricuspid valve or the right ventricular wall, causing inflow obstruction. He was transferred to our hospital for further investigation. On arrival, he showed signs of cardiovascular collapse and was admitted to the ICU. Fluid resuscitation was undertaken with acceptable response, and surgery for mass removal was performed early next day. Blood cultures were taken. There was also evidence of disseminated intravascular coagulation with platelet count of 70.000 mm−3 and INR of 1.6. The patient was operated on using cardiopulmonary bypass (CPB) with cannulation of both venae cava and return to the ascending aorta and antegrade cardioplegia. On atriotomy, the mass was occupying most of the right atrium and obstructing the inflow to the right ventricle. It was heterogenous and had atrial and ventricular elements. It was attached to the tricuspid annulus at the level of the posterior leaflet, destroying it entirely. There was a short and 15 mm wide stalk located away from the atrioventricular (AV) node, which was partially calcified and deeply attached to the AV junction: for this reason it was not considered completely resectable. The atrial component was 80 mm × 65 mm cyst with a wall of 15 mm, filled
Received 13 December 2004; received in revised form 23 January 2006; accepted 27 January 2006; available online 4 April 2006 ∗
Corresponding author. Tel.: +61 411210658; fax: +61 293820493. E-mail address: [email protected] (S.C. Provenzano).
with approximately 70 mL of haemorrhagic fluid. The ventricular component was lobulated myxoid tissue measuring about 20 mm × 40 mm × 10 mm. The gap of posterior tricuspid leaflet was repaired with a gusset of autologous pericardium, and an annuloplasty was performed with a malleable Dacron band (Duran An CoreTM ring 620R, Medtronic Inc., Minneapolis, United States). The patient was weaned from CPB with minimal support and transferred to ICU. On the second postoperative day he presented transient junctional rhythm that recovered to sinus without intervention. He was discharged on warfarin on the seventh post-operative day. The histological features of this tumour were those of a biphasic synovial sarcoma. The mass was focally biphasic, with epithelial and sarcomatous elements. The latter varied in appearance: it was partially composed of small, fairly uniform spindle cells in a background myxoid stroma. Elsewhere it was more cellular with interlacing bundles of more elongated spindle cells with a focally collagenous stroma. In some areas the stroma was hyalinized. The epithelial component was composed of cuboidal to columnar cells arranged in cords, nests and forming glandular structures, with PAS-positive diastase-resistant mucinous material. The cystic component was free of malignant cells. A CT scan of the chest, abdomen and pelvis, and a gallium scan performed 10 days after surgery showed no metastatic disease. He was considered to have incomplete surgical resection, and this was confirmed on a postoperative MRI, which showed residual calcification of the tricuspid annulus. Chemotherapy was commenced on an Ewing’s tumour protocol derived from the Australian and New Zealand Children’s Cancer Group Ewing’s Tumour Study. After 12 months follow-up, he was asymptomatic and chemotherapy was discontinued. An echo showed a peak and mean gradient across the tricuspid valve of 10 and 4 mmHg, respectively. An MRI of the heart was performed
© 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved.
1443-9506/04/$30.00 doi:10.1016/j.hlc.2006.01.009
and showed no evidence of tumour. After 18 months follow-up, he is still asymptomatic, writing his Year 11 examination, and part of a dancing group.
Discussion An excellent review by Salm found that about 75% of primary cardiac tumours are benign (75% of these are atrial myxomas) and 25% malignant. These were sarcomas, including the rare synovial sarcoma.1 Primary synovial sarcoma of the heart (SSH) is mentioned in the literature mainly as case reports. It is an extremely rare tumour with very poor prognosis: in most cases patients succumbed within 12 months from diagnosis, irrespective of the therapy employed.2,3 The longest survival was reported at five years follow-up, but it is a fortunate exception. Attempt to classify SSH by its proximity to the AV node as a prognostic indicator by some authors seems at least arbitrary, and it lacks solid evidence due to the small number of cases. In two reports of SSH, when its stalk was close to the AV node, survival was 3 and 6 months.4,5 Nicholson et al.2 reported a case with similarities to the present: stalk on AV junction away from the AV node and no distant metastasis. These patients survived at least 10 months follow-up with surgery and chemotherapy. In our case, tumour resection was considered incomplete;
Provenzano et al. Synovial Sarcoma of The Heart
279
nevertheless, he is alive and well after 18 months followup. The anatomical location of SSH might not be as important as prognostic factor as the advent of new chemotherapy agents. In conclusion, the ability to identify the disease early in its clinical course before distant metastasis occurs and current chemotherapy agents must play an important role in the natural history of the SSH. Longer follow-up and alertness to diagnose this rare tumour are imperative to determine an optimal clinical management.
References 1. Salm VTJ. Unusual primary tumours of the heart. Semin Thorac Cardiovasc Surg 2000;12(2 (April)):89–100. 2. Nicholson AG, Rigby M, Lincoln C, Meller S, Fisher C. Synovial sarcoma of the heart. Histopathotology 1997;30:349– 52. 3. Hannachi Sassi S, Zargouni N, Saadi Dakhlia M, Mrad K, Cammoun M, Ben Romdhane K. Primary synovial sarcoma of the heart: a clinicopathologic study of one case and review of the literature. Pathologica 2004;96(1 (February)):29–34. 4. Stebenmann R, Jenni R, Makek M, Oelz O, Turtna M. Primary synovial sarcoma of the heart treated by heart transplantation. J Thoracic Cardiovasc Surg 1990;99:567–8. 5. Shefield EA, Corrin B, Addis BJ, Gelder C. Synovial sarcoma of the heart arising from a so-called mesothelioma of the atrioventricular node. Histopathology 1988;12:191–202.
CASE REPORT
Heart, Lung and Circulation 2006;15:278–279