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Primary synovial sarcoma of the right heart involving the tricuspid valve in an elderly Chinese woman: a case report.

Huo Z, Lu H, Mao Q, Jin Z, Wu H, Feng X, Xiao Y, Wang Y, Guo L - Diagn Pathol (2015)

Bottom Line: The patient had no significant medical history, and no tumour was found at any other site.This is a highly intriguing rare case that may represent a diagnostic pitfall, particularly regarding frozen section.The patient will receive chemotherapy, and we will pursue follow-up.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No.1Shuaifuyuan, Wangfujing Street, Dongcheng District, Beijing, 100730, China. huozhen1976@126.com.

ABSTRACT
Described herein is a 51-year-old woman with abdominal discomfort who was found to have a pericardial effusion and a large mass in her right heart by computed tomography scan and who then underwent tumour resection surgery. The tumour was so extensive that it involved the right atrium, the right ventricle and the tricuspid valve, and encompassed the right coronary artery. The patient had no significant medical history, and no tumour was found at any other site. The morphology of the tumour mimicked carcinosarcoma, exhibiting mixed epithelioid and spindle elements and it was difficult to differentiate the diagnosis even by immunohistochemical stains. Then, the final diagnosis of primary biphasic synovial sarcoma of the heart was established based on the finding of SS18 rearrangement. This is a highly intriguing rare case that may represent a diagnostic pitfall, particularly regarding frozen section. The patient will receive chemotherapy, and we will pursue follow-up.

No MeSH data available.


Related in: MedlinePlus

a. Gross appearance of the resected tumour showing a main tumour mass and pieces of fragmented tissue, tumour surface with partial encapsulation, and visible cardiac papillary muscle (indicated by the arrow). b. The cut surface of the tumour is solid and tan-white in colour with a few haemorrhagic areas and the stem of the right coronary artery is encompassed by the tumour (indicated by the arrow)
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Fig2: a. Gross appearance of the resected tumour showing a main tumour mass and pieces of fragmented tissue, tumour surface with partial encapsulation, and visible cardiac papillary muscle (indicated by the arrow). b. The cut surface of the tumour is solid and tan-white in colour with a few haemorrhagic areas and the stem of the right coronary artery is encompassed by the tumour (indicated by the arrow)

Mentions: Gross examination showed the tumour to be generally grey-white tissue with a total size of 8x8x5 cm, with partial surface encapsulation (Fig. 2a); the cut surface was grey-pink, solid and firm with a few haemorrhagic areas (Fig. 2b). The size of the largest tissue fragment was 6.5x5.5x5 cm. Microscopically, the tumour displayed a mixture of spindle cell and epithelioid components in most areas, which mimicked carcinosarcoma (Fig. 3a) with a few slit-like structures (Fig. 3b) and necrosis. Only a small number of tumour areas were composed of pure spindle cell elements, which exhibited eosinophilic cytoplasm and round to oval nuclei with nuclear pleomorphism. Mitoses were readily observed (8-10/10 high-powered fields) (Fig. 3c). The epithelioid element was the main component in most mixed areas, comprising well-differentiated glandular structures with lighter cytoplasm and intraluminal eosinophilic material, and was observed to be blended with less voluminous spindled components (Fig. 3d). In very few areas close to the tumour border, there were solely obvious glandular structures mimicking adenocarcinoma (Fig. 3e). The right coronary artery was involved by the tumour and thrombus could be observed within the lumen (Fig. 3f). A small tumour embolus could be seen in the blood vessel. Immunohistochemical staining showed that the epithelioid components were positive for cytokeratin, CK19 (Fig. 4a) and S-100(focally). The spindle cell components were positive for calponin (Fig. 4b) and vimentin. Both components were positive for BCL-2 and CD99 (Fig. 4c). The Ki-67 index was 25 % in both components. Desmin, WT-1, calretinin and SMA were negative in both components. Details regarding the antibodies used are given in Table 1. Fluorescence in situ hybridization (FISH) showed the rearrangement of SS18 (Vysis SS18 Break Apart FISH Probe Kit, Abbott Molecular Inc., USA) in the tumour cells (Fig. 4d). Based on the above-mentioned findings, the final diagnosis was primary synovial sarcoma of the right heart involving the tricuspid valve. This study was approved by the Ethics Committee of the Peking Union Medical College Hospital, and informed consent was obtained from the patient.Fig. 2


Primary synovial sarcoma of the right heart involving the tricuspid valve in an elderly Chinese woman: a case report.

Huo Z, Lu H, Mao Q, Jin Z, Wu H, Feng X, Xiao Y, Wang Y, Guo L - Diagn Pathol (2015)

a. Gross appearance of the resected tumour showing a main tumour mass and pieces of fragmented tissue, tumour surface with partial encapsulation, and visible cardiac papillary muscle (indicated by the arrow). b. The cut surface of the tumour is solid and tan-white in colour with a few haemorrhagic areas and the stem of the right coronary artery is encompassed by the tumour (indicated by the arrow)
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4482032&req=5

Fig2: a. Gross appearance of the resected tumour showing a main tumour mass and pieces of fragmented tissue, tumour surface with partial encapsulation, and visible cardiac papillary muscle (indicated by the arrow). b. The cut surface of the tumour is solid and tan-white in colour with a few haemorrhagic areas and the stem of the right coronary artery is encompassed by the tumour (indicated by the arrow)
Mentions: Gross examination showed the tumour to be generally grey-white tissue with a total size of 8x8x5 cm, with partial surface encapsulation (Fig. 2a); the cut surface was grey-pink, solid and firm with a few haemorrhagic areas (Fig. 2b). The size of the largest tissue fragment was 6.5x5.5x5 cm. Microscopically, the tumour displayed a mixture of spindle cell and epithelioid components in most areas, which mimicked carcinosarcoma (Fig. 3a) with a few slit-like structures (Fig. 3b) and necrosis. Only a small number of tumour areas were composed of pure spindle cell elements, which exhibited eosinophilic cytoplasm and round to oval nuclei with nuclear pleomorphism. Mitoses were readily observed (8-10/10 high-powered fields) (Fig. 3c). The epithelioid element was the main component in most mixed areas, comprising well-differentiated glandular structures with lighter cytoplasm and intraluminal eosinophilic material, and was observed to be blended with less voluminous spindled components (Fig. 3d). In very few areas close to the tumour border, there were solely obvious glandular structures mimicking adenocarcinoma (Fig. 3e). The right coronary artery was involved by the tumour and thrombus could be observed within the lumen (Fig. 3f). A small tumour embolus could be seen in the blood vessel. Immunohistochemical staining showed that the epithelioid components were positive for cytokeratin, CK19 (Fig. 4a) and S-100(focally). The spindle cell components were positive for calponin (Fig. 4b) and vimentin. Both components were positive for BCL-2 and CD99 (Fig. 4c). The Ki-67 index was 25 % in both components. Desmin, WT-1, calretinin and SMA were negative in both components. Details regarding the antibodies used are given in Table 1. Fluorescence in situ hybridization (FISH) showed the rearrangement of SS18 (Vysis SS18 Break Apart FISH Probe Kit, Abbott Molecular Inc., USA) in the tumour cells (Fig. 4d). Based on the above-mentioned findings, the final diagnosis was primary synovial sarcoma of the right heart involving the tricuspid valve. This study was approved by the Ethics Committee of the Peking Union Medical College Hospital, and informed consent was obtained from the patient.Fig. 2

Bottom Line: The patient had no significant medical history, and no tumour was found at any other site.This is a highly intriguing rare case that may represent a diagnostic pitfall, particularly regarding frozen section.The patient will receive chemotherapy, and we will pursue follow-up.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No.1Shuaifuyuan, Wangfujing Street, Dongcheng District, Beijing, 100730, China. huozhen1976@126.com.

ABSTRACT
Described herein is a 51-year-old woman with abdominal discomfort who was found to have a pericardial effusion and a large mass in her right heart by computed tomography scan and who then underwent tumour resection surgery. The tumour was so extensive that it involved the right atrium, the right ventricle and the tricuspid valve, and encompassed the right coronary artery. The patient had no significant medical history, and no tumour was found at any other site. The morphology of the tumour mimicked carcinosarcoma, exhibiting mixed epithelioid and spindle elements and it was difficult to differentiate the diagnosis even by immunohistochemical stains. Then, the final diagnosis of primary biphasic synovial sarcoma of the heart was established based on the finding of SS18 rearrangement. This is a highly intriguing rare case that may represent a diagnostic pitfall, particularly regarding frozen section. The patient will receive chemotherapy, and we will pursue follow-up.

No MeSH data available.


Related in: MedlinePlus