Dataset Information

Biphasic synovial sarcomas of the liver: a case report and literature review.

ABSTRACT:

Background

Synovial sarcoma is a soft tissue sarcoma of temporarily unknown histologic origin with the ability for biphasic differentiation, occurring mostly in the vicinity of large joints of the extremities. Synovial sarcoma that originates in the liver is extremely rare. Only 7 cases have been reported in the domestic and international literature.

Case presentation

We report an 11-year-old female patient who underwent partial hepatectomy for a liver mass. Microscopically, she was diagnosed with hepatic biphasic synovial sarcoma. Cytogenetic examination revealed the fusion gene SS18-SSX1 (+), which confirmed the diagnosis.

Conclusion

Synovial sarcoma of the liver is a rare malignancy that is difficult to diagnose. Confirmation of diagnosis is based on histopathological assessment combined with immunohistochemical staining and, if necessary, cytogenetic aids.

SUBMITTER: Liang D

PROVIDER: S-EPMC9161578 | biostudies-literature | 2022 Jun

REPOSITORIES: biostudies-literature

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Publications

Biphasic synovial sarcomas of the liver: a case report and literature review.

Liang Defeng D Meng Lingyu L Wang Shanshan S Yi Dan D Liu Yahui Y

Diagnostic pathology 20220602 1

<h4>Background</h4>Synovial sarcoma is a soft tissue sarcoma of temporarily unknown histologic origin with the ability for biphasic differentiation, occurring mostly in the vicinity of large joints of the extremities. Synovial sarcoma that originates in the liver is extremely rare. Only 7 cases have been reported in the domestic and international literature.<h4>Case presentation</h4>We report an 11-year-old female patient who underwent partial hepatectomy for a liver mass. Microscopically, she w ...[more]

PMID: 35655305

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Project description:Tumor embolisms (TE) are an underappreciated source of pulmonary embolisms in sarcoma. Most evidence in the literature is limited to case reports and none have described the presence of TE secondary to myxofibrosarcoma. We report the first case of myxofibrosarcoma TE and perform a review of the literature for TE secondary to bone and soft tissue sarcomas (STS).A 36-year-old female presented with debilitating pain of the right upper extremity secondary to a recurrent soft tissue sarcoma. She had distant metastasis to the lung. An MRI revealed a 25-cm shoulder mass involving the proximal arm muscles with encasement of the axillary artery, vein, and brachial plexus. A palliative forequarter amputation was performed and tumor thrombus was evident within the axillary artery and vein. Postoperatively, she developed an acute onset of dyspnea and hypoxia. A computed tomography scan revealed a pulmonary saddle embolism. A bilateral lower extremity venous duplex was negative. She became hemodynamically unstable despite resuscitation and was placed on vasopressor support. A transthoracic echocardiogram revealed elevated pulmonary artery pressure, tricuspid regurgitation, right heart dilation, and reduced right heart systolic function consistent with acute cor pulmonale. The patient did not want to pursue a median sternotomy with pulmonary artery embolectomy and expired from cardiopulmonary arrest within 24 h of the operation. The final pathology revealed a 25?×?16?×?13 cm high-grade myxofibrosarcoma with invasion into the bone, skin, and neurovascular bundle as well as evidence of tumor thrombus.TE is a rare but deadly cause of pulmonary embolism in sarcoma. A high index of suspicion is necessary in individuals who present with respiratory-related symptoms, especially dyspnea. Diagnostic confirmation with a computed tomography scan of the chest and echocardiogram should be rapid. Unlike venous thromboembolism, pulmonary embolectomy remains the preferred therapeutic approach.

Dataset Information

Biphasic synovial sarcomas of the liver: a case report and literature review.

Background

Case presentation

Conclusion

Publications

Biphasic synovial sarcomas of the liver: a case report and literature review.

Similar Datasets

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