CHEST Guidelines-Patient-With-Slow-Growing-Mediastinal-Mass-Present

Patient-With-Slow-Growing-Mediastinal-Mass-Present

Pdf Summary

A 52-year-old woman presented with severe right upper abdominal and right lower chest pain, which worsened over five months. A smoker on levothyroxine for hypothyroidism, she had elevated right-side hemidiaphragm in past chest radiographs but no further workup. Physical examination showed reduced breath sounds and percussion dullness on the right side, but her vital signs were stable. Blood tests were normal. CT scans revealed a large mass in the right thorax with intralesional hemorrhage, coarse calcifications, and compression of the inferior vena cava, suggesting a liposarcoma. A clamshell thoracotomy removed a 22 cm, 1,510 g mass adherent only to the pericardium, which was also partially removed. The mass, confirmed as a liposarcoma, did not invade the lung or pleura.<br /><br />Histological analysis showed well-differentiated and myxoid liposarcoma features, with fat cells, lipoblasts, a myxoid matrix, and necrosis. Immunohistochemistry confirmed tumor positivity for S-100 and focal positivity for CD34, while fluorescein in situ hybridization (FISH) was negative for genetic rearrangements typically present in liposarcomas. <br /><br />Liposarcomas, rare in the mediastinum, primarily affect adults and are associated with compression symptoms like chest pain and dyspnea. Surgical resection is the treatment of choice, with a 66-85% five-year survival rate. Recurrence is common, and this case had features making it unique, like its origin in the pericardium and lack of genetic markers.<br /><br />Imaging shows fat-attendant mediastinal masses, with distinct features differentiating liposarcomas from similar tumors like lipomas. Histologically, liposarcomas are classified into atypical lipomatous tumors, myxoid, dedifferentiated, and pleomorphic variants, with varying implications for recurrence and metastasis. The patient had no tumor recurrence two years post-surgery, and no additional chemotherapy or radiation was employed.

Keywords

liposarcoma

thorax mass

clamshell thoracotomy

immunohistochemistry

mediastinal tumor

pericardium origin

myxoid liposarcoma

S-100 positivity

chest pain

surgical resection