Inflammatory calcified de‐differentiated liposarcoma of the anterior mediastinum

Abstract

Inflammatory calcified de-differentiated liposarcoma of the anterior mediastinumA 51-year-old female patient was assessed for progressive exertionrelated cough and dysphagia. Physical examination and laboratory tests were normal. A chest radiograph posterior-anterior (PA) and lateral demonstrated a multilobulated, densely calcified mass lesion located within the central left sided chest cavity ( Fig. 1).Contrast-enhanced computed tomography (CT) confirmed a solid calcified anterior-mid mediastinal tumour with sporadic soft tissue components and a diameter of 82 × 91 mm (Fig. 2). The lesion extended from the left sided pericardium into the left upper pulmonary lobe segment 3 without associated atelectasis. No lipomatous tumour matrix was found. Additional imaging studies including bone scan, cranial and abdominal CT showed no secondary lesions elsewhere. Due to diagnostic uncertainty, a percutaneous CTguided coaxial core biopsy was performed from a left parasternal intercostal approach, targeting the soft tissue component. Histopathologic examination revealed an inflammatory, calcified variant of a de-differentiated liposarcoma (Fig. 3a). On immunohistochemistry, tumour cells demonstrated amplification of MDM2 (murine double minute oncogene) by fluorescence in situ hybridization (Fig. 3b). Surgical R0 resection with adjacent pericardium and left upper lobectomy was performed, followed by adjuvant radiotherapy. Clinical recovery was uneventful. Follow-up imaging after 6 and 12 months showed no recurrence.Typically, liposarcomas are characterized by a lipomatous matrix, mixed with a soft tissue component of various degrees. Sporadically calcifications are seen in 32% of liposarcomas, representing heterologous osteosarcoma components. 1 Liposarcoma accounts for approximately 20% of all adult sarcomas. Primary mediastinal manifestations are extremely rare, comprising <1% of all mediastinal tumours. 2,3 Peak occurrence is between 50 and 65 years of age without gender preference. 4 According to the World Health Organization classification, liposarcomas are divided

Primary intrathoracic liposarcomas: A clinicopathologic and molecular study of 43 cases in one of the largest medical centers of China

“…In the study cohort, all tested DDL tumors (16/16, 100%) exhibited MDM2 amplification. However, only 11 of 52 (21.1%) historical cases were subjected to FISH analysis, and all of them displayed positive results (10,18,40,(42)(43)(44)(45)(46)(47). We endorsed ancillary tests for cases in rare locations, such as the mediastinum, lung and/or ambiguous morphology.…”