Lymphangiosarcoma of the Thorax and Thoracic Vertebrae in a 16-Year-Old Girl

Lymphangiosarcoma of the Thorax and Thoracic Vertebrae in a 16-Year-Old Girl Case ReportA 16-year-old girl with a 2-year history of moderate back pain presented to an outside hospital emergency room with acute onset of chest pain and shortness of breath.A chest x-ray showed a large left pleural effusion and pneumothorax.Pleural fluid analysis showed elevated red cells, white cells, and lactate dehydrogenase levels.Cultures were negative.Computed tomography and magnetic resonance imaging revealed bony destruction of several ribs and T4-T6 vertebral pedicles.A skeletal survey was negative.A bone scan showed minimal uptake in the ribs and affected thoracic vertebrae.The biopsy of a rib lesion was suggestive of Langerhans cell histiocytosis.The patient was referred to our institution for additional management.On arrival, she was in significant pain, with absent breath sounds in the left lower lobe and left chest tenderness on palpation.Videoassisted thoracoscopy showed a loculate diffusion (Fig 1A), yellowish nodules on the diaphragmatic surface (Fig 1B), and adhesions (Fig 1C) around the left lung.The patient underwent biopsy of a diaphragmatic nodule and chest tube placement.Review of the rib biopsy from the outside hospital showed soft tissue with papillary vascular structures lined by a single layer of cuboidal and hobnail epithelioid cells (Fig 2A).Initial cytology fluid from the outside hospital was not available for evaluation.A videoassisted thoracoscopy biopsy demonstrated similar findings, with the addition of atypical vessels lined by epithelioid endothelial cells insinuating between collagen and adipose tissue (Fig 2B).Mitosis was present.On immunohistochemistry, tumor cells were reactive to Prox-1 (lymphatic marker) strongly and diffusely (Fig 2C), podoplanin (D2-40) weakly and diffusely, and CD31 focally.Ki-67 immunostain demonstrated a proliferative index of 40% in the neoplastic lymphatic endothelial cells (Fig 2D).CD34, CD68, calretinin, and cytokeratin were all negative.High proliferative index and endothelial cell atypia including papillae, dissecting growth pattern, and immunostaining phenotype were consistent with a high-grade angiosarcoma (AS), lymphangiosarcoma (LAS).Initial management consisted of sorafenib at a dose of 400 mg once per day for a month, which was reduced to 200 mg per day secondary to toxicity, and rapamycin twice per day (0.7 to 3.2 mg titrated to blood levels) for 5 months.We chose sorafenib because it targets the vascular endothelial growth factor (VEGF) receptor blockade, which demonstrated some response in an adult AS study, 1 and sirolimus, which targets the mammalian target of rapamycin pathway 2 ; our aim was to use a multitargeted approach in inhibiting angiogenesis/lymphangiogenesis and promoting apoptosis.Chest radiation and pleurocentesis also helped decrease profuse chylous effusion and pain.The patient improved clinically and was discharged after a 4-month inpatient stay.