Chest / Cardiac for Tuesday, June 2nd, 2026

History

A 20-year-old previously well male presenting to the ED with hemoptysis and shortness of breath progressively worsening over 1 week, fatigue, 25-30 pound weight loss over a 2 month period, and left lower extremity discomfort. On admission, the patient has leukocytosis and mild anemia

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Question

What is the most likely diagnosis?

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Correct answer

Metastatic angiosarcoma

Discussion

Pulmonary hemorrhage has a myriad of etiologies, including infectious, inflammatory, idiopathic, and neoplastic. In an otherwise healthy young adult presenting with hemoptysis and diffuse ground glass opacities, initial differentials include autoimmune or infectious processes, but the presence of a lytic bone lesion can pivot towards an oncologic process or infectious process.

Primary angiosarcoma of the bone (PAB) is a rare and aggressive malignant vascular tumor representing less than 1% of all primary skeletal malignancies. Most cases of PAB occur in adults older than 30 years, making this case in a previously healthy 20-year-old male an unusual presentation.

PAB lesions often present with pain. Radiographically, the lesions are osteolytic, often with destructive cortical permeation and invasion into adjacent tissue, and usually occur in the tubular long bones of the lower extremities, usually the femur. In one series, lesions were usually unifocal (71%), but multifocality in contiguous skeletal sites is not uncommon, especially in the lower extremity. In this patient, his left lower extremity pain radiologically correlated with an expansile lytic lesion in the medullary compartment of the distal left femoral diaphysis and ill-defined lucencies in the proximal medial tibial metaphysis on initial plain film radiographs. Magnetic resonance imaging later further characterized these lesions as a marrow infiltrative process in the left distal femur with soft tissue component and near complete involvement of the tibia.

This patient’s presentation to the emergency department with hemoptysis and 25-30 pound weight loss highlights the pulmonary involvement of his disease secondary to metastasis. Pulmonary metastases can present as multiple thin-walled cysts or solid nodules surrounded by ground glass opacities (termed the CT “halo sign”). The solid nodules surrounded by ground glass opacities correspond to perilesional alveolar hemorrhage, which is a direct result of the fragile, incompetent neovascular channels that are characteristic of this tumor type. On the other hand, the thin-walled cysts are at a high risk of spontaneous rupture, which can present clinically as pneumothorax or hemothorax.

The prognosis for metastatic PAB has been historically poor, with the 5-year survival rate in metastatic patients being 0-8%. Treatment options include surgical resection or amputation for localized disease and chemotherapy, radiotherapy, and, recently, immunotherapy.

Differential diagnosis

Lymphoma can present as either primarily in bone or secondary as part of widespread disease with nodal and extranodal organ involvement. In the setting of widespread disease, pulmonary involvement usually manifests as unifocal or multifocal nodules or consolidations, but not with a ground-glass halo, as seen in this patient. Osseous lymphoma can present as permeative lytic lesions with a large transition zone and soft tissue focus or as a sclerotic focus without soft tissue extension. Primary osseous lymphoma most frequently involves the metadiaphyseal equivalent in the femur, like in this patient, followed by the pelvis, spine, and humerus and can involve multiple bones, like in this patient, but, by definition, will not have extraosseous disease. Secondary osseous lymphoma is the more common form of osseous lymphoma and usually presents more frequently in the thoracolumbar spine, pelvis, and ribs than in the femur but also in a background of lymphadenopathy or other extranodal disease.

Systemic lupus erythematosus (SLE) can present with diffuse alveolar hemorrhage and constitutional symptoms or the reverse halo sign but not nodules within the ground glass densities. Acute SLE musculoskeletal diagnoses include entities such as avascular necrosis, while nonacute SLE musculoskeletal involvement includes nondeforming and nonerosive arthritis, deforming nonerosive arthropathy, and erosive arthropathy. Destructive and expansile osseous lesions are not characteristic of SLE.

Tuberculosis (TB) primarily involves the lung and can involve the musculoskeletal system, particularly in the spine and peripheral joints. Pulmonary TB typically presents as innumerable micronodules (miliary) or consolidation or cavitary lesion with necrotic mediastinal adenopathy (primary or latent), but tuberculomas rarely present as a nodule with ground glass halo. Musculoskeletal TB typically involves the spine (Pott’s disease) and less commonly large, weight-bearing joints, such as the knee, hip, and sacroiliac joints, usually in a monoarticular fashion. Radiographically, this disease can mimic idiopathic arthritis with joint space narrowing and erosions, but additional cross-sectional imaging can reveal abscesses. Though our patient had multifocal osseous involvement in the bones around the knee, the expansile lytic femoral shaft lesion and extensive femoral and tibial involvement with minimal joint distension and absent bone abscesses would not be typically seen in tuberculous

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References

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