Case report and review: Angiosarcoma with thrombocytopenia after total hip arthroplasty

Abstract

Total hip arthroplasty (THA) is a common treatment for osteoarthritis and is also performed for other conditions, such as secondary arthritis due to developmental dysplasia of the hip. Various THA types may be complicated by osteolysis and an inflammatory pseudotumor due to an adverse reaction to metal debris. Rarely, THA has been associated with malignant tumors, but their causality remains unclear. In this case report, we describe a female patient with developmental dysplasia of the hip. She had undergone left metal-on-polyethylene THA, acetabular revision of the THA, and left total knee arthroplasty. In addition, she had a history of dyslipidemia and telangiectasia of the eyes, anemia, hiatal hernia, and pleuritis. A THA-associated mass (suspected to be a pseudotumor) had been detected during a previous hospital admission due to pleuritis. She was hospitalized due to swelling in her left lower limb, fatigue, and bruises. A clinical examination revealed anemia, thrombocytopenia, and growth of the suspected pseudotumor. Within 6 weeks, she presented with bleeding of the oral mucosa, hemoptysis, melena, severe thrombocytopenia that did not respond to treatment, elevated D-dimer and C-reactive protein levels, severe pain, increased osteolysis, and fractures around the THA. Infection or malignancy was suspected, but two trocar biopsies suggested an inflammatory pseudotumor. Since her anemia and thrombocytopenia were considered to have been caused by an inflammatory process within the suspected pseudotumor, her suspected pseudotumor and all THA components were surgically removed. However, she developed severe alveolar hemorrhaging and hypoxia and died 2 weeks after her surgery. Histopathological analysis of her surgical and autopsy samples revealed highly malignant angiosarcoma. Although individual cases of malignancies associated with THA have been reported, the literature lacks a clear association between THA and increased cancer risk. Most pseudotumors are non-malignant. The patient's case presented in this report exemplifies the challenges to the differential diagnosis of a THA-associated pseudotumor and rare angiosarcoma. Atypically rapid tumor growth, severe osteolysis, and deterioration in the general wellbeing suggest a malignant disease.

Keywords: angiosarcoma; endoprosthesis; pseudotumor; thrombocytopenia; total hip arthroplasty.

Figure 1

A pelvic x-ray when the patient was 66 years old presents the dislocation of the right hip and the left total hip arthroplasty after acetabular revision. The acetabular cup is augmented with cement and titanium mesh.

Figure 2

(A) A chest CT scan when the patient was 71 years old showed pleural fluid, suggesting empyema. On the right side, the pleura is thickened at the mediastinum and the interlobular septa, suggesting malignancy (arrows). (B) At the same time, an abdominal CT scan showed a mass, which was presumed to be a total hip arthroplasty (THA)-associated pseudotumor (arrows). (C) When the patient was 74 years old, upon hospital admission, a body CT scan showed ground-glass opacity areas in the lungs (arrow) and (D) enlargement of the THA-associated pelvic mass (size: 6 cm × 7.5 cm × 9 cm; arrows). Metal artifact–reducing sequence magnetic resonance imaging (MARS-MRI) showed an enlarged THA-associated mass in multiple locations: (E) the iliopsoas region (7.1 cm × 6.6 cm × 8.9 cm, arrows), (F) posteromedial to the acetabulum (4.7 cm × 4.8 cm × 8.8 cm, arrows), and (G) near the trochanter major (4.1 cm × 2.1 cm × 6.4 cm, arrows). (H) A preoperative pelvic CT scan showed remarkable osteolysis of the pubic and acetabular regions (arrows).

Figure 3

(A) Hematoxylin–eosin staining (HE) of autopsy specimens from the THA-associated mass shows a focal, patchy, atypical endothelial cell proliferation. (B) The atypical endothelial cell proliferation of the THA-associated mass is highlighted by CD31-positive cells by immunohistochemistry (IHC). (C) HE staining of autopsy specimens from the lungs shows atypical endothelial cell proliferation. (D) The atypical endothelial cell proliferation of the lungs is shown as CD31-positive cells by IHC. (E) HE staining of autopsy specimens from bone marrow shows atypical endothelial cell proliferation. (F) The atypical endothelial cell proliferation of the bone marrow is shown as CD31-positive cells by IHC. These findings are diagnostic of angiosarcoma. Scale bar 0.5 mm.