Endocrine Abstracts

JOINT1527

Introduction: Cystic Angiomatosis (CA) is a rare condition with several dozen cases reported in the literature. It is characterized by multifocal skeletal and visceral angiomatous lesions. The aetiology of the condition remains unclear and the exact pathological background has not been determined so far, similarly as the diagnostic and therapeutic procedures.

Case report: We report the case of a 26-year-old male patient with a 5-year history of severe bone pain located in pelvis, spine, ribs and limping. CT scan showed osteolytic lesions with osteosclerotic rim in the capitula of humerus, pelvis, spine and several lesions in the right acetabulum. It also revealed gross splenomegaly with multiple heterogenous lesions and multiple hepatic lesions. Next, PET-CT was performed showing low FDG uptake in overmentioned lesions. It was followed by bone scintigraphy that showed hyperconcentration of the tracer in the same skeletal locations. Based on the obtained results, the main suspected diagnosis was malignant sarcoma with multiple bone metastases. The US-guided biopsy of the hepatic lesion was performed showing haemangioma. Next, the patient underwent splenectomy and histological examination revealed multiple small haemangiomas. Finally, the biopsy of the left ilium was performed that revealed spaces of bone tissue replaced with fibrous tissue with multiple, thin-walled blood vessels and the absence of myeloid neoplasia, lymphoma or carcinoma. The histological features were characteristic for systemic CA.

Conclusion: Whereas CA is a condition of mostly benign course, routinely performed radiological examination usually suggests more aggressive disorders, including malignant neoplasia, which should be excluded in the differential diagnosis. Therefore, the biopsy of the bone should be performed in the early stages of the diagnostic process to prevent patients from unnecessary stress. To our knowledge this is the first report on a patient with CA in Poland.