Gorham-Stout disease in the rib and thoracic spine with spinal injury treated with radiotherapy, zoledronic acid, vitamin D, and propranolol: A case report and literature review.

Abstract

Gorham-Stout disease (GSD) is a rare bone condition that is characterized by the spontaneous, idiopathic and progressive proliferation of blood or lymphatic vessels, which replace the bone and marrow space. The precise etiology and pathophysiology of GSD remain poorly understood. Therefore, there is no consensus on the treatment of this disease. In the current study, a rare case of GSD in the rib and thoracic spine with spinal injury that was treated with radiotherapy, zoledronic acid, vitamin D and propranolol, is reported. A 77-year-old man visited the hospital complaining of constipation for 10 days. Within a few days, the patient had recognized complete paralysis of the bilateral lower legs. Radiologically, large osteolytic lesions were confirmed in the 6, 7 and 8th right ribs, the 6 and 7th thoracic vertebrae and in the liver and spleen. The lesions were diagnosed as GSD based on clinical findings, imaging characteristics and needle biopsy results. The patient was treated with zoledronic acid, activated vitamin D, propranolol and radiotherapy to the thoracic vertebrae. However, approximately 5 months after the first treatment, the rapidly increasing hemorrhagic pleural effusion compressed the left lung and caused a mediastinal shift to the right thorax. Unfortunately, the patient succumbed to the disease 7 months after first admission. To the best of our knowledge, this is the first reported case of GSD in the rib and thoracic spine with spinal injury to be treated with radiotherapy, propranolol, vitamin D and zoledronic acid. Furthermore, there have been no previous reports of a mediastinal shift caused by intralesional hemorrhage in GSD. For future reference, it should be noted that such processes may occur in GSD lesions of the thoracic spine and/or thorax. The present case will therefore contribute to a deeper understanding of GSD, a rare clinical entity.