Successful Management of Gorham-Stout Disease in the Cervical Spine by Combined Conservative and Surgical Treatments: A Case Report

Abstract

Gorham-Stout disease (GSD) is a rare condition characterized by intraosseous proliferation of endotheliallined vessels and progressive osteolysis. The precise etiology and pathophysiology of the disease remain poorly understood. Current therapeutic options for GSD include chemotherapy, radiotherapy, and surgical resection, but the surgical treatment of GSD is difficult, especially in the spinal lesion. The indication of wide-margined resection was limited because of anatomical features. Herein, we report a case of GSD of the cervical spine in which the lesions were successfully stabilized with combined conservative and surgical treatments. A 15-year-old male patient was admitted because of severe neck pain. The patient presented no neurological deficiency. However, the radiological findings revealed osteolytic lesions on the laminae and vertebrae between C1 to C5. An open biopsy confirmed an irregular, thin-walled vessel formation in the bone trabeculae, which was diagnosed as GSD. Conservative treatment was initiated with chemotherapy and radiotherapy. After one and a half year, the osteolytic condition had regressed. Spinal fusion surgery was then performed from C2 to C5 to prevent for progression of the cervical kyphotic changes, and spinal fusion was confirmed 7 months after the surgery. The patient showed no recurrence of GSD in the 5-year follow-up period after surgery. We were able to provide successful treatment by giving priority to the combined conservative treatments. If a patient has no severe deformity or progressive neurologic deficits, it might be better to prioritize conservative treatments and to perform the surgery after the osteolytic changes have stopped.

Extensive osteolytic disease in the mandible of a pediatric patient

“…On his literature review, Patel 10 concludes that most patients have been treated with surgery and/ or radiation therapy 8,10,11 . Which corroborates with Tateda et al 12 , that combined conservative and surgical treatments for a 15-year-old patient with massive osteolytic lesions in cervical spine, from C1 to C5. Authors started treatment with chemotherapy using interferon α-2b (1,000,000-4,000,000 units/day) and pamidronate disodium (30 mg/week) for about one and a half years, until the osteolytic condition regressed, and then a 36 Gy radiotherapy was performed.…”

Usefulness of 99mTc-ASC lymphoscintigraphy and SPECT/CT in the evaluation of rare lymphatic disorders

“…The diagnosis of GSD is mainly based on the clinical information and the histopathologic and radiographic findings. [ 26 ] The commonly used radiological examinations for the evaluation of GSD are X-ray, ultrasound, CT, bone scan, and MRI. [ 5 , 14 , 27 – 29 ] As lymphoscintigraphy was found to be helpful for the diagnosis of lymphedema and chylous effusion, [ 22 , 23 ] and GSD is a lymphatic disorder, lymphoscintigraphy might play a role in the evaluation of GSD.…”

Surgical management of Gorham‑Stout syndrome involving the cervical spine with bilateral pleural effusion: A case report and literature review

“…Between 1997 and 2019, only 17 cases affecting the cervical spine (including the present case) have been reported in the English literature. Details of such cases are summarized in Table I (14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25). Surgical treatment was performed in 9 patients (52%), of whom 2 patients also received radiotherapy.…”