Gorham-Stout Disease with Multiple Bone Involvement-Challenging Diagnosis of a Rare Disease and Literature Review.
Alina MomanuLavinia CabaNicoleta Carmen GorduzaOana Elena ArhireAlina Delia PopaVictor IanoleEusebiu Vlad GorduzaPublished in: Medicina (Kaunas, Lithuania) (2021)
Gorham-Stout disease is a rare disorder, which may result in a poor prognosis. This disease, a rare lymphangiomatosis, is defined by progressive bone disappearance due to massive unicentric and multicentric osteolysis. Osteolytic lesions of the spine and pleura effusion are poor prognostic factors. Herein, we will present a case where the onset of disease occurred at the age of 18 with asthenia, myalgia, and major bone pain, followed by incomplete motor deficiency in the lower limbs and, later, in the upper limbs. Imaging studies (CT scan and MRI) of the patient revealed osteolytic lesions (cervical and thoracic vertebrae, rib, and clavicle) and a pathological fracture of the C7 vertebra. Surgical procedures undertaken involved replacing the affected vertebrae with bone grafting and prosthesis. The investigations performed allowed for the exclusion of inflammation, thyroid or parathyroid disease, lymphoma, neoplasia, or autoimmune disorders. A bone marrow biopsy showed osteolysis, the replacement of bone tissues with connective tissue, and chronic non-specific inflammation. The evolution was negative with almost complete osteolysis of the left clavicle, the emergence of new osteolysis areas in the lumbar vertebrae, pelvic bones, and the bilateral proximal femur, splenic nodules, chylothorax, and associated major neurological deficits. Unfortunately, this negative evolution resulted in the patient's death a year after onset.
Keyphrases
- poor prognosis
- bone mineral density
- bone marrow
- case report
- oxidative stress
- computed tomography
- magnetic resonance imaging
- long non coding rna
- gene expression
- multiple sclerosis
- chronic pain
- soft tissue
- spinal cord
- contrast enhanced
- minimally invasive
- body composition
- bone regeneration
- bone loss
- mesenchymal stem cells
- diffuse large b cell lymphoma
- mass spectrometry
- high grade
- hip fracture
- finite element
- fluorescence imaging