Gorham syndrome of the thorax and cervical spine: CT and MRI findings

Abstract

Gorham syndrome is a rare disorder that is characterized by local osseous invasion and surrounding soft tissues by an angiomatous mass, eventually causing lysis of the affected bone. To date, only four cases have reported the MR imaging appearance of this disease and the findings have been variable. We present a case involving the cervical and thoracic spine and part of the osseous hemithorax with attention to the MR findings.

Gorham Disease of the Spine: A Case Report and Treatment Strategies for This Enigmatic Bone Disease

“…Heffez et al (1983) suggested the following diagnostic criteria: 1) a positive biopsy for angiomatous tissue, 2) absence of cellular atypia, 3) minimal or no osteoblastic response and absence of dystrophic calcification, 4) evidence of local progressive osseous resorption, 5) non-expansile, non-ulcerative lesion, 6) absence of visceral involvement, 7) osteolytic radiographic pattern, and 8) negative hereditary metabolic, neoplastic, immunologic, or infectious etiology. Any bone can be affected (Chung et al 1997). However, only 28 cases of spinal involvement, to our knowledge, have been reported in English literature.…”

“…Only 28 cases, to our knowledge, have been reported in the English literature (Table 1) (Bickel and Broders 1947;Gorham and Stout 1955;Falkmer and Tilling 1956;Hambach et al 1958;Castleman and McNeely 1964;Ellis and Adams 1971;Thompson and Schurman 1974;Heyden et al 1977;Woodward et al 1981;Heffez et al 1983;Brown et al 1986;Bohlman et al 1986;Hejgaard and Olsen 1987;Joseph and Bartal 1987;Mitchell et al 1993;Drewry et al 1994;Halliday et al 1994;Stöve and Reichelt 1995;Aoki et al 1996;Livesley et al 1996;Chung et al 1997;Hagberg et al 1997;Mawk et al 1997;Benhalima et al 2001;Bode-Lesniewska et al 2002;Chong and Shell 2003;Ceroni et al 2004). Eighteen patients were males and 10 patients were females.…”

“…Some authors stated that radiotherapy could not cease the progression of osteolysis (Cannon 1986;Stöve and Reichelt 1995;Livesley et al 1996;Chung et al 1997;Bode-Lesniewska et al 2002), and others reported favorable outcomes of radiotherapy alone, or in combination with a brace or halo traction, which not only prevented further progression of the disease but also lead to new bone formation in involved vertebrae (Heyden et al 1977;Heffez et al 1983;Mawk et al 1997). In the present case, radiotherapy was not chosen because its effect was controversial and there were too many vertebrae involved.…”

“…However, only 28 cases of spinal involvement, to our knowledge, have been reported in English literature. The progressive osteolysis causes kyphosis, kyphoscoliosis, subluxation or even dislocation of the spine (Castleman and McNeely 1964;Ellis and Adams 1971;Heyden et al 1977;Woodward et al 1981;Bohlman et al 1986;Joseph and Bartal 1987;Drewry et al 1994;Aoki et al 1996;Livesley et al 1996;Chung et al 1997; Bode-Lensniewska et T1-weighted magnetic resonance (MR) images (T1WI) and heterogeneously high intensities on T2-weighted images (T2WI). The lesions were unevenly enhanced by gadolinium-diethylenetriamine-pentaacetic acid (Gd-DTPA) (Figs.…”

Gorham's Disease or Massive Osteolysis

“…Plain radiographs, 6,28,36,71,77,79,116,136,143 radioisotope bone scans, 50,79,81,111,116,143 computed tomography (CT), 71,76,77,79,87,106,143 and magnetic resonance imaging (MRI) 76,77,87,106,116,138,143 have all been used in such evaluations.…”