Skip to main content
SpringerLink
Log in

Gorham-Stout syndrome in mainland China: a case series of 67 patients and review of the literature

  • Published:
Journal of Zhejiang University SCIENCE B Aims and scope Submit manuscript

Abstract

Objective

Gorham-Stout syndrome (GSS) is a rare disorder of uncertain etiology and unpredictable prognosis. This study aims to present a comprehensive understanding of this rare entity.

Methods

A literature search in PubMed and three Chinese databases was performed to screen histologically proven GSS cases among Chinese residents in the mainland. We analyzed the patients’ clinical characteristics, the value of different treatment modalities and their influence on the clinical outcome.

Results

Sixty-seven cases were finally enrolled. There were 43 men (64.2%) and 24 women (35.8%). The mean age at diagnosis was 28 years (1.5–71 years). The most common clinical symptoms included pain (n=40, 59.7%), functional impairment (n=13, 19.4%), and swelling (n=12, 17.9%). The radiographic presentation of 37 cases (55.2%) was disappearance of a portion of the bone. The others presented as radiolucent foci in the intramedullary or subcortical regions. A total of 42 cases provided data on therapy, these included surgery (n=27, 40.3%), radiation therapy (n=6, 9.0%), surgery combined with radiation therapy (n=2, 3.0%), and medicine therapy (n=7, 10.4%). For 30 of these 42 cases, follow-up data were available: 21 cases had the disorder locally controlled and 9 had a symptom progression. Fortunately, the disease is not fatal in the majority of cases.

Conclusions

GSS has no specific symptoms and it should be taken into consideration when an unclear massive osteolysis occurs. The efficacies of different treatment modalities are still unpredictable and further research is required to assess the values of different treatments.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  • Aviv, R., McHugh, K., Hunt, J., 2001. Angiomatosis of bone and soft tissue: a spectrum of disease from diffuse lymphangiomatosis to vanishing bone disease in young patients. Clin. Radiol., 56(3):184–190. [doi:10.1053/crad. 2000.0606]

    Article  PubMed  CAS  Google Scholar 

  • Barleon, B., Sozzani, S., Zhou, D., Weich, H.A., Mantovani, A., Marme, D., 1996. Migration of human monocytes in response to vascular endothelial growth factor (VEGF) is mediated via the VEGF receptor flt-1. Blood, 87(8): 3336–3343.

    PubMed  CAS  Google Scholar 

  • Boyer, P., Bourgeois, P., Boyer, O., Catonné, Y., Saillant, G., 2005. Massive Gorham-Stout syndrome of the pelvis. Clin. Rheumatol., 24(5):551–555. [doi:10.1007/s10067-005-1088-7]

    Article  PubMed  Google Scholar 

  • Browne, J.A., Shives, T.C., Trousdale, R.T., 2011. Thirty-year follow-up of patient with Gorham disease (massive osteolysis) treated with hip arthroplasty. J. Arthropl., 26(2):339.e7–339.e10. [doi:10.1016/j.arth.2010.03.003]

    Article  Google Scholar 

  • Bruch-Gerharz, D., Gerharz, C.D., Stege, H., Krutmann, J., Pohl, M., Koester, R., Ruzicka, T., 2007. Cutaneous lymphatic malformations in disappearing bone (Gorham-Stout) disease: A novel clue to the pathogenesis of a rare syndrome. J. Am. Acad. Dermatol., 56(2): S21–S25. [doi:10.1016/j.jaad.2006.01.063]

    Article  PubMed  Google Scholar 

  • Choma, N.D., Biscotti, C.V., Bauer, T.W., Mehta, A.C., Licata, A.A., 1987. Benign diseases syndrome: a case report and review of the literature. Am. J. Med., 83(6):1151–1156.

    Article  PubMed  CAS  Google Scholar 

  • Collins, J., 2006. Case 92: Gorham syndrome1. Radiology, 238(3):1066. [doi:10.1148/radiol.2383032126]

    Article  PubMed  Google Scholar 

  • Deveci, M., İnan, N., Çorapçıoğlu, F., Ekingen, G., 2011. Gorham-Stout syndrome with chylothorax in a six-year-old boy. Ind. J. Pediatr., 78(6):737–739. [doi:10.1007/s12098-010-0328-2]

    Article  Google Scholar 

  • Devlin, R.D., Bone, H.G.3rd, Roodman, G.D., 1996. Interleukin-6: a potential mediator of the massive osteolysis in patients with Gorham-Stout disease. J. Clin. Endocrinol. Metabol., 81(5):1893–1897. [doi:10.1210/jc.81.5.1893]

    Article  CAS  Google Scholar 

  • Dunbar, S.F., Rosenberg, A., Mankin, H., Rosenthal, D., Suit, H.D., 1993. Gorham’s massive osteolysis: the role of radiation therapy and a review of the literature. Int. J. Rad. Oncol. Biol. Phys., 26(3):491–497. [doi:10.1016/0360-3016(93)90968-2]

    Article  CAS  Google Scholar 

  • Dupond, J.L., Bermont, L., Runge, M., de Billy, M., 2010. Plasma VEGF determination in disseminated lymphangiomatosis-Gorham-Stout syndrome: a marker of activity? A case report with a 5-year follow-up. Bone, 46(3): 873–876. [doi:10.1016/j.bone.2009.11.015]

    Article  PubMed  CAS  Google Scholar 

  • Escande, C., Schouman, T., Fran Oise, G., Haroche, J., Ménard, P., Piette, J.C., Bertrand, J.C., Ruhin-Poncet, B., 2008. Histological features and management of a mandibular Gorham disease: a case report and review of maxillofacial cases in the literature. Oral Surg. Oral Med. Oral Pathol. Oral Radiol. Endodontol., 106(3):e30–e37. [doi:10.1016/j.tripleo.2008.02.028]

    Article  Google Scholar 

  • Foult, H., Goupille, P., Aesch, B., Valat, J.P., Burdin, P., Jan, M., 1995. Massive osteolysis of the cervical spine: a case report. Spine, 20(14):1636.

    Article  PubMed  CAS  Google Scholar 

  • Fujiu, K., Kanno, R., Suzuki, H., Nakamura, N., Gotoh, M., 2002. Chyothorax associated with massive osteolysis (Gorham’s syndrome). Ann. Thoracic Surg., 73(6): 1956–1957. [doi:10.1016/S0003-4975(02)03413-6]

    Article  Google Scholar 

  • Gorham, L.W., Stout, A.P., 1955. Massive osteolysis (acute spontaneous absorption of bone, phantom bone, disappearing bone). Its relation to hemangiomatosis. J. Bone Joint Surg. Am., 37(5):985–1004.

    PubMed  Google Scholar 

  • Hagberg, H., Lamberg, K., Astrom, G., 1997. Alpha-2b interferon and oral clodronate for Gorham’s disease. Lancet, 350(9094):1822–1823. [doi:10.1016/S0140-6736(05) 63639-2]

    Article  PubMed  CAS  Google Scholar 

  • Hammer, F., Kenn, W., Wesselmann, U., Hofbauer, L.C., Delling, G., Allolio, B., Arlt, W., 2005. Gorham-Stout disease stabilization during bisphosphonate treatment. J. Bone Mineral Res., 20(2):350–353. [doi:10.1359/JBMR. 041113]

    Article  Google Scholar 

  • Heffez, L., Doku, H.C., Carter, B.L., Feeney, J.E., 1983. Perspectives on massive osteolysis: report of a case and review of the literature. Oral Surg. Oral Med. Oral Pathol., 55(4):331–343.

    Article  PubMed  CAS  Google Scholar 

  • Heyd, R., Micke, O., Surholt, C., Berger, B., Martini, C., Füller, J., Schimpke, T., Seegenschmiedt, M.H., 2011a. Radiation therapy for Gorham-Stout syndrome: results of a national patterns-of-care study and literature review. Int. J. Rad. Oncol. Biol. Phys., 81(3):e179–185. [doi:10.1016/j.ijrobp.2011.01.006]

    Article  Google Scholar 

  • Heyd, R., Rabeneck, D., Rnenburg, O., Tselis, N., Zamboglou, N., 2011b. Gorham-Stout syndrome of the pelvic girdle treated by radiation therapy. Strahlenther. Onkol., 187(2):140–143. [doi:10.1007/s00066-010-2174-6]

    Article  PubMed  Google Scholar 

  • Hirayama, T., Sabokbar, A., Itonaga, I., Watt-Smith, S., Athanasou, N.A., 2001. Cellular and humoral mechanisms of osteoclast formation and bone resorption in Gorham-Stout disease. J. Pathol., 195(5):624–630. [doi:10.1002/path.989]

    Article  PubMed  CAS  Google Scholar 

  • Jackson, J., 1838. A boneless arm. Boston Med. Surg. J. 18:368–369.

    Article  Google Scholar 

  • Johnstun, J., Brady, L., Simstein, R., Duker, N., 2010. Chronic recurrent Gorham-Stout syndrome with cutaneous involvement. Rare Tumors, 2(3):e40. [doi:10.4081/rt. 2010.e40]

    Article  PubMed  Google Scholar 

  • Kulenkampff, H.A., Richter, G.M., Hasse, W.E., Adler, C.P., 1990. Massive pelvic osteolysis in the Gorham-Stout syndrome. Int. Orthop., 14(4):361–366.

    Article  PubMed  CAS  Google Scholar 

  • Lehmann, G., Pfeil, A., Böttcher, J., Kaiser, W.A., Füller, J., Hein, G., Wolf, G., 2009. Benefit of a 17-year long-term bisphosphonate therapy in a patient with Gorham-Stout syndrome. Arch. Orthop. Trauma Surg., 129(7):967–972. [doi:10.1007/s00402-008-0742-3]

    Article  PubMed  Google Scholar 

  • Li, X., Liu, B., Xiao, J., Yuan, Y., Ma, J., Zhang, Y., 2011. Roles of VEGF-C and Smad4 in the lymphangiogenesis, lymphatic metastasis, and prognosis in colon cancer. J. Gastrointest. Surg., 15(11):1–10. [doi:10.1007/s11605-011-1627-2]

    Google Scholar 

  • McNeil, K.D., Fong, K., Walker, Q., Jessup, P., Zimmerman, P., 1996. Gorham’s syndrome: a usually fatal cause of pleural effusion treated successfully with radiotherapy. Thorax, 51(12):1275–1276.

    Article  PubMed  CAS  Google Scholar 

  • Möller, G., Priemel, M., Amling, M., Werner, M., Kuhlmey, A., Delling, G., 1999. The Gorham-Stout syndrome (Gorham’s massive osteolysis). A report of six cases with histopathological findings. J. Bone Joint Surg., 81(3): 501–506.

    Article  Google Scholar 

  • Ng, C.L., Sell, P., 2003. Gorham disease of the cervical spine—a case report and review of the literature. Spine, 28(18):E355–E358. [doi:10.1097/01.BRS.0000084557.38858.85]

    Article  Google Scholar 

  • Paley, M., Lloyd, C., Penfold, C., 2005. Total mandibular reconstruction for massive osteolysis of the mandible (Gorham-Stout syndrome). Br. J. Oral Maxillofac. Surg., 43(2):166–168. [doi:10.1016/j.bjoms.2004.09.008]

    Article  PubMed  CAS  Google Scholar 

  • Patel, D.V., 2005. Gorham’s disease or massive osteolysis. Clin. Med. Res., 3(2):65–74.

    Article  PubMed  Google Scholar 

  • Radhakrishnan, K., Rockson, S.G., 2008. Gorham’s disease: An osseous disease of lymphangiogenesis?. Ann. N. Y. Acad. Sci., 1131:203–205. [doi:10.1196/annals.1413.022]

    Article  PubMed  Google Scholar 

  • Rauh, G., Gross, M., 1997. Disappearing bone disease (Gorham-Stout disease): report of a case with a follow-up of 48 years. Eur. J. Med Res., 2(10):425–427.

    PubMed  CAS  Google Scholar 

  • Ricalde, P., Ord, R., Sun, C.C.J., 2003. Vanishing bone disease in a five year old: report of a case and review of the literature. Int. J. Oral Maxillofac. Surg., 32(2):222–226. [doi:10.1054/ijom.2002.0306]

    Article  PubMed  CAS  Google Scholar 

  • Spieth, M., Greenspan, A., Forrester, D.M., Ansari, A.N., Kimura, R.L., Gleason-Jordan, I., 1997. Gorham’s disease of the radius: radiographic, scintigraphic, and MRI findings with pathologic correlation. Skel. Radiol., 26(11): 659–663.

    Article  CAS  Google Scholar 

  • Stove, J., Reichelt, A., 1995. Massive osteolysis of the pelvis, femur and sacral bone with a Gorham-Stout syndrome. Arch. Orthop. Trauma. Surg., 114(4):207–210.

    Article  PubMed  CAS  Google Scholar 

  • Takahashi, A., Ogawa, C., Kanazawa, T., Watanabe, H., Suzuki, M., Suzuki, N., Tsuchida, Y., Morikawa, A., Kuwano, H., 2005. Remission induced by interferon α in a patient with massive osteolysis and extension of lymph-hemangiomatosis: a severe case of Gorham-Stout syndrome. J. Pediatr. Surg., 40(3):E47–E50. [doi:10.1016/j.jpedsurg.2004.11.015]

    Article  PubMed  Google Scholar 

  • Tie, M.L., Poland, G.A., Rosenow, E.C.3rd, 1994. Chylothorax in Gorham’s syndrome. A common complication of a rare disease. Chest, 105(1):208–213.

    CAS  Google Scholar 

  • Tong, A.C.K., Leung, T.M., Cheung, P.T., 2010. Management of massive osteolysis of the mandible: a case report. Oral Surg. Oral Med. Oral Pathol. Oral Radiol. Endodontol., 109(2):238–241. [doi:10.1016/j.tripleo.2009.08.044]

    Article  Google Scholar 

  • Vinée, P., Tanyü, M., Hauenstein, K., Sigmund, G., St Ver, B., Adler, C., 1994. CT and MRI of Gorham syndrome. J. Comput. Assist. Tomogr., 18(3):985–989.

    Article  PubMed  Google Scholar 

  • von Marschall, Z., Scholz, A., Cramer, T., Schäfer, G., Schirner, M., Öberg, K., Wiedenmann, B., Höcker, M., Rosewicz, S., 2003. Effects of interferon α on vascular endothelial growth factor gene transcription and tumor angiogenesis. J. Nat. Cancer Inst., 95(6):437–438.

    Article  Google Scholar 

  • Wang, H.C., Lin, G.T., 2004. Close-wedge osteotomy for bony locking stiffness of the elbow in Gorham disease patients: a case report. Kaohsiung J. Med. Sci., 20(5):250–255. [doi:10.1016/S1607-551X(09)70115-9]

    Article  PubMed  Google Scholar 

  • Woodward, H.R., Chan, D.P.K., Lee, J., 1981. Massive osteolysis of the cervical spine a case report of bone graft failure. Spine, 6(6):545–549.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Cardiovascular Key Lab of Zhejiang Province, Department of Cardiology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, 310009, China

    Po Hu, Xin-yang Hu & Jian-an Wang

  2. Department of Cardiology, Zhejiang Hospital, Hangzhou, 310013, China

    Po Hu & Fa-rong Shen

  3. Department of Internal Medicine, Hangzhou Binjiang Hospital/the Second Affiliated Hospital (Binjiang Branch), School of Medicine, Zhejiang University, Hangzhou, 310009, China

    Xiang-gui Yuan

Authors
  1. Po Hu
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Xiang-gui Yuan
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Xin-yang Hu
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Fa-rong Shen
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Jian-an Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Jian-an Wang.

Additional information

The two authors contributed equally to this work

Electronic supplementary material

Rights and permissions

Reprints and permissions

About this article

Cite this article

Hu, P., Yuan, Xg., Hu, Xy. et al. Gorham-Stout syndrome in mainland China: a case series of 67 patients and review of the literature. J. Zhejiang Univ. Sci. B 14, 729–735 (2013). https://doi.org/10.1631/jzus.B1200308

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1631/jzus.B1200308

Key words

CLC number

Search

Navigation