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Commentary: massive osteolysis or Gorham-Stout disease
Commentary: massive osteolysis or Gorham-Stout disease
In this issue of the Journal, three interesting case reports, one in the print version1 and two online,2,3 discuss a rare condition characterized by a massive and progressive loss of bone. Although rare, this disease is recognized by several names, including disappearing bone disease (phantom bone), Gorham syndrome, Gorham-Stout syndrome, Idiopathic massive osteolysis, massive Gorham osteolysis, massive osteolysis, Morbus Gorham-Stout disease, progressive massive osteolysis, vanishing bone disease, and essential osteolysis. This bone resorption abnormality can affect different sites of the skeleton and in some instances the head and neck region, as described in these 3 cases. Gorham-Stout disease (GSD) is characterized by massive bone loss, which can affect different skeletal sites, and excessive intraosseous proliferation of small blood or lymphatic vessels, resulting in resorption and destruction of bone. The case reports and literature review update the reader in the various aspects that involve this rare condition, including the fact that the condition is often diagnosed by exclusion and late. The etiology is unknown and the appropriate management of GSD is controversial. Because the process is not associated with any systemic abnormality, local curettage, resective surgery, radiation therapy, and reconstruction have been used to stop the disease process and to correct morbidity with variable success. Surgery followed by reconstruction was part of the management protocol used in 2 cases,1,2 whereas surgery alone was used in the third case.3 One of the patients, a 9-yearold girl, was initially treated with an intravenous bisphosphonate medication (pamidronate).1 During the period of 3.5 years, this patient received a total of 11 infusions of pamidronate, leading to recovery of the osteolytic process but creating a possible dilemma for the reconstruction phase because of the associated risk for the development of osteonecrosis. Bisphosphonates are effective in suppressing osteoclastic bone resorption and have been widely used in different areas of medicine. In many instances, such as in this case, the use is considered to be off-label, because there is no FDA approval for the use in the treatment of GSD. One of the patients reported2 was a 79-year-old woman with a history of osteoporosis and who was taking an oral bisphosphonate (alendronate) for several years when she was diagnosed with GSD. Thus, bisphosphonate use in this case was directed to the treatment of osteoporo242
sis and GSD developed in spite of bisphosphonate therapy. What is important for the reader to be aware of is that although GSD is a rare condition, a growing number of cases have been reported in recent literature, and selection of bisphosphonates can influence the clinical outcome.4-6 Cases can be confused with other osteolytic processes resulting from infection and tumors. The basic mechanism of GSD involves abnormal bone resorption. Therefore, it is likely that antiresorptive medications such as bisphosphonates may be part of the management protocol, because they can retard progress of the osteolytic activity. During reconstructive surgery the risk for bisphosphonate osteonecrosis must be considered, especially for patients who have been treated with intravenous bisphosphonates. The management of the 3 cases reported involved surgery and reconstruction and, so far, in the 2 cases with a history of bisphosphonate use, there is no evidence of osteonecrosis development. In the case involving the 9-year-old girl, the use of intravenous pamidronate infusions caused the destructive bone process to stop, resulting in healing and allowing future oral rehabilitation. However, the long-term use of oral alendronate in the 79-year-old woman to treat osteoporosis did not prevent the development of GSD. This suggests that high-potency bisphosphonates, such as pamidronate and intravenous zoledronic acid infusion should be the type of bisphosphonate used in the treatment of GSD. Finally, it is important to point out that because of the potent antiresorptive action, bisphosphonates are now being used as off-label indications in the treatment of several other pathologic bone processes, such as giant cell lesions, giant cell tumor of bone, osteogenesis imperfecta, fibrous dysplasia, Gaucher disease, and osteomyelitis, and as an adjunct in orthopedic implants.7 Furthermore, alveolar bone resorption is a major component of periodontal diseases. Therefore, potent antiresorptive agents such as the bisphosphonates could potentially be used to deter bone destruction in the periodontium. A recent comprehensive literature review evaluates this new potential use of bisphosphonates as an adjunct of periodontal therapy.8 Cesar A. Migliorati, DDS, MS, PhD Department of Biologic and Diagnostic Sciences
OOOOE Volume 109, Number 2
College of Dentistry, University of Tennessee Memphis, Tennessee doi:10.1016/j.tripleo.2009.09.030
REFERENCES 1. Tong ACK, Leung TM, Cheung PT. Management of massive osteolysis of the mandible: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;109:239-42. 2. Pedroletti F, Rangarajan S, McCain JP, Velez I. The conservative treatment of a pathologic fracture in a patient with Gorham-Stout disease. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;109:e49-52. 3. Gondivkar SM, Gadbail A. Gorham-Stout syndrome—a rare clinical entity and a review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;109:e41-8.
Commentary 243 4. Raghuveer HP, Jayalekshmy R. Gorham’s massive osteolysis of the mandible—a progressive radiographic presentation. Dentomaxillofac Radiol 2009;38:292-5. 5. Zacharia B, Chundarathil J, Meethal KC, Ramakrishnan V, Krishnankutty RM, Veluthedath R. Gorham’s disease of the fibula: a case report. J Foot Ankle Surg 2009;48:347-52. 6. Kose M, Pekcan S, Dogru D, Akyuz C, Ozcelik U, Ozsurekci Y. Gorham-Stout syndrome with chylothorax: successful remission by interferon alpha-2b. Pediatr Pulmonol 2009;44:613-5. 7. Landesberg R, Eisig S, Fennoy I, Siris E. Alternative indications of bisphosphonate therapy. J Oral Maxillofac Surg 2009;67(Suppl 1): 27-34. 8. Badran Z, Kraehenmann MA, Guicheaux J, Soveidan A. Bisphosphonates and periodontal treatment: a review. Oral Health Prev Dent 2009;7:3-12.
In this issue of the Journal, three interesting case reports, one in the print version1 and two online,2,3 discuss a rare condition characterized by a massive and progressive loss of bone. Although rare, this disease is recognized by several names, including disappearing bone disease (phantom bone), Gorham syndrome, Gorham-Stout syndrome, Idiopathic massive osteolysis, massive Gorham osteolysis, massive osteolysis, Morbus Gorham-Stout disease, progressive massive osteolysis, vanishing bone disease, and essential osteolysis. This bone resorption abnormality can affect different sites of the skeleton and in some instances the head and neck region, as described in these 3 cases. Gorham-Stout disease (GSD) is characterized by massive bone loss, which can affect different skeletal sites, and excessive intraosseous proliferation of small blood or lymphatic vessels, resulting in resorption and destruction of bone. The case reports and literature review update the reader in the various aspects that involve this rare condition, including the fact that the condition is often diagnosed by exclusion and late. The etiology is unknown and the appropriate management of GSD is controversial. Because the process is not associated with any systemic abnormality, local curettage, resective surgery, radiation therapy, and reconstruction have been used to stop the disease process and to correct morbidity with variable success. Surgery followed by reconstruction was part of the management protocol used in 2 cases,1,2 whereas surgery alone was used in the third case.3 One of the patients, a 9-yearold girl, was initially treated with an intravenous bisphosphonate medication (pamidronate).1 During the period of 3.5 years, this patient received a total of 11 infusions of pamidronate, leading to recovery of the osteolytic process but creating a possible dilemma for the reconstruction phase because of the associated risk for the development of osteonecrosis. Bisphosphonates are effective in suppressing osteoclastic bone resorption and have been widely used in different areas of medicine. In many instances, such as in this case, the use is considered to be off-label, because there is no FDA approval for the use in the treatment of GSD. One of the patients reported2 was a 79-year-old woman with a history of osteoporosis and who was taking an oral bisphosphonate (alendronate) for several years when she was diagnosed with GSD. Thus, bisphosphonate use in this case was directed to the treatment of osteoporo242
sis and GSD developed in spite of bisphosphonate therapy. What is important for the reader to be aware of is that although GSD is a rare condition, a growing number of cases have been reported in recent literature, and selection of bisphosphonates can influence the clinical outcome.4-6 Cases can be confused with other osteolytic processes resulting from infection and tumors. The basic mechanism of GSD involves abnormal bone resorption. Therefore, it is likely that antiresorptive medications such as bisphosphonates may be part of the management protocol, because they can retard progress of the osteolytic activity. During reconstructive surgery the risk for bisphosphonate osteonecrosis must be considered, especially for patients who have been treated with intravenous bisphosphonates. The management of the 3 cases reported involved surgery and reconstruction and, so far, in the 2 cases with a history of bisphosphonate use, there is no evidence of osteonecrosis development. In the case involving the 9-year-old girl, the use of intravenous pamidronate infusions caused the destructive bone process to stop, resulting in healing and allowing future oral rehabilitation. However, the long-term use of oral alendronate in the 79-year-old woman to treat osteoporosis did not prevent the development of GSD. This suggests that high-potency bisphosphonates, such as pamidronate and intravenous zoledronic acid infusion should be the type of bisphosphonate used in the treatment of GSD. Finally, it is important to point out that because of the potent antiresorptive action, bisphosphonates are now being used as off-label indications in the treatment of several other pathologic bone processes, such as giant cell lesions, giant cell tumor of bone, osteogenesis imperfecta, fibrous dysplasia, Gaucher disease, and osteomyelitis, and as an adjunct in orthopedic implants.7 Furthermore, alveolar bone resorption is a major component of periodontal diseases. Therefore, potent antiresorptive agents such as the bisphosphonates could potentially be used to deter bone destruction in the periodontium. A recent comprehensive literature review evaluates this new potential use of bisphosphonates as an adjunct of periodontal therapy.8 Cesar A. Migliorati, DDS, MS, PhD Department of Biologic and Diagnostic Sciences
OOOOE Volume 109, Number 2
College of Dentistry, University of Tennessee Memphis, Tennessee doi:10.1016/j.tripleo.2009.09.030
REFERENCES 1. Tong ACK, Leung TM, Cheung PT. Management of massive osteolysis of the mandible: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;109:239-42. 2. Pedroletti F, Rangarajan S, McCain JP, Velez I. The conservative treatment of a pathologic fracture in a patient with Gorham-Stout disease. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;109:e49-52. 3. Gondivkar SM, Gadbail A. Gorham-Stout syndrome—a rare clinical entity and a review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;109:e41-8.
Commentary 243 4. Raghuveer HP, Jayalekshmy R. Gorham’s massive osteolysis of the mandible—a progressive radiographic presentation. Dentomaxillofac Radiol 2009;38:292-5. 5. Zacharia B, Chundarathil J, Meethal KC, Ramakrishnan V, Krishnankutty RM, Veluthedath R. Gorham’s disease of the fibula: a case report. J Foot Ankle Surg 2009;48:347-52. 6. Kose M, Pekcan S, Dogru D, Akyuz C, Ozcelik U, Ozsurekci Y. Gorham-Stout syndrome with chylothorax: successful remission by interferon alpha-2b. Pediatr Pulmonol 2009;44:613-5. 7. Landesberg R, Eisig S, Fennoy I, Siris E. Alternative indications of bisphosphonate therapy. J Oral Maxillofac Surg 2009;67(Suppl 1): 27-34. 8. Badran Z, Kraehenmann MA, Guicheaux J, Soveidan A. Bisphosphonates and periodontal treatment: a review. Oral Health Prev Dent 2009;7:3-12.