Chronic recurrent Gorham-Stout syndrome with cutaneous involvement
Type IV osteolysis or Gorham-Stout syndrome is a rare condition characterized by recurrent vascular tumors that disrupt normal anatomical architecture. Gorham-Stout syndrome is most commonly associated with the skeletal system with resulting replacement of bone with scar tissue following tumor regre...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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SAGE Publishing
2010-09-01
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| Series: | Rare Tumors |
| Subjects: | |
| Online Access: | http://www.pagepress.org/journals/index.php/rt/article/view/1803 |
| _version_ | 1829483979396349952 |
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| author | Nahum Duker Rebecca Simstein Luther Brady Jared Johnstun |
| author_facet | Nahum Duker Rebecca Simstein Luther Brady Jared Johnstun |
| author_sort | Nahum Duker |
| collection | DOAJ |
| description | Type IV osteolysis or Gorham-Stout syndrome is a rare condition characterized by recurrent vascular tumors that disrupt normal anatomical architecture. Gorham-Stout syndrome is most commonly associated with the skeletal system with resulting replacement of bone with scar tissue following tumor regression. The loss of entire bones has given Gorham-Stout syndrome the moniker vanishing bone disease. Natural progression of Gorham-Stout syndrome is characterized by spontaneous disease resolution. However, rare variants of recurrent, progressive, and/or systemic disease have been reported. We present a patient with a history of recurrent Gorham-Stout disease refractory to all treatment options considered. In addition to skeletal disease, our patient had soft tissue and cutaneous involvement, thus reflecting the more aggressive disease variant. Previous surgical attempts to control disease had been ineffective and the patient was referred to us for radiation therapy. Treatment with external beam radiation therapy resulted in good local control and symptom palliation, but full disease resolution was never accomplished. In addition to presentation of this patient, a review of the literature on etiological hypotheses and past/future treatment options was conducted and is included. |
| first_indexed | 2024-12-14T22:18:28Z |
| format | Article |
| id | doaj.art-23180d4bb538409eb5e465e7b9443feb |
| institution | Directory Open Access Journal |
| issn | 2036-3605 2036-3613 |
| language | English |
| last_indexed | 2024-12-14T22:18:28Z |
| publishDate | 2010-09-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Rare Tumors |
| spelling | doaj.art-23180d4bb538409eb5e465e7b9443feb2022-12-21T22:45:34ZengSAGE PublishingRare Tumors2036-36052036-36132010-09-0123e40e4010.4081/rt.2010.e40Chronic recurrent Gorham-Stout syndrome with cutaneous involvementNahum DukerRebecca SimsteinLuther BradyJared JohnstunType IV osteolysis or Gorham-Stout syndrome is a rare condition characterized by recurrent vascular tumors that disrupt normal anatomical architecture. Gorham-Stout syndrome is most commonly associated with the skeletal system with resulting replacement of bone with scar tissue following tumor regression. The loss of entire bones has given Gorham-Stout syndrome the moniker vanishing bone disease. Natural progression of Gorham-Stout syndrome is characterized by spontaneous disease resolution. However, rare variants of recurrent, progressive, and/or systemic disease have been reported. We present a patient with a history of recurrent Gorham-Stout disease refractory to all treatment options considered. In addition to skeletal disease, our patient had soft tissue and cutaneous involvement, thus reflecting the more aggressive disease variant. Previous surgical attempts to control disease had been ineffective and the patient was referred to us for radiation therapy. Treatment with external beam radiation therapy resulted in good local control and symptom palliation, but full disease resolution was never accomplished. In addition to presentation of this patient, a review of the literature on etiological hypotheses and past/future treatment options was conducted and is included.http://www.pagepress.org/journals/index.php/rt/article/view/1803RadiationGorham Stoutvanishing bone diseasevascularhemangiomahemangiomatous |
| spellingShingle | Nahum Duker Rebecca Simstein Luther Brady Jared Johnstun Chronic recurrent Gorham-Stout syndrome with cutaneous involvement Rare Tumors Radiation Gorham Stout vanishing bone disease vascular hemangioma hemangiomatous |
| title | Chronic recurrent Gorham-Stout syndrome with cutaneous involvement |
| title_full | Chronic recurrent Gorham-Stout syndrome with cutaneous involvement |
| title_fullStr | Chronic recurrent Gorham-Stout syndrome with cutaneous involvement |
| title_full_unstemmed | Chronic recurrent Gorham-Stout syndrome with cutaneous involvement |
| title_short | Chronic recurrent Gorham-Stout syndrome with cutaneous involvement |
| title_sort | chronic recurrent gorham stout syndrome with cutaneous involvement |
| topic | Radiation Gorham Stout vanishing bone disease vascular hemangioma hemangiomatous |
| url | http://www.pagepress.org/journals/index.php/rt/article/view/1803 |
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