Radiation-Induced Sarcoma on 18F-FDG PET/CT After Treatment of Gorham-Stout Disease of the Maxilla.

Gorham-Stout disease is a rare disorder characterized by proliferation of lymphatic and vascular channels within bone resulting in osteolysis. A 53-year-old man with Gorham-Stout disease involving the left maxilla underwent previous treatment including radiation therapy and intralesional chemotherapeutic injections. He later presented with anemia, facial pain, weight loss, and nasal cavity hemorrhage. CT imaging demonstrated a mass centered within the right maxillary sinus with locoregional involvement. PET/CT showed prominent FDG activity involving the mass centered in the right maxillary sinus with low-grade avidity involving the contralateral maxilla in regions of treated Gorham-Stout disease. Biopsy of the mass confirmed radiation-induced sarcoma.

Vanishing Mandible in a 7-year Old Child: Response to Radiation Therapy.

Massive osteolysis in a 7-year old child is a rare condition. The etiology of massive osteolysis is unknown and it results in the progressive destruction of bony structures. There is no standard therapy available in the literature. Conservative treatment is often used for its management. Radiotherapy is considered as an accepted form of treatment with greater chance of success when it is used in the early course of disease. There are few case reports in the literature in which radiotherapy has been used for the treatment. This article highlights the literature update on various treatment modalities and a case managed by radiation therapy.

Gorham disease: lymphangiomatosis with massive osteolysis.

Gorham disease (also referred to as vanishing bone disease or idiopathic massive osteolysis) is a rare skeletal condition that results in the localized replacement of bone with angiomas and fibrosis. The etiology and most effective treatment for this nonhereditary condition remain controversial in the medical literature. To our knowledge, we present the first case of post-traumatic Gorham disease that includes MR imaging (before and after radiation therapy), post-radiation CT with evidence of treatment effect (sclerosis), as well as asymptomatic bilateral renal lymphangiomata.

Gorham-Stout syndrome of the pelvic girdle treated by radiation therapy: a case report.

BACKGROUND: The Gorham-Stout syndrome (GSS) is a rare, benign idiopathic and progressive disorder causing massive osteolysis due to a vascular hyperproliferation replacing the bony structure. Clinical experience concerning the efficacy of radiation therapy (RT) is limited to about 50 of an overall 200 cases reported worldwide. CASE REPORT: A 24-year-old bedridden woman had histologically proven GSS with destruction of the anterior pelvic girdle and received RT for a total dose of 45.0 Gy applied in 5 weekly fractions of 1.8 Gy. In addition, the patient received intravenously 4 mg zoledronic acid once a month. One year after the combined treatment, complete pain relief occurred, and the patient was able to walk without the use of appliances. Imaging studies revealed no progression of the osteolysis but only minimal signs of remineralization. CONCLUSION: Combined treatment with RT and bisphosphonate administration can prevent the progression of osteolysis in GSS. Total doses of 40-45 Gy are recommended.

Radiation therapy for Gorham-Stout syndrome: results of a national patterns-of-care study and literature review.

PURPOSE: The German Cooperative Group on Radiotherapy for Benign Diseases conducted a national patterns-of-care study to investigate the value of radiation therapy (RT) in the management of Gorham-Stout syndrome. METHODS AND MATERIALS: In 2009 a structured questionnaire was circulated to 230 German RT institutions to assess information about the number of patients, the RT indication and technique, and the target volume definition, as well as accompanying treatments, outcome data, and early or late radiation toxicity. RESULTS: In November 2009 responses were available from 197 departments (85.6%): 29 university hospitals (14.7%), 89 community hospitals (45.2%), and 79 private RT offices (40.1%). Of these institutions, 8 (4.0%) had experience using RT, for a total of 10 cases in various anatomic sites. Four patients underwent irradiation postoperatively, and six patients received primary RT. The total doses applied after computed tomography-based treatment planning ranged from 30 to 45 Gy. After a median follow-up period of 42 months, local disease progression was avoided in 8 cases (80.0%). In 2 of these cases a progression occurred beyond the target volume. Acute and late toxicity was mild; in 4 patients RT was associated with Grade I side effects according to Radiation Therapy Oncology Group/European Organisation for Research and Treatment of Cancer criteria. The literature analysis of 38 previously published articles providing results after the use of RT in 44 patients showed stable or regressive disease in 77.3%. CONCLUSIONS: RT may prevent disease progression effectively in Gorham-Stout syndrome in 77% to 80% of cases. Total doses ranging from 30 to 45 Gy applied after computed tomography-based treatment planning are recommended.

Early surgical management of chylothorax complicated by Gorham's disease.

Gorham's disease is a rare disorder and its etiology remains unknown. It is characterized by the proliferation of vascular structures in bone, and results in progressive osteolysis. Chylothorax is a severe complication of Gorham's disease with a mortality of 50%. We report a 14-year-old boy with Gorham's disease complicated by chylothorax who underwent early surgical management followed by radiotherapy.

Conservative treatment of a pathologic fracture in a patient with Gorham-Stout disease.

Gorham-Stout disease, or massive osteolysis, is an extremely rare idiopathic condition characterized by spontaneous, localized relentless resorption of one or several contiguous bones. There is no known successful treatment. Autologous bone graft also resorbs. It is a condition with difficult diagnosis, treatment, and prognosis. We report an extreme case of massive osteolysis of the maxillofacial complex. Unique to this case is that the patient presented with a mandibular fracture, and was taking alendronate (Fosamax), one of the treatment options for patients with Gorham-Stout disease. We discuss our treatment and add to the growing list of patients who have presented with this devastating disease.

Gorham disease of the mandible: radiographic findings and radiotherapy response.

Gorham disease, a rare condition of unknown etiology, is characterized histologically by bone disintegration and endothelial proliferation. We describe serial imaging findings, the treatment course, and radiotherapy response in a patient with pathologically confirmed Gorham disease involving the right mandible. Progressive mandibular resorption was managed initially with multiple surgical resections and reconstruction, followed by external-beam radiotherapy, which was an effective treatment method in this case. The patient's reconstructed mandible is functional and he is asymptomatic. Our literature review provides further insights regarding the clinical, radiologic, and pathologic behavior of this entity and examines the available treatment strategies.

A case of Gorham's disease with chylothorax treated curatively with radiation therapy.

Gorham's disease, also known as vanishing bone disease or massive osteolysis, is a rare disorder of uncertain etiology. It is characterized by uncontrolled proliferation of vascular or lymphatic capillaries within bone, leading to resorption and replacement with angiomatous tissue. It can be complicated by chylous pericardial and pleural effusions, which can be life threatening. Patients are also at risk of mortality or serious morbidity due to bone destruction, especially when the disease involves the spine. We report the case of a 31-year-old female with Gorham's disease involving several bones along with chylous pericardial and pleural effusions. She was effectively treated with definitive radiation therapy.

[Gorham-Stout disease: report of a case affecting the right hand with a follow-up of 24 years]. / Das Gorham-Stout-Syndrom der Hand: Fallbericht eines progressiven Krankheitsverlaufes über 24 Jahre.

Gorham-Stout disease is a rare idiopathic syndrome with distinctive clinical, pathologic and radiologic features. It is a variant form of osseus angiomatosis associated with massive osteolysis of bone. Usually appearing after trauma, the disease is described to occur at any age. Especially in case of thoracic involvement (chylothorax), lethal outcomes are reported. In Medline, about 200 cases have been described. A patient with osteolysis of the right hand following contusion at the age of two years is reported. Despite radiotherapy and repeated bone grafting, the osteolysis progressed until today (24 years). The pathologic features and various treatment methods for hand involvement are discussed.

Radiation therapy in the treatment of Gorham disease.

An adolescent male with Noonan syndrome presented with a nonhealing fracture of the proximal right humerus. Over a 7-month period there was progressive loss of bone in this region, resulting in a flail arm at 9 months. Radiographic review was consistent with Gorham disease. In addition, there was significant bleeding in the soft tissues and pain. Radiation was delivered. Seven years passed, until the patient re-presented with right hemithorax near collapse secondary to chylothorax. A chest tube was placed with temporary relief, but significant effusion remained. Radiation was again administered, and by the end of therapy the chest tube was removed. The effusion has not recollected at last follow-up, which is now 6 months. The use of radiation in the treatment of Gorham disease has been demonstrated to have excellent palliative ability.

Chylothorax in Gorham's disease.

A 25-yr-old woman presented with a right pleural effusion. Destruction of 9th through 12th ribs, adjacent vertebral bodies, and transverse processes was noted on plain radiograph and a large low-attenuated, irregular shaped mass lesion with peripheral rim enhancement, destroying vertebral body and transverse process, was revealed on the computed tomographic scan. Magnetic resonance imaging showed high signal on T1- weighted image and iso- and low signal on T2-weighted image for the mass lesion replacing the vertebral bony cortex and marrow space. An open rib biopsy revealed the histopathological changes of Gorham's disease (essential osteolysis), even though only bloody fluid filling the empty space and rib and vertebral transverse process destruction were grossly observed on operation. Even though there was no definite response to radiotherapy and pleurodesis, the patient showed stable condition up to 20 months after diagnosis.

Massive osteolysis of the maxillo-facial bones: case report and review of the literature.

An unusual case of massive osteolysis destroying the left side of the mandible, the maxilla, the orbit, the cranium bones and the upper cervical spine is reported. The evolution of this disease was observed over a period of 4 years. The literature was reviewed, only 39 cases have been found involving the maxillo-facial bones. In our case, attempts at surgery was without success. However, further radiotherapy with 35 Gy controlled the progression of this osteolysis.

[Massive lytic osteodystrophy or Gorham-Stout disease of the craniomaxillofacial area]. / L'ostéodystrophie lytique massive ou maladie de Gorham-Stout à localisation craniomaxillofaciale.

Gorham's disease also called idiopathic massive osteolysis represents a rare affection with unknown aetiology. This disease was described in different bones of the body but its location on maxillo-facial skeleton is particular by the number of cases in the world (only 32), by morphological, functional consequences and the prognosis. The authors describe a new case of male teenager (17 years old) who had a massive osteolysis of mandibular ramus, upper maxillary, malaire, sphenoid, temporal and occipital left bone. The authors give precision about epidemiological data, methods of diagnosis and therapeutic procedures.