原文題目(出處)：
Vanishing bone disease: A review. J Oral Maxillofac Surg 2011;69 :199-203.
原文作者姓名：
D. N. Kiran, A. Anupama
通訊作者學校：
Department of Oral and Maxillofacial Surgery, College of Dental Sciences and Research, Maharishi Markandeshwar University, Mullana, Ambala, Haryana, India
報告者姓名(組別)： R2 葉宏偉
報告日期： 2011.1.25
內文：
Introduction
I. Vanishing bone disease, or Gorham disease, was first defined as a specific entity by Gorham and Stout in 1955; it is a rare disorder characterized by proliferation of vascular channels, which results in destruction and resorption of osseous matrix.
II. Only a few cases have been reported in the jaws. Vanishing bone disease or massive osteolysis of the lower jaw will initially affect the mandibular basal and alveolar bone, which subsequently involves the rami and the condyles.
III. The etiology remains unknown, the prognosis is unpredictable, and effective therapy has still not been determined.
IV. Numerous names have been used in the literature to describe this condition, such as phantom bone, disappearing or vanishing bone disease,acute spontaneous absorption of bone,hemangiomatosis, lymphangiomatosis, idiopathic osteolysis, and Gorham disease.
V. Idiopathic osteolysis is characterized by the spontaneous and progressive destruction of one or more of the skeletal bones.
VI. Idiopathic osteolysis was described first in 1838 and again in 1872 by Jackson, who reported a case of a “boneless arm.” Romer reported the first case in the jaws in 1924, in a 31-year-old woman.
VII. In 1954, Gorham reported on 2 patients with massive osteolysis of the bone. One was a boy, aged 16 years, with right clavicle and scapula involvement. Chylothorax eventually developed, and the patient died. The other patient was a man, aged 44 years, who also had involvement of the right clavicle and scapula.
VIII. Gorham disease is usually associated with angiomatosis of blood
vessels and sometimes of lymphatic vessels.
IX. Any bone can be affected, although there is a predilection for the pelvis, humerus, axial skeleton, and mandible.
Etiopathology
Wildly proliferating neovascular tissue causes massive bone loss, but non-neoplastic vascular tissue, similar to a hemangioma or lymphangioma.
In early stage, bone is replaced by an abundance of thin-walled capillary-sized vascular channels and, at a later stage, by fibrous connective tissue.
One main structural feature of the lesion is the presence of unusually wide capillary-like vessels, and therefore it is likely that the blood flow through these vessels is slow. It has been suggested that the slow circulation produces local hypoxia and lowering of the pH, favoring the activity of various hydrolytic enzymes.
Gorham and Stout reported that active hyperemia, changes in local pH, and mechanical forces promote bone resorption. They hypothesized that trauma may trigger the process by stimulating the production of vascular granulation tissue and that “osteoclastosis” is not necessary.
In contrast,Devlin have suggested that bone resorption in Gorham disease is due to enhanced osteoclast activity and IL-6 may play a role.
Moller reported 6 cases of Gorham-Stout syndrome with histopathologic findings and presented evidence that osteolysis is due to an increased number of stimulated osteoclasts.
Hirayama has also been suggested thyroid C cells and calcitonin may play an important role in the pathogenesis of Gorham disease
The disease can be monostotic or polyostotic, although multicentric involvement is exceptional.
No ethnic or gender predilection has been noted.
The disease appears to be nonhereditary and is most common in children and young adults.
In 30% of cases maxillofacial involvement is seen with pain, malocclusion, and deformity.
Clinical Features
Most cases occur in children or in adults aged less than 40 years.
However, the disease has been described in patients aged as young as 1 month to as old as 75 years.
Approximately 60% of all cases occur in men.
More than 200 cases have been reported in the literature. The process may affect the appendicular or axial skeleton. Cases have been reported in the skull (8 cases), maxillofacial region (42 cases), spine (18 cases), pelvis (18 cases), trunk (including clavicle and ribs) (35 cases), upper extremity (including scapula) (41 cases), and lower extremity (22 cases), in addition to multicentric involvement (11 cases).
The mandible was affected alone by the osteolysis, partially or completely, in 23 cases, whereas the maxilla was never involved alone.
Some patients present with a relatively abrupt onset of pain and swelling in the affected area, whereas others present with a history of insidious onset of pain in the involved jaw.
The medical, personal, and family histories are usually noncontributory.
Investigations
The standard laboratory blood tests are usually within normal limits and are not helpful. The serum alkaline phosphatase level may be slightly elevated.
Plain radiographs,radioisotope bone scans,CT,and MRI must been used.
There will be resorption and decreased vertical height in panoramic radiograph.
T1-weighted spin echo MRI shows uniformly low signal intensity in the involved bones, whereas an increased signal intensity generally is observed on T2-weighted spin echo images.
Differential Diagnosis
Differential Diagnosis is needed to rule out other common underlying causes of osteolysis, such as infection, cancer, and inflammatory or endocrine disorders.
D.D：(can be confirmed with biopsy)
Aneurysmal bone cyst
Extensive metastatic bone disease due to carcinomaof the breast
Osteosarcoma
Treatment
There is no standard therapy.
Non-OP ： radiation therapy, anti-osteoclastic medication (bisphosphonates), and interferon alfa-2b
OP：surgical resection and reconstruction by use of a bone graft or prosthesis.
The success rate after the use of a bone graft is low.(dissolution)
A moderate doses (40-45 Gy in 2-Gy fractions) appears to result in a good clinical outcome
In children and adolescents who receive high-dose RT,exists：
Potential for secondary malignancy
Growth restriction
The prognosis of Gorham disease is generally good unless vital structures are involved.
Discussion
The term used by Gorham and Stout was “haemangiomatosis,”implying:
This connective tissue contains many thin-walled vessels, sometimes with red blood cells. In other areas, freely
anastomosing vascular spaces lined by endothelial cells can be seen. The fatty marrow also contains some dilated blood vessels.
(1958)Johnson and McClure: Frequency as follows
clavicle, scapula, proximal end of humerus, ribs, iliac bone, ischium, and sacrum.
The disease has not been observed in the calvaria or in the distal bones of the extremities.
In recent years, most patients have been treated with surgery and/or radiation therapy
Heffez suggested 8 criteria for definitive diagnosis of massive osteolysis:
1. Positive biopsy findings in terms of angiomatous tissue presence 2. Absence of cellular atypia
3. Minimal or no osteoclastic response and absence of dystrophic calcifications
4. Evidence of local bone progressive resorption 5. Non-expansive, nonulcerative lesion
6. Absence of visceral involvement 7. Osteolytic radiographic pattern
8. Negative hereditary, metabolic, neoplastic, immunologic, and infectious etiology
題號 題目
1 Massive osteolysis is primarily related to a proliferation of blood or lymphatic vessels and has been termed _____ of bone (A) Resorption
(B) Destruction (C) Angiomatosis (D) Osteosclerosis 答案
(C)
出處： Oral and Maxillofacial Pathology , 3rd edition , p622
題號 題目
2 What kind of treatment is the most successful and widely accepted mode of therapy?
(A) Radiation therapy (B) Surgical resection (C) Chemotherapy
(D) No further treatment, but keep follow up closely 答案
(A)
出處：Oral and Maxillofacial Pathology , 3rd edition , p623
