Enchondroma
Description Enchondroma (en-kon-DRO-ma) is one type of benign (non-cancerous) cartilage tumor that appears on the inside of the bone. These tumors usually begin and grow in childhood, then stop growing but remain present throughout adulthood. They are often found in patients between the ages of 10 and 20. Some cases become dormant or burned out. Incidence These tumors are very common and often occur in the small bones of the hand and feet. In fact, they are the most common tumor of the hand. They also occur in the long bones of the upper arm and thigh. In rare cases, multiple tumors can appear as part of a syndrome. These syndromes are Ollier's disease and Maffucci's syndrome. Single enchondromas can become cancerous, but this is very rare. The rate of change to a cancerous tumor is a little higher in Ollier's disease and Maffucci's syndrome. Being able to tell the difference between these benign tumors and very low-grade forms of cancerous tumors can be difficult, even for orthopaedic tumor surgeons. Diagnosis Most enchondromas are found when X-rays are taken for another reason, such as injury or arthritis. The diagnosis of an enchondroma is important to make sure it is not a more aggressive or cancerous tumor. The physician evaluates the history and physical exam along with images. It is important to know if the tumor is painful and the kind of pain it causes. There is greater concern if the pain occurs at rest or night pain that doesn't go away. Pain caused by activity is not as troublesome. Often pain from a different reason in a nearby joint may have prompted the X-ray. The next most important information is found on the X-rays. Enchondromas appear as small (smaller than 5 cm) lobe-shaped white tumors in the middle of the bone (Figure 1). The white areas of the tumor usually show a pattern of rings and arcs that indicates the tumor contains cartilage. Characteristics of a more aggressive tumor or malignant chondrosarcoma include: - Scalloping of the inner surface of the bone
- Thickening of the bone cortex
- Reactive bone growth on the outer surface of the bone
- Destruction of the bone by the tumor
- Soft tissue mass
- Large amounts of erosion of the bone
- Bone erosion that is growing
- Erosion surrounded by reactive bone
Although most enchondromas are diagnosed on plain X-rays (Figure 2), a computed tomography (CT) or magnetic resonance imaging (MRI) scan of the affected bone may sometimes be helpful. These scans give a more complete picture of the bone around the tumor (Figure 3). Bone scans can be helpful in some circumstances. However, both benign and malignant tumors can show increased uptake of the radioactive tracer used to detect increased bone activity (Figure 4). Under the microscope, enchondromas have islands of cartilage that are easy to tell apart from the normal bone that surrounds them. Usually, cartilage is not found in the center of bones. However, these tumors in the hand and foot or in Ollier's disease or Maffucci's syndrome may contain more odd-looking cartilage. They may be more difficult to tell apart from a mildly cancerous malignant tumor called low-grade chondrosarcoma. Low-grade chondrosarcomas (cancers) look more cellular under a microscope than enchondromas and there is less normal bone in the tumor. Because both these tumors look similar under the microscope, a group effort between experienced surgeons, radiologists and pathologists is important to get the best interpretation of the tumor. Risk Factors/Prevention The cause of enchondromas is not completely clear, but they don't seem to be caused by exposure to chemicals or radiation or patient activities. The chance that a single one of these tumors will become malignant is very rare. For the very few that become cancer, when they become malignant, they usually become a chondrosarcoma. There is a greater risk of a change to a malignant cancer in patients with Ollier's disease or Marfucci's syndrome. | Symptoms These tumors are usually painless. They are usually found during an X-ray evaluation of another problem in the same area. When these tumors appear in the hands or feet, or in multiple lesions, they can deform the bone. The symptoms for enchondromas of the hands and feet are enlarged fingers, pathologic fracture or deformities. On an X-ray, they appear as a dark hole in the bone, but usually they have calcification or white spots in the hole. The deformities in patients with Ollier's disease and Maffucci's syndrome can be quite severe. If pain from other nearby sources has been excluded, the tumor should be carefully studied to determine whether it could be a low-grade chondrosarcoma. Pain at night or at rest is more likely to mean a malignant tumor. But pain is also very common with some shoulder conditions like rotator cuff tendonitis. | 
| Treatment Options: Non-surgical Most enchondromas require no treatment at all. When needed, treatment for enchondroma can vary. This sometimes causes debate even among orthopaedic oncology surgeons due to the difficulty in determining the aggressiveness of the lesion. Stage 1 latent lesions that show no symptoms can be watched with regular X-rays to ensure the tumor is not growing. Most surgeons think that tumors without symptoms do not need to be removed. Treatment Options: Surgical When enchondromas are treated surgically, it is usually with scraping out and filling of the cavity with bone graft or other filling substances. Although they can come back (recur), most of them will not. Tumors that cause pathologic fractures are usually treated by allowing the fracture to heal. Then the tumor is scraped out to prevent another fracture. More aggressive tumors with bone destruction or with a mass growing outside the bone are usually chondrosarcomas. These tumors need to be removed. Malignant tumors are either scraped out or the entire bone around the lesion must be removed. This decision is made depending on the grade of the tumor. The grade of the tumor is determined by imaging studies and biopsy. Methods to treat tumors that look like simple enchondromas on X-ray studies, but are painful, can be controversial. Some surgeons consider this an in situ low-grade chondrosarcoma and recommend that the tumor should be scraped out. Surgeons call these tumors "grade ½" chondrosarcoma. Other surgeons think that these tumors are completely benign. These surgeons feel these tumors: - Are not likely to be the cause of pain in the area
- Can be observed with regular X-rays
- Do not require surgical removal
Unfortunately, a biopsy will not often help in these cases. Even specialized bone pathologists have trouble determining the difference between a benign enchondroma and a "grade ½" chondrosarcoma. Research on the Horizon/What's New? There is a lot of ongoing research on enchondromas and chondrosarcomas. Ongoing studies are trying to identify chemical markers that can tell doctors the difference between benign and malignant cartilaginous tumors. |