Enchondroma
| Classification according to ICD-10 | |
|---|---|
| D16.- | Benign neoplasm of bone and articular cartilage |
| Q78.4 | Enchondromatosis |
| ICD-10 online (WHO version 2019) | |
An enchondroma is a common benign tumor derived from cartilage tissue . It is usually found in the medullary canal of the long tubular bones between the ages of 20 and 40 . Multiple enchondromas can be found in various syndromes , with the risk of degeneration.
clinic
The lesions are usually painless and slowly growing. Usually these are incidental findings during an x-ray examination . Enchondromas are rarely noticed as a result of pathological fractures or pain. Then a malignancy exclusion is mandatory. In the case of multiple enchondromas, a syndrome must be ruled out, as syndromic enchondromas can degenerate (see syndromes).
localization
Enchondromas are predominantly found in the long tubular bones of the fingers (60%). More rarely, enchondromas are found in the area of the foot bones, the femur , the humerus and the pelvis .
The location influences the likelihood of malignant degeneration. Lesions close to the trunk show a higher probability of degeneration (see chondrosarcoma ). Especially pelvic enchondromas should therefore be removed very carefully.
pathology
Macroscopically, in most cases it is gray-blue tumor nodules, which are usually less than three centimeters in size and lobulated. These are usually located in the medullary canal of the long tubular bones. Histologically, enchondromas are mature cartilage tissue with a hyaline matrix typical of cartilage and histologically benign cartilage cells ( chondrocytes ). There is usually an enchondral ossification in the tumor margin . The tumor center can also calcify after necrosis . The surrounding bone tissue is usually displaced, but not destroyed.
From an aetiological point of view, it is now assumed that these are embryonic remains of the growth plate. Healings after fractures could speak against the tumorous nature of some enchondromas.
Diagnosis
The diagnosis of an enchondroma can usually be made relatively reliably using imaging methods. In the case of classic symptoms, the X-ray image is often sufficient for the primary diagnosis and for monitoring the progress. In unclear cases, the cross-sectional imaging methods of computed tomography and especially magnetic resonance tomography are of decisive help . In addition to the primary diagnosis, imaging methods are also used to detect possible malignant degeneration of an enchondroma into a chondrosarcoma at an early stage. This can be difficult at times. The most important clinical indication of degeneration is the occurrence of pain.
roentgen
The x-ray shows the classic enchondroma as an oval lesion, elongated to the axis of the bone, centrally in the medullary canal of long tubular bones. An eccentric position does not rule out an enchondroma. The center of the lesion is usually in the metaphysis or in the distal diaphysis of a long bone. A small or short bone can be completely filled. Typical for enchondromas is a thinning of the cortex of the bone from the inside, the so-called scalloping and, in the case of expansion beyond the volume of the bone, the formation of the cortex ( neocortex ). However, a periosteal reaction is practically never observed. The appearance of an enchondroma is also mainly characterized by the strongly varying extent of the calcifications of the primarily cartilaginous tumor matrix: these can be punctiform, ring-shaped, curved ( "rings and arcs" ) and flaky. The picture is often popcorn-like. The calcifications are more common and more pronounced in the long tubular bones, while the lesions in the smaller bones of the fingers and feet are less calcified. The X-ray image only records the calcifications and the scalloping, so that the true extent of an only slightly calcified enchondroma can be underestimated with this method. Disappearance of previously calcified areas within an enchondroma in the course must be interpreted as an indication of degeneration.
Computed tomography (CT)
The importance of CT mainly relates to the superimposition-free display so that even the finest matrix calcifications can be recognized. The structure of the cortex can also be better assessed.
Magnetic resonance imaging (MRI)
Since 95% of the enchondromas consist of hyaline cartilage matrix , the enchondroma is homogeneously bright in the MRI on T2-weighted sequences . This particularly clearly shows the typical pattern of many enchondromas ( "popcorn-like", "rings and arcs" ). In addition, MRI is the best method to precisely assess the extent of an enchondroma. This is critical to planning prior to surgical removal.
Scintigraphy
Scintigraphically, the lesions usually enrich, so differentiating them from malignancies can be difficult. However, the storage activity is lower than that of malignant bone processes. There is also no increased perfusion in 3-phase scintigraphy. The storage depends on the degree of ossification, so a comparison with the X-ray findings is necessary.
biopsy
The biopsy of the lesion is usually necessary when a malignant tumor cannot be excluded. If possible, the entire tumor should be excised, as malignant and well-differentiated chondrosarcomas can also contain areas that histologically resemble an enchondroma.
therapy
If the x-ray is typical and benign and there are no clinical symptoms, therapy is not absolutely necessary (en bloc excision or curettage ). If a malignant tumor cannot be excluded with certainty, the tumor should be removed or monitored closely. Symptomatic lesions are usually excised surgically. Enchondromas in the rare locations also require thorough clarification.
Syndromes
Syndromes with multiple enchondromas are called enchondromatoses .
Maffucci syndrome
In Maffucci's syndrome (syn. Dyschondroplasia haemangiomatosa ) there are multiple, asymmetrical enchondromas v. a. of the limbs in combination with multiple hemangiomas of the skin and internal organs. These enchondromes often degenerate. Patients are also prone to developing other malignant tumors (e.g. angiosarcoma ). It is a complex developmental disorder of the mesoderm .
Ollier syndrome
When Ollier syndrome (syn. Hemichondrodysplasia type Ollier , also Enchondromatosis Ollier ) it is a half-page Enchondromatosis multiple long bone. This leads to stunted growth and fractures. The cause is not clear.
Multiple enchondromas often tend (approx. 25–30%) to degenerate in old age (see chondrosarcoma ).
Differential diagnosis
According to radiological criteria - in older patients and metaphyseal involvement of long tubular bones - only chondrosarcoma is possible .
swell
- J. Freyschmit, H. Ostertag, G. Jundt: Bone tumors. Springer, 2003, ISBN 3-540-40364-7 .
- C. Adler: Bone Diseases. Springer, 1983, ISBN 3-540-62836-3 .
Individual evidence
- ^ Rüdiger Döhler : Multiple Chondromas - Healing after Pathological Fractures. In: Advances in the field of X-rays and nuclear medicine (RöFo). 141 (1984), pp. 716 f.
- ^ R. Döhler, G. Heinemann, W. Busanny-Caspari, MD Farrar: Chondrosarcoma of the first metatarsal - primary or secondary to enchondroma? In: Archives of Orthopedic and Traumatic Surgery . 1979, 95, pp. 221-225.
- ↑ Barbara I. Tshisuaka: Ollier, Louis Xavier Edouard Léopold. In: Werner E. Gerabek , Bernhard D. Haage, Gundolf Keil , Wolfgang Wegner (eds.): Enzyklopädie Medizingeschichte. De Gruyter, Berlin / New York 2005, ISBN 3-11-015714-4 , p. 1069.
Web links
- Diagnosis and therapy of enchondromas
- Guideline for Benign Bone Tumors ( Memento of September 29, 2007 in the Internet Archive )
