Cartilagenous Tumors of Bone

D r. A t h m a r a m. M,
athmaramadisesh@yahoo.com Government Medical College , Anantapur.

There is confusion among many about cartilaginous tumors and cartilage forming tumors. When I was requested to deliver this lecture, I came to understand from the postgraduates that they wanted me to cover all the cartilage forming tumors rather than the cartilaginous tumors of bone. Broadly the cartilage forming tumors can be classified as follows: • Benign: o Solitary Osteocartilaginous Exostosis or Solitary Osteochondroma o Multiple Exostosis or Dyaphyseal Aclasis o Solitary Enchondroma o Juxtacortical (periosteal) Chondroma (Enchondroma) o Multiple enchondromatosis (Ollier’s Disease) o Chondroblastoma o Chondromyxoid Fibroma • Malignant: o Chondrosarcoma  Spindle celled  Myxoid  Mesenchymal I intend to confine my lecture on the main points rather than routine statistics of incidence, age, region and common mode of treatment. Osteochondroma: Def: An osteochondroma is a cartilage capped bony protrusion on external surface of a bone. First described by Sir Astely Cooper,(surgeon and anatomist at St. Thomas Guy’s Hospital, London in 1818) is the commonest benign bone tumor. This is usually a painless swelling. Pain can be caused by various factors as follows; • Irritation and pressure on surrounding structures • Osteonecrosis of the osseous component (Infracted Osteochondroma) • Perilesional bursal inflammation and swelling • Pseudoaneurysms due to pressure on adjacent major vessels • Fracture thru the base of stalk (Commonest cause of pain) • Kyphosis (if the location is in spine)

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Continued stimulation of these foci along with endochondral bone growth forms an exostosis. Wide based lesions in pelvis undergoing sarcomatous change are termed as Epiexostotic Chondromas. It rotates and moves towards shaft away from the epiphysis.Spondylolisthesis if it is attached to intervertebral joint Fascial asymmetry and masticatory dysfunction in lesions in head and neck • Malignant transformation Spontaneous resolution of these lesions. Hypotheses on mode of development: Lichtenstein proposed anomalous periosteal development that produces displaced nests of cartilage. • loss of blood supply due to the fracture. mandibular condyle. coronoid process. • termination of cartilaginous cap and active resorption. pedicle. Bilateral sessile osteochondromas were found in external auditory canal in cold water swimmers! Intra articular osteochondroma like lesions are demonstrated in femoral neck in Perthese disease. They can be due to • cessation of lesional growth. Some lesions can occur in the capsule • • 2 . At the site of origin cortex is absent and the bone of osteochondroma merges into the underlying spongy bone. costovertebral joint. Apart from the ends of long bones they are seen in scapula. The common sites of occurrence are familiar to all. vertebral arch. A congenital anomaly of the diaphyseal blood vessels is postulated as being responsible for the development of osteochondromas. Pathogenesis: Osteochondromas form from bones developing from cartilage like all the long tubular bones. origin in the perichondral groove of Ranvier (which surrounds the growth plate) or a defect at the periphery of the growth plate in the perichondral groove. are well documented. The four most popular hypotheses include – origin in epiphyseal growth plate. These can also be caused due to irradiation in children – well documented. not a true neoplasm but rather an enchondromatous hyperplasia. because the lesion is formed by endochondral ossification and the bone substance produced is in every way normal. This theory was proved by reproducing these lesions by implanting epiphyseal cartilage next to epiphysis towards metaphysis in rabbits. tip of spinous process. ribs. innominate bone. • incorporation of lesion by appositional growth into adjacent cortex. They arise on the cortex next to epiphysis from zone of endochondral growth. origin in the periosteum. subungual (below nails) and as ivory exostosis on craniofascial bones. Osteochondromas are considered by many a perversion in the direction of bone growth.

Many divergent theories are postulated in the formation. free osteochondral bodies appear in the joint cavity. Keith called it diaphyseal aclasis.of the knee – periarticular chondroma or osteochondroma – as nodules deeply embedded in the synovium. He said that defective deposition of bone in this area permits the expansion of cartilage preformed bone to grow in anomalous directions precluding normal osseous modeling. Virchow and Von Recklinghausen postulated that both exostosis and enchondromas are derived from the cartilaginous growth plates. Miller postulated that exostosis originated in nests of cartilage arising from the disordered osteogenic layer of the periosteum. This unrestrained growth of bone at the epiphyseal plate ‘runneth over’ onto the outer surface of the bone constituting the exostosis. DD – Peripheral Chondrosarcoma. Keith postulated that it is related to the defective development of periosteal ring surrounding the growth plate. In the early stage the process is intra-synovial without any loose bodies. X-rays: flat sessile / plateau like / with a stalk / Kleiderhaken appearance (tubular appearance ending in a hook). small portions of cartilage may become separated from the lateral aspect of the epiphyseal plate and make a right angle turn shaft wards. They can present as a single large loose body or multiple small synovial chondromatosis. Multiple Osteocartilaginous Exostosis ( Hereditary multiple Exostosis. Juxtacortical osteosarcoma. New exostoses do not form in adults. Lichtenstein considered 1 cm as the upper limit. In any case of the cartilage cap is irregular and more than few millimeters thick. they can be mistaken for enchondromas. Malignant transformation is suspected when the demarcation is interrupted and a soft tissue mass is present with streaky and blotchy radio-opacity is seen. of bones causes deformities in forearm. Calcifications are seen as fluffy amorphous opacities. Myositis ossificans Epiphyseal Dysplasia – Dysplasia epiphysealis hemimelica – Trevors Disease (DD in distal femur and tibia) In Trevors disease isolated irregular centers of ossification form on one side of epiphysis of involved articular segment. it should be suspected as a Chondrosarcoma. Unequal growth. Later in the advanced stage. This condition manifests during childhood. establishing the onset of an exostosis. Half the progeny of affected parent express the trait. Diaphyseal Aclasis): Boyer described this condition originally in 1814. If there is an end-on view. Thus. It is inherited by single autosomal dominant gene. Tibio fibular growth discrepancies cause deformities 3 . In later stages these separate ossification centers fuse together. Shortness of stature is manifested. resulting in symmetrical epiphyseal enlargement causing limb length discrepancy and deformities.

These cartilage cells giving raise to the lesion may be embryonic rests derived from epiphyseal cartilage plate. we have to suspect malignant transformation. Calcification causes grittiness of the lesion. This is the commonest tumor in the patella. The transformation of a benign enchondroma to a Chondrosarcoma has been traced and well documented histologically. especially on limited biopsy material. Lesions at the base of skull or vertebrae may cause neurologic symptoms. central lesion. If present in long or flat bones they have increased propensity for malignant transformation. If it is peripheral it is cammed a ‘periosteal’ of ‘juxtacortical’ enchondroma. Local trauma can cause a pathological fracture and cause severe pain. they are coarsely lobular or nodular myxomatous structure with bluish opalescence due to the hyaline cartilage. In multiple variety. Solitary Enchondroma: Def: Solitary enchondroma. The pathologist’s tendency to under diagnose chondrosarcomas. Thus an enchondroma is produced by disordered endochondral bone formation that results in occurrence of a cartilage tumor in the metaphyseal and diaphyseal regions of the shaft. previously stable 4 . makes evaluation of this contention rather difficult in most cases. The beginning of persistent unrelenting pain or cortical penetration by the tumor withour apparent trauma are foreboding signs. They arise during the growth period and manifest in third or fourth decade. They are usually asymptomatic. it is called an Enchondroma. 35% arise in the hand. If it is central. they are common in distal ends of proximal and middle phalanges. There can be a calcifying enchondroma in large lesions with central calcifications. Indications for surgical removal of enchondromas as per Memorial SolanKettering Cancer Institute – all enchondromas arising in the long bones or in the pelvis and shoulder girdles showing  renewed.of knee and ankle. Caballes called these peripheral lesions as “Enchondroma Protruberans”. Pathogenesis: It evolves from heterotrophic cartilage rests found frequently in otherwise normal metaphyses. If there is pain without trauma and pathological fracture. Grossly. also known as central chondroma is a benign hyaline cartilage growth that develops within the medullary cavity of a single bone. They are easy to diagnose on x-rays with a well demarcated edge. pelvis and scapula are most frequently affected areas. paper thin cortex and clear center. Ribs. nasal cavity and sinuses. In the vertebral body it has to be differentiated from a Schmorl’s node. Lesions in long and flat bones are rare. no periosteal reaction. cause fusiform shape of the phalanx.

these lesions are not related to areas of cartilaginous growth plates. This condition is compatible with long life. persistent unrelenting pain. Pathogenesis: Unlike enchondromas. Pathogenesis: Jaffe postulated that cartilage derived from cambium layer of periosteum penetrated the shaft of involved long bone and is distributed throughout. osteochondroma and Chondrosarcoma on the x-rays. Secondary malignance can also be a chordoma and ostoegenic srcoma. Skeletal enchondromatosis is a cartilage dysplasia of one representing an inborn anomaly of osseous development. poorly defined margin and focal periosteal reaction. They cause deviation of proper growth. glomus tumor. 5 . These are common in long bones like humerus and ribs. x-ray signs of cortical destruction without trauma. a developmental error in endochondral calcification. Transformation into Chondrosarcoma is very common. skull. Differential diagnosis – Metachondromatosis (described by Maroteaux in 1971) mimics multiple enchondromatosis. Bones of hands are most commonly involved. This is an autosomal dominant condition and there are multiple osteocartilagenous exostoses in digits and long bones which point towards the joint and disappear spontaneously. but are probable produces by sub-periosteal cartilage formation. They are rarely seen in the hyoid bone. It is differentiated as it is charecterised by multiple enchondromas in iliac crests and metaphyses of long bones along with periarticular multiple joint calcification and ossification. X-rays show clear bubble like lesion on the cortex with a rim of reactive new bone and bone deposition beneath the saucer like cortical erosion. These unossified remnants of cartilage in metaphysis and diaphysis may evolve to form large tumors. They form and continue to grow past skeletal maturity. femur and tibia. Multiple Enchondromatosis (Ollier’s Disease): (Ollier – French physician from Lyon). There is no evidence of generic factor. They also involve the flat bones. nonspecific tenosynovitis. deformities with eventual brachydactyly. They have to be differentiated from Pigmented villonodular tenosynovitis. ganglion. The association of Ollier’s disease with a functioning ovarian tumor has been reported.growth. Juxtacortical Enchondroma: This is a benign cartilage growth beneath the periosteum and external to the bone. Incidence of malignant transformation according to Mayo clinic data is 30% and 50% according to Jaffee.

Bilateral fibroadenomas are also seen in females affected with Maffuci’s. Non specific pain and wasting of muscles are common. characteristically presenting in the epiphyses of long bones such as the humerus. On arteriographic study. Malignant transformation rage is very high upto 19%. Buttress type of periosteal reaction is seen. Codman in 1931 described it in detail – epiphyseal chondromatous giant cell tumor. numerous phleboliths can be seen in the accompanying hemangiomas. Histogenesis: Proposed by Jaffe and Lichtenstein – it is a primary tumor of bone that developed from cartilage germ cells. also called Maffuci-Kast syndrome. usually benign bone tumor of immature cartilage cell derivation with preferential localization in the epiphysis. less than 1% of all primary bone tumors. a cartilage containing giant cell tumor. tibia. multiple arteriovenous malformations may be noted. Occasionally patella. Although secondary involvement of the metaphysis after skeletal maturity is recognised. Enchondromas are associated with soft tissue hemangiomas. Tissue culture studies showed that the cell of origin was of histiocytic derivation. Secondary synovial reaction with 6 . May present like early arthritis. is affected. Also known as Codman tumor. and femur. primary.Maffucci’s Disease: Described by Maffuci in 1881. The radiographic features are identical with the lesions in Olliers disease. Ewing in 1928 called it – calcifying giant cell tumor. It is noted that a Chondrosarcoma arising within the complex of Maffuci’s located in the craniofascial bones may be associated with a carotid body tumor in the same patient. which is considered equivalent to an epiphysis. Pathological fracture is rare. Chondroblastoma: Chondroblastoma is a rare. occasionally some patients may have a fully malignant osteogenic sarcoma or other non-osseous malignant tumors like pancreatic or biliary adenocarinoma or an astrocytoma. representing. Equally unusual is involvement of the vertebra or intracortical location in the long bones. Kolondy in 1927 called it – giant cell tumor variant. In some cases. Jaffe and Lichtenstein called it – benign chondroblastoma. a predominantly metaphyseal or diaphyseal location is exceedingly rare. 50% lesions are unilateral. is a benign lesion occurring before skeletal maturity. Although a low grade Chondrosarcoma is the likeliest outcome in a patient with Maffuci’s syndrome.

Synovial membrane and articular recurrences follow transarticular curettage. If it appears after cessation of skeletal growth. All these lesions. necessitating major ablation. It has a well defined sclerotic margin and hemispheric “bite-like” cortical destruction. grey yellow to grey brown. The heavier the calcification. chondroblastoma and fibrous dysplasia. 7 . Intermediate Phase – focal areas of calcification. Late Phase – connective tissue replacement of necrotic tissue. Chondromyxoid fibroma: This is a benign tumor of bone characterized by chondroid and myxoid differentiaton of its basic tissue growing in a lobular pattern. Long standing. Chondroid or even osseous matrix formation. On gross examination the curetted tissue shows soft or friable mottled grayish material that appears to be focally gritty. S-100 protein immunostaining provides help in histologic differential diagnosis of chondroblastoma. Curettage with intra-operative implantation often results in clinical soft tissue recurrence or tumor invasion of adjacent bones. although histologically benign. Innominate or the sacrum. With curettage with grafting the recurrence rates dip steeply. the heavier the degeneration and necrosis of tumor cells. bones of skull. Radiologically it is an eccentric round or oval lesion in the metaphysis of a long bone. subarticular location is a rule. Large nuclei. In some planes the external shell is difficult to outline if there is no sup-periosteal new bone formation. mandible and are common in the calcaneum. Axial and appendicular skeleton is equal not sparing the tarsal. Lesions can also occur in the carpal and tarsal bones. There are lot of pseudo-trabeculations. Mictoscopically the pericellular lattice like fine calcification attanged in the characteristic “chicken wire” or “picket fence” pattern is the hall mark of this lesion. neglected lesions may extend spontaneously into adjacent joints. Thus the differential diagnoses will be a giant cell tumor. The evolutionary progression in chondroblastoma as studied by light microscopy is depicted as – Earliest Phase – compact round or polyhedral cells of moderate size.synovitis and effusion is common. unicameral bone cyst. thereby emphasizing the importance of avoiding entering the adjacent joints. Curettage without grafting has a recurrence rate of 15%. Exclusive epiphyseal lesions are smaller than the ones extending on to the metaphysis. occasionally more than one nucleus with a sprinkling of multinucleated giant cells. eccentric aneurismal bone cyst. non-ossifying fibroma. articular surfaces and soft tissues. did indeed exhibit aggressive but self-limited clinical behavior. They often straddle the adjacent epiphysis and metaphysis.

of a cystic radiolucent appearance may even be presented. peripheral. Ollier’s disease and Maffuci’s disease. No medullary involvement is demonstrated and the cortex is rarely affected. central lesions demonstrate large thick walled areas of radiolucency with trabeculation and central areas of multilocular medullary bone destruction. ‘popcorn’. juxtacortical chondroma. Foci of irregularly scattered spotty or blotchy calcification represented by densities throughout the lesion are noted. Pain. Once the lesion is in an advanced stage. In peripheral lesions (from exostosis) the progressive gradual erasure of the smooth outlines of a cartilaginous cap from the periphery inward toward the cortex and a soft tissue mass with foci of irregular calcific speckles represent fairly good evidence that malignant transformation had taken place. tenderness are vital symptoms but absent in pelvic lesions. Some lesions show thinning and bulging expansion of cortex. On x-rays. and infiltration with scattered foci of ossification is noted. ‘breadcrumb’ configuration. Types – Nomenclature – Primary chondrosarcoma arises de novo in bone. with periosteal new bone formation it mimics Pagets disease. juxta-cortical and rarely periosteal. calcification. with radiating spicules or large speckles arranged at fight angles to the cortex. These calcifications have also been described as appearing as ‘fluffy’. Codman’s triangle is seen in early stages due to the elevation of the periosteum. Such central lesion should be differentiated from bone infract before jumping into this malignant diagnosis. Histologic grading of chondrosarcoma – 8 . Sometimes if the lesion is extending all along the shaft. ‘cotton wool’. Adjacent periosteum may be elevated. Excluding multiple myeloma. chondrosarcoma is second to osteogenic sarcoma in frequency as a malignant tumor of bone. Secondary chondrosarcoma develops from a pre-existing benign cartilage tumor like – enchondroma. cap of osteocartelagenous exostosis. Secondary chondrosarcomas from central lesions (enchondroma) show the original lesion and the superimposed malignant change is characterized by a fuzzy infiltrating periosteal activity. with sparsely calcified shadows in the soft tissues next to the lesion.Malignant cartilage tumor – Chondrosarcoma: Chondrosarcoma is a malignant tumor in which the basic neoplastic tissue is fully developed cartilage without tumor osteoid being directly formed by a sarcomatous stroma. Peripheral lesions are faintly visible. or ossification may be present. the entire lesion becomes irregularly and coarsely granular. Myxoid changes. The adjacent soft tissues show evidence of stippled calcification. These can be central. These present with symptoms of pressure effects on nerves or vessels.

chemo and radio-theraputic options for each tumor. because chondrosarcomas are known to be resistant to radiotherapy and chemotherapy. Nuclei are small and dense. and pleomorphism. atypia. The reported recurrence rate ranges from 4-12%. aneuploidy of the tumor coupled with a high mean DNA index. with extended intralesional curettage being considered a viable alternative to wide resection. and high (grade 3). the material is abundant and obviously chondroid. and is much less biologically aggressive than the other mentioned subtypes. Subtypes of chondrosarcoma such as dedifferentiated or mesenchymal have a poor prognosis and are beyond the scope of this review. histologic grade 3. wide resection has generally been accepted as recommended treatment for low-grade chondrosarcoma. tumor volume greater than 100 cc. The cartilage cells are present in lacunae. and binucleate cells are sparse. Thus. Grade 3 lesions are characterized by marked hypercellularity and extreme plemorphism with bizarre giant-cell tumors and mitoses. and foci of necrosis almost are invariably present. The incidence of bi-nucleate cells is increased. which may be enlarged. Occasional microscopic areas of necrosis may be found. depending on initial treatment modality and tumor grade Classically. Evans et al reported that if there are >2 mitoses per 10 high-power fields. Only rarely is there myxoid material. and multinucleated cells are seen. intermediate (grade 2). recommended treatment of these borderline tumors has ranged from marginal curettage and grafting to limited local resection.Conventional chondrosarcoma is separated into three histological grades: low (grade 1). Low-grade tumors have mild hypercellularity and mild cellular atypia. pelvic location. and the nuclei often enlarged. and dedifferentiated chondrosarcomas. Borderline cartilage tumors have an even lower metastatic potential than the true lowgrade (grade 1) malignant chondrosarcoma. There has been a push for more conservative treatment of borderline and low-grade malignant chondrosarcomas. Rare mitoses may be found. correspondingly less matrix. These features usually are especially prominent at the periphery of tumor lobules. common bones. Myxomatous change often is prominent. Clear-cell chondrosarcoma is another rare variant that resembles chondroblastoma in its presentation. Grading is highly subjective and based on relative degrees of cellularity. 9 . I encourage the postgraduate to build up his answers based on these finer points by filling up details like incidence. increased cell numbers. Lee et al monitored 227 patients with chondrosarcoma for an average of 6 years and reported predictive factors for metastasis to be local recurrence. the tumor is most likely high grade. and treatment options and latest surgical. The cartilage cells usually are not located in lacunae. Intermediate chondrosarcomas have significant atypical.

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