Cartilage tumours of the bone. Diagnosis and therapy

Primary malignant bone tumours are rare. The annual incidence of these tumours is 10 per 1 million. Nearly 30% of the primary malignant bone tumours are malignant cartilage tumours. The frequency of benign cartilage tumours cannot be definitely estimated because these tumours are normally clinically inapparent and therefore often diagnosed as an incidental finding. The cartilage tumours appear as benign lesions (e.g. chondroma), as borderline tumours (proliferative chondroma vs grade I chondrosarcoma) or as highly malignant chondrosarcoma (e.g. dedifferentiated chondrosarcoma). Commensurate with the different clinical and oncological manifestations of the cartilage tumours, there are wide differences in the treatment and clinical course of the individual tumour. This article discusses the problems in the diagnosis and treatment of cartilage tumours from an orthopaedic point of view.     

Primary sternal tumours

Nine cases of primary sternal tumour were retrospectively reviewed in regard to clinical, radiologic and surgical features. The tumours were chondrosarcoma (4), chondroma (3), solitary plasmacytoma (1) and osteochondroma (1). All the benign lesions were excised without complication or recurrence. Three chondrosarcomas were radically excised en bloc, and one was only locally excised because of gross involvement of underlying structures. The solitary plasmacytoma was treated with incisional biopsy and radiotherapy. Two of the three patients with radical excision of sternal chondrosarcoma were alive after 5 years. Careful preoperative assessment, including use of computed tomography, is important. Wide excision should be the procedure of choice for all sternal tumours, since differentiation between benign and malignant lesions may be difficult in cartilaginous tumours, which are the commonest types.     

Primary tumours of the ribs

Thirty-four primary rib tumours (24 benign, 10 malignant) were surgically treated in 1966-1985. The mean age was higher and the tumour diameter was greater in the patients with malignant, than in those with benign neoplasm. The benign tumours were excised without operative death. At follow-up after a mean of 12.3 years there was no recurrence of benign growth, but in two cases with initial diagnosis of chondroma a regrowth at the same site proved to be chondrosarcoma. Among the cases of malignant tumour there was one operative death from pulmonary embolism, after radical resection of sarcoma. None of the four patients with chondrosarcoma had recurrence 6-13 years after surgery. There was no long-term survival among the patients with other forms of sarcoma or malignant tumour of the reticuloendothelial system.     

Long-term follow-up of cartilaginous tumors of the larynx.

BACKGROUND: Cartilaginous tumors of the larynx are uncommon. A literature review disclosed approximately 250 cases since 1816; the cricoid cartilage is the most common site. The rarity of these tumors has made for limited experience and, as a consequence, our knowledge is incomplete. OBJECTIVE: To report surgical results as well as long-term follow-up on 6 patients with cartilaginous tumors of the larynx. DESIGN: A 28-year retrospective study with the patients followed-up from 6 to 28 years (average, 17.8 years). METHODS: Six adult white male patients with cartilaginous tumors of the larynx: 4 low-grade chondrosarcoma (1 of the thyroid and 3 of the cricoid) and 2 chondroma of the cricoid. Surgical treatment included total laryngectomy of the thyroid and 1 of the cricoid chondrosarcoma, and conservation surgery of the other 4 cricoid tumors: the 2 patients with chondrosarcoma had total resection of the cricoid cartilage with thyrotracheal anastomosis, and the 2 patients with chondroma had local tumor resection using a laryngofissure approach. RESULTS: The margins of the specimen were negative for tumor in the 6 patients. On follow-up, none of the patients had regional or distant metastasis or tumor-related death. One of the patients with cricoid chondrosarcoma developed recurrence 8 years after conservation surgery, and required a total laryngectomy for salvage. Survival rate tumor-free at 5 years was 100% and at 10 years 67%, co-morbidity being responsible for the decrease in survival rate. CONCLUSIONS: Based on this small series of patients, the long-term follow-up of benign and low-grade malignant tumors suggests that the surgical approach and prognosis does not depend on histologic distinction and, importantly, underdiagnosed malignancy on tumor sampling and recurrent chondrosarcoma, managed with salvage surgery, have no adverse impact on patient survival. Total resection of the cricoid cartilage with thyrotracheal anastomosis over a stent proved an alternative surgical technique in chondrosarcoma who otherwise would have been treated by total laryngectomy.     

Knorpelbildende Tumoren

Primary cartilage-forming tumors of the bone are a large group among the rare bone tumors. The clinical and radiographic findings of the different entities show similar findings. Bone biopsy is still the most relevant examination in the final diagnosis of the lesion. The requirements for a correct biopsy and the main features of the macroscopic and histologic findings of cartilage tumors are presented from the viewpoint of the pathologist. Differentiation between benign enchondroma and grade I chondrosarcoma requires close interdisciplinary cooperation to avoid over-treatment and relapse. Rare low-grade malignant cartilage tumors such as clear-cell chondrosarcoma need to be diagnosed in specialized centres to arrive at the correct therapy. The morphologic features of mesenchymal chondrosarcoma, clear-cell chondrosarcoma and secondary chondrosarcoma in osteochondroma are demonstrated. The aim is to correlate the morphologic and radiographic features. The same applies to benign entities such as osteochondroma, chondroblastoma and chondromyxoid fibroma.     

Cartilage tumors. Classification, conditions for biopsy and histologic characteristics

Primary cartilage-forming tumors of the bone are a large group among the rare bone tumors. The clinical and radiographic findings of the different entities show similar findings. Bone biopsy is still the most relevant examination in the final diagnosis of the lesion. The requirements for a correct biopsy and the main features of the macroscopic and histologic findings of cartilage tumors are presented from the viewpoint of the pathologist. Differentiation between benign enchondroma and grade I chondrosarcoma requires close interdisciplinary cooperation to avoid over-treatment and relapse. Rare low-grade malignant cartilage tumors such as clear-cell chondrosarcoma need to be diagnosed in specialized centres to arrive at the correct therapy. The morphologic features of mesenchymal chondrosarcoma, clear-cell chondrosarcoma and secondary chondrosarcoma in osteochondroma are demonstrated. The aim is to correlate the morphologic and radiographic features. The same applies to benign entities such as osteochondroma, chondroblastoma and chondromyxoid fibroma.     

Strategy of the surgical treatment of primary tumors of the spine

The surgical treatment of the primary spinal tumors is started after the analysis of the following data: --location of the tumor, --pathological identification after the biopsy examination, --staging of the lesion according to Enneking. In case of benign tumors it is possible to realize: a total resection by morcellement until healthy margins in case of chondroma or osteochondroma, intralesional excision until healthy borders in case of osteoid osteoma, total excision by partial anterior or posterior vertebrectomy (with previously embolization) according to the location (body or neural arch resection) in case of giant cells tumour or aneurysmal bone cyst. In case of malignant tumors, the total resection is an absolute goal when the feasability occurs (total vertebrectomy), in case of chordoma, chondrosarcoma and malignant giant cells tumours; the stabilization surgery is indicated without attempt of total tumor removal because others means are availables and/or accurates (radio and/or chemotherapy) in cases of Ewing's sarcoma, plasmocytoma and lymphoma.     

Malignant tumours of bone: clinical aspects and natural course.

An accurate pathological diagnosis must be made prior to treatment of a primary malignant bone tumour. Consideration must be given to the clinical and radiologic aspects as well as the histology. Both benign and malignant tumours occur more frequently in certain decades. A search should be made for precursor lesions such as Paget's disease. The presenting manifestations of pain, a mass and dysfunction are not specific for tumours. Laboratory tests may be helpful, especially in distinguishing tumours from infections and metabolic diseases. Metastasis is usually via the blood stream to the lungs and bones. The low survival rate following amputation for osteosarcoma and radiation therapy for Ewing's sarcoma has been improved by chemotherapy. The lower-grade tumours such as aggressive giant cell tumour and low-grade chondrosarcoma can often by treated successfully by resection and insertion of an autograft, an allograft or a metallic implant.     

Chondrosarcoma of the larynx: a clinicopathologic study of 111 cases with a review of the literature

Chondrosarcomas of the larynx are rare tumors accounting for about 0.5% of all laryngeal primary tumors. A total of 111 laryngeal chondrosarcoma cases, diagnosed between 1970 and 1997, were retrieved from the Otorhinolaryngic-Head & Neck Tumor Registry of the Armed Forces Institute of Pathology. There was a 3.6:1 male/female ratio of patients 25-91 years of age (mean, 64.4 years). Patients presented most frequently with hoarseness (n = 72 patients) present for a mean of 28.2 months. The majority of tumors involved the cricoid cartilage (n = 77) with a mean size of 3.5 cm. All tumors were invasive and malignant by radiology and/or histology (into bone within the ossified laryngeal cartilages in 52 tumors). Most tumors were low-grade lesions: grade 1 (n = 51), grade 2 (n = 54); there were six grade 3 tumors. An associated benign chondroma with (n = 41 tumors) or without ischemia (n = 24 tumors) was noted. All patients had surgery and five had radiation therapy. Wide excision or voice-sparing surgery was used in 73 patients, whereas 37 patients had a laryngectomy. Recurrences occurred in 20 (18%) patients, 10 of whom underwent salvage laryngectomy. At the last follow-up, 102 patients had no evidence of disease (alive or dead, mean 11.2 years) and five patients had evidence of disease (alive, one patient, 6.5 years; dead, four patients, mean 6.4 years). The six patients with high-grade chondrosarcoma were all without disease at the last follow-up (mean, 15.1 years). There was no difference in clinical outcome based on grade (p = 0.210), location (p = 0.078), or treatment (p = 0.607) but was worse for patients with a myxoid-type chondrosarcoma (p = 0.044). Primary laryngeal chondrosarcomas are typically low- to moderate-grade lesions involving the cricoid cartilage, frequently associated with a chondroma. They usually portend an excellent overall long-term prognosis with initial conservative voice-sparing surgery.     

Review of para‐articular soft tissue osteochondromas of the knee

Para‐articular osteochondromas (PAOCs) are a rare benign subtype of chondroma that occur in the soft tissues around the joints. Initially hypothesized to be derived from cartilaginous metaplasia, its aetiology and pathogenesis are still not fully understood. This tumour often causes a diagnostic and management dilemma due to similar clinical and histopathological features compared with other pathology. We report the fourth case of PAOC arising from the suprapatella region of the knee joint and review the literature to update our understanding of this tumour. From the data analysed, a distinctive pattern of tumour formation within or adjacent to a fat pad structure was evident, while trauma appears to play a role in its pathogenesis. Histologically, the tumour consists of a cartilage cap, with varying degrees of trabecular bone and endochondral ossification. Importantly, there are no features of nuclear pleomorphism or increased mitotic activity suggestive of a malignant process. All tumours were surgically removed and behaved in a benign fashion without evidence of recurrence. PAOC may often be suspected for more malignant processes; thus, recognition of this entity is important as simple excision is curative.     

Periosteal chondroma and periosteal chondrosarcoma

A clinicopathologic study of 46 patients with periosteal chondroma and 14 patients with periosteal chondrosarcoma revealed that periosteal chondroma tended to affect younger patients and that the lesion was usually smaller. Radiographically, the typical periosteal chondroma was a small, well-marginated tumor on the outer surface of a long bone. Erosion of the cortical surface and marginal buttresses were usually present. Periosteal chondrosarcoma had a more aggressive appearance and was seen as a large mass located superficially on the cortex; the margins of the mass were more irregular than those of chondroma. Histologically, periosteal chondroma frequently showed hypercellularity, plump nuclei, and binucleation. Thus, the differentiation of chondroma from chondrosarcoma is difficult and is based mainly on evidence of invasion. The prognosis in periosteal chondroma is good: only one patient had a local recurrence, none of the tumors underwent malignant change, and excision seems to be curative. However, the prognosis in periosteal chondrosarcoma is not as good: two patients died of metastasis to the lungs after local excision and two patients had recurrences after local resection. Periosteal chondrosarcoma should be treated more aggressively than periosteal chondroma.     

Long-term follow—up of cartilaginous tumors of the larynx

Background: Cartilaginous tumors of the larynx are uncommon. A literature review disclosed approximately 250 cases since 1816; the cricoid cartilage is the most common site. The rarity of these tumors has made for limited experience and, as a consequence, our knowledge is incomplete. Objective: To report surgical results as well as long—term follow—up on 6 patients with cartilaginous tumors of the larynx. Design: A 28—year retrospective study with the patients followed-up from 6 to 28 years (average, 17.8 years). Methods: Six adult white male patients with cartilaginous tumors of the larynx: 4 low-grade chondrosarcoma (1 of the thyroid and 3 of the cricoid) and 2 chondroma of the cricoid. Surgical treatment included total laryngectomy of the thyroid and 1 of the cricoid chondrosarcoma, and conservation surgery of the other 4 cricoid tumors: the 2 patients with chondrosarcoma had total resection of the cricoid cartilage with thyrotracheal anastomosis, and the 2 patients with chondroma had local tumor resection using a laryngofissure approach. Results: The margins of the specimen were negative for tumor in the 6 patients. On follow-up, none of the patients had regional or distant metastasis or tumor—related death. One of the patients with cricoid chondrosarcoma developed recurrence 8 years after conservation surgery, and required a total laryngectomy for salvage. Survival rate tumor-free at 5 years was 100% and at 10 years 67%, co-morbidity being responsible for the decrease in survival rate. Conclusions: Based on this small series of patients, the long—term follow—up of benign and low—grade malignant tumors suggests that the surgical approach and prognosis does not depend on histologic distinction and, importantly, underdiagnosed malignancy on tumor sampling and recurrent chondrosarcoma, managed with salvage surgery, have no adverse impact on patient survival. Total resection of the cricoid cartilage with thyrotracheal anastomosis over a stent proved an alternative surgical technique in chondrosarcoma who otherwise would have been treated by total laryngectomy. (Otolaryngol Head Neck Surg 2001;124:634-40.)     

A pathologic fracture of an intracortical chondroma masking as an osteoid osteoma

The differential diagnosis of a tibial intracortical diaphyseal lesion includes osteoid osteoma, periosteal chondroma, nonossifying fibroma, osteofibrous dysplasia, and adamantinoma. While osteoid osteomas represent 5% of all primary bone tumors, little is understood about intracortical chondromas. Intracortical chondroma was first described in 1990 and 7 reported cases have since been published. This article presents the first reported case of a pathologic fracture of an intracortical lesion in a child that shared radiographic and clinical features similar to those of osteoid osteoma, but on histopathologic examination revealed an intracortical chondroma. Our patient exhibited radiographic features of a poorly circumscribed cortical bone sclerosis, a centralized radiolucent nidus on computed tomography, and a hot bone scan of a lesion <1 cm in size that was consistent with an osteoid osteoma. An excision of the bone lesion was performed. The histopathology of the lesion revealed nodules of benign hyaline cartilage in cortical bone, consistent with an intracortical chondroma. Demarcated by cortical bone with mature Haversian systems rather than periosteum or cancellous bone, intracortical chondroma differs from the other 2 chondroma variants, periosteal chondroma and enchondroma, by its relationship to the surrounding bone. Enchondromas are characteristically understood to be asymptomatic. Intracortical chondromas along with periosteal chondromas have been found to present as painful lesions. The similarities with osteoid osteoma and intracortical chondroma in our patient make it circumspect in regards to ablating lesions (ie, needle radiofrequency ablation) without acquiring a biopsy in pediatric patients that both clinically and radiographically are presumably an osteoid osteoma.     

Chondroma and chondrosarcoma.

A chondrosarcoma is the most common primary malignant tumor of the chest wall. It usually presents on the anterior chest wall arising from the costochondral arches or sternum. It may occur as the result of malignant degeneration of a benign chondroma. Both chondrosarcomas and benign chondromas present as painful, slow-growing, hard, fixed, and nontender anterior chest wall masses. Microscopic differentiation between the two tumors is difficult and the treatment for both tumors is wide excision with a margin of at least 4 cm. Chemotherapy is ineffective and radiation therapy is used only for patients with tumors that are either not amenable to surgical resection or have positive resection margins.     

The myth regarding bone or cartilage involvement by cancer and the likelihood of cure by radiotherapy

A myth persists that tumor involvement of bone and/or cartilage represents a clinical situation in which cure by radiotherapy would be considered unusual. There are sufficient data to the contrary. This essay reviews the radiotherapy results for (1) primary tumors of bone (benign and malignant), (2) primary malignant tumors of cartilage, and (3) benign and malignant tumors that secondarily involve bone and/or cartilage. It is hoped that the myth concerning radiocurability and bone and/or cartilage involvement will be replaced by an appreciation of the relative rates of cure depending on the clinical situation.     

Periosteal chondroma of the proximal phalanx of the thumb

A 52-year-old man presented with a periosteal chondroma in the base of the proximal phalanx of the right thumb. Magnetic resonance imaging (MRI) showed invasion of the bone marrow, which suggested malignancy. Although the clinical and MRI findings indicated a malignant tumour, the histopathological examination showed a benign periosteal chondroma.     

Tumors of the ribs: experience with 47 cases.

OBJECTIVES: To emphasise the existing difficulties in differentiating benign from malignant rib tumours, and especially the problems that a clinical doctor encounters when dealing with a hyperplastic rib. METHODS: Forty-seven patients with rib tumour underwent surgery in a period of 12 years (1984-1996). In 40 cases (85%), the lesion was benign and in seven (15%) was malignant. Twenty-one benign tumours originated from cartilage and bone, seven were inflammatory, six originated from the bone marrow, and minor percentages (2.5-5%) had vascular, neurogenous, degenerative or miscellaneous origin. Three of the malignant tumours were primary chondrosarcomas and two were metastatic from kidney. The rest were metastatic from stomach (adeno-Ca), and skin (melanoma). The mean age in the benign group was 25.2 years and in the primary malignant group was 20.7 years. Related symptoms were pain (47%) and swelling (42.5%). One-third (32%) of the patients were asymptomatic and the lesion was accidentally found during routine chest radiography. All patients were treated surgically with wide excision of the tumour and the diagnosis was established histologically. RESULTS: Resection was complete and curative in all cases without recurrence. CONCLUSIONS: Since the likelihood of malignancy cannot be excluded, all rib tumours should be considered malignant until proven otherwise. Therefore, prompt intervention is necessary and wide and radical initial excision of the involved rib is advocated.     

Periosteal chondroma of the proximal phalanx of the thumb.

A 52-year-old man presented with a periosteal chondroma in the base of the proximal phalanx of the right thumb. Magnetic resonance imaging (MRI) showed invasion of the bone marrow, which suggested malignancy. Although the clinical and MRI findings indicated a malignant tumour, the histopathological examination showed a benign periosteal chondroma.     

Limb conservation in primary bone tumours by resection, extracorporeal irradiation and re-implantation

En-bloc resection, extracorporeal irradiation and re-implantation of the irradiated bone have been used to treat 15 patients suffering from primary malignant tumours of bone or cartilage and two with benign lesions. This treatment is an alternative to replacement by prosthesis or allograft bridging techniques. After a mean follow-up of over five years results are encouraging, despite some complications and the relatively long period before weight-bearing is allowed.     

Clinical signs and methods of treatment of enchondromas located outside the hand]

160 patients (72 men and 88 women) in the age from 12 to 71 were admitted to Orthopaedic and Traumatology Department Skubiszewski Medical University in Lublin because of enchondromas. In 126 patients chondromas were located in hand, and in 34 remaining patients (21,3%) in: humerus, forearm, femur, tibia, fibula and foot. The typical signs of enchondroma were: pain, little limitation of movement, thickening of tissues or pathological fracture. 31 patients were operated on. Enchondroma was resected with cutterage, the walls of osseus cavity cleaned with reamer and bone loss filled up with bone grafts or bone cement. 1 case of recurrence of chondroma of toe was noted. In one patent malignant change of chondroma of proximal metaphysis of tibia to chondrosarcoma was observed. Lesion resection and filling up the bone defect is the best method of treatment of enchondroma.