Outcome of conservative and surgical treatment of enchondromas and atypical cartilaginous tumors of the long bones: retrospective analysis of 228 patients.

BACKGROUND: Sufficient data on outcome of patients with clinically and radiologically aggressive enchondromas and atypical cartilaginous tumors (ACT) is lacking. We therefore analyzed both conservatively and surgically treated patients with lesions, which were not distinguishable between benign enchondroma and low-grade malignant ACT based upon clinical and radiologic appearance. METHODS: The series included 228 consecutive cases with a follow-up > 24 months to assess radiological, histological, and clinical outcome including recurrences and complications. Pain, satisfaction, functional limitations, and the musculoskeletal tumor society (MSTS) score were evaluated to judge both function and emotional acceptance at final follow-up. RESULTS: Follow-up took place at a mean of 82 (median 75) months. The 228 patients all had comparable clinical and radiological findings. Of these, 153 patients were treated conservatively, while the other 75 patients underwent intralesional curettage. Besides clinical and radiological aggressiveness, most lesions were histologically judged as benign enchondromas. 9 cases were determined to be ACT, while the remaining 7 cases had indeterminate histology. After surgery, three patients developed a recurrence, and a further seven had complications of which six were related to osteosynthesis. Both groups had excellent and almost equal MSTS scores of 96 and 97%, respectively, but significantly less functional limitations were found in the non-surgery group. Further sub-analyses were performed to reduce selection bias. Sub-analysis of histologically diagnosed enchondromas in the surgery group found more pain, less function, and worse MSTS score compared to the non-surgery group. Sub-analysis of smaller lesions (< 4.4 cm) did not show significant differences. In contrast, larger lesions displayed significantly worse results after surgery compared to conservative treatment (enchondromas > 4.4 cm: MSTS score: 94.0% versus 97.3%, p = 0.007; pain 2.3 versus 0.8, p = 0.001). The majority of lesions treated surgically was filled with polymethylmethacrylate bone-cement, while the remainder was filled with cancellous-bone, without significant difference in clinical outcome. CONCLUSION: Feasibility of intralesional curettage strategies for symptomatic benign to low-grade malignant chondrogenic tumors was supported. Surgery, however, did not prove superior compared to conservative clinical and radiological observation. Due to the low risk of transformation into higher-grade tumors and better functional results, more lesions might just be observed if continuous follow-up is assured.

Chondrosarcoma of sinonasal cavity: a case report and brief literature review.

BACKGROUND: Chondrosarcomas are slow-growing malignant tumors that usually arise from cartilaginous structures. It may occur in the head and neck region with a predilection for the maxillofacial skeleton, where it has been reported to occur particularly in the mandible and maxilla. Chondrosarcoma of the sinonasal tract is very rare. AIM: Report a new case CASE: We present the case of a 43-year-old man presenting with an incidental finding of a chondrosarcoma of the maxillary and ethmoid sinus with nasal extension. The tumor was completely resected using a transnasal endoscopic approach. Treatment has followed by a radiation therapy and the patient was considered free of disease at her 5 years follow-up. CONCLUSION: Surgery is the mainstay treatment of chondrosarcomas. In selected patients, complete resection can be achieved using transnasal endoscopic approach.

[Tumors affecting the temporomandibular joint - a literature review]. / Tumeurs de l'articulation temporomandibulaire ­ revue de la littérature.

Benign and malign tumors can affect the temporomandibular joint (TMJ) as any other articulation. Nevertheless, TMJ tumors are rare and mostly benign. Their clinical expression is varied including symptomatology similar to TMJ dysfunctional disorders, otologic or neurologic pathologies. In some cases, they remain totally asymptomatic. Hence, diagnosis is difficult since the symptomatology can be misleading with TMJ dysfunctional disorders or otologic disorders wrongly diagnosed. There is thus frequently a long delay between symptoms onset and diagnosis. The great variety of TMJ lesions explains the wide range of possible treatment modalities, mostly based on surgery. We provide here a review of the lesions originating from the TMJ. Tumoral or cystic mandibular lesion affecting the TMJ through local extension will not be discussed. Osteoma, osteoid osteoma, osteoblastoma, chondroma, osteochondroma, chondroblastoma, tenosynovial giant cell tumors, giant cell lesions, non-ossifying fibroma, hemangioma, lipoma or Langerhans cell histiocytosis are all possible diagnosis among the benign tumors found in the TMJ. Pseudotumors include synovial chondromatosis and aneurysmal bone cyst. Finally, malign tumors of the TMJ include mainly sarcomas (osteosarcoma, chondrosarcoma, synovial sarcoma, Ewing sarcoma, and fibrosarcoma), but also multiple myeloma and secondary metastases. We will review the clinical, radiological and histological aspects of each of these lesions. The treatment and the recurrence risk will also be discussed.

Chondrosarcoma of the head and neck.

Chondrosarcoma is a malignancy rarely encountered in the head and neck. In an attempt to define this tumor's characteristics and response to therapy, all cases of chondrosarcoma treated at the University of Michigan over the past 25 years were retrospectively studied. Fourteen cases originating in the nose and paranasal sinuses, mandible, temporal bone, and larynx were reviewed. Aggressive surgical resection was the mainstay of treatment, and resulted in an overall survival of 70%, with an average follow-up of 3.5 years. Survival was highest in primary temporal bone lesions, and lowest in paranasal sinus lesions. Unresectable lesions were not cured by other modalities. This study, therefore, continued to support the crucial role of wide surgical resection in the treatment of head and neck chondrosarcoma, but conservative resection, when needed to preserve important structures, has resulted in long-term survival.

Surgery for locally aggressive bone tumours.

Treatment of 16 patients with aggressive benign bone tumours and one patient with a low grade malignancy with a combined regimen of cryosurgery, phenolization and acrylic cementation is reported. Patients were aged between 9 and 51 years and were treated by this method between the years 1986 and 1993. Minimal follow up was 13 months. The commonest histological diagnosis was giant cell tumour (7), followed by aneurysmal bone cyst (6), chondromyxoidfibroma (3) and low grade chondrosarcoma (1). Patients were assessed for functional outcome and local recurrence. On average 86 per cent of premorbid function was restored at follow up and there was one local recurrence (6.29 per cent). We conclude that this is a satisfactory method of gaining local control of these tumours.

[Primary oral peripheral chondrosarcoma. Anatomo-clinical aspects and presentation of 2 cases with periodontal onset]. / Il condrosarcoma periferico orale primitivo. Aspetti anatomo-clinici e presentazione di due casi ad insorgenza parodontale.

After a brief analysis of the anatomoclinical aspects of chondrosarcoma in general and of the characteristic and particular aspects of the forms with maxillofacial onset (lower age of onset, elective sites of onset anterior in the maxillary and posterior in the mandible, often insignificant standard X-ray pictures, high percentage of error in clinical and histological diagnosis) two cases of peripheral periodontal onset, with low degree of malignity (grade 1), with aspecific clinical and radiological aspects, both locally recurrent several times after nonradical surgery are reported.

Conventional chondrosarcoma in a survivor of rhabdoid tumor: enlarging the spectrum of tumors associated with SMARCB1 germline mutations.

SMARCB1 germline mutations mainly predispose to rhabdoid tumors. However, less aggressive tumors with a later onset have also been reported in a context of SMARCB1 constitutional mutation-that is, schwannomatosis and meningiomatosis. No other tumor type has formally been observed in such a context thus far. We report on a patient treated for a thoracic malignant rhabdoid tumor at 8 years of age who subsequently developed a mandibular conventional chondrosarcoma at 13 years of age. Both tumors showed a loss of BAF47 expression. The malignant rhabdoid tumor exhibited a large 22q11.2 deletion and an intragenic deletion of SMARCB1 (exons 1 to 3), thus leading to a biallelic inactivation. A 2.8 Mbp deletion encompassing SMARCB1 was found in the germline. This context was a strong incentive to investigate SMARCB1 alterations in the second tumor. As expected, the chondrosarcoma showed the large 22q11.2 deletion but also an additional c.243C>G(p.Tyr18X) premature stop codon in the remaining allele. This report relates for the first time a pediatric conventional chondrosarcoma to the wide family of SMARCB1-deficient tumors. Moreover, we report here the first case of conventional chondrosarcoma arising in a context of constitutional SMARCB1 deletion and, thus, enlarge the spectrum of this tumor predisposition syndrome.

Chondrosarcoma metastatic to the oral cavity.

Metastases to the oral cavity from nonoral malignant lesions occur rarely. We report the case of a woman with chondrosarcoma of the pelvis in whom gingival metastases, in addition to pulmonary, skin, and brain metastases, developed. Clinical course, x-ray correlation, histopathologic review, and treatment modalities are described. The six previously reported cases of chondrosarcoma metastatic to the oral cavity are tabulated and analyzed. Metastases should be included in the differential diagnosis of gingival lesions in patients with prior or current nonoral malignant tumors.

Chondrosarcoma of the jaw: review of fourteen cases.

From 1960 to 1983 a total of 14 cases of chondrosarcoma of the jaws were treated at the University College Hospital in Ibadan, Nigeria. The age of the patients ranged from 19 to 55 years (average age, 35 years), and nine of the patients were male. Six of the lesions were located in the maxilla and eight in the mandible. Most of the patients presented too late in the course of the disease to benefit from treatment; only one is known to be still alive.

Osteosarcoma of the upper jaw.

8 cases of osteosarcoma of the upper jaw from a personal series of tumours are described, and their treatment discussed in the light of past experience. Of the cases, four are still alive and well 6 months to 11 years after treatment. A case is put forward for the initial use of radical surgery, especially maxillectomy with orbital exenteration. The addition of routine postoperative Adriamycin therapy is recommended in the light of its known activity against sarcomas. The palliation of advanced cases is also discussed.

[Treatment of primary bone sarcoma of the jaws]. / Traitement des sarcomes osseux primitifs des maxillaires.

Head and neck bone sarcomas are very uncommon tumors. Therefore, treatment modalities are not clearly established. The medical record of 12 patients with bone sarcoma of the jaw were reviewed. Six patients with osteosarcoma underwent primary chemotherapy followed by wide surgical resection, radiation therapy or combined radiosurgical treatment. The 2 and 5 years survivals were 66% and 40% respectively. Five patients with chondrosarcoma were treated by wide surgical resection alone or combined with postoperative radiotherapy and possibly chemotherapy. All patients were alive; the mean follow-up was 9 years. One patient had Ewing's tumor. Osteosarcoma and chondrosarcoma in head and neck patients have a high rate of local recurrences. Surgery is the mainstay of treatment. Patients with voluminous tumors and high-grade lesions should receive postoperative radiotherapy. The role of chemotherapy has not been defined.

Chondrosarcoma of the jaw and facial bones.

BACKGROUND: Osteosarcomas of the jaw frequently have chondroblastic differentiation, causing confusion with chondrosarcomas. METHOD: Clinicopathologic features and results of treatment were analyzed for a series of 56 patients (27 males and 29 females from 1.5 to 88 years of age) with chondrosarcoma of jaw and facial bones. Twelve patients (21.4%) were younger than 20 years. RESULTS: The major symptom was nasal obstruction or a painless mass; the median interval from the first symptom until initial treatment was 1 year. Of the 56 chondrosarcomas, 25(44.6%) involved the alveolar portion of the maxilla and maxillary sinus; 23 (41.1%) involved the nasal septum, ethmoid, and sphenoid; 6 (10.7%) involved the mandible; and 2 (3.6%) involved the nasal tip. Of the 19 patients with radiographic studies, 15 (78.9%) had an expanding soft tissue mass with varied matrix calcification and destruction of bone and 2 had a purely lytic lesion. The lesion was difficult to assess in the two others. Most tumors had a lobulated growth pattern of hyaline cartilage. Hypercellularity, nuclear pleomorphism, and binucleation were common features. Forty-three tumors were grade 1, 13 were grade 2, and none were grade 3. Modalities of treatment were known for 51 of the 56 patients. Forty-six patients (90.2%) had surgical treatment, 2 (3.9%) had combination radiation therapy and chemotherapy, 1 (2%) had radiation therapy alone, and 2 (3.9%) had biopsy only. Follow-up adequate for analysis was obtained for 42 patients. Of these, 14 (33.3%) had local recurrence; uncontrolled recurrence developed in 9 (21.4%) patients. No distant metastases were documented. Overall actuarial survival at 5, 10, and 15 years was 80.7%, 65.3%, and 56%, respectively. Survival was analyzed for location, size, and histologic grade of tumor. No statistically significant differences were found. CONCLUSIONS: Chondrosarcomas of the jaw and facial bones are extremely rare, locally aggressive tumors.

Chondrosarcoma of the jaws and facial skeleton: a review of the Japanese literature.

From the 35 cases of chondrosarcoma, the maxilla was the most frequently affected site (35.5%) and the mandible was the next (29.4%). Painless swelling is the conspicuous symptom for chondrosarcoma of the lower jaw and nasal complications are often recorded with the lesions of the upper jaw. Several kinds of histologically malignant featurs coexisted which made diagnosis complicated. The three-year survival rate was about 50% for chondrosarcoma of the lower jaw and much lower for the upper jaw.

Sarcomas of the jaw.

A retrospective analysis is made of 61 patients with clinical and histological diagnosis of pure sarcomas of the jaw treated between 1950 and 1984. Surgery was the treatment of choice in 41 cases. Biopsy or palliative treatment were undertaken in the remaining patients. Twenty-four cases relapsed before 12 months, and 12 relapsed before 3 years. Twenty-four died before 12 months, nine before 2 years, and three after 2 years. At present there is no evidence of disease in eight patients: three out of 25 fibrosarcomas (one more than 1 year, one more than 13 years, and one more than 14 years), three out of 15 chondrosarcomas (one more than 1 year, one more than 13 years, and one more than 14 years), and two out of 15 osteosarcomas (one more than 18 years). The prognosis is gloomy. However, an early diagnosis and radical surgery contribute to an improvement in the course of the disease.

Condrossarcoma / Chondrossarcoma

É relatado um caso de condrossarcoma bem diferenciado em uma paciente de 75 anos e que assumiu proporçöes exageradas. Säo comentadas algumas dificuldades de diagnóstico como aspecto clínico, radiográfico, histopatológico e as possibilidades terapêuticas como tratamento cirúrgico, quimioterápico e radioterápico. Ressalta-se também a dificuldade do clínico na indicaçäo do tratamento adequado levando em consideraçäo a idade da paciente, a dificuldade de se estabelecer limites entre benignidade e malignidade e a extensäo da neoplasia