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Plasma cell tumors with calcified amyloid deposition mistaken for chondrosarcoma.

PURPOSE: To report cases of plasmacytoma with a radiologic appearance simulating that of chondrosarcoma. MATERIALS AND METHODS: The authors describe four cases of plasma cell tumors with scattered foci of calcification within their matrix, causing them to resemble chondrosarcoma radiographically. Histologic examination was performed in three of these cases. RESULTS: Biopsy specimens demonstrated that the calcification in these tumors was contained in areas of amyloid deposition. Although amyloid formation in association with plasma cell dyscrasias is well known, sufficient calcification within the amyloid to become radiographically identifiable has only rarely been reported. CONCLUSION: Calcification in amyloid produced by plasma cell tumors may at times simulate the appearance of chondrosarcoma.

Amyloid↗

Primary chondrosarcoma arising in the parotid gland.

We report an extremely rare case of chondrosarcoma arising in the left parotid gland in a 45-year-old man who complained of painless swelling of the postauricular region. Computed tomography revealed a well-circumscribed tumor in the parotid area with a rim of scattered calcification. Under the diagnosis of benign parotid tumor, the tumor mass was removed with adequate margin. Histologic features were consistent with a low-grade chondrosarcoma showing lobular growth but clearly separated from adjacent glandular tissue of the parotid gland. Entire examination of the tumor disclosed no component of pleomorphic adenoma. There has been no evidence of recurrence for 2 months after the operation. The current case indicates that the parotid gland could be the site of occurrence of de novo primary chondrosarcoma.

Chondrosarcoma↗

Cyclooxygenase-2 expression in chondrosarcoma.

OBJECTIVE: In recent years it has become evident that tissue cyclooxygenase-2 (COX-2) may play a role in carcinogenesis and tumor malignancy. There is now a mounting body of information that strongly implies that COX-2 inhibitors may be of some value in the management of patients with carcinomas, and most recently several similar reports have appeared relating to sarcomas. METHODS: The authors studied 32 samples of cartilage tumors from our tumor tissue bank for the presence of COX-2 by a Western blot technique. There were 29 patients from whom the samples were obtained, including 8 with enchondromas and 21 with chondrosarcomas. RESULTS: Thirteen of the 24 chondrosarcoma samples and none of the 8 enchondromas were positive for COX-2. An attempt was made to correlate these results with clinical data including age, gender, staging according to the Musculoskeletal Tumor Society, anatomical site, ploidic pattern, presence of metastases and death rate but no statistically valid correlation could be found. CONCLUSION: It is evident that COX-2 may play some role in chondrosarcoma but not in the benign enchondroma and that further studies with COX-2 inhibitors are warranted.

Adult↗

Mesenchymal chondrosarcoma of the ethmoid sinus.

Mesenchymal chondrosarcoma arising from primitive mesenchymal cells is a distinctive type of chondrosarcoma that exists in both skeletal and extraskeletal forms. The tumor is composed of undifferentiated cells with focal areas of cartilaginous or chondroid matrix. An extremely rare case of mesenchymal chondrosarcoma of the anterior and middle ethmoid sinuses in a 16-year-old girl is presented. Magnetic resonance imaging provided more anatomic information about the tumor than computerized tomography and aided in surgical planning.

Adolescent↗

Fine needle aspiration cytology of primary extraskeletal myxoid chondrosarcoma. A case report.

BACKGROUND: Extraskeletal myxoid chondrosarcoma is a rare soft tissue tumor of the extremities. Since it usually lacks obvious chondroid differentiation on light microscopy, it needs to be distinguished from other myxoid soft tissue sarcomas. CASE REPORT: The diagnosis of extraskeletal myxoid chondrosarcoma was made on fine needle aspiration in a patient with a swelling in the right calf. Cellular myxoid fragments having round to oval cells with grooved nuclei arranged in a cordlike pattern suggested chondroid differentiation. The diagnosis was confirmed by histopathology. CONCLUSION: Fine needle aspiration cytology can be diagnostic of extraskeletal myxoid chondrosarcoma even in the absence of obvious chondroid differentiation.

Biopsy, Needle↗

Laryngeal chondrosarcoma: incidence, pathology, biological behavior, and treatment.

Chondrosarcoma is the most common mesenchymal tumor of the larynx and approximately 200 cases have been collected in the world medical literature. It is less aggressive in the larynx than elsewhere: cervical or distant metastases are rare (8.5%), and although local recurrences are not uncommon, they are not catastrophic. A retrospective study was made on eight cases of laryngeal chondrosarcoma. Three cases had originally been diagnosed as idiopathic vocal cord paralysis, possibly because of early involvement of the cricoarytenoid joint or the distal portion of the recurrent nerve. This fact emphasizes the need for accurate laryngeal computed tomography in cases of vocal cord paralysis of unknown origin. Surgical excision is the treatment of choice for laryngeal chondrosarcoma, and conservative techniques may sometimes be appropriate. Supraglottic laryngectomy may be the technique of choice in the rare cases of epiglottic involvement.

Adult↗

Extraskeletal myxoid chondrosarcoma of the head and neck: a case report.

Extraskeletal myxoid chondrosarcomas are rare, soft tissue tumors with a characteristic histologic appearance. The majority of these tumors occur in the extremities. To our knowledge, this is the first reported case in the head and neck. As a group, extraskeletal myxoid chondrosarcomas behave in a less aggressive manner than do other forms of chondrosarcomas, but their clinical course can vary considerably. Surgery is the treatment of choice, and adjuvant radiotherapy or chemotherapy should be considered for cases that display a more cellular pattern and a less conspicuous myxoid matrix.

Chin↗

Cricoid chondrosarcoma coexisting with a thyroid mass: case report and review of the literature.

Laryngeal chondrosarcomas are rare cartilaginous tumors that usually present with hoarseness, dyspnea and, occasionally, dysphagia and dysphonia. We report the case of a low-grade chondrosarcoma of the cricoid cartilage coexisting with a right thyroid nodule, which presented with homolateral vocal fold paralysis. After a precautionary tracheotomy the patient underwent a laryngofissure in which the mass arising from the posterior plate of the cricoid cartilage was entirely enucleated. The histopathological analysis was compatible with a low-grade chondrosarcoma involving the posterior resection margin. For this reason, in agreement with the patient's wishes, a total laryngectomy and simultaneous right hemithyroidectomy were performed. We here discuss the peculiarity of the presentation and the therapeutic dilemma posed by this rare neoplasm.

Chondrosarcoma↗

Falcine chondrosarcoma: case report and literature review.

We present a case of falcine chondrosarcoma in a 58-year-old man. Only a few other examples of chondrosarcomas in this location and a total of approximately 50 intracranial cases have been reported. Because its rarity prevents any group of neurosurgeons from collecting a large experience in managing this tumor, we reviewed descriptions of previous cases to determine the natural history of intracranial chondrosarcomas. The distinguishing features of this rare tumor are compared with previous examples of intracranial tumors derived from cartilage and more common tumors in the parasagittal region, such as meningioma.

Chondrosarcoma↗

Extraskeletal myxoid chondrosarcoma of the falx.

Intracranial cartilaginous tumors are unusual lesions, of which myxoid chondrosarcoma is the rarest. We describe this tumor arising from the falx in a 28-year-old woman treated at recurrence with a second operation and a radiation implant. The behavior of classic chondrosarcoma and mesenchymal chondrosarcoma is also reviewed.

Adult↗

Management of cranial base chondrosarcomas.

OBJECTIVE: Chondrosarcomas are rare, infiltrative, progressive lesions that occur at the cranial base. Their intimate association with cranial nerves and major vessels of the head and neck often precludes complete surgical resection. METHODS: Between 1983 and 2003, 23 patients (14 females, 9 males) were treated at our institution with the diagnosis of chondrosarcoma of the cranial base (mean age at presentation, 43 yr). A retrospective chart review was performed to evaluate presentation, management, and adjunctive treatment. All living patients were contacted for a current examination and disease status. RESULTS: The 23 patients underwent 43 surgical resections. Follow-up ranged from 8 months to 25 years (mean, 97 mo). Ten patients underwent various adjuvant radiation therapies. Five patients have died. Four patients have no evidence of disease, and 13 have residual tumor. One was lost to follow-up. Of 14 patients with 5 years of follow-up, 13 are living. Therefore, the absolute 5 year survival rate is 93%. The 10 year survival rate is 71%. CONCLUSION: Because of the intricate nature of the cranial base, a team approach is preferable for managing these challenging lesions. Maximum cytoreductive surgery should be pursued as an initial strategy to minimize neurological injury. Adjuvant stereotactic radiosurgery can be used to treat residual disease or small recurrences. This cohort also illustrates that patients with chondrosarcomas have better long-term survival rates than patients with chordomas of the cranial base.

Adolescent↗

Patient outcome at long-term follow-up after aggressive microsurgical resection of cranial base chondrosarcomas.

OBJECTIVE: To evaluate patient clinical outcome and survival at long-term follow-up after aggressive microsurgical resection of chondrosarcomas of the cranial base. METHODS: Over a 20-year period, 47 patients underwent 72 operative procedures for resection of cranial base chondrosarcomas. Thirty-three patients were previously untreated, whereas 14 patients previously had undergone surgery or radiation. Twenty-three patients had a single operation and 24 underwent staged (more than one) operations because of extensive disease. Patients who underwent subtotal resection also underwent radiotherapy or radiosurgery. Patients were evaluated at follow-up clinically and by imaging studies. RESULTS: Gross total resection was accomplished in 29 (61.7%) patients, and subtotal resection was accomplished in 18 patients (38.3%). The resection was better in patients who underwent a primary operation (gross total resection, 68.8 versus 46.7%) rather than a reoperation. Patients who underwent incomplete resection underwent postoperative radiotherapy, which included proton beam radiotherapy (15.6%), radiosurgery (68%), and fractionated radiation (15.6%). There were no operative deaths. Postoperative complications (cerebrospinal fluid leakage, quadriparesis, infections, cranial nerve palsies, etc.) were observed in 10 patients (18%). The follow-up ranged from 2 to 255 months, with an average of 86 months. At the conclusion of study, 36 (76.6%) patients were alive, and 21 (44.7%) patients were alive without disease. Recurrence-free survival was 32% at 10 years in all patients, 42.3% in primary patients and 13.8% in those who underwent reoperation. The Karnofsky performance score was 82.4 +/- 9.8 before surgery, 85 +/- 12.5 at 1 year after surgery, and 85.3 +/- 5.8 at the latest follow-up. Two patients died as a result of radiotherapy complications (malignancy, radiation necrosis). CONCLUSION: Cranial base chondrosarcomas can be managed well by complete surgical resection or by a combination of surgery and radiotherapy. The study cannot comment about the efficacy of radiotherapy. Approximately half of the patients survived without recurrence at long-term follow-up (>132 mo). The functional status of the surviving patients was excellent at follow-up.

Adolescent↗

Chondrosarcoma of the larynx.

Cartilaginous tumors of the larynx are rare, and of those reported, most are chondromas. To date, approximately 157 cartilaginous tumors of the larynx have been reported, but only 37 have been chondrosarcomas. A review of the records of the past 25 years (1948-1974) of the Johns Hopkins University Hospital and the Greater Baltimore Medical Center was made and revealed only two previous cartilaginous tumors, both chondromas. A case of an exceptionally large chondrosarcoma of the larynx which appeared as a neck mass invading the thyroid cartilage in a patient with a six-month history of hoarseness is presented. A complete review of the literature was made which shows that most chondrosarcomas occur in middle-aged males originating most often from the posterior cricoid lamina, next from the thyroid cartilage. Misdiagnosis has been a problem with low-grade tumors, and although wide local excision has been stated as being the treatment of choice, the authors emphasize the need for more aggressive surgery because of the high recurrence rate discovered in the literature.

Age Factors↗

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.

Adolescent↗

Juxtacortical chondrosarcoma.

The features are described of seven cases of "juxtacortical" chondrosarcoma, the term introduced by Jaffe for a rare but distinct entity and now accepted in the World Health Organisation classification as preferable to the term "periosteal" chondrosarcoma. In all cases the lesion involved the shaft of a long bone, most often the femur, and in two cases two different long bones were affected. Six of the seven patients were male and all were in the second decade. The characteristic appearance was that of a small tumour adjacent to the cortex with areas of spotty calcification often accompanied by radiating bone spicules perpendicular to the bone shaft and a typical Codman's triangle. Histologically all the tumours showed a cartilaginous lobular pattern, well limited on the surface and seldom infiltrating the cortex; areas of spotty calcification and enchondral ossification were often present but tumour osteoid and bone were conspicuously absent. Despite the ominous histological aspect, the prognosis proved to be relatively favourable compared with the usual central chondrosarcoma of a similar grade of malignancy.

Adolescent↗

A somatostatin analogue inhibits chondrosarcoma and insulinoma tumour growth.

The in vivo effects on tumour growth of a potent somatostatin analogue, SMS 201-995 [H-(D)Phe-Cys-Phe-(D)Trp-Lys-Thr-Cys-Thr-(ol)], were measured in two characterised transplantable tumours: a) the Swarm rat chondrosarcoma, known to be insulin-, growth hormone (GH)-, somatomedin- and corticosteroid-dependent, b) a hamster insulinoma, bearing specific high affinity somatostatin receptors. SMS 201-995 (1.25 mg/kg/day) given for 25 days to rats bearing freshly transplanted chondrosarcomas inhibited tumour volume by 48%. A significant tumour growth inhibition was measured also in well developed tumours treated with high doses of SMS 201-995 (1.25mg/kg/day) for 7 days. In the treated animals, GH was significantly inhibited. In hamsters bearing a freshly transplanted insulinoma, the daily application of SMS 201-995 (200 micrograms/kg/day, sc) for 33 days could significantly inhibit the growth (as measured by tumour volume) of the tumour. A moderate inhibitory effect of SMS 201-995 on the growth of well grown insulinomas could also be observed. This study shows that SMS 201-995 under the present experimental conditions has a moderate but significant growth inhibitory effect in two different transplantable tumour models. In the rat chondrosarcoma, the effect of SMS 201-995 is probably indirect, due to inhibition of GH, somatomedin and insulin. In the hamster insulinoma, the effect is possibly due to a more direct action of SMS 201-995 on specific somatostatin receptors present in this tumour.

Adenoma, Islet Cell↗

[Osteosarcoma and chondrosarcoma--radiographic differentiation with computerized tomography].

The purpose of this paper was to associate and compare the radiographic patterns of osteosarcoma and those of chondrosarcoma by means of computed tomography (CT). Computed tomographs of five cases of osteosarcoma and five cases of chondrosarcoma of the head and neck region were analyzed. The sensitivity of CT was calculated based on the results of histopathological examinations. We concluded that CT can facilitate the establishment of the final diagnosis since it is possible to distinguish the individual characteristics of osteosarcomas and chondrosarcomas in that exam.

Bone Neoplasms↗

Relationship between surgical procedure and outcome for patients with grade I chondrosarcomas.

PURPOSE: To evaluate the oncological outcome of patients with grade I chondrosarcomas according to the type of surgical treatment performed, since there is still controversy regarding the need for aggressive resections to reach a successful outcome. MATERIALS AND METHODS: The records of 23 patients with grade I chondrosarcomas were reviewed. The mean age was 38.4 years, ranging from 11 to 70 years; 52% were men and 48% were women. The femur was the site of 13 tumors. The tumors were staged as IA (17, 74%) and IB (6, 26%). Regarding tumor location, 74% (17) were medullary, 22% (5) were peripheral, and 4% (1) was indeterminate. Tumor size ranged from 2 to 25 cm, mean 7.9 cm. Regarding the surgical procedure, 11 patients underwent intralesional resection, 9 patients underwent wide resection, and 3 underwent radical resection. The follow-up period ranged from 24 to 192 months. RESULTS: None of the patients developed local recurrence or metastases; 7 patients had other general complications. CONCLUSIONS: This data supports the use of less aggressive procedures for treatment of low-grade chondrosarcomas.

Adolescent↗