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PubMedDate not supplied

Chondrosarcoma of the sternum: CT features.

Eleven sternal chondrosarcomas were reviewed with radiologic emphasis on the CT findings. Typical clinical presentation was a large painless anterior chest wall mass in an elderly individual. In six cases the chest radiograph strongly suggested an underlying cartilaginous neoplasm owing to the appearance of typical mineralized cartilage matrix, i.e., a pattern of stippled, flocculent, or curvilinear ("rings and arcs") calcifications. Calcifications were detected on CT in all 11 patients. Excepting the three smallest lesions, averaging 4 cm in diameter, on CT the tumors exhibited a scalloped or lobulated contour, also typical for cartilaginous neoplasms. Six tumors were centered in the sternal body, four in the xyphoid process, and one in the manubrium. In addition, CT was especially important in localizing the lesion when the tumor was either small in size or centered in the xyphoid process (where all four chondrosarcomas grew in an inferior direction). Because of its ability to detect subtle matrix mineralization and delineate the extent and probable origin of the tumor, CT strongly favored the preoperative diagnosis of sternal chondrosarcoma and was also helpful in surgical management.

Adult↗
PubMedDate not supplied

MR appearance of periosteal chondrosarcoma of the foot.

OBJECTIVE: To assess the role of MRI in evaluating periosteal chondrosarcoma in the foot, an anatomically complicated location. MATERIALS AND METHODS: Three cases of surgically and histologically confirmed periosteal chondrosarcomas occurring in the foot (58-year-old woman, 57-year-old woman, and 63-year-old man) were retrospectively reviewed with an emphasis on MR findings. RESULTS: Magnetic resonance successfully delineated the hypovascular nature and lobulating pattern of the lesion suggesting the cartilaginous component, the periosteal location with sparing bone marrow, and the extent of involvement of adjacent structures. These findings were either not seen or less clearly seen on CT in the former two cases. CONCLUSION: In cases of periosteal chondrosarcomas in the foot MRI can play a significant role both in the characterization of the lesion and in planning surgical therapy.

Amputation, Surgical↗
PubMed2003

Mesenchymal chondrosarcoma of the sinonasal tract: a clinicopathological study of 13 cases with a review of the literature.

OBJECTIVES/HYPOTHESIS: Mesenchymal chondrosarcoma of the sinonasal tract is a rare, malignant tumor of extraskeletal origin. Isolated cases have been reported in the English literature, with no large series evaluating the clinicopathological aspects of these tumors. STUDY DESIGN: Retrospective review. METHODS: Thirteen patients with sinonasal mesenchymal chondrosarcoma were retrieved from the Otorhinolaryngologic-Head and Neck Registry of the Armed Forces Institute of Pathology. RESULTS: Nine women and 4 men (age range, 11 to 83 y; mean age, 38.8 y) presented with nasal obstruction (n = 8), epistaxis (n = 7), or mass effect (n = 4), or a combination of these. No patients reported prior head and neck irradiation. The maxillary sinus was the most common site of involvement (n = 9), followed by the ethmoid sinuses (n = 7) and the nasal cavity (n = 5). Tumors had an overall mean size of 5.1 cm. Microscopically, the tumors displayed a small, blue, round cell morphology appearance arranged in a hemangiopericytoma-like pattern with foci of cartilaginous matrix. All cases were managed by surgery with adjuvant radiation therapy (n = 4) and/or chemotherapy (n = 3). The overall mean survival was 12.1 years, although five of six patients who developed local recurrences died of disease (mean survival, 6.5 y). Six patients were alive and disease free (mean survival, 17.3 y), and two patients were lost to follow-up. CONCLUSIONS: Mesenchymal chondrosarcoma of the sinonasal tract is an aggressive tumor with a predilection for young women. The pattern of growth and scarcity of cartilaginous matrix result in frequent misdiagnosis. Recurrence develops in approximately one-third of patients and seems to predict a poor prognosis. Aggressive, exenterative surgery combined with adjuvant therapy appears to yield the best clinical outcome.

Adolescent↗
PubMed1983

High-energy irradiation in the management of chondrosarcoma.

We present a retrospective analysis of seven patients with chondrosarcoma of the bone treated by high-energy irradiation between 1961 and 1976. Its major role in this series was prevention of local recurrence in cases with inadequate resection. In three of the five cases in which radiation therapy was adjuvant rather than primary treatment, long-term local control was obtained in a dose of 5,000 to 6,500 rads in five to six weeks. Although primary treatment of chondrosarcoma is surgical, high-dose radiation therapy is indicated when surgical resection is not possible. Chondrosarcoma can respond to high doses of irradiation even though the response is slow.

Adult↗
PubMed2006

Cochlear implantation with ipsilateral petroclival chondrosarcoma.

OBJECTIVE: To highlight a case of cochlear implantation in the setting of ipsilateral petrous apex chondrosarcoma. BACKGROUND: A patient with bilateral progressive hearing loss was incidentally found to have a destructive right petrous apex lesion on computed tomography before cochlear implantation. The patient had no associated symptoms and a magnetic resonance imaging scan was obtained, narrowing the differential diagnosis. A middle cranial fossa approach was performed for synchronous biopsy of the lesion and cochlear implantation. RESULTS: Frozen sections revealed a low-grade chondroid lesion, and a Med-El Combi 40+ cochlear implant with a split electrode array was inserted via the middle fossa. Final pathologic examination revealed a Grade I chondrosarcoma. The patient suffered no complications postoperatively and was followed-up over 5 years with serial computed tomographic scans and clinical examinations. No additional treatment was administered. Eighteen months postoperatively, the patient experienced episodic vertigo. There were no new findings on computed tomography, and the vertigo improved with a low-salt diet. Otherwise, the patient had excellent hearing results, and the lesion has not progressed under observation. CONCLUSION: The implications of observing low-grade chondrosarcomas in well-selected patients and the unique aspect of cochlear implantation on the affected side are discussed.

Acoustic Stimulation↗
PubMed2006

Influence of cryosurgery on treatment outcome of low-grade chondrosarcoma.

Successfully managing low-grade chondrosarcomas with margins considered less than wide would minimize the need for extensive reconstruction. We report our experience using cryotherapy as an adjuvant to treat patients with low-grade intracompartmental chondrosarcomas. Ten consecutive patients had intralesional resections including curettage, cryo-surgery, and polymethylmethacrylate application. Eight of these patients required prophylactic skeletal stabilization. We retrospectively reviewed the outcomes for tumor recurrence, disease progression, and complications. The Musculo-skeletal Tumor Society rating scale was used to evaluate functional outcome, and the mean score was 27 points (range, 25-30 points). The mean age of the patients was 54.4 years (range, 29-83 years), and the average followup was 38.5 months (range, 24-60 months). Patients were treated for lesions of the femur (n = 3), humerus (n = 3), scapula (n = 2), tibia (n = 1), and acetabulum (n = 1). There was no evidence of recurrence or metastases. At the latest followup, all patients were well, however, one patient had hardware loosening. In this small group of patients, intralesional resection with adjuvant cryoablation provided an alternative to more radical procedures for low-grade intracompartmental chondrosarcoma.

Adult↗
PubMed2005

Skull base chondrosarcoma originating from the petroclival junction.

OBJECTIVE: To define the presentation of patients with skull base chondrosarcoma, to elucidate surgical strategies, and to identify the role of postoperative radiotherapy. STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: All patients (n = 33) with skull base chondrosarcoma managed at our institution. The average follow-up time was 7.7 years (range, 0-20 years). MAIN OUTCOME MEASURES: Tumor location, presenting symptoms, presence of residual or recurrent tumor, and mortality. RESULTS: The most common tumor location was the petroclival junction (n = 29). Common presenting symptoms were diplopia (48%) and headache (45%). Surgical approaches included retrosigmoid, transtemporal, transfacial, and frontotemporal craniotomies. Biopsy only was performed in four patients, subtotal resection in 19 patients, and total resection in nine patients. Most patients received postoperative radiotherapy (82%). Follow-up revealed residual, stable disease in 28% of patients and recurrent disease in 24% of patients. The mean time to recurrence was 3.0 +/- 2.8 years. The lack of postoperative radiation was significantly correlated with an increased risk of recurrence (odds ratio, 28; p = 0.007) but incomplete tumor resection was not (p = 0.6). Life-table analysis revealed that the 5-year survival rate was 85% and the 10-year survival rate was 77%. Five patients died; four of the deaths attributable to recurrent disease. CONCLUSION: The characteristic growth pattern of skull base chondrosarcoma is tumor eroding the petroclival junction. Current therapeutic strategy is resection through an extradural subtemporal craniotomy with removal of the petrous apex and clivus. Radical resection of uninvolved structures is often not necessary. Nonetheless, gross total removal is often achievable. Postoperative radiotherapy reduces the chance of tumor recurrence.

Adult↗
PubMed2006

Chondrosarcoma of the skull base: long-term follow-up.

OBJECTIVE: Chondrosarcoma of the skull base is an uncommon neoplasm comprising 0.15% of all intracranial tumors and 6% of skull base neoplasms. The outcome of treatment is difficult to assess because the slow growth rate means that there is a long interval before detecting the recurrence. We describe the use of lateral skull base techniques for these lesions and examine the long-term outcomes. The pathological features, radiological findings, and radiotherapy options are also discussed. STUDY DESIGN: Retrospective case review with current follow-up where possible. SETTING: Tertiary referral neurotologic private practice. PATIENTS: Eight patients with histologically confirmed skull base chondrosarcoma operated on since 1979. At the time of the surgery, the five women and three men ranged in age from 31 to 63 years, with a mean of 42.9 years. The follow-up ranged from 9 months to 25 years and 5 months. INTERVENTIONS: All patients underwent surgical removal; the earliest patient underwent surgical removal via transcochlear and retrosigmoid approaches, and seven via an infratemporal fossa approach, with or without exenteration of the otic capsule. MAIN OUTCOME MEASURES: Number of patients with residual and recurrent tumors. RESULTS: In the patient who underwent surgery via the transcochlear approach, recurrence was noted within 3 months, and new exploration using the retrosigmoid approach was performed for the removal of the recurrent lesion. This patient showed no signs of recurrence for 25 years since last surgery. Of the seven patients who underwent surgery via the infratemporal fossa approach, one had residual tumor found at 1 year, but remained alive and well for 21 years after the surgery. The other six had gross total resection and showed no signs of recurrence at the last known follow-up. One patient died as a result of an unrelated cause 18 years after surgery for the chondrosarcoma. CONCLUSION: The gross total resection of these lesions is possible because of the evolution of lateral skull base techniques and can be routinely accomplished with the infratemporal fossa approach. The improved exposure afforded by this approach seems to have resulted in more complete extirpation of the tumor and a decrease in the recurrence rate.

Adult↗
PubMed1981

Primary chondrosarcoma of the ovary.

A case of a large, completely excised left-sided primary ovarian chondrosarcoma occurring in a 61-year-old woman is described. There was no evidence of metastases. Histologically the tumour was a pure chondrosarcoma, which was mainly well differentiated. The patient is well and disease free at 4 years. Review of the literature revealed no other reported cases of pure chondrosarcoma of the ovary. The possible histogenetic origin of this neoplasm is discussed.

Chondrosarcoma↗
PubMed1985

Non-conventional chondrosarcomas and chondrogenesis.

Pattern formation in chondroid development is compared with growth models exhibited by mesenchymal and myxoid chondrosarcomas. The results reveal strong similarities between mesenchymal chondrosarcoma and normal chondrogenesis. The fine vascularization of the tumor lobules of myxoid chondrosarcoma is discussed in relation to chondroid differentiation. A common histogenetic approach to the interpretation and classification of these neoplasias is proposed.

Animals↗
PubMed1988

Dedifferentiated chondrosarcoma with muscle and cytokeratin differentiation in the anaplastic component.

Dedifferentiated chondrosarcoma is a biphasic tumour, comprising well-differentiated chondrosarcoma and an anaplastic non-cartilaginous sarcoma juxtaposed but distinct from each other. Two cases of dedifferentiated chondrosarcoma, one primary and one recurrent, demonstrated muscle differentiation when studied with monoclonal antibodies to muscle specific actin, desmin and myoglobin. One of the tumours was also positive for cytokeratin, identified by AE1/AE3 and CAM 5.2 antibodies. Our findings are consistent with the concept that these tumours are capable of diverse patterns of morphological and immunophenotypic differentiation.

Adult↗
PubMed2005

Extraskeletal myxoid chondrosarcoma: updated clinicopathological and molecular genetic characteristics.

Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft-tissue sarcoma characterized by distinctive morphological and cytogenetical features. As its name implies, EMC was believed to represent a variant of soft-tissue chondrosarcoma owing to its histological resemblance to chondroblastic tissue in the early stages of cartilage development or chondroid tumors such as skeletal chondrosarcoma. However, the chondroid nature has been a subject of controversy, and its line of differentiation remains to be determined. Consequently, the tumor is provisionally classified into a group of tumors of uncertain differentiation in the revised World Health Organization classification of tumors of soft tissue and bone. Moreover, immunohistochemical and ultrastructural features of neural or neuroendocrine differentiation have been recently reported in a subset of EMC, providing a new insight into their histogenetic nature. Chromosomal rearrangements involving 9q22, such as t(9;22)(q22;q12), and resultant NR4A3 fusion genes are tumor-type specific or pathognomotic for this entity and are assumed to play an important role in the development of EMC. Although the biological mechanisms and functions are largely unknown, the NR4A3-related pathway is considered a potential molecular target for future therapeutic intervention. Because of its protracted but resilient nature, a tenacious and long-term follow up is necessary for any patient.

Chondrosarcoma↗
PubMed1986

Cutaneous metastases from chondrosarcoma.

This report describes a patient with histologically confirmed multiple cutaneous metastases from a skeletal chondrosarcoma and reviews the two previously published cases of this very unusual pattern of metastasis. We conclude that metastatic chondrosarcoma should be considered in the differential diagnosis of multiple cartilaginous tumors of the skin, and that radiotherapy may play a role in the management of metastatic chondrosarcoma.

Bone Neoplasms↗
PubMed1990

An unusual cutaneous metastasis of a chondrosarcoma.

We report a case of cutaneous metastases from a scapular chondrosarcoma in a 85-year-old man. The metastatic skin lesion was noted nine years after resection of the primary tumor. Metastases to lung and soft tissues had occurred four years earlier. The patient also had a history of malignant melanoma. Although this case illustrates an unusual pattern of metastases, we conclude that the appearance of cutaneous lesions in a patient with a prior history of chondrosarcoma should alert the clinician to the possibility of a metastatic chondrosarcoma.

Aged↗
PubMed1988

Chondrosarcoma. I. A selected literature review.

More than 200 articles and texts relating to chondrosarcoma in the general skeleton, in the head and neck area, and in extraskeletal soft tissues, were reviewed and summarized. In the interest of space conservation and avoidance, to the extent possible, of distracting referential interjections in the text, the entire list of materials reviewed has not been referenced. For example, articles that contribute essentially nothing more than single case reports are not included in the list of references. Effort has been made, however, to include articles of historical significance, articles reporting series of cases, and articles exploring ideas and concepts. The authors believe this review to be a comprehensive summary of the chondrosarcoma literature. An analysis of 37 cases of chondrosarcoma of the jaws will be published as Part II.

Bone Neoplasms↗
PubMed2005

Chondrosarcoma of the proximal humerus in a cat.

Chondrosarcoma of the proximal humerus was diagnosed in a four-year-old, castrated male, domestic shorthair cat that was presented with a slowly growing solid mass in the region of the proximal humerus. Forequarter amputation was advised, but declined by the owners. Following surgical debulking clinical signs resolved, but two months after surgery the cat was readmitted because the mass had recurred in the same region. The forequarter was amputated. Histopathological evaluation of the tumour confirmed the diagnosis of chondrosarcoma. Follow-up examinations performed over a period of 15 months from initial presentation revealed that the cat was doing well and had no signs of metastatic disease. To the authors' knowledge, this is the first reported case of chondrosarcoma of the proximal humerus in a cat to be diagnosed and surgically treated. This case demonstrates that animals with such tumours may recover well after complete excision.

Amputation, Surgical↗
PubMed1984

Benign exostoses and exostotic chondrosarcomas: evaluation of cartilage thickness by CT.

Computed tomograms of 16 benign exostoses and 15 exostotic chondrosarcomas were generally accurate in delineating anatomy for purposes of planning surgery, but they were inaccurate in the detection and measurement of the cartilage caps of the lesions. CT studies of 14 of the benign exostoses failed to show any cartilage cap, although the maximum cartilage thickness of these 14 lesions ranged from 0.1 to 2.5 cm pathologically. CT did demonstrate thick cartilage in 14 of the 15 chondrosarcomas, but the CT measurements of maximum thickness often were imprecise. Altogether, 15 CT studies failed to show any cartilage cap; 14 of these lesions were benign. CT did not reliably detect cartilage caps less than 2.5 cm in maximum thickness, and CT measurement of cartilage thickness was imprecise when the cartilage was 1.5 to 2.5 cm thick. For this reason, CT did not solve the difficult problem of distinguishing between benign exostoses with relatively thick cartilage caps and exostotic chondrosarcomas with relatively thin cartilage.

Adolescent↗
PubMed1986

Dedifferentiated chondrosarcoma: radiographic features.

Dedifferentiated chondrosarcomas, unlike ordinary chondrosarcomas, are highly aggressive tumors that frequently metastasize. The lesion is characterized by a lytic intraosseous or extraosseous soft-tissue mass that is devoid of the calcifications that are usually associated with a chondroid tumor. We describe the radiographic and pathologic features of five cases involving dedifferentiated chondrosarcoma.

Acetabulum↗