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A rare case of chondrosarcoma at the skull base is presented. Computerized tomography scan was very helpful in delineating the exact extent of the tumor. Chondrosarcoma is a slow growing malignant tumor which metastasizes very late. Usually it is radioresistant, but when surgery is not feasible, irradiation treatment is worth a trial.
The incidence, clinical findings, location, etiology, clinical diagnosis, and surgical treatment of chondrosarcoma of the larynx are discussed. Important features in the diagnosis and treatment of a chondrosarcomatous tumor of the larynx are demonstrated in a case report. The patient's chief complaint was episodic shortness of breath occurring with increasing frequency over a period of 12 months. Her dyspnea progressed in severity until an emergency tracheotomy was required for airway control. CT scan documented the attachment of the tumor to the right posterior lateral wall of the cricoid cartilage. Direct laryngoscopy and biopsy were done to obtain tissue for diagnosis. The histopathological examination revealed low-grade chondrosarcoma. The tumor was removed totally with a wide-margin mucous membrane incision and careful curettement. A definite difference in the consistency of the tumor compared to normal cartilage allowed complete excision of the tumor with minimal sacrifice of healthy cricoid cartilage. This presentation emphasizes the conservative surgical removal of these tumors and notes how a curette can be used for this removal.
Chondrosarcoma of the skull base is an uncommon neoplasm that comprises 0.15% of all intracranial tumors and 6% of skull base lesions. Compression of vital neurologic structures by tumor expansion as well as the histologic characteristics contribute to the malignant potential of these neoplasms. Tumor recurrence has been associated with incomplete resection in this traditionally surgically inaccessible region. This review of five cases of chondrosarcoma of the skull base examines the surgical approaches to resection in a chronologic fashion to emphasize that total gross resection of these lesions has become possible through the evolution of lateral skull base techniques. The difficulty in pathologic diagnosis and the efficacy of postoperative radiotherapy in enhancing survival are also addressed.
Chondrosarcoma of the larynx is a rare tumor; worldwide only about 250-300 cases have been described in the literature. We present a clinical case of laryngeal chondrosarcoma manifesting as a swollen mass of 10 x 7 x 6 cm in the infrahyoid and left lateral cervical region. The patient underwent total laryngectomy, thyroidectomy and bilateral neck dissection. A review of the literature on this disease is also reported.
The authors report a rare case of primary chondrosarcoma of the anterior mediastinum showing unusual pathological and clinical features, namely 1) the lack of any anatomical relationship between the tumor and cartilage-containing organs, and 2) an indolent behavior with long-term survival. In spite of early disease recurrence and repeated surgery, the patient is in good health five years after primary surgery. The reported case suggests that 1) primary chondrosarcomas of the anterior mediastinum may have a better prognosis than previously recognized, 2) the disease can remain confined within the chest for as long as five years, and 3) repeated surgery may contribute to long-term survival.
A cardiac chondrosarcoma was found in the right atrium of a Golden Retriever dog. Macroscopically, the right atrial lumen was filled with a 6- x 12- x 8-mm white glossy mass, which was diffusely attached to the underlying myocardium. The mass was composed of spindle-shaped mesenchymal neoplastic cells loosely packed in light basophilic matrix, with focal areas of tightly packed cells in linear formation similar to the pattern of a growth plate. Tumor cells were positive when stained for vimentin and neuron-specific enolase, and weakly positive for S-100 protein. Ultrastructurally, neoplastic cells and abundant, dilate rough endoplasmic reticulum. Golgi apparatus, bundles of intermediate fibers, and primitive intercellular junctions between adjacent tumor cells. Based on morphologic, ultrastructural, histochemical, and immunohistochemical characteristics, this tumor was diagnosed as a chondrosarcoma.
A young adult female Mastiff dog developed a large retroperitoneal mass, pleural effusion, and multiple pulmonary and pleural nodules. All masses were diagnosed as mesenchymal subtype chondrosarcomas, using histological and immunohistochemical criteria. Reports of canine extraskeletal mesenchymal chondrosarcomas (EMCs) are rare but involved animals less than 3 years of age in 60% of the cases. This is the first description of this type of tumor developing distant metastases. Evidence from this case and previous reports suggests that EMCs are associated with a poor prognosis.
We report the case of a well-differentiated chondrosarcoma of the proximal humerus in a 60-year-old man that featured a prominent component of granular cells, the granules being shown by electron microscopy to be lysosomal. Although secondary granular cell changes have been described in a large variety of tumor types, this is, to the best of our knowledge, the first documentation of this phenomenon in a skeletal chondrosarcoma.
Primary chondrosarcoma originating from the bronchus is very rare. We describe the case of a 71 year old man with a large chondrosarcoma originating from the left lower lobe bronchus. The tumour was resected and, in contrast to most patients with such tumours, this patient is well, without complaints or abnormalities on X-ray or computed tomographic (CT) scan, 3 yrs after surgery. Tumour characteristics, symptoms, diagnosis and treatment are discussed. Radical surgical resection is the treatment of choice.
A 52 year old man developed an extraskeletal mesenchymal chondrosarcoma (ESMC) arising from the pleura. Clinically, the tumour mimicked a mesothelioma. Fine needle biopsy was consistent with the diagnosis of sarcomatoid mesothelioma. Histological examination of multiple tumour samples, supported by immunohistochemical characterization, made it possible to correctly diagnose extraskeletal mesenchymal chondrosarcoma.
A case of extraskeletal mesenchymal chondrosarcoma in a 62-year-old woman is reported. This unusual sarcoma was localized in the right thigh (quadriceps) and treated with a combination of surgery, chemotherapy and radiotherapy. Sixty-three cases were found in the literature, with neurologic localization in 29 cases and muscular localization in 34 cases. Among clinical features, a significant difference in age at the time of diagnosis was found between the two localizations, which strongly suggests the possibility of two different types of extraskeletal mesenchymal chondrosarcoma.
A 37-yr-old woman with clinical, endocrinological, and radiological features suggestive of a nonfunctioning pituitary tumor was found to have a chondrosarcoma of the pituitary sella. The bony structures around the sella were relatively uninvolved, other than showing minor erosion of the left side of the dorsum and the posterior wall of the sphenoid sinus. After partial resection of the tumor by the transsphenoidal route the patient received postoperative radiosurgery by a linear accelerator, stereotactic multiarc radiotherapy. Subsequent follow-up revealed reduction of the residual tumor. This case demonstrates that a chondrosarcoma may apparently arise directly from the pituitary fossa and suggests the efficacy of stereotactic radiosurgery, at least in the medium term. The origin, areas of involvement, management, and long-term prognosis of these rare tumors are reviewed.
Although chrondrosarcomas are common primary bone tumors, their occurrence in the spine is very rare. Because total excision in such cases is seldom possible, few long term survivors have been reported. We are reporting one patient with a high thoracic spinal chondrosarcoma who survived with useful function for over 18 years because of repeated local tumor excisions. The literature is reviewed, and an aggressive surgical treatment of spinal chondrosarcoma is recommended.
Chondrosarcomas of the skull base are indolent, locally invasive tumors with a marked tendency to recur. Surgery is the mainstay of treatment because these tumors are generally resistant to other forms of treatment. A surgical approach with wide access to the skull base and one that is easily repeatable is required, because recurrence is common. We have used the LeFort I maxillotomy or mobilization of the zygoma at the time of craniotomy to obtain wide access to the skull base in eight cases of chondrosarcoma. Three patients have undergone subsequent procedures by us for recurrent disease. One patient died 30 days after the operation, and one has required an open repair of a cerebrospinal fluid leak. Good palliation of symptoms has been achieved in all survivors. These approaches fulfill the criteria for the surgical management of these difficult tumors by allowing excellent exposure, safe repetition if required, satisfactory palliation, and acceptable morbidity.
Tenascin-C is an oligomeric glycoprotein of the extracellular matrix that has been found to have both adhesive and anti-adhesive properties for cells. Recent elucidation of the two major TNC splice variants (320 kDa and 220 kDa) has shed light on the possibility of varying functions of the molecule based on its splicing pattern. Tenascin-C is prominently expressed in embryogenesis and in pathologic conditions such as tumorogenesis and wound healing. Fibronectin is a prominent adhesive molecule of the extracellular matrix that is often co-localized with tenascin-C in these processes. We studied the chondrosarcoma cell line JJ012 with enzyme-linked immunoabsorbance assays, cell attachment assays and antibody-blocking assays to determine the adhesive/anti-adhesive properties of the two major tenascin-C splice variants with respect to fibronectin and their effect on chondrosarcoma cell attachment. We found that the small tenascin-C splice variant (220 kDa) binds to fibronectin, whereas the large tenascin-C splice variant (320 kDa) does not. In addition, the small tenascin-C splice variant was found to decrease adhesion for cells when bound to fibronectin, but contributed to adhesion when bound to plastic in fibronectin-coated wells. Antibody blocking experiments confirmed that both the small tenascin-C splice variant and fibronectin contribute to cell adhesion when bound to plastic. The large tenascin-C splice variant did not promote specific cell attachment. We hypothesize that the biologic activity of tenascin-C is dependent on the tissue-specific splicing pattern. The smaller tenascin-C isoform likely plays a structural and adhesive role, whereas the larger isoform, preferentially expressed in malignant tissue, likely plays a role in cell egress and metastasis.
We report a case of chondrosarcoma of the heart that was managed surgically. As chondrosarcoma of cardiac origin is extremely rare, this case is described with a brief comment.
We investigated the antitumor effects and tissue distribution of adriamycin (ADR) incorporated into a hydroxyapatite (HAP) bead in a cancer rat model bearing Swarm rat chondrosarcoma. The Porous HAP bead (8.48 mm in diameter, 531 +/- 0.7 mg in weight) was used as a model bone graft. One ADR-HAP bead (ADR 0.4 mg-6.0 mg/bead) was implanted s.c. into a Sprague-Dawley rat at 6 days postinoculation of Swarm rat chondrosarcoma. ADR-HAP beads showed strong antitumor activities in a dose dependent manner. The dose of 6.0 mg/bead showed the highest efficacy with no toxic death: It caused a 98% growth inhibition on Day 31 postinoculation and a survival advantage of a 339% increase in life span. After the implantation of the ADR-HAP bead (0.4 mg/bead/body) and the i.v. administration of an equal dose of free adriamycin, we determined the tissue distribution of ADR for up to 90 days. ADR-HAP bead implanted in the tumors released ADR over a 12-week period in the target area. The diffusion of the drug to other organs such as the heart and liver was very low compared with the tumors. The area under the ADR concentration-time curve (AUC) of the tumors was 181.6 micrograms.day/g and 5.22 micrograms.day/g after the implantation of the ADR-HAP bead and the i.v. administration of free ADR, respectively. The targeting index of the tumors, defined as the ratio of the AUC after the implantation of the ADR-HAP bead to that after administration of free ADR, was 34.8.(ABSTRACT TRUNCATED AT 250 WORDS)
Mesenchymal chondrosarcoma (MC) is a rare tumour, with a predilection for the head and neck region. We describe a case of mesenchymal chondrosarcoma arising in the right maxilla extending to the basi-sphenoid. Its computed tomography (CT) and magnetic resonance imaging (MRI) and histopathological features and the management are presented. We also reviewed the literature of reported cases involving the maxilla.