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PubMed2002

Multiple basaloid follicular hamartomas associated with acrochordons, seborrhoeic keratoses and chondrosarcoma.

Basaloid follicular hamartoma is an uncommon neoplasm with distinctive histopathological findings. It presents as four distinctive clinical forms: a solitary papule, a localized plaque of alopecia, a localized linear and unilateral type, and generalized papules with associated alopecia and myasthenia gravis. Histologically, basaloid follicular hamartomas are characterized by thin branching strands and thick cords of basaloid or squamoid cells extending from a follicle into a loose, fibrillar, fibrocytic or mucinous connective tissue stroma. We report a case of long-standing, generalized basaloid follicular hamartomas associated with acrochordons, seborrhoeic keratoses, and a history of chondrosarcoma. In general, solitary tumours are sporadic; multiple tumours are inherited and frequently associated with a syndrome. Further surveillance is warranted to determine if the association of multiple basaloid follicular hamartomas and chondrosarcoma constitutes an inherited syndrome.

Acrodermatitis↗
PubMed1992

Chondrosarcoma of the skull base: report of six cases.

Five patients with chondrosarcoma, grade I or II, and one with mesenchymal chondrosarcoma of the skull base were treated at our clinic between 1967 and 1991. The patients were three men and three women who ranged in age from 21 to 39 years. The presenting symptoms were diplopia, decreased visual acuity, or symptoms of involvement of VIIIth and lower cranial nerves. The duration of the symptoms ranged from 18 months to 14 years. A plain skull x-ray and tomography of the skull, computed tomographic (CT) scans, and magnetic resonance images (MRI) revealed a calcified mass. Angiograms showed an occlusion of the arteries or venous sinuses, or both, in four patients, and a distinct tumor stain in one. The tumors were subtotally removed in all six cases. Two patients received postoperative irradiation for the residual tumor. In one patient, extraocular movement was compromised postoperatively, although in the other five patients there was no aggravation of the neurologic signs. Follow-up CT scans and MRI demonstrated no recurrent tumors nor any enlargement of residual tumors. Based on these findings, we advocate aggressive surgical removal of these tumors, which should be augmented by radiation therapy if the postoperative CT scans or MRI reveal residual tumors.

Journal Article↗
PubMed2001

Mesenchymal chondrosarcoma of the orbit: a clinicopathological study.

Extraskeletal mesenchymal chondrosarcoma of the orbit is a rare tumour and generally presents in young females. We report the clinical presentation and histopathological features of a case of orbital mesenchymal chondrosarcoma in a middle-aged man. The prognosis of this tumour is poor and though rare it should be kept in the differential diagnosis of orbital masses.

Journal Article↗
PubMed1981

The use of computed tomography to distinguish osteochondroma and chondrosarcoma.

Computed tomography (CT) was performed prior to surgery on six patients with osteochondroma and six with chondrosarcoma. Criteria for distinguishing these two types of cartilage tumors were developed from classical radiological descriptions and known pathological characteristics. Application of these criteria to CT images permitted accurate diagnosis of eleven of the tumors and provided anatomical information important for surgical planning. Review of six previously published cases of cartilage tumors supported the findings. CT appears to be effective in the preoperative differentiation of osteochondroma and chondrosarcoma, including cases where this distinction cannot be made with conventional radiographs.

Adolescent↗
PubMed2006

Chondrosarcoma of the spine: a rare case with unusual presentation.

Chondrosarcoma is the third most common primary malignancy of bone, affecting primarily the pelvic and shoulder girdles and being extremely rare in the spine. Herein, we present a case of a 65-year-old woman with a rare chondrosarcoma of the spine, who presented with clinical symptoms from the lung metastasis. The neoplasm was grade II and exhibited overexpression of the p53 tumor suppressor protein. The latter has been associated with a high propensity for distant metastases.

Journal Article↗
PubMedDate not supplied

Testicular chondrosarcoma.

A case of primary chondrosarcoma of the testis is reported. A 40-year-old man presented a painless swelling of the right testis that he has been observing for 3 years. Gross examination of the resected specimen showed an encapsulated, gray to tan colored, roughly rounded tumor. Histologically, the tumor revealed a well-differentiated chondrosarcoma.

Journal Article↗
PubMed2005

Evaluation of a new concept for the management of skull base chordomas and chondrosarcomas.

OBJECT: Chordomas and chondrosarcomas of the skull base are rare locally invasive tumors associated with high recurrence rates. The aim of this study was to evaluate the concept of microsurgical tumor volume reduction followed by early gamma knife surgery (GKS). METHODS: Thirteen patients with 15 tumors were treated between October 2000 and June 2003. There were three patients (23.1%) with chordomas and 10 (76.9%) with chondrosarcomas. There were nine men and four women who ranged in age between 19 and 69 years. All patients first underwent maximal tumor resection. Within 2 to 10 months after surgery they were treated with GKS. The mean postoperative tumor volume treated with GKS was 9.7 cm3 (range 1.4-20.3 cm3). Follow-up computerized tomography and magnetic resonance imaging examinations with volumetric tumor analysis were performed every 6 months after GKS. The mean treatment dose was 17 Gy and the mean isodose was 52%. The mean follow-up duration was 17 months during which there was only one tumor recurrence at the margin of the radiation field. The mean volume reduction was 35.4%. CONCLUSIONS: Results of this treatment strategy are encouraging but the efficacy of this multimodal treatment combining surgery and early GKS requires a longer follow up.

Adult↗
PubMed1983

Purification of an insulin-like growth factor II receptor from rat chondrosarcoma cells.

An insulin-like growth factor II (IGF-II) receptor was purified from rat chondrosarcoma cells by Triton X-100 solubilization of a 100,000 X g membrane preparation and affinity chromatography on a multiplication-stimulating activity (MSA)-Sepharose column. Analysis of the purified receptor by sodium dodecyl sulfate-gel electrophoresis and silver staining showed a major band of Mr = 210,000 (Mr = 250,000 after reduction with dithiothreitol). When 125I-MSA was chemically cross-linked to the purified receptor and analyzed by sodium dodecyl sulfate-gel electrophoresis (with and without dithiothreitol) and autoradiography, the radioactive bands coincided with the Mr = 210,000 and 250,000 bands identified by silver staining. The purified receptor also appeared to contain an Mr = less than 68,000 species identified by silver staining in addition to the Mr = 250,000 binding component. IGF-I, IGF-II, and MSA-II inhibited binding of 125I-MSA to the purified receptor with the same relative potency as for binding to the intact chondrosarcoma cell (IGF-II greater than MSA-II greater than IGF-I), and insulin did not inhibit binding. The association constant (K alpha) for MSA-II binding to the purified receptor was 2 X 10(9) M-1. The purified receptor bound to concanavalin A-Sepharose and wheat germ lectin-Sepharose columns and was eluted with alpha-methyl-D-mannoside and N-acetyl-D-glucosamine, respectively, showing that the receptor is a glycoprotein.

Animals↗
PubMed2006

Primary chondrosarcoma of ethmoid bone in a 6-year-old child.

Chondrosarcoma (CS) is a distinctly uncommon tumor in young patients and tends to be located in the extremities. Cartilaginous tumors in young patients are benign tumors. We report a case of a childhood CS in a 6-year-old boy who presented with right-sided proptosis. Radiological examination revealed a large destructive mass in right ethmoidal sinus extending into the surrounding sphenoidal sinus, intracranially, and the into right orbit, causing proptosis. Biopsy of the mass showed characteristic features of a well-differentiated CS. There are no reports of CS presenting as proptosis and arising from ethmoid bone. This case highlights the rarity of this tumor with regard to age of presentation and site of origin.

Bone Neoplasms↗
PubMed2001

Chondrosarcoma of bone: an oncological and functional follow-up study.

We retrospectively analysed the course of 42 out of 45 patients suffering from different chondrosarcomas which were treated surgically. We found a prospective 5- and 10-year survival of 64% for both time intervals. Follow-up examination was possible in 21 of 45 patients. Most of them were staged as NED, one as AWD, and 8 of 45 were lost to follow-up. From 16 dead patients 12 died of the disease and 4 of unknown but not to tumor related reasons. Survival was depending significantly on the histopathological grade, and the stage according to Enneking's surgical staging system. Eleven out of forty-five patients developed metastases within a mean period of twelve months after surgery. The survival of these patients was significantly reduced. Patients with centrally located tumors exhibited a distinct but non-significant worse survival than those suffering from peripheral tumors. In 18% (8 of 45) recurrence of the tumor was evident within a mean period of 24 months (5-85). Functional evaluation was performed in 21 out of 28 alive patients. After a mean follow-up time of 72 months the mean score was 64% (23-100). Females exhibited a distinct but non-significant better result, the same was observed for peripheral locations compared to centrally located tumors. Regarding age, grade, and line of resection no tendency of any dependence was detected, but worst results were seen in those with a stage III tumor and dedifferentiated CS.

Adult↗
PubMed1977

Gardner's syndrome and chondrosarcoma of the hyoid bone.

There appears to be a total of five cases of chondrosarcoma of the hyoid bone (not all associated with Gardner's syndrome). The case reported herein is one that was first reported in the literature on Gardner's syndrome and the second reported instance of a patient with Gardner's syndrome who had a malignant bone tumor.

Adenocarcinoma↗
PubMed1979

Maxillary chondrosarcoma.

Chondrosarcoma is a malignant tumor that arises from cartilage. In the maxillary area, the tumors spread locally but may also invade blood vessels and metastasize systemically. Initial surgical resection is the treatment of choice.

Chondrosarcoma↗
PubMed1981

Chondrosarcoma of the nasal septum.

Chondrosarcoma of the nasal septum is a rare pathological entity. The histologic and roentgenographic features as well as the clinical presentations are diverse. Treatment of choice is a wide surgical excision, which demands a craniofacial approach. We are presenting the sixth reported case and, to our knowledge, the first one in which this surgical approach was used.

Chondrosarcoma↗
PubMed1999

Tumor-induced osteomalacia and symptomatic looser zones secondary to mesenchymal chondrosarcoma.

Tumor-induced osteomalacia is a rare clinical entity that is associated with soft-tissue or skeletal tumors. We present a case report of a patient with a chest wall mesenchymal chondrosarcoma who presented with bone pain. The patient had skeletal changes in the femoral neck and fibula consistent with osteomalacia and laboratory values suggesting phosphate diabetes. The patient was treated with tumor resection and phosphate supplementation with reversal of the signs and symptoms of osteomalacia. Tumor-induced osteomalacia is vitamin-D-resistant and often reversed by complete removal of the tumor. Most commonly, the causative tumors are of vascular, mesenchymal, or fibrous origin. The osteomalacia is associated with bone pain, muscle weakness, and radiographic changes. Tumor-induced humoral factors have been implicated in causing the osteomalacia, but the definite etiology has yet to be determined. Current treatment includes complete tumor resection and electrolyte supplementation.

Bone Neoplasms↗
PubMed1996

Chondrosarcoma of the pelvis. Prognostic factors for 67 patients treated with definitive surgery.

BACKGROUND: Chondrosarcoma (CS) most commonly involves the pelvis. Local and systemic failures often result in poor outcome. Prognostic factors that determine patient outcome remain ill-defined. METHODS: We retrospectively analyzed 67 patients with CS of the pelvis treated by definitive surgery from January 1970 to December 1992. All patients had localized disease including Grade 1 (19 patients), Grade 2 (18 patients), Grade 3 (17 patients), and dedifferentiated (13 patients) tumors. Thirty-two patients underwent a limb-sparing surgical resection and 35 patients underwent hemipelvectomy. Follow-up was available for all patients. The median duration of follow-up for the survivors was 115 months (range, 24-288 months). RESULTS: At last follow-up, 52% of the patients were disease free, 8% were alive with disease, 36% died of disease, and 4% died of other causes. Nineteen patients (28%) had developed a local recurrence (LR). The median time to LR was 23 months (range, 1-111 months). Independent variables in a multivariate analysis associated with increased risk of LR included inadequate surgical margin, tumor epicenter in the pubis, and high grade histology. LR did not influence overall patient survival. Twenty-three patients (36%) developed distant metastases at a median time of 9 months (range, 1-111 months) postoperatively. Metastases developed in 0% of the patients with Grade 1, 20% of the patients with Grade 2 60% of the patients with Grade 3 and 75% of patients with dedifferentiated CS. On multivariate analysis, histologic high grade was the only significant predictive variable for distant relapse (P = 0.005). CONCLUSIONS: The critical issue for a favorable outcome in low grade CS of the pelvis is adequate surgical excision (i.e., negative surgical margin). The high rate of systemic failure in high grade and dedifferentiated CS, despite adequate surgery, emphasizes the need for more effective systemic therapy.

Adult↗
PubMed1968

Malignant degeneration of benign cystic teratomas of the ovary. Review of the literature and report of a chondrosarcoma and carcinoid tumor.

The development of cancer in a pre-existing benign cystic teratoma of the ovary is rare. A total of 272 cases have now been recorded in the literature, although detailed reports are unavailable in 75. Excluding cases of doubtful classification, 93% of tumors were carcinomas and only 7% sarcomas. In the epithelial group squamous carcinoma was by far the most common (83%). The incidence of adenocarcinoma and argentaffinoma was approximately equal at 6%, although the number of cases of carcinoid tumor reported in recent years appears to be increasing. The present report tabulates the findings in 43 cases described since the last extensive review in 1957. The average age of patients was 55 years and the only symptom or sign was an abdominal mass, except when the tumor had extended through the cyst wall. Right and left ovaries were involved with equal frequency. Treatment varied from simple removal of the cyst to hysterectomy and bilateral salpingo-oophorectomy. In some inoperable cases only biopsy of the lesion was possible. Prognosis was poor when extension through the cyst wall had occurred and good otherwise. The first case of chondrosarcoma and the fourteenth case of carcinoid tumor arising in a benign cystic teratoma are reported.

Adult↗
PubMed1975

Increased incidence of cerebral metastases in sarcoma patients with prolonged survival from chemotherapy. Report of cases of leiomysarcoma and chondrosarcoma.

Soft tissue and bony sarcomas rarely metastasize to the central nervous system, particularly to the cerebral hemispheres. In 456 patients with metastatic sarcoma, only 6 (1.3%) had cerebral metastases documented by brain scan at the time of referral for chemotherapy. Adriamycin-containing combination chemotherapeutic regimens have led to a significant increase in the median survival of patients from the start of chemotherapy (18 + months for responders compared, to 7 months in nonresponders). Of 14 patients relapsing after a response or stabilization of disease of 6 months or greater, the cause of relapse was the development of cerebral metastases in 5 (36%). Two of these cases, one a patient with leiomyosarcoma and one with chondrosarcoma, were documented by autopsy and are reported in detail because of their rarity in the medical literature. Although the numbers are small, the increased incidence of cerebral metastases in the group relapsing after a lengthy response suggests that improved chemotherapy for sarcomas resulting in improved survival may be chaning the pattern of metastatic disease, and may require new the;apeutic approaches.

Adult↗