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Chondrosarcoma of the maxilla. Case report.

Chondrosarcoma is a rare malignant neoplasm of the jaws. The diagnosis of this lesion is mainly based on the histopathological appearance. A case of chondrosarcoma of the maxilla is reported. The origin of this rare tumor in the maxilla, a bone with exclusive membranous ossification, is discussed.

Adult↗

The treatment of large extraskeletal chondrosarcoma of the leg: comparison of IMRT and conformal radiotherapy techniques.

Extraskeletal chondrosarcoma of the leg is a rare, malignant neoplasm with very few cases having been reported in the literature. In this study we investigate the possibility of using intensity modulated radiotherapy (IMRT) for this type of disease and demonstrate its advantages over conventional three-dimensional (3D) conformal treatment. A case was presented of a patient with extraskeletal chondrosarcoma of the lateral compartment of the leg in which the target volume was 50 cm in length and twisted around the surrounding bones. Both the 3D conformal plan and IMRT plan were designed using the Memorial Sloan-Kettering Cancer Center planning system. The IMRT plan produced a superior dose distribution to the patient as compared to the 3D conformal plan both in terms of dose conformity and homogeneity in the target volumes, and reduction of the maximum dose to the bone. The planning time of the IMRT plan was about 3-5 times shorter than that of the 3D conformal plan. It was demonstrated that the IMRT technique can be used not just for small tumors, but also for large and spiral-shaped tumors close to critical organs. The IMRT method requires less planning time, and provides better target coverage with more sparing of critical structures. When planning patients with multiple target volumes receiving different prescribed doses, the IMRT technique can more easily meet this requirement.

Adult↗

Dedifferentiated chondrosarcoma arising in the orbit.

Orbital chondrosarcomas are extremely rare and are usually an extension of tumours involving the paranasal sinuses. A unique case of dedifferentiated chondrosarcoma arising solely within the orbit is presented.

Adult↗

Malignant melanoma in families of children with osteosarcoma, chondrosarcoma, and adrenal cortical carcinoma.

Seven cases of malignant melanoma in the close relatives of children with osteosarcoma and chondrosarcoma are described. The association between certain childhood malignancies (adrenal cortical carcinoma, osteosarcoma, chondrosarcoma, retinoblastoma) and malignant melanoma is discussed and it is proposed that in certain families malignant melanoma may be another manifestation of the same gene defect which results in susceptibility to tumours characteristic of the SBLA cancer family syndrome.

Adolescent↗

Primary non-skeletal intracranial cartilaginous neoplasms: report of a chondroma and a mesenchymal chondrosarcoma.

The clinical and pathological features of two rare, primary non-skeletal intracranial cartilaginous tumours, a chondroma and a mesenchymal chondrosarcoma, are presented. The literature with respect to clinical, pathological, and possible pathogenetic features is reviewed. The chondroma was successfully resected and the patient has had no recurrence since the operation in 1956. The clinical course of the mesenchymal chondrosarcoma was characterized by rapidly recurring tumour, slowed somewhat by cobalt therapy and frequent surgical interventions. Death occurred some 16 months after the initial resection. There were no metastases.

Adolescent↗

Intracranial chondrosarcoma: review of the literature and report of 15 cases.

The available data in the literature (177 cases), two current clinical patients, and cases which occurred in The Netherlands (13) were reviewed concerning the clinical presentation, pathological features, radiological data, and treatment options of chondrosarcoma of the cranial base. The mean age of patients was 37 years, the male/female ratio 1:1.1. The most frequent complaints were diplopia with oculomotor disorders (51%), headache (31%), and decreased hearing, dizziness, and tinnitus with statoacusticus dysfunction (21%). The mean duration of symptoms before diagnosis was 27 months. The chondrosarcomas were located in the petrosal bone in 37% (47 cases), in the occipital bone and clivus in 23% (30 cases), in the sphenoid bone in 20% (25 cases) and to a lesser extent in frontal, ethmoidal, and parietal bones (14%). In 6% (eight cases) the primary location was in dural tissue. Radiological examinations showed bone destruction and variable calcification (CT), involvement of neuronal and vascular structures (MRI), and mostly hypovascularity on angiography. On histological examination 51% of tumours were classified as grade I, 11% grade II, 30% mesenchymal, and 8% myxoid. The mesenchymal type was the most malignant as illustrated by a strong tendency to intradural and cerebral growth and possibly occurrence in younger age groups. The treatment of choice until recently was surgery because of the critical location and local aggressive nature. Regrowth of tumour after surgery occurred in 53% of the patients (average after 32 months). Charged particle irradiation gave a five year survival of 83-94% and a local control rate of 78%-91%. Both in surgery and radiotherapy there is treatment related morbidity and mortality that should be considered when offering these therapies. Recent promising results imply that charged particle radiotherapy, in combination with surgery, may be the therapeutical choice of the future.

Adolescent↗

A new variant of Carney's triad: phaeochromocytoma and chondrosarcoma.

A 61 year old hypertensive woman presented in 1986 with a right scapular chondrosarcoma. She developed type 1 diabetes mellitus in 1991 and suffered a stroke in 1991. Chest radiography showed pulmonary metastases in 1997. Further radiological staging detected a right sided phaeochromocytoma, which was subsequently removed in 1998. Before this, repeated urine estimations of vanillylmandelic acid had been normal. Her diabetes was cured by adrenalectomy. It is believed that the combination of phaeochromocytoma and extrapulmonary chondrosarcoma represents a new variant of Carney's triad.

Adrenal Gland Neoplasms↗

Chondrosarcoma of thumb metacarpal--a case report with literature review.

Function preserving management of a dominant thumb metacarpal chondrosarcoma is reported, with a literature review for this uncommon neoplasm. Wide local excision of the metacarpal followed by temporary silicone block interposition and definitive iliac crest bone grafting was performed. A persistent wound defect was managed by a radial artery forearm flap. The functional result was acceptable. In the literature, there were seven cases of thumb metacarpal chondrosarcoma reported; two were treated by ray amputation, and the other five were treated by excision of the metacarpal. Three of those five had bone graft and the other two had simple resection. Of these seven cases, three had local recurrence and one a distant metastasis.

Aged↗

Clear cell chondrosarcoma.

The clinical, radiologic, and histopathologic features of three cases of clear cell chondrosarcoma are described. On radiographs, this rather benign-appearing tumor resembles a chondroblastoma when it occurs at the end of a long bone, and may occasionally show a calcified matrix. However, it has distinctive tumor cells with a centrally placed vesicular nucleus surrounded by clear cytoplasm. The lesion has a low-grade malignancy and is amenable to en bloc surgical resection, which results in a much better prognosis than that of conventional chondrosarcoma.

Adult↗

Regulation of chondrosarcoma metabolism by cyclic adenosine 3'5'-monophosphate.

The in vitro effects of cyclic AMP on amino acid transport and synthesis of macromolecules in the Swarm rat chondrosarcoma were investigated using the cyclic AMP analogue, N6-monobutyryl cyclic AMP (MBcAMP), and the phosphodiesterase inhibitor, 3-isobutyl-1-methylxanthine (MIX). Amino acid transport was assessed by measuring alpha-amino-[1-14C]isobutyrate (AIB) uptake. The synthesis of macromolecules was estimated by measuring radiolabeled precursor incorporation into total proteins, proteoglycan, and RNA. MBcAMP stimulated [14C]AIB uptake, [3H]uridine transport, and UTP formation. MBcAMP inhibited 35SO4 and [3H]leucine incorporation into proteoglycan and stimulated [3H]uridine incorporation into RNA. MIX elevated endogenous cyclic AMP levels in the Swarm rat chondrosarcoma and mimicked the effects of MBcAMP on AIB transport and radiolabeled precursor incorporation into macromolecules. For comparative purposes, the effects of MBcAMP on AIB uptake and macromolecule synthesis in rat costal cartilage segments were investigated. MBcAMP and MIX stimulated AIB uptake by costal cartilage segments, inhibited [3H]leucine incorporation into total protein and 35SO4 incorporation into proteoglycan, and had no effect on [3H]uridine incorporation into RNA.

Amino Acids↗

Chondrosarcoma of the temporal bone and otosclerosis.

Chondrosarcoma constitutes 6% of all primary bone tumors and 11% of malignant primary bone tumors. Nevertheless, in a review of the tumor registry of the University of Michigan covering a period of 50 years, there were only 3 cases involving the temporal bone. A case of a woman with a chondrosarcoma of the temporal bone that was partially resected by means of an infratemporal approach at the skull base is presented. This patient had previously undergone surgical treatment for otosclerosis of the other ear. Several considerations regarding survival factors in this type of tumor are dealt with in terms of the histological features, therapeutic options and anatomic location. The possibility that this lesion may originate from the persistence of the cartilaginous inclusions that some authors consider to be involved in the origin of otosclerosis is discussed.

Chondrosarcoma↗

Hypofractionated stereotactic radiation therapy for skull base and upper cervical chordoma and chondrosarcoma: preliminary results.

INTRODUCTION: Chordoma and chondrosarcoma are rare tumors of the base of the skull and are difficult candidates for surgical treatment. They are also usually resistant to conventional radiation therapy. We report preliminary results of hypofractionated stereotactic radiation therapy (SRT) using the Cyberknife system (Accuray Inc., Sunnyvale, Calif., USA) for primary and recurrent chordomas and chondrosarcomas of the skull base and upper cervical region. MATERIAL AND METHODS: Nine pathologically proven chordoma/chondrosarcoma patients underwent Cyberknife treatment, and in 4 patients Cyberknife was performed as a primary adjuvant treatment after operation. Remainder of the patients had previously received conventional radiotherapy except 1 who had received Gamma Knife treatment. The prescribed tumor dose ranged from 21 to 43.6 Gy in three to five fractions. The dosimetric characteristics were evaluated for conformity and coverage indices. Dose volume histograms of both the tumor and the critical structure were obtained, and the dose delivered to a specific volume (25, 50 and 100%) of the critical structure was calculated in each case. Mass response was measured on follow-up MRI scans. Total tumor doses of different fractionation numbers were converted into single session equivalent doses and linear quadratic equivalent doses of conventional radiation for comparison among patient groups. RESULT: No significant complications were observed during the treatment and early follow-up periods except one instance of transient esophagitis and one instance of otitis. All treatment plans met the criteria for standard protocol of radiosurgery suggested by the Radiation Therapy Oncology Group, specifically in terms of conformity index, which ranged from 1.01 to 1.83. Three plans had a coverage index that was rated as a minor acceptable deviation. All patients were followed from 11 to 30 (median 24) months following the treatment with regular magnetic resonance images, and 4 patients showed mass reduction. Disease progression was not noted in any patient during the above follow-up period except 1 patient who showed asymptomatic recurrence on 27-month follow-up MRI. Dose volume histograms revealed that the relative dose to volume percent of critical structure, measured at 25, 50 and 100%, was apparently lower in the pretreatment surgical decompression group than in the nondecompression group. Two patients developed radiation-induced myelopathy. The delivered radiation dose to the critical structure calculated using the linear-quadratic formula was within the acceptable range in one case and exceeded 70 Gy at 50% volume of the spinal cord in the other. CONCLUSION: The hypofractionated Cyberknife SRT is effective in generating therapeutic response in these radioresistant tumors, with minimal toxicity during the procedure and early follow-up period. Repeated radiosurgical treatment may also be feasible for tumor recurrences but deserves great caution with respect to the biological effects of the accumulated dose on the adjacent critical structures. Cyberknife SRT may be a potentially valuable treatment option once the long-term results and appropriate dose calculators are optimally defined.

Adult↗

A case of chondrosarcoma developing in a recurrent retroperitoneal mass after chemotherapy for testicular germ cell tumor.

Teratomas with malignant transformation occur in a small proportion of patients with metastatic germ cell tumors treated with platinum-based chemotherapy. Chondrosarcoma has rarely been reported as a component of the second non-germ cell malignancy. We report the case of a 37-year-old man who developed a chondrosarcoma in a recurrent retroperitoneal mass after chemotherapy for testicular germ cell tumor. Malignant transformation of the retroperitoneal teratomatous mass occurred in the absence of any symptoms or clinical signs, elevation in serum tumor markers, or the presence of atypical elements in previously resected specimens, suggesting the need for close radiographic follow-up of these patients.

Adult↗

Primary chondrosarcoma of the left inferior lobar bronchus.

A primary chondrosarcoma arising in the left inferior lobar bronchus is described in a 67-year-old man. The symptoms upon admittance were dyspena, cough with purulent sputum and weight loss. The tumor was removed by pneumonectomy. Eight months later the patient died of massive mediastinal lymph node involvement. While tracheobronchially located primary pulmonary chondrosarcoma tends to remain localized, the peripheral variety tends toward mediastinal lymph node involvement and thoracic metastasis. The treatment of choice is resection in a radical manner, whenever possible.

Aged↗

Chondrosarcoma of the larynx.

Chondrosarcoma of the larynx is a rare neoplasm. The characteristic features of this tumor are described, and a case in a 69-year-old woman is reported. This case demonstrates the difficulties of histological classification. On the first biopsy specimen a benign chondroma was diagnosed. However, the microscopical features of the excised tumor were those of a chondrosarcoma. It is generally accepted that radiotherapy is of no value; surgical treatment by laryngectomy is advised. Some authors recommend a conservative attitude towards these tumors, which are of low malignancy, the growth slow and the risk of local or distant metastases slight.

Aged↗

Chondrosarcoma of the proximal femur with myxoid degeneration mistaken for chondromyxoid fibroma in a young adult. A case report.

BACKGROUND: Fine needle aspiration cytology (FNAC) is effective in the diagnosis of bone tumors when combined with careful radiologic and clinical evaluation. However, cases where clinical or radiologic findings are atypical or unusual may lead to an erroneous diagnosis. CASE: A 19-year-old male presented with a pain in the left hip area that had been slowly progressive over a 10-month period. Clinical and radiologic findings suggested either giant cell tumor or chondroblastoma. The smeared aspiration specimen showed loosely cohesive, oval to round cells with moderate amounts of pale pink cytoplasm admixed with pinkish-blue, chondromyxoid material. The individual cells contained a single nucleus with evenly distributed, fine chromatin. A few osteoclastic giant cells were scattered in the smears. A cytologic diagnosis of myxoid lesion with a few giant cells, suspicious for chondromyxoid fibroma, was made. The diagnosis of chondrosarcoma was made by subsequent histologic examination. CONCLUSION: Absence of the usual clinicoradiologic features of chondrosarcoma combined with an unusual cytologic presentation in this case led to a misdiagnosis. In most centers, FNAC has achieved undisputed status as a diagnostic tool, and cytologic diagnosis often forms the basis of the therapeutic protocol. However, at some sites FNAC diagnosis is more problematic. Awareness of the limitations and pitfalls of FNAC is just as important as knowledge of the scope of FNAC in bone tumors. Tumors with chondromyxoid features provide particular difficulties.

Adult↗

Chondrosarcoma of the urinary bladder: report of a case with immunohistochemical and ultrastructural findings and review of the literature.

A primary chondrosarcoma arising in the urinary bladder is described in a 73-year-old female, together with a review of the 2 previously reported cases in the literature. The clinical symptoms are similar to transitional cell carcinoma, but chondrosarcoma usually presents at an advanced stage, and the outcome is rather poor. This very rare and commonly poorly differentiated tumor should not be confused with poorly differentiated transitional cell carcinoma. The differential diagnosis from other lesions with chondroid features, such as chondroid metaplasia, osteosarcoma and carcinosarcoma, is discussed. The value of immunohistochemistry and electron microscopy in the differential diagnosis is demonstrated.

Aged↗

Conservative surgical treatment of chondrosarcoma of the larynx.

Chondrosarcoma of the larynx is a rare malignant tumor sometimes encountered by otolaryngologists. Since they are usually of low grade malignancy, total laryngectomy is indicated only in cases of high grade malignancy or where total excision by conservative (partial) laryngectomy is not feasible. This paper reviews chondrosarcoma of the larynx, discusses a patient who developed this tumor, and describes a method of conservative surgical excision and laryngotracheal reconstruction when the tumor involves the cricoid, the most common site of occurrence in the larynx.

Adult↗