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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A Case of chordoma of the base of the skull is reported. It had been present with minimal symptoms for six years during which the clinical picture was limited to nasal obstruction and headache. Comparison of initial X-rays and the preoperative assessment revealed progressive destruction of the base of the skull which indicated the likelihood of considerable difficulties in excision. Surgical treatment posed the problem of the approach and emphasised the absence of encapsulation and the incomplete nature of the operation. The development of new symptoms (diplopia, dysphagia) resulted in complementary radiotherapy. The authors take the opportunity to point out the polymorphous clinical nature of these tumours in relation to their site and the criteria of the histological diagnosis. The possibilities of treatment are discussed. Abstention from treatment is formally rejected and, on the contrary, the emphasis is placed upon the need for early treatment. The consequences of excision should be limited by using a minimal surgical technique when possible. Complementary radiotherapy is necessary and may be repeated, up to certain limits, in the case of recurrence if the first irradiation is felt to be effective. There is no parallel between histological appearances and the response to treatment. The possibilities of chemotherapy would appear to be limited.
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PMID:[Chordoma of the base of the skull. Clinical and histological study. Therapeutic possibilities (author's transl)]. 52 48

Chondrosarcoma of the skull base is a rare neoplasm. The most common presenting symptoms are hoarseness, dysphagia, and diplopia, which is associated with palsies of cranial nerves X, IX, and VI. The temporal bone is the most common site of tumor origin, followed by the sphenoid bone. These tumors must be differentiated from chordoma, chondroid chordoma, osteogenic sarcoma, enchondroma, and meningioma. Diagnosis is made by patient history, radiologic imaging, and biopsy. The treatment is surgical excision followed by radiation therapy. Five-year survival rates for grades I, II, and III are 90, 81, and 43%, respectively.
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PMID:Extracranial skull base chondrosarcoma. 268 2

The patient, a 48-year-old man, was first admitted in 1981 for the chief complaints of retropharyngeal tumor and anosmia. Computerized tomography scan disclosed destruction of the lower half of the clivus and retropharyngeal tumor predominantly located in the third prevertebral region. The tumor was removed partially via the transcervical approach and was histologically a typical chordoma. Two years later the tumor recurred and was operated on by the same approach. However, only partial removal of the tumor was realized due to difficulties in dissecting the fibrous adhesion. In 1986 (7 years after onset of the symptoms), the patient was readmitted due to severe dysphagia. The sagittal MRI scan demonstrated a huge oval retropharyngeal tumor with precise delineation between the tumor and clivus, clivus upper cervical spine and brain stem. The tumor was extirpated subtotally on the midline via the transoral approach. Dysphagia completely improved postoperatively and the patient regained normal smell sensation. The advantages and disadvantages of the transcervical and transoral approach to clival lesion are discussed and the advantage of the MRI imaging is stressed for preoperative information for the transoral approach.
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PMID:[Transcervical and transoral approach to clival and upper cervical chordoma: a case report]. 343 30

A 26-year-old woman experienced bilateral hearing loss, progressive nasal obstruction, and rhinorrhea. Examination disclosed a retropharyngeal mass. A needle biopsy specimen of the mass showed that it was a chordoma. The patient underwent surgery to remove the mass and received a postoperative course of radioactive cobalt. She did well for 18 months, at which time proptosis gradually developed in her right eye. Although the optic nerve heads and visual fields appeared normal, roentgenograms showed a large lesion involving the anterior and middle cranial fossae and destruction of the right posterior ethmoid sinus and right superior orbital fissure. Shortly after completing a course of methotrexate therapy (total dose, 89 mg), the patient experienced sudden pain and visual loss in her right eye. Surgical decompression of the orbit failed to restore light perception. The following year, her left eye became involved. A transfrontal craniotomy and extradural orbital decompression provided only temporary improvement. Four months later, left lateral rectus muscle palsy developed and her visual acuity decreased to 6/60 (20/200). Radiation therapy (400 rads per week; total dose, 3,200 rads) and treatment with methotrexate, vincristine sulfate, and prednisone did not improve her condition. At the time of her death, six years after the first symptom appeared, the patient was blind in both eyes, almost completely deaf, and suffered from severe dysphagia.
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PMID:Orbital invasion by an intracranial chordoma. 725 80

A 57-year-old male with clival chordoma developed severe hoarseness, dysphagia, and dysphonia 1 month after a second removal of the tumor. Magnetic resonance imaging demonstrated a mass 10 cm in diameter in the region of the middle clivus enhanced inhomogeneously by gadolinium-diethylenetriaminepentaacetic acid, and a defect in the skull base. There was evidence of compression of the anterior surface of the pons. He received proton irradiation employing a pair of parallel opposed lateral proton beams. The dose aimed at the tumor mass was 75.5 Gy, to the pharyngeal wall less than 38 Gy, and to the anterior portion of the pons less than 30 Gy. Time dose and fractionation factor was calculated at 148. Thirty-one months following treatment, he was free of clinical neurological sequelae. Proton therapy should be considered in treatment planning following initial surgical removal or for inoperable clivus chordoma.
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PMID:Proton radiation therapy for clivus chordoma--case report. 768 25

A 27-month-old boy of Hispanic background developed multiple cranial nerve palsies, difficulty swallowing, bloody nasal discharge, and irritability. Radiographic evaluations showed extensive destruction of the clivus by a large tumor that invaded the sphenoid bone, left cavernous sinus, ethmoid sinus, nasal cavity, and left orbit. Multiple pulmonary nodules were also noted. The bone marrow and spinal fluid showed no evident tumor cells. Transnasal biopsy revealed a chordoma. Treatment was initiated with a combination of ifosfamide, mesna, and etoposide along with radiation therapy to the cranial tumor. Shifting pulmonary densities were noted on serial films. Despite some clinical improvement, the child developed rapidly progressive hypoxemia 3 weeks after admission and died. Autopsy showed persistent viable tumor in the primary site and massive pulmonary arteriolar tumor emboli, infarcts, and widespread lung parenchymal metastases. No other sites of tumor involvement were discovered. This is the second child reported with intracranial chordoma, pulmonary metastases at diagnosis, and early death attributed to pulmonary tumor emboli.
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PMID:Metastatic intracranial chordoma in a child with massive pulmonary tumor emboli. 780 74

Chordomas are rare neoplasms of notochordal origin that arise along the vertebral axis. In the cervicofacial area, they show a marked proclivity for the sphenooccipital region. These slow-growing and infiltrating tumors are often discovered because of neglected symptoms related to the ENT field such as nasal obstruction, snoring, dyspnea or dysphagia in the case of anterior development, serous otitis media, cervical pain, or even palsy of the X, XI, or XII cranial nerves when the tumor develops toward the foramen jugulare or the foramen magnum. Prognosis is usually poor because of local malignancy, proximity to critical central nervous system structures, and volume of the tumor. Surgery is the preferred treatment for these extradural tumors, but most authors recommend postoperative irradiation because of surgical spillage or residual tumor. However, conventional irradiation is limited by the sensitivity of surrounding structures, which results in a poor rate of local control. We present 9 cases of histologically proven diagnosis of chordoma treated from 1984 to 1994 at our institution. Prognosis and therapeutic modalities are discussed. Therapeutic improvement might be brought the protontherapie, which ensures a better local control, and therefore may transform the prognosis of the disease.
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PMID:[Therapeutic management of craniocervical chordoma]. 876 68

A chordoma was removed from the tail base of a 6.5-year-old ferret (Mustela putorius furo). A nodule was observed in the area of tumor development when the ferret was purchased at 3 months of age. Although the nodule did not enlarge for 2 years, slow, steady growth of the tumor was observed for 4 years before surgical removal. Eight months after removal of the chordoma, the ferret developed 2 cutaneous masses. One was adjacent to the vulva, close to where the chordoma had been removed from, whereas the other was in the nasofacial region. After 4 months of slow growth, both masses were removed and both were histologically and immunohistochemically consistent with chordoma. Over the next 8 weeks, additional masses developed in the facial, maxillary gingival, and scapular regions. Enlargement of the gingival mass caused dysphagia, and the ferret was euthanized. Although a necropsy was not performed, these additional masses had a clinical appearance and texture that was similar to the 2 previously removed cutaneous chordomas. To the authors' knowledge, this is the first report of a ferret coccygeal chordoma that developed close to the base of the tail. Ferret chordomas have been reported previously to metastasize to the subcutis overlying the tumor. However, this is the first report of a ferret chordoma that metastasized to a location distant to the primary site of neoplasm development. Cell proliferation indices did not predict this metastatic behavior. It is hypothesized that the long clinical period before removal may have predisposed this neoplasm to metastasis. Observations from this case suggest that chordomas in ferrets may have metastatic potential and so should be removed promptly.
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PMID:Suspected metastatic coccygeal chordoma in a ferret (Mustela putorius furo). 1546 Mar 32

Chondroid chordoma commonly presents as clival osseous and extradural mass. A 15-year-old boy presented with progressive visual deficit, headaches and diplopia since three years. Computed tomography (CT scan) showed a skull base tumour, but was wrongly reported at the time as chronic sphenoidal sinusitis and nasal polyps. In the past three months, he developed dysphagia, urinary retention and constipation. Terminally, he had weakness of all limbs. Fundoscopy showed optic atrophy. Temporal and spatial variation in symptoms led to a clinical diagnosis of multiple sclerosis with optic neuritis. Partial brain autopsy revealed small gelatinous tumour nodules in the subarachnoid space of middle cranial fossa encasing base of brain like arachnoiditis. Tumour deposits extended down into the spinal cord along the subarachnoid space as far as vision allowed. Histopathology and immunohistochemistry confirmed a diagnosis of chondroid chordoma. Awareness of this rare mode of dissemination will avoid misdiagnosis and delay in treatment.
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PMID:Craniospinal dissemination of clival chondroid chordoma. 1633 98

Twelve patients with histologically confirmed clivus chordoma were treated at the Johns Hopkins Hospital between 1971 and 1989. Eight of the patients were men and four were women. The mean age at first operation was 51 years (range, 10 to 80). The most common presenting symptoms were headache, diplopia, dysphagia and dysarthria, and facial sensory changes. Computed tomography, with and without contrast enhancement, proved adequate for tumor identification and localization. Magnetic resonance imaging and angiography were occasionally employed to localize the tumors further and to define tumor vascular supply and proximity to vascular structures. Twenty-two resections were performed in 11 patients, and another patient underwent biopsy only. Seven were also treated with radiation therapy. Tumors recurred in eight patients, six of whom underwent further operations. The mean time to first recurrence was 22 months (range 8 to 36 months). Six of the patients are still alive, with a mean follow-up of 31 months (range, 3 to 89 months) from first surgical resection. The mean survival time from first treatment was 31 months (range, 4 to 62 months) among those patients who died. There was no operative mortality. The 5-year cumulative survival rate was 20%. Six patients with long follow-up have had fair to good results, being free of recurrences for at least a year. However, none of the patients returned to their premorbid baseline of activities. Five of the patients had tumors with the histologic diagnosis of chondroid chordoma. Three of these patients are still alive. The mean age at first treatment was 44 (compared with 62 for typical chordomas). The mean time from symptoms to diagnosis was 29 months (typical chordomas, 18 months). The mean length of survival and time to tumor recurrence were not significantly different between chondroid and typical chordomas.
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PMID:Clivus chordoma: a report of 12 recent cases and review of the literature. 1717 Aug 37


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