Chordoma: Difference between revisions
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{{SK}} Notochordoma; chordocarcinoma; chordoepithelioma | {{SK}} Notochordoma; chordocarcinoma; chordoepithelioma | ||
==Overview== | ==Overview== | ||
Chordomas are uncommon malignant tumors that account for 1% of intracranial tumors and 4% of all primary bone tumors. They originate from embryonic remnants of the primitive notochord (earliest fetal axial skeleton, extending from the Rathke's pouch to the coccyx). Since chordomas arise in bone, they are usually extradural and result in local bone destruction. They are locally aggressive, but uncommonly metastasise. Fluid and gelatinous mucoid substance (associated with recent and old haemorrhage) and necrotic areas are found within the tumor. In some patients, calcification and sequestered bone fragments are found as well. The variety of these components may explain the signal heterogeneity observed on MRI. Incomplete delineation of the tumor and microscopic distal extension of tumor cells may explain the frequency of recurrences. Physaliphorous cells are classically seen on microscopy | Chordomas are uncommon malignant tumors that account for 1% of intracranial tumors and 4% of all primary bone tumors. They originate from embryonic remnants of the primitive notochord (earliest fetal axial skeleton, extending from the Rathke's pouch to the coccyx). Since chordomas arise in bone, they are usually extradural and result in local bone destruction. They are locally aggressive, but uncommonly metastasise. | ||
Metastatic spread of chordomas is observed in 7-14% of patients and includes nodal, pulmonary, bone, cerebral or abdominal visceral involvement, predominantly from massive tumors. True malignant forms of chordomas occasionally have areas of typical chordoma and undifferentiated areas, most often suggestive of fibrosarcoma; the prognosis is poor. | ==Pathophysiology== | ||
Fluid and gelatinous mucoid substance (associated with recent and old haemorrhage) and necrotic areas are found within the tumor. In some patients, calcification and sequestered bone fragments are found as well. The variety of these components may explain the signal heterogeneity observed on MRI. Incomplete delineation of the tumor and microscopic distal extension of tumor cells may explain the frequency of recurrences. Physaliphorous cells are classically seen on microscopy. Metastatic spread of chordomas is observed in 7-14% of patients and includes nodal, pulmonary, bone, cerebral or abdominal visceral involvement, predominantly from massive tumors. True malignant forms of chordomas occasionally have areas of typical chordoma and undifferentiated areas, most often suggestive of fibrosarcoma; the prognosis is poor. | |||
==Epidemiology== | ==Epidemiology== | ||
Chordomas occur at any age but are usually seen in adults (30-70 years). Those located in the spheno-occipital region most commonly occur in patients 20-40 years of age, whereas sacrococcygeal chordomas are typically seen in a slightly older age group (peak around 50 years 10). They are commonly found in Caucasians. | Chordomas occur at any age but are usually seen in adults (30-70 years). Those located in the spheno-occipital region most commonly occur in patients 20-40 years of age, whereas sacrococcygeal chordomas are typically seen in a slightly older age group (peak around 50 years 10). They are commonly found in Caucasians. | ||
Revision as of 16:02, 23 December 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Notochordoma; chordocarcinoma; chordoepithelioma
Overview
Chordomas are uncommon malignant tumors that account for 1% of intracranial tumors and 4% of all primary bone tumors. They originate from embryonic remnants of the primitive notochord (earliest fetal axial skeleton, extending from the Rathke's pouch to the coccyx). Since chordomas arise in bone, they are usually extradural and result in local bone destruction. They are locally aggressive, but uncommonly metastasise.
Pathophysiology
Fluid and gelatinous mucoid substance (associated with recent and old haemorrhage) and necrotic areas are found within the tumor. In some patients, calcification and sequestered bone fragments are found as well. The variety of these components may explain the signal heterogeneity observed on MRI. Incomplete delineation of the tumor and microscopic distal extension of tumor cells may explain the frequency of recurrences. Physaliphorous cells are classically seen on microscopy. Metastatic spread of chordomas is observed in 7-14% of patients and includes nodal, pulmonary, bone, cerebral or abdominal visceral involvement, predominantly from massive tumors. True malignant forms of chordomas occasionally have areas of typical chordoma and undifferentiated areas, most often suggestive of fibrosarcoma; the prognosis is poor.
Epidemiology
Chordomas occur at any age but are usually seen in adults (30-70 years). Those located in the spheno-occipital region most commonly occur in patients 20-40 years of age, whereas sacrococcygeal chordomas are typically seen in a slightly older age group (peak around 50 years 10). They are commonly found in Caucasians.