Chordoma are very rare tumors. Approximately 1 person out of 1 million will develop the disease. These numbers are similar for the US and Europe. For other regions no data is available. Approximately 2% of all bone tumors are chordoma. The ratio men to women is about 2:1 in sacral chordoma. The average onset of the disease is approximately 60 - 65 years in sacral chordoma [5], an onset before the age of 40 is rather unusual. However, even children may develop chordoma [6].

Symptoms

The typical symptom of a sacral chordoma is pain, mostly in the region of the so-called sacroiliac joint as well as in the gluteal region [3]. Furthermore, there might be nerval pain caused by irritation of the ischiadic nerv. This type of pain is typically and in contrast to "regular" ischiadic nerv pain localized at both sides of the body and may change sides, respectively [3]. It is also possible that lumbal nerv irritation may cause pain (yellow on the picture; this is approx. from the hip downwards). All these symptoms are relatively unspecific, and therefore the tumor might already be quite large at time of diagnosis [4]. If the lesion occurs above S3 ("S" stands for sacral segment and counting is from top to down) bowel and bladder problems may occur, in particular obstipation as well as emptying of the bladder. If a sacral root (nerves that step outside from the segments are called "roots") is directly irritated by the tumor, neurological symptoms may occur, in particular severe pain, disturbance of sensory perception, sudden weakness in the legs or in men sudden erectile dysfunctions.

Prognosis

The prognosis for sacral chordoma and those at mobile segments of the spine is in contrast to earlier assumptions not particularly good. A Swedish study reports [7] 5-, 10-, 15- ad 20-year survival rates of 84%, 64%, 52% und 52%, respectively .

Other studies report an average diseasefree period of time of approximately 63 months and an average survival time of approximately 7 years [8].

The most important criteria here seem to be the width of the margins , i.e. how close the operated tumor comes to the surgical margin. The best prognosis is for so-called R0-margins, i.e. wide margins where the tumor was removed within healthy tissue. The next best prognosis is for marginal margins and then those that go through the tumor (intralesional margins). Large tumor size, too little surgical margins, microscopic tumor growth as well as local recurrences have been shown to be negative progostic factors. Local recurrence is relatively common in chordoma, an average time interval is 18-24 months following initial diagnosis. Relatively late onset of metastatic growth is reported, particularly into the lungs, in approximately 5% - 40% [12] of patients. This is particularly true for patients with large lesions which grow relatively far towards the head, i.e. for example those including the complete sacrum or even further including lumbal parts of the spine.

However, it seems that chordoma are difficult to judge tumors, and longer survival rates are reported despite multiple metastases.

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		Sacral Chordoma General information Chordoma are primary bone tumors, i.e. they are no metastases from other tumors but represent an own cancer entity. It is assumed that chordoma develop from remnants of the notochord [1] (during the development of the embryo the notochord is the basis of the future spine). This explains the typical location of the chordoma at the sacrum (i.e. the last part of the spine; see green part of the spine picture), at the skullbase as well as at mobile segments of the spine. Approximately 50% of the chordoma are localized at the sacrum, 35% at the skullbase mostly in the region of the so-called clivus as well as approximately 15% at segments of the spine [2]. Prevalence and Incidence Chordoma are very rare tumors. Approximately 1 person out of 1 million will develop the disease. These numbers are similar for the US and Europe. For other regions no data is available. Approximately 2% of all bone tumors are chordoma. The ratio men to women is about 2:1 in sacral chordoma. The average onset of the disease is approximately 60 - 65 years in sacral chordoma [5], an onset before the age of 40 is rather unusual. However, even children may develop chordoma [6]. Symptoms The typical symptom of a sacral chordoma is pain, mostly in the region of the so-called sacroiliac joint as well as in the gluteal region [3]. Furthermore, there might be nerval pain caused by irritation of the ischiadic nerv. This type of pain is typically and in contrast to "regular" ischiadic nerv pain localized at both sides of the body and may change sides, respectively [3]. It is also possible that lumbal nerv irritation may cause pain (yellow on the picture; this is approx. from the hip downwards). All these symptoms are relatively unspecific, and therefore the tumor might already be quite large at time of diagnosis [4]. If the lesion occurs above S3 ("S" stands for sacral segment and counting is from top to down) bowel and bladder problems may occur, in particular obstipation as well as emptying of the bladder. If a sacral root (nerves that step outside from the segments are called "roots") is directly irritated by the tumor, neurological symptoms may occur, in particular severe pain, disturbance of sensory perception, sudden weakness in the legs or in men sudden erectile dysfunctions. Prognosis The prognosis for sacral chordoma and those at mobile segments of the spine is in contrast to earlier assumptions not particularly good. A Swedish study reports [7] 5-, 10-, 15- ad 20-year survival rates of 84%, 64%, 52% und 52%, respectively . Other studies report an average diseasefree period of time of approximately 63 months and an average survival time of approximately 7 years [8]. The most important criteria here seem to be the width of the margins , i.e. how close the operated tumor comes to the surgical margin. The best prognosis is for so-called R0-margins, i.e. wide margins where the tumor was removed within healthy tissue. The next best prognosis is for marginal margins and then those that go through the tumor (intralesional margins). Large tumor size, too little surgical margins, microscopic tumor growth as well as local recurrences have been shown to be negative progostic factors. Local recurrence is relatively common in chordoma, an average time interval is 18-24 months following initial diagnosis. Relatively late onset of metastatic growth is reported, particularly into the lungs, in approximately 5% - 40% [12] of patients. This is particularly true for patients with large lesions which grow relatively far towards the head, i.e. for example those including the complete sacrum or even further including lumbal parts of the spine. However, it seems that chordoma are difficult to judge tumors, and longer survival rates are reported despite multiple metastases.

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