Sacrococcygeal teratoma
Updates to Article Attributes
Sacrococcygeal teratoma refers to a teratoma arising in the sacrococcygeal region. The coccyx is almost always involved 6.
Epidemiology
It is the most common congenital tumour in fetus 11 and neonate 3. The incidence is estimated at ~1:35,000-40,000. There is a recognised female predilection with a M:F ratio of 1:4.
Associations
Clinical presentation
Presentation varies depending on if a tumour has an intrapelvic location or has an extra-fetal extension (see further classification below). Intrapelvic tumours can manifest after birth with genitourinary and gastrointestinal symptoms given the compression of those structures.
Pathology
The sacrococcygeal region is the most common location for non-CNS teratomas. They are thought to arise from totipotent cells from the node of Hensen 1,3 at the anterior aspect of the coccyx by about the 2nd to 3rd weeks of gestation. They are most often mixed solid/cystic, although purely cystic types can occur in ~15% of cases.
A tumour is composed of all three germ cells (i.e. ectoderm, mesoderm and endoderm).
Classification
A pathology-based classification is as:
benign (mature): much more common, comprising ~ 60-70%
malignant (immature)
A location-based classification system according to the American Academy of Paediatric Surgery Section Survey is:
type I: developing only outside the fetus (can have small pre-sacral component); accounts for the majority of cases, 47% 12
type II: extra-fetal with intrapelvic presacral extension
type III: extra-fetal with extension through the pelvis into the abdomen
type IV: tumour developing entirely in the fetal pelvis
Markers
Can have elevated levels of:
Genetics
most cases tend to be sporadic 12
Radiographic features
Plain radiograph
may show a large mass projecting from the lower pelvic region or within the abdominopelvic cavity
may show calcification
Ultrasound
Mature types tend to be more cystic which appear as anechoic components. Solid types (which are much rarer) often appear as an echogenic mass within the pelvis.
The correlation between sonographic appearances and malignant components is thought to be poor 7.
Colour Doppler interrogation in some tumours may show marked hypervascularity with arteriovenous (AV) shunting.
CT
Not part of a routine investigation. Identifies bone, fat and cystic components. Calcification may again be seen.
MRI
Superior to ultrasound especially in the assessment of the following areas 2:
colonic displacement
ureteric dilatation
associated hip dislocation
intraspinal extension
vaginal dilatation
metastatic assessment in malignant lesions
Signal characteristics can significantly vary depending on the constituent of the teratoma 1.
T1: fat components appear high signal, calcific/bony components low signal
T2: fluid (cystic) components appear high signal, calcific bony components low signal
T2* GRE: magnetic susceptibility artefact because of calcifications
T1 C+ (Gd): enhancing solid components
Treatment and prognosis
A sacrococcygeal teratoma can be benign or malignant depending on whether they are mature or immature. The majority, however, tend to be benign (~80% 11). Those presenting in older infants tend to have a higher malignant potential while those presenting in utero have a poor prognosis due to complications. Malignant change may be also more common in males.
Treatment is with surgical excision inclusive of coccygectomy with additional chemotherapy for malignant tumours 5.
Complications
high output cardiac failure from AV shunting: which in turn can cause hydrops fetalis
ureteric obstruction
gastrointestinal tract obstruction
compression of underlying nerves: giving urinary/faecal incontinence
anaemia
tumour rupture
Differential diagnosis
General imaging differential considerations include:
sacral chordoma
terminal myelocystocoele: for cystic types on ultrasound 9
For type IV lesions also consider:
low-lying neuroblastoma
low-lying rhabdomyosarcoma
small round blue cell tumour in the sacral region
enteric (tailgut) cyst: for a purely cystic SCT
-<p><strong>Sacrococcygeal teratoma</strong> refers to a <a href="/articles/teratoma-1">teratoma</a> arising in the sacrococcygeal region. The coccyx is almost always involved <sup>6</sup>.</p><h4>Epidemiology</h4><p>It is the most common congenital tumour in fetus<sup> 11</sup> and neonate <sup>3</sup>. The incidence is estimated at ~1:35,000-40,000. There is a recognised female predilection with a M:F ratio of 1:4.</p><h5>Associations</h5><ul>-<li><p><a href="/articles/myelomeningocele-1">myelomeningocele</a></p></li>-<li><p><a href="/articles/vertebral-anomalies">vertebral anomalies</a></p></li>-</ul><h4>Clinical presentation</h4><p>Presentation varies depending on if a tumour has an intrapelvic location or has an extra-fetal extension (see further classification below). Intrapelvic tumours can manifest after birth with genitourinary and gastrointestinal symptoms given the compression of those structures. </p><h4>Pathology</h4><p>The sacrococcygeal region is the most common location for non-CNS teratomas. They are thought to arise from totipotent cells from the <a href="/articles/node-of-hensen">node of Hensen</a> <sup>1,3 </sup>at the anterior aspect of the coccyx by about the 2<sup>nd</sup> to 3<sup>rd</sup> weeks of gestation. They are most often mixed solid/cystic, although purely cystic types can occur in ~15% of cases.</p><p>A tumour is composed of all three germ cells (i.e. ectoderm, mesoderm and endoderm).</p><h5>Classification</h5><p>A pathology-based classification is as:</p><ul>-<li><p>benign (mature): much more common, comprising ~ 60-70%</p></li>-<li><p>malignant (immature)</p></li>-</ul><p>A location-based classification system according to the American Academy of Paediatric Surgery Section Survey is:</p><ul>-<li><p><strong>type I:</strong> developing only outside the fetus (can have small pre-sacral component); accounts for the majority of cases, 47% <sup>12</sup></p></li>-<li><p><strong>type II:</strong> extra-fetal with intrapelvic presacral extension</p></li>-<li><p><strong>type III:</strong> extra-fetal with extension through the pelvis into the abdomen</p></li>-<li><p><strong>type IV:</strong> tumour developing entirely in the fetal pelvis</p></li>-</ul><h5>Markers</h5><p>Can have elevated levels of:</p><ul>-<li><p><a href="/articles/alpha-fetoprotein-1" title="Serum AFP elevation">alpha-fetoprotein (AFP)</a></p></li>-<li><p><a href="/articles/beta-hcg-1" title="BHCG">beta HCG</a></p></li>-</ul><h5>Genetics</h5><ul><li><p>most cases tend to be sporadic <sup>12</sup></p></li></ul><h4>Radiographic features</h4><h5>Plain radiograph</h5><ul>-<li><p>may show a large mass projecting from the lower pelvic region or within the abdominopelvic cavity</p></li>-<li><p>may show calcification</p></li>-</ul><h5>Ultrasound</h5><p>Mature types tend to be more cystic which appear as anechoic components. Solid types (which are much rarer) often appear as an echogenic mass within the pelvis.</p><p>The correlation between sonographic appearances and malignant components is thought to be poor <sup>7</sup>.</p><p>Colour Doppler interrogation in some tumours may show marked hypervascularity with arteriovenous (AV) shunting.</p><h5>CT</h5><p>Not part of a routine investigation. Identifies bone, fat and cystic components. Calcification may again be seen.</p><h5>MRI</h5><p>Superior to ultrasound especially in the assessment of the following areas <sup>2</sup>:</p><ul>-<li><p>colonic displacement</p></li>-<li><p>ureteric dilatation</p></li>-<li><p>associated hip dislocation</p></li>-<li><p>intraspinal extension</p></li>-<li><p>vaginal dilatation</p></li>-<li><p>metastatic assessment in malignant lesions</p></li>-</ul><p>Signal characteristics can significantly vary depending on the constituent of the teratoma <sup>1</sup>.</p><ul>-<li><p><strong>T1:</strong> fat components appear high signal, calcific/bony components low signal</p></li>-<li><p><strong>T2:</strong> fluid (cystic) components appear high signal, calcific bony components low signal</p></li>-<li><p><strong>T2* GRE:</strong> magnetic susceptibility artefact because of calcifications</p></li>-<li><p><strong>T1 C+ (Gd):</strong> enhancing solid components</p></li>-</ul><h4>Treatment and prognosis</h4><p>A sacrococcygeal teratoma can be benign or malignant depending on whether they are mature or immature. The majority, however, tend to be benign (~80% <sup>11</sup>). Those presenting in older infants tend to have a higher malignant potential while those presenting in utero have a poor prognosis due to complications. Malignant change may be also more common in males.</p><p>Treatment is with surgical excision inclusive of coccygectomy with additional chemotherapy for malignant tumours <sup>5</sup>.</p><h5>Complications</h5><ul>-<li><p><a href="/articles/high-output-cardiac-failure">high output cardiac failure</a> from AV shunting: which in turn can cause <a href="/articles/hydrops-fetalis">hydrops fetalis</a></p></li>-<li><p>ureteric obstruction</p></li>-<li><p>gastrointestinal tract obstruction</p></li>-<li><p>compression of underlying nerves: giving urinary/faecal incontinence</p></li>-<li><p>anaemia</p></li>-<li><p><a href="/articles/dystocia">dystocia</a></p></li>-<li><p>tumour rupture</p></li>-</ul><h4>Differential diagnosis</h4><p>General imaging differential considerations include:</p><ul>-<li><p>sacral <a href="/articles/chordoma" title="Chordoma">chordoma</a></p></li>-<li><p><a href="/articles/terminal-myelocystocoele">terminal myelocystocoele</a>: for cystic types on ultrasound <sup>9</sup></p></li>-<li><p><a href="/articles/anterior-sacral-meningocele-1" title="Anterior sacral meningocele">sacral meningocele</a></p></li>-<li><p><a href="/articles/sacral-hemagioma">sacral haemangioma</a></p></li>-</ul><p>For type IV lesions also consider:</p><ul>-<li><p>low-lying <a href="/articles/neuroblastoma">neuroblastoma</a></p></li>-<li><p>low-lying <a href="/articles/rhabdomyosarcoma">rhabdomyosarcoma</a></p></li>-<li><p><a href="/articles/small-round-blue-cell-tumours">small round blue cell tumour</a> in the sacral region</p></li>-<li><p><a href="/articles/enteric-tail-gut-cyst">enteric (tailgut) cyst</a>: for a purely cystic SCT</p></li>- +<p><strong>Sacrococcygeal teratoma</strong> refers to a <a href="/articles/teratoma-1">teratoma</a> arising in the sacrococcygeal region. The coccyx is almost always involved <sup>6</sup>.</p><h4>Epidemiology</h4><p>It is the most common congenital tumour in fetus<sup> 11</sup> and neonate <sup>3</sup>. The incidence is estimated at ~1:35,000-40,000. There is a recognised female predilection with a M:F ratio of 1:4.</p><h5>Associations</h5><ul>
- +<li><p><a href="/articles/myelomeningocele-1">myelomeningocele</a></p></li>
- +<li><p><a href="/articles/vertebral-anomalies">vertebral anomalies</a></p></li>
- +</ul><h4>Clinical presentation</h4><p>Presentation varies depending on if a tumour has an intrapelvic location or has an extra-fetal extension (see further classification below). Intrapelvic tumours can manifest after birth with genitourinary and gastrointestinal symptoms given the compression of those structures. </p><h4>Pathology</h4><p>The sacrococcygeal region is the most common location for non-CNS teratomas. They are thought to arise from totipotent cells from the <a href="/articles/node-of-hensen">node of Hensen</a> <sup>1,3 </sup>at the anterior aspect of the coccyx by about the 2<sup>nd</sup> to 3<sup>rd</sup> weeks of gestation. They are most often mixed solid/cystic, although purely cystic types can occur in ~15% of cases.</p><p>A tumour is composed of all three germ cells (i.e. ectoderm, mesoderm and endoderm).</p><h5>Classification</h5><p>A pathology-based classification is as:</p><ul>
- +<li><p>benign (mature): much more common, comprising ~ 60-70%</p></li>
- +<li><p>malignant (immature)</p></li>
- +</ul><p>A location-based classification system according to the American Academy of Paediatric Surgery Section Survey is:</p><ul>
- +<li><p><strong>type I:</strong> developing only outside the fetus (can have small pre-sacral component); accounts for the majority of cases, 47% <sup>12</sup></p></li>
- +<li><p><strong>type II:</strong> extra-fetal with intrapelvic presacral extension</p></li>
- +<li><p><strong>type III:</strong> extra-fetal with extension through the pelvis into the abdomen</p></li>
- +<li><p><strong>type IV:</strong> tumour developing entirely in the fetal pelvis</p></li>
- +</ul><h5>Markers</h5><p>Can have elevated levels of:</p><ul>
- +<li><p><a href="/articles/alpha-fetoprotein-1" title="Serum AFP elevation">alpha-fetoprotein (AFP)</a></p></li>
- +<li><p><a href="/articles/beta-hcg-1" title="BHCG">beta HCG</a></p></li>
- +</ul><h5>Genetics</h5><ul><li><p>most cases tend to be sporadic <sup>12</sup></p></li></ul><h4>Radiographic features</h4><h5>Plain radiograph</h5><ul>
- +<li><p>may show a large mass projecting from the lower pelvic region or within the abdominopelvic cavity</p></li>
- +<li><p>may show calcification</p></li>
- +</ul><h5>Ultrasound</h5><p>Mature types tend to be more cystic which appear as anechoic components. Solid types (which are much rarer) often appear as an echogenic mass within the pelvis.</p><p>The correlation between sonographic appearances and malignant components is thought to be poor <sup>7</sup>.</p><p>Colour Doppler interrogation in some tumours may show marked hypervascularity with arteriovenous (AV) shunting.</p><h5>CT</h5><p>Not part of a routine investigation. Identifies bone, fat and cystic components. Calcification may again be seen.</p><h5>MRI</h5><p>Superior to ultrasound especially in the assessment of the following areas <sup>2</sup>:</p><ul>
- +<li><p>colonic displacement</p></li>
- +<li><p>ureteric dilatation</p></li>
- +<li><p>associated hip dislocation</p></li>
- +<li><p>intraspinal extension</p></li>
- +<li><p>vaginal dilatation</p></li>
- +<li><p>metastatic assessment in malignant lesions</p></li>
- +</ul><p>Signal characteristics can significantly vary depending on the constituent of the teratoma <sup>1</sup>.</p><ul>
- +<li><p><strong>T1:</strong> fat components appear high signal, calcific/bony components low signal</p></li>
- +<li><p><strong>T2:</strong> fluid (cystic) components appear high signal, calcific bony components low signal</p></li>
- +<li><p><strong>T2* GRE:</strong> magnetic susceptibility artefact because of calcifications</p></li>
- +<li><p><strong>T1 C+ (Gd):</strong> enhancing solid components</p></li>
- +</ul><h4>Treatment and prognosis</h4><p>A sacrococcygeal teratoma can be benign or malignant depending on whether they are mature or immature. The majority, however, tend to be benign (~80% <sup>11</sup>). Those presenting in older infants tend to have a higher malignant potential while those presenting in utero have a poor prognosis due to complications. Malignant change may be also more common in males.</p><p>Treatment is with surgical excision inclusive of coccygectomy with additional chemotherapy for malignant tumours <sup>5</sup>.</p><h5>Complications</h5><ul>
- +<li><p><a href="/articles/high-output-cardiac-failure">high output cardiac failure</a> from AV shunting: which in turn can cause <a href="/articles/hydrops-fetalis">hydrops fetalis</a></p></li>
- +<li><p>ureteric obstruction</p></li>
- +<li><p>gastrointestinal tract obstruction</p></li>
- +<li><p>compression of underlying nerves: giving urinary/faecal incontinence</p></li>
- +<li><p>anaemia</p></li>
- +<li><p><a href="/articles/dystocia">dystocia</a></p></li>
- +<li><p>tumour rupture</p></li>
- +</ul><h4>Differential diagnosis</h4><p>General imaging differential considerations include:</p><ul>
- +<li><p>sacral <a href="/articles/chordoma" title="Chordoma">chordoma</a></p></li>
- +<li><p><a href="/articles/terminal-myelocystocoele">terminal myelocystocoele</a>: for cystic types on ultrasound <sup>9</sup></p></li>
- +<li><p><a href="/articles/anterior-sacral-meningocele-1" title="Anterior sacral meningocele">sacral meningocele</a></p></li>
- +<li><p><a href="/articles/sacral-hemagioma">sacral haemangioma</a></p></li>
- +</ul><p>For type IV lesions also consider:</p><ul>
- +<li><p>low-lying <a href="/articles/neuroblastoma">neuroblastoma</a></p></li>
- +<li><p>low-lying <a href="/articles/rhabdomyosarcoma">rhabdomyosarcoma</a></p></li>
- +<li><p><a href="/articles/small-round-blue-cell-tumours">small round blue cell tumour</a> in the sacral region</p></li>
- +<li><p><a href="/articles/enteric-tail-gut-cyst">enteric (tailgut) cyst</a>: for a purely cystic SCT</p></li>
Image 1 Diagram ( create )
Image 2 X-ray (Frontal) ( update )
Image 3 X-ray (Frontal) ( update )
Image 5 Ultrasound (Sagittal) ( update )
Unable to process the form. Check for errors and try again.