Sacrococcygeal teratoma. The sacrococcygeal teratoma (in English sacrococcygeal teratoma or SCT), is the most frequent extragonadal germ cell tumor in childhood. Most are detected in the neonatal period as a tumor mass. Because these patients are prone to developing malignancies , it is important to obtain surgical treatment followed by clinical surveillance.
Summary
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- 1 Story
- 2 Pathological anatomy
- 3 Diagnosis
- 1 Congenital disorders
- 2 Malignancy
- 4 Differential diagnosis
- 5 Operative treatment
- 1 Urgent removal
- 6 Mortality
- 7 Conclusions
- 8 Sources
History
The term teratoma was introduced by Virchow, and means malformed. The tumor appears mainly in girls. Although in principle it is a tumor from childhood, cases have been described in the third decade of life. It can be cystic or solid, malignant or benign. The histology of the tumor (potentially malignant in all cases), ranges from conjoined twins to simple glial and mesodermal cell nests.
Because the sacrococcygeal teratoma is a rare tumor, with a frequency of 1 for every 40 thousand live births, and because it presents various anatomical dispositions, the surgeon rarely achieves a complete experience about the different aspects of the disease , however , in childhood constitutes the most common type of teratoma.
Its rarity and inferior anatomical location does not mean that its importance should be minimized. The need to clarify some of the existing confusions regarding the diagnosis , the operative moment and the type of surgical intervention and the long-term results of sacrococcygeal teratoma must be continually emphasized .
Sacrococcygeal teratoma is a treatable lesion in the newborn and may contain very malignant tissues. If it is carefully and completely removed (benign or malignant), the long-term results are generally excellent
Pathological anatomy
Glial tissue was found in the studied sample; brain fragments ; choroid plexus; peripheral nerves; smooth and striated muscle; embryonic and adult fat; mucous, sweat and sebaceous glands; squamous, cylindrical, and respiratory stratified epithelium; fibrous connective tissue; hair follicles ; pancreatic tissue; cartilage , and bone , heart muscle has rarely been found . Excluding malignant tumors , glial tissue is usually found in all anatomical parts.
Diagnosis
The main symptom is the existence of a globular mass (cystic or solid), of variable size, above the sacrum and the coccyx and which, when very large, displaces the anal opening. In all cases, a complete neurological examination should be performed to demonstrate the presence of any abnormality before the operation. Most of the patients do not suffer from neurological deficit. If the presence of a pelvic extension is suspected, a descending pyelogram should be performed. These examinations can demonstrate anterior and inferior displacement of the rectum, but without obstruction of this segment. Some of these patients may also suffer bilateral hydroureter and hydronephrosis.[[. Radiographic examination of the tumor shows calcifications in 35% of cases, which are rarely observed in malignant lesions, but calcification of teratomas is not a differential factor in stopping the absence of malignancy.
Congenital disorders
It has been proven that more than a third of patients with sacrococcygeal teratomas have other associated disorders, in these cases are: adenomatous polyps of the rectum, spindle cell sarcoma, hemivértebrae of the thoracic region (VIII-X and I-VI ), absence of the four distal segments of the coccyx and an omphalocele.
Malignancy
From the clinical point of view, two facts should be highlighted:
- Only malignant teratomas have a solid structure and consistency, with no cystic component.
- In most malignant tumors, the neoplasm is intimately linked to the coccyx and has a hidden part that extends to a variable distance within the pelvis .
Differential diagnosis
Other tumors of the sacrococcygeal region that can be confused with teratoma are sacral meningocele, abscesses , chordomas, neurofibromas, ganglioneuromas, ependymomas, and cystic hemangiomas. The most easily confused lesion is non-communicating sacral meningocele, because the current differential signs of spina bifida are not appreciated.and communication with the spinal canal. Meningocele is extraordinarily attached to the lamina terminalis by a fibrous band. The apparent absence of spina bifida is due to the radiological difficulty in making this diagnosis in the newborn. In reality, some patient with a teratoma will undergo surgery with a preoperative diagnosis of sacral meningocele, this should not be a problem as long as it precedes the operation, the necessary reflection and careful preparation.
Operative treatment
In general, the intention of this communication goes beyond commenting on all aspects of the surgical removal of this tumor, however, we must cite some fundamental principles.
- The transverse incision of the skin of the buttocks is the route of choice. It maintains the suture line at a reasonable distance from the anus and allows the proper resection of the coccyx and at the same time the correct exposure of the rectum . Wound infections have only occurred in a small group of patients who underwent a vertical skin incision. It is important not to close the wound with tension, since the suture line may become necrotic. Occasionally, the double-0 Y-shaped incision “in a letter envelope”, recommended by Swenson, has been useful.
- The coccyx must always be removed along with the tumor.
- Before the operation, and under local anesthesia, a plastic tube as large as possible should be placed in the anterior ulnar vein. Endotracheal anesthesia is recommended in all cases, due to the patient’s position in the prone position . The anesthetic of choice is Halothane, mixed with small amounts of ether as necessary to stimulate respiratory movements.
- Efforts should be made to maintain body temperature and adequate support of the chest . The shoulders should be raised with rolled towels, to allow normal chest and abdominal movements.
- A water blanket should always be used to regulate the temperature appropriately (recorded by a thermoelectric pair placed in the esophagus ). It is useful, but not absolutely necessary, to obtain a continuous electrocardiogram during the intervention. The loss of blood during the operation can be considerable, ranging from 30 to 3,000 cubic cm, with an average of 175, this requires dissecting a vein before of the operation. Since the tumor is intimately attached to the rectum, dissection is facilitated by filling this organ, preoperatively, with Vaseline gauze. An additional aid during dissection is the use of a nerve stimulator, which helps to identify the sacral plexus.
Urgent excisions
Almost a third of patients have large, dilated veins and necrotic areas of skin over the tumor, which may be the cause of intense preoperative bleeding that requires emergency intervention to restrain bleeding .
Mortality
In general, the average mortality for surgical treatment before one month of age is 18%, while it rises to 55% if started later. These figures support the other reasons that suggest early intervention. The mortality in benign recurrences is double (12% versus 25%) and due to malignant relapse is increased up to five times (6% vs. 30%), in cases operated after the first month of life. The appearance of malignant sacrococcygeal teratomas in the combined series is slightly more than double, after the first month of life (18% vs. 40%). This is not to say that these statistics support the belief that malignant degeneration occurs in older tumors, but more abundantly, there is no reason to wait until the child is older to intervene.
Conclusions
Most sacrococcygeal teratomas appear in children . The best results are obtained through careful preoperative evaluation and immediate removal in the newborn . If a prolonged tumor in the pelvis is suspected by physical examination, including digital rectal examination , a descending pyelogram and a barium enema should be performed before the operation . Tumors with large pelvic prolongation that cause hydronephrosis should be operated by a combined abdominosacral route in a single time, in order to facilitate the complete removal of the tumor and avoid injury to the urinary tract. The removal of the coccyx together with the tumor is mandatory if success is to be achieved.
If the patient undergoes surgery before the first month of life, a frequency of malignancy of 18% is found, while if the treatment is applied after the first month, 40% of malignant cases are found. In those patients whose treatment was delayed beyond the first month of life, the total mortality was higher. Tumors with large superficial venous trunks should be considered as surgical emergencies, to avoid rupture of these vessels and possible appearance of serious bleeding. Long-distance observations have shown that patients have normal intelligence and physical and mental development. Since these are potentially normal individuals, it is important, when they are born, to adequately assess the character and extent of the lesion and to remove the tumor completely and early.
