Coccygeal teratoma is a prenatal tumor of the coccyx that is usually benign and associated with maldevelopment of the primitive streak. The tumor can be detected prenatally and treated if necessary. Prenatal treatment mainly stabilizes the circulation of the embryo.
What is a coccygeal teratoma?
Germ cell tumors are tumors that originate from the germ cells. In women, the tumors are usually benign. The male sex is more often affected by malignant forms. Coccygeal teratoma is the most common manifestation of germ cell tumor in infancy. The teratoma is located within a coccyx tumor in the area of the coccyx and corresponds to a malformation tumor of the spine. In some cases, the tumor infiltrates the fetal pelvis from the spine. Teratoma literally means “monster tumor.” Often, fittingly for this name, breech teratomas are already large at birth and visible to the naked eye. Although these are usually benign tumors, the risk of degeneration should not be underestimated. Large coccygeal teratomas are already visible prenatally by fine ultrasound. Depending on the degree of perfusion, even a previously benign breech bone tumor can have serious to lethal consequences.
Causes
Coccygeal teratoma is a malformation tumor. As this name implies, the growth has its cause in a malformation during embryonic development. The malformation involves the developmental phase of the primitive streak. The primitive streak actually regresses during gastrulation. Patients of coccygeal teratoma suffer from incomplete regression of the primitive streak. In coccygeal teratoma, the remnants of the strip become a tumor at the posterior end of the embryonic spine. The resulting tumor often causes defective unfolding of the individual cotyledons. This connection can disturb all possible tissues, organs and limbs of the embryo during development. Particularly heavily perfused coccygeal teratomas, for example, can result in heart failure and thus cause the unborn child to die.
Symptoms, complaints, and signs
The coccygeal teratoma may manifest as a mature tumor the size of a child’s head. In milder cases, the teratoma is not necessarily noticeable immediately after birth, but manifests only as mild swelling in the coccyx area. The teratoma may have a low or high blood supply. In cases of highly perfused coccygeal teratomas, the newborn often suffers from functional cardiac impairment. Pelvic teratomas often compress the bladder or bowel and cause associated concomitant symptoms. For example, particularly large, compressive teratomas may also cause pain. If there is infiltration of the spinal canal, there may be limited range of motion, usually due to the severe pain. In some cases, the pregnant woman also suffers symptoms while still pregnant. For example, mirror syndrome in pregnancy may be due to a breech bone teratoma of the unborn child. Abdominal pain, dyspnea, and massive edema formation have been observed in part as a reflection of fetal disease in the pregnant woman.
Diagnosis and course of the disease
The diagnosis of a large coccygeal teratoma is usually made prenatally by fine ultrasound. If the teratoma is not prominent in size, the physician makes the diagnosis postnatally. He usually develops an initial suspicion of the disseminated tumor by palpation findings. For diagnosis, he uses radiological methods in addition to blood tests. In the child’s blood, alpha-fetoprotein is determined in particular. Imaging such as sonography is used to accurately visualize the coccyx area. Imaging is used to determine the size of the growth in addition to its location. For fine diagnosis, magnetic resonance imaging is the tool of choice. At the same time, MRI helps physicians plan treatment steps. The prognosis for patients with coccygeal teratomas depends primarily on the malignancy and the degree of its perfusion. A prenatal time of diagnosis has a positive effect on prognosis.
Complications
In most cases, coccygeal teratoma does not cause any particular complications or discomfort. The tumor is usually benign in this case, so metastases do not develop. The coccygeal teratoma does not have to lead to complaints in every case.In many people, the symptom is discovered only by chance, although it may be manifested by a swelling of the coccyx. The swelling is often not associated with pain. However, due to the increased blood flow, this can cause heart problems, which can be severe, especially in newborns. Movement restrictions can also occur in this area and possibly negatively influence the child’s development. Furthermore, patients often suffer from abdominal pain or shortness of breath, so the coccyx teratoma must be removed in any case. Usually, there are no special complications during the removal and treatment of this tumor. However, chemotherapy is necessary afterwards, which is associated with side effects. Furthermore, the affected person is dependent on regular examinations in order to avoid further tumors. With successful treatment of coccygeal teratoma, there is no decreased life expectancy of the patient.
When should you see a doctor?
A visit to a doctor is necessary in any case of breech bone teratoma. Since it cannot heal on its own and in most cases the symptoms intensify if left untreated, the condition must always be treated by a doctor. Only early examination with subsequent treatment can prevent further complications. A doctor should be consulted in the case of coccyx teratoma if the patient experiences severe movement restrictions. In most cases, patients can no longer manage their daily lives on their own and are dependent on the help and support of their friends and family. Severe pain in the legs or severe shortness of breath can also indicate a coccyx teratoma. If these complaints occur permanently and above all without any particular reason, a doctor should be consulted. In most cases, severe and persistent fatigue or exhaustion also points to a coccyx teratoma and should also be examined by a medical professional. As a rule, the coccyx teratoma can be detected by a pediatrician or by a general practitioner. Further treatment is then based on exact symptoms and their severity.
Treatment and therapy
If the diagnosis of coccygeal teratoma is made before birth, prenatal fetal surgical procedures may be used. From the 20th to the 32nd week of gestation, for example, fetal surgical interventions can decrease blood flow to the teratoma and thus improve the circulatory situation of the unborn child. After birth, excision of the tumor should be attempted as soon as possible. In the case of prenatal diagnosis, therapy planning is carried out by an interdisciplinary team of pediatricians, gynecologists, pediatric surgeons and anesthesiologists with links to perinatal centers. The date of birth is usually determined by an interdisciplinary team so that all necessary preparations can be made for the best possible treatment. For excision, the circulatory situation of the child should be stable. For this reason, postnatal diagnosis worsens the prognosis, because in this case fetal surgical circulatory stabilizing measures can no longer be taken. During excision, in most cases, removal of the coccyx is indicated to eliminate the tumor as completely as possible. After excision, physicians decide whether chemotherapy is necessary in individual cases. After removal and follow-up treatment of the teratoma, children must attend regular checkups. Doctors of oncology and hematology care for the patients permanently in the outpatient clinic of a children’s hospital. Thus, if there is a recurrence, doctors detect the recurred tumor at the early stage and can intervene in time.
Prevention
Coccygeal teratoma is a malformation during embryonic development. To date, however, it remains unclear which factors are decisive for this maldevelopment. Therefore, the teratoma cannot be prevented promisingly so far. Nevertheless, fine ultrasound can be regarded as a kind of preventive measure, since teratomas of the child can be detected by imaging and pretreated prenatally.
Follow-up
Follow-up care for a coccygeal teratoma depends on the shape of the teratoma and the form of treatment chosen.The sooner the teratoma together with a part of the coccyx was surgically removed, the sooner the necessary medical follow-up can be determined. Ideally, the coccyx teratoma will show up before or directly at birth. After the surgical intervention and an analysis of the tissue, it must be determined whether further therapy is necessary. This may consist, for example, of chemotherapy, which itself entails various follow-up examinations and treatments. Once it has been established that nothing remains of the teratoma and that there are no more malignant tumor cells to be feared, follow-up care includes wound care and an examination of all parts of the body adjacent to the coccyx. In this way, it is checked whether the coccyx teratoma has affected the bladder or the intestine, for example. If necessary, surgery or therapy will be performed here. If, on the other hand, the coccyx teratoma appears in the first months or years of life, malignancy is much more likely. The aftercare that follows the treatment of such a coccygeal teratoma is similar to that following cancer therapy. Accordingly, medical follow-up here involves a great many examinations, including of the blood and by imaging techniques.
What you can do yourself
In the best case, a coccygeal teratoma can be diagnosed and removed before the baby is born. The mother should watch for warning signs and inform the gynecologist of any symptoms. If the tumor is too large, it will be removed immediately after the baby is born. The mother’s task is to carefully observe the child. If the surgical scar opens or the child shows other signs of pain or discomfort, call emergency medical services or the ambulance. Usually, however, a breech bone teratoma is relatively symptom-free. If it is detected and removed at an early stage, the child will not experience any further symptoms. However, a tumor disease of the child can be a heavy psychological burden for the parents and especially the mother. Affected parents should consult a therapist if they feel stressed or overwhelmed by the situation. The gynecologist or surgeon in charge can also put the child in touch with a therapist or psychologist. If necessary, the child may need to take pain medication or anti-inflammatories. Parents should seek support from a specialist or other parents for this.