Dermoid Tumor

Dermoid Tumor

Summarize 

What is a teratoma?

 

        In the human body, there is a type of cell called a pluripotent germ cell that normally develops into an egg or a sperm. When the growth process of the pluripotent germ cell goes wrong, it can form a tumor called a germ cell tumor.

        The cause of germ cell tumors can be traced back to the process of conception.

        In the mother's womb, a fertilized egg develops into a child. It is like planting a seed; it sprouts, grows a main stem, branches out, and eventually produces leaves and flowers. Similarly, a fertilized egg undergoes differentiation, starting with an undifferentiated stem cell (the main stem), branching out into several types of pluripotent stem cells (branches), and finally developing into various organs and tissues (leaves and flowers). This process, known as differentiation, requires precise regulation. Any errors can lead to various diseases.

        Pluripotent germ cells are supposed to migrate to the gonads (ovaries in females and testes in males) during fetal development, where they develop into eggs and sperm during puberty. However, during their migration in the embryo, they may mistakenly enter other areas and develop into tumors in the wrong environment; or even if they reach the correct location, they may develop into tumors due to errors during further development.

        Although called pluripotent germ cells, they actually have a great potential to differentiate into many tissues (such as skin, teeth, hair, muscle, bone, etc.) rather than being limited to germ cells.

        Teratomas are the most common type of germ cell tumor, accounting for the majority of germ cell tumors that occur in various parts of the body. The most distinctive feature of teratomas is that they contain multiple different tissues, such as hair or teeth.

        Clinically, in addition to simple teratomas, there are many cases of teratoma mixed with other germ cell tumors, such as mixed yolk sac tumor or choriocarcinoma.

Epidemiological

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Etiology & Risk Factors

1.1 Where do teratomas occur?

 

        In young children, teratomas often develop outside the gonads, resulting from the migration of multipotent germ cells to incorrect locations. The most common site is the sacrococcygeal region, where the coccyx is located. Teratomas can also occur in the gonads, neck and face, mediastinum (the area between the two lungs), and retroperitoneum. Most teratomas are found along the body's midline and rarely develop in the limbs.

        Teratomas are most commonly found in the ovaries and testes of older children and adolescents. Among pediatric ovarian and testicular tumors, teratomas are the most common type, often resulting from abnormal differentiation of pluripotent germ cells. Additionally, teratomas can also develop in other areas, such as the brain (intracranial).

Classification & Staging

How are teratomas classified?

 

        Teratomas are mainly divided into mature teratomas and immature teratomas.

        Mature teratomas contain well-differentiated cells, such as skin, hair, muscle, fat, and possibly parts of organs and limbs.

        Immature teratomas contain poorly differentiated cells, similar to flowers that have grown branches but stopped growing leaves and flowers. These cells are more primitive, with strong division potential and a higher degree of malignancy. Immature teratomas can be mixed, meaning they contain both well-differentiated and poorly differentiated cells, as well as other types of germ cells. Some may secrete hormones or specific proteins.

 

 

Is a "tacit fetus" a teratoma?

 

        Sometimes we see news like this, a child with another "fetus" inside him, with hands and feet, and even some formed organs.

        Whether this rare "tacit fetus" is a teratoma is still controversial.

        Some people believe that 'embryonic embryo' is a type of 'parthenogenetic embryo.' It forms when, in early pregnancy, the mother carries twins, with one being enveloped by the other and ceasing development at some stage. In this case, 'embryonic embryo' does not originate from the patient's own pluripotent germ cells and is not a teratoma.

        Others believe that "tacit fetus" is a teratoma, which is a very differentiated, mature teratoma with multiple tissues and organs.

 

 

Are teratomas benign or malignant?

 

        It depends on the classification and specific condition of the teratoma. Mature teratomas are benign. In very rare cases, mature teratomas may become malignant, possibly developing into squamous cell carcinoma or carcinoid, which almost only occurs in adults.

        Immature teratomas may be malignant. Immature teratomas can be classified into grades 0 to 3, depending on the amount of immature neural tissue in the tumor, with higher grades being more malignant.

        Interestingly, in rare cases, some immature teratomas may gradually differentiate and mature during the recurrence process. This means that the tumor cells transform from a poorly differentiated type to a well-differentiated type, essentially converting from malignant to benign. In individual cases, immature teratomas located in the ovaries or brain may also transform into mature teratomas.

Clinical Manifestations

How to find a teratoma and what are the symptoms?

 

        The most common symptom of a teratoma is a lump, which can be felt with the hand in larger cases and may be accompanied by pain.

        But the symptoms of each patient are mainly determined by the size and location of the tumor:

        If the mass is located in the sacrococcygeal region, it may compress the intestines and cause constipation, or compress the bladder and cause urinary incontinence, or compress nerves and cause weakness in the legs.

        If the mass is on the ovary, it may compress nerves and cause abdominal pain or vomiting; it may also twist due to changes in position or movement, leading to sudden severe abdominal pain.

        If the lump is large and located in the neck or chest, it may cause difficulty breathing.

        If the mass is in the brain, depending on the area of the brain it is pressing on, it can cause symptoms such as headaches, vomiting, convulsions or slurred speech.

Clinical Department

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Examination & Diagnosis

To diagnose a teratoma, the doctor will take a medical history and perform a physical examination. They will also conduct a series of tests, including blood tests to check for tumor markers, imaging tests to examine the tumor's shape, and histological tests to examine the details of the tumor cells. If the teratoma is located in the brain, additional neurological and cerebrospinal fluid tests will be necessary. These tests are all crucial.

 

 

2.1 What are the markers of teratoma?

 

        Teratomas have two tumor markers: alpha-fetoprotein (AFP) and human chorionic gonadotropin (beta-HCG). To check these markers, blood is usually drawn.

 

        The alpha-fetoprotein is a protein that is secreted by the fetus while in the womb and should drop rapidly within a few weeks after birth. If the alpha-fetoprotein level in the blood is found to be too high, it is considered abnormal and likely to be associated with tumors.

        Human chorionic gonadotropin is a hormone secreted by the placenta that can be measured in the urine and blood of women with normal pregnancies. A positive test in other cases is likely to be associated with germ cell tumors.

 

        Cells in mature teratomas do not express these markers, so blood tests for both are negative (normal).

        The levels of these two markers are elevated (abnormal) in the blood of patients with malignant transformation of immature and mature teratomas.

        These two indicators can not only be used to detect and diagnose diseases, but also to assess the effectiveness of treatment. If the decrease is significant, it indicates that the treatment is effective; if the increase after treatment is reappeared, we should pay attention to whether there is tumor recurrence.

        But these two markers can also occur in other tumors, so even if an abnormality is found, it's important to carefully identify which tumor it is.

 

 

2.2 What are the imaging examinations for teratomas?

 

        There are a lot of them, including B ultrasound, X-ray, CT, MRI (magnetic resonance imaging), etc. These imaging tests allow doctors to visually observe the tumor, not only the overall tumor, but also its internal condition, as well as the relationship between the tumor and surrounding organs.

B ultrasound can be used to detect teratomas. If the tumor is large enough, it can be detected while the child is still in the womb.

CT can effectively differentiate tissues of varying textures, while MRI is better at distinguishing between liquids and soft tissues. Together, these imaging techniques can help observe the texture and composition of tumors: mature teratomas are typically cystic (hollow) with clear boundaries, and may contain internal structures such as teeth or hair. Immature teratomas, on the other hand, are mostly solid (solid) with irregular edges and varying amounts of fat and irregular calcifications. These imaging tests can provide an initial assessment of the malignancy of teratomas.

        Imaging tests can also be used to see if the teratoma has metastasized: chest CT or X-rays can be used to examine the lungs, MRI can be used to examine the brain, and bone scans can be used to examine the entire skeleton to see if the tumor has metastasized to these sites.

 

 

2.3 What does histological examination look for?

 

        Histological examination (also called pathological examination) is a microscopic examination of a section of tumor tissue. The section of tumor tissue may come from surgery or a biopsy performed before surgery.

        According to the type and morphology of the cells, doctors classify teratomas, stage them, and select treatment methods. The previously mentioned mature and immature teratomas, as well as malignant transformation of mature teratomas, all require histological examination for final diagnosis.

 

 

2.4 What other tests are needed for intracranial teratoma?

 

        In addition to the above tests, intracranial teratomas can affect the nervous system by compressing the brain and require a series of additional tests, including neurological reflexes, body coordination, mobility, visual field, language ability, etc.

        Additionally, doctors may perform a lumbar puncture —— to obtain the patient's cerebrospinal fluid (the fluid that nourishes nerve cells in the brain and spinal cord). By examining the cerebrospinal fluid, doctors can not only observe for tumor cells under a microscope but also detect tumor markers such as alpha-fetoprotein (AFP) or human chorionic gonadotropin (beta-HCG).

Clinical Management

In the face of teratomas, the big boss, what are some ways to fight them? Let's look at them in categories.

 

 

How to treat mature teratoma?

 

        At present, the primary treatment for mature teratomas is surgical treatment to remove the tumor as much as possible. Of course, the treatment plan will vary depending on the type and location of the tumor.

        For mature teratomas located in the sacrococcygeal region, the primary treatment methods are surgery and postoperative monitoring. However, it is important to remember to have regular follow-up examinations for several years after surgery. During these follow-ups, checking the levels of tumor markers in the serum can help monitor for any recurrence of the tumor. Two markers commonly tested for teratomas are alpha-fetoprotein (AFP) and human chorionic gonadotropin (beta-HCG).

        Although 43%-50% of the recurrent tumors are malignant, if detected early, the recurrent malignant tumors can be well treated by surgery and chemotherapy. Therefore, postoperative review is very important!!!!

        For mature teratomas that grow in other parts of the body, the main treatments are surgery and postoperative observation.

        Patients with mature teratomas generally have a good prognosis after surgical treatment.

 

 

How to treat immature teratoma?

 

        In addition to surgery and postoperative observation, chemotherapy is sometimes needed for the treatment of immature teratomas according to the specific situation of the tumor. Factors that need to be considered include whether the tumor is completely removed and the grading of malignancy.

        Commonly used chemotherapy regimens are BEP (bephenamine, etoposide and cisplatin) and JEB (bephenamine, etoposide and carboplatin).

        For patients with low-risk teratomas, the survival rate can reach 94%-100% after four courses of BEP treatment. For patients with high-risk teratomas, high-dose BEP treatment can also have a good overall survival rate, but at the cost of increased toxicity and side effects.

 

 

How to treat intracranial teratoma?

 

        The treatment of teratomas in the brain mainly includes surgical treatment and adjuvant treatment, which includes chemotherapy and radiotherapy.

        Surgical treatment for intracranial teratomas is best when it can be completely removed. However, the challenge lies in the fact that tumors can be located in various parts of the brain. Some can be entirely removed through surgery, while others require partial removal. For instance, if a tumor is completely removed, it might affect important brain functions. In such cases, the pros and cons must be carefully weighed, and only partial removal may be chosen.

        For intracranial mature teratomas, there is no consensus on whether adjuvant therapy should be added if the tumor has been completely removed by surgery. For patients who can only partially remove the tumor by surgery, adjuvant therapy is generally recommended.

        For intracranial immature teratomas, even if they can be completely removed by surgery, adjuvant therapy should be added. However, it is complicated that immature teratomas have very different sensitivity to adjuvant therapy, which directly affects the treatment effect.

 

        There is currently no unified guideline for the treatment of teratomas. Treatment philosophies can vary among different hospitals and doctors. These are just basic treatment methods. For each patient, the specific teratoma treatment plan will vary based on the condition and should be based on the doctor's advice. We also hope that a treatment guideline for teratomas will be released soon.

Prognosis

What is the prognosis of different teratomas?

 

 

        Overall, the prognosis for teratomas is good: overall survival rates for children with immature teratomas are over 80%, and for adults over 90%. The prognosis is even better for patients with mature teratomas.

        However, when it comes to individual cases, the prognosis of teratomas is influenced by several factors: the immature grade of the teratoma, whether it has progressed, and the location of the tumor. Some types of teratomas have a less favorable prognosis. For instance, a study on 13 cases of immature intracranial teratomas reported a 5-year survival rate of only about 40%. The survival rate for fetuses with congenital intracranial teratomas is even lower.

        In addition, the prognosis of congenital teratomas is also related to the duration of pregnancy. For example, if an infant with a teratoma in the sacrococcygeal region is born prematurely before 30 weeks of gestation, the survival rate is only 7%, while the survival rate of infants who can survive beyond 30 weeks is 75%.

        During and after the treatment of teratomas, various issues may arise, such as the recurrence of teratomas in the same or adjacent areas, the development of germ cell tumors, and the less common Growing Teratoma Syndrome. Early detection of these issues is crucial for better outcomes. Therefore, regular follow-ups and long-term monitoring are essential after treatment!!!

 

        Growth-type teratoma syndrome: During or after chemotherapy, some patients with teratomas may experience an increase in the number or size of their tumors. If serum markers such as alpha-fetoprotein and human chorionic gonadotropin are normal, and the pathological examination confirms a benign mature teratoma, it may indicate growth-type teratoma syndrome. There are various explanations for this syndrome. It is important to note that tumors in patients with growth-type teratoma syndrome are generally not sensitive to chemotherapy and radiotherapy, and surgery remains the primary treatment. Therefore, during clinical diagnosis, it is crucial to differentiate these cases from other germ cell tumors that are more responsive to chemotherapy.

 

 

What are the long-term effects of teratoma treatment?

 

        In addition to the disease itself, chemotherapy can lead to chronic respiratory diseases, hearing damage, or other health issues during treatment. Therefore, long-term care for patients is very important. This has also been mentioned in previous articles by Sunflower Children. [Link: Article on Long-term Chronic Diseases https://mp.weixin.qq.com/s/4xVpLynIWBWHk2v7JrPfHA]

        Additionally, teratomas often occur in reproductive organs, such as the ovaries in women and the testes in men. Ideally, surgery should completely remove the tumor without damaging surrounding tissues. However, when drugs may affect reproductive organs, it is crucial to consider how to protect the future fertility of pediatric patients, such as by developing techniques to preserve the ovaries or testes. [Link: Saving the Future Fertility of Children https://mp.weixin.qq.com/s/mhzmQ30P350d9GFIWW-WKA]

Follow-up & Review

Patient family member experience sharing

 

        The treatment of teratomas in the cranium is much more troublesome than that outside the cranium, and once they recur, craniotomy is faced. For treatment, the most important thing is to try to perform total surgery.

        According to our experience, if adjuvant chemotherapy is used before surgery for immature teratomas, the tumor volume can be reduced, which is conducive to complete resection of the tumor and also beneficial to prevent recurrence. Now some doctors will use the "sandwich" scheme of radiotherapy and chemotherapy, surgery and re-chemotherapy.

        We have also learned that some children with intracranial immature teratomas can still lead a normal life after surgery, radiotherapy and chemotherapy, or even a second operation.

        As parents, what we can do is to pay more attention to new medical knowledge and technology, pay attention to children's nutrition in daily life, improve children's immunity. I hope all children can grow up healthily and happily!

 

 

Daily Care

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Cutting-edge therapeutic and clinical Trials

Are there any clinical trials for teratomas?

 

        Of course!

        First, let's discuss the chemotherapy mentioned earlier. A Phase III clinical trial is studying the effectiveness of bleomycin, carboplatin, etoposide, or cisplatin in treating germ cell tumors in both children and adults (clinical trial number NCT03067181). Additionally, several clinical trials are exploring the therapeutic effects of targeted drugs and immunotherapies on teratomas.

        Many people have heard of the renowned tumor immunotherapy drug —— Opdivo (O) or Opdivo. This is a PD-1 antibody. A clinical trial used Opdivo in combination with Yervoy (ipilimumab, an anti-CTLA-4 antibody) to treat rare tumors, including malignant teratomas. (Clinical trial number NCT02834013)

        In terms of tumor-targeted therapy, Palbociclib and ribociclib are both CDK4/6 inhibitors that have been approved for marketing. Ribociclib has completed a phase II clinical trial in patients with teratomas, although the results have not yet been published. (Clinical trial number NCT02300987)

Additionally, among patients with mature teratomas, some are unable to undergo surgery for various reasons, and there is currently a lack of effective treatment options. In a phase II clinical trial, 12 patients with mature teratomas who were not eligible for surgery were treated with palbocitinib. The results showed that palbocitinib treatment provided clinical benefits to these patients. Although the number of cases was small, this suggests that palbocitinib may be a viable option for treating mature teratomas in patients who are not suitable for surgery.

 

        It is believed that these clinical trials will bring more effective and relatively less side effects to teratomas, especially those with poor current treatment results. Let's look forward to new ways to fight monsters!

References

 reference material ：

1. Childhood Extracranial Germ Cell Tumors Treatment. https://www.cancer.gov/types/extracranial-germ-cell/hp/germ-cell-treatment-pdq

2. Childhood Central Nervous System Germ Cell Tumors Treatment. https://www.cancer.gov/types/brain/hp/child-cns-germ-cell-treatment-pdq

3. Immature teratoma presenting as a soft-tissue mass with no evidence of other sites of involvement: a case report. Diagn Pathol. 2016; 11: 76. doi: 10.1186/s13000-016-0527-x

4. Malignant Transformation in a Mature Teratoma with Metastatic Deposits in the Omentum: A Case Report. Case Reports in Pathology. Volume 2012, Article ID 568062. doi: 10.1155/2012/568062

5. Teratoma with malignant transformation: report of three cases and review of the literature.

. Clin Imaging. 2014 Sep-Oct;38(5):589-93. doi: 10.1016/j.clinimag.2014.04.011.

6. Immature Teratoma – an Overview https://www.sciencedirect.com/topics/agricultural-and-biological-sciences/immature-teratoma

7. Maturation of intracranial immature teratomas. J Neurosurg. 1996 Oct;85(4):672-6. doi: 10.3171 /jns.1996.85.4.0672.

8. Clinical and pathological characteristics of malignant transformation of ovarian mature teratoma http://news.ipathology.cn/article/3317.html

9. Pediatric Teratomas and Other Germ Cell Tumors. https://emedicine.medscape.com/article/939938-overview

10. Fetus in fetu: Review of the literature over the past 15 years. Journal of Pediatric Surgery Case Reports. Volume 3, Issue 12, December 2015, Pages 554-562. https://doi.org/10.1016/j.epsc.2015.10.006

11. Pediatric Teratomas and Other Germ Cell Tumors. https://emedicine.medscape.com/article/939938-overview

12. Treatment and outcomes of primary intracranial teratoma. Childs Nerv Syst. 2009 Dec;25(12):1581-7. doi: 10.1007/s00381-009-0974-8.

13. Growing Teratoma Syndrome. Indian J Surg Oncol (March 2017) 8(1):46–50. doi: 10.1007/s13193-016-0568-3

14. Diagnosis and Treatment of Intracranial Immature Teratoma. Pediatr Neurosurg 2009;45:354–360 . doi: 10.1159/000257524.

15. Cyclin-Dependent Kinase 4/6 Inhibition for the Treatment of Unresectable Mature Teratoma: Long-Term Follow-Up of a Phase II Study. Clin Genitourin Cancer. 2016 Dec;14(6):504-510. Doi: 10.1016/j.clgc.2016.03.010.

16. https://clinicaltrials.gov/ct2/show/NCT02834013

17. https://clinicaltrials.gov/ct2/show/NCT03067181

18. https://clinicaltrials.gov/ct2/show/NCT02300987

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