TERATOMA

Teratoma
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Teratoma
Classification and external resources
ICD-O: 9080
DiseasesDB 3604 12952 12966
eMedicine med/3449
MeSH D013724
Look up teratoma in Wiktionary, the free dictionary.
A teratoma, is an encapsulated tumor with tissue or organ components resembling normal derivatives of all three germ layers. There are rare occasions when not all three germ layers are identifiable. The tissues of a teratoma, although normal in themselves, may be quite different from surrounding tissues, and may be highly disparate; teratomas have been reported to contain hair, teeth, bone and very rarely more complex organs such as eye,[1][2] torso,[3][4] and hands, feet, or other limbs.[5] Usually, however, a teratoma will contain no organs but rather one or more tissues normally found in organs such as the brain, thyroid, liver, and lung. Sometimes, the teratoma has within its capsule one or more fluid-filled cysts and when a large cyst occurs there is a potential for the teratoma to produce a structure within the cyst that resembles a fetus. Because they are encapsulated, teratomas are usually benign, although several forms of malignant teratoma are known and some of these are common forms of teratoma. A mature teratoma is typically benign and found more commonly in females, while an immature teratoma is typically malignant and is more often found in males.

Teratomas are thought to be present at birth (congenital), but small ones often are not discovered until much later in life.

Definitive medical diagnosis of a teratoma is based on its histology; this diagnosis is made by a pathologist.

Contents [hide]
1 Etymology
2 Natural history
2.1 Location and incidence
2.2 Hypotheses of origin
2.3 Mature teratoma
2.4 Dermoid cyst
2.5 Fetus in fetu and fetiform teratoma
2.6 Struma ovarii
3 Pathology classification of individual teratomas
3.1 "Benign" teratoma may prove to be malignant
3.2 Teratoma with malignant transformation
3.3 Extraspinal ependymoma
4 Initial diagnosis
5 Time of Presentation
6 Complications
7 Treatment
7.1 Surgery
7.2 Chemotherapy
7.3 Clinical trials
7.4 Follow-up
8 Use in basic research
9 Teratoma in non-humans
10 See also
11 References
12 External links


[edit] Etymology
The word teratoma comes from classical Greek and means roughly "monstrous tumor".

There are some differences in terminology in the US and UK. The words "teratoma" (US terminology) and "mature teratoma" (UK terminology) both may be used to refer to a benign growth, while the word "teratoma" (UK terminology) may refer to "immature teratoma", a cancerous growth. It is therefore important to specify whether one is using US or UK terminology.

The term "malignant teratoma" has sometimes been used as a synonym for non-seminomatous germ cell tumor.[6]

[edit] Natural history
Main article: Germ cell tumor
Teratomas belong to a class of tumors known as nonseminomatous germ cell tumor (NSGCT). All tumors of this class are the result of abnormal development of pluripotent cells: germ cells and embryonal cells. Teratomas of embryonal origin are congenital; teratomas of germ cell origin may or may not be congenital (this is not known). The kind of pluripotent cell appears to be unimportant, apart from constraining the location of the teratoma in the body.

[edit] Location and incidence
Teratomas derived from germ cells occur in the testes in males and ovaries in females. Teratomas derived from embryonal cells usually occur on the body midline: in the brain, elsewhere inside the skull, in the nose, in the tongue, under the tongue, and in the neck (cervical teratoma), mediastinum, retroperitoneum, and attached to the coccyx. However, teratomas may also occur elsewhere: very rarely in solid organs (most notably the heart and liver) and hollow organs (such as the stomach and bladder), and more commonly on the skull sutures. Embryonal teratomas most commonly occur in the sacrococcygeal region: sacrococcygeal teratoma is the single most common tumor found in newborn babies.

Of teratomas on the skull sutures, approximately 50% are found in or adjacent to the orbit.[7] Limbal dermoid is a choristoma, not a teratoma.

Teratoma qualifies as a rare disease, but is not extremely rare. Sacrococcygeal teratoma alone is diagnosed at birth in 1 out of 40,000 babies. Given the current world population birth rate, this equals 5 per day or 1800 per year. Add to that number sacrococcygeal teratomas diagnosed later in life, and teratomas in other locations, and the incidence approaches 10,000 new diagnoses of teratoma per year.

Teratoma also occurs, rarely, in non-human animals.[8]

[edit] Hypotheses of origin
Concerning the origin of teratomas, there exist numerous hypotheses.[9] These hypotheses are not to be confused with the unrelated hypothesis that fetus in fetu (see below) is not a teratoma at all but rather a parasitic twin.

[edit] Mature teratoma
A mature teratoma is a grade 0 teratoma. Mature teratomas are highly variable in form and histology, and may be solid, cystic, or a combination of solid and cystic. A mature teratoma often contains several different types of tissue such as skin, muscle, and bone. Skin may surround a cyst and grow abundant hair (see Dermoid cyst). Mature teratomas generally are benign; malignant mature teratomas are of several distinct types.

[edit] Dermoid cyst

A small (4 cm) dermoid cyst of an ovary, discovered during a C-sectionA dermoid cyst is a mature cystic teratoma containing hair (sometimes very abundant) and other structures characteristic of normal skin and other tissues derived from the ectoderm. The term is most often applied to teratoma on the skull sutures and in the ovaries of females.

[edit] Fetus in fetu and fetiform teratoma
Fetus in fetu and fetiform teratoma are rare forms of mature teratoma that include one or more components resembling a malformed fetus. Both forms may contain or appear to contain complete organ systems, even major body parts such as torso or limbs. Fetus in fetu differs from fetiform teratoma in having an apparent spine and bilateral symmetry.[9]

Most authorities agree that fetiform teratomas are highly developed mature teratomas; the natural history of fetus in fetu, however, is controversial.[9] There also may be a cultural difference, with fetiform teratoma being reported more often in ovarian teratomas (by gynecologists) and fetus in fetu being reported more often in retroperitoneal teratomas (by general surgeons). Fetus in fetu has often been interpreted as a fetus growing within its twin. As such, this interpretation assumes a special complication of twinning, one of several grouped under the term parasitic twin. In this regard, it is noteworthy that in many cases the fetus in fetu is reported to occupy a fluid-filled cyst within a mature teratoma.[10][11][12][13] Cysts within mature teratoma have also been reported to contain a rudimentary beating heart.[14]

Regardless of whether fetus in fetu and fetiform teratoma are one entity or two, they are distinct from and not to be confused with ectopic pregnancy.

[edit] Struma ovarii
Main article: Struma ovarii
A struma ovarii (literally: goitre of the ovary) is a rare form of mature teratoma that contains mostly thyroid tissue.

[edit] Pathology classification of individual teratomas
Regardless of location in the body, a teratoma is classified according to a cancer staging system. This indicates whether chemotherapy or radiation therapy may be needed in addition to surgery. Teratomas commonly are classified using the Gonzalez-Crussi[9] grading system: 0 or mature (benign); 1 or immature, probably benign; 2 or immature, possibly malignant (cancerous); and 3 or frankly malignant. If frankly malignant, the tumor is a cancer for which additional cancer staging applies.

Teratomas are also classified by their content: a solid teratoma contains only tissues (perhaps including more complex structures); a cystic teratoma contain only pockets of fluid or semi-fluid such as cerebrospinal fluid, sebum, or fat; a mixed teratoma contains both solid and cystic parts. Cystic teratomas usually are grade 0 and, conversely, grade 0 teratomas usually are cystic.

Grade 0, 1 and 2 pure teratomas have the potential to become malignant (grade 3), and malignant pure teratomas have the potential to metastasize. These rare forms of teratoma with malignant transformation may contain elements of somatic (non germ cell) malignancy such as leukemia, carcinoma or sarcoma.[15] A teratoma may contain elements of other germ cell tumors, in which case it is not a pure teratoma but rather is a mixed germ cell tumor and is malignant. In infants and young children, these elements usually are endodermal sinus tumor, followed by choriocarcinoma. Finally, a teratoma can be pure and not malignant yet highly aggressive: this is exemplified by growing teratoma syndrome, in which chemotherapy eliminates the malignant elements of a mixed tumor, leaving pure teratoma which paradoxically begins to grow very rapidly.

[edit] "Benign" teratoma may prove to be malignant
A "benign" grade 0 (mature) teratoma nonetheless has a risk of malignancy. Recurrence with malignant endodermal sinus tumor has been reported in cases of formerly benign mature teratoma,[16][17] even in fetiform teratoma and fetus in fetu.[18][19] Squamous cell carcinoma has been found in a mature cystic teratoma at the time of initial surgery.[20]

A grade 1 immature teratoma that appears to be benign (e.g., because AFP is not elevated) has a much higher risk of malignancy, and requires adequate follow-up.[21][22][23][24] This grade of teratoma also may be difficult to diagnose correctly. It can be confused with other small round cell neoplasms such as neuroblastoma, small cell carcinoma of hypercalcemic type, primitive neuroectodermal tumor, Wilm's tumor, desmoplastic small round cell tumor, and non-Hodgkin lymphoma.[25]

[edit] Teratoma with malignant transformation
A teratoma with malignant transformation or TMT is a very rare form of teratoma that may contain elements of somatic (non germ cell) malignant tumors such as leukemia, carcinoma or sarcoma.[15] Of 641 children with pure teratoma, nine developed TMT:[26] five carcinoma, two glioma, and two embryonal carcinoma (here, these last are classified among germ cell tumors).

[edit] Extraspinal ependymoma
Extraspinal ependymoma, usually considered to be a glioma (a type of non-germ cell tumor), may be an unusual form of mature teratoma.[27]

[edit] Initial diagnosis
Teratomas are thought to be present since birth, or even before birth, and therefore can be considered congenital tumors. However, many teratomas are not diagnosed until much later in childhood or in adulthood. Large tumors are more likely to be diagnosed early on. Sacrococcygeal and cervical teratomas are often detected by prenatal ultrasound. Additional diagnostic methods may include prenatal MRI. In rare circumstances, the tumor is so large that the fetus may be damaged or die. In the case of large sacrococcygeal teratomas, a significant portion of the fetus' blood flow is redirected toward the teratoma (a phenomenon called steal syndrome), causing heart failure, or hydrops, of the fetus. In certain cases, fetal surgery may be indicated.

Beyond the newborn period, symptoms of a teratoma depend on its location and organ of origin. Ovarian teratomas often present with abdominal or pelvic pain, caused by torsion of the ovary or irritation of its ligaments. Testicular teratomas present as a palpable mass in the testis; mediastinal teratomas often cause compression of the lungs or the airways and may present with chest pain and/or respiratory symptoms.

Some teratomas contain yolk sac elements, which secrete alpha-fetoprotein (AFP). Detection of AFP may help to confirm the diagnosis and is often used as a marker for recurrence or treatment efficacy, but is rarely the method of initial diagnosis. (Maternal serum alpha-fetoprotein, or MSAFP, is a useful screening test for other fetal conditions, including Down syndrome, spina bifida and abdominal wall defects such as gastroschisis).

[edit] Time of Presentation
Teratomas of germ cell origin usually are found (i.e., present) in adult men and women, but they may also be found in children and infants. Teratomas of embryonal origin are most often found in babies at birth, in young children, and, since the advent of ultrasound imaging, in fetuses.

The most commonly diagnosed fetal teratomas are sacrococcygeal teratoma (Altman types I, II, and III) and cervical (neck) teratoma. Because these teratomas project from the fetal body into the surrounding amniotic fluid, they can be seen during routine prenatal ultrasound exams. Teratomas within the fetal body are less easily seen with ultrasound; for these, MRI of the pregnant uterus is more informative.[28][29]

[edit] Complications
Teratomas are not dangerous for the fetus unless there is either a mass effect or a large amount of blood flow through the tumor (known as vascular steal). The mass effect frequently consists of obstruction of normal passage of fluids from surrounding organs. The vascular steal can place a strain on the growing heart of the fetus, even resulting in heart failure, and thus must be monitored by fetal echocardiography.

After surgery, there is a risk of regrowth in place, or in nearby organs.[30]

[edit] Treatment
[edit] Surgery
The treatment of choice is complete surgical removal (i.e., complete resection).[31][32] Teratomas normally are well encapsulated and non-invasive of surrounding tissues, hence they are relatively easy to resect from surrounding tissues. Exceptions include teratomas in the brain, and very large, complex teratomas that have pushed into and become interlaced with adjacent muscles and other structures.

Prevention of recurrence does not require en bloc resection of surrounding tissues.

[edit] Chemotherapy
For malignant teratomas, usually, surgery is followed by chemotherapy.

Teratomas that are in surgically inaccessible locations, or are very complex, or are likely to be malignant (due to late discovery and/or treatment) sometimes are treated first with chemotherapy.

[edit] Clinical trials
The examples and perspective in this article may not represent a worldwide view of the subject. Please improve this article and discuss the issue on the talk page. (June 2007)

As of 2007, there have been two clinical trials in progress that address germ cell tumors, both of which include teratomas.[33][34] A predominant theory of reversing or delaying teratomic lymphatic spread was tested by Kevin Smith incoorporating NJD-S enzyme with common lymphocyte co-stimulators.

[edit] Follow-up
Although often described as benign, a teratoma does have malignant potential. In a UK study of 351 infants and children diagnosed with "benign" teratoma reported 227 with MT, 124 with IT. Five years after surgery, event-free survival was 92.2% and 85.9%, respectively, and overall survival was 99% and 95.1%.[35] A similar study in Italy reported on 183 infants and children diagnosed with teratoma. At 10 years after surgery, event free and overall survival were 90.4% and 98%, respectively.[36]

Depending on which tissue(s) it contains, a teratoma may secrete a variety of chemicals with systemic effects. Some teratomas secrete the "pregnancy hormone" human chorionic gonadotropin (βhCG), which can be used in clinical practice to monitor the successful treatment or relapse in patients with a known HCG-secreting teratoma. This hormone is not recommended as a diagnostic marker, because most teratomas do not secrete it. Some teratomas secrete thyroxine, in some cases to such a degree that it can lead to clinical hyperthyroidism in the patient. Of special concern is the secretion of alpha-fetoprotein (AFP); under some circumstances AFP can be used as a diagnostic marker specific for the presence of yolk sac cells within the teratoma. These cells can develop into a frankly malignant tumor known as yolk sac tumor or endodermal sinus tumor.

Adequate follow-up requires close observation, involving repeated physical examination, scanning (ultrasound, MRI, or CT), and measurement of AFP and/or βhCG.[37][38]

[edit] Use in basic research
In light of the ethical issues surrounding the source of human stem cells, teratomas are being looked at as an alternative source for research since they lack the potential to grow into functional human beings.

[edit] Teratoma in non-humans
Ovarian teratomas have been reported in mares.[39][40]

Home Sweet Home

Seronok rasanya bila dah balik rumah...alhamdulillah ya Allah..rindunya saya nak menatap anak teruna dan anak dara saya yang gebu tu...rindu juga saya nak menyapa ikan-ikan dalam akuarium...(teringat Awatif paling suka duduk tepi akuarium dan cakap..."Fish...angun..angun")"angun" maksudnya bangun...tak tau kenapa agaknya dia ingat ikan-ikan kt dalam akuarium tu semua tidur kot. Saya juga rindu pada pokok-pokok di halaman, pokok tembikai yang saya tanam, pokok bunga nasi kerabu (bunga telang)..yg ni org Kelantan mesti kenal ni..cili yg ayah tanam...dan kehijauan rumput carpet...semuanya menggamit saya pulang.

Jam 4.30 petang 15/3/2010, saya berjaya "melarikan diri" dari hospital Sungai Buloh. Doktor bagi cuti 6 minggu sampai 28/4/2010. Balik je pergi jemput si gebu dan abang. Alhamdulillah anak-anak saya sihat dan selamat...Alhamdulillah, ikan dan pokok2 kesayangan saya juga sihat...meliuk lintuk ditiup angin petang bersama pancaran sinaran mentari...cantiknya...

16/3/2010....memikirkan apa la yang saya nak buat selama 6 minggu ni...aduh bosannya, saya di rumah sorang-sorang...abang pegi tuisyen...adik lak kena hantar kt nursery sbb mama dilarang keras dukung dia...cuma boleh peluk dan cium je...saya dok tepi akuarium tengok ikan berenang, merenung pokok-pokok yang bercahaya disirami mentar pagi. Boring dok depan, saya beralih ke belakang konon nak makan nasi lemak..yang abah beli tadi. Tengok pinggan yang dh berbasuh tapi belum letak dalm kabinet, saya tanpa ragu kemaskan pinggan2 tu semua. Lepas tu masak air..tiba-tiba rasa sesak nafas..penat, mencungap-cungap saya seketika. Cepat penat (agaknya ini lah yang dirasai oleh orang yang baru lepas operation...patutlah orang cakap, jangan buat sebarang kerja pun, rehat, jangan bergerak banyak sangat). Saya gagahkan diri buat nescafee dan kononya nak bawa nasi lemak makan kat kerusi batu tepi akuarium..Bersusah nk buka pintu depan, dengan sudu dan garfu, sebungkus nasi lemak dan muga nescafee tiba2 nescafee panas tertumpah kena tangan. Dalam kekalutan,saya mencari minyak gamat, tak jumpa,(dah la jalan terbongkok2, pergerakan pun lembab),saya ambik ubat gigi..lega sikit. Kemudian call abah tanya mana minyak gamat. Kesian dia yang dalam perjalanan ke pejabat patah balik, mungkin kesian kat saya yang mengaduh kesakitan. minyak gamat kat tingkat atas (pastinya ambik masa yang lama untuk saya menapak keatas dengan keadaan saya yg macam ni)

Alamak...ni baru tak sampai suku hari saya kat rumah, dah ada tragedi..tak apa...esok saya kena lebih berhati-hati kerana saya dah tak sekuat dulu..hmm apa pun bestnya duduk rumah....hehe HOME SWEET HOME...

Sakit Itu Menyiksakan

Selaku manusia, hidup dan mati sepatutnya kerana Allah s.w.t sebagaimana firmannya dalam surah Al-An'am ayat 162 yang bermaksud "Katakanlah, sesungguhnya ibadatku, hidupku dan matiku hanyalah untuk Allah tuhan semesta alam....."syukur yang teramat pada Mu ya Allah kerana masih memberi kesempatan untuk hambaMu ini bernafas dan melangkah di bumi ini. Kesakitan ini walau amat menyiksakan, namun cukup banyak untuk menginsafkan saya. Banyak yang saya janjikan dengan diri ini sepanjang sakit ni..terutamanya hendak menambah amal ibadat (saya tak patut cakap kat sini kan, takut tak terbuat), keduanya saya hendak taat sepenuhnya pada suami dan kedua ibu bapa saya, pas tu saya nak didik anak2 saya betul supaya menjadi seorang muslim dan mslimat yang disayangi Allah....banyak lagi...bla-bla-bla.
Saya tak boleh utarakan semuanya...bahaya...tapi itulah hakikatnya. Saat saya nak dibius saya mengucap dan mohon kemapunan dari Allah sebanyak-banyaknya...manalah tau..waktu tu saya terus tidur untuk selama-lamanya...besar kekuasaanMu Ya Allah..syukur padaMu..
Sebelum masuk hospital, saya sering bermonolog sendiri, kenapa saya yang kena macam ni...orang lain boleh hidup riang ria, sihat walafiat...saya? Saya sering menyalahkan diri sendiri dan takdir, tapi bila dah masuk kat sini macam-macam penyakit yang saya jumpa...Subhanallah.
Satu lagi bila kita sakit ni...kita boleh kenal sebenarnya siapa kawan kita yang sebenar-benarnya..rupanya kawan susah memang payah nak cari. Terima kasih yang tidak terhingga untuk Mira dan Nairul...korang memang kawan sejati...juga kita dapat kenal siapa antara adik beradik yang benar-benar sayangkan kita. Terima kasih untuk adik Salma...semoga tali silaturrahim kita akan kekal sehingga mati.

Sakit Itu Menyiksakan

Hari ni dah masuk hari yang keempat saya kt hospital ni. Rasa seronok sikt hari ni...sebab kawan-kawan datang melawat..pas tu sbb husband bawakan lap top..so boleh lah buka FB untuk menghilangkan sunyi. kalau tak..dok kira masa je...bila la nk balik rumah ni..Bila sakit ni macam-macm yang terkenang..selain boleh rasa keinsafan dalam keperitan menanggung kesakitan...semakin rasa takut menghadapi hari kematian. Ini adalah first time saya menghadapi operation. Walapun dah dua kali bersalin, saya tak pernah kena operate. Rasa lucu pulak bila teringat waktu bersalin kali kedua dulu, bila dah rasa tak tahan lagi menaggung sakit, saya request dgn doktor untuk operation...saya ingat operation lagi senang..nasib baik doktor tu halang. Walaupun doktor tu berbangsa India..tapi dia sentiasa ingatkan saya erti kekuatan dan kesabaran. Balik pada cerita operation ni...sebelum operation hati saya dah gundah gulana...para doktor pun serius semacam je...dah sampai lima enam kali scan...tak puas hati kerana ct scan yang saya jalani tak sama dgn pandangan doktor. Saya jadi takut sendiri. Setiap saat hanya berdoa supaya Allah panjangkan umur saya kerana anak-anak saya masih kecil dan saya masih ada kedua org tua yang akan tua yang memerlukan penjagaan saya. Doktor pun sama...mula2 dia org cakap...saya akan dibius penuh, pas tu...cakap pulak separuh...dah saya mula ketakutan..tapi dalam hati bagus juga bius separuh sbb waktu operate tu saya boleh berdoa pada tuhan, tak pun berzikir sebanyak mungkin. Dan di saat2 akhir, bagi tahu pulak..bius penuh...dan saya tak tahu apa terjadi sehingga 2 jam kemudian iaitu selepas operation dimana saya dikejutkan dan saya alami kesakitan yang amat. (Hmm..waktu entry ni ditulis...pesakit kt depan saya sedang menangis..tak pasti dia sakit apa.yg pasti berkaitan dengan kandungannya..sian dia...)saya nk stop la kejap...husband datang nk sembang ngan dia.