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 a kind of tumor (neoplasm). Definitive diagnosis of a teratoma is based on its

histology: a teratoma is a 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 eyeball[citation needed], torso[citation needed], and
hands, feet, or other limbs[1] . 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. A teratoma is
an encapsulated tumor. Sometimes the capsule encompasses 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. In part because it is encapsulated, a teratoma usually is benign,
although several forms of malignant teratoma are known and some of these are common. A
mature teratoma typically is benign and more commonly is found in females, while an immature
teratoma typically is malignant and more commonly is found in males.

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

Contents
[hide]
1 Etymology
2 Natural history
 o 2.1 Location and incidence

o 2.2 Hypotheses of origin

o 2.3 Dermoid cyst

o 2.4 Fetus in fetu and fetiform teratoma

o 2.5 Struma ovarii

3 Pathology classification of individual teratomas

o 3.1 "Benign" teratoma may prove to be malignant

o 3.2 Teratoma with malignant transformation

o 3.3 Extraspinal ependymoma

4 Initial diagnosis

5 Time of Presentation

6 Complications

7 Treatment

o 7.1 Surgery

o 7.2 Chemotherapy

o 7.3 Clinical trials

o 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".

[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[2].
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.[3]

[edit] Hypotheses of origin

Concerning the origin of teratomas, there exist numerous hypotheses.[4] 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] Dermoid cyst

A small (4 cm) dermoid cyst of an ovary, discovered during a C-section

A 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.[4]

Most authorities agree that fetiform teratomas are highly developed mature teratomas; the natural
history of fetus in fetu, however, is controversial.[4] 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.[5][6][7] Cysts within mature teratoma have also been reported to
contain a rudimentary beating heart.[8]

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

A struma ovarii (literally: goitre of the ovary) is a rare form of mature teratoma that contains
mostly thyroid tissue. Despite its name, struma ovarii is not restricted to the ovary. Only 5% of
struma ovarii are malignant.[citation needed]

[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[4] 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.[9] 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,[10][11] even in fetiform teratoma and fetus in fetu.[12][13] Squamous cell carcinoma has
been found in a mature cystic teratoma at the time of initial surgery.[14]

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.[15][16][17][18] 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.[19]

[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.[9] Of 641 children with pure teratoma, 9 developed TMT[20]: 5 carcinoma, 2 glioma, and
2 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.[21]

[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.[22][23]

[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.[24]

[edit] Treatment
[edit] Surgery

The treatment of choice is complete surgical removal (i.e., complete resection).[25][26] 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 or 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.[27][28]

[edit] Follow-up

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 (hCG), 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 hCG.[29][30]

[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.[31][32]

[edit] See also

Tumor pages for locations in which teratoma can occur:

Brain
Mouth

Head/Neck

Mediastinum (chest)

Small intestine

Pelvis/Tailbone

Ovaries

Testicles

Other conditions that may resemble a teratoma:

Pilonidal cyst in humans

Dermoid sinus in dogs

[edit] References
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Series, Fascicle 18. Armed Forces Institute of Pathology, Washington D.C.

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6. ^ Kajbafzadeh AM, Baharnoori M (2006). "Fetus in fetu". Can J Urol 13 (5): 32778. PMID
17076951.

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teratoma in the retroperitoneum in a child: is a mature teratoma a premalignant condition?". Hum.
Pathol. 29 (10): 11679. doi:10.1016/S0046-8177(98)90432-4. PMID 9781660.

11. ^ Utsuki S, Oka H, Sagiuchi T, Shimizu S, Suzuki S, Fujii K (Jun 2007). "Malignant
transformation of intracranial mature teratoma to yolk sac tumor after late relapse. Case report".
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12. ^ Chen YH, Chang CH, Chen KC, Diau GY, Loh IW, Chu CC (2007). "Malignant transformation
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14. ^ Arioz DT, Tokyol C, Sahin FK, et al (2008). "Squamous cell carcinoma arising in a mature
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Gynaecol. Oncol. 29 (3): 2824. PMID 18592797.

15. ^ Muscatello L, Giudice M, Feltri M (2005). "Malignant cervical teratoma: report of a case in a
newborn". European archives of oto-rhino-laryngology : official journal of the European
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005-0917-2. PMID 15895292.

16. ^ Ukiyama E, Endo M, Yoshida F, Tezuka T, Kudo K, Sato S, Akatsuka S, Hata J (2005).
"Recurrent yolk sac tumor following resection of a neonatal immature gastric teratoma". Pediatr.
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17. ^ Bilik R, Shandling B, Pope M, Thorner P, Weitzman S, Ein SH (1993). "Malignant benign
neonatal sacrococcygeal teratoma". J. Pediatr. Surg. 28 (9): 115860. doi:10.1016/0022-
3468(93)90154-D. PMID 7508500.

18. ^ Hawkins E, Issacs H, Cushing B, Rogers P (1993). "Occult malignancy in neonatal

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PMID 7692755.

19. ^ Ramalingam P, Teague D, Reid-Nicholson M (Jul 2008). "Imprint cytology of high-grade

immature ovarian teratoma: A case report, literature review, and distinction from other ovarian
small round cell tumors". Diagn. Cytopathol. 36 (8): 5959. doi:10.1002/dc.20849. PMID
18618728.
20. ^ Biskup W, Calaminus G, Schneider DT, Leuschner I, Gbel U (2006). "Teratoma with
malignant transformation: experiences of the cooperative GPOH protocols MAKEI 83/86/89/96".
Klinische Pdiatrie 218 (6): 3038. doi:10.1055/s-2006-942272. PMID 17080331.

21. ^ Aktu T, Hakgder G, Sariolu S, Akgr FM, Olguner M, Pabucuolu U (2000).

"Sacrococcygeal extraspinal ependymomas: the role of coccygectomy". J. Pediatr. Surg. 35 (3):
5158. PMID 10726703. http://linkinghub.elsevier.com/retrieve/pii/S0022346800129197.

22. ^ Danzer E, Hubbard AM, Hedrick HL, et al (2006). "Diagnosis and characterization of fetal
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23. ^ Kocaoglu M, Frush DP (2006). "Pediatric presacral masses". Radiographics 26 (3): 83357.
doi:10.1148/rg.263055102. PMID 16702458.

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26. ^ Gbel U, Schneider DT, Calaminus G, Haas RJ, Schmidt P, Harms D (2000). "Germ-cell
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11 (3): 26371. PMID 10811491. http://annonc.oxfordjournals.org/cgi/reprint/11/3/26.

27. ^ GCT1P1 Protocol / Clinical Study: Pilot study of Cisplatin, Etoposide, Bleomycin and
Escalating Dose Cyclophosphamide Therapy for Children with High-Risk Malignant Germ Cell
Tumors

28. ^ GCT132 Protocol / Clinical Study: A Phase III Study of Reduced Therapy in the Treatment of
Children with Low and Intermediate Risk Extracranial Germ Cell Tumors (AGCT0132)

29. ^ Marina NM, Cushing B, Giller R, Cohen L, Lauer SJ, Ablin A, Weetman R, Cullen J, Rogers P,
Vinocur C, Stolar C, Rescorla F, Hawkins E, Heifetz S, Rao PV, Krailo M, Castleberry RP (1999).
"Complete surgical excision is effective treatment for children with immature teratomas with or
without malignant elements: A Pediatric Oncology Group/Children's Cancer Group Intergroup
Study". J. Clin. Oncol. 17 (7): 213743. PMID 10561269.

30. ^ Cushing B, Giller R, Ablin A, Cohen L, Cullen J, Hawkins E, Heifetz SA, Krailo M, Lauer SJ,
Marina N, Rao PV, Rescorla F, Vinocur CD, Weetman RM, Castleberry RP (1999). "Surgical
resection alone is effective treatment for ovarian immature teratoma in children and adolescents:
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181 (2): 3538. doi:10.1016/S0002-9378(99)70561-2. PMID 10454682.

31. ^ Catone G, Marino G, Mancuso R, Zangh A (April 2004). "Clinicopathological features of an

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0531.2003.00476.x. PMID 15065985.

32. ^ Lefebvre R, Theoret C, Dor M, Girard C, Laverty S, Vaillancourt D (November 2005).

"Ovarian teratoma and endometritis in a mare". Can. Vet. J. 46 (11): 102933. PMID 16363331.
[edit] External links
humpath pathology images #2657 (Teratomas), #4541 (Mature teratoma), #5350
(Immature teratoma)
Orphanet page on teratoma

cystic teratoma at eMedicine (also search EMedicine for all articles containing the word
teratoma)
Monster Tumors Show Scientific Potential in War Against Cancer article in the NYTimes

[show]
vde
Germ cell tumors (ICD-O 9060-9119) (C45-C49/D17-D21, 171/214-215)

[show]
vde
Endocrine pathology: endocrine diseases (E00-35, 240-259)

Hyperpituitarism (Acromegaly, Hyperprolactinaemia, SIADH)

Hypopituitarism (Sheehan's syndrome, Kallmann syndrome, Growth hormone

Pituitarydeficiency, Diabetes insipidus)

Adiposogenital dystrophy Empty sella syndrome Pituitary apoplexy

ACTH deficiency

Hypothyroidism (Iodine deficiency, Cretinism, Congenital hypothyroidism,

Goitre, Myxedema)

Hyperthyroidism (Graves disease, Toxic multinodular goitre, Teratoma with

thyroid tissue or Struma ovarii, Hashitoxicosis)
Thyroid
Thyroiditis (Graves disease, De Quervain's thyroiditis, Hashimoto's
thyroiditis, Riedel's thyroiditis)

Euthyroid sick syndrome Thyroid hormone resistance Thyroid nodule

Hypoparathyroidism (Pseudohypoparathyroidism) Hyperparathyroidism

Parathyroid
(Primary, Secondary, Tertiary)

AdrenalAdrenocortical hyperfunction: Cushing's syndrome (Nelson's syndrome,

Pseudo-Cushing's syndrome) Hyperaldosteronism (Conn syndrome, Bartter
syndrome) Glucocorticoid remediable aldosteronism
 CAH (Lipoid, 3, 11, 17, 21)

Adrenal insufficiency (Addison's disease, Waterhouse-Friderichsen

syndrome) Hypoaldosteronism

ovarian (Polycystic ovary syndrome, Premature ovarian failure)

testicular (5-alpha-reductase deficiency, 17-beta-hydroxysteroid

Gonadsdehydrogenase deficiency) Androgen receptor (Androgen insensitivity
syndrome)

general (Hypogonadism, Delayed puberty, Precocious puberty)

[show]
vde
Tumors: urogenital neoplasia genital neoplasia (C51-C63/D25-29, 179-187/218-
222)

Surface epithelial-stromal tumor Luteoma Meigs syndrome Krukenberg

Ovariestumor Teratoma Clear cell adenocarcinoma Endometrioid tumor
Fibroma

Fallopian tubePrimary fallopian tube cancer Adenomatoid tumor

Uterine sarcoma Leiomyosarcoma

Endometrium (Endometrioid tumor), (Uterine papillary serous carcinoma),

Uterus
(Clear cell carcinoma)

Cervix (SCC, Cervical intraepithelial neoplasia)

SCC Botryoid rhabdomyosarcoma Adenocarcinoma/Clear cell

Vagina
adenocarcinoma

VulvaPapillary hidradenoma Extramammary Paget's disease

Intratubular germ cell neoplasia (Carcinoma in situ) Seminoma

Spermatocytic seminoma Endodermal sinus tumor (yolk sac tumor)
Testicles
Embryonal tumor Choriocarcinoma Teratoma Leydig cell tumor Sertoli
cell tumor
 Transitional cell carcinoma Prostatic intraepithelial neoplasia Small cell
Prostate
carcinoma

Penis cancerCarcinoma (Extramammary Paget's disease)

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