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often all 3. Arising from totipotential cells, these tumors typically are midline or paraxial. [3] The most
common location is sacrococcygeal (57%). Because they arise from totipotential cells, they are
encountered commonly in the gonads (29%). By far, the most common gonadal location is the ovary,
although they also occur somewhat less frequently in the testes. Cystic teratomas occasionally occur in
sequestered midline embryonic cell rests and can be mediastinal (7%), retroperitoneal (4%), cervical
(3%), and intracranial (3%). [4] Cells differentiate along various germ lines, essentially recapitulating any
tissue of the body. Examples include hair, teeth, fat, skin, muscle, and endocrine tissue. See images
above.
Complications of ovarian teratomas include torsion, rupture, infection, hemolytic anemia, and
malignant degeneration.
Torsion is by far the most significant cause of morbidity, occurring in –3-11% of cases. Several
series have demonstrated that increasing tumor size correlates with increased risk of torsion. [2, 20]
Rupture of a cystic teratoma is rare and may be spontaneous or associated with torsion. Most
series report a rate of less than 1%, [2, 1] though Ahan et al reported a rate of 2.5% in their report
of 501 patients. [21] Rupture may occur suddenly, leading to shock or hemorrhage with acute
chemical peritonitis. Chronic leakage also may occur, with resultant granulomatous peritonitis.
Prognosis after rupture is usually favorable, but the rupture often results in formation of dense
adhesions.
Infection is uncommon and occurs in less than 1-2% of cases. Coliform bacteria are the
organisms most commonly implicated. [21, 20]
A recently recognized encephalitis associated with antibodies against the N -methyl D-aspartate
receptor (NMDAR) is associated with ovarian mature teratomas. In a series of 400 cases, of
which 335 were women, 165 (49%) had tumors and all but six were ovarian teratomas. The
syndrome is characterized by a viral-like prodrome followed by a multistage progression of
symptoms that includes psychosis, memory deficits, seizures, language disintegration, decreased
consciousness, dyskinesias, and autonomic instability. Substantial recovery is usually seen with
tumor resection and immunotherapy. [22]
Autoimmune hemolytic anemia has been associated with mature cystic teratomas in rare cases.
In these reports, removal of the tumor resulted in complete resolution of symptoms. Theories
behind the pathogenetic mechanism include (1) tumor substances that are antigenically different
from the host and produce an antibody response within the host that cross reacts with native red
blood cells, (2) antibody production by the tumor directed against host red blood cells, and (3)
coating of the red blood cells by tumor substance that changes red blood cell antigenicity. In this
context, radiologic imaging of the pelvis may be indicated in cases of refractory hemolytic
anemia. [23, 24]
In its pure form, mature cystic teratoma of the ovary is always benign, but in approximately 0.2-
2% of cases, it may undergo malignant transformation into one of its elements, the majority of
which are squamous cell carcinomas. The prognosis for patients with malignant degeneration is
generally poor but dependent on stage and degenerated cell type.
Mature cystic teratomas are encapsulated tumours with mature tissue or organ components. They are
composed of well-differentiated derivations from at least two of the three germ cell layers (i.e.
ectoderm, mesoderm, and endoderm). They, therefore, contain developmentally mature skin complete
with hair follicles and sweat glands, sometimes luxuriant clumps of long hair, and often pockets of
sebum, blood, fat, bone, nails, teeth, eyes, cartilage, and thyroid tissue. Typically their diameter is
smaller than 10 cm, and rarely more than 15 cm. Real organoid structures (teeth, fragments of bone)
may be present in ~30% of cases.
Plain radiograph
Pelvic ultrasound
Ultrasound is the preferred imaging modality. Typically an ovarian dermoid is seen as a cystic
adnexal mass with some mural components. Most lesions are unilocular.
CT
CT has high sensitivity in the diagnosis of cystic teratomas 6 though it is not routinely
recommended for this purpose owing to its ionising radiation.
Typically CT images demonstrate fat (areas with very low Hounsfield values), fat-fluid level,
calcification (sometimes dentiform), Rokitansky protuberance, and tufts of hair. The presence of
most of the above tissues is diagnostic of ovarian cystic teratomas in 98% of cases 5. Whenever
the size exceeds 10 cm or soft tissue plugs and cauliflower appearance with irregular borders are
seen, malignant transformation should be suspected 5.
When ruptured, the characteristic hypoattenuating fatty fluid can be found as anti dependant
pockets, typically below the right hemidiaphragm, a pathognomonic finding 2. The escaped cyst
content also leads to a chemical peritonitis and the mesentery may be stranded and the
peritoneum thickened, which may mimic peritoneal carcinomatosis 2.
Pelvic MRI
MR evaluation usually tends to be reserved for difficult cases but is exquisitely sensitive to fat
components. Both fat suppression techniques and chemical shift artefact can be used to confirm
the presence of fat.
Enhancement is also able to identify solid invasive components, and as such can be used to
accurately locally stage malignant variants.
Mature ovarian teratomas are slow growing (1-2 mm a year) and, therefore, some advocate
nonsurgical management. Larger lesions are often surgically removed. Many recommend annual
follow-up for lesions <7 cm to monitor growth, beyond which a resection is advised.
Complications
ovarian torsion: ~3-16% of ovarian teratomas, in general: considered the most common
complication
rupture: ~1-4%
malignant transformation: ~1-2%, usually into squamous cell carcinoma (adults) or rarely
into endodermal sinus tumours (paediatrics)
superimposed infection: 1%
autoimmune haemolytic anaemia: <1%
paraneoplastic anti-N-methyl-D-aspartate receptor (anti-NMDA receptor) associated
limbic encephalitis (uncommon) 8
Differential diagnosis