Teratoma20150424

Introduction

● due to abnormal differentiation of fetal germ cells that arise from the fetal yolk sac

● Teratomas are typically found in the midline or gonads.

Sacrococcygeal - 40%Ovary - 25%Testicle - 12%Brain - 5%Other (including the neck and mediastinum) - 18%

Introduction

Teratomas have been reported to contain hair, teeth, bone (rarely: eyes,torso,and hands, feet, or other limbs)

usually benign

mature/benign

immature/malignant

women

men

more commonly

95%

5%

Introduction

● Congenital (unknown: germ cells)● nonseminomatous germ cell tumor (N.S.G.C.T.)● abnormal development of pluripotent cells● germ cells and embryonal cells● ectoderm, endoderm, and mesoderm

germ cells testes or ovaries

embryonal cells Congenital

unknown

Mature teratoma

● grade 0 teratoma● solid, cystic, or a combination● skin, muscle, and bone● generally benign

Dermoid cystSkin may surround a cyst and grow abundant hair

Dermoid cyst

● a cystic teratoma● grows slowly and contains mature tissue● always benign

rare malignant in adult: squamous cell carcinomain infants and children: endodermal sinus tumor

Dermoid cyst

Ovarian dermoid cysts● totipotential germ cells differentiated abnormally● Complications: torsion, rupture and infection

Periorbital dermoid cysts● in young children● near the lateral aspect of the eyebrow● can recur if not completely excised

Dermoid cyst

Spinal dermoid cysts● benign ectopic growths of

neural tube closure● lumbosacral region

Gonzalez-Crussi grading system

Grade 0mature (benign)Grade 1immature, probably benignGrade 2immature, possibly malignant (cancerous)Grade 3frankly malignant

depends on amount of immature elements

<10%

10-50%

>50%

mature

Diagnosis

● Prenatal ultrasound● Prenatal MRI● “steal syndrome”fetus' blood flow is redirected toward the teratoma

● AFPSome teratomas contain yolk sac elements, which secrete AFP.

Introduction

The most commonly diagnosed fetal teratomas are sacrococcygeal teratoma (Altman types I, II, and III) and cervical (neck) teratoma.

Fetal Sacrococcygeal Teratoma

● the most common congenital germ cell tumor● 1 in 35,000-40,000● female predominance (3:1-4:1 ratio)● prenatally diagnosed: 30-50%● 36-41% require fetal intervention● survival rate of prenatally diagnosed SCT is 47-83%● 50% have long-term morbidityobstructive uropathy, bowel and bladder incontinence

http://emedicine.medscape.com/article/2109544-overview#showall

Cervical teratoma

● 1:20,000-40,000● 50% presence of calcification● 20-40% polyhydramnios● hyper-extended to flexed towards a side● neonatal airway obstruction

http://radiopaedia.org/articles/congenital-cervical-teratoma

Fetiform teratoma

● Fetus in fetu● highly developed mature teratomas● retroperitoneal teratomas

Teratoma theory

Parasitic twin theory

http://en.wikipedia.org/wiki/Fetus_in_fetu

anencephalic

Case

http://www.nownews.com/n/2014/06/18/1282162

http://www.ettoday.net/news/20140619/369401.htm

Treatment

● Surgery

non-invasive of surrounding tissues

● Chemotherapy

for malignant teratomas

● Follow-up

Introduction

http://en.wikipedia.org/wiki/Dermoid_cysthttp://en.wikipedia.org/wiki/Teratomahttp://emedicine.medscape.com/article/939938-overview#a0104http://www.iap-ad.org/lectures/sudan/Germ%20cell%20tumours.pdfhttps://www.youtube.com/watch?v=9qoohmd-kUM

http://en.wikipedia.org/wiki/Sacrococcygeal_teratoma