extragonadal teratoma - non SCT Flashcards
In a fetus with cervical teratoma, what radiological findings should trigger suspicion of airway compromise?
1) important polyhydramnios (may require serial amnioreduction)
2) deviated esophagus / trachea
3) lung hypoplasia (due to mediastinal/thoracic extension, measure volume MRI)
US + Fetal MRI
On US, fetal cervical teratoma is cystic / partially cystic
Assess for EXIT
what is the differential diagnosis of a fetal cervical mass?
90% are teratoma or lymphatic malformation.
Classification of fetal neck mass includes: tumoral, vascular malformation, congenital cyst.
Tumor
- Teratoma
- neuroblastoma
Vascular malformation
- hemangioma
- lymphatic malformation
Congenital cyst
- branchial
- foregut duplication
also, congenital goiter
What is the postnatal workup a cervical mass?
Urine VMA / HVA US CT/MRI FP/BHCG TSH if stuck to thyroid
how is the differential diagnosis impacted if a cervical cyst is identified on during the first vs second trimester?
Posterior nuchal translucency is identified during the first or early second trimester and sometimes called cystic hygroma. it represents a unilocular cystic collection in the occipital region. it is associated with chromosomic syndromes such as T21, turner, noonan.
A cervical cystic lesion identified during T2 US is at a much smaller risk of such syndromic association. Nevertheless, amniocentesis is recommended.
Name 2 fetal complications which should be monitored in the context of a cervical mass.
Polyhydramnios (for serial punction)
Hydrops (high output heart failure)
Corticosteroids should be administered to mother in preparation of preterm labor. What is the optimal timing?
steroids should be given for preterm labor between 23+0 and 33+6 weeks.
What syndrome is associated with mediastinal teratomas in male?
Klinefelter (47 XXY)
-decreased testosterone, associated with infertility, cryptorchidism, gynocomastia. Taller and some with intelectual disabilities.
If presence of precocious puberty, suspect choricarcinoma (BHCG secretion) within teratoma
What is the management of malignant mediastinal germ cells tumor?
if complete resection possible without significant morbidity, proceed to surgery.
If not, biopsy and give neoadjuvant chemotherapy.
Be aware of growing teratoma syndrome: after initial decrease in size, tumor starts to grow under chemo. this
typically is secondary to growth of mature (benign) elements of the tumor.
What is the overall survival of malignant mediastinal GCT?
71%, which is less than for other extragonadal sites.
Name 2 abdominal sites of extra-gonadic teratomas?
Retroperitoneal, typically supra renal
Gastric, often involving posterior wall. presents as a gastric obstruction in infants.
Describe COG’s staging for extragonadal GCT
1: R0, N-, including resection of coccyx for SCT
2: R1
3: N+, R2, bx only
4: mets
Should lymph node sampling be conducted in retroperitoneal GCT?
biopsy only grossly abnormal lymph nodes.
send peritoneal fluid cytology.
What to do if phrenic is involed post neoadjuvant chemo in mediastinal GCT?
aim for complete surgical resection. some advocate for upfront diaphragmatic plication givent toxic effect of bleomycin on lungs (APSA guideline)
note: given high rate of mature teratoma identified post neoadj, I wonder if sending the nerve preserving specimen for fresh before commiting to phrenic resection would be acceptable?
Surgical principles of cervical GCT resection?
vital structures preservation (vagus, carotid). can be ligated if are involved…
expect adhesion ++ pharynx, larynx. may need reconstruction.
Sometime originates from thyroid and requires hemi-thyroidectomy.
what is the concept of growing teratoma syndrome?
when a patient is receiving chemo (PEB), a portion of the tumor is growing on serial imaging. although the malignant component is receding, a benign mature teratoma compenent keeps on growing.
Highlight the importance of using chemo only for proven malignant GCT.
