CASE PRESENTATION

Dr. Lynda Ayade

BIODATA
Miss K.W. is a 20 year old student who
hails from Okrika in Rivers State and is a
Christian of the pentecostal denomination.
 PRESENTATION
Histology report of Yolk Sac Tumour
following an exploratory laparotomy at a
private clinic (25/09/03).
Had presented at the Gynaecology clinic a
month earlier (25/08/03) with USS report
of pedunculated uterine leiomyoma.
She was given a 2 week appointment to
prepare for myomectomy
Developed acute abdomen that
necessitated the emergency exploratory
laparotomy at the private clinic.
• Admitted to Gynaecology ward.
• Was commenced on adjuvant
chemotherapy using the BEP regimen; 3
cycles were given every 21 days.
• Received a total of 8 units of blood for the
period of chemotherapy.
• Developed facial nerve palsy, proptosis,
and vomiting- symptoms of secondary
metastasis to the CNS.
• Consult sent to Neurosurgical team to
review patient.
• However, she absconded before they
could review.
SUMMARY
Miss K.W., a 20 year old student who
presented with a histological diagnosis of
Yolk Sac Tumour following an exploratory
laparatomy in a private clinic. Had 3
courses of chemotherapy. She developed
symptoms of CNS metastasis but
absconded before a neurosurgical review.
GERM CELL TUMOURS

Dr. James E. Omietimi

Department of Obstetrics and Gynaecology, UPTH.
 INTRODUCTION
Tumours of the ovaries are common forms of
neoplasm in women.
They are the 3rd commonest gynaecological
tumours. However, because they present
very late, death from ovarian tumours
supercedes death from cervical &
endometrial tumours combined.
Fortunately, 80%-90% of ovarian tumours
are benign (20-45years) while 10%-20% are
malignant (40-65years).
Malignant Germ Cell Tumours account for
about 5% of ovarian cancers
 ASSOCIATED RISK FACTORS FOR
OVARIAN TUMOURS
Risk factors are much less clear than for other gynaecological
tumours. Accepted risk factors:
NULLIPARITY & FAMILY HX.
Nulliparity & low parity are associated with carcinoma of the
ovaries.

Gonadal dysgenesis in children is associated with a higher risk

of ovarian cancer.

Genetic mutation
Tumour suppressor genes; p53 -50% of ovarian
carcinoma
BRCA-1 & BRCA-2 :Serous
cystadenocarcinomas
HER2/neu oncogene -30% adenocarcinoma
(correlates with a poor prognosis)
CA-125 –80% of Serous & endometrioid CA`s
 CLASSIFICATION OF OVARIAN TUMOURS

Ovarian tumours are classified according

to their cell of origin:
SURFACE EPITHELIAL CELLS
Serous tumour
Mucinous tumour
Endometroid tumour
Clear cell tumour
 SEX CORD STROMAL TUMOURS
 Granulosa–Stromal cell tumour
 Sertoli-Stromal cell tumour
 Sertoli-Leydig cell tumour
 GERM CELL TUMOURS
 Teratoma:
• Mature(adult)
– Solid
– Cystic e.g Dermoid cyst

• Immature
• Monodermal (e.g Struma ovarii, carcinoid)
 Dysgerminoma
 Yolk sac tumour (Endodermal Sinus tumour)
 Mixed germ cell tumours
 Choriocarcinoma
 METASTATIC (NON-OVARIAN) TUMOURS
 From Uterus, Fallopian tubes, Contralateral
Ovary, Breast & GIT e.g. Krukenberg tumour
 Germ Cell Tumours
15-20% of all ovarian tumours
Most are benign cystic teratomas like Dermoid
Cysts.
Malignant Germ Cell Tumours are very
uncommon accounting for only 5% of ovarian
cancers
They are found mainly in children (teenagers)
and young adults in their twenties
They arise from germ cell differentiation.
Endodermal Sinus (Yolk Sac) Tumour accounts
for 20% of malignant germ cell tumours
 Teratomas
Mature (Benign) teratomas:
Most are dermoid cysts
Benign and derived from ectodermal differentiation of
totipotential cells.
Found in young women during active reproductive years.
Originate from a meiotic germ cell with a karyotype of
46,XX.
Morphology:
Bilateral in 10-15% of cases
Unilocular cysts containing hair and cheesy sebaceous
material.
Thin wall lined by an opaque, gray-white, wrinkled
apparent epidermis.
Within the wall, it is common to find tooth structures and
areas of calcification.
Histology:
Cyst wall is composed of stratified squamous
epithelium with underlying sebaceous glands,
hair shafts, and other skin structures.
Structures from other germ layers can be
identified, e.g., bone, cartilage, thyroid tissue.
About 1% of dermoids undergo malignant
transformation of any one of its components
e.g., thyroid carcinoma, melanoma but most
commonly squamous cell carcinoma.
Rarely, a teratoma is solid but is composed of
benign-looking heterogenous structures derived
from all three germ layers.
Immature (Malignant) teratomas
Rare tumours. Its component tissue resembles that in
the embryo or foetus. Found in prepubertal adolescents
and young adults. Mean age 18 years.
Morphology:
Bulky and have a smooth external surface.
Have a predorminantly solid structure.
Hair, grumous material, cartilage, bone and calcification
may be present.
Microscopy:
Varying amounts of immature tissue differentiating
toward cartilage, bone, glands, muscle and others.
They grow rapidly and frequently penetrate the capsule
with spread or metastases.
Stage 1 tumours have an excellent prognosis.
 Monodermal or Specialized teratomas

Rare , always unilateral.

Most common are Struma ovarii and carcinoid.
Struma ovarii is composed entirely of thyroid tissue
which may hyperfunction producing hyperthyriodism.
Ovarian carcinoid arises from intestinal epithelium in a
teratoma and may be functioning, producing 5-
hydroxytryptamine and the carcinoid syndrome.
Rarely there is a combination of both in the same ovary
– strumal carcinoid.
Primary carcinoids are uncommonly (< 2%) malignant.
Dysgerminoma
Uncommon tumours. 2% of all ovarian cancers yet 50% of all
malignant germ cell tumours. 75% occur in the 2nd and 3rd
decades.
Composed of large vesicular cells having clear cytoplasm, well
defined boundaries and centrally placed regular nuclei.
Morphology:
Usually unilateral, frequently solid tumours.
Yellow-white to gray-pink cut surface and are often soft and fleshy.
Histology:
Cells are dispersed in sheets or cords separated by scant fibrous
stroma.
Fibrous stroma is infiltrated with mature lymphocytes and occasional
granulomas.
All dysgerminomas are malignant.
Only about one third are aggressive.
A unilateral tumour that has not spread has an excellent prognosis.
They are extremely radiosensitive.
Overall survival exceeds 80%.
Endodermal Sinus (Yolk Sac) Tumour
Rare but the 2nd most common malignant germ
cell tumour (20%).
Generally, Germ Cell Tumours are unilateral &
slow growing but Endodermal Sinus (Yolk Sac)
Tumours grow rapidly and aggressively.
 Mostly seen in children or young women with
abdominal pain and rapidly developing pelvic
mass.
 Characteristic histological feature is a
glomerulus-like structure composed of a central
blood vessel enveloped by germ cells within a
space lined by germ cells (Schiller-Duval body)
 Intracellular and extracellular hyaline droplets
are present in all tumours.
 Prognosis is improved with combination
chemotherapy.
Choriocarcinoma
Most exist in combination with other germ cell
tumours
Pure choriocarcinomas are extremely rare.
Histologically identical to the more common
placental lesions.
Ugly tumours that generally have metastasized
widely through the blood stream to the lungs,
liver, bone, and other viscera by the time of
diagnosis.
They elaborate high levels of chorionic
gonadotropins- B-hCG.
They are generally unresponsive to
chemotherapy and are highly fatal.
Other Germ Cell Tumours
Embryonal carcinoma:
Highly malignant tumour of primitive
embryonal elements.
Polyembryoma:
A malignant tumour containing so-called
embryoid bodies.
Mixed germ cell tumours:
Containing various combinations of
dysgerminoma, teratoma, endodermal
sinus tumour, and choriocarcinoma.
 Clinical Presentation
Symptomatology is vague;
May be assymptomatic & incidental
Symptoms may include; weight loss
GIT upset -vomiting, constipation or
diarrhoea
Urinary frequency, urgency or dysuria
Abdominal mass
Abdominal pain; haemorrhage, rupture or
torsion
Abnormal vaginal bleeding
 DIAGNOSIS; clinical, radiological,
biochemical & surgical
Clinical; May be cachetic, lymphadenopathy,
pale & ascites
An abdomino-pelvic mass; Smooth & Cystic
Hard & Irregular
Bimanual exam; mass separate from uterus
Radiological; Abd. USS for large masses
Colour flow Doppler
Trans-vaginal USS for small
pelvic masses
CT Scan
 DIAGNOSIS contd.
Biochemical;
CA 125 –protein produced by most ovarian
cancers
Also produced in pregnancy & other benign
conditions; endometriosis, fibroids, diverticulitis
Alpha- FP & Alpha-1 anti-trypsin are specific for
York Sac Tumours
B-hCG is a useful tumour marker for
Choriocarcinoma & other GCTs
Tumor markers are also useful for post operative &
chemotherapy phase of mgt.
Rate of decline following Rx. is prognostic.
 DIAGNOSIS Contd.
Surgical;
Definitive diagnosis is histopathological after
surgery; laparotomy or laproscopy
Staging is also done at laparotomy
Frozen section at surgery or histopathology
thereafter –
Our patient had laparotomy &
histopathology revealed York Sac Tumor
 FIGO staging for Ovarian Cancers
I Growth limited to the ovaries
Ia Tumor in one ovary; capsule intact, no tumor on
surface, no ascites
Ib As in Ia but tumor in both ovaries
Ic As in Ia or Ib, but ascites with cancer cells, or
capsule ruptured or tumor on surface, or positive
peritoneal washings
II Growth on one or both ovaries with peritoneal
implants within the pelvis
IIa Extension or metastases to fallopian tubes or
uterus
IIb Extension to other pelvic organs
IIc As in IIa or IIb, but ascites with cancer cells, or
capsule ruptured or tumor on surface, or positive
peritoneal washings
Staging of Ovarian Cancers Contd.
III Tumor in one or both ovaries with peritoneal implants
outside the pelvis, or retroperitoneal node metastases

IIIa Tumor grossly limited to the true pelvis, microscopic

implants on abdominal peritoneal surfaces; no nodal
involvement

IIIb As in IIIa, but abdominal implants are <2cm in

diameter

IIIc As in IIIa, but abdominal implants are >2cm in

diameter +_ retroperitoneal lymph node metastases

IV Tumor involving one or both ovaries with distant

metastases, e.g. parenchymal liver metastases,
malignant pleural fluid
MANAGEMENT OF GERM
CELL TUMOURS

DR. B.O. ALTRAIDE

DEPT. OF OBSTETRICS AND GYNAECOLOGY
UPTH
 History
Physical Examination
Investigations
Treatment
Prognosis
Conclusion
INVESTIGATIONS
A) GENERAL
-FBC plus ESR

-Serum E/U/Cr

- Coagulation Studies (when indicated)

- LFT (when indicated)

- Urinalysis/ Urine MCS

B) SPECIFIC
- Ultrasonography
- Colour Flow Doppler Studies
- Computed Tomography
- Magnetic Resonance Imaging
- Chest X-ray
- Barium Enema
- Mammography
- Intravenous Urography
- Serum Tumour Markers
- Radio Immunoscintigraphy
TREATMENT
- Surgical
- Adjunct Combination Chemotherapy
- Radiotherapy

SURGICAL TREATMENT
- Aims at conserving future fertility
-Normally appearing contralateral adnexa and
uterus preserved.
- Biopsy of contralateral ovary not recommended
because of risk of peritubal and periovarian
adhesions.
COMBINATION CHEMOTHERAPHY
-Can be curative
-Much more effective than single agent regimens
-Tumour cells resistance is reduced.
-Cumulative toxic effects of each drug reduced
-Act at different cell sites to give synergistic effect
- Usually Vinblastine, Bleomycin and Cisplatin or
Bleomycin, Etoposide and Cisplatin are used.
-Doses are;-
-Bleomycin 0.25-0.5 U/kg I.V.,I.M. or S.C.
wkly
-Etoposide 50-100mg/m² per day for 5 days
-Cisplatin 15-20mg/m² I.V. daily for 5 days
-Cisplatin & Etoposide are repeated every 3-4 wks
- May be associated with a variety of potentially life
threatening side effects.
- FBC with differentials, LFT and Serum E/U/Cr are used
to monitor between cycles of therapy.
RADIOTHERAPY
- Especially useful in the treatment of
dysgerminoma.
- Otherwise germ cell tumours are generally not
responsive to radiotherapy.

PROGNOSIS
- Significantly improved when the disease is
detected while still confined to the ovary.
-Amongst the malignant Ovarian Tumours, Germ
Cell Tumours are associated with a better
prognosis.
- Overall 5 year survival rate for Ovarian
Tumours is about %.