MALIGNANT SACROCOCCYGEAL YOLK SAC (ENDODERMAL
Mufti*, M.Saeed Khan***, Muzher-U- Dua**
and Farah Waqar**
of Surgery* and* *Oncology Ayub Teaching Hospital, Abbottabad and ***Institute
of Nuclear Medicine Oncology & Radiotherapy (INOR), Abbottabad
report a 2 ½ years old female child who presented with Malignant
Sacrococcygeal Yolk sac (Endodermal Sinus) tumor as presacral mass with
ulceration of overlying skin. Her ultrasonography (USG)abdomen revealed
a huge presecral mass with irregular margins extending in to the
pelvis, pushing the rectum antero-laterally. CT scanning of abdomen confirmed
USG finding with out enlargement of retroperitoneal lymph nodes and no
involvement of the liver. Serum AFP was abnormally elevated. The tumor was
excised along with Coccyx in-toto through posterior approach dividing the
sacrum and 3-Drugs (VAC) Chemotherapy started post–operatively i.e. 2-weeks
after surgery. Here we discuss our experience with this extremely rare
malignant extra-gonadal germ-cell tumor and review of literature.
can say with some assurance that although children may be victims of
fate, they will not be the victims of our neglect (John
sacrococcygeal Yolk sac (Endodermal Sinus) tumor is an extremely rare extra-gonadal
germ cell tumor found in infants and children. Although, most yolk sac tumors
present as infantile testicular tumors yolk sac tumors occurring as pure or
mixed germ cell tumors are rather common in the ovaries of young girls and in
several extra-gonadol sites including the sacro-coccygeal area, pelvis,
mediastinum, stomach, retro-peritoneum, vagina and brain2.
yolk sac tumors of infant and children reflect the transformation of
primordial cells that have failed to migrate to their pre-destined location.
Toti-potent germ cells normally arise from the Yolk sac of the 04 weeks old
human embryo and migrate along the Gonadal ridge before their final decent in
to the pelvis. During embryogenesis, some of these cells fail to complete
migration and come to rest along the dorsal mid-line of the embryo. The
primordial germ cells give rise to an un-differentiated germ cell line. The
un-diff-germ cell undergoes differentiation into embryonic (somatic cells) or
extra –embryonic cells of Yolk sac, chorion & allantoin cells.3,8
transformation of these cells give rise to tumors that reflect their embryonic
features. Tumors of extra-embryonic cells have trophoblastic features as in
chorio-carcinoma or characteristic of
yolk sac endoderm etc. as shown in Fig-1.3
parents of this young baby gave history of appearance of painless swelling
over sacral region with gradual increase in size of 5-6 months duration. The
child was taken to the nearest doctor. A biopsy piece was taken for laboratory
diagnosis. The histopathology report (AFIP) revealed Malignant Yolk sac (endodermal
sinus) tumor. The patient was referred to Ayub Teaching Hospital Complex (ATHC),
Abbottabad for necessary treatment. Where
she got admitted in Surgical-A unit. Her USG abdomen revealed a huge (solid)
presacral mass with irregular margins extending into the pelvis pushing the
rectum antero-laterally. CT scanning of abdomen also confirmed these findings.
Retro-Peritoneal lymph nodes were not enlarged, no metastases in liver were
reported in C.T scan. Her x-ray chest was clear. Her AFP was raised. Patient
was staged as stage-II according to St. Jude Children research hospital
staging system and operated i.e removing the tumor in one piece along with
coccyx after dividing the sacrum through posterior approach. No serious
complications occurred during surgery and her post- operative recovery was
3-drugs (VAC) chemotherapy was started in following dosage schedule.
Inj. Vincristine (VCR) 1.4 mg/m2 I/V wkly. Planned for 10
Inj. Actinomycin-D (Cosmegen) 0.5mg i/v day 1-5 and
Inj. Cyclophosphamide (Endoxan) 5-7mg/kg day1-5
2 & 3 to be continued at monthly interval for 1 ½ - 2 years
Fig-1: Schematic representation of germ cell maturation and the corresponding malignant germ cell tumors3
extra-gonadal germ cell tumors are uncommon neoplasm accounting for approx. 3%
of childhood tumors that may arise in a variety of anatomic sites3.
The incidence of malignant yolk sac carcinoma is less than 1 per million per
year4. The Male to Female ratio is about 1:2.55. In a
reported series by Gross-field et al6 58 of 85 (68%) patients were
girls and 27(30%) were boys. The sites of origin benign and malignant in
decreasing frequency were sacro-coccygeal in 55 (64.8%), mediastinal in 10
(11.7%), Gonadal in 10 (11.7%), Presacral in 4 (4.8%), retro-peritoneal in 3
(3.5%)and neck in 3(3.5%) of cases. Of the total 67 (78.8%) were benign and
18(21.2%) malignant. Site of malignant tumors were sacro-coccygeal 11 of 55
(20%), mediastinal 2 of 10(20%) , ovarian 3 of 8 (37.5%), testicular 2 of 2
yolk sac tumors can be external or internal i.e presacral and intra-pelvic or
intra-abdominal extension. Tumors that are predominantly external have a lower
malignant potential than prescral that are always malignant2. For
infant with external masses in the sacro-coccygeal area, the differential
diagnosis must include Menin-gomyelocoele, chordoma, neurogenic tumors, Lipoma,
vestigial tail and Hemingioma etc.2
infants without external masses, a careful rectal examination is essential in
the evaluation of these infants. Presacral or intra-abdominal extension of
tumors occurs in 27% of patients6. Symptoms of Urinary tract or
colonic obstruction are associated more often with malignant than benign
tumors. Rarely, invasion of the lumbo-sacral plexus or spinal cord may result
in lower extremity weakness and pain.
Sacro-coccygeal lesions when malignancy is suspected, abdominal and chest
x-rays plus sacral film are taken to look for tumor invasion or metastasis.
IVP, Ba-enema, USG & CT scanning of the abdomen are obtained in patients
who have malignant lesion. Patients with sacro-coccygeal lesions may have an
elevated AFP which can indicate malignancy and also should predict recurrence8.
surgical excision should be attempted in malignant lesion. Since complications
with sacro-coccygeal lesions usually involve haemorrhage, control of the tumor
vasculature is important. Failure to excise malignant lesions completely had
always resulted in death from the disease. Prior to 1950, only patients with
completely removed tumors survived the disease9. The outlook is
improving with the use of combination chemotherapy and long-term responses are
being noted7. Adjuvant VAC chemotherapy (vincristine, actinomycin-D
and Cyclophosphamide) has been most extensively used in children with
endodermal sinus yolk sac tumors.
incidence of re-growth of the tumor at the primary site is extremely high in
the absence of radiation10. However, in a more common yolk sac
tumor of extra gonadol origin, the role of radiotherapy is less certain7.
Metastatic lesions may require palliative treatment with local radiation.
Patients with Sacro-coccygeal tumors have a greater probability for survival
than those with tumors at other
sites (50 versus 13%)11-18.
Yolk sac (endodermal sinus) tumors are highly malignant and lethal germ cell
tumors which can kill by early metastasis and rapid invasion of
abdominal and pelvic structures. There is growing body of evidence
documenting complete responses of metastatic and primary disease to
chemotherapy with long term survival.
it appears reasonable to conclude that all patients even those with completely
resectable tumors should receive adjuvant chemotherapy.
role of retro-peritoneal lymph nodes dissection and post-operative irradiation
is not fully defined in these more common malignant yolk sac (endodermal sinus) tumors of extra-gonadol origin.
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