CASE REPORTS

Congenital Vulvar Teratoma in a Newborn

zbasar, O
. Candr, H. Kaya, and F. Caglayan
By M. Cakmak, C. Savas, D. O
Krkkale, Turkey and Isparta, Turkey

The most common site of teratomas in neonates is in the

sacrococcygeal region. Herein the authors describe a congenital teratoma, and to their knowledge it is the first reported case of vulvar site in a newborn. Tumor was removed
on the fourth day of life, and she was completely normal 18
months after the operation. Because of possible malignancy

or recurrence, complete surgical excision of the tumor and

careful follow-up is the treatment of choice.
J Pediatr Surg 36:620-621. Copyright 2001 by W.B.
Saunders Company.

healthy after evaluation of the other organ systems. Hemogram and

biochemistry findings were all within normal limits, except the unconjugated bilirubin level (8.7 mg/dL). Alpha-fetoprotein (AFP) and carcinoembryogenic antigen (CEA) levels also were normal (AFP, 0.02
ng/mL; CEA, 1.38 ng/mL). Plain abdominal and pelvic radiographs in
both sides were normal. The patency of the urethra and the bladder
capacity were normal in cysto-urethrogram. A computed tomography
scan showed a polypoid and pedinculated mass, 5 cm in diameter and
in fatty density (40 HU). All the other abdominal and pelvic structures were normal. The tumor was resected on the fourth day of her life,
and the space between the clitoris and urethra was approximated, which
was the result of resection of the base of the tumor. The recovery was
uneventful, and she was discharged at the fifth postoperative day. She
was completely normal 18 months after the operation, and the CEA and
AFP levels were normal.

HE MOST COMMON site of teratomas in neonates

is in the sacrococcygeal and presacral region
(47%). The other most common sites are the gonads
(36%) and the mediastinum (6%).1 The remaining 11%
are distributed in the mesenterium, retroperitoneum,
pericardium, spinal canal, cervical, nasopharyngeal and
orbital regions, and the neurocranium. In rare cases,
hepatic, gastric, anorectal, adrenal, and umbilical cord
teratomas also have been reported.1-5
In the current study, we report a congenital vulvar
teratoma, which is a very unusual site in a newborn and
a unique case appearing in vulva. We also review the
literature relevant to teratomas and neonatal tumors of
the vulva.
CASE REPORT
A girl, who is a first child of the family, was delivered vaginally in
the 38th week of gestation. Her birth weight was 2,600 g. There was no
familial relationship between the parents, and the pregnancy was
uneventful. The neonate was transferred to the hospital because of a
solid tumor in the vulva on her first day of life. A pedinculated spheric
mass 55 45 40 mm in diameter was seen on her physical
examination. The mass was soft and covered with skin. It was originating from the vulva with a pedicle, and the base of the mass was just
below the clitoris and above the urethral opening. The base of the mass
(its pedicle) was 1 cm in diameter. Two reddish openings on the mass
were catheterized, and 2 blind sinuses approximately 1 cm in length
were determined (Fig 1). All the external genital structures, urethral,
and vaginal orifices were completely normal. The baby was found

From the Departments of Pediatric Surgery, Obstetrics and Gynecology, and Pathology, Suleyman Demirel University, Medical School,
Isparta, Turkey, and the Department of Pediatric Surgery, Krkkale
University, Medical School, Krkkale, Turkey.
Address reprint requests to Murat Cakmak, MD, Bars Sitesi, 80 Sok.
No: 5, Mustafa Kemal Mah, Ankara, Turkey.
Copyright 2001 by W.B. Saunders Company
0022-3468/01/3604-0018$35.00/0
doi:10.1053/jpsu.2001.22302
620

INDEX WORDS: Teratoma, vulva.

Pathology
The specimen was fixed in 10% formalin and embedded in paraffin,
processed in the usual manner, and stained with H&E. Some sections
also were immunostained with monoclonal antibodies to S100 (Dako
Corp, Carpinteria, CA) and desmin (Dako Corp).

Macroscopy
The mass was soft and largely covered by skin. There were 2 reddish
openings that were blind sinuses. On the cut surface, yellow, lobulated
adipous tissue, grayish-white areas, and several little cysts were seen.

Microscopy
Microscopic examination found a mature teratoma consisting of a
lipomalike mature fat tissue with fibrous septae, lymphoid follicles, and
cystic areas, which were lined internally with intestinal epitelium
changed into stratified squamous epitelium underlying fibrous connective tissue, which contained skin adnex, peripheral nerves, islands of
fat, and smooth muscle. There were normal fibrous tissue of the
resection margins.

DISCUSSION

Teratomas are embryonal neoplasms consisting of

tissues of the 3 germ layers.4 Excluding testicular teratomas, 75% of these tumors occur in girls, and approximately 80% are benign. Teratomas occuring in infancy
Journal of Pediatric Surgery, Vol 36, No 4 (April), 2001: pp 620-621

VULVAR TERATOMA

621

Fig 1. A pedinculated spheric mass 55 45 40 mm in diameter

and 2 reddish openings.

and early childhood usually are extragonadal, whereas

older children predominantly present with gonadal teratomas.6
Although it has been reported that mature teratomas
were the most common tumors among the neonatal solid
tumors, teratomas are tumors that rarely exist in children.7 The most common site for a neonatal teratoma is
the sacrococcygeal region. Their location in vulva, however, is an unusual finding.1 Congenital tumors of the
vulva are rare conditions. Some benign or malignant
tumors including benign hemangiopericytoma, neurofibroma, malignant schwannoma, rhabdomyosarcoma, and
primary endodermal sinus tumor also should be considered in addition to the teratoma.8-10
Levard et al11 reported a case of congenital neoplasm
of the clitoris, which was a choristoma. This was an
aberrant rest or heterotopic tissue, located at the tip of the

clitoris, which contained intestinal tissue.11 The investigators explained that the urogenital membrane at the
origin of the vulvar mucosa derives from endodermlike
intestine. Choristoma is defined as microscopically normal cells or tissues that are present in abnormal locations
as a result of embryologic developmental anomaly,
whereas teratoma contains tissues of the 3 germ layers.12
Although there are different widely accepted theories
about the histogenesis of teratomas, one is the germ cell
theory. Because primitive germ cells possess all the
DNA for any type of cell, they have the ability to form
the tissues seen in teratomas. The migration pattern of
these cells also explains the gonadal, midline, and paramedian locations of the teratomas.1
Jona3 reported the first case of congenital anorectal
teratoma that originated in the rectal wall, which had no
connection with the coccyx and presented as a prolapsing
posterior perineal mass at birth. It contained 2 sinuses 1
and 2 cm in depth, and pathologic diagnosis was benign
mature teratoma that contained intestinal tissue, like our
case.3 There were 2 recurrences at 9 and 12 months in
this case, which required reexcision.
Congenital teratomas of the vulva are rare conditions
seen in the newborn. Some other benign or malignant
tumors of the vulva should also be considered. Because
of possible malignancy or recurrence, complete resection
of the tumor is the treatment of choice. Long-term
follow-up and control of the patient by physical examination, tumor markers, and by radiologic studies must be
done after the operation.

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reported case in a female infant. J Pediatr Surg 30:889-890, 1995
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6. Azizkhan RG, Caty MG: Teratomas in childhood. Curr Opin

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8. Bond SJ, Seibel N, Kapur S, et al: Rhabdomyosarcoma of the
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