Sacrococcygeal teratoma (SCT) is a well known tumor of the newborn with a male: female ratio of about 1: 3 has an excellent prognosis provided adequate surgical treatment is given to the patient. Teratoma is believed to arise from the totipotent somatic cells that originate from the primitive knot (Hensen’s node) presenting usually attached to the coccyx. We present a case report of a 1 year female who presented with midline swelling in the saccrococcygeal region since birth. The baby was operated and specimen sent for histopathology, where a diagnosis of extra spinal teratoma was made. The patient was kept on one month follow-up after 15 day post operative period and is healthy.
Sacrococcygeal teratoma (SCT) is a well known tumor of the newborn with a male: female ratio of about 1: 3 has an excellent prognosis provided adequate surgical treatment is given to the patient 1. It has a reported incidence of approximately one in 35,000-40,000 live births [1-3]. Although generally a rare condition, it is said to be the most common tumor in the neonatal period. Historically, teratomas were attributed to demons, sexual misconduct and abnormal fertilization . However scientifically there are many conflicting theories for its origin, the most commonly accepted theory being the presence of three germ cell layers giving rise to teratomas of various sizes and shapes [4,5]. Teratoma is believed to arise from the totipotent somatic cells that originate from the primitive knot (Hensen’s node) presenting usually attached to the coccyx [5,6]. Here with we present a case report of a 1 year female who presented with midline swelling in the saccro-coccyxegeal region since birth. The baby was operated and specimen sent for histopathology, where a diagnosis of extra spinal teratoma was made. The patient was kept on one month follow-up after 15 day post operative period and is healthy.
A 1 year female presented with swelling in the lumbar region with difficulty in walking. Spinal MRI revealed a heterogeneously enhancing mass lesion in the saccrococcygeal region. All other routine investigations were within normal limits.
Gross findings: A globular soft tissue piece with attached skin measuring 6x5x3cms was received. The growth measured 4x2cms while the skin ellipse measured 2x3 cms. Outer surface of growth had mucosal rugosities while inner surface had fibrofatty tissue and cartilaginous areas. Specimen was fixed in 10% formalin after giving multiple incisions for the formalin to penetrate.
Microscopic examination: Section from tissue
show stratified squamous epithelial lining. Underlying
fibrocollagenous stroma show glands lined by mucin containing
columnar cells. Section also showed smooth muscle fibres,
adipocytes, gastrointestinal epithelium, nerve fibres, lymphoid
tissue and cartilage and mild chronic inflammatory infiltrate
was also seen. A diagnosis of extra spinal teratoma was made
Immature malignant sacrococcygeal teratoma (SCT) is a rare
tumor occurring in the neonates with a female predilection. The
most frequent site of tumor occurrence is the lumbar region and
the child usually presents with a lumbar region growth. The most
common theory accepted for its development is that it is a benign
tumor that has been derived from the three germinal layers in
the sacrococcygeal region . The incidence of this tumor type is
one in 35,000 to 40,000 live births .The most common diagnostic
modality available for its diagnosis is the prenatal ultrasound
and magnetic resonance imaging (MRI). Due to lack of study data
in the field of Sacrococcygeal and cervical teratomas, they are
being considered an interesting field for research .
Human tails and pseudotails are rare sacrococcygeal
lesions that are associated with a wide variety of anomalies
and syndromes. These tails and pseudotails occur in common
conjunction with Anorectal malformations. These malformations
are relatively uncommon congenital defects that often occur
in with syndromes or other congenital abnormalities . The
anomalies associated with both disorders determine the timing
and approach to surgical correction. Presence of both imperforate
anus and a pseudo tail in the absence of a syndrome or other
associated anomalies is an unusual phenomenon necessitating
the need of a thorough preoperative evaluation. A true tail is
defined as the remnant of the embryonic tail, which usually
regresses during the seventh and eighth weeks of gestation. A
pseudo tail is a protrusion from the lumbosacrococcygeal area
that may be composed of normal or abnormal tissues but is not
derived from the embryologic tail .
In contrast to the newborn with SCT, the fetus in utero with
SCT remains at high risk of perinatal complications and death
. Fetuses with SCT detected antenatally have three times
mortality rate compared with postnatally diagnosed neonates
. The cause of in utero fetal death can be maternal obstetric
complications of tumor rupture, preterm labor, or dystocia .
The typical presentation of SCT as a skin-covered lumbosacral
masses in the newborns leads to an array of possible diagnoses,
ranging from benign hamartomas to aggressive malignancies
and meningoceles .
A neonate with sacrococcygeal teratoma (SCT) has an
excellent prognosis depending on the timing of diagnosis, the
ease of surgical resection and malignant potential of the tumor
Anorectal malformations and skin-covered midline
growths present as a challenging diagnostic and therapeutic
entities because of the extensive differential diagnoses ranging
from benign hamartomas to aggressive malignancies and
meningocoele and the array of associated anomalies. Hence
extensive discussion and research is needed to improve the
neonatal morbidity and mortality due to extra spinal teratomas.