Teratomas are tumors composed of multiple cell types from different germ layers. They most commonly occur in the sacrococcygeal region. Sacrococcygeal teratomas present as large masses protruding from the buttocks in neonates or as presacral tumors detected later in infants. Diagnosis involves imaging and tumor markers. Treatment involves complete surgical resection of the tumor and coccyx through a chevron incision in the first week of life to prevent recurrence.
Teratomas are tumors composed of multiple cell types from different germ layers. They most commonly occur in the sacrococcygeal region. Sacrococcygeal teratomas present as large masses protruding from the buttocks in neonates or as presacral tumors detected later in infants. Diagnosis involves imaging and tumor markers. Treatment involves complete surgical resection of the tumor and coccyx through a chevron incision in the first week of life to prevent recurrence.
Teratomas are tumors composed of multiple cell types from different germ layers. They most commonly occur in the sacrococcygeal region. Sacrococcygeal teratomas present as large masses protruding from the buttocks in neonates or as presacral tumors detected later in infants. Diagnosis involves imaging and tumor markers. Treatment involves complete surgical resection of the tumor and coccyx through a chevron incision in the first week of life to prevent recurrence.
Introduction Teratomas are tumors comprising more than a single cell type derived from more than one germ layer
Teratomas range are :
benign
well-differentiated cystic lesions (mature)
solid and malignant (immature)
Pathophysiology Teratomas are made up of a variety of parenchymal cell types representative of more than a single germ layer Teratoma arise from totipotential cells, which typically are midline or paraxial The most common location is sacrococcygeal (57%). The frequency of sacrococcygeal teratomas is 1 per 20,000-40,000 births. Sacrococcygeal Teratoma Sacrococcygeal teratomas more common in females than in males with ratio of approximately 3-4:1 Can be diagnosed in the prenatal period Complications may occur in utero or during or after birth Approximately 10% of sacrococcygeal teratomas are associated with other congenital anomalies, primarily defects of the hindgut and cloacal region. Clinical Findings Sacrococcygeal teratomas not diagnosed antenatally present in 2 patterns neonates, who present with a large, predominantly benign tumor protruding from the sacral area the newborn may exhibit only asymmetry of the buttocks or present when aged 1 month to 4 years with a presacral tumor that may extend into the pelvis. The latter group is at higher risk for malignancy. Differential diagnosis Sacrococcygeal teratomas should be differentiated from neural tube defects (meningoceles or meningomyeloceles) epidermoid cysts anal duct or pilonidal cysts rectal abscesses Lymphangiomas imperforate anus rectal prolapse. Workup Level of serum AFP and beta HCG may indicating benign or malignant teratomas CT scanning of the abdomen and pelvis before surgical exploration can further delineate sacrococcygeal tumor from normal anatomic features. Fine-needle aspiration or core biopsy can be used to differentiate benign from malignant mediastinal masses in 90% of cases. Type I Staging tumors are predominantly external, attached to the coccyx, and may have a small presacral component (45.8%) No metastases were associated with this group. Type II tumors have an external mass and significant presacral pelvic extension (34%) have a 6% metastases rate. Type III tumors are visible externally, but the predominant mass is pelvic and intraabdominal (8.6%). 20% rate of metastases was found in this group. Type IV lesions are not visible externally but are entirely presacral (9.6%) and have an 8% metastases rate. Treatment The treatment of mature teratomas is largely surgical may benefit from in utero surgery should be resected electively in the first week of life, Complete excision with chevron-shaped buttock incision, with preservation of the muscles of the rectal sphincter. The coccyx always should be resected with the tumor, to prevent recurrency Hemorrhage from the middle sacral vessels and hypogastric arteries is the most common complication.