Unusual presentations of sacrococcygeal teratomas in pediatric patients

The present study aims to review the unusual modes of presentation of sacrococcygeal teratomas (SCT) in pediatric patients. In this study we managed 23 cases of SCT, at NICH Karachi and Children's Hospital Islamabad from Jan 2004 to June 2008. Their mode of presentation and previous management issues were studied. All patients had U/S, Alpha Fetoprotein levels, CT or MRI scan beside the routine investigation. Patients were labeled as having unusual presentation, if they had either a erroneous diagnosis at referral, had complication of previous surgeries or presented late due to erroneous or missed diagnosis. Patients were managed on standard protocol with preoperative staging. Of the 23 patients been registered, there were 17 Female and 6 male suggesting a 3:1 preponderance of female. Nearly half of the patients had atypical presentation. Five patients were referred with the diagnosis of meningocele. Hematuria, inguinal lymphadenopathy, suspected abscess, ruptured SCT, fecal fistula, simultaneous abdominal and sacrococcygeal teratoma, laminectomy for lower limb weakness and fetus in fetu were presentations in one patient each. Outcome was most favorable in external SCT which was presented early. Of the 11 patients with type-I and Type-II teratomas, only one died and he had ruptured teratoma. All other survived inspite of the large external tumor masses. Of the seven patients with type-III teratomas, five survived, one died and one lost to follow-up. Of the six babies with type-IV SCT, three survived, one died and two lost to follow-up. It could be concluded that, there were many cases of unusual presentation of SCT. In some cases, it caused significant delay in diagnosis and poor outcome. The reasons were hidden tumors, ignorance of the families, lack of awareness and inadequate evaluation by the treating physicians in peripheral hospitals. In some cases, the unusual presentation was due to the natural process. A high index of suspicion, awareness of treating physicians and parents, can help in early diagnosis and better outcome. Above all, pediatric tumors like SCT need a team approach and all patients with such lesions must be referred early to tertiary care pediatric surgical facilities for optimal care and management.