|CLINICAL CASE STUDY
|Year : 2012 | Volume
| Issue : 1 | Page : 30-34
Unusual presentations of sacrococcygeal teratomas in pediatric patients
Iftikhar A Jan1, Hazratullah1, Nazia Ishaque1, Anwar-ul Haq2, Mudassar Gondal2, Ahmad Sharif3, Sofia Ahmad3
1 National Institute of Rehabilitation Medicine, Islamabad, Pakistan
2 The Children's Hospital, PIMS, Islamabad, Pakistan
3 National Institute of Child Health Karachi, Pakistan
|Date of Web Publication||13-Apr-2012|
Iftikhar A Jan
Professor of Pediatric Surgery and HOD, National Institute of Rehabilitation Medicine, Islamabad
Source of Support: None, Conflict of Interest: None
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.
Keywords: Fetus in fetu, sacrococcygeal, teratoma, unusual presentation
|How to cite this article:|
Jan IA, Hazratullah, Ishaque N, Haq Au, Gondal M, Sharif A, Ahmad S. Unusual presentations of sacrococcygeal teratomas in pediatric patients. Saudi J Health Sci 2012;1:30-4
|How to cite this URL:|
Jan IA, Hazratullah, Ishaque N, Haq Au, Gondal M, Sharif A, Ahmad S. Unusual presentations of sacrococcygeal teratomas in pediatric patients. Saudi J Health Sci [serial online] 2012 [cited 2019 Jun 18];1:30-4. Available from: http://www.saudijhealthsci.org/text.asp?2012/1/1/30/94981
| Introduction|| |
Sacrococcygeal teratomas are the most common tumors in the newborn babies.  Prenatal diagnosis is now a routine in developed countries due to advances in fetal sonography, where more than 50% cases may be detected antenatally.  After delivery, the diagnosis is easy in teratomas with an external mass, however, in pure presacral teratomas the diagnosis may be significantly delayed. Large external SCT may be misdiagnosed as meningomyelocele. Unusual presentations of SCT are not uncommon, especially in the developing countries and presentations like lower limb weakness,  urinary and bowel obstruction,  fetus in fetu,  ruptured teratoma  have been reported in the literature. During the last few years, we have noticed that, we are getting many patients with significant delayed in diagnosis or erroneous diagnosis due to unusual presentations of the tumors. We reviewed these cases in detail with a view to understand pattern of unusual presentations of patients with SCT and factors responsible for the delay in diagnosis.
| Methods and Techniques|| |
We managed 23 cases of SCT at NICH Karachi and Children's Hospital Islamabad, from Jan 2004 to June 2008. We reviewed their mode of presentation, age of presentation, complications and previous management issues. All patients were fully evaluated by ultrasonography, Serum alpha Fetoprotein levels and CT or MRI scans along with the routine investigations. Patients were categorized by Altman et al. classification for the type of SCT. There is no standard classification of unusual presentation of SCT, We therefore, labeled the patients as having unusual presentation if they had either a erroneous diagnosis at referral, had complication of previous surgery, had other symptoms for referral or presented late due to erroneous or missed diagnosis. On confirmation of the diagnosis, the patients were managed according to standard protocols. Primary excision was performed in patients with resectable tumors and those with advance disease had chemotherapy before surgery.
| Results|| |
Twenty three patients were registered. There were 17 Female and 6 male suggesting a nearly 3:1 preponderance of females. Ten (43%) presented in the newborn period. Five (22%) presented from one month to one year of age. Eight (35%) patients presented after one year of age. Unusual presentations were noted in 12 (52%) patients. Five babies were referred with the diagnosis of meningomyelocele [Figure 1]. All these babies had a large presacral masses; one had a ruptured teratoma and was in sepsis [Figure 2]. He later died of fulminant sepsis. All other were managed by primary excision and survived.
One baby presented in the newborn period with an abdominal mass. He had an absent right kidney. Complete excision of the mass was performed in newborn period [Figure 3]a. Histology confirmed it to be a teratoma. He initially had high serum alpha fetoprotein (SAFP) levels that came to normal after six months. Baby presented on his first birthday with a small coccygeal mass with normal SAFP levels. MRI showed SCT with extension in presacral area. Excision was performed and histology confirmed it to be a SCT [Figure 3]b. Child is well at one-year follow-up.
Another baby presented with hematuria in the newborn period. Ultrasound image suggested a presacral mass that was confirmed as type-IV, SCT and excision was performed.
A three-year-old child was brought with intestinal obstruction and anuria. She initially developed weakness of lower limbs and had a myelogram in a peripheral hospital. Diagnosis of vertebral disc prolapse was made and she underwent laminectomy but the symptoms worsened, and she also developed intestinal obstruction and anuria. The parents brought her in a terminal stage [Figure 4]. On arrival, the child was very sick. She had a large presacral SCT causing bowel and urinary obstruction. A suprapubic cannula was already placed with no urinary drainage as the mass was obstructing the ureter. The child died within few hours in spite of resuscitation, due to sepsis and other factors.
|Figure 4: Spinal laminectomy for suspected disc prolapse in a child with type-IV SCT|
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One child presented in emergency with a soft fluctuant swelling on the back. On call resident thought it was an abscess and made an incision for drainage [Figure 5]. Only blood was drained. Later evaluation revealed that she had a type IV, SCT with advance disease.
One child presented with a fecal fistula at the back, she was 4 years and evaluation revealed that she had a type-IV SCT.
One child had surgery for a sinus in the sacral area in a tertiary care hospital. She developed multiple fistulae and was operated four times before referral [Figure 6]. Evaluation revealed that she had a type-IV, SCT that was missed and as excision of the coccyx was not performed, therefore she was getting a recurrence. En-block excision of the mass along with coccyx was performed. She had a mature teratoma on histology and was symptoms free at one-year follow-up.
One eight-year-old child presented with urinary incontinence. She was initially evaluated and a diagnosis of spina bifida occulta made with neuropathic bladder. She was referred for Mitroffanof procedure. MRI of the spine was performed, which showed a large presacral mass attached to the coccyx. Diagnosis of SCT was made and excision of the mass performed. It was a large mass and contained axial skeleton on X-ray. Histology showed mature bone, cartilage, intestine and neural tissue. Final diagnosis was fetus in fetu presenting as a presacral mass [Figure 7].
A two-year-old girl presented with inguinal lymphadenopathy [Figure 8]. Examination revealed that, she had a type-IV SCT with inguinal metastasis.
Outcome was most favorable in external SCT. Of the ten patients with type-I and Type-II teratomas only one died and he was the baby with 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 was interesting to note that except for two, all other cases of type-I and type- II SCT presented in the first month of life ensuring good prognosis. All type-IV presented late and had the worse outcome.
| Discussion|| |
Sacrococcygeal teratoma is the most common congenital neoplasm, and is seen in one in every 35,000 live births.  SCT occurs more often in girls, than in boys with a ratio of 3:1 to 4:1.  This was also noted in our study. Antenatal diagnosis has significantly improved by the good quality ultrasound evaluation. Fetal sacrococcygeal teratomas diagnosed in utero does not ensure good outcome and caries high risk of preterm delivery, mortality rate of 15-35%, and a morbidity rate of 12-68%. , Families with autosomal dominant inherited presacral teratomas have been are reported. 
The diagnosis of SCT after birth is easy in type-I and type -II tumors due to the location and appearance of the mass. The confusion may be with lumbosacral meningomyelocele (MMC), as large cystic teratomas and lumbosacral meningomyelocele may have a similar appearance. SCT rarely cause neurological deficit of lower limbs, which may be the main presentation in MMC. Furthermore, other associations like hydrocephalus are absent in SCT. In some patients, SCT and MMC may occur together making the diagnosis more difficult, which may be confirmed by lumbosacral MRI. Elevated SAFP are present in both NTD and SCT and cannot be used as an effective tool for differential diagnosis.
The delay in diagnosis is seen mostly in type-III and Type-IV SCT due to hidden tumor mass and patients may present with local complications. Intestinal obstruction, urinary retention and lower limb weakness are the common symptoms in these patients. Other unusual presentations are fetus in fetu, curarino's triad and ruptured teratomas.
Five of our patients presented with the diagnosis of meningomyelocele and of them one had a ruptured cystic teratoma. This is also reported in the literature.  There are also case reports of SCT associated with anterior meningocele.  Some of our patients presented in the most unusual way, which have not been reported previously. One baby presented with hematuria and an ultrasound was advised for any urological pathology, which suggested a pelvic mass. The diagnosis of SCT was confirmed by MRI scan and tumor resected. No communication of urogenital system was noted with the teratoma in this patient and the hematuria may be vaginal bleeding by the hormonal affect.
Another child, who was misdiagnosed as an abscess with a soft swelling over lower back had a hemorrhage in the tumor. This has also not been reported before and also highlights the importance of good local examination in suspected abscesses at unusual location and loss of constitutional symptoms should have alerted the resident for a proper evaluation.
Weakness of lower limbs is a known complication of SCT.  It has previously been reported but our case with lower limbs weakness was unique as the treating physician performed a myelogram on the child and then suspecting disc prolapse performed a laminectomy. Disc prolapse is virtually unknown in children. In our setup, the common cause of acquired spinal problem is tuberculosis of the spine. Most children with tuberculosis have vertebral erosions and diagnosis may be confirmed by MRI scan rather than myelogram. In any case, the missed diagnosis and wrong treatment caused significant delay in the management and the child died due to advance disease. A good local examination could have suggested the diagnosis earlier.
The problem of inadequate treatment is another dilemma responsible for unusual presentation of SCT.  Patient with multiple sinuses was a classical example where a surgeon performed inadequate tumor resection by leaving the coccyx behind. This resulted in residual disease and local infection causing multiple sinuses. Repeated surgeries did not improve the patient due to residual disease. Complete tumor excision along with coccygectomy was curative. The patient also has mature teratoma and remained symptoms free one year after surgery.
There are several reports of fetus in fetu arising from coccyx. , Our patient was unique. She was having urinary symptoms for the last 4 years and was diagnosed as a case of spina bifida occulta with neuropathic bladder. She had wide sacrum on external examination but no evidence of a large presacral mass. Spina bifida, presacral mass and anorectal malformation constitute the currarino's triad. , This child however, had normal anal canal. The presacral mass was picked when MRI scan was performed to exclude tethered cord syndrome. The resected specimen was diagnosed as fetus in fetu due to the formation of axial skeleton. There may be a debate regarding the terminologies but definitely it was a mature teratoma with well formed axial skeleton and other mesenchymal tissue.
Patient with a concomitant abdominal and SCT is also unique as this has not been reported previously. Initially, we thought that the patient had an extension of the SCT teratoma, which was missed during initial surgery. When, we retrieved the record and photographs, we found that there was complete tumor resection on initial surgery and repeated ultrasound image did not reveal any pelvic mass. Furthermore, SAFP levels came to normal after 6 months. Therefore, this was a unique case of concomitant abdominal and SCT.
After reviewing our cases, we noted that there were many cases of unusual presentation of SCT. In some cases it caused significant delay in diagnosis and poor outcome. The reason was hidden tumors, ignorance of the families, lack of awareness, inadequate evaluation by the treating physicians in peripheral hospitals. In some cases, the unusual presentation was due to the natural process and was unavoidable. 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 shall be referred to tertiary care pediatric surgical facilities for optimal care and management.
| References|| |
|1.||Tailor J, Roy PG, Hitchcock R, Grant H, Johnson P, Joseph VT, et al. Long-term functional outcome of sacrococcygeal teratoma in a UK regional center (1993 to 2006). J Pediatr Hematol Oncol 2009;31:183-6. |
|2.||Swamy R, Embleton N, Hale J. Sacrococcygeal teratoma over two decades: Birth prevalence, prenatal diagnosis and clinical outcomes. Prenatal Diag 2008;28:1048-51. |
|3.||Sharma MC, Aggarwal M, Ralte AM, Vaishya V. Clinicopathological study of spinal teratomas. A series of 10 case. J Neurosurg Sci 2003; 47:95-100. |
|4.||Wakhlu A, Misra S, Tandon RK, Wakhlu AK. Sacrococcygeal teratoma. Pediatr Surg Int 2002; 18:384-7. |
|5.||de Lagausie P, de Napoli Cocci S, Stempfle N, Truong QD, Vuillard E, Ferkadji L, et al. Highly differentiated teratoma and fetus-in-fetu: A single pathology? J Pediatr Surg 1997;32:115-6. |
|6.||Hoehn T, Krause MF, Wilhelm C, Lattermann U, Rueckauer KD. Fatal rupture of a sacrococcygeal teratoma during delivery. J Perinatol 1998;19:596-8. |
|7.||Sharma AK, Sharma CS, Gupta AK, Sarin YK, Agarwal LD, Zaffar M. Teratomas in pediatric age group: Experience with 75 cases. Ind Pediatr 1993;30:689-94. |
|8.||Makin EC, Hyett J, Ade-Ajayi N, Patel S, Nicolaides K, Davenport M. Outcome of antenatally diagnosed sacrococcygeal teratomas: Single-center experience (1993-2004). J Pediatr Surg 2006;41:388-93. |
|9.||Gopal M, Turnpenny PD, Spicer R. Hereditary sacrococcygeal teratoma-not the same as its sporadic counterpart! Eur J Pediatr Surg 2007;17:214-6. |
|10.||Bader I, Akhter N, Haq AU, Khattak J, Abassi Z. Anterior sacral meningomyelocele with sacrococcygeal teratoma. J Coll Physicians Surg Pak 2004;14:302-3. |
|11.||Basu A, Jagdish S, Iyengar KR, Basu D. Fetus in fetu or differentiated teratomas? Ind J Pathol Microbiol 2006;49:563-5. |
|12.||O'Riordain DS, O'Connell PR, Kirwan WO. Hereditary sacral agenesis with presacral mass and anorectal stenosis: The Currarino triad. Br J Surg 1991;78:536-8. |
|13.||Schropp KP, Lobe TE, Rao B, Mutabagani K, Kay GA, Gilchrist BF, et al. Sacrococcygeal teratoma: The experience of four decades. J Pediatr Surg 1992;27:1075-8. |
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