|Year : 2020 | Volume
| Issue : 2 | Page : 717-719
Type 3 sacrococcygeal teratoma presenting as a huge abdominal lump in a neonate
Aditya P Singh1, Arun K Gupta1, Maryem Ansari2, Ramesh Tanger1
1 Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India
2 Department of Pathology, SMS Medical College, Jaipur, Rajasthan, India
|Date of Submission||29-Apr-2018|
|Date of Decision||03-Jun-2018|
|Date of Acceptance||09-Jun-2018|
|Date of Web Publication||27-Jun-2020|
Aditya P Singh
MS, M Ch in Pediatric Surgery, Near The Mali Hostel, Main Bali Road, Falna, Pali, Rajasthan - 306 116
Source of Support: None, Conflict of Interest: None
Sacrococcygeal teratoma (SCT) is the commonest solid tumor in neonates, occurring in one in 40 000 births. Early removal of the tumor is advised in neonates as there is increased chance of malignant transformation with age. There are four types of the SCT. Type 3 is a relatively rare and usually present in older children. We are presenting here a case of type 3 SCT in a neonate with huge abdominal lump.
Keywords: abdominal lump, excision, sacrococcygeal teratoma
|How to cite this article:|
Singh AP, Gupta AK, Ansari M, Tanger R. Type 3 sacrococcygeal teratoma presenting as a huge abdominal lump in a neonate. Menoufia Med J 2020;33:717-9
|How to cite this URL:|
Singh AP, Gupta AK, Ansari M, Tanger R. Type 3 sacrococcygeal teratoma presenting as a huge abdominal lump in a neonate. Menoufia Med J [serial online] 2020 [cited 2020 Aug 11];33:717-9. Available from: http://www.mmj.eg.net/text.asp?2020/33/2/717/287754
| Introduction|| |
Sacrococcygeal teratoma (SCT) has an incidence of ∼1/40 000 live births and is the commonest solid tumor in the neonate. In 1973, Altman classified SCT into four types based on the external component and intrapelvic/intra-abdominal extension of the tumor (American Academy of Pediatrics Surgical Section classification). The SCTs seen at birth are usually Altman type I and II (87%). In rare cases, type III can also be seen in neonates. Type IV is typically seen later in life as there is no external component. We are presenting here a type 3 SCT in a neonate with a huge cystic abdominal lump.
| Case Report|| |
The parent of a male newborn presented to us with the complaint of abdominal distension since birth. There was no history of any vomiting and baby had passed meconium. Baby was the product of normal vaginal delivery with birth weight of 2.8 kg. Antenatal ultrasonography was not done. Abdomen was distended and a cystic lump was palpable around 9 × 8 cm in the whole abdomen [Figure 1]a. There was also swelling present in the sacrococcygeal area around 3 × 2 cm in size [Figure 1]b. Routine blood investigations were within normal limits including complete blood counts, renal function test, liver function test and serum electrolytes. Two-dimensional echo showed patent foramen oval with normal study finding. Ultrasonography of the abdomen showed a large cyst in abdominal pelvic cavity of size 10 × 11 cm with few septation, suggestive of lymphangioma or mesenteric cyst. In sacrococcygeal region, there was a multiloculated cystic lesion (1.5 × 4.5 cm) with multiple septation, suggestive of SCT. We planned for surgical exploration. After opening the abdomen through right supraumbilical region, there was large retroperitoneal cystic mass displacing the bowel [Figure 1]c. We dissected the swelling all round with blunt and sharp dissection. It extended deep in the pelvis. Then we aspirated the fluid of cyst and dissected the swelling deep in the pelvis [Figure 1]d. We excised the swelling completely. Histopathology report showed mature cystic teratoma having intestinal epithelium, adipose tissue, glial tissue and nerve tissues [Figure 2]. The patient was in follow-up for last 6 months without recurrence.
|Figure 1: (a and b) Preoperative photograph, (c) peroperative photograph, (d) Follow-up images after 6 months per operative photo (e and f) follow up image.|
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|Figure 2: Histopathology images: (a) nerve bundles, (b) glial and nerve tissue, (c) nerve and ganglion tissue and (d) glial tissue brain.|
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| Discussion|| |
There are four types of SCTs. The neonate in this case was diagnosed with Altman type 3 SCT which has both an intra-abdominal presacral and extra-abdominal presentation. This tumor has a female preponderance, and factors like antenatal diagnosis before 20 weeks of gestation, prematurity, low birth weight (LBW), low Apgar scores, placentomegaly, nonimmune hydrops, polyhydramnios and malignant histotypes have worse prognosis. In this case, it was a full-term male baby with normal Apgar score with a postnatal diagnosis of SCT. These tumors are seen as heterogeneous well-circumscribed predominantly external caudal masses.
The differential diagnoses of intrapelvic cystic masses include meconium pseudocyst, enteric duplication cyst, fetal ovarian cyst, hydrocolpos and its causes, dilated bowel, bowel duplication, mesenteric cyst, rectal duplication, cystic neuroblastoma, teratoma, or bladder duplication.
There are several reports on the hemorrhage associated with the tumor, complications of massive blood transfusion and even cardiac arrests in neonates.
Hydrops is due to arteriovenous shunting from a large vascular tumor leading to high-output cardiac failure. It carries poor prognosis and is associated with high mortality. It sometimes results in a mirror syndrome where the foetal hydrops causes placentomegaly and ultimately features of maternal hydrops with preeclampsia and oedema develop which requires urgent delivery of the foetus.
Hypervascularity of the tumor has been described in previous reports; the arterial supply being middle sacral artery, which is a branch of internal iliac artery. Apart from the high vascularity, associated coagulopathy, which becomes worse with time, and hypothermia are contributory factors to hemorrhage.
Another problem in these cases is electrolyte imbalance particularly hyperkalaemia which can be due to tumor handling in intra-abdominal tumors, respiratory and metabolic acidosis, hypocalcaemia and also from massive Packed RBC (PRBC) transfusion.
Abraham et al. described a case of ventilatory difficulty from an intra-abdominal tumor pressing on the diaphragm, managed with lifting of the tumor. This was observed in case of intra-abdominal resection in supine position and resulted in increase in peak airway pressure more than 25 mm H2O, reduced tidal volume and air blood gas (ABG) changes and also cardiac arrest. No such problem was encountered in this case. Complete excision of the SCT including coccygectomy is advised to avoid recurrence.
| Conclusion|| |
Type 3 is very rare type of the SCT with a huge abdominal swelling in the neonate.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Altman RP, Randolph JG, Lilly JR. Sacrococcygeal teratoma: American Academy of Pediatrics Surgical Section Survey-1973. J Pediatr Surg 1974; 9
Ashley DJ. Origin of teratomas. Cancer 1973; 32
Abraham E, Parray T, Ghafoor A. Complications with massive sacrococcygeal tumor resection on a premature neonate. J Anesth 2010; 24
Girwalkar-Bagle A, Thatte WS, Gulia P. Sacrococcygeal teratoma: a case report and review of literature. Anaesth Pain Intensive Care 2014; 18
Legbo JN, Opara WE, Legbo JK. Mature sacrococcygeal teratoma: case report. Afr Health Sci 2008; 8
[Figure 1], [Figure 2]