Immature congenital teratoma of the neck: a very rare entity

Immature congenital teratoma of the neck: a very rare entity

Abderrahim Siati^1,&, Mohammed Boukhechba², Abdelaziz Baidada¹, Aicha Kharbach¹

¹Department of Gynecology and Obstetrics, Maternity Souissi Hospital, University Hospital Center IBN SINA, University Mohammed V, Rabat, Morocco, ²Department of Pathology, Mohamed V Military Hospital, University Hospital Center IBN SINA, University Mohammed V, Rabat, Morocco

^&Corresponding author
Abderrahim Siati, Department of Gynecology and Obstetrics, Maternity Souissi Hospital, University Hospital Center IBN SINA, University Mohammed V, Rabat, Morocco

Introduction    

Teratoma is a congenital tumor derived of tissues from the three embryonic germ layers: ectoderm, mesoderm and endoderm [1]. These tumors are commonly found in the sacrococcygeal region. The cervical area is rarely affected [2]. This exceptional cervical location led to a significant risk of compression and the occurrence of acute respiratory distress in the newborn. Here, we present a case of a congenital teratoma of the neck. Fortunately, the diagnosis was made before delivery.

Patient and observation     

A 22-year-old woman, G1P1, who came from a rural area, was admitted to our obstetrics department for delivery. She had no document of pregnancy follow-up. The ultrasound showing a live fetus at term with voluminous cervical mass (Figure 1). No other malformation was detected. After consultation with the neonatal resuscitation team, a caesarean section was performed urgently. A live female neonate was delivered weighing 3300g and the Apgar score was 07/09 at respectively 3/5 minutes of life. The baby was admitted immediately at the neonatology department and remained asymptomatic. On local examination, there was a solitary left cervical mass measuring 100mm of size (Figure 2A). The consistency was firm with moderate mobility. There were no skin changes. A cervical computed tomography (CT) was done, that showed a 92 x 55 x 48 mm mass, with solid and cystic components and calcifications. The contralateral airway was displaced with a posterior displacement of the left carotid artery. No invasions were observed of adjacent structures (Figure 2B). The diagnosis that was proposed by the radiologist was a congenital teratoma, but a reshaped hemolymphangioma was also suggested. The biological analyzes including tumor markers were unremarkable. The infant underwent a surgical excision at 25 days of life, and was discharged from the hospital on the 4th postoperative day. Macroscopically, It was a whitish tumor, surrounded by a capsule (Figure 3A). The histological report was revealed mature and immature elements derived from all three germ layers (Figure 3B). Mature elements included glands, neural tissue, and mature cartilage. Immature elements comprised of immature neuroepithelial tissue, neuroectodermal rosettes, and immature cartilage. The diagnosis was considered as an immature cervical teratoma without any malignant elements.

Discussion     

Teratoma is a tumor derived from the transformation of multipotent germ cells. They are composed of ectodermal, endodermal and mesodermal tissues in varying proportions, hence the term embryonic tumor [3,4]. It is a rare tumor, 1/40000 births. Cervical localization represents 1.5 to 5% of all locations [5]. There is a clear female predominance (75% of cases). Its volume, preventing the normal growth of the fetus, may be responsible for hypotrophy or prematurity. Germ cells migrate at the expense of the yolk sac during the first weeks of intrauterine life, and colonize the sexual cord forming undifferentiated primitive gonads. They can stop along their migration to transform themselves and form a benign or malignant germ cell tumor, which can thus be located from the head to the coccyx of the child [5,6]. Teratoma is a very heterogeneous tumor. We can find fragments of hair, bone or cartilage. It is necessary to analyze all parts of the tumor so as not to ignore an undifferentiated malignant area whose presence could change the prognosis [5,7]. The antenatal diagnosis makes it possible to prepare the care of the newborn by a multidisciplinary team, in front of the risk of respiratory distress, but also to prevent a dystocia at the birth, or a rupture of the tumor. Unfortunately, the diagnosis in the most cases is done only at birth [5,7]. Thus respiratory distress, caused by the tumor, would be fatal for the newborn. The case of our study fortunately had little or no respiratory signs and the diagnosis was performed before delivery. At birth, the scanner or better the MRI, allow a good study of the tumor, by determining its characteristics, the presence of the calcifications and by specifying its relations with the organs and vessels. The assessment will be completed by the determination of alpha fetoprotein, which will be repeated after excision [7,8]. The prognosis is especially respiratory. Thus the antenatal diagnosis allows a good care. If prenatal diagnosis is made, two procedures may be used: EXIT (ex-utero intra partum technique) and OOPS (operation on placenta support). In the EXIT procedure, the fetal head is exteriorized to allow exploration of the airways, the rest of the body with the umbilical cord and the placenta remain in utero. In the OOPS procedure, the newborn is completely exteriorized, the umbilical cord is clamped only after securing the airways with intubation or tracheostomy [9]. These techniques require maximal uterine relaxation, which may be responsible for severe uterine bleeding, and complications for the newborn such as thrombocytopenia, ascites or pleurisy [4,8,10]. The treatment of congenital cervical teratoma is surgical. It must be done very early because the tumor may have an unpredictable evolution and suddenly increase in volume due to intratumoral hemorrhage. Difficulties related to intubation is not negligible [11]. The quality of excision conditions the prognosis; in case of tumor residues, chemotherapy is necessary, based on platinum salts and other drugs [12].

Conclusion     

The congenital cervical teratoma is a very rare tumor. Prenatal diagnosis is necessary for good multidiciplinary management. The treatment is surgical. This must be done very early to allow good results, with a low rate of complications.

Competing interests     

The authors declare no competing interests.

Authorscontributions     

All the authors have read and agreed to the final manuscript.

Tables and figures     

Figure 1: antenatal ultrasound showing a voluminous and heterogeneous mass

Figure 2: (A) representing clinical appearance of the cervical mass; (B) cervical computed tomography showing a 92 x 55 x 48 mm mass, with solid and cystic components and calcifications

Figure 3: (A) the macroscopic appearance of the tumor; (B) histological examination of immature teratoma, showing immature elements admixed with mature tissue (HES)

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