Fetal Nasopharyngeal Teratoma: An Autopsy Case Report

Document Type : Case report

Authors

1 Department of Pathology, Mashhad Branch, Islamic Azad University, Mashhad, Iran

2 Department of Radiology, Mashhad Branch, Islamic Azad University, Mashhad, Iran

Abstract

Teratoma is a rare type of tumor that can contain fully developed tissues and organs, including hair, teeth, muscle, and bone. Teratomas are most common in the tailbone, ovaries, and testicles, but can occur elsewhere in the body. These tumors originate from all three embryonic germ layers: ectoderm, mesoderm, and endoderm. Nasopharyngeal teratomas are responsible for a high birth mortality rate from acute respiratory distress. We report a rare case of pharyngeal mass in a fetus with antenatal ultrasound and autopsy findings. A 31-year-old primigravid woman with history of insulin-dependent diabetes mellitus and treated hypothyroidism presented with severe polyhydarmnios, prenatal ultrasound revealed a heterogenous solid cystic mass in submandibular region with no vascularity on Doppler exam. The postmortem and pathologic examination confirmed the nasopharyngeal teratoma with extensive cervical extension. The aim of this study was updating current knowledge about this disease.  

Keywords


Introduction
In 1863 Virchow used the term “teratoma” that derived from the Greek word “teraton” (meaning monster(1,2). Teratoma is a true neoplasm and is the most common mass encountered in neonates (3). This is consists of tissues from all three embryonic germ layers: ectoderm, mesoderm, and endoderm (4-6).
Teratomas occur in 1 of 4000 live births with head and neck teratomas accounting for less than 5% of the total (5, 7, 8) and representing 25–35% of neonatal tumours (9). Head and neck teratomas are most commonly cervical with the nasopharynx being the second commonest location (10, 11). Teratomas in the upper areo-digestive tract (UADT) are rare neoplasms, accounting for fewer than 2% of all teratomas (12).

The appearance of nasopharyngeal teratomas varies according to the size, degree of tissue heterogeneity and degree of tissue maturation (9). The most common location for teratomas to be seen within the UADT mucosa is the nasopharynx; other less commonly involved sites include the oral cavity (tonsil, tongue, and palate), sinonasal cavity and the ear and temporal bone. Nasopharyngeal teratoma presents as a mass protruding into the oral cavity or pharynx causing dysphagia and/or airway obstruction (9,13).
True teratoma is a congenital neoplasm of tridermal origin (14). Teratomas may be associated with maternal polyhydramnios, preeclampsia, preterm delivery and respiratory compromise after birth due to upper airway obstruction by the mass and stillbirth (2,15).
A fetal teratoma originating from the base of the skull is called pharyngeal teratoma or epignathus and is associated with other anomalies (16,17). Epignathus is a rare type of teratoma for which accurate prenatal diagnosis is definitive to plan proper peripartum management (10). It usually carries a poor prognosis (15).
Here we report a case of fetal pharyngeal teratoma with ultrasound postmortem findings and review literature.

Case report
A 31-year-old primigravid woman with history of insulin-dependent diabetes mellitus and treated hypothyroidism, underwent a first-trimester ultrasound examination at 13 weeks, which was normal. Neither the first trimester screening test (ultrasound and biochemical markers screening test for aneuploidies) nor second trimester anomaly scan and quadrant blood test (Alpha-fetoprotein (AFP), free β-human chorionic gonadotropin (β-HCG), free estriol, and inhibin A) were performed.
She had no positive history for stillbirth or previous abortion and no malformed neonates within her first relatives. Sonographic examination by a Medison V20 with multi-frequency 3-5 MHz convex probe at 29 weeks of gestation, revealed a single live fetus with biparietal diameter of 72mm, head circumference of 267 mm, femur length of 55mm and abdominal circumference of 251 mm. The placenta was located on the uterine fundus. The amount of amniotic fluid was severely increased (Amniotic fluid Index was 40 cm).
There was a heterogeneous solid-cystic appearance mass with diameters of 48*32*21mm filling the submandibular region bilaterally caused partial opening of the mouth and deformity of profile fetal face (Fig 1a&b). No calcified or echogenic elements were found within the mass and no vascularity was detected by color Doppler ultrasound.
Other fetal organs were unremarkable. The differential diagnosis included cystic hygroma, hemangioma and teratoma. After counseling the parents decided to terminate the pregnancy at the 30 weeks’ gestation. Cesarean section was performed and a 1300gm live girl was born but because airway access was too difficult due to the mass, intubation was failed and it shortly died.

Other examinations were normal, and there was no sign of arthritis or cellulite on the examination of the organs. There was only a slight tenderness in the thighs. The result of the bone marrow aspiration procedure was normal, and a skin biopsy showed the EN pathology.

The patient underwent naproxen treatment, an anti-inflammatory drug. Afterward, the patient’s pain and fever decreased, but the patient still suffered from several ulcerative lesions in the mouth, on the last day of hospitalization.
An infectious disease consultation was performed, and he was discharged with a diagnosis of EN in the context of oral herpes. Two days later, the patient had hematochezia.
Consequently, he was hospitalized and underwent colonoscopy, which showed multiple mucosal lesions in the esophagus and duodenum, and a sample was sent for pathology. The test result revealed Crohn’s disease, and the patient was treated immediately. The results of the laboratory tests of the patient are provided in Table 1.
Most cases of Crohn’s disease occur with gastrointestinal manifestations, and there have only been a few reports on skin problems. This patient was selected to present a rare case of the disease with unusual manifestations and how to diagnose and treat the patient based on symptoms.

 

Antimicrobial susceptibility testing
Antibiotic susceptibility testing was performed based on Kirby-Bauer disk diffusion method. The antibiotic discs used for this purpose included: Cefotaxime, Ceftazidime, Cefepime, Azithromycin, Erythromycin, Clindamycin, Cefoxitin, Levofloxacin, Gentamicin, Trimethoprim/ Sulfamethoxazole, Ciprofloxacin, Amopenem, Meropenem, Amikacin, and Imipenem. Interpretation of inhibitory zone (susceptible, intermediate, and resistant) was performed using clinical and laboratory standard institute (CLSI) instructions.

Statistical analysis
The time series analyses was applied for evaluation of antimicrobial resistance pattern over the times. All statistical analyses was performed using Microsoft Excell software. the results considered as significant, if p-value was ≤0.05. 

Results and Discussion
Changes in the pattern of antibiotic resistance of the most common nosocomial pathogens were investigated in the period 2018-2021 to monitor the drug resistance of these bacteria. During 4 years, 70,234 isolates were identified (A. baumannii: n = 19,374; K. pneumonia n=17,206; E. coli n =23,777; S. epidermidis: n=9,877). The bacteria were isolated from the clinical specimens such as sputum, urine, wounds, biopsy, blood, and sterile fluids.
The overall antibiotic resistance rate of S. epidermidis to different classes of antibiotics included Azithromycin: 84.63%, Erythromycin: 81.25%, Clindamycin: 68.19%, Cefoxitin: 62.20%, Levofloxacin: 45.18%, and Gentamicin: 26.35%. The total resistance to E.coli strain was as follows: Trimethoprim/Sulfamethoxazole: 45.31%, Cefotaxime: 48.62%, Ceftazidime: 59.16%, Ciprofloxacin: 62.0%, Cefepime: 62.73%, Gentamicin: 50.23%, Ceftazidime/Clavulanic acid: 15.60%, Meropenem: 36.39%, Amikacin: 44.74%, and Imipenem: 49.87%. In addition, the overall antibiotic resistance for K. pneumonia included: Cefotaxime: 81.11%, Ceftazidime: 77.46%, and Cefepime: 74.55%. Also, the resistance in relation to A. baumannii was reported as Ceftriaxone: 95.78%, Ceftazidime: 89.54%, Meropenem: 87.03%, Ciprofloxacin: 84.88%, Imipenem: 81.61%, Gentamicin: 80.08% and Amikacin: 76.80%.
The pattern of antibiotic resistance varied over 4 years. Regarding the antibiotic resistance pattern of S. epidermidis, the trend of antibiotic resistance was uniform over four years, and except for levofloxacin, which had a significant increase (p-value: 0.002), the rates of antibiotic resistance for other antibiotics showed no significant difference (Fig 1). Over the last four years, Azithromycin resistance has changed from 85.8% to 83.8%, the resistance of Erythromycin has altered from

 cantly higher than that of the neonates who did not recover (2141.7 ± 755.2 g) (p < 0.01). There was also a significant relationship between the TSH level and birth weight (p < 0.01). Moreover, the mean age of mothers in participants was 26.9 ± 3.7 years, with a minimum and maximum of 12 and 35 years, and did not have any significant relation with the level of TSH and with the recovery rate (p > 0.05).

The mean level of TSH in neonates who recovered within three-month was 9.4 ± 3 mIU/L, and in neonates who did not recover was 22 ± 6.5 mIU/L. The relation between the recovery and TSH level

 Discussion

The present study showed that the prevalence of febrile seizures was associated with gender, living place, temperature, family history of seizure, and the serum level of zinc. In this regard, the frequency of zinc deficiency was higher in patients with febrile seizures compared to febrile patients without seizure, before and after adjusting for gender.
Zinc plays a vital role in the neuronal terminals of the hippocampus and amygdala by producing pyridoxal phosphate and affecting glutamatergic, gamma-aminobutyric acidergic (GABAergic), and glycinergic synapses (13).
Glutamic acid decarboxylase (GAD) acts as a major inhibitory neurotransmitter in the synthesis of gamma-aminobutyric acid (GABA) (14). A study by Ganesh R. and Janakiraman L. on 38 children with febrile convulsion and 38 children as a control group, aged between 3 months and 5 years, indicated that a serum zinc deficiency was significantly more prevalent in their case group than in the control group (15). Another study has reported that there is a correlation between disruption in Zn2+ homeostasis and fever seizure (16).
In studies by Papierkowski A., Mollah M.A., and Gündüz Z. et al., the mean serum zinc level in the febrile convulsion group was significantly lower than in the control group, which indicates the role of zinc in febrile seizure. Comparing the groups in terms of age and gender, no significant difference was found, similar to our study (17-19). Abdel Hameed Z.A. et al. (20), in a study on 100 infants in Egypt, observed that temperature had no significant difference between the case and control groups. But Berg A.T. (21), Ahmed B.W. (22), and our study showed the importance of temperature in febrile seizure. The geographic area can be the cause of this difference. Duangpetsang J. in a study from 2014 to 2017 reported that a high fever with electrolyte disturbance hyponatremia has an important role in FS (23). Sharifi R. et al., in a study in 2007-2014, showed the importance of family history in febrile seizure (24), which is similar to our results.

Conclusion
The findings of this study show that zinc deficiency is significantly associated with the occurrence of febrile seizures. Zinc supplementation in children can therefore be helpful for the prevention and treatment of FS.

Conflict of interest
The authors declare no conflicts of interest.



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