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Epignathus with fetiform features.

Kumar SY, Shrikrishna U, Shetty J, Sitaram A - J Lab Physicians (2011)

Bottom Line: Epignathus is an extremely rare oropharyngeal teratoma that commonly arises from the palate, leading to a high mortality (80-100%) due to airway obstruction in the neonatal period.We present a case of epignathus immature teratoma with fetiform features, originating from basisphenoid in a 28-week preterm male baby, who succumbed to death immediately after birth.Since epignathus is a life-threatening condition at the time of delivery, a prenatal diagnosis is essential to coordinate the treatment and appropriate management by securing the airway, either by endotracheal intubation or tracheostomy followed by complete resection of the tumor.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, KS Hegde Medical Academy, Deralakatte, Mangalore, India.

ABSTRACT
Epignathus is an extremely rare oropharyngeal teratoma that commonly arises from the palate, leading to a high mortality (80-100%) due to airway obstruction in the neonatal period. We present a case of epignathus immature teratoma with fetiform features, originating from basisphenoid in a 28-week preterm male baby, who succumbed to death immediately after birth. Since epignathus is a life-threatening condition at the time of delivery, a prenatal diagnosis is essential to coordinate the treatment and appropriate management by securing the airway, either by endotracheal intubation or tracheostomy followed by complete resection of the tumor.

No MeSH data available.


Related in: MedlinePlus

(a) Gross photograph of the dead male baby showing huge intraoral mass. (b) Microphotograph of the mass showing cartilage, adipocytes and smooth muscle tissue (H and E stain, 10×). (c) Microphotograph showing bony tissue and adipocytes (H and E stain, 10×). (d) Microphotograph showing immature neuroectodermal epithelium (H and E stain, 10×)
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Figure 1: (a) Gross photograph of the dead male baby showing huge intraoral mass. (b) Microphotograph of the mass showing cartilage, adipocytes and smooth muscle tissue (H and E stain, 10×). (c) Microphotograph showing bony tissue and adipocytes (H and E stain, 10×). (d) Microphotograph showing immature neuroectodermal epithelium (H and E stain, 10×)

Mentions: On autopsy, a pedunculated mass arising from the posterior end of palate through the basisphenoid, measuring 9.5 × 7.5 × 6 cm, was seen protruding from the oral cavity [Figure 1a]. The external surface showed bilaterally symmetrical skin buds appearing like developing fetal parts. A postmortem plain radiograph [Figure 2a, b] and computed tomography (CT) scan [Figure 2c, d] revealed a heterogeneous dense mass arising from basisphenoid, with no intracranial extension. Cut section of the mass showed variegated appearance, fat tissue and bony areas. Microscopically, the mass was composed of mature components of all the three germ cell layers comprising stratified squamous epithelium, nervous tissue, intestinal epithelium, respiratory epithelium, cartilage, bony tissue, skeletal muscle, adipose tissue and blood vessels [Figure 1b, c]. Foci of immature neuroepithelial components comprising small round cells forming rosettes and tubules were also found [Figure 1d]. So, a final diagnosis of epignathus immature teratoma (grade 1) with fetiform features was made.


Epignathus with fetiform features.

Kumar SY, Shrikrishna U, Shetty J, Sitaram A - J Lab Physicians (2011)

(a) Gross photograph of the dead male baby showing huge intraoral mass. (b) Microphotograph of the mass showing cartilage, adipocytes and smooth muscle tissue (H and E stain, 10×). (c) Microphotograph showing bony tissue and adipocytes (H and E stain, 10×). (d) Microphotograph showing immature neuroectodermal epithelium (H and E stain, 10×)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3118061&req=5

Figure 1: (a) Gross photograph of the dead male baby showing huge intraoral mass. (b) Microphotograph of the mass showing cartilage, adipocytes and smooth muscle tissue (H and E stain, 10×). (c) Microphotograph showing bony tissue and adipocytes (H and E stain, 10×). (d) Microphotograph showing immature neuroectodermal epithelium (H and E stain, 10×)
Mentions: On autopsy, a pedunculated mass arising from the posterior end of palate through the basisphenoid, measuring 9.5 × 7.5 × 6 cm, was seen protruding from the oral cavity [Figure 1a]. The external surface showed bilaterally symmetrical skin buds appearing like developing fetal parts. A postmortem plain radiograph [Figure 2a, b] and computed tomography (CT) scan [Figure 2c, d] revealed a heterogeneous dense mass arising from basisphenoid, with no intracranial extension. Cut section of the mass showed variegated appearance, fat tissue and bony areas. Microscopically, the mass was composed of mature components of all the three germ cell layers comprising stratified squamous epithelium, nervous tissue, intestinal epithelium, respiratory epithelium, cartilage, bony tissue, skeletal muscle, adipose tissue and blood vessels [Figure 1b, c]. Foci of immature neuroepithelial components comprising small round cells forming rosettes and tubules were also found [Figure 1d]. So, a final diagnosis of epignathus immature teratoma (grade 1) with fetiform features was made.

Bottom Line: Epignathus is an extremely rare oropharyngeal teratoma that commonly arises from the palate, leading to a high mortality (80-100%) due to airway obstruction in the neonatal period.We present a case of epignathus immature teratoma with fetiform features, originating from basisphenoid in a 28-week preterm male baby, who succumbed to death immediately after birth.Since epignathus is a life-threatening condition at the time of delivery, a prenatal diagnosis is essential to coordinate the treatment and appropriate management by securing the airway, either by endotracheal intubation or tracheostomy followed by complete resection of the tumor.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, KS Hegde Medical Academy, Deralakatte, Mangalore, India.

ABSTRACT
Epignathus is an extremely rare oropharyngeal teratoma that commonly arises from the palate, leading to a high mortality (80-100%) due to airway obstruction in the neonatal period. We present a case of epignathus immature teratoma with fetiform features, originating from basisphenoid in a 28-week preterm male baby, who succumbed to death immediately after birth. Since epignathus is a life-threatening condition at the time of delivery, a prenatal diagnosis is essential to coordinate the treatment and appropriate management by securing the airway, either by endotracheal intubation or tracheostomy followed by complete resection of the tumor.

No MeSH data available.


Related in: MedlinePlus