PMC10191252_01
(A) Sagittal section (resp. coronal section from perspective of neonate) of T2 weighted MRI depicting the presence of a large epignathus attached to neonate's right oral cavity (white arrows) (B) Coronal section (resp. sagittal section from perspective of neonate) of T2 weighted MRI demonstrating a free upper airway of the neonate (white arrowheads). Epignathus labeled with a white arrow. (C) Coronal section of T1 weighted MRI demonstrating the attachment of the epignathus (white arrows) to the right lateral margin of the tongue (white arrowhead).
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F1
For further specification, a subsequent fetal MRI scan led to the diagnosis of a large epignathus originating from the right palate and masticator space (Figure 1A). A postnatal MRI demonstrated the attachment of the fetiforme mass to the soft palate with extension to the masseter muscle and the parotid gland on the right side (Figure 1C).
(A) Pedunculated and with skin covered mass with multiple round and nodular extensions. (B) Attachment to the lateral margin of the tongue and palatal region on the right sinde.
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F2
Upon physical examination, a 12 cm x 8 cm x 5 cm stalked fetiforme mass protruding out of the mouth was observed (Figure 2A). At surgery, the epignathus was found to be attached to the floor of the mouth, the lateral margin of the tongue and the soft palate via a structure that resembled macroscopically an umbilical cord (Figure 2B).
(A,B) excision of the fetiform mass in external view. White asterisk marks the peduncle and the resection margin, respectively. White arrowhead shows the rudimentary leg, black arrowhead the single toe. (C) Frontal section, unfolded. Abundant adipose tissue, some of which is well perfused, is visible. Black arrows mark rudimentary cartilaginous vertebral anlagen. White double asterisk shows the cerebral vesicle. Other formed organs are not macroscopically delineated.
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F3
In the histopathological preparation, the specimen presented as a pedunculated tumor that was completely covered by skin, showing multiple round nodular distensions (Figure 3).
Microscopy with (A) regularly structured colonic wall with mucosa, submucosa, and bilayered muscularis propria. (HE). (B) Primitive but organized structure of a bronchus with bronchial mucosa, bronchial glands, and a hyaline cartilaginous brace (HE). (C) Section through primitive leg showing centrally located differentiated long tubular bone. This shows a regular anatomical architecture with organized enchondral ossification zone (inset) (HE). (D) Lymph node with regular architecture, capsule and feeding vessels (HE). (E) Section through primitive cerebral vesicle with thin lining by neuronal tissue. Additional neuronal tissue is found in the fibrosed sheath (HE staining, left). In the right half of the image, the central neuronal tissue is marked by GFAP immunohistochemistry staining.
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F4
On section, there was a large cavity that histologically corresponded to a cerebral vesicle (Figure 4E). In addition, there were central cartilage fragments, possibly corresponding to primitive vertebrae, as well as only histologically recognizable tissue differentiations in the form of thyroid tissue, colon wall portions, and rudimentary bronchial structures (Figure 4B).