Pituitary duplication with nasopharyngeal teratoma and cleft palate

Case contributed by Dr Bruno Lorensini

Presentation

Newborn with expanding lesion in the oral cavity, with hair on the surface. Respiratory distress, requiring intubation and mechanical ventilation.

Patient Data

Age: 2 days
Gender: Female

Axial and coronal T1 C+ shows duplicated pituitary stalks extending towards two small paramidline pituitary glands. 

Sagittal T1 shows expansive lesion involving the oral cavity, protruding through the mouth, with apparent pedicle extending the craniofaríngeo channel region, suggesting palatal teratoma.

Sagittal also shows thickened of soft tissue along the floor of the third ventricle from the mesencephalon to the optic chiasm (hypothalamic mass), which may be related to tuber cinereum hamartoma.

CT show persistent of craniopharyngeal canal and cleft palate.

Axial and coronal T1 C+ shows duplicated pituitary stalks (orange arrow) extending towards two small paramidline pituitary glands (red arrow). 

Case Discussion

The lesion of the oral cavity was removed and pathology showed a benign cystic teratoma.

This is a case of duplication of the pituitary gland with oropharyngeal teratoma and cleft palate associated, rare disorder being reported about 40 cases until 2012. Most of the reported cases had other craniofacial abnormalities and the term "syndrome of pituitary duplication - plus" it has been used.

Etiology not fully understood. It is proposed that the development abnormality occurs during blastogenesis (division of the rostral portion of the notochord and prechordal plate). The defects that occur at this stage are often severe, with multiorgan involvement in the midline.

It has not been associated with any specific teratogenic agent.

Craniofacial abnormalities associated with duplication of the pituitary gland:

  • hypertelorism
  • cleft palate
  • persistent craniopharyngeal canal
  • hypothalamic mass
  • midline clival defects
  • oropharyngeal masses

Vertebral malformations, diaphragmatic hernia, cardiac malformations and urinary anomalies were also reported.

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Case information

rID: 47645
Case created: 25th Aug 2016
Last edited: 27th Nov 2016
Inclusion in quiz mode: Included

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