A Massive Congenital Intracranial-orbital Immature Teratoma Tracing Trigeminal Nerve Pathway in One Monozygotic Twin: Report of a Case and Review of the Literature

Background: Congenital intracranial teratomas are extremely rare tumors, which may extend through the extracranial structures, namely orbit, and pose difficulties in determining of original site and also in the surgical procedures.

Methods and materials: In this paper, we presented two newborn male monozygotic twins in which only one twin had a proptosis of the right eye. The magnetic resonance imag¬ing (MRI) revealed a huge tumor along a trigeminal nerve that involved orbit, wall of cavernous sinus, infratemporal fossa, and brain stem. Because of the large involvement of this tumor, the patient underwent a subtotal resection of the middle cranial fossa and debulking of intra orbital portion. The histopathological findings demonstrated a congenital immature teratoma.

Result: To date, the patient is treated with adjuvant chemotherapy, and the other twin remains healthy, with normal brain CT scan.

Conclusion: To our knowledge, given the low incidence of congenital teratomas, there is no certain treatment for these tumors, and perhaps this report is the first case of congenital immature teratoma presented in intracranial or orbital structures in only one monozygotic twin.


Guive Sharifi, Mehrdad Hosseinzadeh Bakhtevari, Farahnaz Bidari Zerehpoosh, Masood Saberi, Mona Rezaei and Omidvar Rezaei

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