Background:
Hemorrhagic lesions of the septum pellucidum are rare and usually occur in neonates. They can be due to a number of etiologies. Here, we report a rare case of adolescent nontraumatic septum pellucidum hemorrhage with a review of literature.
Case Description:
A 16-year-old girl presented with a 1-month history of gradual visual deterioration in the left eye, intermittent headache, and vomiting. Brain imaging showed hematoma located between the leaflets of the septum pellucidum with obstructive hydrocephalus. Transcallosal resection of interventricular mass was done. The patient was discharged with improved neurological symptoms; however, the left eye vision did not recover. Imaging demonstrated a unique anatomical variant in deep vascular structures.
Conclusion:
Cavum septum pellucidum hemorrhage is rare in adults. Many theories were constructed to explain its etiology. Bleeding due a vascular anatomical variant was not previously encountered. Understanding the embryological origin and anatomical details are important for proper clinical assessment and management of these patients.
Background:
Malignant peripheral nerve sheath tumors (MPNSTs) are one of the rarest soft-tissue sarcomas with a prevalence of 0.001% in the general population. It is closely associated with a unique neurocutaneous stigmata under the spectrum of the dermatological manifestations of neurofibromatosis type 1 (NF1). Almost 81% of MPNST arises from a precursor neuroma, and multifocality of these lesions is extremely rare, making up to 0.001% of cases. Moreover, spinal cases are extremely uncommon with only four cases reported internationally. Here, we present the fifth and sixth spinal MPNST cases with a brief review of literature.
Case Description:
We describe two unusual cases of multifocal MPNST in relation to NF1 occurring in the spinal cord. Both patients presented with local pain and myelopathic symptoms. The two patients underwent wide surgical resection, followed by neoadjuvant radiotherapy and reported immediate postoperative improvement of the presented complaint; however, one patient suffered from rapid recurrence and metastasis.
Conclusion:
Due to the scarcity of spinal cases related to MPNST, no clear guidelines regarding the management of these cases are set in the literature. Histopathological diagnosis remains as the most pivotal diagnostic tool as they can mimic other peripheral nerve sheath lesions, such as neuromas and schwannomas, in imaging. Cases that were managed by early surgical intervention in addition to neoadjuvant radiotherapy reported the best outcome. However, cases of MPNST in concomitance with NF1 were found to be resistant to both chemo and radiotherapy and have high recurrence rate.
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