Malignant peripheral nerve sheath tumor (MPNST) is a rare, aggressive sarcomatous tumor that arises from peripheral nerve sheath and shows Schwann cell differentiation. They are commonly seen among cases with existing benign plexiform neurofibromas, prior radiation treatment, and large germline mutations involving the entire neurofibromatosis 1 (NF1) gene. MPNST can have varied presentations; hence diagnosis remains a great challenge. Here we report a rare case of MPNST in an NF1 patient who presented with Horner´s syndrome. A young male reported swelling in the neck, dyspnea on exertion, and dysphagia. Subsequently, he was diagnosed to have a malignant peripheral nerve sheath tumor arising from the mediastinum and compressing the ipsilateral cervical sympathetic plexus causing Horner′s syndrome. The patient underwent surgical resection of the mediastinal mass followed by chemotherapy. His symptoms improved significantly following treatment. This case report emphasizes the fact that high suspicion of MPNST is required when NF1 cases present with mass lesions, so that early surgical clearance with chemoradiation may offer a nearcomplete cure.
Idiopathic intracranial hypertension (IIH) causes visual involvement secondary to papilledema but rarely presents with acute gross diminution of vision. Anemia is associated with IIH. Acute hemorrhage-related anemia causing severe sudden onset bilateral visual loss due to IIH has not been previously reported. A 28year-old female attempted the first-trimester abortion by self-administration of oral drugs. She presented with bleeding per vaginum, followed by bilateral visual loss. Symptoms pertaining to intracranial hypertension were mild. Examination revealed pallor, normal hemodynamic parameters, bilaterally dilated pupils, bilateral lateral rectus palsy, and only perception of light in both eyes. Lumbar puncture demonstrated high pressures; neuroimaging was noncontributory. Blood transfusion and supportive therapy in the form of acetazolamide and pulse methylprednisolone improved her vision in the right eye to six of 24; optic nerve sheath fenestration was performed in the left eye. During follow-up, her vision improved to six of 24 (right) and two of 60 (left), respectively. The IIH can present with severe acute onset bilateral visual loss even if features of raised intracranial pressure are minimal or absent. Immediate correction of anemia and supportive measures may significantly improve visual outcomes in fulminant IIH without the necessity of surgery.
Subretinal abscesses due to endogenous staphylococcal blood stream infection is a rare occurrence. A young adult male presented with subretinal abscesses, necrotising pneumonia, pleural empyema, skin and soft tissue infection, muscle abscesses and deep vein thrombosis. Aspirate from one of the abscesses and blood culture revealed meticillin-susceptible Staphylococcus aureus. We present here a case of probable Panton–Valentine leucocidin (PVL) syndrome. PVL is a cytotoxin produced by S. aureus. Infection with PVL-positive S. aureus produces a clinical disease that is characterised by necrotising pneumonia and disseminated infection that often carries a high mortality. Our patient showed prompt clinical response to cloxacillin that was given for a total duration of 6 weeks. At the end of 6 weeks vision also recovered. The successful outcome in our patient was likely due to early and appropriate antibiotic therapy.
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