Lateral Femoral Cutaneous Nerve Angiomyoma.

Angiomyomas are benign tumors derived from smooth muscle cells of vessels. They are principally a pathology of the peripheral nervous system where they involve cutaneous nerves, causing pain and paresthesia. We present a case of a lateral femoral cutaneous nerve angiomyoma and its surgical treatment. A 24-year-old female presented to clinic with right thigh pain in the distribution of the lateral femoral cutaneous nerve, which had been ongoing and progressive for seven months. There was associated numbness and tingling. A lesion was noted in the anterior subcutaneous tissues of the thigh on contrasted CT and MRI. The patient was taken to the operating room where a pearly white lesion was found within the subcutaneous tissue. No effect was seen with stimulation of the lesion. The lesion was removed en bloc, and pathological analysis revealed an angiomyoma. Post-operatively, the patient reported complete resolution of all symptoms, namely, pain and paresthesia. No similar reports were identified within the literature. Together, angiomyomas have been described within the lower extremities to cause pain and paresthesia. This is the first reported case of an angiomyoma involvement within the lateral femoral cutaneous nerve. Complete surgical resection, in this case, was curative and diagnostic.

Long-term outcome in children with gliomas of the anterior visual pathway.

We performed a retrospective assessment of the long-term visual, neurologic, and systemic outcomes of 47 patients with anterior visual pathway gliomas seen at the Johns Hopkins Hospital. All of the patients had follow-up of at least 10 years or died during the follow-up period. Two patients died before 10 years of follow-up were achieved. The remaining 45 patients (including three patients who subsequently died) had follow-up of 10-28 years (mean, 15.3 years; median, 15 years). Sixteen of the patients in this study, most of whom had neurofibromatosis type 1 (NF1), received no treatment. None of these patients died or developed neurologic morbidity as a result of their tumor. Thirty-one of the patients, most of whom did not have evidence of NF1, received treatment. Many of these patients subsequently developed neurologic, endocrine, or visual morbidity. However, although patients with anterior visual pathway gliomas who were not treated fared better visually, neurologically, and systemically than patients who were treated, patients who required treatment for progression generally had a good overall prognosis, particularly patients with tumors that did not involve the hypothalamus. Most of these patients survived and maintained useful vision in at least one eye. We believe that patients with anterior visual pathway gliomas, particularly those with NF1, should not be treated unless there is clear clinical or neuroimaging evidence of progression.