ABSTRACT
Pediatric and NF2-associated meningiomas are uncommon and poorly characterized in comparison to sporadic adult cases. In order to elucidate their molecular features, we analyzed MIB-1, progesterone receptor (PR), NF2, merlin, DAL-1, DAL-1 protein, and chromosomal arms 1p and 14q in 53 meningiomas from 40 pediatric/NF2 patients using immunohistochemistry and dual-color fluorescence in situ hybridization (FISH). Fourteen pediatric (42%) patients, including 5 previously undiagnosed patients, had NF2. The remaining 19 (58%) did not qualify. All 7 of the adult patients had NF2. Meningioma grading revealed 21 benign (40%), 26 atypical (49%), and 6 anaplastic (11%) examples. Other aggressive findings included high mitotic index (32%), high MIB-1 LI (37%), aggressive variant histology (e.g. papillary, clear cell) (25%), brain invasion (17%), recurrence (39%), and patient death (17%). FISH analysis demonstrated deletions of NF2 in 82%, DAL-1 in 82%, 1p in 60%, and 14q in 66%. NF2-associated meningiomas did not differ from sporadic pediatric tumors except for a higher frequency of merlin loss in the former (p = 0.020) and a higher frequency of brain invasion in the latter (p = 0.007). Thus, although pediatric and NF2-associated meningiomas share the common molecular alterations of their adult, sporadic counterparts, a higher fraction are genotypically and phenotypically aggressive. Given the high frequency of undiagnosed NF2 in the pediatric cases, a careful search for other features of this disease is warranted in any child presenting with a meningioma.
Subject(s)
Neurofibromatosis 2/genetics , Neurofibromatosis 2/pathology , Tumor Suppressor Proteins , Adolescent , Adult , Astrocytoma/diagnosis , Astrocytoma/genetics , Astrocytoma/pathology , Child , Child, Preschool , Cohort Studies , Contraindications , Female , Follow-Up Studies , Genotype , Humans , Male , Membrane Proteins/biosynthesis , Microfilament Proteins , Middle Aged , Neurofibromatosis 2/diagnosis , PhenotypeABSTRACT
A case of malignant (ciliary block) glaucoma apparently induced by a large posterior chamber intraocular lens (PC-IOL) is presented. The involved eye was small, with an axial length of 21.7 mm and a preoperative refractive error of +8.25 D. An uncomplicated extracapsular cataract extraction with implantation of a PC-IOL with a 7 mm optic was performed. Within 1 week postoperatively, malignant glaucoma developed, for which surgical intervention was required. We recommend avoiding implantation of PC-IOLs with large optics in certain small eyes, since these implants may be more likely than lenses with smaller optics to induce malignant glaucoma in such eyes.
Subject(s)
Glaucoma/etiology , Lenses, Intraocular/adverse effects , Aged , Anterior Chamber/pathology , Ciliary Body/pathology , Female , Glaucoma/surgery , Humans , Iatrogenic Disease , Iris/surgery , Visual Acuity , VitrectomyABSTRACT
In an effort to achieve the early postoperative safety and stability provided by trabeculectomy plus the late lower intraocular pressure (IOP) advantages of full-thickness glaucoma filtration surgery, 43 eyes of 38 patients with chronic open-angle glaucoma had laser suture lysis after primary trabeculectomy. The technique of laser suture lysis afforded serial release of resistance to aqueous outflow through the newly performed trabeculectomy, allowing initial tight closure of the trabeculectomy to avoid the dangers of hypotony. Serial gradual loosening of the trabeculectomy closure decreased resistance to outflow through the trabeculectomy and thereby lowered the IOP to desired levels in a controlled, titrated manner. Complications decreased with experience. For 25 eyes with at least 6 months follow-up (average, 54 weeks), the average IOP decreased from 25.0 mmHg preoperatively to 12.2 mmHg postoperatively.