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BACKGROUND: The tumor invasion of the skull base structures is very frequent in spheno-orbital meningiomas. The aim of the present study is to evaluate the invasion rate of skull base structures and the best surgical approach and management. METHODS: The surgical series of 80 spheno-orbital meningiomas was reviewed. The tumors were classified according to the intraorbital location with respect to the optic nerve axes into three types: I-lateral: II-medial; III-diffuse. The invasion of the orbital apex, optic canal, superior orbital fissure, anterior clinoid, ethmoid-sphenoid sinuses, and infratemporal fossa was evaluated. The rate and extension of involvement of these structures was correlated with the intraorbital location and the surgical approach. The preoperative ophtalmological symptoms and signs and their outcome were also evaluated. RESULTS: Proptosis was found in 79 patients (97%), variable decrease of the visual function in 47 patients (59%), and deficits of the eye movements in 28(35%). The invasion of the optic canal (74%), superior orbital fissure (65%), anterior clinoid (60%), and orbital apex (59%) was more frequently found, whereas the tumor extension into the ethmoid-sphenoid sinuses (4%) and infratemporal fossa (4%) was rare. Types II and III meningiomas showed significantly higher involvement of the skull base structures than type I ones, which only had 15% invasion of the optic canal. Remission or significant improvement of the visual function occurred postoperatively in 24 among 47 cases (51%), with a higher rate for type I meningiomas vs. other types (p = 0.021, p = 0.019) and worsening in 7 (15%). CONCLUSIONS: Spheno-orbital meningiomas growing in the lateral orbital compartment show no involvement of the skull base structures excepting the optic canal as compared to those growing medially or diffusely. The surgical resection of tumor invading the skull base structures should be more extensive as possible, but the risk of optic and oculomotor deficits must be avoided.
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BACKGROUND: The progesterone receptor (PR) is variably expressed in most meningiomas and was found to have prognostic significance. However, the correlation with patient age, tumor location, time to recurrence, and pattern of regrowth has scarcely been discussed. METHODS: A surgical series of 300 patients with meningiomas is reviewed. The PR expression was classified as: 0. absent; 1. low (<15%); 2. moderately low (16-50%); 3. moderately high (51-79%); 4. high (≥80%). The PR values were correlated with the patient age and sex, meningioma location, WHO grade, Ki-67 MIB1, recurrence rate, pattern of recurrence (local-peripheral versus multicentric diffuse), and time to recurrence. RESULTS: The PR expression has shown lower rate of high expression in the elderly group (p = 0.032) and no sex difference (including premenopausal versus postmenopausal women), higher expression in medial skull base and spinal versus other locations (p = 0.0036), inverse correlation with WHO grade and Ki67-MIB1 (p < 0.0001). Meningiomas which recurred showed at initial surgery higher rates of low or moderately low PR expression than the non-recurrent ones (p = 0.0004), whereas the pattern of regrowth was not significant. Higher rates of PR values ≥80% were found in cases with time to recurrence >5 years (p = 0.036). CONCLUSION: The higher PR expression in medial skull base meningiomas, the significant correlation with the time to recurrence, the lack of difference of PR expression between premenopausal and postmenopausal women and between local-peripheral versus multicentric-diffuse recurrences are the most relevant unreported findings of this study. The rate of PR expression must be included in the routine pathological diagnosis of meningiomas because of its prognostic significance.
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BACKGROUND: Familial forms of cavernous malformations (CMs) often occur as multiple lesions. Nevertheless, the presence of a single CM does not exclude the familiarity. The aim of this study is to establish which patients who undergo surgery for a single cerebral cavernous malformation (CCM), with no family history at initial diagnosis, should be investigated for familiarity through genetic testing and counseling. METHODS: Eight families with 2 or more members affected by CCM have been studied. A control group of sporadic cases operated on, with no family history and followed up 10 years or more, was also included. Analyzed factors were patient age and sex, location, number and size of the lesions, associated developmental venous anomaly, presence of epileptic seizures, symptomatic hemorrhage, focal neurological deficits, and documented growth of the malformation and Ki67 MIB1 proliferation index. RESULTS: The familial group of CCMs showed higher incidence of pediatric patients (P = 0.01), more frequent occurrence of multiple lesions (P = 0.0004), higher rate of large CCMs, and symptomatic hemorrhage; besides, all 3 cases with documented growth belonged to the familial group (14%). The expression of Ki67 MIB1 was positive in 79% of the familial cases versus 0% in the sporadic ones (P < 0.00001). CONCLUSIONS: Patients with CCM and no known family history at the time of the initial diagnosis who present specific features should be studied by genetic screening. The Ki67 MIB1 is a useful biomarker in favor of familial occurrence and may be studied in all patients with CMs to define the indication to the genetic tests.
Asunto(s)
Hemangioma Cavernoso del Sistema Nervioso Central/genética , Hemangioma Cavernoso del Sistema Nervioso Central/cirugía , Antígeno Ki-67/genética , Adolescente , Adulto , Anciano , Biomarcadores , Niño , Preescolar , Femenino , Pruebas Genéticas , Humanos , Lactante , Masculino , Persona de Mediana Edad , Adulto JovenRESUMEN
BACKGROUND: Lemierre's syndrome is a rare but potentially life-threatening clinical condition characterized by bacteremia and thrombophlebitis of the internal jugular vein, usually secondary to oropharyngeal infection and often caused by Fusobacterium necrophorum; rarely, it occurs after surgical procedures. The most common clinical presentation includes acute pharyngitis, high fever, and neck pain. The diagnosis is based on blood culture and cranial and cervical spine computed tomography (CT)/magnetic resonance imaging (MRI) with contrast. Antibiotic therapy for 3-6 weeks is the mainstay of treatment, while the use of anticoagulant drugs is controversial. OBSERVATIONS: The authors describe a case of Lemierre's syndrome that occurred after transoral surgery. The patient underwent a combined surgical approach from above (transoral) and below (anterolateral transcervical) to the upper cervical spine for the resection of a large anterior osteophyte causing dysphagia, globus sensation, and dysphonia. Three weeks after the surgical procedure, she developed fever and severe neck pain. LESSONS: The aim of this paper is to consider Lemierre's syndrome as a possible complication after the transoral approach, underlining the importance of its early diagnosis and with a suggestion to perform cranial and cervical spine CT or MRI venous angiography in patients who undergo surgery with a transoral approach and exhibit local or systemic signs of infection such as neck pain, persistent fever, and positive blood culture results.
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BACKGROUND: Anterior cervical osteophytes (ACOs) may rarely cause dysphagia, dysphonia, and dyspnea. Symptomatic ACOs are most commonly located between C3 and C7, whereas those at higher cervical (C1-C2) levels are rarer. We report a case series of 4 patients and discuss the best surgical approach according to the ostheophyte location and size, mainly for those located at C1-C2, and the related surgical problems. MATERIALS AND METHODS: Four patients (two males and two females) aged from 57 to 72 years were operated on for ACOs, causing variable dysphagia (and dyspnea with respiratory arrest in one). Three patients with osteophytes between C3 and C5 were approached through antero-lateral cervical approach, and one with a large osteophyte between C1 and C3-C4 level underwent a two-stage transcervical and transoral approach. All had significant postoperative improvement of dysphagia. RESULTS: The patient operated on though the transoral approach experienced postoperative flogosis of the prevertebral tissues and occipital muscles and thrombosis of the right jugular vein and transverse-sigmoid sinuses (Lemierre syndrome). CONCLUSION: The transoral approach is the best surgical route to resect C1 and C2 ACOs, whereas the endoscopic endonasal approach is not indicated. The anterior transcervical approach is easier to resect osteophytes at C3, as well as those located below C3. A combined transoral and anterior cervical approach may be necessary for multilevel osteophytes.