An unusual giant schwannoma over face diagnosed on FNAC: a case report

Schwannoma is a slow growing benign peripheral nerve sheath tumor arising from schwann cells. It is also known as neurilemmoma. Although it is common in the head and neck region, it rarely exceeds 10 cm in size. Usually Schwannomas are notlarger than 5 to 6 cm in diameter.Large tumors are uncommon and are found in posterior mediastinum or retroperitoneum. We are presenting an unusual giant cell schwannoma over face (cheek) measuring 12×10cmsin size which caused facial deformity to the patient. The tumor was diagnosed on FNAC where cell block was prepared and IHC was done which showed S-100 positivity. This size schwannomaover cheek site has not been reported in the literature as far our knowledge. Therefore this case is unique for its size over face.

Schwannoma lumbosacro gigante / Schwannome lombo-sacré géant / Giant lumbosacral schwannoma

Fundamento: el schwannoma espinal es un tumor de las vainas nerviosas y se comporta intradural y extramedular y provienen de las células embrionarias de la cresta neural de los nervios periféricos. Representan entre el 25 y el 30 por ciento de los tumores y afectan a adultos entre 40 y 60, son raros en niños y no hay predilección por el sexo. Objetivo: presentar un caso de schwannoma lumbosacro gigante que se diagnosticó en el Hospital Universitario Manuel Ascunce Domenech de Camagüey y recibió tratamiento quirúrgico en el Instituto Nacional de Neurología y Neurocirugía. Reporte de caso: paciente MB, de 38 años con antecedentes de salud con historia de dolor lumbosacro de dos años de evolución, intensificado en los últimos 8 meses que se irradiaba a miembros inferiores con parestesias y pérdida de la fuerza muscular. Además presentaba dificultad para la deambulación, constipación, incontinencia urinaria y eyaculación retardada. La tomografía axial computarizada informó un extenso proceso tumoral intrarraquídeo con crecimiento longitudinal anterior, posterior y lateral desde la cuarta vértebra lumbar hasta la tercera sacra (L4-S3), que producía lisis de la porción posterior de los cuerpos vertebrales, los pedículos y el borde anterior de S3 y la imagen de resonancia magnética reveló gran área ligeramente hipointensa en T1 e hiperintensa en T2, que ocupaba el canal vertebral desde L3 hasta la cuarta vertebra sacra (S4). La biopsia informó un tumor constituido por células de aspecto fusocelular dispuesto en fascículos compactos, otras áreas más laxas compuestas por células de citoplasma claro, abundantes vasos sanguíneos, compatible con un schwannoma, tumor benigno de las vainas nerviosas. Se remitió para tratamiento quirúrgico en el Instituto Nacional de Neurología y Neurocirugía con evolución favorable luego de la resección amplia e instrumentación. Conclusiones: el schwannoma lumbosacro es un reto para los cirujanos y la afectación sacra es rara. El diagnóstico anatomopatológico es fundamental y la cirugía es el tratamiento de elección, donde la resección completa del tumor es muy difícil en ocasiones debido a la posibilidad de lesionar estructuras nerviosas importantes. Se recomienda el seguimiento radiológico por el riesgo de recidivas(AU)

Background: The spinal schwannoma is a tumor of the nerve sheaths that presents in intradural and extramedular areas. It comes from the embryonic cells of the neural crest of the peripheral nerves. They represent 25-30 percent of tumors and it affects adults aging 40 and 60. It is rare in children and there is no sex difference. Objective: Present a case of giant lumbosacral schwannoma that was diagnosed at Manuel Ascunce Domenech University Hospital in Camagüey. This patient received surgical treatment at the National Institute of Neurology and Neurosurgery. Case report: 38-year-old white male patient with a history of lumbosacral pain of two years of evolution came to consultation. His pain intensified in the last 8 months, radiating to lower limbs with paresthesias and loss of muscle strength. In addition, he had constipation, urinary incontinence, difficulty at walking, and delayed ejaculation. Computed tomography revealed an extensive intrarectal tumor process with anterior, posterior and lateral longitudinal growth from the fourth lumbar vertebra to the third sacrum (L4-S3), which produced lysis of the posterior portion of the vertebral bodies, pedicles, and S3 anterior border. Magnetic resonance imaging revealed a large area slightly hypointense in T1 and hyperintense in T2, which occupied the vertebral canal from L3 to the fourth sacral vertebra (S4). The biopsy reported a tumor-like fusocellular cells arranged in compact fascicles, other more lax areas of clear cytoplasm cells, abundant blood vessels, compatible with a schwannoma, benign tumor of the nerve sheaths. The patient was referred for surgical treatment at the National Institute of Neurology and Neurosurgery with favorable evolution after extensive resection and instrumentation. Conclusions: Lumbosacral schwannoma is a challenge for surgeons. Sacral involvement is rare. Histopathologic diagnosis is fundamental and surgery is the treatment of choice, in which complete resection of the tumor is very difficult at times due to the possibility of injuring important nerve structures. Radiological follow-up is recommended for the risk of relapses(AU)

Fondement: Le schwannome rachidien est une tumeur de comportement intradural et extramédullaire des gaines nerveuses, provenant des cellules embryonnaires de la crête neurale des nerfs périphériques. Il représente 25 à 30 pourcent des tumeurs, touchant généralement les adultes âgés de 40 à 60 ans. Chez l'enfant, il est rare. Il touche les deux sexes en égal. Objectif: Présenter un cas de schwannome lombo-sacré géant diagnostiqué à l'hôpital universitaire "Manuel Ascunce Domenech", à Camagüey, et traité chirurgicalement à l'Institut national de neurologie et neurochirurgie, à La Havane. Rapport de cas: Patient, blanc, âgé de 38 ans, ayant une histoire de douleur lombo-sacrée de deux ans d'évolution, intensifiée dans ces huit derniers mois et irradiée aux membres inférieurs avec paresthésie et perte de la force musculaire. Il avait aussi des difficultés pour la marche, et souffrait de constipation, incontinence urinaire et éjaculation retardée. La tomographie axiale calculée par ordinateur a montré un large processus tumoral intrarachidien, avec une croissance longitudinale antérieure, postérieure et latérale depuis la quatrième vertèbre lombaire jusqu'à la troisième sacrée (L4-S3), produisant la lyse de la portion postérieure des corps vertébraux, des pédicules et du bord antérieur de S3, et l'imagerie par résonance magnétique a révélé une vaste zone légèrement hypointense en T1 et hyperintense en T2, occupant le canal vertébral depuis L3 jusqu'à la quatrième vertèbre sacrée (S4). La biopsie a indiqué une tumeur constituée de cellules d'aspect fusocellulaire disposées en fascicules compacts, d'autres zones plus détendues composées de cellules à cytoplasme clair, beaucoup de vaisseaux sanguins, évoquant un schwannome, c.-à-d., une tumeur bénigne des gaines nerveuses. Le patient a été renvoyé vers l'Institut national de neurologie et neurochirurgie pour un traitement chirurgical. Après une grande résection et appareillage, son évolution a été favorable. Conclusions: Le schwannome lombo-sacré constitue un défi pour les chirurgiens parce que cette affection est très rare. Son diagnostic anatomopathologique est essentiel. L'intervention chirurgicale est le traitement de choix, mais la résection complète de la tumeur est parfois très difficile due à la proximité de structures nerveuses importantes. Il est conseillé un suivi radiologique de risque de récidive(AU)

Single Stage Posterior Approach for Total Resection of Presacral Giant Schwannoma: A Technical Case Report

The neurogenic tumor of frequent occurrence in the presacral area is a schwannoma. Giant presacral schwannoma has a risk for anterior surgical approach because of its massive size and proximity to abundant vascularity of presacral region. We report a single stage posterior approach for total resection of a giant presacral schwannoma. A 40-year-old female patient experienced left buttock pain and tingling sensation at left S1 dermatome. Magnetic resonance imaging showed that the presacral huge mass at S1–3 level with osseous extension and structural remodeling in left sacral ala. The presacral mass was ranging in maximum diameter from 8.0 to 8.6 cm. S2 foramen laminectomy was performed to expose the mass. The tumor capsule and the root were carefully dissected away. The tumor was removed while preserving the capsule by dissecting the plane between the inner wall of the capsule and the tumor. The single stage posterior approach for presacral giant schwannoma is feasible, and it can be a good surgical alternative to prevent pelvic organ or vascular damage and anterior approach related dystocia and infertility.

Cystic Giant Sacral Schwannoma Mimicking Aneurysmal Bone Cyst : A Case Report and Review of Literatures

To present a rare case of a cystic giant schwannoma of the sacrum mimicking aneurysmal bone cyst (ABC). A 54-year-old man visited our institute complaining left leg weakness and sensory change for several years. Magnetic resonance imaging revealed a large multilocular cystic mass with canal invasion and bone erosion confined to left S1 body. The lesion showed multiple septal enhancement without definite solid component. Initially the tumor was considered as ABC. The patient underwent grossly-total tumor resection with lumbosacral reconstruction via posterior approach. The tumor was proved to be a cystic schwannoma. The postoperative course was uneventful and the patient was relieved from preoperative symptoms. We present a rare case of pure cystic giant schwannoma confined to sacrum mimicking ABC. The surgical treatment is challenging due to the complex anatomy of the sacrum. Schwannoma should be considered in the differential diagnosis of osteolytic sacral cysts.

Giant Cauda Equina Schwannoma with Dystrophic Calcifications : Case Report and Review of the Literature

Giant spinal schwannoma of the cauda equine involving many nerve roots is rare, and ossification is usually not observed in the schwannoma. A 21-year-old man presented with a 12-month history of urinary dysfunction and numbness below the buttocks. Plain radiography showed scalloping of the posterior surface of the vertebral bodies from L4 to the sacrum, and magnetic resonance imaging and computed tomography revealed a giant cauda equina tumor with dystrophic calcification. The tumor was completely removed, with intraoperative neurophysiologic monitoring. Histopathologic examination showed that the tumor was a schwannoma. The patient's postoperative course was uneventful, with urinary function and numbness gradually improving. Although a giant schwannoma accompanied by dystrophic calcification is extremely rare, such a tumor can be removed safely and completely by meticulous dissection and careful neuromonitoring of the cauda equina spinal nerves involved in the tumor.

Giant Ventral Midline Schwannoma of Cervical Spine : Agonies and Nuances

Pure ventral midline giant schwannoma is an extremely rare entity. Spinal intradural extramedullary schwannomas commonly occur posterolateral or anterolateral to the spinal cord. A case of a pure midline ventrally situated giant pan cervical extramedullary schwannoma in an 18-year-old male patient with compressive myelopathy and sphincter involvement is presented. Spinal MR imaging showed a midline ventrally situated extramedullary tumor with severe spinal cord compression extending from clivus to C7 vertebra. It was resected through a posterolateral approach. Histology was consistent with a schwannoma. Post operative MR imaging showed no evidence of the tumor. The radiological features, pathogenesis and surgical strategies in management of these difficult tumors are discussed and the relevant literature is briefly reviewed.