Hybrid Neurofibroma/Schwannoma of the Oral Cavity: A Rare Case Report and Literature Review.

Hybrid peripheral nerve sheath tumors show combined features of more than one type of conventional benign peripheral nerve sheath tumors. There are few cases reported of hybrid peripheral nerve sheath tumors in the head and neck region. A 68-year-old female patient was referred for evaluation of an oral swelling lasting five years. Intraoral examination revealed a small mobile nodule located in the lower vestibule. The patient underwent excisional biopsy and microscopic evaluation showed typical features of neurofibroma enclosing areas with palisading nuclei compatible with Antoni A pattern, which are seen in schwannomas. These regions showed strong and diffuse immunoreactivity for S100 protein and moderate positivity in the neurofibroma area. CD34 was positive in the neurofibroma area and entrapped axons were positive for neurofilament. The final diagnosis was oral hybrid neurofibroma-schwannoma tumor. Hybrid peripheral nerve sheath tumors, although extremely rare, may arise within the oral cavity. To the best of our knowledge, this is the first neurofibroma-schwannoma tumor reported in the oral cavity. Recognizing hybrid peripheral nerve sheath tumors as a distinct clinicopathological entity is important because they may also be associated with syndromic disorders.

Extracranial non-vestibular head and neck schwannomas: a case series with the review of literature.

INTRODUCTION: Schwannomas are tumous that arise from Schwann cells. Schwannoma is one of the differential diagnosis for lateral neck swelling. OBJECTIVE: In this study, we aim to describe the incidence, presenting clinical features and management of extracranial, non-vestibular schwannomas of head and neck region, along with the review of the literature. METHODS: Patients treated at our tertiary care hospital for head and neck schwannomas for the past 15 years were included in the study. A review of literature on the extracranial head and neck schwannoma was also done. RESULTS: Twenty-five cases were assessed in this study. Nineteen cases presented as a neck swelling during the initial evaluation. Vagus nerve was the most common nerve of origin, followed by the cervical sympathetic plexus. A rare presentation arising from brachial plexus C5 nerve root was also encountered. A few rare cases of schwannomas arose from the nasal cavity, paranasal sinuses, and oral cavity. Surgical excision was done in all the cases with histopathology suggestive of schwannoma. The nerve of origin of the tumor was identified in nineteen patients. Among them, 11 (58%) were from the vagus nerve, 7 (37%) from the cervical sympathetic chain, and 1 (4%) from the brachial plexus C5 nerve root. CONCLUSION: A long-standing unilateral neck mass is the most common presenting complaint in head and neck schwannoma. The diagnosis is mainly based on clinical features and investigations such as imaging. The mainstay of treatment is complete surgical excision. The diagnosis is confirmed on the histopathological study after excision of the lesion. Due to the proximity of the tumor with the involved nerve, palsy may occur. Hence, an accurate preoperative diagnosis of schwannoma is essential.

Tumor benigno de la vaina neural en rodilla. Reporte de caso y revisión bibliográfica / Knee neural sheath benign tumor. Case report and bibliographic revision

El schwannoma es el tumor benigno de nervio periférico más frecuente. Su presencia en los nervios de miembros inferiores es excepcional, donde representan el 1% de todos los schwannomas. Presentamos el caso de una mujer de treinta y un años que consulta por dolor en la cara anterior de la rodilla derecha, donde se palpa una masa blanda, dolorosa, de 1 cm aproximadamente y dolor en interlínea externa con signo de McMurray positivo. La RM evidenció una estructura ovoidea de señal quística, superficial al retináculo medial en su tercio proximal, de 10 × 8 × 8 mm y lesión del menisco externo en su tercio medio. Se realizó tratamiento artroscópico de la lesión meniscal externa y por vía abierta la exéresis marginal quirúrgica del tumor de partes blandas, con diagnóstico histopatológico de schwannoma. Los schwannomas de nervio periférico, aunque sean una entidad poco frecuente, deben considerarse en el diagnóstico diferencial de las masas dolorosas de la rodilla. Su tratamiento es la exéresis quirúrgica

Schwannoma is the most common benign peripheral nerve tumor, its presence being exceptional in the nerves of the lower limbs, where it represents 1% of all schwannomas. We present the case of a thirty-one-year-old woman who consulted for anterior knee pain, where a soft, painful mass of approximately 1cm and pain on the lateral joint line was assessed. McMurray's sign was positive. MRI showed an ovoid structure with a cystic signal, superficial and proximal to the medial retinaculum, measuring 10 × 8 × 8 mm and a tear in the body and posterior horn of the lateral meniscus. Arthroscopic treatment for the lateral meniscus tear and open surgical marginal excision of the soft tissue tumor were performed, with pathological diagnosis of schwannoma. Peripheral nerve schwannomas, although a rare entity, should be considered in the differential diagnosis of painful knee masses, their treatment being surgical excision

Schwannoma lingual: presentación de un caso y breve revisión de la literatura / Lingual schwannoma: case report and brief review of the literature

Objetivo: Reportar un caso de schwannoma lingual, así como revisar las características diagnósticas (clínicas, por imágenes e histopatológicas) y terapéuticas de esta patología mediante las publicaciones disponibles. Caso clínico: Se presenta un caso de schwannoma in- traoral en un hombre de 26 años de edad con un tumor ubica- do en el tercio anterior de la lengua. Se resolvió mediante un único procedimiento quirúrgico bajo anestesia local sin obser- varse recidivas ni secuelas al menos a 12 meses de seguimien- to a distancia. El schwannoma de la lengua es poco frecuente pero sus características clínico-imagenológicas presentan ras- gos benignos que favorecen la resolución mediante una única biopsia quirúrgica total cuando el tamaño y la ubicación sean propicios para ello (AU)

To describe a case of lingual schwannoma, and through a literature review, to provide data on clinical, imaging and pathological features, as well as treatment methods. Clinical case: A 26-year-old male patient with a swelling in the tip of the tongue was diagnosed as having oral schwannoma. A single surgery was performed, without recurrence or postoperative complications at least during a 12-month follow-up. Lingual schwannoma is a rare entity with benign clinical and imaging characteristics that ena- ble surgical removal without prior biopsy when size and oral location are favorable (AU))

Case for diagnosis. Infraorbital schwannoma

Abstract We report a 40-year-old man, with an unremarkable personal and family history, who presented for evaluation of an asymptomatic papule located on his right cheek. Histopathology revealed an encapsulated neoplasm within the dermis; composed by narrow, elongated, and wavy cells with an ill-defined cytoplasm, dense chromatin and tapered ends interspersed with collagen fibers. Pathologic findings were consistent with tissue of Antoni B pattern. The diagnosis was an infraorbital schwannoma. The incidental finding of rare tumors like this, should make clinicians consider a greater spectrum of differential diagnoses for a unilateral skin-colored papule on the cheek of patients.

Case for diagnosis. Infraorbital schwannoma.

We report a 40-year-old man, with an unremarkable personal and family history, who presented for evaluation of an asymptomatic papule located on his right cheek. Histopathology revealed an encapsulated neoplasm within the dermis; composed by narrow, elongated, and wavy cells with an ill-defined cytoplasm, dense chromatin and tapered ends interspersed with collagen fibers. Pathologic findings were consistent with tissue of Antoni B pattern. The diagnosis was an infraorbital schwannoma. The incidental finding of rare tumors like this, should make clinicians consider a greater spectrum of differential diagnoses for a unilateral skin-colored papule on the cheek of patients.

Retrospective Analysis of 20 Patients Affected by Schwannomas in the Upper and Lower Limbs / Análise retrospectiva de 20 pacientes acometidos por schwannoma nos membros superior e inferior

Abstract Objective To analyze the epidemiological profile and evolution of 20 patients diagnosed with upper- and lower-limb schwannomas. Methods A group of patients was defined for a retrospective evaluation comprising the period between February 2002 and June 2018, in which we studied and evaluated 20 medical records of patients undergoing surgery due to schwannoma; the diagnosis was confirmed by an anatomopathological examination. Results Male and female patients were equally affected. The average age was 50.85 years, ranging from 12 to 77 years. There was a predominance of the upper limb and of the flexor face. The most affected nerve was the ulnar nerve. In total, 6 (30%) patients had transient postoperative complications. No cases of tumor recurrence were identified. Conclusion Schwannoma is a rare and difficult-to-diagnose lesion. It should always be considered as a hypothesis when facing a soft-tissue tumor affecting the limbs. The Tinel sign should be regarded, given its higher correlation with complications. The patients should be informed of the possible postoperative complications, which are frequent but usually transient.

Resumo Objetivo Analisar o perfil epidemiológico e a evolução de 20 pacientes diagnosticados com schwannoma nos membros superiores e inferiores. Métodos Definiu-se um grupo de pacientes para avaliação retrospectiva, compreendendo o período entre fevereiro de 2002 e junho de 2018, no qual foram estudados e avaliados 20 prontuários de pacientes submetidos a procedimento cirúrgico devido a schwannoma; a confirmação diagnóstica foi feita pelo exame anatomopatológico. Resultados Tanto os pacientes do sexo masculino quanto do feminino foram igualmente acometidos, e a média de idade foi de 50,85 anos, variando de 12 a 77 anos. Houve predomínio do membro superior e da face flexora. O nervo mais acometido foi o ulnar, e 6 (30%) pacientes apresentaram complicações pós-operatórias transitórias. Não foi identificado nenhum caso de recidiva tumoral. Conclusão O schwannoma é uma lesão rara e de difícil diagnóstico. Deve sempre ser considerada como hipótese quando se estiver diante de um tumor de partes moles acometendo os membros. O sinal de Tinel deve ser levado em consideração por conta de sua maior correlação com as complicações. Os pacientes devem ser informados quanto às possíveis complicações pós-operatórias, que são frequentes, mas, geralmente, transitórias.

[Orbital schwannoma in a child with acute proptosis]. / Schwannoma orbitario en un niño con proptosis aguda.

Schwannoma is a usually benign primary tumor. It develops from the Schwann cells, which produce the myelin sheath that surrounds the peripheral nerves. It represents less than 10 % of the intracranial tumors, and it is infrequent in the pediatric age. We hereby present a 6-year-and-11-month-old previously healthy patient, with a history of intermittent generalized cephalea associated with proptosis and a diminished visual acuity of the left eye, epiphora and strabismus, with radiological evidence of retro-ocular mass. A macroscopically complete exeresis was performed, with an anatomopathological diagnosis of orbital schwannoma.

El schwannoma es un tumor primario, habitualmente, benigno, procedente de las células de Schwann, productoras de la vaina de mielina que recubre los nervios periféricos. Constituye menos del 10 % de los tumores intracraneales y es infrecuente en la edad pediátrica. Se presenta a un paciente de 6 años y 11 meses de edad, previamente sano, con antecedente de cefalea holocraneana intermitente asociado a proptosis y disminución de la agudeza visual del ojo izquierdo, epífora y estrabismo, con evidencia tomográfica de una masa retroocular. Se realizó la exéresis macroscópicamente completa, con diagnóstico anatomopatológico de schwannoma orbitario.

Peripheral nerve tumours: A 66-case retrospective study. / Tumores de los nervios periféricos: estudio retrospectivo de 66 casos.

BACKGROUND/OBJECTIVES: Peripheral nerve tumours (PNT) are rare lesions that are generally categorised as soft tissue tumours, so they are usually managed by a range of surgical disciplines such as plastic, orthopaedic and trauma surgery, dermatology, general surgery and neurosurgery. Appropriate knowledge of the therapeutic indication and surgical management are essential in order to avoid neurological deficit. MATERIALS AND METHODS: This is a retrospective study of 66 tumour lesions in 64 patients, acquired from the joint experience of 2surgical teams specialised in peripheral nerve surgery. RESULTS: The most common location was the lower limb and upper limb with 48 tumour lesions. The most common pathological diagnosis was Schwannoma, accounting for 51.5%. Complete tumour resection was achieved in 81.8% of the lesions and always with neurophysiological stimulation and/or monitoring. The most common postsurgical complication was neuropathic pain and/or a transient sensory disorder in 15% of surgeries, with only one persistent motor deficit appearing in 2cases that required nerve graft reconstruction. CONCLUSIONS: Although the therapeutic management of benign tumours such as Schwannomas can be considered to be relatively simple thanks to the application of the basic concepts of peripheral nerve microsurgery, malignant tumors, despite being very rare, require multidisciplinary management. The option of pre-surgical biopsy remains a controversial issue and no consensus has been reached among the different authors. In our opinion, percutaneous biopsy is not necessary in most cases.

Neurofibromatosis tipo 2, entidad a tener presente durante la práctica clínica / Neurofibromatosis type 2, entity to have present during clinical practice

La neurofibromatosis tipo 2 es un trastorno poco frecuente, que pertenece al grupo de las neurofibromatosis, que se caracterizan por la mayor propensión al desarrollo de tumores. Se presenta con múltiples tumores no malignos del sistema nervioso, incluidos schwannomas, meningiomas, ependimomas y gliomas, siendo los schwannomas vestibulares bilaterales una característica clásica. La mayoría de los casos se diagnostican en la adultez, sin embargo, las características clínicas habitualmente están presentes durante muchos años antes del diagnóstico. Es importante un alto índice de sospecha y un adecuado examen cutáneo y neurológico, ya que es crítico para hacer un diagnóstico correcto y precoz, y así, realizar un tratamiento interdisciplinario adecuado, evitando posibles complicaciones como son la pérdida auditiva y el uso de ayudas técnicas.

Neurofibromatosis type 2 is a rare disorder, belonging to the group of neurofibromatosis, which are characterized by the propensity for tumor development. The usual presentation are multiple non-malignant tumors of the nervous system, including schwannomas, meningiomas, ependymomas, and gliomas, with bilateral vestibular schwannomas being a classic feature. Most cases are diagnosed in adulthood; however, the clinical features are usually present for many years before diagnosis. A high index of suspicion and an adequate skin and neurological examination are important, since it is critical to make a correct and early diagnosis, so an appropriate interdisciplinary treatment can be performed, avoiding possible complications such as hearing loss and use of technical aids.

Laparoscopically excised retroperitoneal presacral Schwannoma: atypical pre and postoperative manifestations - case report.

BACKGROUND: We are a reporting a rare case of retroperitoneal schwanomma with atypical pre and postoperative manifestations. Retroperitoneal schwannomas are rare tumors that are difficult to preoperatively diagnose. CASE PRESENTATION: This is a case report of a male patient, 41 years old, with symptoms of hipogastric and lower right member pain, as well as a history of a papilliferous thyroid tumor. Computerized tomography exams were inconclusive, showing a mass in the presacral region with dimensions of 4.4 × 3.9 × 3.4 cm. Removal was carried out by laparoscopic surgery, with self-limited postoperative complications. Diagnosis was carried out by anatomopathological examination, and syndromic hypotheses were discarded. CONCLUSIONS: The postoperative complications of schwanomma are little reported in the literature. In the simultaneous occurrence of schwanomma and other endocrine tumors, further studies are warranted to better differentiate the cases that need investigation of syndromic causes.

Palatal Schwannoma: An Analysis of 45 Literature Reports and of an Illustrative Case

Abstract Introduction Schwannomas are benign tumors originating from differentiated Schwann cells. Being the least common intraoral neoplasm of neural origin, it is rarely seen in the palate. The literature lacks an extensive review of intraoral schwannoma confined to the palate. Objective To review previously reported cases of palatal schwannoma along with an illustrative case, and to provide a better insight regarding clinicopathological and radiological features of this neural tumor in a rare intraoral site. Data Synthesis We present a case of palatal schwannoma in a 16-year-old female. An additional 45 cases were identified in 2 medical database searches (PubMed and Google Scholar) published fromthe year 1985 onwards, and from13 countries, in the 5 continents. The ages of the patients ranged from3 to 84 years old. Palatal schwannoma showed a slight predilection to females, with a male/female ratio of ~ 1:1.81. Hard palate involvement is almost twice greater than soft palate involvement. Surgical excision was employed inalmost all of the cases, and recurrence was reported only once. Conclusion Palatal schwannomas, although rare, have been reported both over the hard and the soft palate. They mostly present as a painless, firm, well-encapsulated, slow-growing solitary lesion over the lateral palatal aspect. Imaging can add to suspicion and can delineate a differential diagnosis, but the diagnosis is confirmed by pathological examination. Fine-needle aspiration cytology (FNAC) is almost always inconclusive. Immunohistochemistry can assist in confirming a diagnosis, but is more important to rule out close differentials. Complete surgical excision is the treatment of choice, and recurrence or malignant transformation are extremely rare.

Trans-oral, Carbon Dioxide-Assisted Excision of an Unusual Schwannoma of the Tongue Base: Case Report and Review of the Literature

Abstract Introduction Schwannomas are benign, solitary, encapsulated tumors that may originate at any site of the peripheral nervous system, with the exception of the olfactory and optic nerves. Schwannomas of the base of tongue are very rare, and only sporadic cases are documented. The tongue base represents a challenge for surgeons. Carbon dioxide (CO2) laser might provide an effective surgical option for such lesions because of the easy access to the lesion, the bloodless surgical field and optimum epithelization of wounds. Objective We present an unusual case of pedunculated schwannoma of the tongue base treated via transoral CO2-assisted excision. We also provide a review of the available literature, in English language, on humans. Data synthesis The authors searched the PubMed database and Google up to July 2018. The following search terms were applied: tongue and lingual, combined with schwannoma and neurilemmoma. Titles and abstracts were screened, and, then, only supraglottic (hypopharyngeal) tongue base masses were considered. Fourteen articles were included in this review, reporting 17 cases. The age of the patients ranged from 9 to 39 years, affecting predominantly females. Dysphagia and lump sensations were the most common presenting symptoms, and the mean follow-up period range was 1.5 to 60 months (mean = 13 months). There was no evidence of recurrence in any of the cases. Conclusion We could conclude that tongue base schwannomas are rare. Transoral complete excision of the tumor is the treatment of choice. CO2 laser surgery is a minimally invasive treatment option that has been performed in few reports with no recurrence and with favorable outcomes.

Malignant Peripheral Nerve Sheath Tumor Arising Within Lumbar Spinal Plexiform Neurofibroma.

A 41-year-old woman presented with a 16-year history of lumbar pain and right leg paresthesia. Twenty-four years previously, she had been diagnosed with neurofibromatosis type 1 syndrome. Magnetic resonance imaging showed a heterogeneous mass extending through widened lumbar neural foramina to the psoas muscles bilaterally, with a dumbbell-like appearance. Biopsy led to the diagnosis of a malignant peripheral nerve sheath tumor, an uncommon soft tissue sarcoma of neural origin that may occur in the context of neurofibromatosis type 1 syndrome. Given the aggressive behavior of these tumors, prompt and accurate diagnosis is critical to improve patient outcomes.

[Facial nerve schwannoma as differential diagnosis of parotid tumors in pediatrics]. / Schwannoma del nervio facial como diagnóstico diferencial en tumores de la región parotídea en pediatría.

Schwannomas are benign tumors which arise from Schwann cells and take place along peripheral nerves. Extra-temporal facial nerve schwannomas are infrequent and present as painless masses in the parotid region, slow-growing and involvement of the facial nerve. Although rare, they should be taken into account as a differential diagnosis in parotid masses in children. Surgical strategies include superficial parotidectomy and surgical tumor resection with electrical nerve stimulation and nerve preservation. The following case describes a paediatric patient treated with the previously mentioned nerve sparing surgical technique.

El schwannoma es un tumor benigno originado de las células de Schwann y puede producirse a lo largo de cualquier nervio en el que estas células formen parte de su vaina. Los schwannomas del nervio facial extratemporales son infrecuentes y se presentan como masas indoloras en la región parotídea, de lento crecimiento y con compromiso del nervio facial. Se los debe tener en cuenta como diagnóstico diferencial en masas parotídeas en los niños, aunque sean raros. La utilización de la punción aspirativa con aguja fina y la resonancia magnética nuclear evidencia la mejor aproximación diagnóstica. El tratamiento quirúrgico de elección en la localización intraparotídea es la parotidectomía superficial. Otra opción es la tumorectomía completa con electroestimulación intraoperatoria y preservación del nervio facial. Se presenta un caso de schwannoma intraparotídeo en una paciente pediátrica operada con esta última técnica.

[Sigmoid intussusception as a clinical presentation of colonic schwannoma: Pediatric case]. / Invaginación sigmoidea como presentación clínica de schwannoma de colon: Caso pediátrico.

Schwannomas are tumours originating in the Schwann cells of the peripheral nerves. The appearance in the colon is extremely rare, and there are no known cases in the literature of schwannomas of the colon and rectum described in children. These tumours are usually asymptomatic, but can produce non-specific and gastrointestinal symptoms. The clinical presentation as intestinal intussusception is infrequent. We present a case of an 11-year-old girl with sigmoid swchannoma presented clinically as a colonic intussusception with abdominal pain, rectal bleeding and constipation of 10 days. An intestinal resection with free surgical margins was performed.

Los schwannomas son tumores que se originan de las células de Schwann de los nervios periféricos. Su localización en el colon es extremadamente rara y no se han descrito, en la literatura, casos en niños. Por lo general, estos tumores son asintomáticos, pero pueden producir síntomas inespecíficos y gastrointestinales. La presentación como invaginación intestinal es muy infrecuente. Se expone el caso clínico de una paciente de 11 años de edad con un schwannoma de colon manifestado clínicamente como invaginación intestinal de sigma con dolor abdominal, rectorragia y estreñimiento de 10 días de evolución, que precisó resección intestinal con márgenes quirúrgicos libres, con buena evolución posoperatoria.