Tumor maligno de las vainas nerviosas periféricas en la región maxilofacial: schwannoma maligno mandibular / Malignant tumor of peripheral nerve sheaths in the maxillofacial region: malignant mandibular schwannoma

Objetivo: Describir el diagnóstico y la resolución de un caso clínico de schwannoma maligno mandibular, una neopla- sia extremadamente rara en la región maxilofacial y con alto porcentaje de mortalidad. Caso clínico: Un paciente masculino de 56 años de edad acudió a la consulta por trismus, dolor y no cierre de la herida posextracción en maxilar inferior, con una evolución de tres meses. Se le solicitó una tomografía computada que evidenció lesión osteolítica y pieza dentaria retenida en la zona afectada. Se realizó la biopsia excisional. El diagnósti- co fue schwannoma maligno mandibular. Nueve meses des- pués del tratamiento quirúrgico y coadyuvante, el paciente falleció. Conclusiones: La derivación a un especialista y el análisis histopatológico tempranos permiten diagnosticar a tiempo este tipo de neoplasias. El schwannoma maligno es una enfermedad agresiva, con una tasa de supervivencia baja, pero la intervención oportuna y el diagnóstico precoz mejoran el pronóstico y la sobrevida del paciente (AU)

Aim: Describe the diagnosis and resolution of a clini- cal case of malignant mandibular schwannoma. An extremely rare neoplasm in the maxillary facial region, and with a high percentage of mortality. Clinical case: A 56-year-old male patient attended the consultation due to trismus, pain and non-closure of the post-extraction wound in the lower jaw, with an evolution of three months. He was asked for a computed tomography scan, that showed an osteolytic lesion and retained tooth in the af- fected area. Excisional biopsy is performed. The diagnosis was malignant mandibular schwannoma. After surgical and adjuvant treatment, the patient dies nine months later. Conclusion: Early referral to a specialist and histo- pathological analysis will allow to diagnose this type of neoplasms early. Malignant schwannoma is recorded as an aggressive disease with a low survival rate, but timely inter- vention and early diagnosis improves the prognosis and pa- tient survival (AU)

Orbitectomía en monobloque en un paciente con sarcoma neurogénico de la órbita / Single block orbitectomy in a patient with neurogenic orbital sarcoma

El sarcoma neurogénico es un tumor maligno que se origina en las células de Schwann de la vaina del revestimiento de los nervios periféricos y son poco frecuentes en la órbita. Se presenta un paciente de 23 años de edad, masculino, blanco, con antecedentes de neurofibromatosis tipo I, con desplazamiento anteroinferior del globo ocular izquierdo, dolor intenso y pérdida de la visión de 4 meses de evolución. Al examen oftalmológico se constató proptosis severa con descenso del globo ocular izquierdo, oftalmoplejia total, quemosis severa, hiperemia, opacidad corneal y aumento de volumen del párpado superior. En los estudios imagenológicos se evidenció lesión tumoral que ocupaba la totalidad del compartimiento orbitario sin infiltración de sus paredes óseas y con desplazamiento del globo ocular por fuera del reborde orbitario. Se realizó un abordaje quirúrgico combinado, y se logró una orbitectomía en monobloque con resección total del tumor y reconstrucción con colgajo rotado de músculo temporal ipsilateral. El estudio histopatológico informó la presencia de un sarcoma neurogénico de la órbita y se complementó con tratamiento adyuvante con radioterapia. El paciente se mantuvo controlado durante un año y a partir de esta fecha comenzó la aparición secuencial de varias lesiones a distancia(AU)

Neurogenic sarcoma is a malignant tumor that starts in Schwann cells of the peripheral nerves sheath and is rarely found in the orbit. Here is a 23 year old, male, Caucasian patient, with a history of Type-I Neurofibromatosis, and a left eye fore and lower side displacement of the left eyeball, intense pain and loss of vision for 4 months. A severe proptosis and the lowering of the left eyeball was detected during the ophthalmologic examination, as well as total ophthalmoplegia, severe chemosis, hyperemia, corneal opacity and increased upper eyelid volume. Imaging studies revealed a tumor lesion occupying the whole orbital compartment, with no bone wall infiltration, but causing the displacement of the eyeball out of the orbit border. A combined surgical approach was performed consisting in a single block orbitectomy with total tumor resection, as well as the reconstruction with the ipsilateral temporal muscle rotated flap. The histopathology study showed the presence of an neurogenic orbital sarcoma, so an adjuvant radiotherapy treatment was indicated. The patient was followed up for a year, after which the sequential occurrence of several lesions began(AU)

Widely Infiltrating Epithelioid Malignant Peripheral Nerve Sheath Tumour of Skull Base

The epithelioid variant of malignant peripheral nerve sheath tumours is a rare histological entity, and the occurrence of a malignant peripheral nerve sheath tumour in the skull base is even more unusual. We report a case of a 52-year-old man who presented with reduced hearing in the left ear, giddiness and left-sided facial weakness of short duration. He was a known hypertensive. On examination, left-sided 7th to 12th cranial nerve palsies were noted. Computed tomography (CT) and brain magnetic resonance imaging (MRI) were reported as an ill-defined heterogeneously enhancing mass left skull base suggestive of chondrosarcoma. Left tympanotomy and biopsy of the lesion were carried out. On light microscopy and immunohistochemical examination of the biopsy, a diagnosis of epithelioid malignant peripheral nerve sheath tumour was established. The patient underwent left extended modified radical mastoidectomy and selective neck dissection. Histopathological study of the resected surgical specimen confirmed left-sided extensive tumour involvement of skull base structures, as well as neck nodal metastases.

A Case of Malignant Peripheral Nerve Sheath Tumor in Parapharyngeal Space / 대한이비인후과학회지

The malignant peripheral nerve sheath tumor is an extremely rare soft tissue sarcoma. It is a highly malignant sarcoma, which is locally invasive, frequently leading to multiple recurrences and eventual metastatic spread. The peak incidence of disease is known to occur sporadically between the age of 20s and 50s, and is usually associated with the neurofibromatosis type I. In human body, the trunk and extremities are the most commonly involved sites, with only 8-14% of all lesions appearing in the head and neck region. We present a case of malignant peripheral nerve sheath tumor involving the right parapharynx in a 48-year-old patient who complained of headaches in the right parietal area and of dysphagia that aggravated over a month. After surgery, tumor was finally diagnosed as malignant peripheral nerve sheath tumor by histopathologic examinations. The authors report a case of malignant peripheral nerve sheath tumor in the right parapharynx with a review of the literature.

Malignant Peripheral Nerve Sheath Tumor Not Associated with Neurofibromatosis / 대한피부과학회지

Malignant peripheral nerve sheath tumor (MPNST) is a rare entity of all soft tissue sarcomas. It has four different types of glandular, melanocytic, rhabdomyoblastic, and epithelioid. Epithelioid MPNST is composed of predominantly epithelioid cells. In contrast, typical MPNST is consisted of spindle cells, which is arranged in a fascicular or whirling pattern. A 33-old man showed two skin-color to reddish brown protruding nodules on the left thigh. Microscopically, one nodule was made up of epithelioid cell, primarily. In addition, the other nodule was composed of epithelioid cells and spindle cells, which also showed Antoni A and B. Immunohistochemical study showed the cells being positive for S-100 protein (weak and focal) and neuron-specific enolase, and negative for HMB-45, smooth muscle actin. Herein, we report a case of two different forms of malignant nerve sheath tumor on the left thigh, which was not associated with neurofibromatosis-1.

Schwannoma maligno en la mandíbula: reporte de un caso / Malignant Schwannoma in mandible: a case report

El schwannoma maligno es una neoplasia cuyo origen está dado por tejido nervioso periférico. Rara vez se presenta en cabeza y cuello, más escasamente se encuentra en el territorio maxilofacial. A continuación se reporta desde el punto de vista radiológico el caso de un paciente de sexo masculino, de 9 años de edad, que presentó un aumento de volumen de crecimiento rápido, expansivo, ubicado en el cuerpo y rama mandibular derecha con diagnóstico de neurosarcoma maligno de mandíbula o schwannoma maligno mandibular con compromiso del canal mandibular, canal y foramen mentoniano, tejido blando y tejido óseo del sector.

The malignant schwannoma is a neoplasia whose origin is given by peripheral nerve tissue. It rarely appears in the head and neck, and even less frequently in maxillofacial territory. Here is a case report of a male patient, 9 years old, with a rapidly growing mass, expansive growth, located in the body and right mandibular ramus diagnosed as a malignant neurosarcoma or malignant schwannoma of the mandible, with mandibular canal, mental canal and foramen, soft tissue and bone involvement of the region.

Apical Lung Cancer Associated with Neurofibromatosis Type 1

No abstract available.

Spinal Malignant Schwannoma with Hematogenous Pulmonary Metastases: Case Report

We report a case of multiple hematogenous pulmonary metastases from a spinal malignant schwannoma. A 54-year-old woman with a known primary spinal malignant schwannoma presented with a 2-month history of painful neck mass, ptosis, anhidrosis, facial edema and mild chest discomfort. She had had the spinal surgeries two times followed by postoperative radiation therapy. The chest radiograph revealed typical multiple metastatic nodules on the both lung fields. Metastatic malignant schwannoma to the lung was confirmed by fine needle aspiration biopsy using a 22-gauge needle under fluoroscopic gauidance. The result of cytology of the lung mass was malignant spindle cell tumors consistent with malignant schwannoma located in cervical spine. The course of metastases might be hematogenous due to direct vascular invasion of tumor.

Retroperitoneal Malignant Schwannoma / 대한비뇨기과학회지

Schwannoma, also known as a neurilemmoma, neurinoma, and perineural fibroblastoma, can be either a benign or malignant tumor, arising from the associated nerve sheath. A retroperitoneal malignant schwannoma is a rare tumor. Immunohistochemical staining for S-100, leucine-7 and the myelin basic protein is useful for diagnosis. Here we report a case of retroperitoneal malignant schwannoma with a brief review of the literatures.

A Case of Cervico-Thoracic Malignant Schwannoma with Lung Metastasis

A case of malignant schwannoma in the cervico-thoracic region with lung metastasis is presented. The patient was 55-year-old man presented with right arm weakness. Magnetic resonance image demonstrated lobulated enhancing soft tissue masses in spinal canal, neural foramen and right paraspinal space at C7-T1 level compressing the dural sac and spinal cord. Subtotal removal was performed and histological diagnosis of malignant schwannoma was made. Reoperation due to recurrence was done but subsequent metastasis to lung was observed.

A Case of Purely Epithelioid Peripheral Nerve Sheath Tumor / 대한피부과학회지

The purely epithelioid malignant peripheral nerve sheath tumor(MPNST) is a rare form of MPNST, first described by McCormack et al. in 1954. This tumor type differs from ordinary epithelioid MPNST because of absence of spindle cell components. The patient was 25-year-old woman with palpable bean-sized subcutaneous mass on the right upper arm for 8 months which enlarged gradually. On physical examinations, we could not find a evidence of lymphadenopathy or von Reklinghausen's disease. Microscopically the tumor showed well circumscribed encapsulated nodular pattern composed of epithelioid cells. The cells were plump round or polygonal cells which had vesicular nuclei and prominent nucleoli with abundant eosinophilic and some clear cytoplasms. Immunohistochemical study showed the cells being positive for S-100 protein and neuron specific enolase and negative for HMB-45, cytokeratin and vimentin. We report a case of purely epithelioid malignant peripheral nerve sheath tumor which is located superficially without evidence of von Recklinghaunsen's disease or associated peripheral nerve.

Three Cases of Primary Spinal Malignant Schwannomas: Case Reports

Malignant peripheral nerve sheath spinal tumours are relatively rare. A primary spinal location at the first onset is rarely reported in the literature, thus the clinical features and therapeutic results of these spinal malignant tumours are not defined. We describle three patients with primary spinal malignant schwannomas and stress the surgical mananement of these cases.

Malignant Schwannoma of the Scalp in Type I Neurofibromatosis: Case Report

It has been reported that malignant transformation of neurofibroma occurs in about 5 to 10 per cent of type I neurofibromatosis patients. This 68 year old female patient presented with a huge fungating mass lesion in the right parietal scalp, which had recently grown rapidly. She had multiple neurofibromas of varying sizes on the entire trunk, extremities, and face. On the basis of family history and opthalomologic examination, type I neurofibromatosis was diagnosed. The mass was totally removed and the pathologic report revealed malignant transformation of the benign neurofibroma to malignant schwannoma. According to the literature, the incidence of head and neck sarcomas is very low, in particular malignant schwannoma occurring in the scalp is extremely rare. This case was, then, a rare event associated with type I neurofibromatosis.

Malignant Schwannomas of the Ethmoid Sinus and the Larynx :Case Report and Review of Literatures / 대한치료방사선과학회지

The incidence of malignant schwannoma in the head and neck is extremly rate. Most tumors apper as a rapidly expanding nonpainful mass and the symptoms are usually attributablet to local expansion of the mass. About one half occurs in association with von Recklinghausen's disease. Wide surgical excision is generally recommended as a primary treatment. Recently, there has been a trend to include postoperative radiation therapy as a primary modality. Prognosis of head and neck malignant schwannoma has been reported as particularly poor. However, recent authors advocate that prolonged survival is possible after adequate therapy including postoperative radiation therapy.We present our experience with these tumors on very rare locations such as the ethmoid sinus and the larynx, with the review of literatures.

Two Cases of Malignant Schwannoma in Association with Neurofibromatosis

We report two cases of malignant schwannoma of skin in association with non-familial neurofibromatosis. Case 1, a 47 year old man, had a large subcutaneous tumor on the sacral area and case 2, a 62 year old woman, a painful, ulcerating tumor on the posterior aspect of the left arm. Both cases were histopathologically confirmed as malignant schwannomas and immunohistochemical studies showed 5-100 protein in the tumor cells. After surgical excision of the tumors, case 1 was lost to follow up, while case 2 remained without evidence of disease for more than one and half years.

Histopathologic and Immunohistochemical observation on Malignant Schwannoma

Histopathologic and immunohistochemical analysis using antibodies for S-100 protein and keratin has been conducted on 21 cases of malignant schwannomas. The 21 cases were divided into the following three groups Group A: tumors originating from the nerve trunk or neurofibroma; Group B: tumors related to von Recklinghausen's disease; and Group C: other tumors not belonging to the above groups but histologically diagnosed as malignant schwannoma. The commonest histological pattern consisted of either closely packed or loosely arranged interlacing fascicles of slender spindle cells with wavy fibrillar cytoplasm, followed by myxoid change, perithelial pattern, hyaline change of the blood vessels, and hyalinlzed cords or nodules. Nine out of 12 cases of malignant schwannomas in group A and B, and 7 out of 9 cases of group C were positive for S-100 protein. None of the above cases showed positive staining reaction for keratin. Since 7 of 9 malignant schwannomas in Group C stained with S-100 protein, we can conclude that careful histological analysis supplemented by immunohistichemical study can make a conclusive diagnosis in most of the cases of malignant schwannomas even in cases that do not fulfil the traditional strict criteria.

Malignant Schwannoma / 대한피부과학회지

Malignant schwannoma is a malignant neoplasm of nerve sheath origin. It develops in an unaltered nerve or in a neurofibroma, and rarely, if ever, within a neurilernmoma. We experienced a malignant schwannoma in a 75-year-old man without von Recklinghausen's syndrome. The lesion was solitary and polypoid, fungating nodule in the posterior portion of the left thigh, The tumor cells had some features of Schwann cells and positively stained to S-100 protein and neuron-specific enolase. Complete excision was performed but the associated nerve could not be demonstrable.

Malignant Schwannoma: Report of Two Cases / 대한정형외과학회잡지

A Malignant Schwannoma is a relatively rare primary nerve sheath tumor arises from Sehwann cells of the peripheral nerves. This tumor is frequently associated with von Recklinghausens disease. A high percentage of patients experience local recurrence even after radical surgical excision, and eventual pulmonary metastases via the intraneural and hematogenous routes. The treatment of choice is radical excision or amputation. Results of treatment with radiation and chemotherapy have been disappointing. The authors have experienced two cases of Malignant Schwannoma, one of which was located in the median nerve of the palm, the other in the ulnar nerve of the arm, but neither were associated with von Recklinghausens disease.

A Case of Malignant Schwannoma in the Sacrum

Malignant schwannoma is a relatively rare malignant neoplasm arising from schwann cell of neuroectodermal origin that infiltrate locally and widely, and metastases. Inreaosseous bone involvement or retroperitoneal location of this tumor is extremely rare. A rare case of solitary malignant schwannoma which originated in the sacrum and extended to the retroperitoneal space in a 34 year-old male without neurofibromatosis reported. Simple X-ray showed not sclerotic, smooth marginated lytic defect in the right sacral bone. Computed tomography(CT) of sacrum revealed homogenous soft mass with intraosseous and dumbel shaped retroperitoneal extension without enhancement after were appeared in serial iliac artery angiogram. We could remove the tumor mass without specific technical difficulty with the help of CT and angiographic findings.