Giant intercostal nerve schwannoma in a patient with neurofibromatosis type 2 (original) (raw)
Related papers
Recurrent Malignant Schwannoma of the Parapharyngeal Space in Neurofibromatosis Type 1
Ear, Nose & Throat Journal, 2003
Ma lignant schwannoma is an agg ressive tumor that ca rries a poor p rognosis despite wid e excision, chemothe rapy, and radio therapy. Ma ligna nt scliwannoma of the pa raphary ngeal space is an uncom mon finding; to our know ledge, only fo ur cases have been described in the literature during the pas t 30 years, and only one ofthem invo lved a patient who had clinical ev idence of neurofibromatosis type 1. In this article , we describe a new cas e of malignant schwannoma of the pa rapharyngeal space in a patient who had clinical evidence ofneurofibromatosis type 1. Following resection of the tum or and a total parotidectomy, the diagnosis was made on the basis of histology and immun ohistoch emistry. The patient und erwent postoperative chemothe rapy with carboplatin and UP16. How ever, 5 months f ollowin g sur gery, the tumor recurred and metastasized. The patient was then placed on a different polychemotherap eutic regim en, which was made up of3 g/m' ofifosfamide, 1.5 mg/m' of vincristine, and 1.5 mg/m ' of doxorubicin (I VA 2 protoco l). The I VN regim en slowed tum or growth, but 13 month s after the initiati on of therapy, the pat ient died of neoplastic cachexia. A lthough chemotherapy is generally ineffecti ve in most cases of malignant schwanno ma, we did expe rience som e positi ve results with the IVA 2 protocol. Therefore, we recommend that this combination be cons idered as a first-line adj uvant therapy following surge ry or as a first-line therapy for pa tients with inoperable tumors.
American Chinese Journal of Medicine and Science, 2009
Intrathoracic neurogenic tumors are not uncommon, but presentation as a giant mass in the thoracic cavity is rare. Although several cases of intrathoracic giant malignant peripheral nerve sheath tumor have been reported, only one case of intrathoracic giant benign neurofibroma appears in the literature. In this report, we describe a very rare case of atypical giant neurofibroma in the right thoracic cavity. The patient was a 57 year old African American man, who developed sudden cardiac arrest and passed away in the emergency room. At autopsy, a huge encapsulated firm tumor was found in the right thoracic cavity, attached to the vertebral bodies and superficially adherent to the upper and middle lobes of the right lung. This giant mass weighed 2140 grams and measured 31 x 30 x 5.5 cm. Microscopically, the tumor consisted of interweaving fascicles of spindle cells with scattered atypical nuclei. Immunohistochemical studies showed that tumor cells were focally positive for S100, and negative for SMA, desmin, calretinin, Pan CK, CK5/6, EMA, CD99, CD34 and p53. The overall morphological and immunohistochemical features were diagnostic of an atypical neurofibroma. [N A
Mixed schwannoma with meningioma of the trigeminal nerve
Indian Journal of Pathology and Microbiology, 2010
The coexistence of schwannoma and meningioma as a mixed intracranial tumor is a rarity usually seen in association with neurofibromatosis type 2 (NF-2). [1] Only 10 cases have been described so far in literature. [1-7] The most common site of involvement is in the cerebello-pontine angle (CPA), involving the vestibular nerve. Involvement of the trigmenial nerve has never been reported previously. We present a mixed tumor comprising of distinct schwannoma and meningioma components within the same tumor, arising from the trigeminal nerve, and review the relevant literature. CASE REPORT A 33-year-old male presented with complaints of redness of the right eye, episodic diplopia, and numbness of the right side of face since two months. There was no history of seizure, ear discharge, visual deterioration or loss of consciousness. On examination there was reduced right sided blinking response, reduced sensation on the right side of face (about 30-40%), and weakness with atrophy of the right side jaw muscles. Patient's visual acuity was normal and there was movement of the extra-ocular muscles. On systemic examination, no subcutaneous nodules were identified. There was no family history of neurofibromatosis. Computerized tomography (CT) scans showed a right sided extra-axial medial temporal region hypo dense mass. A similar small mass was seen on the left side. Contrast enhanced magnetic resonance imaging (MRI) [Figure 1] revealed bilateral parasellar masses (larger on right side) abutting bilateral body and lower wing of sphenoid and extending to the anterior aspect of the petrous apex. The masses were hypo intense on T1 weighted images and hyper intense in T2 with contrast enhancement. For the right sided mass, a right pterional craniotomy and excision of tumor by sub-temporal, intra-dural approach ABSTRACT Mixed tumors composed of schwannoma and meningiomas are extremely rare and are usually associated with neurofibromatosis-2 (NF-2). So far, all the cases reported have involved the cerebello-pontine angle. Only two cases did not have a clear association with NF-2. We report a mixed tumor comprising of meningioma admixed with schwannoma in a 33-year-old male with bilateral trigeminal nerve schwannomas. The patient did not fit the existing diagnostic criteria of NF-2. The relevant literature, along with diagnostic criteria for NF-2 is discussed.
The European respiratory journal, 1990
Neurogenic pulmonary tumours are rare. Neuronbromas are the most common variety and tend to occur In patients with generaUzed neurofibromatosis, whereas schwannomas arise sporadically In patients who have no evidence of this disorder. A case of solitary benign scbwannoma arising In a segmental bronchus Is presented, and the published cases reviewed. Microscopically, the tumour bad a spindle-shaped cell proliferation with palisade formation In several nelds and low cellularlty In other areas. The diagnosis was supported by positive lmmunostalnlng for S-100 protein and by ultrastructural examination which showed elongated cells surrounded by well-preserved basal lamina and numerous cytoplasmic processes also possessing a basal lamina.
Airway management of a neurofibromatosis type 2 with multicompartmental tumours: a case report
Malaysian Journal of Anaesthesiology
Neurofibromatosis type 2 (NF2) is a rare autosomal dominant disorder. We report a case of a 52-year-old woman with underlying NF2 who was scheduled for excision of cervical neurofibromas. The patient had four nerve sheath tumours affecting different parts of the airway, namely, two cervical neurofibromas with spinal cord compression, a large retrosternal goitre extending into the anterior mediastinal compartment causing central airway obstruction, and a large left thoracic paravertebral tumour in the posterior mediastinal compartment. After risk stratification and multidisciplinary discussion, awake nasal fibreoptic intubation with a contingency plan of rigid bronchoscopy and jet ventilation was decided. The patient was intubated successfully with target-controlled infusion remifentanil as the sole sedative and airway topicalization with local anaesthetic. The patient was ventilated in a prone position intraoperatively with no desaturation. The tumours were successfully removed and ...
Neurofibroma at an Aberrant Location -A Case Report
https://ijshr.com/IJSHR\_Vol.7\_Issue.3\_July2022/IJSHR-Abstract.01.html, 2022
Background: Neurofibromas are benign tumors of peripheral nerve sheath and are uncommon in cervical part of vagus nerve. A 35-year female presented with nontender swelling on the left side of the neck since 2 months with no family history. Clinical diagnosis was made as? carotid body tumor/parapharyngeal mass. CT neck showed well defined hypodense oval mass in the left upper neck in the carotid space reported as features suggestive of schwannoma. Post-operative diagnosis of left vagal schwannoma was made and sent for histopathological examination. Gross morphology was well circumscribed grey white mass measuring 6x4x3cms with gelatinous cut surface. On microscopy it was neoplasm composed of spindle cells with poorly defined cell borders having buckled nuclei with pale eosinophilic cytoplasm. These cells are arranged loosely in the background of collagenous matrix. No evidence of atypia or malignancy noted. Features consistent with Neurofibroma. Conclusion: Histopathological confirmation is essential for the diagnosis of neurofibroma in cervical part of vagus nerve as it is an uncommon site for the lesion to develop.
Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia, 2008
A 31-year-old man with neurofibromatosis 1 (NF1) was admitted for the treatment of solitary lung tumor. Nine months earlier he had undergone a large resection for malignant peripheral nerve sheath tumors (MPNSTs) in his back. Surgical resection of the right lower lobe was performed, and the tumor was pathologically diagnosed as a metastasis of MPNST. The survival of patients with pulmonary metastasis of MPNST is extremely poor, especially of those with NF1, but this patient has survived 5 years without recurrence. Based on our knowledge of the literature, a 5-year survival is extremely rare, and select patients have benefited from a resection of pulmonary metastasis.