Maxillary Schwannoma – A Rare Case Report

Background: Schwannoma or neurilemmoma are a type of nerve sheath tumors. Most of the literature, reports of schwan- noma in the head and neck involve tongue. Intraosseous schwannomas account to about less than 1% with affliction to the mandible over maxilla. Very few cases of maxillary schwannomas have been reported till date. Case presentation: We present one such rare case of schwannoma involving the maxilla extending till the nasal cavity in a 35 years old male. Wide excision of the lesion with hemi-maxillectomy of the right side was done using modified Weber–Fergusson approach (Dieffenbach’s extension). The patient’s postoperative recovery was uneventful without recurrence when followed up till 2 years. Conclusion: Though schwannoma of the Head and neck region are rare, they should be considered as a differential diagnosis of unilateral slow growing mass in the head and neck region, particularly in an adult.

Surgical management of sacral neurogenic tumors / 中国骨伤

OBJECTIVE@#To observe the efficacy and complications of one-stage tumor resection to treat primary sacral neurogenic tumors and to discuss some details in the clinically relevant anatomy.@*METHODS@#A retrospective analysis of 26 patients with neurogenic turors of the sacral spine who were surgically treated from January 2001 to January 2018, including 16 males and 10 females, aged from 21 to 69 years old with an average age of (39.3±10.9) years old. The courses of diseases ranged from 3 to 56 months with an average of (17.9±10.1) months. The diameters of presacral components ranged from 3.3 to 19.6 cm with an average of (8.7±4.1) cm. The proximal margin of presacral lesions was above the L5S1 level in 6 cases, and lower than L5S1 in 20 cases. A posterior incision approach for one-stage complete resection of the tumor was used firstly, and an anterior approach was combined when necessary. Spinal-pelvic reconstruction with the modified Galveston technique was also carried out in relevant cases. Whether to preserve the tumor-involved nerve roots depended on the situation during the operation. The operation time, intraoperative blood loss, pain relief, and complications were recorded. The lumbosacral spine stability and sacral plexus neurological function were evaluated during postoperative follow-up, and local recurrence and distant metastasis were examined as well.@*RESULTS@#Total excision was achieved in all 26 patients, with an operation time of (160.4±35.3) mins and an intraoperative blood loss of (1 092.3±568.8) ml. Tumors have been removed via a posterior-only approach in 21 cases and via combined anterior/posterior approaches in 5 cases. The diameter of presacral masses components ranged from 11.3 to 19.6 cm with an average of (15.1±3.2) cm in patients with combined anterior/posterior approaches, and ranged from 3.3 to 10.9 cm with an average of (7.2±2.4) cm in patients with a posterior-only approach. Five of the six patients whose proximal margin of presacral masses was above the L5S1 level adopted combined anterior/posterior approaches, and 20 patients lower than the L5S1 level adopted the posterior-only approach. All the patients were followed up for 6 to 82 months with an average of(45.4±18.2)months. Postoperative lumbosacral pain and lower extremity radicular pain were significantly relieved, and sensation, muscle strength and bowel and bladder function were also improved to varying degrees. The postoperative early complications included superficial wound infection in 1 case and cerebrospinal fluid leakage in 2 cases. Pathology confirmed 17 cases of schwannoma, 7 cases of neurofibroma and 2 cases of malignant schwannoma. Local recurrence was observed in two cases of benign neurogenic tumors. One patient with a malignant nerve sheath tumor had lung metastasis, who died 20 months after the operation. In 17 cases of upper sacral neurogenic tumors, 4 cases did not undergo spinal-pelvic reconstruction with internal fixation, of which 2 cases suffered from postoperative segmental instability. Tumor-involved nerve roots were resected during surgery in 7 cases. One of these patients who had S2 and S3 nerve roots sacrificed simultaneously had an impaired bladder and bowel function postoperatively, and did not recover completely. In the other 6 cases, the neurological function was not damaged obviously or recovered well.@*CONCLUSION@#The posterior approach can directly expose the lesions, and it is also convenient to deal with nerve roots and blood vessels. The operation time, intraoperative blood loss, degree of symptom relief, complication rate, and recurrence and metastasis rate can be controlled at an appropriate level. It is a safe and effective surgical approach. When the upper edge of the presacral mass is higher than the L5S1 level or the diameter of the presacral mass exceeds 10 cm, an additional anterior approach should be considered. The stress between the spine and pelvis is high, and internal fixation should be used to restore the mechanical continuity of the spine and pelvis during resection of neurogenic tumors of the high sacral spine. Most of the parent nerve roots have lost their function. Resection of a single parent nerve root is unlikely to cause severe neurological dysfunction, while the adjacent nerve roots have compensatory functions and should be preserved as much as possible during surgery.

Neurofibromatosis sin neurofibromas con tumor mediastinal como forma de debut / Neurofibromatosis without neurofibromas. Case report with a mediastinal tumor as the first sign of the disease

Se presenta paciente con historia familiar de neurofibromatosis (NF) tipo I, con síntomas y signos sugestivos de esta enfermedad que refiere dolor torácico paravertebral izquierdo al cual mediante estudios de imagen se le diagnostica lesión tumoral en mediastino posterior. Es de notar la presencia de manchas cutáneas características de la neurofibromatosis, localizadas solo en un dermatoma del cuerpo sin neurofibromas en ninguna otra localización. Se intervino quirúrgicamente el enfermo, se resecó una gran masa mediastinal que se confirmó histológicamente ser un neurofibroma. La evolución posquirúrgica fue satisfactoria. Se discuten las singularidades de este enfermo sobre la base de criterios diagnósticos de neurofibromatosis tipo I, pero sin neurofibromas periféricos hasta ese momento. La posibilidad de una neurofibromatosis segmentaria tampoco se descarta. Se hace énfasis en la necesidad de resección de cualquier lesión tumoral en el contexto de este síndrome genético por la frecuencia de lesiones malignas asociadas a la neurofibromatosis y a la progresión hacia la malignidad de lesiones primariamente benignas(AU)

A patient with a family history of Neurofibromatosis type I was presented. The patient referred symptoms and signs suggestive of that disease and complaint of left paravertebral chest pain. Imaging investigations were done and a posterior mediastinal tumor was diagnosed. It is important to highlight the presence of characteristics spot of neurofibromatosis localized only in one dermatome without neurofibromas in any other part of the body. The patient underwent a surgical intervention and a left thoracotomy was done, a large posterior mediastinal tumor was found and totally resected. The histology confirmed a neurofibroma. The postoperative evolution was satisfactory. The singularities of this patient were discussed, especially diagnosis criteria for neurofibromatosis type I, and the lack of peripheral neurofibromas until that moment. The possibility for a segmental neurofibromatosis also was considered. We pointed out about the necessity to remove any neoplastic lesion in the background of this genetic syndrome due to the high frequency of malignancies associated with neurofibromatosis, and also related with the malignant degeneration these tumors can develop(AU)

Clinical experience of primary neurogenic tumors in mediastinum with surgical treatment in 131 cases / 复旦学报（医学版）

Objective To analyze the clinical features,methods of treatment and prognosis of primary neurogenic tumors of mediastinum in patients taking surgical intervention.Methods A database was maintained retrospectively of all patients undergoing surgery for tumor and pathologically diagnosed with primary neurogenic tumors of mediastinum,managed in the Department of Thoracic Surgery,Zhongshan Hospital,Fudan University,Shanghai between Jan.,2008 and Dec.,2014.This work analyzed retrospectively the information about clinical and imaging features,surgical techniques and outcome extracted from medical records.Results Among the 131 cases,78 cases (59.5％) were males,53 cases (40.5％) were females;72 cases were diagnosed incidentally (55.0％),while the other 59 cases (45.0 ％) suffered from different symptoms.The posterior mediastinum was the most principal location with 61 cases in the left and 69 cases in the right,and 1 case remained in the anterior mediastinum.Total 98 cases (74.8％) underwent surgeries via video-assisted thoracic surgery (VATS),5 cases (3.8％) took VATS surgery with small incision,and 28 cases (21.4％) experienced open thoracotomy,with no mortality during perioperative period.Gross total resection was obtained in 130 patients (99.2％).The remaining patient underwent a palliative resection for malignant schwannomas.Of the patients,98 cases had benign schwannomas (74.8％),24 cases had gangliocytomas (18.3％),2 cases had malignant schwannomas (1.5％),2 cases had neurofibromas (1.5％),2 cases had paragangliomas (1.5％),2 cases hadprimitive neurotodermal tumor (PNET) (1.5％) and 1 case had neuroblastomas (0.8％).All patients were followed up from 12 to 95 months with an average of 53 months.A patient with PNET died of tumor metastasis,a patient with malignant schwannomas died after palliative ectomy,and 2 cases died of other reasons.The rest survived until Jan.,2016 with tumor free.Conclusions Nearly no specific clinical symptoms occur in neurogenic tumors of mediastinum.Most of neurogenic tumors of mediastinum are benign with optimistic prognosis after surgical treatment.While malignant neurogenic tumorsusually come with poor prognosis,which places special emphasis on early diagnose together with surgical treatment.

Is Video-assisted Thoracoscopic Resection for Treating Apical Neurogenic Tumors Always Safe? / 대한흉부외과학회지

BACKGROUND: Mediastinal neurogenic tumors are generally benign lesions and they are ideal candidates for performing resection via video-assisted thoracoscopic surgery (VATS). However, benign neurogenic tumors at the thoracic apex present technical problems for the surgeon because of the limited exposure of the neurovascular structures, and the optimal way to surgically access these tumors is still a matter of debate. This study aims to clarify the feasibility and safety of the VATS approach for performing surgical resection of benign apical neurogenic tumors (ANT). MATERIAL AND METHOD: From January 1996 to September 2008, 31 patients with benign ANT (15 males/16 females, mean age: 45 years, range: 8~73), were operated on by various surgical methods: 14 VATS, 10 lateral thoracotomies, 6 cervical or cervicothoracic incisions and 1 median sternotomy. 3 patients had associated von Recklinhausen's disease. The perioperative variables and complications were retrospectively reviewed according to the surgical approaches, and the surgical results of VATS were compared with those of the other invasive surgeries. RESULT: In the VATS group, the histologic diagnosis was schwannoma in 9 cases, neurofibroma in 4 cases and ganglioneuroma in 1 case, and the median tumor size was 4.3 cm (range: 1.2~7.0 cm). The operation time, amount of chest tube drainage and the postoperative stay in the VATS group were significantly less than that in the other invasive surgical group (p<0.05). No conversion thoracotomy was required. There were 2 cases of Hornor's syndrome and 2 brachial plexus neuropathies in the VATS group; there was 1 case of Honor's syndrome, 1 brachial plexus neuropathy, 1 vocal cord palsy and 2 non-neurologic complications in the invasive surgical group, and all the complications developed postoperatively. The operative method was an independent predictor for postoperative neuropathies in the VATS group (that is, non-enucleation of the tumor) (p=0.029). CONCLUSION: The VATS approach for treating benign ANT is a less invasive, safe and feasible method. Enucleation of the tumor during the VATS procedure may be an important technique to decrease the postoperative neurological complications.

En Bloc Resection of a Thoracic Outlet for a Recurred Malignant Schwannoma of the Brachial Plexus: A case report / 대한흉부외과학회지

Neurogenic tumors of the brachial plexus are rare. An malignant schwannoma originates from the schwan cells or nerve sheath cells. Occasionally, schwannomas are associated with Von Rechlinghausen's disease, but this is rare. We were recently presented with a thirty-five year old female patient with a history of pulmonary tuberculosis about ten years prior. The patient also presented with a mass that has been slowly growing for one year. Onset of pain occurred six months after the tumor began to grow. The mass was 5X7 cm in size. The patient underwent en bloc resection of the tumor as the mass recurred twice in spite of postoperative radiotherapy.