Malignant transformation of a neurilemmoma is an exceedingly rare event. We describe a case of intrathoracic ancient neurilemmoma undergoing a malignant change in a 39-yr-old man. The patient presented with right flank and chest pain for several months. Plain radiography and CT scan of the chest showed a soft tissue mass lesion at the extrapleural space with erosion of surrounding ribs at the right basal lung area. The excised mass was encapsulated and measured 4.5 x 3.5 x 2.3 cm. The cut surface showed grayish-white and glistening with a focal cystic change and hemorrhage. Necrosis was not seen. Histologically, the tumor showed the features of classic neurilemmoma composed of the Antoni type A and B areas with perivascular hyalinization. In addition, obviously histo-logically malignant foci manifested by presence of markedly increased cellularity with fascicular arrangement, active mitotic activity, hyperchromasia, and gradual loss of original neurilemmomatous feature were noted.
Adult ; Cell Transformation, Neoplastic* ; Diagnosis, Differential ; Human ; Male ; Neurilemmoma/complications ; Neurilemmoma/pathology* ; Neurofibromatosis 1/complications ; Neurofibromatosis 1/pathology* ; Thoracic Neoplasms/etiology ; Thoracic Neoplasms/pathology* ; Tumor Markers, Biological

Summary A 36 years old patient with hoarseness for 2 years and got worsen for one month, electronic laryngoscopy showed a red smooth-faced wide based neoplasm on the posterior 2/3 of the right side of the vocal cords. The neoplasm was excised under suspension laryngoscope . The pathologic results showed:Cells were weave patterned, infiltrative growth, mitotic figure was rare. Immunohistochemical results showed CD34 (-), SMA (-), DM (-), S - 100 (+). The pathological diagnosis was plexiform schwannoma.
Adult ; Hoarseness ; etiology ; Humans ; Laryngeal Neoplasms ; complications ; diagnosis ; surgery ; Laryngoscopes ; Laryngoscopy ; Neurilemmoma ; complications ; diagnosis ; surgery ; Syndrome ; Vocal Cords ; pathology

OBJECTIVETo investigate the diagnosis and treatment of facial nerve schwannomas with facial nerve function House-Brackmann grade (HB) ≤ grade II.

METHODSA retrospective study was conducted in Peking Union Medical College Hospital. We reviewed eight cases of facial nerve schwannomas with facial nerve function HB ≤ grade II, which were diagnosed and managed between Jan 1996 and March 2011.

RESULTSThe initial presenting symptoms of the eight patients were not facial paralysis. Eight patients were misdiagnosed and six had mistreatment histry. CT and(or) MRI results in all patients showed that the tumors originated from different part of the facial nerves. All patients received operation. Facial nerves were completely preserved in four patients because of easy separation of the tumors from the facial nerves in surgery, facial function was gradeII-III over 17 - 180 months' follow-up. The tumors were attached with the facial nerves in two patients with wide extension involving cochlea and labyrinth, therefore the tumors were removed together with the attached facial nerves, and the nerves were repaired by using the greater auricular nerves. Facial function was grade VI over 56 - 79 months' follow-up. One patient refused to sacrifice the facial nerve, wide decompression of facial nerve and tumor was undertaken, facial function was grade III over 8 months' follow-up. One chorda tympani neuroma was removed with the branch of the facial nerve, facial function was grade II over 8 months' follow-up.

CONCLUSIONSThe facial nerve schwannomas with facial nerve function HB ≤ grade II is difficult to diagnosis. The therapy strategy should depend on the patients' choice, position of the tumor and adherences of the tumor to facial nerve. Facial nerve could be preserved if the tumor is easy to be separated from the facial nerve during operation, if not, total remove the tumor and nerve repairment are indicted when invasion into the inner ear canal, cerebro pontine angle, cochlea or labyrinth. If patients refuse to sacrifice the facial nerve, facial nerve decompression and periodic follow-up are recommended.

Adult ; Diagnostic Errors ; Facial Nerve ; pathology ; Facial Paralysis ; complications ; diagnosis ; surgery ; Female ; Humans ; Male ; Middle Aged ; Neurilemmoma ; complications ; diagnosis ; surgery ; Retrospective Studies ; Young Adult

OBJECTIVE: 10 summarize tne clinical features of the facial nerve tumors involving the internal auditory canal and promote the management of facial nerve tumor. METHOD: We retrospectively reviewed the clinical manifestations, the experiences of diagnosis and treatment of the facial nerve tumor involving the internal auditory canal. All these 5 cases were enrolled during January 2013 to Apr 2015. RESULT: Among the 5 cases, 3 cases were facial neurilemmoma and the others were facial neurofibroma. The main symptoms of facial nerve tumors involving the internal auditory canal most commonly were facial paralysis companied with hearing loss. All the patients accepted the surgical treatment with various approaches, 3 cases of translabyrinthine approach, 1 case of middle fossa approach, and 1 case of combination of translabyrinthine and transotic approach. Total tumor resection were achieved in all 5 cases. Facial-hypoglossal nerve anastomosis was performed in one case, another case was undergone great auricular nerve graft. CONCLUSION Surgical intervention for patients with facial neuroma involving internal auditory canal should be considered when facial weakness has deteriorated to grade 4. The management should be based on the patient's hearing, facial nerve function, tumor size and invasive extension to select the appropriate surgical procedures.
Anastomosis, Surgical ; Cranial Nerve Neoplasms ; diagnosis ; surgery ; Facial Nerve ; pathology ; surgery ; Facial Nerve Diseases ; diagnosis ; surgery ; Facial Paralysis ; complications ; Hearing Loss ; complications ; Humans ; Hypoglossal Nerve ; surgery ; Neurilemmoma ; diagnosis ; Neurofibroma ; diagnosis ; Retrospective Studies

OBJECTIVE: Because the anatomical structure of the brachial plexus is very complex, surgical treatment of tumors in this region is challenging. Therefore, a lot of clinical and surgical experience is required for successful treatment; however, many neurosurgeons have difficulty accumulating this experience owing to the rarity of brachial plexus tumors. The purpose of this report is to share our surgical experience with brachial plexus tumor with other neurosurgeons.METHODS: The records of 18 consecutive patients with brachial plexus tumors who underwent surgical treatment between January 2010 and December 2017 in a single institution were retrospectively reviewed. The surgical approach was determined according to the tumor location and size, and intraoperative neurophysiological monitoring (IONM) was used in most of cases to prevent iatrogenic nerve injury during surgery. In addition, to evaluate the differences in tumor characteristics according to pathologic diagnosis, the tumors were divided twice into two groups, based on two separate classifications, and statistical analysis was performed.RESULTS: The 18 brachial plexus tumors comprised 15 (83.3%) benign peripheral nerve sheath tumors including schwannoma and neurofibroma, one (5.6%) malignant peripheral nerve sheath tumor, one (5.6%) benign tumor of non-neural sheath origin (neurogenic cyst), and one (5.6%) metastatic tumor (papillary carcinoma). The authors analyzed relationship between tumor size/location and tumor characteristic parameters such as age, size, right-left, and pathology. There were no statistically significant differences except a tendency of bigger tumor size in young age.CONCLUSION: For a successful surgical outcome, an appropriate surgical approach is essential, and the appropriate surgical approach is determined by the location and size of the tumor. Furthermore, applying IONM may prevent postoperative complications and it is favorable option for brachial plexus tumors surgery.
Brachial Plexus Neuropathies ; Brachial Plexus ; Classification ; Diagnosis ; Humans ; Intraoperative Neurophysiological Monitoring ; Monitoring, Intraoperative ; Nerve Sheath Neoplasms ; Neurilemmoma ; Neurofibroma ; Neurosurgeons ; Pathology ; Peripheral Nerves ; Postoperative Complications ; Retrospective Studies

We identified Charcot-Marie-Tooth disease type 1A (CMT1A) in a family with schwannomas in the spinal cord and median nerve. The CMT1A in this family showed an autosomal dominant pattern, like other CMT patients with PMP22 duplication, and the family also indicated a possible genetic predisposition to schwannomas by 'mother-to-son' transmission. CMT1A is mainly caused by duplication of chromosome 17p11.2-p12 (PMP22 gene duplication). A schwannoma is a benign encapsulated tumor originating from a Schwann cell. A case of hereditary neuropathy with liability to pressure palsies (HNPP) concurrent with schwannoma has been previously reported. Although it seems that the co-occurrence of CMT1A and schwannomas in a family would be the result of independent events, we could not completely ignore the possibility that the coincidence of two diseases might be due to a shared genetic background.
Adolescent ; Adult ; Charcot-Marie-Tooth Disease/complications/*diagnosis/genetics ; Chromosomes, Human, Pair 17 ; Female ; Genetic Predisposition to Disease ; Humans ; Magnetic Resonance Imaging ; Male ; Median Neuropathy/*diagnosis/genetics ; Myelin Proteins/genetics ; Neurilemmoma/complications/*diagnosis/pathology ; Pedigree ; Peripheral Nervous System Neoplasms/*diagnosis/genetics ; Spinal Cord Neoplasms/*diagnosis/genetics

PURPOSE: In treating schwannoma patients, it is critical to determine the origin of the tumor to preserve nerve function. We evaluated the validity of preoperative imaging studies in distinguishing the neurological origin of the schwannomas of the head and neck, and the efficacy of intracapsular enucleation in preserving nerve function. MATERIALS AND METHODS: In 7 cases of schwannomas in the head and neck region, we predicted whether the tumor originated from the vagus nerve or the cervical sympathetic chain through imaging studies including computed tomography (CT) and magnetic resonance imaging (MRI). All patients were performed intracapsular enucleation, and the function of the vagus nerve and the sympathetic nerve was evaluated preoperatively and postoperatively. RESULTS: Preoperative imaging studies showed 6 cases where the tumor was located between the carotid artery and the internal jugular vein, and 1 case where the tumor was located posteriorly, displacing the carotid artery and the internal jugular vein anteriorly. At the time of operation, we confirmed schwannoma originating from the vagus nerve on the first 6 cases, and schwannoma originating from the sympathetic nervous system on the last case. All patients went through successful intracapsular enucleation, and of the seven schwannoma cases, 6 patients maintained normal postoperative neurological function (85.7%). CONCLUSION: Preoperative imaging studies offer valuable information regarding the location and origination of the tumor, and intracapsular enucleation helped us to preserve the nerve function.
Aged ; Diagnostic Imaging/methods ; Female ; Follow-Up Studies ; Head and Neck Neoplasms/complications/diagnosis/*pathology ; Humans ; Magnetic Resonance Imaging/methods ; Male ; Middle Aged ; Neurilemmoma/complications/diagnosis/*pathology ; Peripheral Nervous System/injuries/physiology ; Sympathetic Nervous System/physiology ; Tomography, X-Ray Computed/methods ; Treatment Outcome ; Vagus Nerve/physiology