A case of macrodactyly associated with neurofiborma of the median nerve, a congenital anomaly of the hand, affecting only one(left middle) finger is reported with a review of the literature. Macrodactyly which is also termed local gigantism, megalodactylism, megalodactylia, or macrodactylism in other literature, is a rare congenital malformation characterized by overgrowth of one or more fingers of hand. Macrodactyly associated with neurofibroma of the median nerve is especially rare. For this reason the following case is presented together with a review of the literature.
Adolescent ; Angiography ; Female ; Fingers/abnormalities* ; Fingers/radiography ; Fingers/surgery ; Human ; Korea ; Median Nerve*/surgery ; Neurofibroma/complications* ; Neurofibroma/pathology ; Neurofibroma/surgery ; Peripheral Nervous System Neoplasms/complications* ; Peripheral Nervous System Neoplasms/pathology ; Peripheral Nervous System Neoplasms/surgery ; Radial Nerve/surgery

BACKGROUNDNeurofibroma is a kind of benign neoplasm that derives from nervous tissues. Though this tumor is the most common types in the peripheral nervous system, it is rarely seen in the choroid and easy to be misdiagnosed of choroidal melanoma. The aim of this study was to review the clinical features of neurofibroma of the choroid in the Chinese race.

METHODSA retrospective case series design was used. Two male and one female patients diagnosed with choroidal neurofibroma in Beijing Tongren Eye Center were included in this study. The clinical histories were abstracted from the patients' medical records. Routine eye examinations including visual acuity, intraocular pressure, slit lamp and ophthalmoscope were performed. Auxiliary examinations included fluorescein fundus angiography (FFA), AB-ultrasound scan, color doppler imaging (CDI), and magnetic resonance imaging (MRI). Local resection of the tumors was performed and the specimens underwent pathological examinations.

RESULTSThe tumors were of yellow-pink color with pigmentation on the surface. CDI showed arterial blood signals in the tumor and MRI showed high-intensity in the T1-weighted image and a slightly increased intensity in the T2-weighted image. FFA and indocyaninegreen angiography demonstrated the tumors were of hypofluorescence at early stage and hyperfluorescence with prominent leakage at late stage. The pathological examination confirmed the tumors were choroidal neurofibroma. After 5 - 10-year follow-up, there were no recurrences of the tumors and the retinas were well attached.

CONCLUSIONSChoroidal neurofibroma is difficult to be diagnosed clinically and pathological confirmation is important. These tumors can be managed conservatively by local resection.

Adult ; Choroid Neoplasms ; diagnosis ; surgery ; Female ; Humans ; Male ; Middle Aged ; Neurofibroma ; diagnosis ; surgery ; Retrospective Studies

OBJECTIVETo study the indication and character of the lateral-cervical approach for treating dumble-shape neurogenic tumors in cervical spine.

METHODSRetrospectively review the clinical data of 14 dumble-shape neurogenic tumors in cervical spine, from October 2005 to October 2011. Among them 8 were males and 6 were females, range from 11 to 60 years old. The maximum tumor diameter range from 3.0 to 8.0 cm, with an average of 4.8 cm; the intraspinal tumor diameter range from 1.3 to 3.8 cm, with an average of 2.1 cm. According to Asazuma classification, 9 cases were type IIc, 2 cases were type IIIb, 2 cases were type IV, 1 case was type VI. Involving the neck segment C(1)-C(2) in 1 case, C(2)-C(3) in 1 case, C(3)-C(4) in 2 cases, C(4)-C(5) in 2 cases, C(5)-C(6) in 3 cases, C(6)-C(7) in 4 cases and C(2)-C(4) in 1 case. All cases performed surgery with general anethesia. The head and neck surgeon performed surgery with lateral cervical approach, in the space between the anterior and the medius scalenus, exposed the transverse process and the intervertebral foramen as the anatomy marker, resected the extraspinal tumor part. The neurosurgery expanded the intervertebral foramen, and resected the intraspinal tumor with microscope, and repaired the dura. Then head and neck surgeon closed the wounds.

RESULTSPathology proved 3 neurolimmoas and 11 Schwannomas, 12 cases received gross total resection, 2 cases received subtotal resection, the average blood loss during operation was 292 ml, the average operation time was 129 minutes, the average stay in hospital days was 7.1 days. The vertebral artery were exposed in 2 cases, and no vertebral artery injury occurred, there were 3 cases dissect the cervical nerve roots. No cerebrospinal fluid leakage, hematoma, newly branchial plexus injury, sympathic nerve injury or tracheal edema occurred. In 3 to 24 months, with an average of 13.5 months follow-up period, 2 cases with subtotal resection had no tumor progression, and 12 cases with gross total resection had no tumor recurrence.

CONCLUSIONSLateral-cervical approach is minimal invasive, easily to perform and recovery fine. It can be adopt for Asazuma type IIc, IIIb and IV tumors which not grow over the midline in spine and expand to deep layer of the deep cervical fascia out spine.

Adolescent ; Adult ; Cervical Vertebrae ; surgery ; Child ; Female ; Humans ; Male ; Middle Aged ; Neurilemmoma ; surgery ; Neurofibroma ; surgery ; Neurosurgical Procedures ; methods ; Retrospective Studies ; Spinal Neoplasms ; surgery ; Young Adult

OBJECTIVETo study the clinical manifestations, diagnostic methods, surgical management and prognosis of patients with neurogenic tumors of the mediastinum.

METHODOne hundred and ten patients with neurogenic tumors of the mediastinum were analyzed retrospectively.

RESULTSAfter operation, 2 patients died in hospitalization and 8 experienced such complications as Horner's syndrome or laryngeal recurrent nerve paralysis. In 102 patients with benign tumors, 2 patients had recurrence, and 4 patients with neurofibrosarcoma or malignant neurilemmoma died within 3 years postoperatively.

CONCLUSIONSMost neurogenic tumors of the mediastinum are benign and could be diagnosed by chest X-ray or CT. The clinical manifestations, diagnosis methods, surgical management of the dumbbell tumors differ from others. Minimal invasive surgery and video assist thoracoscopy surgery are of special value in treatment of the selected neurogenic tumors of the mediastinum. Benign neurogenic tumors rarely recur after complete resection, and malignant neurogenic tumors have poor prognosis.

Adolescent ; Adult ; Aged ; Child ; Female ; Humans ; Male ; Mediastinal Neoplasms ; diagnosis ; mortality ; surgery ; Middle Aged ; Neurilemmoma ; diagnosis ; mortality ; surgery ; Neurofibroma ; diagnosis ; mortality ; surgery ; Prognosis ; Retrospective Studies

OBJECTIVE: To investigate the surgical treatment for cervical dumbbell tumors. METHODS: Clinical manifestation, surgical type and stage, and surgical method of 36 consecutive patients with cervical dumbbell tumor were retrospectively studied. RESULTS: The tumors in 35 patients were completely resected once, and the other one patient underwent subtotal resection. Signs and symptoms of all patients were recovered to different degrees, without significant complications and tumor recurrence. CONCLUSION According to the type and stage of dumbbell tumors, we could adopt surgical methods through posterior or posterior combined with anterior approach, together with instruments and fusion, which could completely resect tumors and prevent complications.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Cervical Vertebrae ; pathology ; surgery ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neurilemmoma ; surgery ; Neurofibroma ; surgery ; Neurosurgical Procedures ; methods ; Retrospective Studies ; Spinal Canal ; pathology ; surgery ; Spinal Neoplasms ; classification ; surgery ; Young Adult

OBJECTIVETo provide the experience for early diagnosis and management of facial nerve neuromas, and to discuss the clinic and imaging feature of facial nerve schwannoma and facial nerve fibroma in 22 cases.

METHODSTwenty cases facial nerve schwannoma and two cases of facial nerve neurofibroma were diagnosed and reviewed retrospectively. Surgical removal were performed through the middle cranial fossa in 2 cases, through intratemporal approach in 8 cases, through intraparotid approach in 2 cases, and combined intra-temporal with out-temporal approaches in 10 cases. Seventeen cases underwent facial nerve graft for repairing a facial nerve defect. Great auricular nerve was used in 3 cases with intratemporal approach and 1 case with intratemporal combined intraparotid approach. Sural nerve graft was used in 5 cases with intratemporal approach and 8 cases with intra-temporal combined intraparotid approach. Two cases were employed two-stage facial muscle flap-plasty.

RESULTSFacial nerve neuromas were totally removed in 21 cases and subtotal neuroma removed in 1 case. In these cases, 20 patients were no recurrence and 1 patient was lost follow-up. One patient with subtotal neuroma removal received Gamma Knife treatment before and after surgery, and this case was no recurrence. The CT imaging of the temporal bone showed that schwannoma was separated "white mass" with smooth margin along the region of facial nerve without intact canal. But neurofibroma locate in enlarge fallopian with intact canal. Magnetic resonance imaging had the advantage of evaluating all segments of the facial nerve and showed continuity of intratemporal and intraparotid mass with the facial nerve. Pathological results indicated that 20 cases were diagnosed as facial nerve schwannoma and 2 cases were neurofibroma.

CONCLUSIONSAlthough tumors originating from the facial nerve are extremely rare, it is possible to make early diagnosis through finding clinical feature and imaging methods. Generally, systematic surgical approach for tumor removal and facial nerve reconstruction should be considered in the cases with facial neurinoma.

Adolescent ; Adult ; Child ; Cranial Nerve Neoplasms ; diagnosis ; pathology ; surgery ; Facial Nerve ; pathology ; transplantation ; Female ; Humans ; Male ; Middle Aged ; Neoplasm Staging ; Neurilemmoma ; diagnosis ; pathology ; surgery ; Neurofibroma ; diagnosis ; pathology ; surgery ; Neuroma ; diagnosis ; pathology ; 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

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.
Ants ; Brachial Plexus Neuropathies ; Chest Tubes ; Drainage ; Female ; Ganglioneuroma ; Humans ; Mediastinum ; Neurilemmoma ; Neurofibroma ; Retrospective Studies ; Sternotomy ; Thoracic Surgery, Video-Assisted ; Thoracoscopy ; Thoracotomy ; Vocal Cord Paralysis

OBJECTIVE: This study was conducted to assess the surgical results of one-stage posterior minimal laminectomy and video-assisted thoracoscopic surgery (VATS) for the treatment of thoracic dumbbell tumor and to describe its precise technique. In addition, we investigated the technique's usefulness and limitations. METHODS: Seven cases of thoracic dumbbell tumor (two men and five women, mean age, 43 years) were analyzed retrospectively. Pathological findings included schwannoma in four patients, neurofibroma in two patients, and hemangioma in one patient. The location of tumors varied from T2/3 to T12/L1. Dumbbell tumors were resected by one-stage operation using posterior laminectomy followed by VATS without instrumentation. Clinical data were reviewed. RESULTS: The mean follow-up period was 25 months (range, 3–58 months), and the operative time ranged from 255 to 385 min (mean, 331 min), with estimated blood loss ranging from 110 to 930 mL (mean, 348 mL). The tumor was completely resected without instrumentation and postoperative instability in all cases. Postoperative complications included atelectasis and facial anhydrosis in one case each. CONCLUSION: One-stage posterior minimal laminectomy and VATS may be a safe and less invasive technique for removal of thoracic dumbbell tumor without instability. This method has the advantage of early ambulation and rapid recovery because it reduces blood loss and postoperative pain.
Early Ambulation ; Female ; Follow-Up Studies ; Hemangioma ; Humans ; Laminectomy* ; Male ; Methods ; Neurilemmoma ; Neurofibroma ; Operative Time ; Pain, Postoperative ; Postoperative Complications ; Pulmonary Atelectasis ; Retrospective Studies ; Thoracic Surgery, Video-Assisted*

OBJECTIVETo investigate the surgical mode, recurrence and prognosis for patients with head and neck neurofibromas and explore their treatment strategies.

METHODSThe clinicopathological features, operation mode, prognosis and neural function of 46 patients with head and neck neurofibroma were analyzed retrospectively, and 41 of the cases were followed up for 24-170 months (median 74 months).

RESULTSAmong the 41 followed-up patients, 26 patients were cured and 15 patients were not cured (two died). The cure rate of the neurofibromas with neurofibromatosis type 1 (NF1) and the neurofibromas without NF1 were 42.9% and 85.0%, respectively (P = 0.005). The cure rate of localized, aggressive, diffuse and beaded neurofibromas were 100.0%, 46.6%, 40.0% and 66.7%, respectively (P = 0.009). The cure rate of radical resection (including expanding excision and complete resection) and partial resection were 73.5% and 14.3%, respectively (P = 0.011). The cure rates of expanding excision and partial resection were 80.0% and 14.3% (P = 0.029). The cure rates of complete resection and partial resection were 70.8% and 14.3%, respectively (P = 0.026). However, the cure rates of expanding excision and complete resection were not significantly different (P = 0.581). Multivariate Cox model analysis indicated that thoroughness of surgery was the independent risk factor for the prognosis for patients with head and neck neurofibromas.

CONCLUSIONSNeurofibroma is a kind of aggressive benign tumors. Some neurofibromas have a high recurrence rate and low recovery rate, and some nerves are essay to be injured in the operation. Lots of factors impact on the prognosis and recovery of the neural function. Therefore, operation opportunity and mode should be carefully selected.

Follow-Up Studies ; Head and Neck Neoplasms ; pathology ; surgery ; Humans ; Neoplasm Recurrence, Local ; Neurofibroma ; pathology ; surgery ; Neurofibromatosis 1 ; pathology ; surgery ; Prognosis ; Proportional Hazards Models ; Retrospective Studies ; Risk Factors ; Time Factors