The malignant peripheral nerve sheath tumor (MPNST) originates from neurofibromatosis type 1 (NF1). Because NF1 patients have many accompaniments with growth of additional masses, they usually overlook potential malignant changes in their masses. Our patient had two growing mass near the left elbow for several months; however, she ignored these masses until 7 days prior to writing this article, at which time they began bleeding. Traditionally, sarcoma including MPNST treatment consisted of amputation of the involved extremity. However, treatment now consists of surgical resection with adjuvant therapy. Therefore, we conducted resection of the mass and subsequent coverage with a local advancement flap. We believe that the most effective treatment for MPNST is early diagnosis and fast surgery, coupled with notification that there is always potential for malignant change in NF1 patient's masses.
Amputation ; Diagnosis ; Drug Therapy ; Early Diagnosis ; Elbow ; Extremities ; Hemorrhage ; Humans ; Neurilemmoma ; Neurofibromatoses* ; Neurofibromatosis 1* ; Peripheral Nerves* ; Sarcoma ; Writing

OBJECTIVETo explore the clinical characteristics, diagnosis and treatment regimens for retroperitoneal schwannoma.

METHODSClinicopathological data of 53 retroperitoneal schwannoma patients treated from January 1999 to April 2013 in our hospital were collected and analyzed using SPSS 13.0 statistical software.

RESULTSSymptoms of the retroperitoneal schwannoma were vague and nonspecific. 12 patients had interrupted abdominal pain, 9 patients had abdominal discomfort, and only 6 patients presented with abdominal mass while 24 patients were detected by health checkup. There were some characteristics but not specific findings in imaging examination such as CT, ultrasonography and MRI, so preoperative diagnosis rate was low with only 9 patients diagnosed as retroperitoneal schwannoma and 21 patients diagnosed as neurogenic tumor. S-100 immunohistochemisty was very important in pathological diagnosis, and the patients with benign retroperitoneal schwannoma got 100% tumor specific 5-year survival after complete excision while the 5-year survival of malignant retroperitoneal schwannoma was only 50.0%.

CONCLUSIONSRetroperitoneal schwannoma is a rare disease. Most of them are benign tumors, and complete surgical excision is the effective treatment.

Abdominal Pain ; Diagnostic Imaging ; Humans ; Magnetic Resonance Imaging ; Neurilemmoma ; diagnosis ; pathology ; therapy ; Physical Examination ; Retroperitoneal Neoplasms ; diagnosis ; pathology ; therapy ; Retroperitoneal Space ; Treatment Outcome

We report a case of 46-year-old xanthoderm woman who was diagnosed as malignant peripheral nerve sheath tumors of right maxillary sinus, and have a literature review. Histology confirmed a diagnosis of malignant peripheral nerve sheath tumor. The woman had the right total maxillectomy and postoperative adjuvant radiotherapy. There is no local recurrence or metastasis of one year following up. Literature review revealed MPNST in the nasal cavity and para-nasal sinuses were not common with poor prognosis. The main cause of death is local recurrence and metastasis. Surgical resection showed more advantage than adjuvant radiotherapy and chemotherapy.
Female ; Humans ; Maxillary Sinus ; Middle Aged ; Neoplasm Recurrence, Local ; Nerve Sheath Neoplasms ; pathology ; therapy ; Neurilemmoma ; pathology ; therapy ; Paranasal Sinus Neoplasms ; pathology ; therapy ; Radiotherapy, Adjuvant

If a testicular cancer patient has a mass in the retroperitoneum, a metastasis is often the first suspicion, probably leading to improper diagnosis and overtreatment. Here we report a case of retroperitoneal schwannoma mimicking metastatic seminoma. A 29-year-old man, who had a history of seminoma, presented with a single retroperitoneal mass suspected to be a metastasis. Because the patient refused radiotherapy, 3 cycles of cisplatin, etoposide, and bleomycin were offered. Post-chemotherapy computed tomography scan revealed persistence of the retroperitoneal mass, with no change in tumor size or characteristics. Subsequently, retroperitoneal lymph node dissection was performed. The dissected tissue contained negative lymph nodes but a single mass in the attached fat. Pathology revealed retroperitoneal schwannoma, which was confirmed by immunohistochemistry. Thus, clinicians should be aware of retroperitoneal schwannoma and its distinction from metastatic seminoma to avoid misdiagnosis and ensure proper treatment.
Adult ; Antibiotics, Antineoplastic ; therapeutic use ; Antineoplastic Agents ; therapeutic use ; Antineoplastic Agents, Phytogenic ; therapeutic use ; Bleomycin ; therapeutic use ; Cisplatin ; therapeutic use ; Diagnostic Errors ; Etoposide ; therapeutic use ; Humans ; Lymph Node Excision ; Lymph Nodes ; pathology ; Lymphatic Metastasis ; Male ; Neoplasms, Multiple Primary ; Neurilemmoma ; diagnostic imaging ; drug therapy ; pathology ; Radiography ; Retroperitoneal Neoplasms ; diagnostic imaging ; drug therapy ; pathology ; secondary ; Retroperitoneal Space ; Seminoma ; secondary ; surgery ; Testicular Neoplasms ; surgery

Actins ; metabolism ; Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Breast Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Calcium-Binding Proteins ; metabolism ; Carcinoma ; metabolism ; pathology ; Cyclophosphamide ; therapeutic use ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Fibrosarcoma ; metabolism ; pathology ; Fluorouracil ; therapeutic use ; Follow-Up Studies ; Humans ; Leiomyosarcoma ; drug therapy ; metabolism ; pathology ; surgery ; Mastectomy, Segmental ; Methotrexate ; therapeutic use ; Microfilament Proteins ; metabolism ; Neurilemmoma ; metabolism ; pathology ; Phyllodes Tumor ; metabolism ; pathology ; Postoperative Period ; Vimentin ; metabolism

Objective: To present a rare case of facial schwannoma manifesting as a parotid mass and discuss its diagnosis and treatment. Methods: Design: Case Report Setting: Tertiary Government Hospital Patient: One Results: A 48-year-old female was seen for a 2-year progressive left hemifacial paralysis and a 5-month gradually enlarging left infraauricular mass with episodes of tinnitus but intact hearing and balance. Physical examination showed a left-sided House Brackmann grade VI facial paralysis and a 5 x 4 x 3 cm soft, ill-defined, slightly movable, nontender, left infraauricular mass. Gadolinium-enhanced magnetic resonance imaging revealed a 5 cm heterogeneouslyenhancing lobulated mass centered within the deep lobe of the left parotid gland extending to the left mastoid, with facial nerve involvement. A diagnosis of a facial nerve tumor, probably a schwannoma, was entertained. Pure tone audiometry revealed normal hearing thresholds for both ears with dips at 6-8 KHz on the left. The patient underwent total parotidectomy with facial nerve tumor resection via transmastoid approach, with simultaneous facial – hypoglossal nerve anastomosis reconstruction. Histopathologic findings confirmed the diagnosis of a schwannoma. Postoperative facial function was Grade VI. Hearing and hypoglossal nerve function were preserved. Conclusion A progressive hemifacial paralysis of chronic duration with or without the presence of an infra-auricular mass should raise the suspicion of a facial nerve tumor. Gadolinium-enhanced magnetic resonance imaging is valuable since intraparotid facial nerve schwannomas are mostly diagnosed intraoperatively when the neoplasm and the nerve are exposed and determined to be contiguous. The clinician should be aware that not all parotid masses are salivary gland in origin.
Human ; Female ; Middle Aged ; NEURILEMMOMA ; PARALYSIS ; NEUROLOGIC MANIFESTATIONS ; FACIAL PARALYSIS ; MAGNETIC RESONANCE IMAGING ; DIAGNOSTIC IMAGING ; PHYSICAL EXAMINATION ; DIAGNOSIS ; THERAPEUTICS ; THERAPY

OBJECTIVES: To present a case of cervical vagal schwannoma and describe our experience with the clinical presentation, surgical and outcome of an elderly patient with this pathology.

METHODS:
Design: Case report
Setting: Tertiary Public Hospital
Patient: One

RESULTS: A 65-year-old lady presented with a recently enlarging, pulsatile right sided neck mass that had been asymptomatic for 15 years. Contrast CT revealed a circumscribed non-enhancing heterogenous 4 x 4 x 7 cm mass splaying the right internal jugular vein and common carotid artery. A neurogenic tumour was considered, and the mass was exercised from the vagus nerve with preservation of adjacent structures. Final histopathologic reading was schwannoma. However, the patient succumbed to complications following a second surgery for expanding hematoma.

CONCLUSION:  Schwannomas are benign, slow growing tumours that arise from Schwann cells of the nerve sheath. Cervical schwannomas originating from the vagus are rare but should be considered in patients presenting with solitary neck masses. Surgical extirpation is still the treatment of choice for nerve sheath tumours and recurrence is uncommon. Efforts should be made to preserve unaffected structures and patients should be counseled preoperatively on the possible high risk of morbidity especially in the elderly group where close follow up and aggressive rehabilitation should be instituted following surgery.

Human ; Female ; Aortic Bodies ; Neurilemmoma-therapy ; General Surgery ; Neoplasms ; Tomography Scanners, X-ray Computed ; Schwann Cells ; Vagus Nerve

Neurogenic neoplasms involving the head and neck are uncommon. We report a case of supraglottic schwannoma. The tumor was located in the right false vocal cord level. It was removed by transoral CO2 laser surgery.
Head ; Larynx ; Laser Therapy ; Lasers, Gas ; Neck ; Neurilemmoma ; Vocal Cords

INTRODUCTIONWe present a series of head and neck extracranial non-vestibular schwannomas treated during a ten-year period, assessing epidemiology, presenting signs and symptoms, location, nerve of origin, diagnostic modalities, treatment and clinical outcome.

MATERIALS AND METHODSClinical records of all patients with head and neck schwannomas treated at our department from April 1995 to July 2005 were retrospectively reviewed.

RESULTSThere was female predominance (67%). The mean age at diagnosis was 48 years. Sixteen (76%) presented with a unilateral neck mass. Eleven schwannomas (52%) were in the parapharyngeal space. The most common nerves of origin were the vagus and the cervical sympathetic chain. The tumour may masquerade as a cervical lymph node and other myriad conditions. Treatment for all but 2 cases was complete excision with nerve preservation. Two cases of facial schwannoma required sacrifice of the affected nerve portion with nerve reconstruction. All facial schwannoma patients suffered postoperative facial palsy with only partial resolution (mean final House-Brackman grade, 3.25/6). Among non-facial schwannoma patients, postoperative neural deficit occurred in 12 with partial to complete resolution in 7. The median follow-up period was 24 months. No schwannoma was malignant and none recurred.

CONCLUSIONNon-vestibular extracranial head and neck schwannomas most frequently present as an innocuous longstanding unilateral parapharyngeal neck mass. Preoperative diagnosis may be aided by fine-needle cytology and magnetic resonance imaging or computed tomographic imaging. The mainstay of treatment is complete intracapsular excision preserving the nerve of origin, but for extensive tumour or facial schwannomas, subtotal resection or nerve sacrifice with reconstruction and rehabilitation are considerations. Surgery on intraparotid facial schwannomas carries considerable morbidity and conservative management has a place in treatment. Early recognition of facial schwannomas is key to optimal treatment.

Adult ; Aged ; Female ; Head and Neck Neoplasms ; diagnosis ; epidemiology ; pathology ; therapy ; Hospitals, General ; Humans ; Male ; Medical Audit ; Middle Aged ; Neurilemmoma ; diagnosis ; epidemiology ; pathology ; therapy ; Outcome Assessment (Health Care) ; Retrospective Studies ; Risk Factors ; Singapore ; epidemiology ; Time Factors

Tracheal neurilemoma, an extremely rare benign tracheal tumor that there has been only one case reported in 1996 throughout the nation, is a slowly progressing disease that obliterates the upper airway, delays diagnosis for its symptom similarity to asthma, and makes intubation for operation difficult. Bronchoscopic is therefore needed for diagnosis. There are two options for the treatment methods, a bronchoscopic resection or open surgical resection; however if intubation is difficult, then the bronchoscopic resection is used first to keep the airway open for the surgical resection. In this case, the severe tracheal stenosis impeding intubation made the surgical resection of the primary tracheal neurilemoma with extratracheal mass impossible; therefore, bronchoscopic laser resection was applied first to optain the airway passage for endotracheal intubation, followed by a successful open surgical resection.
Asthma ; Diagnosis ; Humans ; Intubation ; Intubation, Intratracheal ; Laser Therapy* ; Neurilemmoma* ; Tracheal Neoplasms ; Tracheal Stenosis*