Malignant Peripheral Nerve Sheath Tumor Arising in Association With the Sural Nerve

Malignant peripheral nerve sheath tumor is a rare sarcoma of peripheral nerves found most often in deep soft tissue. This aggressive tumor is difficult to diagnose clinically and must be surgically excised for therapy. An incisional biopsy will allow for testing and in most cases, diagnosis. The authors present a case of a 39-year-old African American woman with malignant peripheral nerve sheath tumor in association with the sural nerve. The tumor was surgically removed and sent for pathologic studies. The patient reported to her first postoperative appointment and was referred to an oncologist for follow-up. Despite multiple attempts at contacting the patient and explaining the prognosis of the diagnosis, the patient refused further follow-up care including referral to an oncologist.     

Cutaneous Malignant Peripheral Nerve Sheath Tumor

Cutaneous malignant peripheral nerve sheath tumors (MPNSTs) are rare sarcomas of neuroectodermal origin arising in the dermis and/or subcutis. In contrast with their deep soft tissue and visceral counterparts, cutaneous MPNSTs are rarely associated with neurofibromatosis type 1. Two main subtypes of cutaneous MPNST can be distinguished histologically: conventional (ie, spindle cell) and epithelioid MPNST. The 2 subtypes also differ in predilection for deep versus superficial locations, association with preexistent benign peripheral nerve sheath tumors and S100 immunohistochemistry. Herein, we review current knowledge of cutaneous MPNST and discuss its differential diagnosis.     

Melanocytic Malignant Peripheral Nerve Sheath Tumor of the Male Breast

Summary

Background

Malignant peripheral nerve sheath tumors are rare tumor entities that originate from peripheral nerve sheaths and have an unfavorable prognosis. Common sites include deeper soft tissues, usually in the proximity of a nerve trunk. Breast is an absolutely rare location of this lesion, and presentation as a breast lump in the male breast is even rarer.

Case Report

A 65-year-old man presented with a 6-month history of a painless mass of the left breast. Tissue biopsy was performed. Histopathology revealed a malignant spindle cell tumor which was confirmed to be a melanocytic malignant peripheral nerve sheath tumor on the basis of immunopositivity for HMB45 and S-100.

Conclusion

There are no generally accepted guidelines for the treatment of malignant peripheral nerve sheath tumors in the male breast. The patient was referred for radiation therapy after simple mastectomy.Key Words: Malignant peripheral nerve sheath tumor, Breast, Melanocytic     

Malignant Peripheral Nerve Sheath Tumor Arising from the Left Ventricle

Abstract We report a case of a 32-year-old female who underwent complete excision of a malignant left ventricular peripheral nerve sheath tumor. Malignant peripheral nerve sheath tumors in the mediastinum, including the pericardium, are rare. This case report describes a malignant peripheral nerve sheath tumor arising from the left ventricular origin for which complete excision was possible. (J Card Surg 2012;27:567-570).     

Sporadic Multifocal Malignant Peripheral Nerve Sheath Tumor—A Rare Presentation

Malignant peripheral nerve sheath tumors(MPNST) are uncommon neoplasms with an incidence of 0.001% in general population. Multifocality is a rare manifestation of MPNST . A case of a 65 year old patient who presented with multiple swellings involving the neck, extremity and back without associated neurofibromatosis is reported for its rarity of presentation.. Diagnosis was made by FNAC and confirmed by peroperative findings and histopathology.     

Malignant Peripheral Nerve Sheath Tumor: An Unusual Cause of Intussusception

Malignant peripheral nerve sheath tumors (MPNST) are defined as any tumor arising from a peripheral nerve or showing nerve sheath differentiation. The majority of these tumors arise on the trunk, extremities, or head and neck region. The literature to date has fewer than 14 cases of MPNST arising in the gastrointestinal tract, and only two cases were ever reported in the small intestine, one of which was a recurrent disease. In this paper, we report the first US case of an MPNST arising in the small intestine and presenting as intussusception.     

Malignant Peripheral Nerve Sheath Tumor of the Orbit: Case Report and Literature Review

A 68-year-old woman with progressive visual loss and exophthalmos in her right eye had been operated on for a mass in her right calf 3 years earlier. Imaging showed a huge mass invading the orbital structures and temporal pole. The presumptive diagnosis was a malignant orbital tumor. The tumor was resected totally and eroded tissues such as the lateral rectus muscle and dural compartments were repaired. The histological diagnosis was a malignant peripheral nerve sheath tumor (MPNST). The patient recovered uneventfully and was discharged 8 days after surgery. Two years later she died from a liver tumor. Few MPNSTs involving the orbit have been reported.     

Total Occlusion of the Superior Vena Cava Due to Bronchogenic Cyst

Bronchogenic cysts are the most common form of congenital cystic lesions in the mediastinum. Of all cases with bronchogenic cysts, 1/3 are symptomatic. The symptoms vary depending on the location and compression of the adjacent structures of the cyst. Some mediastinal bronchogenic cysts can cause severe respiratory distress due to airway and vascular compression. We herein present a case with a bronchogenic cyst that required venoplasty to the superior vena cava (SVC) due to total occlusion of the SVC.     

Supraclavicular Catheterization of the Superior Vena Cava

In seeking to develop a simple and safe technique of central vein catheterization, a supraclavicular route to the superior vena cava was chosen. Advancing from a point above the medial portion of the clavicle, the needle punctures the wall at the confluence of the subclavian and internal jugular veins, and a siliconized catheter is introduced straight into the superior vena cava. The technique is described in detail.
The results of 600 catheterizations are reported. Unsuccessful attempts at cannulation were recorded in 6.3%, malposition of the tip of the catheter in 1.7%, pneumothorax in 0.3% and other complications in altogether 3.3% of the cases. Attention is drawn to the potential dangers inherent in cannulation of major central veins. The supraclavicular route described seems to provide a straightforward, reproducible approach to the superior vena cava, and it seems to have a low incidence of technical failures and early and late complications.     

Surgical Relief of Thrombotic Superior Vena Cava Obstruction Caused by Endocardial Pacing Catheter

Superior vena cava obstruction, an uncommon thrombotic complication of transvenous endocardial pacing, can usually be managed with long-term anticoagulation therapy. In a 55-year-old woman with a permanent pacemaker, progressive obstruction of the superior vena cava developed despite nine months of warfarin sodium therapy. Relief was obtained by interposing a spiral vein graft from the left internal jugular vein to the right atrium. The literature is reviewed, and recommendations are made for management of this unusual problem.     

Surgical Treatment of Complex Malignant Anterior Mediastinal Tumors Invading the Superior Vena Cava

Determining the appropriate surgery-based treatment for complicated anterior mediastinal malignancies (CAMM), especially those invading the superior vena cava (SVC) and its branches, remains a challenge for general thoracic surgeons. In this report, we summarize our experience and lessons regarding this issue in order to discuss a reasonable strategy for diagnosis and treatment of CAMM. Between January 2001 and April 2003, 15 patients with CAMM invading the SVC and/or its branches with or without invasion of other neighboring organs were surgically treated in our institution by a single surgeon team. We collected clinical data from the medical charts and from surgeons’ specific notes for complicated cases, and performed a comprehensive analysis. There were 9 patients with malignant thymoma. Thymic carcinoma, teratoma, embryonal carcinoma, Hodgkin’s lymphoma, non-Hodgkin’s lymphoma, and mixed teratoma with thymoma were diagnosed in 1 patient each. All procedures were performed via median sternotomy. Some angioplasty techniques were successfully used to resect and reconstruct the SVC. Ten of the 15 patients also underwent pulmonary resection due to involvement of pulmonary parenchyma. Four of the patients underwent perioperative chemotherapy. There were no perioperative deaths. Two patients suffered prolonged ventilation after surgery, and there were no other severe complications related to surgery. One patient died 10 months after surgery. The remaining 14 patients were still living and their progress is still monitored. As of August 2004, the median follow-up duration for all patients was 35 months, and the disease-free survival duration was 10–43 months. CAMM can be safely and completely resected via a median sternotomy, even if it has invaded other mediastinal structures. CAMM should be pathologically identified before initial treatment. A good outcome for patients with CAMM is possible if a suitable strategy combining accurate diagnosis and appropriate treatment, especially surgical resection, is established. Ke-Neng Chen and Shao-Fa Xu contributed equally to this work.