Malignant peripheral nerve sheath tumor mimicking carotid body tumor--case report and review

Malignant peripheral nerve sheath tumor is a rare neurogenic tumor that usually presents in geriatic patients. Typically, it is found in the trunk and extremities and rarely presents in the head and neck region. It may mimic a carotid body tumor when it presents in the neck. We report the first case of malignant peripheral nerve sheath tumor of the vagus nerve in an adolescent boy. He presented with an asymptomatic lateral neck lump that was thought to be a benign schwannoma on preoperative imaging. We describe the diagnostic dilemma and management difficulties in this patient and review the literature.     

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 of the vagus nerve in a teenager with the neurofibromatosis 1 gene mutation: a case report

Malignant peripheral nerve sheath tumors account for approximately 5% to 10% of all soft tissue sarcomas in which 25% to 50% are diagnosed in patients with neurofibromatosis 1 (NF1). Tumors are often located in the proximal portion of the upper and lower extremities and trunk, whereas cervical vagus nerve localizations are extremely rare, and the English literature is limited to isolated case reports. Malignant peripheral nerve sheath tumors usually affect adults. However, earlier presentation is described in patients with the NF1 mutation. The authors describe a very rare case of malignant peripheral nerve sheath tumor of the vagus nerve in a teenage patient with NF1 focusing on surgical management of this uncommon pathology and its histopathologic features to underline the importance of differential diagnosis and early treatment of this rare and aggressive tumor.     

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.     

Nerve Sheath Myxoma (Neurothekeoma) of the Gingiva,A Case Report and Review of the Literature

Nerve sheath myxoma (NSM) is a benign peripheral nerve sheath tumor that rarely occurs in the oral cavity. Among the 23 reported intraoral cases, no lesion has previously been reported on the gingiva. In this report, we describe the first gingival case of oral neurothekeoma with histopathologic and immunohistochemical characteristics. The patient, a 32 year old female presented with a slowly growing gingival mass diagnosed clinically as an epulis. The lesion was surgically excised. Histopathologically, the lesion presented as a submucosal multinodular mass composed of spindle and stellate-shaped cells with a myxoid background. Immunohistochemically, the tumor cells were sporadically positive for S-100 and NSE and negative for GFAP, EMA, SMA, CD68 and HMB45. The immunoprofile of this lesion confirmed a Schwann cell origin. The lesion was followed up for 10 months with no reports of recurrence.     

Concurrent epithelioid malignant peripheral nerve sheath tumor and papillary thyroid carcinoma in the treated field of Hodgkin's disease

BACKGROUND: Simultaneous malignancies in the field of radiation for Hodgkin's disease is an extremely rare event. A unique case of concurrent thyroid and neck mass in the postirradiation field of a young patient with Hodgkin's disease is presented. METHODS AND RESULTS: Thyroidectomy and excision biopsy of the neck mass were performed. A 1.5-cm papillary thyroid carcinoma was identified in thyroidectomy and an initial diagnosis of undifferentiated malignant neoplasm was rendered on the neck mass biopsy. Subsequent surgical excision of the neck mass and immunohistochemical analysis revealed malignant peripheral nerve sheath tumor. CONCLUSION: Concurrent malignancies in the field of treatment of Hodgkin's disease may occur. Rare malignancies including malignant peripheral nerve sheath tumor may be encountered along with the more common papillary thyroid carcinoma.     

Would a Massive Intra-abdominal Malignant Peripheral Nerve Sheath Tumor with Growth into the Inguinal Canal and Scrotum Preclude Surgical Option? A Case Report and Review of Literature

Malignant peripheral nerve sheath tumors (MPNST) are rare spindle-cell sarcomas derived from Schwann cells or pluripotent cells of the neural crest accounting for less than 10 % of all soft tissue sarcomas. They arise from major or minor peripheral nerve fibers or their sheaths. The World Health Organization coined the term MPNST for tumors of neurogenic origin with similar biological behavior replacing all the previous heterogeneous and, often, confusing nomenclature including malignant schwannoma, malignant neurilemmoma, and neurofibrosarcoma. The retroperitoneum and the lower extremities are the most common sites, but MPNST may arise anywhere in the body. Its location in the retroperitoneum in a patient without neurofibromatosis is an exceedingly rare occurrence. Imaging is routinely performed to assess the extent of the disease and to plan surgical resection. Surgical resection is the first line of therapy, ideally with total removal of the tumor. Owing to a high risk of recurrence with incomplete resection, postoperative irradiation and chemotherapy are necessary; however, they are often used as adjuvant therapy even if the tumor is completely resected.     

Malignant peripheral nerve sheath tumor arising from the colon in a newborn: report of a case and review of the literatures

A malignant peripheral nerve sheath tumor (MPNST) is a rare neoplasm arising from peripheral nerve sheath. Here, we report the first case of MPNST arising in the colon and also the youngest case of MPNST in the gastrointestinal tract. The patient was a 2-day-old neonate with symptoms and signs of intestinal obstruction. The patient had no family history or stigmata of neurofibromatosis type 1. A computed tomographic scan revealed a 5-cm-sized mass in ascending colon causing intestinal obstruction, and emergent right hemicolectomy was performed.The microscopic examination showed atypical spindle cells with hyperchromatic nuclei and high mitotic activity. The results of immunohistochemical staining, which showed positivity for S-100 and vimentin as well as negativity for smooth muscle actin, CD34, and c-Kit, supported the final diagnosis of MPNST. Genetic analysis of the patient revealed no abnormalities.After surgery, the patient recovered uneventfully and has been free of the disease for 17 months.     

Malignant peripheral nerve sheath tumor of the kidney

Malignant peripheral nerve sheath tumors are rare in the genitourinary organs, with few reports of occurrence in the kidney. We describe a patient with a renal malignant peripheral nerve sheath tumor, discovered after excision of a malignant peripheral nerve sheath scalp lesion, with additional masses in the lung and shoulder on metastatic evaluation. This patient underwent neoadjuvant intravenous doxorubicin therapy, followed by surgical resection of the scalp, lung and shoulder lesions in addition to a radical nephrectomy.     

Posterior Tibial Nerve Lymphoma Presenting as Tarsal Tunnel Syndrome: A Case Report

We report a case of isolated posterior tibial B-cell lymphoma of the posterior tibial nerve presenting as tarsal tunnel syndrome. This diagnosis was considered because of the clinical presentation and electrophysiologic abnormalities. It was further confirmed by the magnetic resonance imaging findings of the ankle and tissue pathologic findings. Whole body positron emission tomography confirmed this to be a localized lymphoma involving the peripheral nerve. The patient underwent chemotherapy with complete tumor resolution. She had had no relapse after 8 months of follow-up. Isolated peripheral nerve lymphomas are very rare, and involvement of the posterior tibial nerve has not been previously reported. Furthermore, the present case report highlights the importance of the clinical examination in the diagnosis of tarsal tunnel syndrome before performing surgical decompression.     

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.     

Peripheral Nerve Schwannoma: A Review of Varying Clinical Presentations and Imaging Findings

A schwannoma or neurilemmoma is a benign, isolated, noninvasive, and encapsulated tumor originating from Schwann cells of the peripheral nerve sheath. The incidence of a schwannoma occurring in the foot and ankle is rare, with prevalence rate of 1% to 10%. Schwannomas have no sex predilection, and they commonly occur in patients in their fourth decade. Malignant transformation of benign schwannoma is unusual; however, it is important to note that malignant variants of schwannomas do exist and account for about 5% to 10% of all soft tissue sarcomas. We present 3 cases of benign schwannoma in the lower extremity. All 3 patients presented with varying clinical symptoms, including pain, paresthesia, weakness, and a palpable mass. A schwannoma was eventually diagnosed in all 3 patients. We discuss and review the known entities of peripheral nerve schwannoma and describe the clinical and imaging findings and therapeutic strategies for treating and diagnosing peripheral nerve schwannoma.     

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.     

Primary Intraosseous Malignant Peripheral Nerve Sheath Tumor of the Medial Cuneiform: A Case Report and Review of the Literature

Peripheral nerve sheath tumors (benign and malignant) usually arise in the soft tissues and are unusual in bone. Intraosseous peripheral nerve sheath tumors are usually benign and constitute approximately 0.2% of all bone tumors. Intraosseous malignant peripheral nerve sheath tumors (MPNSTs) are uncommon and usually result from secondary invasion. Only a few cases of primary intraosseous MPNSTs have been reported in published studies, and these were localized mostly in the mandible (approximately 50%) or maxilla, spine, and, occasionally, in the appendicular skeleton. To the best of our knowledge, we report the first case of primary intraosseous MPNST involving a midtarsal bone (medial cuneiform). The patient was a 62-year-old female who presented with pain and tenderness but without swelling. Imaging revealed nonspecific findings, and the preoperative computed tomography-guided biopsy findings were consistent with MPNST. The patient was treated with neoadjuvant radiotherapy, followed by wide local excision and allograft reconstruction. At the final follow-up examination (24 months), the graft had been incorporated without evidence of local recurrence or distant disease. The patient with primary intraosseous MPNST of the medial cuneiform described in the present report presented with nonspecific clinical and radiologic findings. Thus, a high index of suspicion and histopathologic examination, including immunohistochemistry, are necessary for an accurate diagnosis.     

Malignant triton tumor of the gluteal region in a patient unaffected by neurofibromatosis: A case report

Malignant triton tumor (MTT) is a rare variant of malignant peripheral nerve sheath tumor (MPNST) made up of both malignant schwannoma cells and malignant rhabdomyoblasts.A 26-years-old male patient was admitted with an asymptomatic gluteal mass. Magnetic resonance imaging showed heterogeneous soft tissue mass and he underwent open biopsy. Malignant peripheral nerve sheath tumor was diagnosed. He was given adjuvant chemotherapy following the removal of the tumor with hip disarticulation. The tumor was diagnosed as “malignant triton tumor” based on pathological examination including immunohistochemical studies. There were no signs of metastasis but recurrence was observed at 9 months follow up.MTT is usually associated with Neurofibromatosis 1 and located in head, neck region. In this case sporadic involvement of gluteal region and aggressive behavior of the lesion despite radical surgery was demonstrated.     

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).     

Superficial Peroneal Nerve to Deep Peroneal Nerve Transfer With Allograft Conduit for Neuroma in Continuity

The anterior approach to the ankle for surgery can result in injury to the superficial peroneal nerve, resulting in a painful neuroma and significant patient morbidity. A paucity of data is available evaluating the role of the superficial peroneal nerve to deep peroneal nerve transfer as a method of treatment of neuromas in continuity after ankle arthrodesis. We describe 11 patients who underwent nerve transfer with nerve allograft and conduit repair to treat recalcitrant painful neuromas after ankle arthrodesis. At a mean follow-up period of 31 months, the mean visual analog pain scale score had improved from 7.9 preoperatively to 2.45 postoperatively (p?<?.0001). These data suggest that nerve transfer with a nerve allograft can provide significant clinical improvement for painful neuromas of the peripheral nerves at the ankle.     

Salvage of the Foot for Recurrent Malignant Peripheral Nerve Sheath Tumor

Malignant peripheral nerve sheath tumors are rare soft tissue tumors accounting for 3% to 10% of all soft tissue tumors. They are strongly related to neurofibromatosis type 1, an autosomal dominant disease, and are characterized by aggressive biologic behavior, high local recurrence rates, and frequent metastases. Although the major nerves of the lower extremities are a common location of these tumors, scarce cases have been reported of malignant peripheral nerve sheath tumors involving the interdigital nerves of the foot. We report the case of a patient with non-neurofibromatosis type 1 and a recurrent malignant peripheral nerve sheath tumor of the first interdigital nerve of the foot treated successfully with limb salvage surgery with wide resection margins and reconstruction with an autogenous fibula graft.     

A rare case of a solitary peripheral nerve sheath sarcoma of the scalp

Summary Malignant peripheral nerve sheath tumors are rare and most often appear in the clinical setting of von Recklinghausen's neruofibromatosis. On rare occasions they are seen in patients lacking any of the overt stigmata of this systemic disease. We report on a case of a peripheral nerve sheath sarcoma (PNSS) on the scalp and the back of the neck of a 13-year-old female arising in a solitary benign neurofibroma with no evidence of systemic neurofibromatosis. The tumor was examined by ultrastructural and immunohistochemical techniques as well as by routine histopathology. It was defined as a peripheral nerve sheath sarcoma, fibrosarcomatous type, of poorly differentiated perineurial cell origin. The possible significance of histogenetic subclassification in terms of surgical management and the oncological approach as well as the prognosis is discussed.