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.     

A malignant peripheral nerve sheath tumor of the mediastinum in a patient with neurofibromatosis type 1: Report of a case

Malignant peripheral nerve sheath tumor (MPNST) is rare, but its association with neurofibromatosis type 1 (NF1) is well known. A 56-year-old man with NF1 was referred to our hospital for investigation of dyspnea. Computed tomography showed a huge mass occupying the right thorax and invading the inferior vena cava. A salvage resection was performed to alleviate the developing hypoxemia. Histological examination confirmed an MPNST. The tumor regrew rapidly and the patient died 6 weeks after the resection. This case report reinforces the importance of monitoring patients with NF1 carefully because of their increased risk of the development of malignant neoplasms.     

Malignant peripheral nerve sheath tumor of kidney—A case report

INTRODUCTIONMalignant peripheral nerve sheath tumors (MPNSTs) are very rare soft-tissue tumors. These tumors originate from Schwann cells or pluripotent perineural cells of the neural crest. An isolated MPNST of the kidney is extremely rare and has been reported in only six cases to date.PRESENTATION OF CASEA 30-year-old female with an unremarkable past medical and surgical history presented with an enlarging, right-sided abdominal mass for the last 3 months. A CT scan of the abdomen and pelvis showed a large exophytic mass arising from the right kidney. A metastatic work-up was negative, so a right radical nephrectomy was performed. Histopathological and immunohistochemical studies confirmed the diagnosis of an MPNST.DISCUSSIONMPNSTs are rare and aggressive soft-tissue sarcomas that are usually associated with neurofibromatosis type 1 (NF-1) but that also occur post-radiation and sporadically. These tumors are highly malignant neoplasms with a high recurrence rate and distant metastases. Complete surgical excision remains the mainstay of treatment due to the limited response of MPNSTs to both chemotherapy and radiotherapy.CONCLUSIONMPNSTs arising from the kidney are very rare. Complete surgical resection provides the only hope for cure. Postoperative chemotherapy and radiotherapy are still controversial, although postoperative radiotherapy is currently recommended by an oncology consensus group.     

Malignant peripheral nerve sheath tumor of the back

A 65-year-old woman developed a tumor on the medial aspect of the lumbosacral spine. The diagnosis on biopsy was neurogenic sarcoma, T3N0M0. Despite wide surgical excision and regular follow-up, the patient developed a local recurrence requiring several supplementary operations before her death 4 years after the initial diagnosis. This case illustrates the highly aggressive and recurrent nature of these rare neurogenic tumors; they require early multidisciplinary management and regular careful follow-up. Received: 13 November 1999 / Accepted: 23 February 2000     

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.     

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.     

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.     

Intrasellar malignant peripheral nerve sheath tumor (MPNST)

Summary Intracranial malignant peripheral nerve sheath tumors (MPNST) and intrasellar schwannomas are rare tumors. We describe a case of an intrasellar schwannoma with progression to a MPNST, a finding that, although very rare, extends the differential diagnosis of intrasellar lesions.     

Vaginal epithelioid malignant peripheral nerve sheath tumor nearly misdiagnosed as advanced cervical cancer: A case report

A 56-year-old woman was referred to our hospital with a pathological diagnosis of squamous cell carcinoma of the cervix. We performed a re-biopsy of the vaginal mass and cervical conization. The mass was originally reported as an epithelioid MPNST after re-biopsy. Strong diffuse S-100 positivity, epithelioid morphology of the lesion, and negativity to all other immune histochemical markers confirmed the diagnosis of epithelioid MPNST. Cervical conization specimen was negative for any neoplasms.     

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.     

De novo malignant peripheral nerve sheath tumor of the breast: case report number one

Summary BACKGROUND: Sarcomas of the breast are rare, representing less than 1% of malignant breast tumours. Malignant peripheral nerve sheath tumour (MPNSTs) is the malignant counterpart to benign soft tissue tumours such as neurofibromas and schwannomas. It is the most common sarcoma arising in the setting of von Recklinghausen's disease. METHODS: We report a de novo malignant peripheral nerve sheath tumour of the breast in 18-year-old patient. To the best of our knowledge, this is the first reported case of sporadic MPNST occurring in the breast in the absence of neurofibromatosis. RESULTS: Immunohistochemical examination is essential to establish the diagnosis and helpful in excluding other lesions in the differential diagnosis. CONCLUSIONS: The surgeon should approach these tumours according to the established guidelines for soft tissue sarcoma surgery.      

Malignant peripheral nerve sheath tumors: The St. Jude Children's Research Hospital experience

Background: Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon in young patients. To contribute to the understanding of these tumors, we reviewed the records of all patients treated for PNSTs at one institution over a 30-year period. Methods: We reviewed the records of eight patients treated for benign PNSTs and 28 patients treated for 29 MPNSTs. We focused on the latter group, statistically testing several clinical factors for their significance in affecting survival. Results: Five-year survival in patients with MPNSTs was 39%. The most significant prognostic factor was gross tumor resectability (p=0.0004). Five-year survival for patients with resectable tumors was 65%, whereas no patient with unresectable disease survived >25 months. Tumor grade, site, and patient race were also significant factors by univariate analysis but were not significant when adjusted for resectability. Conclusion: Gross tumor resection is crucial in treating malignant PNSTs. Supplemental radiation therapy is recommended for positive microscopic margins. More effective treatment is still being sought for unresectable disease. Presented at the 47th Annual Cancer Symposium of The Society of Surgical Oncology, Houston, Texas, March 17–20, 1994.     

Malignant peripheral nerve sheath tumour of median nerve - an unusual presentation: a case report

Malignant peripheral nerve sheath tumours (MPNST) are rare tumours, which usually occur in patients with multiple neurofibromatosis. They are common in the head and neck and trunk and only a few cases have been reported in the hand. Many of these occur in patients with von Recklinghausens disease and rarely as a malignant transformation of benign schwannomas. When they appear in the hand they often present with various symptoms. We encountered a patient who presented with a longstanding asymptomatic swelling of the palm. On excision the histopathology showed a high-grade malignant peripheral nerve sheath tumour, which led us to speculate that a malignant transformation had occurred in a pre-existing benign swelling. This is reported for its unusual presentation, which, to our knowledge, has never been described previously.     

Surgical strategies for managing foraminal nerve sheath tumors: the emerging role of CyberKnife ablation

Sixteen Stanford University Medical Center (SUMC) patients with foraminal nerve sheath tumors had charts reviewed. CyberKnife radiosurgery was innovative in management. Parameters were evaluated for 16 foraminal nerve sheath tumors undergoing surgery, some with CyberKnife. Three neurofibromas had associated neurofibromatosis type 1 (NF1). Eleven patients had one resection; others had CyberKnife after one (two) and two (three) operations. The malignant peripheral nerve sheath tumor (MPNST) had prior field-radiation and adds another case. Approaches included laminotomy and laminectomies with partial (three) or total (two) facetectomies/fusions. Two cases each had supraclavicular, lateral extracavitary, retroperitoneal and Wiltze and costotransversectomy/thoracotomy procedures. Two underwent a laminectomy/partial facetectomy, then CyberKnife. Pre-CyberKnife, one of two others had a laminectomy/partial facetectomy, then total facetectomy/fusion and the other, two supraclavicular approaches. The MPNST had a hemi-laminotomy then laminectomy/total facetectomy/fusion, followed by CyberKnife. Roots were preserved, except in two. Of 11 single-operation-peripheral nerve sheath tumors, the asymptomatic case remained stable, nine (92%) improved and one (9%) worsened. Examinations remained intact in three (27%) and improved in seven (64%). Two having a single operation then CyberKnife had improvement after both. Of two undergoing two operations, one had symptom resolution post-operatively, worsened 4 years post-CyberKnife then has remained unchanged after re-operation. The other such patient improved post-operatively, had no change after re-operation and improved post-CyberKnife. The MPNST had presentation improvement after the first operation, worsened and after the second surgery \and CyberKnife, the patient expired from tumor spread. In conclusion, surgery is beneficial for pain relief and function preservation in foraminal nerve sheath tumors. Open surgery with CyberKnife is an innovation in these tumors’ management.     

恶性外周神经鞘瘤2例报告并文献回顾

目的 提高对恶性外周神经鞘瘤（MPNSTs）的认识,改善患者生存率。方法报告我院2例恶性外周神经鞘瘤的临床表现、辅助检查及治疗情况,并结合文献资料进行回顾分析。结果入院后予行CT、MR、肿瘤活检等辅助方法明确诊断后,行手术治疗及辅助放化疗,或行根治性截肢手术,临床效果满意。结论MPNSTs在诊断和治疗上仍然存在困难,预后不理想。手术切除是最为有效的治疗方法,术后辅助放疗亦能有效降低复发率,化疗的效果尚不确切。     

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.     

Malignant epithelioid peripheral nerve sheath tumor arising in a benign schwannoma

The case of a patient with malignant degeneration of a solitary abdominal schwannoma and endobronchial metastasis is presented. The patient presented clinically with dyspnea referable to her lung mass, anorexia, and night sweats. The lung mass, initially diagnosed as a large-cell undifferentiated carcinoma, was later found to be histologically identical to the malignant portion of the abdominal tumor. The light microscopic, electron microscopic, and immunoperoxidase staining characteristics of the tumor are reported, and previous reports in the literature are reviewed.     

Malignant peripheral nerve sheath tumour of penis

Malignant peripheral nerve sheath tumour (MPNST) is a rare variety of soft tissue sarcoma that originates from Schwann cells or pluripotent cells of neural crest origin. They have historically been difficult tumours to diagnose and treat. Surgery is the mainstay of treatment with a goal to achieve negative margins. Despite aggressive surgery and adjuvant therapy, the prognosis of patients with MPNST remains poor. MPNST arising from penis is a very rare entity; thus, it presents a diagnostic and therapeutic challenge. We present a case of penile MPNST in a 38‐year‐old man in the absence of neurofibromatosis treated with surgery followed by post‐operative radiotherapy to a dose of 60 Gray in 30 fractions and adjuvant chemotherapy with ifosfamide and adriamycin.     

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 tumours of the spine: clinical manifestations,classification, treatment,and prognostic factors

Background and objectives  

To summarise our experience treating patients with spinal malignant peripheral nerve sheath tumours (MPNSTs).