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![A 31-year-old man with mediastinal malignant peripheral nerve sheath tumor. A. Axial contrast-enhanced CT (CECT) shows a low attenuating mildly enhancing posterior mediastinal mass (arrow) displacing the aorta, azygous vein and esophagus. The mass encompasses more than 50% circumference of the descending thoracic aorta. B. Coronal T2-weighted MR image shows a heterogeneous lobulated hyperintense mass (arrow). C. Coronal maximum intensity projection (MIP) PET image shows a moderately fluorodeoxyglucose (FDG) avid mediastinal mass (arrow, standardized uptake value [SUV]max 5.2). Physiologic activity is noted in the heart (arrowhead).](profile/Nikhil-Ramaiya/publication/257889896/figure/fig1/AS:203216080904200@1425461897728/A-31-year-old-man-with-mediastinal-malignant-peripheral-nerve-sheath-tumor-A-Axial.png)
A 31-year-old man with mediastinal malignant peripheral nerve sheath tumor. A. Axial contrast-enhanced CT (CECT) shows a low attenuating mildly enhancing posterior mediastinal mass (arrow) displacing the aorta, azygous vein and esophagus. The mass encompasses more than 50% circumference of the descending thoracic aorta. B. Coronal T2-weighted MR image shows a heterogeneous lobulated hyperintense mass (arrow). C. Coronal maximum intensity projection (MIP) PET image shows a moderately fluorodeoxyglucose (FDG) avid mediastinal mass (arrow, standardized uptake value [SUV]max 5.2). Physiologic activity is noted in the heart (arrowhead).
Source publication
The aim of the study was to analyze the clinical and imaging characteristics of primary intrathoracic malignant peripheral nerve sheath tumors (MPNSTs).
In this institutional review board (IRB)-approved retrospective study, clinical and imaging features of 15 patients (eight men; mean age 50 years [range 18-83)] with pathologically proven malignant...
Similar publications
A malignant peripheral nerve sheath tumor (MPNST) is defined as any malignant tumor that develops or differentiates from cells in the peripheral nerve sheath. This tumor is commonly associated with neurofibromatosis type 1 (NF1) and previous radiotherapy treatment. Primary intraosseous MPNSTs are extremely rare and a case of the lumbar spine in a p...
Background
Neurofibromatosis Type 1 (NF-1) and previous irradiation are two common risk factors that can result in malignant peripheral nerve sheath tumors (MPNSTs), extremely rare soft-tissue sarcomas. Here, a 63-year-old male with NF-1 presented with diffuse spinal metastases from a subcutaneous MPNST.
Case Description
A 63-year-old male with NF...
Gender-based differences in disease onset in murine models of malignant peripheral nerve sheath tumor (MPNST) and in patients with Neurofibromatosis type-1-(NF-1)-associated or spontaneous MPNST has not been well studied.
Forty-three mGFAP-Cre+;Pten
loxp/+;LSL-K-ras
G12D/+ mice were observed for tumor development and evaluated for gender disparit...
Schwannomas are benign nerve sheath tumors commonly found in the head, neck, vestibular system, and extremities. Primary hepatic schwannomas are exceptionally rare, with 34 cases reported to date according to our review of the literature. This case report describes a 79-year-old man with a medical history of skin and thyroid cancer, who presented w...
Citations
... Due to its rarity, most experience published in the literature refers to single case reports or small patient series until now. [13][14][15][16][17][18] Therefore, the aim of the present study was to systematically examine imaging features of MTT in the available literature and validate these findings in a cohort from a tertiary care centre. ...
Objectives
Malignant triton tumours (MTTs) are rare but aggressive subtypes of malignant peripheral nerve sheath tumours (MPNSTs) with a high recurrence rate and 5-year survival of 14%. Systematic imaging data on MTTs are scarce and mainly based on single case reports. Therefore, we aimed to identify typical CT and MRI features to improve early diagnosis rates of this uncommon entity.
Methods
A systematic review on literature published until December 2022 on imaging characteristics of MTTs was performed. Based on that, we conducted a retrospective, monocentric analysis of patients with histopathologically proven MTTs from our department. Explorative data analysis was performed.
Results
Initially, 29 studies on 34 patients (31.42 ± 22.6 years, 12 female) were evaluated: Literature described primary MTTs as huge, lobulated tumours (108 ± 99.3 mm) with central necrosis (56% [19/34]), low T1w (81% [17/21]), high T2w signal (90% [19/21]) and inhomogeneous enhancement on MRI (54% [7/13]). Analysis of 16 patients (48.9 ± 13.8 years; 9 female) from our institution revealed comparable results: primary MTTs showed large, lobulated masses (118 mm ± 64.9) with necrotic areas (92% [11/12]). MRI revealed low T1w (100% [7/7]), high T2w signal (100% [7/7]) and inhomogeneous enhancement (86% [6/7]). Local recurrences and soft-tissue metastases mimicked these features, while nonsoft-tissue metastases appeared unspecific.
Conclusions
MTTs show characteristic features on CT and MRI. However, these do not allow a reliable differentiation between MTTs and other MPNSTs based on imaging alone. Therefore, additional histopathological analysis is required.
Advances in knowledge
This largest published systematic analysis on MTT imaging revealed typical but unspecific imaging features that do not allow a reliable, imaging-based differentiation between MTTs and other MPNSTs. Hence, additional histopathological analysis remains essential.
... Spinal MPNST may also invade the vertebrae and cause bony erosion [11]. MPNSTs have poor outcomes, with low progression-free survival (PFS) and overall survival (OS) [6,12]. Outcomes are worst in craniospinal axis MPNST, with a five-year OS as low as 25%, and PFS ranging from 5 to 32.2 months [6,9,13]. ...
... Survival in MPNST is very poor, with 5-year survival rates as low as 25% owing to increased local recurrence and metastasis rates [6,12]. Local recurrence rates reported range from 31 to 75%, with a median PFS of 5 to 32.2 months [6,9,13] and distal metastasis rates of 22% to 45% [9,13]. ...
Background
Malignant peripheral nerve sheath tumor (MPNST) is an exceedingly rare and aggressive tumor, with limited literature on its management. Herein, we present our series of surgically managed craniospinal MPNSTs, analyze their outcomes, and review the literature.
Methods
We retrospectively reviewed surgically managed primary craniospinal MPNSTs treated at our institution between January 2005 and May 2023. Patient demographics, tumor features, and treatment outcomes were assessed. Neurological function was quantified using the Frankel grade and Karnofsky performance scores. Descriptive statistics, rank-sum tests, and Kaplan–Meier survival analyses were performed.
Results
Eight patients satisfied the inclusion criteria (4 male, 4 female). The median age at presentation was 38 years (range 15–67). Most tumors were localized to the spine (75%), and 3 patients had neurofibromatosis type 1. The most common presenting symptoms were paresthesia (50%) and visual changes (13%). The median tumor size was 3 cm, and most tumors were oval-shaped (50%) with well-defined borders (75%). Six tumors were high grade (75%), and gross total resection was achieved in 5 patients, with subtotal resection in the remaining 3 patients. Postoperative radiotherapy and chemotherapy were performed in 6 (75%) and 4 (50%) cases, respectively. Local recurrence occurred in 5 (63%) cases, and distant metastases occurred in 2 (25%). The median overall survival was 26.7 months. Five (63%) patients died due to recurrence.
Conclusions
Primary craniospinal MPNSTs are rare and have an aggressive clinical course. Early diagnosis and treatment are essential for managing these tumors. In this single-center study with a small cohort, maximal resection, low-grade pathology, young age (< 30), and adjuvant radiotherapy were associated with improved survival.
... MPNSTs have poor outcomes, with low progression-free survival (PFS) and overall survival (OS) [6,10]. The ve-year OS is as low as 25%, and the PFS ranges from 5 to 32.2 months [6, 9,11]. ...
Background
Malignant peripheral nerve sheath tumor (MPNST) is an exceedingly rare and aggressive tumor, with limited literature on its management. Herein, we present our series of surgically managed craniospinal MPNSTs, analyze their outcomes, and review the literature.
Methods
We retrospectively reviewed surgically managed primary craniospinal MPNSTs treated at our institution between January 2005 and May 2023. Patient demographics, tumor features, and treatment outcomes were assessed. Neurological function was quantified using the Frankel grade and Karnofsky performance scores. Descriptive statistics, rank-sum tests, and Kaplan-Meier survival analyses were performed.
Results
Eight patients satisfied the inclusion criteria (4 male, 4 female). The median age at presentation was 38 years (range 15–67). Most tumors were localized to the spine (75%), and 3 patients had neurofibromatosis type 1. The most common presenting symptoms were paresthesia (50%) and visual changes (13%). The median tumor size was 3 cm, and most tumors were oval-shaped (50%) with well-defined borders (75%). Six tumors were high grade (75%), and gross total resection was achieved in 5 patients, with subtotal resection in the remaining 3 patients. Postoperative radiotherapy and chemotherapy were performed in 6 (75%) and 4 (50%) cases, respectively. Local recurrence occurred in 5 (63%) cases, and distant metastases occurred in 2 (25%). The median overall survival was 26.7 months. Five (63%) patients died due to recurrence.
Conclusions
Primary craniospinal MPNSTs are rare and have an aggressive clinical course. Early diagnosis and treatment are essential for managing these tumors. Maximal resection, low-grade pathology, young age (< 30), and adjuvant radiotherapy were associated with improved survival.
... Although, it is plausible that some CT imaging features are masked or obscured in the presence of an abundant blood supply whereby the mass of the MTT increases rapidly and disproportionally within a short time frame or in the presence of Schwann cell metaplasia of neural crest during a state high-malignancy [20]. At times, hemorrhage, necrosis and cystic morphological changes are observed in the lesions with a distinct high-density calcification shadow [21,22]. Importantly, contrast-enhanced CT scan can mildly enhance the peripheral features of the mass, allowing the specificity of MTT grading based on the heterogeneity of the mass and the linear or annular shape of the atrial septal shadow. ...
Background
Malignant ‘triton’ tumor is an extremely rare subtype of malignant periphery nerve sheath tumors. Clinical diagnosis of malignant triton tumor is difficult before surgery due to its low incidence and the lack of knowledge. Therefore, to describe and summarize the CT imaging characteristics of malignant triton tumor is of great assistance for early and preoperative diagnosis.
Case presentation
Two cases suspected of MTT by CT scan before operation were closely observed. The diagnosis of malignant triton tumor was eventually confirmed by immunochemical assay, which verified speculation of CT scans. Huge, irregular, well-circumscribed lobulated mass-like shadows can be observed from these patients by CT scans. Besides, heterogeneity of density within the body of tumor was well-established by CT scans, together with linear septum. Meanwhile, CT scans demonstrated that calcifications were remarkable at the margin of tumor body.
Conclusions
Some CT image features from two cases were presented as a reference for the preoperative consideration of MTT: (i) enormity of mass-like shadow; (ii) presence of well-circumscribed lobulated shape; (iii) septum within the well-defined mass accompanied with hemorrhage, necrosis and cystic changes as well as calcification, especially within neurofibromatosis type 1 patients.
... Radiological differentiation of benign and malignant nerve sheath tumors is difficult. A rapid increase in size, presence of high attenuation and necrosis or hemorrhage on CT, and heterogeneity on magnetic resonance imaging point towards a malignant tumor [7]. Positron emission tomography (PET) scans may be useful in differentiating a malignant tumor from a benign neurofibroma. ...
Neurofibromatosis type 1 (NF-1) is a genetic disorder associated with dermatological, musculoskeletal, and neurological features. Apart from these, knowledge of other uncommon manifestations, including intrathoracic and pulmonary involvement, is crucial for early diagnosis and treatment. These patients are predisposed to various sarcomatous and non-sarcomatous malignancies. We report the case of an elderly lady with NF-1 who presented with pleural effusion related to the genetic disorder, which was missed, and elaborate on the diagnostic workup done to reach a diagnosis.
... Asymptomatic presentation also has been documented. 9 MPNSTs usually arise from pre-existing plexiform neurofibromas or perineuriomas, and exhibit a 20-fold increased risk with the former. MPNSTs can arise de novo from normal nerves as well. ...
... Patients with nonradical resection receive adjuvant chemotherapy. 9 This patient's tumour was not completely resectable and she underwent debulking surgery followed by chemotherapy. Neoadjuvant chemotherapy/chemoradiation was not considered in this patient as there was no convincing evidence for malignant transformation before surgery. ...
Neurofibromatosis is a genetic disorder withmultisystem involvement. Malignant peripheral nerve sheath tumours (MPNST) are one of the soft tissue sarcomas associated with neurofibromatosis type1(NF-1). These tumours are relatively rare and mostly occur in proximal portions of the upper and lower extremities and the trunk. MPNST of the thoracic cavity has rarely been reported. This case report describes a woman with NF-1 who presented with progressive dyspnoea and was found to have a mass in the thoracic cavity. She underwent partial resection and was found to have MPNST. Intra-thoracic MPNST have a bad prognosis often needing neoadjuvant chemotherapy or chemoradiation pre/post resection.
... Whole-body MRI and PET CT help assess tumour burden of internal neurofibromas which may not be apparent on general physical examination. FDG-PET also helps distinguish high-grade MPNST from benign plexiform neurofibroma [11]. Malignant lesions have maximum standardised uptake values (SUVmax) ≥3.5, whereas benign lesions have SUV<2.5 [12]. ...
... MPNSTs are highly aggressive tumours with high local recurrence rate (40-65%) and early metastasis (40-68%), 5 and 10-year Case Report survival rates being 34-60% and 22-45%, respectively [2,11]. Truncal location is associated with poor prognosis which can be explained by the difficulty in obtaining R0 resection and subsequent locoregional recurrence [5]. ...
Malignant peripheral nerve sheath tumours (MPNSTs) are rare soft tissue tumors that arise from pre-existing plexiform neurofibromas or within a normal peripheral nerve. They are aggressive tumors with high rates of recurrence and distant metastases, the most common sites of metastasis being the lung followed by bone.
A 46 year old gentleman presented with breathlessness and chest pain three years after post amputation of left thumb for an ulcerative growth. CECT thorax showed a left upper lobe mass with pleural and pericardial effusion. Within a month of presentation he worsened and succumbed to the disease. Antemortem biopsy of the left hand ulcerative growth showed features suggestive of malignant peripheral nerve sheath tumour- epithelioid variant and post mortem liver and lung biopsy showed metastasis of MPNST. The diagnosis was a malignant peripheral nerve sheath tumor with lung, liver and cardiac metastasis.
This case report aims to highlight the importance of upfront aggressive multimodality local therapy for achieving local disease control in patients presenting with localised MPNST and regular follow up for early detection of relapse and metastasis.
... We present a rare case of malignant nerve sheath tumor (MNST) of pleura referred for the They tend to relapse locally and spread homogeneously. Prognosis is worse with trunk involvement (3,4), most likely due to failure of achieving a margin free status following surgery (4,5). Herein we report a 56-year-old woman with a previous history of colon cancer who presented for PET/CT evaluation of a mass in the right pleural cavity. ...
We present a rare case of malignant nerve sheath tumor of pleura referred for the evaluation of metastases and local invasion. FDG PET/CT demonstrated a hypermetabolic tumoral lesion extensively involving the right pleura with no involvement of mediastinal structures or pulmonary parenchyma and no clear evidence of distant metastasis. Malignant nerve sheath tumor of pleura is an extremely rare entity, and FDG PET/CT is valuable in demonstrating the extent of disease and can have potential role for postsurgical as well as postchemotherapy assessment of possible residual disease.
... MPNST is a tumor of neural origin which can occur in the mediastinum and which arises most commonly in patients with neurofibromas and neurofibromatosis type 1 (NF1) (5,9). The tumors occur most often within the posterior mediastinum in association with nerves located within that compartment, although rare cases have been described in the anterior mediastinum (84)(85)(86)(87). These lesions may arise from malignant transformation of benign neural tumors, but may also occur sporadically (88,89). ...
Mediastinal sarcomas represent rare neoplasms of mesenchymal origin. Most published data on mediastinal sarcomas is primarily derived from small series and case reports. Although rare, primary mediastinal sarcomas have a clinically aggressive course with worse 10-year survival rates than other types of mediastinal tumors, highlighting the importance of adequate diagnosis of these lesions. The diagnosis of mediastinal sarcomas is complicated by the varied histologic subtypes of tumors that can occur and which can sometimes display overlapping clinical, morphological, imaging, and immunohistochemical features. Cytogenetic analysis and more recently, molecular techniques, have provided new methods by which these tumors can be differentiated. Sarcomas occurring within the mediastinum are an extremely heterogenous group of tumors, although the specific incidence of the different subtypes of mediastinal sarcomas varies among studies, there is a subset of lesions that appear to occur more commonly across most published studies. These tumors include synovial sarcoma, liposarcoma, malignant peripheral nerve sheath tumor (MPNST), small round blue cell sarcomas (including Ewing sarcoma) and leiomyosarcoma (LMS). Other rare sarcoma subtypes may also less commonly occur within the mediastinum. Many of these sarcomas have specific, recurrent genetic abnormalities that can be identified through cytogenetic and molecular testing allowing for accurate diagnosis. This review aims to cover the role of molecular pathology, specifically with regards to diagnosis, as well as discuss the salient molecular genetic features of the various types of sarcoma that occur within the mediastinum. In addition, the various types of cytogenetic and molecular diagnostic tests available for the diagnosis of different types of sarcomas will be reviewed.
... Our patient, a 33-year-old and non-smoker woman, complained of pain on her right chest. Pain is the most common symptom [5]. MPNST is often associated with NF1 with varying incidence rate of 20-30% [5]. ...
... Pain is the most common symptom [5]. MPNST is often associated with NF1 with varying incidence rate of 20-30% [5]. The contrary was found in our patient. ...
... The contrary was found in our patient. MPNST is usually large, ovoid, with white margin with necrotic and haemorrhagic area [4,5]. MPNST 4,5]. ...
Pulmonary malignant peripheral nerve sheath tumours (MPNSTs) are extremely rare soft tissue sarcomas that develop from the cell constituting the nerve sheaths, approximately 5–10% of all soft tissue sarcomas. We present a rare case of primary lung MPNST in an adult female non‐smoker patient, in whom surgical thoracotomy approach has obtained a good control of the disease. Low‐grade MPNST was established from excisional biopsy followed by immunohistochemistry. We present a rare case of primary lung malignant peripheral nerve sheath tumours (MPNSTs) in an adult female non‐smoker patient, in whom surgical thoracotomy approach has obtained a good control of the disease. Low‐grade MPNST was established from excisional biopsy followed by immunohistochemistry.
