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Chest Imaging and Pathology for Clinicians |

A 40-Year-Old Woman With Back Pain FREE TO VIEW

Andreu Fernández-Codina, MD; Santiago Aranda-Rodríguez, MD; Cleofé Romagosa, PhD; Maria Deu-Martin, PhD; Carmen Parra-Fariñas, MD; Segundo Bujan-Rivas, PhD
Author and Funding Information

aSystemic Autoimmune Diseases Unit, Internal Medicine Department, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain

bEmergency Department, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain

cPathology Department, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain

dThoracic Surgery Department, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain

eRadiology Department, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain

CORRESPONDENCE TO: Andreu Fernández-Codina, MD, Hospital Universitari Vall d’Hebron, Unitat de Malalties Autoinmunes Sistèmiques, Servei de Medicina Interna, 3a planta edifici Hospital General, Passeig de la Vall d’Hebron 119-129, 08035, Barcelona, Spain


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;150(6):e159-e165. doi:10.1016/j.chest.2016.08.1449
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Published online

A 40-year-old woman consulted our ED for a 7-month history of left dorsal back pain and dyspnea. The pain was initially dull and mechanical. Her general practitioner started nonsteroidal antiinflammatory drugs and physiotherapy, which provided partial relief. One week before consulting, the intensity of the pain increased, and she started to feel shortness of breath when performing her daily activities. She had lost 5 kg during the previous month. The patient was a healthy woman who lived in an urban area of Barcelona, Spain. She did not smoke or take drugs of abuse, and she worked as a butcher. During the initial evaluation, her blood pressure was 131/76 mm Hg, heart rate was 120 beats/min, temperature was 36.2°C, and ambient air pulse oximetry was 98%.

Figures in this Article

The physical examination revealed hypophonesis of the entire left hemithorax; the cardiovascular, abdominal, and neurologic explorations were normal. Scoliosis of the lumbar column was present; no other bone abnormalities were noticed. Interestingly, the patient had uncountable freckles (< 1 mm) in the armpits, as well as multiple large brown papules (> 3 cm) along the trunk and the proximal region of her limbs (Fig 1). The patient stated that she had never been questioned about these skin alterations by a physician. No lymph nodes were palpable.

Figure Jump LinkFigure 1 Presence of freckling in the patient’s left armpit. A brown papule is also noticeable.Grahic Jump Location

A posteroanterior conventional chest radiograph showed a large mass occupying almost the entire left hemithorax, with displacement of the mediastinal structures (Fig 2A). A contrast-enhanced thorax CT scan with coronal reconstruction revealed a heterogeneous mass with foci of vascular enhancement and necrosis (Fig 2B) producing a mediastinal shift to the contralateral side. Passive atelectasis of the adjacent lung and a moderate pleural effusion were also noted. These findings correlated with her back pain and dyspnea. Finally, a full-body 18-fluorodeoxyglucose (18-FDG) photon emission tomography (PET)/CT scan was performed. Axial enhanced CT images revealed a huge, well-defined, heterogeneous mass (Fig 3A). The axial PET/CT fusion image focused on the mass showed intense 18-FDG uptake with a maximum standardized uptake value (SUVmax) of 26 (Fig 3B). Small nodules with an SUVmax of 3.7 were also visualized in several intercostal spaces bilaterally (Fig 3C) and in the left thigh. Gadolinium-enhanced MRI of the dorsal and lumbar regions confirmed the presence of these nodules. They corresponded with multifocal thickening of the cervical lumbar nerve roots with gadolinium enhancement (Fig 3D). No thoracic wall invasion was seen.

Figure Jump LinkFigure 2 A, Posteroanterior conventional chest radiograph. There is an image of increased density over the left middle lung field with no infiltration of the adjacent structures and an incomplete border sign that is typical for chest wall tumors. It corresponds to a huge mass occupying almost the entire left hemithorax with contralateral displacement of mediastinal and lung structures. B, Contrast- enhanced chest CT image with coronal reconstruction. A heterogeneous and hypodense soft tissue mass (asterisk) is producing a mediastinal shift to the contralateral side. A moderate amount of loculated pleural effusion and passive atelectasis of the adjacent lung are also noted (arrow).Grahic Jump Location

Figure Jump LinkFigure 3 A, Axial contrast-enhanced CT image with a low attenuating and mildly enhancing mass (asterisk), which is displacing the aorta, azygos vein, and esophagus. B, Axial fused photon emission tomography/CT image with an intensely 18-fluorodeoxyglucose avid mediastinal mass (arrow) with a maximum standardized uptake value of 26. C, Axial fused photon emission tomography/CT image with small nodules (maximum standardized uptake value, 3.7) in the right intercostal spaces, suggestive of neurofibromas (arrow). D, Coronal T2-weighted fat saturation MR image of the lumbosacral roots and plexus. Proximal lumbosacral nerves appear to be thickened by the presence of neurofibromas (arrow).Grahic Jump Location

Results of blood tests showed normal WBC and platelet counts, as well as microcytic anemia (hemoglobin, 5.7 g/dL [normal, 11-15 g/dL]; mean corpuscular volume, 67.8 fL [normal, 84-96 fL]) and increased ferritin levels (167 ng/mL [normal, 25-125 ng/mL]). Renal and liver function, electrolytes, thyroid function, and protein electrophoresis were within normal parameters. Her C-reactive protein level was elevated (25.53 mg/dL [normal, < 0.5 mg/dL]), and prothrombin time was persistently prolonged (17.6 s; international normalized ratio, 1.59). The study of the extrinsic coagulation pathway resulted with factor VIIs’ activity slightly diminished (45.8% [normal, 50%-129%]). No spontaneous macroscopic bleeding was detected. An ophthalmologic examination with a slit lamp microscope revealed multiple hamartomas in both irises. The cranial MRI was normal, as was the transthoracic echocardiography.

Due to the high bleeding risk (ie, vascularized mass, coagulation alteration), a core-needle biopsy (18-G) was performed under CT guidance. However, results of the pathologic study revealed only normal pleural tissue. Finally, a surgical approach (simple removal of the tumor by hemiclamshell thoracotomy) was used. Pleural decortication was performed, with no visual thoracic wall invasion after the procedure. The macroscopic study of the tumor samples showed a 20 × 17 × 12 cm tumor with pleural and muscular infiltration, weighing 2 kg (Fig 4). The tumor had well-defined margins, with a polylobulated shape and necrotic areas. Histologically, the tumor exhibited a transition from low- to high-grade spindle cell proliferation areas. The low-grade areas showed spindle cells, with relatively small hyperchromatic nuclei in a collagenous background (Fig 5A). However, the spindle cells of these areas were focally positive for S100 staining (Fig 5B). The higher grade areas showed necrosis (40%) and clear pleomorphic cells with focal giant cells (Fig 5C and 5D). These areas were negative for S100 staining. In the most cellular areas, 21 mitoses per 10 high-power fields were recorded. The rest of the immunohistochemical analysis showed wide positivity for CD34; results of desmin, smooth muscle actin, acid fibrillary glial protein, BCL-2, STAT6, cytokeratin, and TLE1 staining were negative. The Ki-67 proliferative index was 20%.

Figure Jump LinkFigure 4 Macroscopic image of the tumor once it was removed from the patient’s chest.Grahic Jump Location

Figure Jump LinkFigure 5 A, Tumoral area with spindle cells in a collagenized background at 200×. B, Focal staining for S100 monoclonal antibodies at 200× is noticeable. C and D, Highly cellular area with clear atypia and focal pleomorphism (hematoxylin-eosin; 400×) and an area of tumoral necrosis, respectively (hematoxylin-eosin; 200×).Grahic Jump Location

What is the diagnosis?

Diagnosis: Malignant peripheral nerve sheath tumor within a plexiform neurofibroma and neurofibromatosis type 1.

The initial diagnostic approach to this patient was based on the presence of a mass that occupied the entire hemithorax (Table 1) according to results of the imaging tests. In the patient with a diagnosis of neurofibromatosis type 1 (NF1), clinicians must take into consideration malignant peripheral nerve sheath tumors (MPNSTs).

Table Graphic Jump Location
Table 1 Differential Diagnosis of Complete Opacification of a Hemithorax Associated With Underlying Masses in Patients With Neurofibromatosis Type 1

NF1, also known as von Recklinghausen’s disease, is an inherited genetic disorder caused by mutations in the NF1 tumor suppressor gene located in chromosome 17. The estimated incidence is 1 in 2,600 to 3,000 individuals., Two-thirds of the patients have de novo mutations, with no family history. Heterozygous single-germline mutation carriers may develop nontumoral symptoms starting at birth. The appearance of a second somatic mutation during the growth period is related to tumor development. Diagnosis is based on the 1988 consensus criteria (Table 2). The specter of the disease caused by NF1-related mutations is wide. Multiple manifestations have been described, and they have been reviewed elsewhere extensively. Given that NF1 is an uncommon disease, patients with mild cutaneous changes or with no clinically relevant symptoms may be undiagnosed.

Table Graphic Jump Location
Table 2 National Institutes of Health Consensus Criteria for Diagnosis of NF1

Two or more of the following clinical features are enough to diagnose neurofibromatosis type 1 (NF1).

Clinical Discussion

Although 10% to 15% of the normal population may exhibit up to three café au lait macules, the patient raised clinicians’ suspicions due to the presence of multiple café au lait spots and the probable neurogenic origin of the tumor suggested by the first thorax CT scan. Café au lait macules are present in 95% of subjects with NF1. These macules usually develop in the first 2 years of life. Axillary and inguinal freckling is also common and appears by the age of 5 years. Lisch nodules are melanocytic hamartomas of the iris, with no impact on visual acuity. The diagnosis is made by slit-lamp examination. These nodules commonly develop over the fifth year of life, and they are an almost constant finding.

Patients with NF1 have an increased risk of tumor development. Neurofibromas, the most common type of tumor in these patients, are formed by a mixture of Schwann cells and other cells that conform to the nerve sheath. Nerve sheath tumors present with three subtypes: cutaneous, plexiform, and nodular. The last two subtypes have a higher risk for malignant degeneration. Optic nerve gliomas are frequent at childhood, appearing in 15% of the patients aged < 6 years. In addition to MPNSTs, other sarcomas can develop in patients with NF1 (ie, rhabdomyosarcomas, GI stromal tumors, glomus tumors). Moreover, there is an increased risk for developing nonsarcomatous tumors such as breast cancer, among others.

Other nontumoral manifestations include skeletal malformations (eg, scoliosis, sphenoid wing dysplasia, congenital tibial dysplasia, pseudarthrosis, short height), neurocognitive disorders (eg, cognitive deficits, autism spectrum disorders, learning disabilities), and cardiovascular alterations (eg, congenital malformations in the arterial system and in the heart, early cerebrovascular disease, renal disorders).

Radiologic Discussion

Intrathoracic MPNSTs are uncommon. The largest study to date, describing the imaging characteristics of intrathoracic MPNSTs, included 15 patients. On CT scanning, tumors were hypoattenuating (53%) or isoattenuating (47%) compared with muscle. Sixty-six percent of the masses had mild contrast enhancement, and 26% had moderate enhancement. One-half of the nearby masses were heterogeneous, probably due to the presence of necrotic areas.

MPNSTs may not differ from plexiform neurofibromas on CT scanning. On MRI, MPNSTs are isointense on T1 and hyperintense on T2. Because differentiation between benign and malignant peripheral nerve sheath tumors is difficult, 18-FDG PET/CT scanning has been proposed as a new technique to solve this problem. MPNSTs present as 18-FDG avid masses, with a mean SUVmax of 8.5. Sensitivity and specificity have been estimated at 95% and 72%, respectively, for patients with NF1. This technique has been proposed as an effective method of staging and restaging patients with MPNST versus use of other standard image techniques.

Pathologic Discussion

Usual histologic findings are those mentioned previously. The pathologic differential diagnosis basically included cellular neurofibroma, cellular schwannoma, and other sarcomas. The immunohistochemical profile of the spindle cells did not correspond to the one expected for mesothelioma, and the patient had no history of asbestos exposure. Malignant solitary fibrous tumor of the pleura could be an option because of the location and positivity for CD34 immunostaining, but results of STAT6 staining were negative. Pleomorphism and S100-negative staining in the most cellular areas ruled out the diagnosis of schwannoma. Finally, in the clinical setting of NF1, MPNST should be considered. Moreover, the presence of less cellular and atypical areas corresponding with a plexiform neurofibroma being part of the lesion confirmed the diagnosis. It is also interesting to see the progressive loss of S100 staining, while cellularity and atypia increased, confirming the malignant transition of the lesion. Other sarcomas that can mimic MPNST, such as synovial sarcoma, were easily ruled out because of the absence of keratin and TLE1 expression, along with the clinical context of NF1.

The patient’s surgery was complicated by major intraoperative bleeding (7 L); multiple transfusions of blood, fresh frozen plasma, and fibrinogen were required. The postoperative period occurred without incidence, with significant respiratory improvement. Postsurgical adjuvant therapy included radiotherapy and a chemotherapy regimen consisting of ifosfamide and epirubicin. One month after the surgery, the patient felt well, and results of her blood tests (ie, CBC, coagulation, hepatic and renal function) were within normal ranges. We hypothesize that the alteration of the extrinsic pathway of coagulation was caused by the massive tumor and, subsequently, resolved after the tumorectomy. We found no published literature describing this phenomenon in MPNSTs. NF1 gene sequencing revealed a c.1799del (p.Leu600Cysfs*5) mutation in exon 16. No mutations were identified in the SPRED1 gene. No family members presented with the NF1 phenotype, but they did not give consent to perform genetic studies.

Patients with NF1 can go unnoticed, and clinicians must be aware of this entity. MPNSTs, inside the sphere of sarcomas, originate in degenerated neurofibromas and, on rare occasions, can develop inside the thorax. Diagnosis may be challenging, and multidisciplinary units specialized in sarcomas should be consulted to administer neoadjuvant treatments when possible, followed by surgery and adjuvant treatment. Genetic counseling is mandatory.

Author contributions: All authors contributed equally in the design, interpretation, and writing of the paper.

Financial/nonfinancial disclosures: None declared.

Other contributions:CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met.

Lammert M. .Friedman J.M. .Kluwe L. .Mautner V.F. . Prevalence of neurofibromatosis 1 in German children at elementary school enrollment. Arch Dermatol. 2005;141:71-74 [PubMed]journal. [PubMed]
 
Evans D.G. .Howard E. .Giblin C. .et al Birth incidence and prevalence of tumor-prone syndromes: estimates from a UK family genetic register service. Am J Med Genet A. 2010;152A:327-332 [PubMed]journal. [CrossRef] [PubMed]
 
Gutmann D.H. .McLellan M.D. .Hussain I. .et al Somatic neurofibromatosis type 1 (NF1) inactivation characterizes NF1-associated pilocytic astrocytoma. Genome Res. 2013;23:431-439 [PubMed]journal. [CrossRef] [PubMed]
 
Anonymous  . National Institutes of Health Consensus Development Conference Statement: neurofibromatosis. Bethesda, Md., USA, July 13-15, 1987. Neurofibromatosis. 1988;1:172-178 [PubMed]journal. [PubMed]
 
Hirbe A.C. .Gutmann D.H. . Neurofibromatosis type 1: a multidisciplinary approach to care. Lancet Neurol. 2014;13:834-843 [PubMed]journal. [CrossRef] [PubMed]
 
Friedman J.M. . Neurofibromatosis 1: clinical manifestations and diagnostic criteria. J Child Neurol. 2002;17:548-554 [PubMed]journal. [CrossRef] [PubMed]
 
Lewis R.A. .Riccardi V.M. . Von Recklinghausen neurofibromatosis. Incidence of iris hamartomata. Ophthalmology. 1981;88:348-354 [PubMed]journal. [CrossRef] [PubMed]
 
Seminog O.O. .Goldacre M.J. . Risk of benign tumours of nervous system, and of malignant neoplasms, in people with neurofibromatosis: population-based record-linkage study. Br J Cancer. 2013;108:193-198 [PubMed]journal. [PubMed]
 
Tucker T. .Riccardi V.M. .Sutcliffe M. .et al Different patterns of mast cells distinguish diffuse from encapsulated neurofibromas in patients with neurofibromatosis 1. J Histochem Cytochem. 2011;59:584-590 [PubMed]journal. [CrossRef] [PubMed]
 
Lewis R.A. .Gerson L.P. .Axelson K.A. .Riccardi V.M. .Whitford R.P. . von Recklinghausen neurofibromatosis. II. Incidence of optic gliomata. Ophthalmology. 1984;91:929-935 [PubMed]journal. [CrossRef] [PubMed]
 
Madanikia S.A. .Bergner A. .Ye X. .Blakeley J.O. . Increased risk of breast cancer in women with NF1. Am J Med Genet A. 2012;158A:3056-3060 [PubMed]journal. [CrossRef] [PubMed]
 
Friedman J.M. .Arbiser J. .Epstein J.A. .et al Cardiovascular disease in neurofibromatosis 1: report of the NF1 Cardiovascular Task Force. Genet Med. 2002;4:105-111 [PubMed]journal. [CrossRef] [PubMed]
 
Stucky C.C. .Johnson K.N. .Gray R.J. .et al Malignant peripheral nerve sheath tumors (MPNST): the Mayo Clinic experience. Ann Surg Oncol. 2012;19:878-885 [PubMed]journal. [CrossRef] [PubMed]
 
Kamran S.C. .Shinagare A.B. .Howard S.A. .et al Intrathoracic malignant peripheral nerve sheath tumors: imaging features and implications for management. Radiol Oncol. 2013;47:230-238 [PubMed]journal. [PubMed]
 
Kim J.G. .Sung W.J. .Kim D.H. .Kim Y.H. .Sohn S.K. .Lee K.B. . Malignant peripheral nerve sheath tumor in neurofibromatosis type I: unusual presentation of intraabdominal or intrathoracic mass. Korean J Intern Med. 2005;20:100-104 [PubMed]journal. [CrossRef] [PubMed]
 
Bredella M.A. .Torriani M. .Hornicek F. .et al Value of PET in the assessment of patients with neurofibromatosis type 1. Am J Roentgenol. 2007;189:928-935 [PubMed]journal. [CrossRef]
 
Khiewvan B. .Macapinlac H.A. .Lev D. .et al The value of 1⁸F-FDG PET/CT in the management of malignant peripheral nerve sheath tumors. Eur J Nucl Med Mol Imaging. 2014;41:1756-1766 [PubMed]journal. [CrossRef] [PubMed]
 
Antonescu C.R. .Scheithauer B.W. .Woodruff J.M. . Tumors of the Peripheral Nervous System.  2013;:381-458 [PubMed] American Registry of Pathology Annapolis Junction, MDjournal
 

Figures

Figure Jump LinkFigure 1 Presence of freckling in the patient’s left armpit. A brown papule is also noticeable.Grahic Jump Location
Figure Jump LinkFigure 2 A, Posteroanterior conventional chest radiograph. There is an image of increased density over the left middle lung field with no infiltration of the adjacent structures and an incomplete border sign that is typical for chest wall tumors. It corresponds to a huge mass occupying almost the entire left hemithorax with contralateral displacement of mediastinal and lung structures. B, Contrast- enhanced chest CT image with coronal reconstruction. A heterogeneous and hypodense soft tissue mass (asterisk) is producing a mediastinal shift to the contralateral side. A moderate amount of loculated pleural effusion and passive atelectasis of the adjacent lung are also noted (arrow).Grahic Jump Location
Figure Jump LinkFigure 3 A, Axial contrast-enhanced CT image with a low attenuating and mildly enhancing mass (asterisk), which is displacing the aorta, azygos vein, and esophagus. B, Axial fused photon emission tomography/CT image with an intensely 18-fluorodeoxyglucose avid mediastinal mass (arrow) with a maximum standardized uptake value of 26. C, Axial fused photon emission tomography/CT image with small nodules (maximum standardized uptake value, 3.7) in the right intercostal spaces, suggestive of neurofibromas (arrow). D, Coronal T2-weighted fat saturation MR image of the lumbosacral roots and plexus. Proximal lumbosacral nerves appear to be thickened by the presence of neurofibromas (arrow).Grahic Jump Location
Figure Jump LinkFigure 4 Macroscopic image of the tumor once it was removed from the patient’s chest.Grahic Jump Location
Figure Jump LinkFigure 5 A, Tumoral area with spindle cells in a collagenized background at 200×. B, Focal staining for S100 monoclonal antibodies at 200× is noticeable. C and D, Highly cellular area with clear atypia and focal pleomorphism (hematoxylin-eosin; 400×) and an area of tumoral necrosis, respectively (hematoxylin-eosin; 200×).Grahic Jump Location

Tables

Table Graphic Jump Location
Table 1 Differential Diagnosis of Complete Opacification of a Hemithorax Associated With Underlying Masses in Patients With Neurofibromatosis Type 1
Table Graphic Jump Location
Table 2 National Institutes of Health Consensus Criteria for Diagnosis of NF1

Two or more of the following clinical features are enough to diagnose neurofibromatosis type 1 (NF1).

References

Lammert M. .Friedman J.M. .Kluwe L. .Mautner V.F. . Prevalence of neurofibromatosis 1 in German children at elementary school enrollment. Arch Dermatol. 2005;141:71-74 [PubMed]journal. [PubMed]
 
Evans D.G. .Howard E. .Giblin C. .et al Birth incidence and prevalence of tumor-prone syndromes: estimates from a UK family genetic register service. Am J Med Genet A. 2010;152A:327-332 [PubMed]journal. [CrossRef] [PubMed]
 
Gutmann D.H. .McLellan M.D. .Hussain I. .et al Somatic neurofibromatosis type 1 (NF1) inactivation characterizes NF1-associated pilocytic astrocytoma. Genome Res. 2013;23:431-439 [PubMed]journal. [CrossRef] [PubMed]
 
Anonymous  . National Institutes of Health Consensus Development Conference Statement: neurofibromatosis. Bethesda, Md., USA, July 13-15, 1987. Neurofibromatosis. 1988;1:172-178 [PubMed]journal. [PubMed]
 
Hirbe A.C. .Gutmann D.H. . Neurofibromatosis type 1: a multidisciplinary approach to care. Lancet Neurol. 2014;13:834-843 [PubMed]journal. [CrossRef] [PubMed]
 
Friedman J.M. . Neurofibromatosis 1: clinical manifestations and diagnostic criteria. J Child Neurol. 2002;17:548-554 [PubMed]journal. [CrossRef] [PubMed]
 
Lewis R.A. .Riccardi V.M. . Von Recklinghausen neurofibromatosis. Incidence of iris hamartomata. Ophthalmology. 1981;88:348-354 [PubMed]journal. [CrossRef] [PubMed]
 
Seminog O.O. .Goldacre M.J. . Risk of benign tumours of nervous system, and of malignant neoplasms, in people with neurofibromatosis: population-based record-linkage study. Br J Cancer. 2013;108:193-198 [PubMed]journal. [PubMed]
 
Tucker T. .Riccardi V.M. .Sutcliffe M. .et al Different patterns of mast cells distinguish diffuse from encapsulated neurofibromas in patients with neurofibromatosis 1. J Histochem Cytochem. 2011;59:584-590 [PubMed]journal. [CrossRef] [PubMed]
 
Lewis R.A. .Gerson L.P. .Axelson K.A. .Riccardi V.M. .Whitford R.P. . von Recklinghausen neurofibromatosis. II. Incidence of optic gliomata. Ophthalmology. 1984;91:929-935 [PubMed]journal. [CrossRef] [PubMed]
 
Madanikia S.A. .Bergner A. .Ye X. .Blakeley J.O. . Increased risk of breast cancer in women with NF1. Am J Med Genet A. 2012;158A:3056-3060 [PubMed]journal. [CrossRef] [PubMed]
 
Friedman J.M. .Arbiser J. .Epstein J.A. .et al Cardiovascular disease in neurofibromatosis 1: report of the NF1 Cardiovascular Task Force. Genet Med. 2002;4:105-111 [PubMed]journal. [CrossRef] [PubMed]
 
Stucky C.C. .Johnson K.N. .Gray R.J. .et al Malignant peripheral nerve sheath tumors (MPNST): the Mayo Clinic experience. Ann Surg Oncol. 2012;19:878-885 [PubMed]journal. [CrossRef] [PubMed]
 
Kamran S.C. .Shinagare A.B. .Howard S.A. .et al Intrathoracic malignant peripheral nerve sheath tumors: imaging features and implications for management. Radiol Oncol. 2013;47:230-238 [PubMed]journal. [PubMed]
 
Kim J.G. .Sung W.J. .Kim D.H. .Kim Y.H. .Sohn S.K. .Lee K.B. . Malignant peripheral nerve sheath tumor in neurofibromatosis type I: unusual presentation of intraabdominal or intrathoracic mass. Korean J Intern Med. 2005;20:100-104 [PubMed]journal. [CrossRef] [PubMed]
 
Bredella M.A. .Torriani M. .Hornicek F. .et al Value of PET in the assessment of patients with neurofibromatosis type 1. Am J Roentgenol. 2007;189:928-935 [PubMed]journal. [CrossRef]
 
Khiewvan B. .Macapinlac H.A. .Lev D. .et al The value of 1⁸F-FDG PET/CT in the management of malignant peripheral nerve sheath tumors. Eur J Nucl Med Mol Imaging. 2014;41:1756-1766 [PubMed]journal. [CrossRef] [PubMed]
 
Antonescu C.R. .Scheithauer B.W. .Woodruff J.M. . Tumors of the Peripheral Nervous System.  2013;:381-458 [PubMed] American Registry of Pathology Annapolis Junction, MDjournal
 
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