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Year : 2021  |  Volume : 64  |  Issue : 1  |  Page : 158-160
A rare case of malignant peripheral nerve sheath tumor of pleura and review of literature

1 Department of Medical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Delhi, India
2 Department of Pathology, Rajiv Gandhi Cancer Institute and Research Centre, Delhi, India
3 Department of Radiology, Rajiv Gandhi Cancer Institute and Research Centre, Delhi, India

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Date of Submission03-Dec-2019
Date of Decision01-Jan-2020
Date of Acceptance24-Feb-2020
Date of Web Publication8-Jan-2021


Malignant peripheral nerve sheath tumor (MPNST) is a type of soft tissue sarcoma arising from peripheral nerves. They can be found in isolation, but about half of the cases are associated with neurofibromatosis type 1 (NF1), an autosomal dominant genetic disorder. They usually arise from the nerve plexus in extremities and trunk. MPNSTs arising from the viscera or internal organs are very rare. We hereby report a rare case of sporadic MPNST arising from pleural surface in a middle-aged male. The diagnosis of such a rare entity involved multimodal investigations with consideration of wide differential diagnosis.

Keywords: Malignant Peripheral Nerve Sheath Tumor, malignant peripheral nerve sheath tumor, pleural sarcoma

How to cite this article:
Amrith B P, Pasricha S, Jajodia A, Babu Koyyala VP, Batra U. A rare case of malignant peripheral nerve sheath tumor of pleura and review of literature. Indian J Pathol Microbiol 2021;64:158-60

How to cite this URL:
Amrith B P, Pasricha S, Jajodia A, Babu Koyyala VP, Batra U. A rare case of malignant peripheral nerve sheath tumor of pleura and review of literature. Indian J Pathol Microbiol [serial online] 2021 [cited 2022 Sep 30];64:158-60. Available from:

   Introduction Top

Malignant peripheral nerve sheath tumor (MPNST), previously called as neurofibrosarcoma or malignant schwannoma, is a type of soft tissue sarcoma arising from peripheral nerves. These are the tumors which originate from the connective tissue of a peripheral nerve with the exception of those arising from epineurium or nerve vasculature. MPNSTs are rarely seen in general population, with an incidence rate of about 0.001%. They can be found in isolation, but about half of the cases are associated with neurofibromatosis type 1 (NF1), an autosomal dominant genetic disorder. Nerve plexus in the extremities and trunk are the usual site of origin, but it has been rarely reported from any viscera or internal organs. We report a rare case of sporadic MPNST arising from pleura in a middle-aged male with a review of literature.

   Case History Top

A 45-year-old male presented with history of left-sided chest pain, progressive dyspnea, and weight loss for 2 months. The patient was a smoker without any significant past or personal medical history. On examination, the patient had reduced breath sounds on the left side. Rest of the general and systemic examination was unremarkable.

On initial evaluation, the chest X-ray showed left mid and lower zone homogenous opacity. This was further evaluated with CT chest [Figure 1]a, which showed a large well-defined, diaphragmatic pleural based, enhancing mass lesion of size 7 × 7 cm with moderate pleural effusion causing a complete collapse of the left lower lobe. PET CT-whole body showed FDG avidity in the pleural based mass lesion without any other FDG avid lesions in the body [Figure 1]c. Thoracoscopic [Figure 1]b biopsy from the mass lesion showed a highly cellular spindle cell tumor exhibiting marked nuclear atypia with brisk mitotic activity (<20/10 hpf) [Figure 2]. Features were suggestive of high-grade malignant spindle cell tumor and in view of clinicopathological features, the differentials considered were: synovial sarcoma, leiomyosracoma, sarcomatoid mesothelioma, malignant solitary fibrous tumor (MSFT), MPNST, and dedifferentiated liposarcoma (DLPS).
Figure 1: Imaging studies of the patient. (a) CT chest showing a large well defined diaphragmatic pleural based enhancing mass lesion with moderate pleural effusion. (b) Thoracoscopic image showed a nodular mass in the diaphragmatic surface of the pleura. (c)18F-FDG PET-CT image (axial CT and fused axial) showing metabolically active multiple left pleural based lesions and left anterior diaphragmatic lymph node

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Figure 2: Micrographs of histopathology and IHC of the tumor tissue. (a) Diffuse proliferation of neoplastic spindle cells with interspersed blood vessels (H and E, ×100). (b) Neoplastic spindle cells show marked nuclear atypia with brisk mitosis (H and E, ×400). (c) Neoplastic spindle cells are heterogeneously (focally) positive for S100 (DAB; ×200). (d) Neoplastic spindle cells show diffuse nuclear positivity for SOX 10 (DAB; ×200)

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On immunohistochemistry (IHC) [Figure 2], the tumor cells were diffusely positive for CD 99, heterogeneously positive for S100 while focally positive for SMA and TLE-1 and were negative for CK, EMA, CD34, STAT6, SMMH, WT1, desmin, CDK4, MDM2, Calretinin, and Melan A. Subsequently, SOX 10 and CD56 was performed which were positive, hence, provided a tangible evidence for neural differentiation/histogenesis. The overall histopathological and IHC features were suggestive of MPNST (FNCLCC grade III).

The patient was further evaluated by thoracic surgeons, radiation oncologists and plan of management was discussed in multidisciplinary tumor board meeting. Since R0 resection of the tumor was not feasible upfront, the patient was treated with ifosfamide and adriamycin based chemotherapy. There was a partial response to chemotherapy after three cycles and he was continued on the same regimen as the tumor was still unresectable.

   Discussion Top

MPNSTs usually arise in relation to nerve roots and plexus of head and neck, trunk, or extremities like brachial plexus, sacral plexus, and sciatic nerve. These tumors probably arise from the sheets of intercostals nerves. A growing mass, pain, and neurologic deficit are the usual mode of presentation. In a retrospective analysis of 175 patients of MPNSTs, published by mayo clinic, 99% of the tumors were arising from extremities, trunk, and head/neck (45%, 34%, and 19%, respectively).[1] Rarely MPNSTs can also arise from nerve plexus in the mediastinum, breast, prostate, omentum, and pleural cavity. MPNST arising from a pleural surface is very rare and our literature review was limited to two case reports till date.[2],[3] A literature review of such rare sites of MPNSTs is presented in [Table 1].[4],[5],[6],[7],[8],[9],[10]
Table 1: Review of rare site of MPNST

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Although usually MPNSTs can be suspected by imaging (CT/MRI), histopathological examination is the mainstay for definitive diagnosis. However, differentiating a pleural MPNST from that of an intercostals nerve is difficult. Imaging can contribute in early-stage disease as the intercostals nerve MPNST shows elongated growth along the nerve course. Diligent histomorphological assessment and judicious IHC markers panel helps in establishing an accurate diagnosis due to distinct therapeutic implications in terms of selecting the chemotherapy. In the presented case, negative immunoexpression of CK, EMA, and TLE-1 ruled out synovial sarcoma. CDK4 and MDM2 negativity ruled out DPLS while malignant SFT and sarcomatoid mesothelioma were ruled out with negative immunoexpression for CD34/STAT6 and Calretinin/WT1, respectively. There is no single IHC marker with optimal sensitivity and specificity currently available for definitive diagnosis of MPNST. Hence, it is the clinicoradiological and histopathological correlation which leads to the final diagnosis, ruling out competing diagnostic possibilities. S100 is the most commonly used IHC marker, which is usually diffusely positive in grade 1 MPNST; however, the expression is focally positive/negative in grade III MPNST. A diffusely positive S100 can be seen in epitheloid variant of high-grade MPNST, which will be INI-1 deficient on IHC.[11] SOX 10, CD57, and CD56 have been documented as markers of neural differentiation in the prevailing literature, which helped in the presented case for establishing diagnosis of MPNST. Focal and weak expression of the TLE1 has been described in MPNST.

The standard of care for a localized high-grade MPNST is wide excision and radiotherapy. The role of systemic chemotherapy is limited to unresectable or metastatic MPNSTs. Owing to its rarity, the management of pleural MPNSTs needs to be individualized. A multidisciplinary tumor board meeting consisting of medical oncologists, thoracic surgeon, radiation oncologists, and radiologist is of immense of help in taking a judgmental call for planning treatment of such cases with rare sites of involvement for which the standard treatment guidelines are not established owing to a paucity of the literature.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Stucky CCH, Johnson KN, Gray RJ, Pockaj BA, Ocal IT, Rose PS, et al. Malignant Peripheral Nerve Sheath Tumors (MPNST): The Mayo clinic experience. Ann Surg Oncol 2012;19:878-85.  Back to cited text no. 1
Misiak P, Wcislo S, Jabłoński S, Szwalbe K. Primary malignant peripheral nerve sheath tumor of the pleural cavity: Rapid progression. Kardiochirurgia Torakochirurgia Pol Pol J Cardio-Thorac Surg 2014;11:213-5.  Back to cited text no. 2
Ghosh A. Multidisciplinary management of giant malignant endo-thoracic nerve sheath tumor. Eur J Cardiothorac Surg 2003;24:165-7.  Back to cited text no. 3
Seno N, Fukushima T, Gomi D, Kobayashi T, Sekiguchi N, Matsushita H, et al. Successful treatment with doxorubicin and ifosfamide for mediastinal malignant peripheral nerve sheath tumor with loss of H3K27me3 expression. Thorac Cancer 2017;8:720-3.  Back to cited text no. 4
Kalra B, Kingsley PA, Bedi HS, Kwatra KS, Negi P. Malignant peripheral nerve sheath tumor of the anterior mediastinum: A rare presentation. Rare Tumors 2014;6:140-2.  Back to cited text no. 5
Shuayb M, Begum R. Unusual primary breast cancer – malignant peripheral nerve sheath tumor: A case report and review of the literature. J Med Case Reports 2017;11:161.  Back to cited text no. 6
Bonnet SE, Kang-Chapman JK, Buckley KA, Cui X, Grignol VP, Hawley JR. Malignant peripheral nerve sheath tumor of the breast in a patient with neurofibromatosis 1. Breast J 2018;24:1066-8.  Back to cited text no. 7
Senapati S, Mishra S, Dhir M, Das S. Malignant peripheral nerve sheath tumor in spine: Two case reports. South Asian J Cancer 2013;2:141.  Back to cited text no. 8
[PUBMED]  [Full text]  
Kim H, Kim DY, Seol YM, Ku JY, Choi KU, Choi YJ. Primary malignant peripheral nerve sheath tumor of prostate in a young adult: A case report. Medicine (Baltimore) 2018;97:39.  Back to cited text no. 9
Miguchi M, Takakura Y, Egi H, Hinoi T, Adachi T, Kawaguchi Y, et al. Malignant peripheral nerve sheath tumor arising from the greater omentum: Case report. World J Surg Oncol 2011;9:33.  Back to cited text no. 10
Laskin WB, Weiss SW, Bratthauer GL. Epithelioid variant of malignant peripheral nerve sheath tumor (malignant epithelioid schwannoma). Am J Surg Pathol 1991;15:1136-45.  Back to cited text no. 11

Correspondence Address:
Ullas Batra
Department of Medical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_948_19

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