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CASE REPORT – OPEN ACCESSInternational Journal of Surgery Case Reports 4 (2013) 914– 916

Contents lists available at ScienceDirect

International Journal of Surgery Case Reports

journa l h om epage: www.caserepor ts .com

alignant peripheral nerve sheath tumor of kidney—A case report�

adr Alharbi ∗

epartment of Urology, Prince Sultan Military Medical City, Riyadh, Saudi Arabia

r t i c l e i n f o

rticle history:eceived 6 July 2013eceived in revised form 22 July 2013ccepted 23 July 2013vailable online 3 August 2013

eywords:alignant peripheral nerve sheath tumorPNST

idney tumor

a b s t r a c t

INTRODUCTION: Malignant 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 isolatedMPNST of the kidney is extremely rare and has been reported in only six cases to date.PRESENTATION OF CASE: A 30-year-old female with an unremarkable past medical and surgical historypresented with an enlarging, right-sided abdominal mass for the last 3 months. A CT scan of the abdomenand pelvis showed a large exophytic mass arising from the right kidney. A metastatic work-up wasnegative, so a right radical nephrectomy was performed. Histopathological and immunohistochemicalstudies confirmed the diagnosis of an MPNST.DISCUSSION: MPNSTs are rare and aggressive soft-tissue sarcomas that are usually associated with neu-rofibromatosis 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 excisionremains the mainstay of treatment due to the limited response of MPNSTs to both chemotherapy andradiotherapy.CONCLUSION: MPNSTs arising from the kidney are very rare. Complete surgical resection provides theonly hope for cure. Postoperative chemotherapy and radiotherapy are still controversial, although post-operative radiotherapy is currently recommended by an oncology consensus group.

© 2013 The Author. Published by Elsevier Ltd on behalf of Surgical Associates Ltd. All rights reserved.

. Introduction

Malignant peripheral nerve sheath tumors (MPNSTs) are rareoft-tissue sarcomas that commonly occur in the extremities andre often associated with neurofibromatosis. MPNSTs accounts for–10% of all soft-tissue sarcomas, and up to 50% occur in patientsith neurofibromatosis type 1 (NF-1), 10% are radiation-induced,

nd 40% are sporadic.2 MPNSTs affect adults aged 20–50 yearsnd are typically connected to the main trunks of nerves. MostPNSTs are aggressive and have high rates of recurrence and dis-

ant metastases, with the lungs being the most common site ofPNST metastases. An MPNST of the kidney is an exceedingly rare

ccurrence.4 An aggressive surgical approach, when feasible, is con-idered to be the treatment of choice.

. Case presentation

A 30-year-old female patient not known to have any medicalllnesses presented with a dull, constant pain and an enlarging

� This is an open-access article distributed under the terms of the Creativeommons Attribution License, which permits unrestricted use, distribution andeproduction in any medium, provided the original author and source are credited.∗ Correspondence to: Department of Urology, Prince Sultan Military Medical City,

O Box 7897, Riyadh 11159, Saudi Arabia. Tel.: +966 555181799.E-mail address: dr badr@hotmail.com

210-2612/$ – see front matter © 2013 The Author. Published by Elsevier Ltd on behalf ottp://dx.doi.org/10.1016/j.ijscr.2013.07.013

mass in the right side of her abdomen for the last 3 months. Themass was associated with backache and nausea. Upon examina-tion, a large abdominal mass of firm consistency and occupyingthe upper and lower right quadrants was identified. The vitalsigns were normal, and there was no icterus or pallor. A bloodanalysis showed a hemoglobin (Hb) level of 13.1 g/dL. The totalbilirubin; renal profile; and alkaline phosphatase, serum albumin,serum urea and electrolyte levels were normal. Testing for anti-bodies against hepatitis C virus (anti-HCV) was negative. A CTscan and MRI of the abdomen (Fig. 1) revealed a large, encapsu-lated exophytic mass involving the medial aspect of the kidney,which was displaced inferolaterally. The mass extended beyondGerota’s fascia and caused a mass effect on adjacent organs, dis-placing the liver dome superiorly; the hepatic flexure inferiorly;and the gallbladder, duodenum and pancreas medially. However,there was no definite invasion of adjacent organs. The mass mea-sured approximately 14.5 cm × 19.0 cm and encased the right renalartery. The right renal vein and IVC were severely compressed bythe mass; however, there was no definite invasion. The growth wassurrounded by parasitic vessels but no enlarged regional lymphnodes. The post-contrast images showed gradual enhancement ofthe mass. The liver showed a normal contour and signal intensityand four hemangiomas, with the largest in segment VIII, measuring

4.7 cm × 5.1 cm. There were two simple hepatic cysts in segmentVIII.

A right radical nephrectomy was performed. The operationwent smoothly, with no major blood loss. The post-operative

f Surgical Associates Ltd. All rights reserved.

CASE REPORT – OPEN ACCESSB. Alharbi / International Journal of Surgery Case Reports 4 (2013) 914– 916 915

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ig. 1. CT (A) and MRI (B) showing a large right kidney tumor (T) arising from the mnd four hemangiomas, with the largest (H) in segment VIII, measuring 4.7 cm × 5.1

ecovery was uneventful. Histopathology of the resectedass revealed a right nephrectomy specimen measuring

4.0 cm × 15.0 cm × 10.0 cm and weighing 3.0 kg. The right adrenalland had unremarkable histology. The right kidney was involvednd infiltrated by a neurofibrosarcomatous tumor consistent withn MPNST. High-grade features were observed, including a highitotic rate and necrosis. The tumor invaded the renal capsule

o Gerota’s fascia. Renal pelvis invasion was present. No vascularnvasion was noted. Ureteric, vascular and all surgical resection

argins were free of malignancy. The case was discussed by combined tumor board, and postoperative radiotherapy wasecommended. Six months after the operation, the patient is still

oing well, and a contrast-enhanced CT scan did not reveal anyecurrence of the tumor (Fig. 2).

ig. 2. CT scan follow-up 6 months after surgery, with no recurrence or residualumor and no evidence of distant metastasis. Stable appearance of multiple heman-iomas of the liver (arrow) with no interval development of new hepatic lesions.

aspect of the right kidney. The liver showed a normal contour and signal intensity

3. Discussion

MPNSTs are rare and highly aggressive soft-tissue sarcomasthat are associated with neurofibromas in NF-1 patients but thatalso occur in association with radiation or sporadically. Patientwith NF-1 usually present with multiple rapidly enlarging massesor a new onset of pain associated with pre-existing plexiformneurofibromas, whereas the sporadic forms present with a newsolitary, enlarging and painless mass.2 The extremities are mostcommon sites for MPNSTs, followed by the head and neck regionand the trunk. Kidney involvement is uncommon, and it is verydifficult to differentiate other kidney sarcomas from MPNSTs clin-ically or upon gross examination.5 Thus, histopathological andimmunohistochemical studies are needed for the diagnosis andgrading of an MPNST.3 Grossly, MPNSTs appear fusiform, havea large tumor size (>5 cm) and are gray-tan in color. Micro-scopically, these tumors exhibit features of spindle cells, with afascicular pattern and varying degrees of mitosis, necrosis andtumor calcification. Associated benign neurofibroma or schwan-nanian cells that have not undergone malignant transformationcan be observed. MPNSTs stain positive for S-100 protein; neuron-specific enolase; and other proteins, such as actin, cytokeratin (CK),smooth muscle actin (SMA), desmin and vimentin, to differen-tiate from other spindle cell sarcomas.6 CT and MRI are helpfulimaging techniques for identifying the tumor extent and maysuggest the tumor’s origin. The presence of heterogeneity, necro-sis and hemorrhage in MRI and increased uptake on a positronemission tomography scan may help to detect malignant change.1

Tumor size, local recurrence and the completeness of surgicalresection are the most important prognostic factors for MPNSTs.Complete surgical resection is the treatment of choice for MPN-STs and provides the only hope for cure. MPNSTs are consideredto be chemotherapy- and radiotherapy-resistant tumors. However,postoperative radiotherapy has a definite role in both disease-freeand overall survival and is currently recommended by an oncologyconsensus group.7 Despite multimodal therapy, including aggres-sive surgical resection and adjuvant radiotherapy, the prognosisremains poor.

4. Conclusions

MPNSTs of the kidney are rare malignant tumors. Wide localexcision, if feasible, is considered to be the preferred approach

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CASE REPORT16 B. Alharbi / International Journal o

ecause the prognosis is largely dependent on the completenessf surgical resection. Postoperative radiotherapy is recommended.

onflict of interest

No conflicting interests.

unding

None.

thical approval

Written informed consent was obtained from the patient prioro publication of this case report and the accompanying images. Aopy of the written consent is available for review by the Editor-in-hief of this journal upon request.

uthor contribution

Study design, data collections, data analysis, and writing doney Dr. Badr Alharbi.

7

pen Accesshis article is published Open Access at sciencedirect.com. It is distribermits unrestricted non commercial use, distribution, and reproductredited.

PEN ACCESSery Case Reports 4 (2013) 914– 916

References

. Jankulovski N, Stankov O, Banev S, Petrovski D, Mickovski A, Mitevski A, et al.Isolated malignant peripheral nerve sheath tumor of kidney capsule. Prilozi2008;29(December (2)):361–9.

. Beer TC. Malignant peripheral nerve sheath tumor (MPNST): an overview withemphasis on pathology, imaging and management strategies. Department of Neu-rosurgery Faculty Papers; 2012, paper 18.

. Kar M, Deo S, Shukla N, Malik A, Dattagupta S, Mohanti B, et al. Malig-nant peripheral nerve sheath tumors (MPNST)-clinicopathological study andtreatment outcome of 24 cases. World Journal of Surgical Oncology 2006;4:55–7.

. Khandelwal A, Gupta A, Khandelwal K. Malignant peripheral nerve sheathtumor of kidney. Iranian Journal of Kidney Diseases 2011;5(November (6)):373.

. Gogate BP, Anand M, Deshmukh SD, Purandare SN. Malignant periph-eral nerve sheath tumor of facial nerve: presenting as parotid mass.Journal of Oral and Maxillofacial Pathology 2013;17(January–April (1)):129–31.

. Kar M, Deo SV, Shukla NK, Malik A, DattaGupta S, Mohanti BK, et al. Malignantperipheral nerve sheath tumors (MPNST)-clinicopathological study and treat-ment outcome of twenty-four cases. World Journal of Surgical Oncology 2006;4:55.

. Ferner RE, Gutmann DH. International consensus statement on malignant periph-eral nerve sheath tumors in neurofibromatosis. Cancer Research 2002;62:1573–7[PubMed].

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