DOI No.: 10.21176/ ojolhns.0974-5262.2016.10.

A CASE OF MALIGNANT PERIPHERAL NERVE SHEATH TUMOR

IN PARAPHARYNGEAL SPACE
*Souvagini Acharya, **Utkal P Mishra, ***Debasish Jena
Date of receipt of article -23-2-2016
Date of acceptance -2-5-2016
DOI-10.21176/ojolhns.2016.10.1.13
ABSTRACT
Malignant peripheral nerve sheath tumors (MPNSTs) are spindle-cell sarcomas which are aggressive tumors
accounting for 510% of all soft tissue sarcomas. It is locally invasive, frequently leading to multiple recurrences
and eventual metastatic spread. In human body, the trunk and extremities are the most commonly involved sites,
with only 8-14% of all lesions appearing in the head and neck region. Diagnosis is difficult in most cases. Wide
surgical excision followed by Radiotherapy is the treatment of choice.We present a case of malignant peripheral
nerve sheath tumor involving the left parapharynx in a 25-year-old patient who complained of swelling & dysphagia
that aggravated over a month. After surgery, tumor was finally diagnosed as malignant peripheral nerve sheath
tumor by histopathologic examinations.
Key words:- Malignant peripheral nerve sheath tumor, Parapharyngeal, Neurofibrosarcoma, Malignant
schwannoma, Malignant neurilemmoma.
INTRODUCTION

CASE REPORT

Malignant peripheral nerve sheath tumour

(MPNST) is the term coined by the World Health
Organization and corresponds to the malignant
proliferation of any cell of the nerve sheath: Schawnn
cell, perineural fibroblast or endoneural fibroblast.1,5
MPNSTs account for 510 % of all soft-tissue sarcomas
and are one of the most common nonrhabdomyosarcoma
soft-tissue
sarcomas. 3
Approximately 50 % of MPNSTs are diagnosed in
patients with NF1; the lifetime risk of MPNST in NF1
is 813 % as compared to 0.001 % in the general
population.3 The histological features of MPNSTs are
those of a highly cellular, spindle-cell neoplasm
resembling a soft-tissue sarcoma, but with
differentiation toward elements of the nerve sheath,
Schwann cell, and perineural cell.4 Fifty to seventy
percent of MPNST are S-100 immunopositive.1,5 Highgrade MPNSTs metastasize widely and have a poor
prognosis, but low-grade tumors that are diagnosed
early and treated appropriately are compatible with
longterm survival.2 Complete surgical resection is the
only curative treatment for sporadic and NF1-associated
MPNSTs. The surgical goal is to resect the MPNST
with wide negative margins. If complete surgical
resection is not achieved, postoperative radiation
therapy has a role in improving local control.1

A 25 yr old man presented with a 7 month history

of gradually enlarging swelling over left side of neck &
dysphagia to solids that aggravated over a month. On
physical examination a non tender, firm to hard, fixed,
bossolated swelling measuring 12 7 cm over left side
of neck extending from infra-auricular region down to
lower border of cricoid cartillage vertically, from
posterior border of sternomastoid to midline
horizontaly which has displaced the thyroid cartilage
to opposite side.

No neck node enlargement was there. Intraoraly

the mass was extending upto nasopharynx with no
other mass palpable in the body.
Affiliations:
*Associate Professor,**,*** P G Students
Dept of ENT and Head and Neck Surgery
VIMSAR,Burla,Sambalpur,Odisha,India
Address of Correspondence:
Dr Utkal Priyadarsi Mishra
P G Students Dept of ENT and Head and Neck Surgery,
VSS Institute of Medical Science And Research, Burla,
Sambalpur, Odisha, India
Email: drutkal.mishr@gmail.com
Mob: 9439755093

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Vol.-10, Issue-I, Jan-June - 2016

No neck node enlargement was there. Intraoraly

the mass was extending upto nasopharynx with no
other mass palpable in the body.

DOI No.: 10.21176/ ojolhns.0974-5262.2016.10.1

sympathetic chain. The cut surface of excised mass was

mostly yellowish without hemorrhage or necrosis.

Fig.1: preoperative photograph

Laryngealendoscopy revealed normal vocal cord
movements. X-ray neck suggestive of shifting of trachea
towards right. Routine blood investigations were within
normal limit. FNAC revealed benign parapharyngeal
neurofibroma CT scan revealed a large mass in
parapharyngeal space displacing carotid sheath
extending from nasopharynx upto hypopharynx
without any bone erosion.

Fig.4: the mass after removal

Microscopic examination showed alternating hypo
and hypercellular areas in spindle- or polyhedral-shaped
cells. There was a moderate degree of cellular
pleomorphism and mitoses were found frequently.
Immunohistochemistry showed a strong positive
reaction for S-100. The pathologic diagnosis was
malignant peripheral nerve sheath tumor arising from
cervical sympathetic chain.Postoperatively patient
recovered well & discharged after 7 days. Radiotherapy
was administered as an adjuvant therapy, with 6000 cGy
given to the parapharyngeal space.
The patient was grossly free of symptoms 5 months
after surgery.
DISCUSSION

Vol.-10, Issue-I, Jan-June - 2016

Fig.2:per operative photograph of the mass

Fig.3: Immediate post operative photograph

Wide excision of mass was performed by
transcervical approach with mandibular swing. At
surgery, the mass appeared pinkish white in color
attached tightly to nasopharynx, hypopharynx and
carotid sheath. Site of origin was from cervical

62

Tumors of the parapharyngeal space are relatively

uncommon, representing only 0.5% of all head and

Fig.5: Microscopic features showing alternating hypo and

hypercellular areas in spindle- or polyhedral-shaped cells.
neck tumors. [6] About half the parapharyngeal tumors
are nerve sheath tumors, and another 30% are of salivary
gland origin.7 Malignant peripheral nerve sheath tumors
are rare, highly aggressive tumors capable of arising de
novo or from pre-existing benign neurofibromas or
schwannomas. The peak incidence of disease is known

DOI No.: 10.21176/ ojolhns.0974-5262.2016.10.1

2.

Ferner RE. Neurofibromatosis 1. Ferner RE,

Huson SM, Evans DGR. Neurofibromatosis in
Clinical Practice. 1st ed. Springer; 2011. pp. 19-23.
3. Hooly M, Brigitte C, Ratner N, editors.
Malignant Peripheral Nerve Sheath Tumours:
Prognostic and Diagnostic Markers and
Therapeutic Targets. In: Upaddhyaya M, Cooper
DN. Neurofibromatosis Type 1. 1st ed. Springer;
2012. pp. 445-467.
4. Chitale AR, Dickersin GR. Electron microscopy
in the diagnosis of malignant schwannomas. A
report of six cases. Cancer. 1983;51:144861.
[PubMed]
5. M.S. Kenali, P.G. Bridger, M. Baldwin, R.
Smee.Malignant peripheral nerve sheath tumour
the tongue. Aust N Z J Surg, 69 (3) (1999), pp.
243246.
6. Barnes L, Dekker M. Surgical pathology of the
head and neck. Tumours of the Head and
Neck.2nd ed. Madison Avenue Inc; New York;
2001. p. 836-41.
7. Lee H, Lee C, Jin S, Lee S :A Case of Malignant
Peripheral Nerve Sheath Tumor in
Parapharyngeal
Spacehttp:
//synapse.
koreamed.org / DOIx.php ? doi10.3342/kjorlhns.
2012.55.3.181&vmode =PUBREADER#! po=
25.0000.
8. Wanebo JE, Malik JM, VandenBerg SR, Wanebo
JH, Driesen N, Persing JA: Malignant peripheral
nerve sheath tumors. A clinicopathologic study
of 28 cases.Cancer 1993, 71:1247-1253.
9. Kar M, Deo S, Shukla N, Malik A, Gupta S,
Mohanti B, Thulka S : Malignant peripheral nerve
sheath tumors (MPNST) Clinicopathological
study and treatment outcome of twenty-four cases.
10. Stout AP: Tumors of peripheral nervous system:
Atlas of tumor pathology, Sect. 2, fasc. 6.
Washington, D.C, Armed forces Institute of
pathology; 1949.
11. Ferner RE, Gutmann DH: International
consensus statement on malignant peripheral nerve
sheath tumors in neurofibromatosis.Cancer Res
2002, 62:1573-1577.
12. Basso-Ricci S: Therapy of malignant
schwannomas: usefulness of an integrated
radiologic. surgical therapy.J NeurosurgSci 1989,
33:253-257.
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to occur sporadically between the age of 20s and 50s,

and is usually associated with the neurofibromatosis
type I. About 40-50% of MPNSTs are associated with
a family history of neurofibromatosis-1.7
Microscopically the tumour consists of spindle
cells with a high mitotic rate and indistinct cytoplasmic
borders arranged in bundles or fascicles. The diagnosis
of MPNST is difficult and elusive in the soft tissue
disease due to lack of standardized criteria. It is not
always possible to demonstrate the origin from a nerve,
especially when it arises from a small peripheral branch.
Immuno-histochemistry plays an important part in the
diagnosis, with tumor cells showing specific positivity
for S-100.1
Radical surgical resection is the treatment of choice
in MPNST. A good three-dimensional clearance is
mandatory for a successful outcome. Routine nodal
dissection is not indicated. However, when a major
nerve is identified, the cut end should be sent for frozen
section to assess the tumor free margin of the
resection.Although MPNSTs are generally considered
chemotherapy and radiotherapy resistant tumors.9
Currently, postoperative radiotherapy is recommended
by oncology consensus group as part of a uniform
treatment policy for MPNSTs, much like other high
grade soft tissue sarcomas [8][10], despite having clear
surgical margins. 11
Basso-Ricci demonstrated 56% disease free survival
using combined surgery and radiation therapy for
MPNST.12
CONCLUSION
MPNST is a highly aggressive tumour, which
could be difficult to treat, despite substantial progress
in treatment modalities available in present era. The
wide spreading nature of this tumour has a strong hold
in determining the prognosis. Early detection of this
aggressive tumour may help reduce morbidity.
DISCLOSURES
(a) Competing interests/Interests of Conflict- None
(b) Sponsorships - None
(c) Funding - None
(d) No financial disclosures
REFERENCES
1. F.M. Enzinger, S.W. Weiss. Malignant tumors of
the peripheral nerves. F.M. Enzinger, S.W. Weiss
(Eds.), Soft tissues tumors, Mosby-Year Book, St.
Louis (1995), pp. 889928