Malignant peripheral nerve sheath tumour is a rare tumour of the breast and this report highlights the radiological and histological features and the difficulties in making a diagnosis. A high index of suspicion when dealing with a breast lesion that has both cystic and solid components in a background of von Recklingausen’s neurofibromatosis will make the diagnosis easie

Akhator, A

Inikori, A

Nwanchokor, FN

Oside, CP

English

Cognitive Sciences ePrint Archive

Case Report:
Malignant peripheral nerve sheath tumour: A rare tumour of the breast
Afeyodion Akhator, Senior lecturer, Dept. of Surgery, Faculty of Clinical Medicine, Delta State University, Abraka,
Oside CP, Dept. of Surgery, Central Hospital, Warri; Assoc. Lecturer, Dept. of Surgery, Faculty Of Clinical Medicine, Delta State 
University, Abraka,
Inikori A, Senior Lecturer, Dept. of Radiology, Faculty of Clinical Medicine, Delta State University, Abraka,
Nwanchokor FN, Dept. of Pathology, Central Hospital, Warri; Assoc. Lecturer, Dept. of Pathology, Faculty of Clinical Medicine, 
Delta State University, Abraka
Address For Correspondence:
Dr. Afeyodion Akhator,
Senior lecturer, Dept. of Surgery, 
Faculty of Clinical Medicine,
Delta State University, Abraka.
E-mail: doc_akhator@yahoo.com
Citation:  Akhator  A,  Oside  CP,  Inikori  A,  Nwanchokor  FN.  Malignant  peripheral  nerve  sheath  tumour:  A  rare  tumour  of  the 
breast. Online J Health Allied Scs. 2010;9(1):9
URL: http://www.ojhas.org/issue33/2010-1-9.htm
Open Access Archives: http://cogprints.org/view/subjects/OJHAS.html and http://openmed.nic.in/view/subjects/ojhas.html
Submitted: Jan 26, 2010; Accepted: Apr 6, 2010; Published: Jul 30, 2010
Abstract:
Malignant peripheral nerve sheath tumour is a rare tumour of 
the breast and this report highlights the radiological and histo-
logical features and the difficulties in making a diagnosis. A 
high index of suspicion when dealing with a breast lesion that 
has both cystic and solid components in a background of von 
Recklingausen’s  neurofibromatosis  will  make  the  diagnosis 
easier.
Key Words: Malignant  peripheral nerve sheath tumour, von 
Recklingausen’s neurofibromatosis, breast 
Introduction:
Primary carcinomas  of  the breast are predominantly  of  epi-
thelial origin, and non epithelial tumours arising in the breast 
are  very  rare.1 Malignant  peripheral  nerve  sheath  tumour 
(MPNST) accounts for 5-10% of all soft tissue sarcomas com-
monly  in  association  with  von  Recklingausen’s  neur-
ofibromatosis (VRN), 4% incidence compared with 0.001% in 
the general population.2 MPNST of the breast (with or without 
VRN) is a rare tumour with only a few cases reported in the 
literature.3
Case Report:
A 41 years  old  teacher  presented to  our  surgical  outpatient 
with a 4 year history of recurrent right breast mass. She had 2 
excisions of the mass done 5years previously, 3 months apart, 
only to have it recur. It has increased rapidly in size to now in-
volve the whole right breast. There was no breast pain, nipple 
discharge or family history of breast disease.
She had developed generalized body nodules 10 years previ-
ously during her first pregnancy. She is a Para 4+3, no use of 
contraceptives; breast fed her children for more than 1 year. 
There were no associated hearing or balance problems.
On examination, there were generalized multiple nodules on 
her skin, café au lait spots and some plexiform nodules. The 
right breast was larger than the left, with an oblique scar; the 
breast was completely taken up by a large multilobulated mass 
with hard and cystic components, measuring 15cm x 20cm, no 
nipple discharge and no palpable axillary lymphnode (Fig 1).
Fig 1: Café au lait spots, plexiform nodules and the mul-
tilobulated hard mass affecting the right breast
Assessment  of  Cystosarcoma  phylloides  in  a  patient  with 
VRN was entertained. Patient was requested to do mammo-
graphy, breast USS and FNAC.
Mammography showed multilobulated masses of varying soft 
tissue  density  and  of  varying  sizes  seen  in  the  entire  right 
breast, the largest measuring 8 x 10cm. Assessment of Giant 
fibroadenoma was made.
1
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Online Journal of Health and Allied Sciences
Peer Reviewed, Open Access, Free Online Journal
Published Quarterly :  Mangalore, South India : ISSN 0972-5997
Volume 9, Issue 1; Jan-Mar 2010
Breast  USS  showed  multiple,  predorminantly  hypoechoic 
masses  with poorly defined margins seen in the entire right 
breast, with marked distortion of the parenchyma of the right 
breast. Some of these lesions show focal anechoic areas con-
sistent  with  necrosis.  Other  show  associated  cystic  lesions 
with posterior acoustic enhancement. The echotexture of the 
underlying  pectoralis  muscle  is  also  distorted.  Features  are 
highly suggestive of a malignant breast mass.
FNAC – Smears  show plump spindle,  oval  and round cells 
with little or no cytoplasm. There are no mitotic figures and 
nuclei  are hyperchromatic.  The cells  form compact  isolated 
sheets. Assessment was of benign spindle cell lesion.
Patient was counseled for mastectomy, at surgery the findings 
were  multilobulated  mass  completely  taken  over  the  right 
breast involving both the pectoralis major and minor muscles. 
A modified radical mastectomy with axillary dissection was 
done.
Histology  of  the  breast  show a malignant  mesenchymal  tu-
mour  composed  of  compact  sheets  of  proliferating  spindle 
cells  with  moderate  eosinophilic  cytoplasm.  The  nuclei  are 
spindle and with blunt ends. Some of the nuclei are round to 
oval in some areas. Some show attempts at rosette formation. 
They  are  arranged  mainly  in  fascicles.  Mitotic  figures  are 
moderate to frequent in numbers. Epitheloid areas are seen in 
some  sections  with  rosette-like  patterns.  Vague  pallisading 
with  cellular  and acellular  areas are seen in some.  There is 
also pleomorphism in some areas.  Assessment  of Malignant 
peripheral nerve sheath tumour (MPNST) in a background of 
Recklinghaunsens disease was made.
Patient was counseled for radiotherapy and chemotherapy, but 
she defaulted and returned 7 months later with recurrent tu-
mour of the right breast, which was excised and she was also 
advised for adjuvant therapy and she has not been able to af-
ford them.
Discussion:
MPNST is a rare sarcomatous tumour, previously known as 
neurofibrosarcoma, neurogenic sarcoma, malignant neurilem-
moma and malignant schwannoma. It is believed to arise from 
a nerve or neurofibroma and usually affects patients who are 
20 – 50 years old but develops earlier in patients with VRN.5
The clinical  diagnosis  of  MPNST is  difficult  because of  its 
rarity. Because of the size and consistency of the tumour a dia-
gnosis  of  cystosarcoma  phylloides  was  entertained initially, 
but the background setting of VRN should have hinted on the 
possibility of MPNST.
The radiological features were also unhelpful in making this 
diagnosis with a discordant between the findings at mammo-
gram and breast ultrasound. Woo et al described the problems 
of radiological diagnosis of MPNST6. The accuracy of cyto-
logy in preoperative diagnosis is uncertain and is difficult to 
distinguish  it  from benign  or  malignant  spindle  tumours4 as 
was in our case. Histological  examination therefore remains 
the most important tool for diagnosis. The presence of spindle 
shaped cells arranged in dense cellular areas with rosette-like 
patterns and pallisading is suggestive of MPNST. Electron mi-
croscopy and immunohistochemistry is necessary for definite 
diagnosis of MPNST,5 However, these are not yet available in 
our facility.
Radical or modified radical mastectomy is the treatment for 
these lesions with post operative radiotherapy helpful in de-
creasing the local recurrence rate.6 Our patient could not  af-
ford  to  go  for  radiotherapy  and  had  recurrence  within  7 
months.
References:
1. Majmudar B. Neurilemoma presenting as a lump in 
the breast. South Med J. 1976;69:463-464
2. Ramanathan RC, Thomas JM. Malignant peripher-
al nerve sheath tumours associated with von Reck-
lingausen’s  neurofibromatosis. Eur  J  Surg 
Oncol 1999;25:190-193
3. Thanapaisal C, Koonmee S, Siritunyaporn S. Ma-
lignant Peripheral Nerve Sheath Tumor of Breast in 
Patient  without  Von  Recklinghausen’s  Neur-
ofibromatosis:  A  case  report. J  Med  Assoc 
Thai 2006;89(3):377-379.
4. Dhingra KK, Mandal S, Somak R, Khurana N. Ma-
lignant peripheral nerve sheath tumor of the breast: 
case  report. World  Journal  of  Surgical  Onco-
logy 2007;5:142. doi 10.1186/1477-7819-5-142.
5. Wanebo JE, Malik JM, VandenBerg SR, Wanebo 
HJ,  Driesen N,  Persing JA.  Malignant  peripheral 
nerve sheath tumors. A clinicopathologic study of 
28 cases. Cancer1993;71:1247-1253
6. Woo OH, Yong HS, Lee JB, Kim A et al. A giant 
malignant  peripheral  nerve  sheath  tumour  of  the 
breast:  CT and pathological  findings. The British 
Journal  of  Radiology2007;80:e44-e47.  doi 
10.1259/bjr/30984808
2


Malignant Peripheral Nerve Sheath Tumor of Breast in Patient without Von Recklinghausen’s Neurofibromatosis: A case report.

Malignant peripheral nerve sheath tumor of the breast: case report.

Malignant peripheral nerve sheath tumors. A clinicopathologic study of 28 cases.

Malignant peripheral nerve sheath tumours associated with von Recklingausen’s neurofibromatosis.

Neurilemoma presenting as a lump in the breast.

Malignant peripheral nerve sheath tumour: A rare tumour of the breast

CogPrints Cognitive Sciences Eprint Archive

Malignant peripheral nerve sheath tumour: A rare tumour of the breast

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