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Case Report

Isolated Nerve Sheath Tumor Of Jejunum Presenting As Acute Abdomen

Mohanty J1, Prasad S2
Abstract 
Nerve   sheath tumors of   gastrointestinal  tract  are  rare and occurs more frequently as systemic manifes-
tations of generalized neurofibromatosis type-1(NF-1).In neurofibromatosis-1 lesions may be scattered or
solitary tumor. Occasionally ,such tumors may be the initial presenting feature of NF-1.Rarely ,cases of iso-
lated nerve sheath tumor of small intestine presenting with bleeding and obstruction have been document-
ed. We report a 19years male presenting with nerve sheath tumor of jejunum in acute abdomen. His inves-
tigations show no features of NF-1.

Keywords: nerve sheath tumor, neurofibromatosis, isolated, intestinal.

Introduction
Nerve sheath tumors of small intestine may manifest
in various forms and accordingly given different
descriptive terms including intestinal neurofibro-
matosis, ganglioneuromatosis, neuronal intestinal
dysplasia and diffuse plexiform neurofibromatosis.
These lesions may be seen in 25% cases of neurofi-
bromatosis-1 and multiple endocrine neoplasia type
2b.There occurrence without NF-1 and MEN-2b are
very rare1. No consensus has been made yet whether
these isolated lesions represent different phenotypic
manifestations of the neurocutaneous or multiple
endocrine neoplasia syndromes or whether they rep-
resent separate and distinct entities. 

Case Report
A 19  years  old  male  presented to us with  acute
abdomen   of   1  day   history  with  symptoms  of
pain  around  umbilicus  and  2  episodes   of  vom-
iting.  On  examination  he  had  tachycardia    and
tenderness  over   umbilical  and  hypogastrium
regions  with  bp-90/60mm  of  Hg. He had  rigidity
and rebound tenderness more marked in lower
abdomen. USG  shows a  oval  well  defined  hypo
echoic  solid  lesion  seen  in  lower  abdomen
infraumblical  region  to  the  left  of  midline. CECT
shows  a  isodense   inhomogeneously   enhancing
soft  tissue   exophytic   tumor   of  size  5.8*4.2*4.3

arising  from  small  bowel  likely  to  represent  gas-
trointestinal  stromal   tumor. There  is  no  lym-
phadenopathy  with  moderate  amount  of  free  fluid
in  the  pelvis. Initially  we  proceed  with  laparo-
scopic   exploration  .But  due  to dense  adhesions
we   converted  it  into  formal   laparotomy  with
lower  midline  incisions. After  entering  into
abdominal  cavity  a  tumor  arising   from  jejunum
approximately  7  feet distal  to  D-J  junction  with
dense  adhesions  with  surrounding  bowels was
seen .[Figure-1] 

After  clearing  all  adhesions    carefully  segmental
resection  of  bowel  having  tumor  carried  out  with

1. Dr. Jignyasu Mohanty, Associate   Professor, Room No- 307,   Mgm Medical Staff Quarters, Mgm
Medical College, Sector 18 Kamothe, Navi Mumbai, Raigad District, Maharashtra, India, E-mail:
Drjignyasu@rediffmail.com

2. Dr. Sujit Prasad, Consultant General Surgeon, Mgm Hospital, Sector 7, Vashi, Navi Mumbai,
Maharashtra, India, E-mail:prasadsujitin@yahoo.com 

Corressponds to: Dr.   Jignyasu Mohanty, Associate   Professor, Room No- 307,   Mgm Medical Staff
Quarters, Mgm Medical College, Sector 18 Kamothe, Navi Mumbai, Raigad District, Maharashtra, India,
E-mail: Drjignyasu@rediffmail.com

Bangladesh Journal of Medical Science Vol. 12 No. 01 January’13

91

Figure I Intra  operative photograph showing tumor
arising from jejunum



end  to  end  anastomosis. [Figure-II] The  postoper-
ative orally  allowded  on  4th  postoperative  day
and recovery was uneventful .Biopsy  sent  to 3  dif-
ferent  labs.  Histopathology  from  all  the  3  labs
reported   uniform  epithelioid   morphology with
spindle  component  .  These  cells  are  very  strong-

ly  positive  for  S100[Figure-3]  and  negative  for
C-kit,  CD34,  SMA,  Calponin,  HMB45,  Melan A.
There   is no  mitoses  or  necrosis.  The   patient is
followed  for    8months  without  any  complication. 

Discussion
Isolated nerve sheath tumors are benign neural
lesions of intestinal tract which may be initial pre-
senting feature of NF-1 and MEN tepe2b. Affected
patient may present with altered bowel habit2,
abdominal pain3, intestinal obstruction4 or with pal-
pable abdominal mass. In setting of NF-1 associated
clinical findings including dermal neurofibromas,
café-au-lait spots and lisch nodules may be seen. In
MEN type 2b thickened lips, marfanoid habitus and
medullary carcinoma of thyroid may be  seen.
Isolated nerve sheath tumors commonly involve
colon , terminal ileum , and appendix5.  Surgical
excision is the treatment of choice for these lesions
These cases  need to be followed up closely to
exclude NF-1 and MEN type2b in future.

References
1. Hochberg FH, Dasilva AB, Galdabini J,

Richardson EP Jr. Gastrointestinal involvement
in von Recklinghausen's neurofibromatosis.
Neurology. 1974;24:1144-1151 http://dx.doi.
org/10.1212/WNL.24.12.1144 PMid:4374669

2. Kim HR, Kim YJ. Neurofibromatosis of the
colon and rectum combined with other manifes-
tations of von Recklinghausen's disease: report
of a case. Dis Colon Rectum. 

3. Bononi M, De Cesare A, Stella MC, Fiori E,
Galati G, Atella F, Angelini M, Cimitan A,
Lemos A, Cangemi V. Isolated intestinal neu-
rofibromatosis of colon. Single case report and

review of the literature. Dig Liver Dis.
2000;32:737-742. http://dx.
doi.org/10.1016/S1590-8658(00)80340-0

4. Urschel JD, Berendt RC, Anselmo JE. Surgical
treatment of colonic ganglioneuromatosis in
neurofibromatosis. Can J Surg. 1991;34:271-
276 PMid:1905194

5. Shekitka KM, Sobin LH. Ganglioneuromas of
the gastrointestinal tract. Relation to Von
Recklinghausen disease and other multiple
tumor syndromes. Am J Surg
Pathol.1994;18:250-257 http://dx.doi.
org/10.1097/00000478-199403000-00004
PMid:7906923

Isolated Nerve Sheath Tumor Of Jejunum Presenting As Acute Abdomen

92

Figure II Immunohistochemistry showing strong s-
100 reaction 


