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Guangming Wu

Lei Lei

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Totipotency in the mouse

Lin-Bo Zhu

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Wei-Hua Xiao

Peng-Bin Zhang

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Ming-Fei Sun

CASE REPORT Open Access
A distal ileum malignant peripheral nerve
sheath tumor causing intussusception in a
patient in China: a case report
Lin-Bo Zhu1, Peng-Fei Li1*, Wei-Hua Xiao2, Peng-Bin Zhang1, Jun-Qiang Li2 and Ming-Fei Sun1
Abstract
Background: Malignant peripheral nerve sheath tumors (MPNSTs) arise from a peripheral nerve or display nerve
sheath differentiation. Most MPNSTs typically originate on the trunk, extremities, head, neck, and paravertebral
regions. Gastrointestinal MPNSTs are rare entities with only 10 cases reported worldwide in the literatures.
Case presentation: Here, we report the first Chinese case of a malignant peripheral nerve sheath tumor of the
distal ileum presenting as intussusception. A 53-year-old female patient without pathological antecedent for
neurofibromatosis was admitted with pain in the right lower abdomen and multiple episodes of vomiting for
1 week. Preoperative diagnosis was intussusception with a contrast-enhanced computed tomography scan (CECT)
of the abdomen showing characteristic target sign. Due to difficulty reducing the ileum-colon intussusception, right
hemicolectomy with ileocolostomy was performed. Histopathology was suggestive of low-grade MPNST. The
patient received postoperative care and was followed up for 9 months. There is no sign of tumor recurrence and
metastatic disease.
Conclusions: This case is unique in terms of a rare tumor presenting with unusual complication.
Keywords: Malignant peripheral nerve sheath tumor, Small bowel
Background
Malignant peripheral nerve sheath tumors (MPNSTs)
are defined as any tumor arising from a peripheral nerve
or exhibiting nerve sheath differentiation. Most MPNSTs
are located along major nerve trunks, commonly arising
on the body trunk, extremities, head, neck, and paraver-
tebral regions. MPNSTs arising from nerves of the small
intestinal wall are extremely rare, with only 10 cases re-
ported worldwide. In this report, to the best of our
knowledge, we describe the first Chinese case of a malig-
nant peripheral nerve sheath tumor of distal ileum pre-
senting as intussusception in a patient.
Case presentation
A 53-year-old female patient presented to the First Affil-
iated Hospital of Beilun Branch of Zhejiang University
China on September 2015 complaining of pain on the
right side of her abdomen associated with abdominal
distension and multiple episodes of vomiting for 1-week
duration. She denied changes in bowel habits, blood in
stools, and past abdominal operations. Her family his-
tory was insignificant for neurofibromatosis type 1. On
abdominal examination, she exhibited abdominal guard-
ing, but no palpable mass was found. Abdominal CECT
demonstrated intussusception in the ascending colon
with a smooth enhancing 3.5 cm mass suspicious for
neoplasm (Fig. 1). Colonoscopy was deferred due to sig-
nificant exacerbation of pain and worsening peritonitis.
The patient subsequently underwent emergent explora-
tory laparotomy. Due to difficulty reducing the ileocolic
(Fig. 2a), right hemicolectomy with ileocolic anastamosis
was performed (Fig. 2b). Exploration of the abdomen re-
vealed no carcinomatosis. The intraoperative frozen sec-
tion displayed spindle cell tumor of the small bowel.
Although there seemed to be no apparent lymphadenop-
athy in the mesentery, we resected the mesentery to in-
clude the potential lymph node metastases. The resected
* Correspondence: nbblyy1288@163.com
1Department of General Surgery, Beilun District People’s Hospital, The First
Affiliated Hospital, College of Medicine, Beilun Branch of Zhejiang University,
No.1288, Lushan East Road, Ningbo, Zhejiang 315800, China
Full list of author information is available at the end of the article
© The Author(s). 2017 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0
International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and
reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to
the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver
(http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
Zhu et al. World Journal of Surgical Oncology  (2017) 15:29 
DOI 10.1186/s12957-017-1101-z
specimen revealed a 4.0 × 3.5 × 2.3 cm submucosal mass
of the distal ileum (Fig. 2c). Macroscopic examination
demonstrated a gray-white appearance of the tumor
cross section, without hemorrhage and necrosis. Histo-
logically, the tumor was composed of bundles and palis-
ading arrangement of malignant spindle cells that
extended into the muscularis mucosa (Fig. 3a). The
tumor had dense cellularity with significant mitotic ac-
tivity of approximately 10 mitoses per 10 high-power
fields. The surgical margins were negative for the tumor.
On immunohistochemical staining, the tumor was posi-
tive for S-100 (Fig. 3b) and CD34, but negative for
CD117, DOG-1, SMA, AE1/AE3, HMB45, and PNL-2.
The Ki67 labeling index was approximately 15–20%
(Fig. 3c). On the basis of these pathological findings, the
tumor was identified as a low-grade malignant periph-
eral nerve sheath tumor (MPNST).
The patient declined to undergo further genetic
analysis and chemotherapy. She had a good postopera-
tive course and was discharged 2 weeks after surgery.
She presented with diarrhea, fever, and moderate anemia
at 3 and 7 months post-operation. Abdominal CECT
demonstrated no sign of tumor recurrence and meta-
static disease. She underwent symptomatic treatment
and was discharged home. In June 2016, she died due to
complications of her disease.
Discussion
According to the WHO, MPNSTs are defined as any
tumor originating from a peripheral nerve or exhibiting
nerve sheath differentiation. MPNST is the sixth most
common type of soft tissue sarcoma [1, 2]. Approxi-
mately 50% of all MPNST cases arise sporadically,
whereas the other cases are observed in patients with
neurofibromatosis type 1 (NF1) [3, 4], who carry an esti-
mated 8 to 13% lifetime risk of developing MPNST [5].
An estimated 3 to 10% of all MPNST patients have a
clinical history of prior radiation exposure after a latent
period of more than 15 years [3]. MPNST is typically
characterized in adults with most tumors occurring in
patients between 20 and 50 years of age with a median
age of 35 [6].
Most MPNSTs are located along major nerve trunks,
commonly arising on the body trunk, extremities, head,
neck, and paravertebral regions [7]. MPNSTs arising from
nerves of the small intestinal wall are extremely rare, with
only fewer than 10 cases reported worldwide [8–11].
There seems to be no characteristic clinical symptoms
of MPNST of the intestine. Most patients experience
fatigue, weight loss, emesis, abdominal pain, and intes-
tinal bleed [11]. Our patient presented with intestinal
obstruction due to intussusception. The diagnosis is
often delayed because these symptoms are usually non-
specific and vague, thereby increasing the difficulty of a
preoperative diagnosis of MPNST of the small intestine.
The quantitative FDG-PET imaging is used to distin-
guish between benign PNST and MPNST based on a
tumor’s metabolic activity [12, 13]. Due to the disability
to effectively confirm malignant transformation of
lesions, CT and MRI are limited to define the anatomic
Fig. 1 Contrast-enhanced computed tomography scan of the
abdomen showing intussusception in the ascending colon with a
smooth enhancing 3.5 cm mass suspicious for neoplasm (white arrow)
Fig. 2 a Difficulty reduction of ileum-colon intussusceptions on emergent exploratory laparotomy. b A right hemicolectomy specimen. c A 4.0 ×
3.5 × 2.3 cm submucosal tumor of distal ileum (white arrow)
Zhu et al. World Journal of Surgical Oncology  (2017) 15:29 Page 2 of 4
tumor size and local invasiveness of PNST [14, 15]. The
quantitative FDG-PET imaging combined with CT or
MRI may be the best way to distinguish MPNST from
benign PNST. However, radiographic imaging of
MPNST has not supplanted histopathologic examination
as the gold standard for the diagnosis of MPNST [16].
In our case, the CECT of the abdomen revealed an
intussusception in the ascending colon with a smooth
enhancing 3.5 cm mass suspicious for neoplasm, but
could not confirm its definite lesion.
It is important to recognize that there is still a lack of
widely accepted diagnostic criteria for MPNST [16].
These tumors have well-described morphological hetero-
geneity, and staining reveals highly cellular spindle cell
tumor in fascicles [10]. S-100 protein has been the classic
and most widely used antigen for documenting nerve
sheath differentiation. CD34 is expressed in some
MPNSTs and is likely a reflection of perineurial differenti-
ation. Many studies suggest that elevated Ki67 expression
is associated with decreased survival in MPNST [6, 17].
The reactivity of S-100 protein and high levels of p53 and
Ki67 can be useful in making the final diagnosis [11]. In
our case, the diffuse expression of S-100 protein and the
level of Ki67 were in favor of MPNST.
Forty to 65% of MPNST patients experience local re-
currence, and 30 to 60% develop metastases within
12 months of initial surgery [16]. Factors that predict re-
currence include anatomic site, tumor size (≥10 cm),
and adequacy of margins. Factors that predict metastases
include tumor size (≥10 cm) or tumors that are Ameri-
can Joint Committee on Cancer stage III [16]. Over two
thirds of metastases develop in the lung, whereas the
other sites include the liver, brain, bone, and adrenal
gland [16]. To date, there is little knowledge on MPNST
of the small bowel, which is thought to have a far worse
prognosis than other soft tissue sarcomas.
Because of its rarity, the optimal treatment of the
small bowel MPNST is not well established [18].
Current recommendations and treatment may be
based only on what is known of this tumor in other
locations of the body. Complete surgical resection
with wide negative margins is the current standard of
care for localized MPNST and is a strong predictor of
survival [16]. Adjuvant radiation therapy can been
used to locally control MPNST [19]. However, small
bowel MPNST may not benefit from the radiotherapy
due to the location in the abdominal cavity [20]. Al-
though chemotherapy has been carried out on gastro-
intestinal MPNST after surgical treatment, there are
no further trials evaluating the role of chemotherapy
in unresectable and metastatic tumors [11]. Recent
advances in therapy have focused on targeting the
molecular pathways in MPNST, but the outcomes of
recently clinical trials demonstrate that further studies
are needed [16, 21]. The multidisciplinary approach
should be adopted to cope with these tumors.
According to the clinical and pathological characteris-
tics, this patient was diagnosed the MPNST of the small
bowel. Unfortunately, further genetic analysis was not
obtained, due to patient preference as this could have
been helpful for creating further treatment plans. Here,
we report a rare case of MPNST of the distal ileum,
which, to the best of our knowledge, was also the first
Chinese case of this disease. Although MPNST arising
from the small bowel is extremely rare, a surgeon should
also be aware of the possibility of MPNST when dealing
with intestinal obstruction or intussusception. Since
patients with MPNST have very poor prognosis, it is
necessary to perform a quick frozen section in the oper-
ation for a clear diagnosis. We suggest an extended
radical operation in case of the fast frozen section
displaying spindle cell tumors of the small bowel.
Conclusions
To date, little is known regarding MPNST of the small
bowel. We report a rare case MPNST presenting as
ileum-colon intussusception. Multidisciplinary approach
is essential for diagnosis and treatment of these tumors.
There is no definitive guideline available for treatment of
small bowel MPNST. We recommend wide excision of
these tumors with very close postoperative follow-up im-
aging. However, the role of adjuvant radiotherapy and
chemotherapy is still under debate reserved only for
positive margins and recurrent tumors or when wide
local excision is unfeasible.
Fig. 3 a The tumor showing bundles and fascicles of atypical spindle cells with hyperchromatic nuclei and indistinct cytoplasm (HE, ×200).
b S-100 staining showing diffuse positive reaction. c IHC staining for Ki67-labeling index 15~20%
Zhu et al. World Journal of Surgical Oncology  (2017) 15:29 Page 3 of 4
Acknowledgements
The author thanks Dr. Chen Hu, who has been my sources of encouragement
and inspiration. The authors would like to thank Clarity Manuscript Consultant
LLC (Indianapolis) for their language editing assistance.
Funding
No funding source.
Availability of data and materials
The data and material are freely available upon requests.
Please contact Dr. Lin-Bo Zhu (nbblyy1288@163.com) for further information,
who is responsible for the dataset.
Authors’ contributions
L-BZ drafted this manuscript. W-HX, P-BZ, J-QL, and M-FS were involved in
the acquisition of data and preparing the figures. P-FL conceived of the
study and revised the manuscript. All authors read and approved the final
manuscript.
Competing interests
The authors declare that they have no competing interests.
Consent for publication
The consent for publication of the individual patient data from the patient
has been obtained by The First Affiliated Hospital, College of Medicine,
Beilun Branch of Zhejiang University.
Ethics approval and consent to participate
The Ethics Committee of the First Affiliated Hospital, College of Medicine,
Beilun Branch of Zhejiang University approved this study. The analysis was
performed in accordance with the ethical standards of the hospital and the
tenets of the Declaration of Helsinki/Declaration of Istanbul. The patient
reported in this study provided written consent.
Author details
1Department of General Surgery, Beilun District People’s Hospital, The First
Affiliated Hospital, College of Medicine, Beilun Branch of Zhejiang University,
No.1288, Lushan East Road, Ningbo, Zhejiang 315800, China. 2Department of
Pathology, Beilun District People’s Hospital, The First Affiliated Hospital,
College of Medicine, Beilun Branch of Zhejiang University, Ningbo, Zhejiang
315800, China.
Received: 24 November 2016 Accepted: 12 January 2017
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Zhu et al. World Journal of Surgical Oncology  (2017) 15:29 Page 4 of 4


A distal ileum malignant peripheral nerve sheath tumor causing intussusception in a patient in China: a case report

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