Case Report
Inflammatory Myofibroblastic Tumor of the Stomach: A Rare Case Report in a 10-Year-Old Girl
Mithat Gunaydin1*, A. Gazi Kalayci3, Unal Bicakci2, Burak Tander2, B. Dilek Demirel2, Bilge Can
Meydan4, Meltem Ceyhan5 and Riza Rizalar6
1Department of Pediatric Surgery, Private Avicenna Hospital, Istanbul, Turkey
2Department of Pediatric Surgery, Ondokuz Mayıs University, Faculty of Medicine, Samsun, Turkey
3Department of Pediatric Gastroenterology, Ondokuz Mayıs University, Faculty of Medicine, Samsun, Turkey
4Department of Pathology, Ondokuz Mayıs University, Faculty of Medicine, Samsun, Turkey
5Department of Pediatric Radiology, Ondokuz Mayıs University, Faculty of Medicine, Samsun, Turkey
6Department of Pediatric Surgery, Medipol University, Faculty of Medicine, Istanbul, Turkey
*Corresponding author: Mithat Gunaydin, Department of Pediatric Surgery, Private Avicenna Hospital, Istanbul, Turkey
Published: 08 Dec 2017
Cite this article as: Gunaydin M, Kalayci AG, Bicakci U,
Tander B, Demirel BD, Meydan BC.
Inflammatory Myofibroblastic Tumor of
the Stomach: A Rare Case Report in
a 10-Year-Old Girl. Clin Surg. 2017; 2:
1811.
Abstract
Inflammatory Myofibroblastic Tumor (IMT), also called inflammatory pseudo tumor, is a rare childhood disease that can mimic malignancy. The etiology is not fully understood. It is usually diagnosed with histopathological examination performed after mass excision. In this case report, we present a rare case of inflammatory myofibroblastic tumor of the stomach.
Introduction
Inflammatory myofibroblastic tumor is a rare disease that can occur in many different parts of
the body but most frequently in the lungs [1]. It is more common in children and young adults [2].
Although it can be diagnosed preoperatively via computed tomography and magnetic resonance
imaging, the final diagnosis is usually established by postoperative histopathological evaluation. In
most cases, lesions are well-circumscribed [3,4]. Although it may result from inflammation, whether
it is a true neoplasm is controversial [5]. There are different opinions in the literature regarding
conservative or medical treatment options and resection in the diagnosis and treatment [1,6].
In this paper, we present a rare case of inflammatory myofibroblastic tumor of the stomach
which was histopathologically diagnosed after excision of the mass.
Case Presentation
A-10-year old female patient referred to the hospital with the complaints of weakness and bloody vomiting two days after the use of acetylsalicylic acid due to respiratory tract infections. In laboratory studies, hemoglobin was 3.4 g/dL. The patient underwent erythrocyte replacement. The patient had a history of anorexia and intermittent epigastric pain in the last three months. After patient's vital signs and laboratory values become stable, gastroduoendoscopy was performed for the etiological evaluation purposes. Gastroduoendoscopy revealed a polyploidy mass in the gastric corpus (Figure 1) and biopsy was taken from the lesion. Intense vascular structures, increased inflammation and fibroblastic proliferation were observed on pathological evaluation. Computed tomography showed a polyploidy mass (5.5 cm x 3.9 cm x 3.7 cm) in the greater curvature of the stomach (Figure 2). Laparotomy was decided. On laparotomy, intraluminal mass (6 cm x 4 cm) in the greater curvature of the stomach was palpated. Polypoid mass was reached through gastronomy. Macroscopically well-defined, solitary mass was removed via gastric wedge resection (Figure 3 and 4). Histopathologically fusiform and plasma cells was observed (Figure 5 and 6). It was reported as inflammatory myofibroblastic tumor. The patient was discharged from the hospital on the tenth postoperative day. Radiograph of esophagus, stomach and duodenum was taken on the ninthpostoperative month (Figure 7) and showed no pathological findings. The patient was followed-up via ultrasonography for two years and no recurrence was observed.
Discussion
Inflammatory Myofibroblastic Tumor (IMT) in stomach is extremely rare in childhood.
The most frequent symptoms of gastric masses include abdominal pain, weight loss, anorexia, epigastric burning [2,5,7]. In our case, the patient was admitted to the clinic with the complaint of bloody vomiting and a history of
anorexia and epigastric pain. IMT is a rare tumor characterized by
unsteady proliferation of inflammatory cells. These lesions are also
called as inflammatory pseudo tumor, fibrous xanthoma, plasma
cell granuloma, pseudo sarcoma, lymphoid hamartoma, myxoid
hamartoma, inflammatory myofibrohistiocytic proliferation and
benign myofibroblastoma. Due to its rarity, the pathogenesis and
treatment are still controversial. Main cell is known to be the
myofibroblast in the development of pseudo tumor [1,7,8]. They
are more frequent in children and young adults [2,3]. Although
lung involvement is more common, airway, gastrointestinal tract,
pancreas, tonsil, liver, gall bladder, duodenum, kidney, bladder,
mesentery, spleen and epididymal involvement have also been
reported [1,9-12]. Extra pulmonary involvement of IMT is more
frequent in the first two decades of life [7]. Although the pathogenesis
of IMT is not clear, there are publications asserting that it result
from infections, multifactorial, vascular disorders, immune system
disorders [8,9]. Pulmonary involvement of IMT has been attributed
to pneumonia agents whereas infections such as gram-positive cocci
and E Coli have been considered to be the cause of liver involvement
[12,13]. In the etiology of inflammatory myofibroblastic tumor (IMT)
of the stomach, helicobacter pylori and EBV virus infection were
investigated but no effect was reported [5]. Although inflammatory
myofibroblastic tumor (IMT) is considered as a benign lesion, there
are cases in the literature which display recurrence, local invasion and
distant metastasis [3,10,14]. Considering the literature are IMT of the
stomach has a low risk of metastasis [2,4,14]. In our literature review,
we have encountered limited number of gastrointestinal involvement
in children and our case is the eighteenth pediatric patient presenting
gastric inflammatory tumor. Localization of the lesion varies;
cardia, antrum and pylorus localized cases have been identified.
As in other gastric tumors, the most common causes of admission
to the hospital are weight loss, anemia, epigastric pain, vomiting,
anorexia, and upper gastrointestinal bleeding, as in our patient. The
size of IMT of the stomach varies between 3 cm and 10 cm [5]. In
cases presenting stomach localization of IMT, establishing diagnosis
via preoperative radiological imaging is difficult. Radiological
imaging does not indicate specific diagnostic findings. They usually
appear as well-defined, hard masses. In patients with postoperative histopathological diagnosis, radiological imaging may be useful
to follow-up other organ involvement and recurrence. Pathologic
evaluation is also important in differential diagnosis [13,14]. In our
case, the boundaries of mass lesion were clearly imaged and excision
was performed. The size of the extracted mass was 6 cm x 4 cm. No
other organ involvement was observed on CT examination. Although
conservative follow-up has been considered as an alternative
treatment option due to the presence of spontaneously regressing
cases, in cases presenting recurrence or local invasion, surgical
excision of the mass is preferred. Steroids and chemotherapy options
can be applied in cases presenting recurrence or incomplete resection
[1,8,10]. Because they are nodular and solid masses and they invade
the tissue which they originate from, inflammatory myofibroblastic
tumors are difficult to distinguish from malignancy. Therefore, the
surgical approach may be more radical. Because the recurrence rate is
very low in cases where the whole mass is removed, we can conclude
that the treatment is administered when the pathological diagnosis
is made. In our case, because the macroscopic appearance of the
mass is well-defined, and the whole mass is completely removed and
surgical margins are clean on microscopic examination and there
is no evidence of metastases on radiological evaluation, the patient
did not receive add-on-therapy. In a study evaluating 13 adult cases
with IMT of the stomach, the initial complaint in majority of the
patients was abdominal pain and mass size ranged from 1.5 cm to
10 cm. All patients underwent surgery and additional treatment was
not administered. No pathology was observed during postoperative
follow-up except recurrence in one patient and peritoneal invasion
[7].
While recurrence was not observed after total excision in one
pediatric case with IMT of the stomach, gastric tumor extending
esophagus and meditational mass extending to the left pulmonary
hilar were observed in the second case during four-year followup.
The patient received chemotherapy after surgery but the tumor
spread to the abdomen and right lung. The patient died 11 months
after diagnosis [5].
Figure 1
Figure 1
On gastroduoendoscopy, polypoid mass was detected in the
gastric corpus and biopsied. Examination of the mass revealed intense
vascular structures, increased inflammation and fibroblastic proliferation.
Figure 2
Figure 2
Abdominal computed tomography; mass (5.5 cm x 3.9 cm x 3.7
cm) protruding into the lumen of the stomach in the greater curvature of the
stomach.
Figure 3
Figure 3
On laparotomy; intraluminal polypoid mass (4 cm x 5 cm) in the
greater curvature of the stomach.
Figure 4
Figure 5 and 6
Figure 5 and 6
Histological appearance of the polypoid mass (4x, H&E),
fusiform and plasma cells (40x, H&E).
Figure 7
Conclusion
IMT should also be considered in children. In most cases, complete surgical resection of the mass is sufficient for treatment. However, patients should be followed-up due to the postoperative risk of recurrence, local invasion and distant metastasis.
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