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Gastric inflammatory myofibroblastic tumor in a young child – A case report and literature review


1 Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
2 Department of Gastroenterology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
3 Department of Pediatric Surgery, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India

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Date of Submission05-Jul-2021
Date of Decision23-Nov-2021
Date of Acceptance05-Dec-2021
Date of Web Publication26-May-2022
 


How to cite this URL:
Panigrahi C, Shahin M, Nayak HK, Padhi S, Tripathy BB, Patra S. Gastric inflammatory myofibroblastic tumor in a young child – A case report and literature review. Indian J Pathol Microbiol [Epub ahead of print] [cited 2023 Jan 29]. Available from: https://www.ijpmonline.org/preprintarticle.asp?id=345892




Dear Editor,

Inflammatory myofibroblastic tumor (IMT) (so-called inflammatory pseudotumor), is a rare mesenchymal neoplasm of uncertain malignant potential characterized morphologically by proliferating spindle cells with myofibrobalstic differentiation admixed with a prominent inflammatory component giving rise to secondary constitutional symptoms. ALK (2p23) and ROS1gene rearrangement are reported to occur in nearly 60% and less than 5% of IMTs, respectively.[1] We describe a 7-year-old girl who presented to the pediatric outpatient department of our institute with 1-month history of insidious onset, gradually progressive, and painless epigastric swelling. Abdominal contrast-enhanced computed tomography scan revealed a well-circumscribed (9 cm × 7.2 cm × 7 cm) lobulated heterogeneously enhancing mass with areas of internal calcification and necrosis arising primarily from the lesser curvature and body of stomach extending both anteriorly and posteriorly, along the gastrohepatic ligament and abutting the left margin of the liver [Figure 1]a. Upper gastrointestinal endoscopy revealed extrinsic bulge seen in the gastric body and incisura along the lesser curvature with mucosal ulceration [Figure 1]b. Her base line routine investigation revealed a microcytic hypochromic anemia (Hb; 91 g/L, ref.; 120–140 g/L), neutrophilic leukocytosis (leukocyte count; 13 × 109/L, ref.; 4–11 × 109/L), and thrombocytosis (Plt; 545 × 109/L, ref.; 150–450 × 109/L). Her liver and renal function tests were within normal reference limits.
Figure 1: (a) Contrast-enhanced computerized tomogram scan of abdomen showing a heterodense mass located along the gastrohepatic ligament infiltrating gastric body and abutting the left margin of the liver (black arrow head). (b) Esophagogastroduodenoscopy showing a large extrinsic bulge in the gastric body and incisura with mucosal ulceration (black arrow head). (c) Peroperative photograph showing a lobulated mass arising from the lesser curvature of stomach. (d) Gross examination of the excised lesion showing an encapsulated, multilobulated, grayish white mass with myxoid cut surface

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Endoscopic mucosal biopsy from the mass was suggestive of a benign spindle cell lesion. Gross evaluation of laparoscopic resected specimen showed a large, firm, well-encapsulated, and multilobulated grayish white mass measuring 6.0 cm × 6.0 cm × 4.5 cm [Figure 1]c and [Figure 1]d. Microscopically, the lesion comprised of both hypocellular (collagenized) and cellular areas displaying whorls and fascicles of spindle cells with elongated plump vesicular nuclei and scant eosinophilic cytoplasm displacing gastric glands and admixed with dense infiltrates of plasma cells, few lymphocytes, and foci of calcification. The lesion was seen infiltrating the overlying mucosa with surface ulceration [Figure 2]a, [Figure 2]b, [Figure 2]c. On immunohistochemistry (IHC), the spindle cells were positive for smooth muscle actin [Figure 2]d and pan cytokeratin (focal) [Figure 2]e and negative for ALK-1, CD117, CD68, and S-100. Her serum IgG4 level was 2.59 g/L (ref.; 0.03–2 gm/L) and tissue IHC demonstrated less than 10% positive plasma cells. The morphology and IHC characteristics were more in favor of IMT than inflammatory fibroid polyp and GIST. The molecular workup for ALK1 and ROS1 gene rearrangement were not done due to financial issues. The child had an uneventful postoperative recovery, and there was no evidence of recurrence of the disease at 10 months follow-up.
Figure 2: Hematoxylin and eosin-stained tissue sections from the lesion demonstrating cellular areas comprising of plump spindle-shaped cells in fascicles admixed with abundant chronic inflammatory cells (a, ×200; inset, ×400) and hypocellular (collagenized) areas showing focal calcification (b, ×400). Note the surface mucosal ulceration with splaying of glands by spindle cells (yellow asterisk) (c, ×200) in this case. The spindle cells showed strong diffuse cytoplasmic positivity for smooth muscle actin (SMA) (d, ×200) and focal positivity for pancytokeratin (PanCK) (e, ×400) (Peroxidase-antiperoxidase)

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There have been 21 reported cases of pediatric gastric IMT that occurred in 13 females and 8 males with median age of 5 years (range: 1–18) [presented in [Table 1]]. The lesions ranged from 0.5 to 12 cm in size (median: 8 cm) with a solid homogeneous appearance along with the areas of calcification. Microscopically, the lesions showed variable cellularity with proliferating fibroblastic and myofibroblastic cells with prominent lymphoplasmahstiocytic infiltrate and areas of collagenous to myxoid stroma, calcification, and foci of ossification. IHC performed on 12/21 cases confirmed the myofibroblastic nature of lesional cells; ALK1 performed in only 4/21 cases (19%) were found to be negative; and clonal cytogenetic abnormalities from tumor cells revealed multiple chromosomal abnormality in one case.[2]
Table 1: Clinicopathological characteristics of gastric inflammatory myofibroblastic tumor in pediatric population (1985.2021) (n=21)

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Recent studies have shown that ALK1 IHC may be used as a useful diagnostic supplement, even though its role is controversial. More than half of spindle cell IMTs are reported to express diffuse cytoplasmic ALK1 expression, whereas epithelioid IMTs show a nuclear membrane and perinuclear pattern, secondary to RANBP2-ALK gene fusion. ALK1 negative IMTs have a higher risk of metastases compared to ALK positive group,[10] though available data from pediatric gastric IMTs have been more in favor of a benign biological behavior amenable to complete surgical resection. Therefore, larger organ specific prospective case series with long-term follow-up is necessary for a better understanding of disease biology.

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Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Yamamoto H, Yoshida A, Taguchi K, Kohashi K, Hatanaka Y, Yamashita A, et al. ALK, ROS1 and NTRK3 gene rearrangements in inflammatory myofibroblastic tumors. Histopathology 2016;69:72-83.  Back to cited text no. 1
    
2.
Lazure T, Ferlicot S, Gauthier F, Doz F, Couturier J, Fabre M, et al. Gastric inflammatory myofibroblastic tumors in children: An unpredictable course. J Pediatr Gastroenterol Nutr 2002;34:319-22.  Back to cited text no. 2
    
3.
Cho MY, Min YK, Kim NR, Cho SJ, Kim HK, Lee KC, et al. Fever of unknown origin as a presentation of gastric inflammatory myofibroblastic tumor in a two-year-old boy. J Korean Med Sci 2002;17:699-703.  Back to cited text no. 3
    
4.
Riedel BD, Wong RC, Ey EH. Gastric inflammatory myofibroblastic tumor (inflammatory pseudotumor) in infancy: Case report and review of the literature. J Pediatr Gastroenterol Nutr 1994;19:437-43.  Back to cited text no. 4
    
5.
Al Hatlani M, Ratcliffe EM. Endoscopic visualization of a gastric polypoid mass: A rare pediatric presentation of an inflammatory myofibroblastic tumor. Gastrointest Endosc 2010;72:894-5.  Back to cited text no. 5
    
6.
Hirschburger M, Enders J, Alzen G, Padberg W, Wagner HJ. An inflammatory myofibroblastic tumor of the stomach as a rare cause of gastric outlet obstruction in an 8-month-old infant. Klin Pädiatr 2010;222:192-3.  Back to cited text no. 6
    
7.
Mirshemirani A, Tabari AK, Sadeghian N, Shariat-Torbaghan S, Pourafkari M, Mohajerzadeh L. Abdominal inflammatory myofibroblastic tumor: Report on four cases and review of literature. Iran J Pediatr 2011;21:543-54.  Back to cited text no. 7
    
8.
Hayashi T, Takahashi A, Otake S, Ishige T, Tatsuki M, Hirato J, et al. An association of hypertrophic pyloric stenosis and pyloric inflammatory myofibroblastic tumor-like lesion: A rare case of an infant with gastric outlet obstruction. J Pediatr Surg Case Rep 2013;1:77-80.  Back to cited text no. 8
    
9.
Jadhav M, Harvi R, Patil R, Kittur S. Inflammatory myofibroblastic tumor of the stomach presenting as an exophytic mass-a diagnostic dilemma. Turk Patoloji Derg 2019;35:151-6.  Back to cited text no. 9
    
10.
Mariño-Enríquez A, Wang WL, Roy A, Lopez-Terrada D, Lazar AJ, Fletcher CD, et al. Epithelioid inflammatory myofibroblastic sarcoma: An aggressive intra-abdominal variant of inflammatory myofibroblastic tumor with nuclear membrane or perinuclear ALK. Am J Surg Pathol 2011;35:135-44.  Back to cited text no. 10
    

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Correspondence Address:
Susama Patra,
Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Bhubaneswar - 751019, Odisha
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_691_21



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