Articles
Inflammatory myofibroblastic tumor in children: Diagnosis and treatment

https://doi.org/10.1053/jpsu.2001.23970Get rights and content

Abstract

Background/Purpose: Inflammatory myofibroblastic tumor (IMT) is a rare benign neoplasm. Although it is commonly seen in children, the number of childhood cases in the current literature is limited. Furthermore, malignant degeneration or transformation to lymphoma in the recurrent or residual IMT have directed attention to this interesting entity. Herein, the authors present their experience with IMT with special emphasis on diagnosis and treatment. Methods: All records of children treated with diagnosis of IMT between 1977 and 1999 inclusive were evaluated retrospectively. Results: Seven children were treated for IMT with the mean age of 9.14 ± 2 years (range, 6 to 12 years). Male to female ratio was 5:2. Respiratory symptoms and clubbing were present in a patient with pulmonary IMT (n = 1). Abdominal pain (n = 3), fever (n = 2), and weight loss (n = 4) were encountered in intraabdominal IMTs. The most frequent physical finding was palpable intraabdominal mass (n = 4). Plain films showed nonspecific findings such as radiodense area in the hemithorax (n = 1), displacement of bowel segments (n = 2), air-fluid levels (n = 1), and amorphous calcification (n = 4). Ultrasonography and CT showed calcified masses in 4 cases. Except the case with intrathoracic IMT, all the tumors were located in the abdomen at various sites such as cardioesophageal junction (n = 1), left hepatic lobe (n = 1), mesentery of the small bowel (n = 2), and antimesenteric wall of the descending colon (n = 1), gastrosplenic region and porta hepatis (n = 1). Tumor sizes ranged from 3 × 2 × 2 cm to 15 × 15 × 13 cm. The masses were excised totally in all but one case. Infiltrated organs (esophagogastric junction, a segment of jejunum, and spleen, stomach wall, and renal capsule) were resected in 3 cases. Total surgical excision of IMT was considered adequate for treatment in 6 cases. One patient with aggressive IMT required further treatments such as immunomodulation and chemotherapy and died of neutropenic sepsis. Conclusions: IMT is a benign neoplasm rarely presented with malignant features such as local invasiveness, recurrence, distant metastasis, or malignant transformation. IMT can be suspected preoperatively through some hematologic abnormalities and radiologic findings, but precise diagnosis should be made on the basis of histologic findings. Complete surgical resection and close follow-up are all necessary for appropriate treatment to avoid recurrences as well as unnecessary and potentially harmful therapy. The optimal management of locally aggressive and recurrent forms should be decided individually for each patient. J Pediatr Surg 36:908-912. Copyright © 2001 by W.B. Saunders Company.

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Materials and methods

Records of children who were treated for plasma cell granuloma, inflammatory pseudotumor, or IMT at our department between 1977 and 1999, inclusive, were evaluated retrospectively. Information recorded for each patient included age, sex, clinical characteristics, diagnostic procedures, treatment, histopathologic characteristics, and outcome. Histopathologic diagnosis was made by using H&E staining and immunohistochemical staining methods and by electron microscope.

Results

There were 7 children treated for IMT in the study period (Table 1).

. Clinical, Radiologic, and Surgical Features of Children With IMT

Case No.Age, SexPresenting SymptomsRadiologic FindingsLocationTreatment
18 yr, FDysphagia, weight lossUGI series: distal esophageal stenosisCardioesophageal junctionProximal esophagogastrectomy plus esophagogastric anastomosis
210 yr, MMinor respiratory symptomsPlain chest x-ray and chest CT: Posterior mediastinal massRight lower lung lobeTotal excision
36 yr, M

Discussion

Inflammatory myofibroblastic tumor was first described in the in 1937, and since then has been reported at various sites such as mesentery (one of the most common extrapulmonary sites) and cardioesophageal region.1, 2, 3, 4, 8, 9, 10 It mainly is a tumor of young adults and children,4, 5 and the lung is the most frequent site of location. Histopathologically, IMT is a benign solid tumor composed mainly of spindle-shaped cells and has a chronic inflammatory component consisting of plasma cells,

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Address reprint requests to Ibrahim Karnak, MD, Department of Pediatric Surgery, Hacettepe University Medical Faculty, 06100 Ankara, Turkey.

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