Ann Thorac Surg 2009;87:610-613. doi:10.1016/j.athoracsur.2008.07.036
© 2009 The Society of Thoracic Surgeons
Case Reports
Inflammatory Myofibroblastic Tumor in the Airway of a Child
Derek A. Uchida, MDa,*,
John A. Hawkins, MDb,
Cheryl M. Coffin, MDc,
Johannes Fredrik Grimmer, MDb
a Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, Utah
b Department of Surgery, University of Utah School of Medicine, Salt Lake City, Utah
c Department of Pathology, University of Utah School of Medicine, Salt Lake City, Utah
Accepted for publication July 9, 2008.
* Address correspondence to Dr Uchida, Pediatric Pulmonology, 100 N Mario Capecchi Dr, Salt Lake City, UT 84113 (Email: derek.uchida{at}hsc.utah.edu).
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Abstract
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This report describes a 9-year-old boy who was found to have an inflammatory myofibroblastic tumor completely occluding the right mainstem bronchus. Initial subtotal resection at an outside facility resulted in prompt recurrence of the tumor. At our institution, total resection with bronchotomy and pericardial patch repair resulted in an excellent outcome with no evidence of recurrence at 1 year post-resection.
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Introduction
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Inflammatory myofibroblastic tumor is an intermediate, locally recurrent neoplasm that prototypically occurs in the lung [1, 2]. Rarely, this tumor can arise within the airway of a young child. We present the case of a young boy who was discovered to have a large inflammatory myofibroblastic tumor in the right mainstem bronchus.
A 9-year-old boy was presented to his primary care physician with chief complaints of cough, abdominal pain, and fever. On physical examination, breath sounds were decreased in the right lower lung field. A chest roentgenogram demonstrated a right lower lobe infiltrate. He was treated with antibiotics for presumed pneumonia. He presented 6 months later with dyspnea and right-sided chest pain. A chest roentgenogram demonstrated persistence of the right lower lobe infiltrate. A computed tomographic scan of the thorax revealed a 14-mm mass present in the right main bronchus.
Rigid bronchoscopy was performed at an outside institution. A nonpulsatile mucosa-covered lesion was removed. Histopathology from this procedure is not available. He was discharged in good condition. Ten months later he presented with cough, dyspnea, and fatigue. A chest roentgenogram showed right lower lobe and right middle lobe infiltrates (Fig 1A). A computed tomographic scan was obtained, which showed a mass projecting into the proximal right mainstem bronchus (Fig 1B). The patient was then referred to our institution.
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Fig 1. (A) Chest roentgenogram frontal view demonstrating atelectasis of the right lower lobe. (B) Computed tomographic scan showing a round mass (arrow) in the right mainstem bronchus.
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Bronchoscopy demonstrated a mass totally obstructing the right mainstem bronchus (Fig 2). Biopsy of the mass was consistent with an inflammatory myofibroblastic tumor. Due to the recurrence risk of inflammatory myofibroblastic tumor and the prior recurrence, we decided an open approach would give us the best chance of success. The child was intubated with a standard endotracheal tube, which was advanced down into the left mainstem bronchus for the resection of the tumor. The patient underwent a right thoracotomy. A mass with rubber-like consistency in the bronchus intermedius was palpable, just at the junction, with the takeoff of the right upper lobe bronchus. There was a purple hue over the membranous portion of the bronchus intermedius; the mass appeared to be almost eroding through the bronchus. A right mainstem bronchotomy was performed to visualize the tumor and assess its extent. The tumor was adherent to the membranous portion of the bronchus just distal to the takeoff of the right upper lobe bronchus and extended right to the bifurcation of the right lower and middle lobes. It was believed that a sleeve resection would have been quite complicated in this child; therefore, a simple excision of the tumor and membranous portion of the bronchus was sufficient. The resultant defect in the bronchus was repaired with a glutaraldehyde-treated autologous pericardial patch. A glutaraldehyde-treated pericardium was used to give the membranous portion of the airway some stiffness immediately postoperatively. This patch was sealed with tissue glue (Tisseel; Baxter, Deerfield, IL) and was covered with a local intercostal muscle pedicle. The endotracheal tube was then withdrawn at the conclusion of the procedure to allow ventilation of the right lung and examination of the pericardial patch under direct vision with positive pressure ventilation. The patient was extubated immediately, and good aeration of the previously collapsed right lower lobe was accomplished. He was discharged home on postoperative day 7 on room air.
Microscopic sections of the submitted tissue showed a cellular mass composed of interlacing fascicles of spindle cells with small foci of overlying pseudo-stratified ciliated columnar mucosa with focal squamous metaplasia. An inflammatory infiltrate accompanied the mass, consisting predominantly of plasma cells with occasional eosinophils and lymphocytes, and focal aggregates of foamy histiocytes. The mitotic rate was less than 1 per 10 high-power field. Immunohistochemical stains with appropriate controls revealed cytoplasmic reactivity for vimentin, smooth muscle actin, and anaplastic lymphoma kinase-1. These features led to a diagnosis of inflammatory myofibroblastic tumor (IMT).
Bronchoscopy performed 10 weeks after the resection demonstrated a small amount of inflammation at the excision site. Histopathology demonstrated granulation tissue without evidence of IMT recurrence.
Bronchoscopy performed at 6 months post-excision showed a widely patent bronchus with evidence of re-mucosalization over the pericardial patch. There was no visual evidence of tumor recurrence (Fig 3A). Repeat bronchoscopy at 1-year post excision showed similar findings (Fig 3B). Clinically, the patient is doing well and is not having any significant respiratory symptoms.
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Fig 3. (A) 6 months post-resection showing widely patent right mainstem bronchus with minimal granulation tissue and no evidence of tumor recurrence. (B) Right mainstem bronchus at 12-months post-resection.
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Comment
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Inflammatory myofibroblastic tumor was originally reported in the lung, but it is subsequently described in other sites including soft tissue, viscera, larynx, and genitourinary tract [1, 2]. Inflammatory myofibroblastic tumor is currently considered as an intermediate, locally recurrent neoplasm that rarely metastasizes [3]. There are many synonymous terms used for inflammatory myofibroblastic tumor, including plasma cell granuloma, inflammatory pseudotumor, and plasma cell pseudotumor [1]. Histopathology reveals spindled myofibroblasts accompanied by an inflammatory infiltrate consisting of plasma cells, lymphocytes, and eosinophils [1]. The immunhistochemical staining pattern noted in this case is consistent with reports of IMT. Inflammatory myofibroblastic tumor must be differentiated from spindle cell squamous carcinoma, a malignant tumor that has metastatic potential [2]. The absence of atypical mitoses, despite the presence of mitotic figures is a key feature of IMT that distinguishes it from spindle cell squamous carcinoma and other malignant neoplasms [2].
Reported cases of this tumor presenting in the trachea or a bronchus in children are few [4–6]. Complete resection of the tumor is the treatment of choice; subtotal resection can result in recurrence. We are confident that the most complete resection possible was achieved in our patient because the biopsy of the site of involvement at 10 weeks post-resection showed no evidence of tumor, and bronchoscopy at 6 and 12 months post-resection showed no evidence of recurrence. In our patient, the initial subtotal removal of the lesion at another facility resulted in rapid recurrence and significant clinical symptoms within a year.
Metastasis, while rare, has been reported. A recent report suggested that anaplastic lymphoma kinase-negative lesions are more prone to metastasize. Positive reactivity for anaplastic lymphoma kinase-1, as was noted in this patient, may be a favorable prognostic marker in this regard [3], although there is still a risk for recurrence.
Achieving complete resection can present significant difficulties if the location of the tumor is in a large airway. Previously reported techniques used for resection of these tumors in the airway of children include bronchoplasty and resection with airway reconstruction [4–6]. In our patient, bronchotomy with local resection of the membranous portion of the mainstem bronchus and pericardial patch repair resulted in an excellent outcome to date, and this represents another approach to consider when encountering a patient with IMT arising within the proximal mainstem bronchus. Although pericardial patch repair for bronchial stenosis has been reported in children [7], its use in this setting of bronchial reconstruction for inflammatory myofibroblastic tumor has not been reported. In addition, although bronchotomy for this entity has been reported in a 17-year-old [8], this is the first report in a young child of using the technique of localized resection and pericardial reconstruction for IMT. This approach offers another option when complex reconstructions of the airway are necessary and when avoidance of complete pneumonectomy or a more complex sleeve resection is desired.
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References
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- Coffin CM, Fletcher JA. Inflammatory myofibroblastic tumourIn: Fletcher CDM, Unni KK, Mertens F, editors. Pathology and genetics of tumours of soft tissue and bone. . World Health Organization Classification of Tumours. Lyon: IARC Press; 2002. pp. 91-93.
- Wenig BM, Devaney K, Bisceglia M. Inflammatory myofibroblastic tumor of the larynx. A clinicopathologic study of eight cases simulating a malignant spindle cell neoplasm. Cancer 1995;76:2217-2229.
- Coffin CM, Hornick JL, Fletcher CD. Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases Am J Surg Pathol 2007;31:509-520.[Medline]
- Chen D, Ryan G, Edwards M. Bronchial sleeve resection for a patient with an inflammatory pseudotumour ANZ J Surg 2001;71:187-189.[Medline]
- Hoseok I, Joungho H, Ahn KM, Lee SI, Kim J. Complete surgical resection of inflammatory myofibroblastic tumor with carinal reconstruction in a 4-year-old boy J Pediatr Surg 2005;40:e23-e25.[Medline]
- Maeda M, Matsuzaki Y, Edagawa M, Shimizu T, Onitsuka T, Kataoka H. Successful treatment of a bronchial inflammatory pseudotumor by bronchoplasty in an 8-year-old boy: report of a case Surg Today 2000;30:465-468.[Medline]
- Waldhausen JH, Richardson M, Hall DG. A new technique for surgical correction of bronchial stenosis J Pediatr Surg 1994;29:199-200.[Medline]
- Hajjar WA, Ashour MH, Al-Rikabi AC. Endobronchial inflammatory pseudotumor of the lung Saudi Med J 2001;22:366-368.[Medline]
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Abstract
Introduction
