Ann Thorac Surg 2010;90:e36-e37. doi:10.1016/j.athoracsur.2010.06.013
© 2010 The Society of Thoracic Surgeons
Case Reports
Endotracheal Inflammatory Pseudotumor: The Role of Interventional Bronchoscopy
Filipe M. Andrade, MDa,*,
Omar M. Abou-Mourad, MDa,
Luiz Felippe Judice, MD, PhDa,
Antonio Bento C.B. Carvalho-Filho, MDa,
Bruno Schau, MDb,
Angela C.G. Carvalho, MDc
a Department of Surgery, Division of Thoracic Surgery, Antonio Pedro University Hospital, Fluminense Federal University, Niterói, Brazil
b Department of Internal Medicine, Antonio Pedro University Hospital, Fluminense Federal University, Niterói, Brazil
c Department of Pathology, Antonio Pedro University Hospital, Fluminense Federal University, Niterói, Brazil
Accepted for publication June 1, 2010.
* Address correspondence to Dr Andrade, Rua Visconde do Rio Branco, 755/812, Centro, Niterói-RJ, 24020-006, Brazil (Email: filipeandrade_torax{at}hotmail.com).
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Abstract
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We present the case of a 31-year-old woman with recurrent pneumonia. A chest roentgenogram, a chest computed tomographic scan, and a rigid bronchoscopy revealed a mass in the left mainstem bronchus, which was protruding into the trachea and right mainstem bronchus, with no extraluminal component. Endoscopic biopsies confirmed an inflammatory myofibroblastic tumor. The patient was treated with two sessions of bronchoscopic resection, followed by medical therapy with corticosteroids and azathioprine. She has been followed-up for 31 months without complications or recurrence. Although the usual treatment for this type of entity is surgical excision, endoscopic resection may be an adequate oncologic procedure when the tumor is completely endoluminal, as in this case.
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Introduction
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Inflammatory myofibroblastic tumor (IMT) is a rare disease, occurring in the larynx, abdomen, heart, and lungs, among other sites. The IMT accounts for 0.04% to 0.7% of all lung tumors [1, 2]. In children who are less than 16 years of age, an IMT is the most frequent primary tumor of the lungs [2]. Because of atypical symptoms and variable imaging features, it is often initially misdiagnosed as pneumonia or bronchogenic carcinoma [3, 4].
If an IMT is a reactive lesion or a true neoplasm, it is controversial. However, recent cytogenetic studies have suggested that it is a true neoplasm [5].
A 31-year-old white woman presented to an outside institution with symptoms of dry cough, dyspnea, and no previous history of any respiratory or other diseases. The chest roentgenogram was normal, and her physician did not prescribe medications. Three months later her complaints got worse and there were also an expiratory wheezing. She was diagnosed and treated for asthma with minimal relief. A chest roentgenogram was then performed, and the patient was referred to our hospital for evaluation and treatment. At the consultation, there were no other complaints and the only finding of interest in the physical examination was diminished vesicular breath sounds at the inferior third of the left hemithorax. Laboratory findings were unremarkable. After an initial evaluation, a chest computed tomographic scan was performed.
An atelectasis of the left lower lobe (Fig 1A) was found on a posteroanterior chest roentgenogram. A computed tomographic scan (Fig 1B) confirmed the atelectasis and also revealed an intraluminal, rounded mass in the left posterolateral wall of the distal trachea, which was occupying approximately 40% of the lumen at its distal third and also the entire circumference of left main bronchus.
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Fig 1. (A) A chest roentgenogram reveals opacity at the left lower hemithorax, which is compatible with left lower lobe atelectasis. (B) A computed tomographic scan of the chest shows a round lesion occupying approximately 40% of the tracheal lumen.
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A rigid bronchoscopy confirmed a friable, exophitic mass arising from left main bronchus, projecting into the trachea and right main bronchus, thus also involving the carina (Fig 2A). It was not possible to measure the mass exactly, given the lesion occupied the entire circumference of the left bronchus. At first bronchoscopy, we performed biopsies and removed most of the tumor piecemeal with a rigid forceps and eletrocautery. At the end of the procedure, the tumor in the trachea, carina, and right main bronchus had been removed, as it was for a part of the lesion in the left main bronchus (Fig 2B). The team decided to finish the procedure, given the anatomy was disrupted after many piecemeal resections and repeated use of cautery. At that moment, the left lung already could be adequately ventilated, albeit the tumor had not been completely resected. It was also necessary to have the histologic diagnosis to choose the definitive treatment. The histologic examination revealed spindle cells arranged in interlaced fascicles with admixed diverse inflammatory cells; the immunohistochemistry was positive for anaplastic lymphoma kinase-1 and vimentin.
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Fig 2. (A) A rigid bronchoscopy shows the lesion inside the tracheal lumen before resection. (B) The trachea and carina after first endoscopic resection of the tumor. (C) After the second and complete resection, the airway was freed from the tumor.
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Once the IMT was confirmed, the patient was scheduled for another endoscopic procedure. At the second rigid bronchoscopy, complete removal of the tumor from the left main stem bronchus was accomplished (Fig 2C). The underlying cartilage was noted to be intact during both procedures. The recovery of the patient was uneventful, and she was discharged home on the same day after both procedures. The interval between both bronchoscopies was 8 days.
To date (ie, as of the writing of this article), our patient has been followed-up for 31 months since the complete resection. Bronchoscopies were scheduled 15 days after resection, then 1 month, 3 months and, finally, every 6 months. A computed tomographic scan was performed once a year to rule out recurrences in adjacent tissues or lung parenchyma [6]. The patient was also treated with systemic corticosteroids [1, 6] and azathioprine. There was no relapse of the disease detected by bronchoscopic examinations or by computed tomography.
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Comment
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The IMT has been referred to by different names, including histiocytoma, fibrous histiocytoma, xanthoma, xanthofibroma, xantogranuloma, and plasma cell granuloma [1, 2]. The most common name for the IMT, and in our opinion also the most adequate denomination, is inflammatory myofibroblastic tumor or inflammatory pseudotumor.
The IMT is defined by the World Health Organization as a distinctive lesion composed of a myofibroblastic spindle cell population accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils [7]. This relatively rare entity mainly affects the soft tissue viscera of children and young adults.
This entity can affect different organs, such as the brain, liver, kidney, heart, and others. This neoplasm, although rare, has a preference for the lungs, where its incidence is reported to be 0.04% to 0.7% of all lung tumors [1, 4, 6]. In the small subset of patients who have endobronchial lesions, the presentation may be more acute and clinically impressive due to postobstructive pneumonia or symptoms related to airflow obstruction. Despite this predilection for pulmonary involvement, a tracheobronchial IMT is exceedingly rare.
Although previous studies have suggested that an IMT could be an inflammatory reactive lesion as a consequence of some types of pulmonary infection, recent reports state that an IMT is a true neoplasm rather than a reactive lesion, because of clonal chromosomal abnormalities [8], chromosomal rearrangements involving the anaplastic lymphoma kinase receptor tyrosine-kinase locus region, and DNA aneuploidy in the IMT [4]. This tumor usually grows locally and slowly; therefore, taking into account these clinical, histopathologic, and biological findings, an IMT may be regarded as a low-grade malignancy. We believe that the designation of benign tumor is not appropriate, because recurrence can occur after resection and some infrequent cases of distant metastasis are reported. Spontaneous regression of an IMT had been reported in the past, but given the difficulty in making the diagnosis preoperatively and the clear necessity of treatment, these are only anecdotal reports.
The experience with tracheobronchial IMT is lacking. Cerfolio and Mathews [6] describe a case of this tumor in a young female patient, in whom after repeated bronchoscopic resections it was still necessary to perform a resection of the entire left main bronchus because the tumor had spread just beyond the bronchial wall.
Although pulmonary resections with parenchyma sparing is the surgical option of choice for most pulmonary IMTs, endobronchial resection can be offered if there is no tumor spreading found beyond the tracheobronchial wall with excellent results and less morbidity [6].
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References
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- Cerfolio RJ, Allen MS, Nascimento AG, et al. Inflammatory pseudotumors of the lung Ann Thorac Surg 1999;67:933-936.[Abstract/Free Full Text]
- Fabre D, Fadel E, Singhal S, et al. Complete resection of pulmonary inflammatory pseudotumors has excellent long-term prognosis J Thorac Cardiovasc Surg 2009;137:435-440.[Abstract/Free Full Text]
- Narla L, Newman B, Spottswood S, Narla S, Kolli R. Inflammatory pseudotumor Radiographics 2003;23:719-729.[Abstract/Free Full Text]
- Sakurai H, Hasegawa T, Watanabe S, Suzuki K, Asamura H, Tsuchiya R. Inflammatory myofibroblastic tumor of the lung Eur J Cardiothorac Surg 2004;25:155-159.[Abstract/Free Full Text]
- Lawrence B, Perez-Atayde A, Hibbard MK, et al. TPM3-ALK and TPM4-ALK oncogenes in inflammatory myofibroblastic tumors Am J Pathol 2000;157:377-384.[Medline]
- Cerfolio RJ, Mathews TC. Resection of the entire left mainstem bronchus for an inflammatory pseudotumor Ann Thorac Surg 2005;79:2127-2128.[Abstract/Free Full Text]
- Venizelos I, Papathomas T, Anagnostou E, Tsanakas J, Kirvassilis F, Kontzoglou G. Pediatric inflammatory myofibroblastic tumor of the trachea: a case report and review of the literature Pediatr Pulmonol 2008;43:831-835.[Medline]
- Snyder CS, Dell' Aquila M, Haghighi P, Baergen RN, Suh YK, Yi ES. Clonal changes in inflammatory pseudotumor of the lung Cancer 1995;76:1545-1549.[Medline]
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Abstract
Introduction
