International Journal of Pediatric Otorhinolaryngology Extra
Volume 1, Issue 3 , Pages 217-219, September 2006

Inflammatory pseudotumor of the trachea

  • Richard J. Vivero
    • ,
  • Sandeep P. Dave
    • ,
  • Soham Roy

      Affiliations

    • Corresponding Author InformationCorrespondence to: Pediatric Otolaryngology-Head and Neck Surgery, Department of Otolaryngology-Head and Neck Surgery, University of Miami, 900 NW 17th Street, Pediatic ENT-Ground Floor, Bascom Palmer Eye Institute, Miami, FL 33136, USA. Tel.: +1 305 326 6332; fax: +1 305 547 3670.

Department of Otolaryngology, Leonard M. Miller School of Medicine, University of Miami, Miami, FL, USA

Received 9 May 2006; accepted 28 May 2006.

Article Outline

Summary 

Although more than a dozen names have been used to describe the so-called inflammatory pseudotumor, the term inflammatory myofibroblastic tumor has been suggested by many authors to be an appropriate designation. It is generally a benign lesion that most commonly arises from the lung, but cases involving nearly every site in the body have been documented. Histopathological confirmation is required to prevent an unwarranted radical intervention. Tracheal involvement is uncommon and rarely, distant metastases and sarcomatous transformation have been reported. The clinical presentation of airway inflammatory pseudotumor varies and can mimic refractory-type asthma or malignancy. Conservative endoscopic removal is the standard of care and the overall prognosis is excellent. We present a pediatric case of tracheal inflammatory pseudotumor, and review the etiology, diagnosis and treatment.

Keywords: Inflammatory myofibrohistiocytic proliferation, Histiocytoma, Xanthoma, Xanthofibroma, Xanthogranuloma, Sclerosing hemangioma, Endothelioma, Plasmacytoma

 

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1. Introduction 

Although infrequently seen in clinical practice, a variety of terms have been used to describe the inflammatory pseudotumor. In contrast to the majority of primary tracheal neoplasms which are malignant, inflammatory pseudotumor is most likely a benign reactive process, and accordingly, otolaryngologists must exercise caution prior to undertaking any radical procedure to treat this lesion. We report the case of a 15-year-old girl who presented with severe respiratory distress secondary to a large inflammatory pseudotumor of the trachea.

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2. Case report 

A 15-year-old Haitian girl presented to an outside institution with wheezing, odynophagia and subjective weight loss. She was diagnosed and treated for asthma with minimal relief. Six weeks later she experienced severe respiratory distress, which resulted in respiratory arrest. The patient was intubated in the field and transferred to an outside pediatric intensive care unit. The episode was attributed to status asthmaticus. She subsequently failed extubation with immediate desaturation and cardiac arrest. She was re-intubated and required a brief period of cardiopulmonary resuscitation. Computed tomography (CT) revealed an intraluminal tracheal mass at the distal end of the endotracheal tube. Bronchoscopy demonstrated a tracheal lesion at the tip of the endotracheal tube which was causing a ball-valve effect and necessitating high pressures for adequate ventilation (Fig. 1A). The patient subsequently developed an apical pneumothorax and was transferred to our institution for further management.

  • View full-size image.
  • Fig. 1. 

    (A) Bronchoscopy demonstrating a pedunculated soft tissue mass arising from the tracheal wall at the distal end of the endotracheal tube and (B) postoperative bronchoscopy at one month demonstrating residual granulation tissue and no evidence of recurrence.

Direct laryngoscopy and bronchoscopy confirmed a 2cm friable, pedunculated mass, which was approximately 5.5cm below the vocal folds and arose from the left lateral tracheal wall. The lesion was completely removed with endoscopic forceps. The underlying cartilage was noted to be intact. Histopathological examination revealed edematous spindle cells with a “tissue-culture” appearance. Plasma cells and eosinophils were abundant. Immunohistochemistry was positive for keratin, but negative for anaplastic lymphoma kinase (ALK) gene and caldesmon.

The patient was extubated the following day and discharged 3 days later. Bronchoscopy was performed one month later and revealed two small posterior glottic granulomas and a small area of granulation tissue approximately 10cm distal to the vocal folds. There was no evidence of recurrence (Fig. 1B).

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3. Discussion 

The confusion surrounding the so-called inflammatory pseudotumor is illustrated by the plethora of terms used to describe this lesion. It has been referred to by more than a dozen names including inflammatory myofibrohistiocytic proliferation, histiocytoma, fibrous histiocytoma, xanthoma, xanthofibroma, xanthogranuloma, sclerosing hemangioma, endothelioma, plasmacytoma, plasma cell–histiocytoma complex and plasma cell granuloma [1], [2], [3], [4]. Although the most appropriate classification is still debated, most authors suggest either inflammatory myofibroblastic tumor or inflammatory fibrosarcoma depending on the tumor's histology and clinical behavior [2], [5]. It is generally a benign lesion that most commonly arises from the lung [1], [2], [5], [6]. Rarely, distant metastases and sarcomatous transformation have been reported [2], [5]. Cases involving nearly every site in the body, including the larynx, maxillary sinus, parapharyngeal space, mediastinum and abdomen have been documented [2], [5], [6], [7]. Inflammatory pseudotumor is more common in the pediatric population with 50% of cases presenting as an intrapulmonary mass involving the parenchyma, pleura or bronchi [6], [7], [8]. Despite this predilection for pulmonary involvement, tracheal inflammatory pseudotumor is uncommon.

There is no consensus regarding the exact etiology of inflammatory pseudotumor. Potential mechanisms include an inflammatory reaction secondary to trauma, an autoimmune reaction or infection [2], [6]. On histopathological examination, the tumor is characterized by a predominance of spindle cells and the presence of plasma cells, lymphocytes, foamy histiocytes, multinucleated giant cells, fibroblasts and blood vessels. This pattern of histiocytes, giant cells and spindle cells grouped in a whorled form is ultimately required for diagnosis [6], [8]. Immunohistochemistry is helpful to exclude other similar appearing lesions [2], [3], [4].

The clinical presentation of airway inflammatory pseudotumor varies and depends on the area of involvement. Dyspnea, stridor, wheezing, chronic cough, hemoptysis, dysphagia, fever, pleuritic pain, right upper quadrant or epigastric pain and severe constitutional symptoms have all been described [3], [4], [6]. Tracheal involvement can manifest as obstructive respiratory symptoms, which, as in our case, are often initially misdiagnosed as asthma [2], [3], [6]. Pulmonary infection, physical exertion, and postural changes may exacerbate symptoms [8].

Given the variability in presentation and its low incidence, accurate diagnosis and treatment of tracheal inflammatory pseudotumor is often delayed. Inflammatory pseudotumor should be considered in any pediatric patient who presents with a pulmonary or tracheal mass. CT scan may demonstrate a well-defined or circumscribed mass [6]. Direct laryngoscopy and bronchoscopy with biopsy is mandatory for diagnosis and to evaluate the patency of the airway. Fine-needle aspiration and frozen section analysis are not always reliable [2]. After histopathological confirmation, conservative resection via endoscopic removal or CO2 laser is the standard of care. The efficacy of these modalities has made tracheal resection largely unnecessary unless gross tracheal wall involvement is present. Radiotherapy has been used with limited results, and should be reserved for non-surgical candidates and inoperable tumors [2], [3].

The overall prognosis for tracheal inflammatory pseudotumor is excellent. Most recurrences occur within 1 year, which mandates follow-up bronchoscopy at regular intervals to ensure complete removal [2], [3], [8].

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4. Conclusion 

Inflammatory pseudotumor should be considered in any pediatric patient who presents with a pulmonary or tracheal mass. This lesion can mimic malignancy and therefore histopathological confirmation is required prior to any aggressive intervention. Endoscopic removal is the standard of care and prognosis is excellent.

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References 

  1. Aijaz F, Salam AU, Muzaffar S, Akbani Y, Hasan SH. Inflammatory pseudotumour of the trachea: report of a case in an 8-year-old child. J. Laryngol. Otol. 1994;108(July (7)):613–616
  2. Browne M, Abramson LP, Chou PM, Acton R, Holinger LD, Reynolds M. Inflammatory myofibroblastic tumor (inflammatory pseudotumor) of the neck infiltrating the trachea. J. Pediatr. Surg. 2004;39(October (10)):e1–e4
  3. Bumber Z, Jurlina M, Manojlovic S, Jakic-Razumovic J. Inflammatory pseudotumor of the trachea. J. Pediatr. Surg. 2001;36(April (4)):631–634
  4. Nikanne E, Sopanen J, Seppa A. Inflammatory pseudotumor of the trachea. Otolaryngol. Head Neck Surg. 2004;130(February (2)):274–276
  5. Amir R, Danahey D, Ferrer K, Maffee M. Inflammatory myofibroblastic tumor presenting with tracheal obstruction in a pregnant woman. Am. J. Otolaryngol. 2002;23(November–December (6)):362–367
  6. Restrepo S, Mastrogiovanni LP, Palacios E. Inflammatory pseudotumor of the trachea. Ear Nose Throat J. 2003;82(July (7)):510–512
  7. Dewar AL, Connett GJ. Inflammatory pseudotumor of the trachea in a 10-month-old infant. Pediatr. Pulmonol. 1997;23(April (4)):307–309
  8. Dunnick NR. Image interpretation session: Inflammatory myoblastic pseudotumor of the trachea (plasma cell granuloma). Radiographics. 2000;20(January–February (1)):274–276

PII: S1871-4048(06)00063-3

doi:10.1016/j.pedex.2006.05.008

International Journal of Pediatric Otorhinolaryngology Extra
Volume 1, Issue 3 , Pages 217-219, September 2006

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