Ann Thorac Surg 2003;76:601-603
© 2003 The Society of Thoracic Surgeons
Case report
Pulmonary inflammatory myofibroblastic tumorinvading the left atrium
Marius Berman, MDa*,
Georgios P. Georghiou, MDa,
Tommy Schonfeld, MDb,
Meora Feinmesser, MDc,
Gadi Horev, MDd,
Bernardo A. Vidne, MDa,
Milton Saute, MDa
a DEPARTMENT OF Cardiothoracic Surgery, Rabin Medical Center, Beilinson Campus, Tel Aviv, Israel
c DEPARTMENT OF Pathology, Rabin Medical Center, Beilinson Campus, Tel Aviv, Israel
b DEPARTMENT OF Intensive Care, Schneider Childrens Medical Center of Israel, Petach Tikva, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
d DEPARTMENT OF Roentgenology, Schneider Childrens Medical Center of Israel, Petach Tikva, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
Accepted for publication November 27, 2002.
* Address reprint requests to Dr Berman, Department of Cardiothoracic Surgery, Rabin Medical Center, Beilinson Campus, Petach Tikva, 49100 Israel
e-mail: mariusb{at}clalit.org.il
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Abstract
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Inflammatory myofibroblastic tumor is a rare solid tumor that most often affects children and young adults. Although benign, the tumor may be very aggressive locally. We describe a 9-year-old boy with primary inflammatory myofibroblastic tumor of the left upper lobe involving the left atrium.
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Introduction
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Inflammatory myofibroblastic tumor (IMT) is a rare solid tumor that most often affects children and young adults [1, 2]. It was first described in 1937 in the lungs and thereafter in various sites such as the mesentery and cardioesophageal region [3]. Although histopathologically benign, IMT cannot be differentiated from a malignancy because of its local invasiveness and its tendency to recur. We describe an unusual case of primary IMT of the left upper lobe with invasion of the left atrium.
A 9-year-old boy was referred to our department for evaluation of recurrent pneumonia of the left lung in the absence of antecedents or apparent risk factors for upper respiratory tract infection. Treatment consisted of amoxicillin and clavulanic acid, prednisone, and inhalations of budesonide and terbutaline. The airway obstruction symptoms failed to respond to the steroids. Chest roentgenogram performed after resolution of the acute febrile phase and inflammatory response showed partial atelectasis of the left upper lobe. Computed tomography scan showed a possible mass at the left hilum (Fig 1)
compressing the left atrium (Fig 2), and bronchoscopy revealed extrinsic compression of the left main bronchus.
Because of the patient’s poor pulmonary condition, a stent was inserted, which successfully maintained a reasonable amount of ventilation of the left lung (Fig 3).
Two days later, exploratory thoracotomy was performed. A palpable solid tumor involving the left upper lobe, the left pulmonary hilum, and the posterior part of the pericardium was noted. The diagnosis on biopsy study was IMT. A therapeutic median dose of steroids was given for 2 weeks with no effect. Echocardiography confirmed the clinical suspicion of left atrium involvement.
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Fig 1. Axial computed tomographic scan at infracarina level, showing tumor of left lung compressing the left bronchus and segmental atelectasis.
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Fig 3. Axial computed tomographic scan showing tumor of left lung with left main bronchus kept open with an endobronchial stent.
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Owing to the invasiveness of the tumor, an aggressive operative approach was adopted. The patient underwent median sternotomy, with connection to a cardiopulmonary bypass machine and cardioplegic arrest. The left pulmonary artery was separated, and the remaining common pulmonary artery was closed. The left atrium was than opened, followed by resection of the free wall together with the origin of the left pulmonary veins, with safe margins. The posterior pericardium was opened, the carina was dissected, and the left main bronchus was separated; left pneumonectomy was completed (Fig 4).
The main bronchus was closed, and the left atrium was reconstructed using an autopericardial patch. At termination of surgery, the patient was weaned from the cardiopulmonary bypass machine with no inotropic support. Pathologic examination confirmed the diagnosis of IMT (Fig 5).
The postoperative course was uneventful, and the patient was discharged after 4 days.
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Fig 4. Postoperative macroscopic view of left lung. (1 = inflammatory myofibroblastic tumor near the hilum, engulfing the left main bronchus and pulmonary arteries; 2 = tumor penetrating the left atrium compressing the orifice of left pulmonary veins; 3 = free left atrium wall.)
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Fig 5. Inflammatory myofibroblastic tumor composed of spindle cell proliferation and scattered inflammatory cells extending into the left atrial myocardium (arrows). (Original magnification x20.)
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At the 10-month follow-up, he appeared to be in good general condition, gaining weight and disease-free.
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Comment
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Inflammatory myofibroblastic tumor has been termed in different studies inflammatory pseudotumor, pseudosarcomatous myofibroblastic proliferation, plasma cell granuloma, xanthomatous pseudotumor, inflammatory myofibrohistiocytic proliferation, and inflammatory fibrosarcoma [4]. Its etiology is still unclear. Some researchers claim it is a true neoplasm, whereas others believe it is an immunologic response to an infectious or noninfectious intervention or insult [1], such as previous abdominal surgery, trauma, ventriculoperitoneal shunt, radiotherapy, or steroid use. Genetic factors may also play a role [5].
The lung is the most frequent site of occurrence of IMT [4, 6], followed by the small and large bowel mesentery, mediastinum, retroperitoneum, omentum, and diaphragm [7]. Less common sites are thyroid, brain, spinal cord meninges, abdominal wall, and liver.
The clinical presentation varies markedly, depending on the site at which the tumor originates. Inflammatory myofibroblastic tumor of the lung is often asymptomatic, although some patients present with cough, shortness of breath, chest pain, dysphagia, and hemoptysis [6]. Clubbing, if present, disappears after tumor resection [8]. Laboratory evidence of an inflammatory response has been described, including hypochromic microcytic anemia, increased immunoglobulins, elevated erythrocyte sedimentation rate, and elevated thrombocyte count. The radiologic appearance of IMT extends the differential diagnosis of coin lesion, although this presentation is really rare. Chest CT usually shows a circumscribed calcified mass. The final diagnosis, however, is based on histopathologic findings of spindle-shaped cells with a chronic inflammatory component consisting of plasma cells, lymphocytes, and occasional histiocytes.
The best accepted treatment is complete surgical resection of the lesion. Corticosteroids, although originally considered a possible etiologic factor, appear to have a beneficial effect on tumor mass and are used in inoperable cases [9]. One study described the use of corticosteroids in a case of IMT with pleural thickening [10]. As pathologic study of all areas of tumor is crucial to confirm the diagnosis, needle biopsy should not be performed; frozen section, too, should be avoided owing to the resemblance of IMT to soft tissue sarcoma.
The rate of local recurrence ranges between 15% and 37%, according to large series of mesentery and retroperitoneum IMT [4]; most recurrences appear within 1 year of surgery. In view of the multifocal nature of the lesion, recurrences are assumed to represent mainly rare distant metastases.
Two series of thoracic IMT treated surgically have been reported [6, 11]. More than 90% of the patients remained disease-free, with no recurrent tumors and no metastatic lesion for up to 27 years of follow-up. A tendency of IMT of the lung to invade the pulmonary vessels was reported by Warter and associates [12]. Hartyanszky and colleagues [13] described a patient with recurrent IMT in the right ventricular outflow tract.
Pediatricians and surgeons should be aware of this entity and its surgical management. At the time of surgery, a meticulous search should be conducted for additional small lesions, as they may provoke early recurrence.
Although IMT may be suspected preoperatively by clinical, laboratory, and radiologic findings, a precise diagnosis can be made only on the basis of permanent histologic sections. Close follow-up is required for early diagnosis of recurrence.
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Acknowledgments
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We thank Gloria Ginzach for her editorial assistance.
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References
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- Pettinato G., Manivel J.C., De Rosa N., Dehner L.P. Inflammatory myofibroblastic tumor (plasma cell granuloma). Am J Clin Pathol 1990;94:538-546.[Medline]
- Monzon C.M., Gilchrist G.S., Burgert E.O., et al. Plasma cell granuloma in the lung in children. Pediatrics 1982;70:268-274.[Abstract/Free Full Text]
- Coffin C.M., Watterson J., Priest J.R., et al. Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). Am J Surg Pathol 1995;19:865-872.
- Karnak I., Senocak M.E., Ciftci A.O., et al. Inflammatory myofibroblastic tumor in children: diagnosis and treatment. J Pediatr Surg 2001;36:908-912.[Medline]
- Snyder C.S., Dell’Aquilla M., Haghighi P., et al. Clonal changes in inflammatory pseudotumor of the lung. Cancer 1995;76:1545-1549.[Medline]
- Souid A.K., Ziemba M.C., Dubansky A.S., Mazur M., et al. Inflammatory myofibroblastic tumor in children. Cancer 1993;72:2042-2048.[Medline]
- Meis J.M., Enzinger F.M. Inflammatory fibrosarcoma of the mesentery and the retroperitoneum: a tumor closely simulating inflammatory pseudotumor. Am J Surg Pathol 1991;15:1146-1156.[Medline]
- Bahadori M., Liebow A.A. Plasma cell granuloma of the lung. Cancer 1990;31:191-208.
- Doski J.J., Priebe C.J., Driessnack M., et al. Corticosteroids in the management of unresected plasma cell granuloma (inflammatory pseudotumor) of the lung. J Pediatr Surg 1991;26:1064-1066.[Medline]
- Ishioka S., Maeda A., Yamasaki M., Yamakido M. Inflammatory pseudotumor of the lung with pleural thickening treated with corticosteroids. Chest 2000;117:923.[Free Full Text]
- Cerfolio R.J., Allen M.S., Nascimento A.G., et al. Inflammatory pseudotumor of the lung. Ann Thorac Surg 1999;67:933-936.[Abstract/Free Full Text]
- Warter A., Satge D., Roeslin N. Angioinvasive plasma cell granulomas of the lung. Cancer 1987;59:435-443.[Medline]
- Hartyanszky I.L., Kadar K., Hubay M. Rapid recurrence of an inflammatory myofibroblastic tumor in the right ventricular outflow tract. Cardiol Young 2000;10:271-274.[Medline]
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Abstract
Introduction
