Surgery for recurrent inflammatory pseudotumor of the lung
© Goto et al; licensee BioMed Central Ltd. 2011
Received: 10 May 2011
Accepted: 18 October 2011
Published: 18 October 2011
Cases of recurrent inflammatory pseudotumor have only rarely been reported. The treatment for recurrent pseudotumor is surgery. Patients not eligible for surgery require different treatment, and the optimal type of the treatment is controversial.
A 54-year-old woman was noted to have an abnormal shadow in the right middle lung field on chest X-ray. Computed tomography of the chest revealed an infiltrative lesion in the right segment 4 and a nodule in the right segment 8. She underwent right middle lobectomy and partial resection of the right segment 8. Histopathology revealed non-atypical lymphocytes and plasma cells infiltrates, leading to the diagnosis of the lymphoplasmacytic type of inflammatory pseudotumor. During postoperative follow-up, chest computed tomography revealed a nodular lesion in the left segment 3 and an infiltrative lesion in the right segment 2. Left segment 3 segmentectomy and right segment 2 wedge resection were performed. The histopathological findings were similar to those of the first surgical specimen, leading to the diagnosis of recurrent lymphoplasmacytic type of inflammatory pseudotumor.
Surgical cases of recurrent inflammatory pseudotumor of the lung have been reported only very rarely. We believe that surgery is the best treatment for recurrent inflammatory pseudotumor of the lung when patients are eligible.
Inflammatory pseudotumor is a rare disease of unknown etiology. The histopathological correlates include a spectrum of fibroblastic or myofibroblastic proliferation with a varying infiltrate of inflammatory cells, including plasma cells, lymphocytes and histiocytes. Inflammatory pseudotumor of the lung is generally treated surgically, and the prognosis of patients undergoing complete surgical resection is favorable [1–7]. However, patients with recurrent inflammatory pseudotumor of the lung have rarely been reported and no effective treatment for such disease has been established. Here, we present a surgically treated case of intrapulmonary recurrence of inflammatory pseudotumor of the lung.
Inflammatory pseudotumor is comprised of a variable mix of inflammatory and mesenchymal cells, including plasma cells, histiocytes, lymphocytes and fibroblasts. Inflammatory pseudotumor usually arises within the lung, although it may also occur at many other sites . Most of the reported cases were in patients younger than 40 years of age, with a mean age of 27 - 50 years . Both genders are equally affected. No special geographic or ethnic predominance has been reported. The incidence of this lesion in the lung is between 0.04 and 1.0% in the general population [10–13]. Whether an inflammatory pseudotumor is a reactive lesion or a spontaneous neoplasm is controversial, and the precise etiology at present is still unknown. An IgG4-related immunopathological process has attracted attention as a cause of inflammatory pseudotumor in recent years ; however, in this patient, the serum IgG4 level was in the normal range, and the IgG4-positive plasma cells in the tumor were immunohistochemically few in number, excluding any association with IgG4-related sclerosing disease. Approximately half of these patients do not exhibit clinical symptoms, and the lesions are often detected incidentally on chest radiographs, while 26-56% of patients have been reported to show clinical symptoms, such as cough, hemosputum, dyspnea and chest pain .
The radiological features of inflammatory pseudotumors of the lung have been analyzed by Agrons et al. . Chest CT reportedly reveals a single nodule or mass in approximately 90% of these patients, with multiple nodules in 5% . Secondary infiltration of the hilum, mediastinum and airways is apparent in 16% of patients . PET/CT is usually positive, as in the present case . It is often difficult to make the diagnosis of inflammatory pseudotumor on preoperative imaging findings alone, as there is a highly complex differential diagnosis. Histology specimen obtained by bronchoscopic biopsy or transthoracic fine-needle aspiration are often equivocal. Hence, surgery, such as video-assisted thoracic resection or open lung biopsy, is often required [10, 16].
Matsubara et al. categorized three types of inflammatory pseudotumor based on the predominant cell types and main histological characteristics : (i) an organizing pneumonia type with gradual resolution of intra-alveolar exudates; (ii) a fibrous histiocytoma type with storiform proliferation of plasmacyte and lymphocyte aggregations; and (iii) a lymphoplasmacytic type with aggregation of both plasmacytes and lymphocytes, as in the case reported here.
The treatment of choice for pulmonary inflammatory pseudotumor is surgery [1–7]. Inflammatory pseudotumors of the lung usually do not recur after complete resection [3, 5]. The prognosis of patients who undergo radical resection is excellent [1–7]. Nevertheless, relapse can occur even many years after resection [6, 17], and disease-related deaths have been reported [6, 10, 18]. In a comparably large series of 23 surgically treated cases over 47 years, only three recurrences were reported, all due to incomplete resection . Two have had subsequent complete excision of enlarged residual tumors with no evidence of recurrence 8 and 9 years later . To our knowledge, this paper is the first report on the surgical resection of metachronous lesions in different parts of the lung, rather than residual tumor growth.
According to Cerfolio et al., patients with recurrent disease who are suitable for surgery should undergo reresection . Their long-term follow-up documented that complete resection leads to extended disease-free intervals. In our case, left S3 segmentectomy and right S2 wedge resection were performed for the purposes of both diagnosis and treatment. We selected surgery because (i) it was necessary to differentiate inflammatory pseudotumor from lung cancer, (ii) even if it was a recurrent inflammatory pseudotumor, there were only two lesions in the lung, and their resection was expected to afford long-term survival, and (iii) the patient did not wish to receive prolonged non-surgical treatment. It is reported that radiotherapy and steroids have each been employed for anatomically and functionally inoperable patients, and also in patients with recurrent disease [19–22]. However, the results of those treatments are widely variable, ranging from ineffective to complete regression [10, 20, 22]. A larger number of long-term follow-up cases will be needed to establish a clear treatment algorithm.
Written informed consent was obtained from the patient for the publication of this case presentation and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
positron emission tomography.
- Dehner LP: The enigmatic inflammatory pseudotumours: the current state of our understanding, or misunderstanding. J Pathol. 2000, 192: 277-279. 10.1002/1096-9896(200011)192:3<277::AID-PATH749>3.0.CO;2-E.View ArticlePubMedGoogle Scholar
- Fabre D, Fadel E, Singhal S, de Montpreville V, Mussot S, Mercier O, Chataigner O, Dartevelle PG: Complete resection of pulmonary inflammatory pseudotumors has excellent long-term prognosis. J Thorac Cardiovasc Surg. 2009, 137: 435-440. 10.1016/j.jtcvs.2008.07.009.View ArticlePubMedGoogle Scholar
- Janik JS, Janik JP, Lovell MA, Hendrickson RJ, Bensard DD, Greffe BS: Recurrent inflammatory pseudotumors in children. J Pediatr Surg. 2003, 38: 1491-1495. 10.1016/S0022-3468(03)00501-3.View ArticlePubMedGoogle Scholar
- Lee HJ, Kim JS, Choi YS, Kim K, Shim YM, Han J, Kim J: Treatment of inflammatory myofibroblastic tumor of the chest: the extent of resection. Ann Thorac Surg. 2007, 84: 221-224. 10.1016/j.athoracsur.2007.03.037.View ArticlePubMedGoogle Scholar
- Mandelbaum I, Brashear RE, Hull MT: Surgical treatment and course of pulmonary pseudotumor (plasma cell granuloma). J Thorac Cardiovasc Surg. 1981, 82: 77-82.PubMedGoogle Scholar
- Melloni G, Carretta A, Ciriaco P, Arrigoni G, Fieschi S, Rizzo N, Bonacina E, Augello G, Belloni PA, Zannini P: Inflammatory pseudotumor of the lung in adults. Ann Thorac Surg. 2005, 79: 426-432. 10.1016/j.athoracsur.2004.07.077.View ArticlePubMedGoogle Scholar
- 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. 10.1016/S1010-7940(03)00678-X.View ArticlePubMedGoogle Scholar
- Tan-Liu NS, Matsubara O, Grillo HC, Mark EJ: Invasive fibrous tumor of the tracheobronchial tree: clinical and pathologic study of seven cases. Hum Pathol. 1989, 20: 180-184. 10.1016/0046-8177(89)90183-4.View ArticlePubMedGoogle Scholar
- Agrons GA, Rosado-de-Christenson ML, Kirejczyk WM, Conran RM, Stocker JT: Pulmonary inflammatory pseudotumor: radiologic features. Radiology. 1998, 206: 511-518.View ArticlePubMedGoogle Scholar
- Cerfolio RJ, Allen MS, Nascimento AG, Deschamps C, Trastek VF, Miller DL, Pairolero PC: Inflammatory pseudotumors of the lung. Ann Thorac Surg. 1999, 67: 933-936. 10.1016/S0003-4975(99)00155-1.View ArticlePubMedGoogle Scholar
- Golbert ZV, Pletnev SD: On pulmonary "pseudotumours". Neoplasma. 1967, 14: 189-198.PubMedGoogle Scholar
- Ishida T, Oka T, Nishino T, Tateishi M, Mitsudomi T, Sugimachi K: Inflammatory pseudotumor of the lung in adults: radiographic and clinicopathological analysis. Ann Thorac Surg. 1989, 48: 90-95. 10.1016/0003-4975(89)90187-2.View ArticlePubMedGoogle Scholar
- Matsubara O, Tan-Liu NS, Kenney RM, Mark EJ: Inflammatory pseudotumors of the lung: progression from organizing pneumonia to fibrous histiocytoma or to plasma cell granuloma in 32 cases. Hum Pathol. 1988, 19: 807-814. 10.1016/S0046-8177(88)80264-8.View ArticlePubMedGoogle Scholar
- Zen Y, Kitagawa S, Minato H, Kurumaya H, Katayanagi K, Masuda S, Niwa H, Fujimura M, Nakanuma Y: IgG4-positive plasma cells in inflammatory pseudotumor (plasma cell granuloma) of the lung. Hum Pathol. 2005, 36: 710-717. 10.1016/j.humpath.2005.05.011.View ArticlePubMedGoogle Scholar
- Slosman DO, Spiliopoulos A, Keller A, Lemoine R, Besse F, Couson F, Townsend D, Rochat T: Quantitative metabolic PET imaging of a plasma cell granuloma. J Thorac Imaging. 1994, 9: 116-119. 10.1097/00005382-199421000-00013.View ArticlePubMedGoogle Scholar
- Kobashi Y, Fukuda M, Nakata M, Irei T, Oka M: Inflammatory pseudotumor of the lung: clinicopathological analysis in seven adult patients. Int J Clin Oncol. 2006, 11: 461-466. 10.1007/s10147-006-0611-4.View ArticlePubMedGoogle Scholar
- Lee HL, Kim L, Lee KH, Kim KH, Kim CW: A case of pulmonary inflammatory pseudotumor: Recurrence appearing as several consolidative lesions after complete resection. Korean J Intern Med. 2005, 20: 168-172.PubMed CentralView ArticlePubMedGoogle Scholar
- Urschel JD, Horan TA, Unruh HW: Plasma cell granuloma of the lung. J Thorac Cardiovasc Surg. 1992, 104: 870-875.PubMedGoogle Scholar
- Bando T, Fujimura M, Noda Y, Hirose J, Ohta G, Matsuda T: Pulmonary plasma cell granuloma improves with corticosteroid therapy. Chest. 1994, 105: 1574-1575. 10.1378/chest.105.5.1574.View ArticlePubMedGoogle Scholar
- Imperato JP, Folkman J, Sagerman RH, Cassady JR: Treatment of plasma cell granuloma of the lung with radiation therapy. A report of two cases and a review of the literature. Cancer. 1986, 57: 2127-2129. 10.1002/1097-0142(19860601)57:11<2127::AID-CNCR2820571107>3.0.CO;2-O.View ArticlePubMedGoogle Scholar
- Ishioka S, Maeda A, Yamasaki M, Yamakido M: Inflammatory pseudotumor of the lung with pleural thickening treated with corticosteroids. Chest. 2000, 117: 923-10.1378/chest.117.3.923.View ArticlePubMedGoogle Scholar
- Kishi K, Fujii T, Kurosaki A, Nakata K, Yoshimura K: Recurrence of inflammatory pseudotumor of the lung after eleven years of remission. Intern Med. 2009, 48: 1079-1083. 10.2169/internalmedicine.48.2043.View ArticlePubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.