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Inflammatory Pseudotumor of the Pleura

Girdhar, Ankur MD; Singh, Amita MD; Bajwa, Abubakr MD; Shujaat, Adil MD

Journal of Bronchology & Interventional Pulmonology: April 2014 - Volume 21 - Issue 2 - p 154–157
doi: 10.1097/LBR.0000000000000070
Case Reports

Inflammatory pseudotumors are rare solid, non-neoplastic masses that can mimic pulmonary malignancy. It occurs most commonly in children and young adults and is usually found incidentally. There are many reports of the existence of this tumor in various organs in the human body. The occurrence of this tumor exclusively in the pleura has not been described before. We present a case of inflammatory pseudotumor of the pleura and its successful management.

Division of Pulmonary and Critical Care Medicine, UF College of Medicine, Jacksonville, FL

Disclosure: There is no conflict of interest or other disclosures.

Reprints: Ankur Girdhar, MD, Division of Pulmonary and Critical Care Medicine, UF College of Medicine, 655, 8th Street West, Jacksonville, FL 32209 (e-mail:

Received June 4, 2013

Accepted March 5, 2014

Inflammatory pseudotumor (IPT) or inflammatory myofibroblastic tumor of the lung, which was first described in 1939, is an extremely rare condition.1 It is an inflammatory, well circumscribed, unencapsulated, and non-neoplastic process characterized by the unregulated growth of inflammatory cells.2 The existing literature describes this tumor to be occurring most commonly in young age.3 There are case reports of pleural thickening in association with an IPT of lung parenchyma.4 To our knowledge there has been no reported cases of IPT in the elderly and exclusively involving the pleura.

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A 74-year-old female presented with complaints of mild left-sided chest heaviness. In the last 1 month before the presentation she had been complaining of progressive dyspnea on exertion. The chest x-ray and computed tomography of the chest revealed a large left-sided pleural effusion with the possibility of a pleural mass (Figs. 1, 2). Laboratory work-up was significant for mild leukocytosis, high C-reactive protein, normal renal function, and mild proteinuria. The patient subsequently underwent a thoracentesis which showed that the pleural fluid was an exudate. Pleural fluid cytology was negative for malignant cells. She was also found to have positive antinuclear antibody and high anti–double-stranded DNA antibody levels.





With a working diagnosis of pleuritis secondary to a malignant versus inflammatory process, medical thoracoscopy was planned to drain the rest of the fluid and to visualize the pleura. She was found to have a well-circumscribed hyperpigmented, soft pleural mass involving the parietal pleura (Fig. 3). There was no associated pulmonary parenchymal abnormality. Multiple biopsies were obtained from this mass that revealed chronic inflammation with reactive mesothelial cells, spindle-shaped myofibroblasts, and some plasma cells with no signs of malignancy.



Considering the entire clinical scenario of the patient, supplemented with histopathologic findings, she was given a final diagnosis of IPT of the pleura secondary to the new-onset systemic lupus erythematosus. She underwent a video-assisted thoracoscopic surgical (VATS) excision of the pleural mass. In addition she was started on high-dose steroids. On a follow-up at 3 months there was near complete resolution of both pleural effusion and proteinuria.

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IPT is a rare, non-neoplastic lesion and is a result of unregulated inflammation.2 Current knowledge about prevalence of pulmonary IPT suggests that it is found in 0.7% of lung tumors and 0.04% in general thoracic surgery procedures.5,6 It is a tumor that is predominantly found at younger age with most of the cases reported in patient below 40 years of age without any sex or race bias.3 Pathogenesis of IPT remains unclear with some authors suggesting it to be primarily an inflammatory process, whereas others describing it as a low-grade malignant lesion with a dominant inflammatory component.7,8 The inciting trigger for the inflammation is equally ambiguous with proposed etiologies as metabolic disturbances or dysfunctional cytokine response cascade to pulmonary infections. Many reported cases have had a history of infection with various pathogens such as Mycobacteria, Epstein Barr virus, and Pseudomonas.7,9,10

IPT has been reported to occur at multiple extrathoracic sites.11 In the thorax it commonly involves the lower lobes of the lungs and occasionally the adjoining pleura or mediastinal lymph nodes.12,13

There have been many documented cases of calcifying fibrous tumors of the pleura that were initially thought to be the end stage of an IPT.14–16 Recently studies have shown that these maybe 2 separate entities based on their immune expression.17,18 In addition there have been reported cases of IPT of the lung presenting with pleural thickening, some of which were found intraoperatively.4,19 To our knowledge there has been no previous recorded case of IPT involving exclusively the pleura.

The clinical presentation of IPT is variable with nonspecific symptoms attributed either to the inflammation in the lesion or the pressure effect.12,13 Patients with IPT of the lung present with cough, chest pain, dyspnea, hemoptysis, and fever. Nail clubbing and hypertropic osteoarthropathy has also been reported.20 Because of the lack of literature on IPT involving only the pleura we will have to extrapolate the information obtained from the cases with pleural involvement from the pulmonary IPT. Exudative pleural effusion rich in cellular elements with pleuritic chest pain may be the most common presentation. Our patient had only mild symptoms that progressed slowly before medical assistance was sought.

Laboratory investigations also may show findings pointing to an ongoing inflammation in the body. There may be elevated C-reactive protein, erythrocyte sedimentation rate, anemia, thrombocytosis, and hypergammaglobulnemia.13 The high levels of double-stranded DNA in our patient was thought to be due to the large size of the tumor and associated inflammation.

Radiologically pleural pseudotumors appear as a solitary or multiple nodular abnormalities.13,21,22 Unlike calcifying fibrous tumor of the pleura where there is extensive calcification easily visualized on imaging, there is scant calcification in the inflammatory myofibroblastic pseudotumor that might get missed.22 IPT of the lung has predilection for the lower lobes and therefore will cause pleural thickening in the adjacent pleura.13 In the case we are reporting we observed the pleural mass to be close to the left lower lobe and in the proximity to the diaphragmatic pleura (Fig. 3).

Semi-invasive diagnostic procedures such as bronchoscopy and percutaneous fine-needle aspiration biopsy have been considered insufficient for the diagnosis of lung IPTs.4,23 Video-assisted thoracoscopic surgery with complete excision of the mass helps in establishing a confirmatory diagnosis.24,25 Our case demonstrates that if there is pleural involvement, primary or secondary, it may be amenable to the diagnosis with less invasive medical thoracoscopy. We were able to get ample amount of tissue specimen through the medical thoracoscopy to help make a histopathologic diagnosis.

As IPT of the lungs do have malignant component with potential to recur, complete surgical resection has been recommended as the best treatment to achieve cure.26,27 Complete tumor excision and tumor size of ≤3 cm are factors associated with a decreased risk of recurrence.28,29 In our patient a VATS-assisted excision of the pleural mass was performed and the patient was started on corticosteroids. The latter treatment decision was based on certain case reports,30,31 which have shown that corticosteroids may have a role in preventing recurrences of IPT. Nonsurgical treatment modalities like radiation, chemotherapy, and steroids can also help manage IPT in patients who are poor surgical candidates or have multiple nodules or unresectable disease.4,32

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To the best of our knowledge there are no case reports in the literature of diagnosis of IPT exclusively involving the inflamed pleura. Our case highlights that with the help of medical thoracoscopy we might be able to diagnose this rare pleural pathology before patients are subjected to more invasive procedures.

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1. Copin MC, Gosselin BH, Ribet ME.Plasma cell granuloma of the lung: difficulties in diagnosis and prognosis.Ann Thorac Surg.1996;61:1477–1482.
2. Morar R, Bhayat A, Hammond G, et al..Inflammatory pseudotumour of the lung: a case report and literature review.Case Rep Radiol.2012;2012:214528doi: 10.1155/2012/214528.
3. Kim JH, Cho JH, Park MS, et al..Pulmonary inflammatory pseudotumor-a report of 28 cases.Korean J Intern Med.2002;17:252–258.
4. Ishida T, Oka T, Nishino T, et al..Inflammatory pseudotumor of the lung in adults: radiographic and clinicopathological analysis.Ann Thorac Surg.1989;48:90–95.
5. Singh RS, Dhaliwal RS, Puri D, et al..Inflammatory pseudotumor of the lung: report of a case and review of literature.Indian J Chest Dis Allied Sci.2001;43:231–234.
6. Cohen MC, Kaschula RO.Primary pulmonary tumors in childhood: a review of 31 years’ experience and the literature.Pediatr Pulmonol.1992;14:222–232.
7. Kovach SJ, Fischer AC, Katzman PJ, et al..Inflammatory myofibroblastic tumors.J Surg Oncol.2006;94:385–391.
8. Yi E, Aubry MC.Pulmonary pseudoneoplasms.Arch Pathol Lab Med.2010;134:417–426.
9. Lewis JT, Gaffney RL, Casey MB, et al..Inflammatory pseudotumor of the spleen associated with a clonal Epstein-Barr virus genome: case report and review of the literature.Am J Clin Pathol.2003;120:56–61.
10. Arul P, Varghese RG, Ramdas A.Pleural tuberculosis mimicking inflammatory pseudotumour.J Clin Diagn Res.2013;7:709–711.
11. Anthony PP.Inflammatory pseudotumour (plasma cell granuloma) of lung, liver and other organs.Histopathology.1993;23:501–503.
12. Agrons GA, Rosado-de-Christenson ML, Kirejczyk WM, et al..Pulmonary inflammatory pseudotumor: radiologic features.Radiology.1998;206:511–518.
13. Narla LD, Newman B, Spottswood SS, et al..Inflammatory pseudotumor.Radiographics.2003;23:719–729.
14. Fetsch JF, Montgomery EA, Meis JM.Calcifying fibrous pseudotumor.Am J Surg Pathol.1993;17:502–508.
15. Pomplun S, Goldstraw P, Davies SE, et al..Calcifying fibrous pseudotumour arising within an inflammatory pseudotumour: evidence of progression from one lesion to the other?Histopathology.2000;37:380–382.
16. Van Dorpe J, Ectors N, Geboes K, et al..Is calcifying fibrous pseudotumor a late sclerosing stage of inflammatory myofibroblastic tumor?Am J Surg Pathol.1999;23:329–335.
17. Hill KA, Gonzalez-Crussi F, Chou PM.Calcifying fibrous pseudotumor versus inflammatory myofibroblastic tumor: a histological and immunohistochemical comparison.Mod Pathol.2001;14:784–790.
18. Sigel JE, Smith TA, Reith JD, et al..Immunohistochemical analysis of anaplastic lymphoma kinase expression in deep soft tissue calcifying fibrous pseudotumor: evidence of a late sclerosing stage of inflammatory myofibroblastic tumor?Ann Diagn Pathol.2001;5:10–14.
19. Rasmussen H, Graudal N, Horn T, et al..Spontaneous regression of a pleural thickening with the histological appearance of an inflammatory pseudotumour.Virchows Arch A Pathol Anat Histopathol.1989;414:253–255.
20. Pichler G, Eber E, Thalhammer G, et al..Arthralgia and digital clubbing in a child: hypertrophic osteoarthropathy with inflammatory pseudotumours of the lung.Scand J Rheumatol.2004;33:189–191.
21. Sleigh KA, Lai W, Keen CE, et al..Calcifying fibrous pseudotumours: an unusual case with multiple pleural and mediastinal lesions.Interact Cardiovasc Thorac Surg.2010;10:652–653.
22. Mito K, Kashima K, Daa T, et al..Multiple calcifying fibrous tumors of the pleura.Virchows Arch.2005;446:78–81.
23. Mandelbaum I, Brashear RE, Hull MT.Surgical treatment and course of pulmonary pseudotumor (plasma cell granuloma).J Thorac Cardiovasc Surg.1981;82:77–82.
24. Berardi RS, Lee SS, Chen HP, et al..Inflammatory pseudotumors of the lung.Surg Gynecol Obstet.1983;156:89–96.
25. Alexiou C, Obuszko Z, Beggs D, et al..Inflammatory pseudotumors of the lung.Ann Thorac Surg.1998;66:948–950.
26. Bahadori M, Liebow AA.Plasma cell granulomas of the lung.Cancer.1973;31:191–208.
27. Matsubara O, Tan-Liu NS, Kenney RM, et al..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.
28. Huellner MW, Schwizer B, Burger I, et al..Inflammatory pseudotumor of the lung with high FDG uptake.Clin Nucl Med.2010;35:722–723.
29. Husong JW, Brown M, Perkins L, et al..Comparison of DNA ploidy, histologic, and immunohistochemical findings with clinical outcome in inflammatory myofibroblastic tumors.Mod Pathol.1999;12:279–286.
30. Ishioka S, Maeda A, Yamasaki M, et al..Inflammatory pseudotumor of the lung with pleural thickening treated with corticosteroids.Chest.2000;117:923doi: 10.1378/chest.117.3.923.
31. Fang FC, Lee SC, Hsu HH, et al..Inflammatory myofibroblastic tumor of the lung: unusual presentation.Lung.2008;186:191–193.
32. Fornell-Perez R, Santana-Montesdeoca JM, Garc´ia-Villar C, et al..Two types of presentation of pulmonary inflammatory pseudotumors.Arch Bronconeumol.2012;48:296–299.

medical thoracoscopy; pseudotumor; pleural effusion

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