1. Clinical Case DIEGO BONILLA M.D.

2. 71 yo remote smoking history 2 months ago self-resolving flu-like illness Followed by persistent dry cough & 30 lb weight loss

8. 3.7 x 3.6 cm spiculated pulmonary mass + Speckled calcifications. + Rt Hilar & Rt paratracheal LN (>1cm) PET Scan SUV 7.2

9. What is the next step? What is the differential?

10. Bronchoscopy Normal airways. Bronchial brush and BAL of superior segment of RLL, posterior segment of RUL and RML were collected and sent for cytology examination. Pathology was negative for cancer

11. Sampled Nodes 4R, 4L and 7 Cervical Mediastinoscopy Pathology was negative for cancer

12. Frozen section (per surgical note) consistent with adenocarcinoma Final pathology showed that specimens from the chest wall, RUL and right rib were benign fibro-connective and adipose tissue and Level 10 and 11 lymph nodes were reactive nodes negative for tumor. VATS resection of RUL

13. Results consistent with inflammatory pseudotumor. Post lung resection patient had SOB. His quality of life was affected. Patient was upset about “this unnecessary procedure” for a nonmalignant tumor. Was the lung mass resection necessary?

14. Inflammatory Pseudotumors Inflammatory pseudotumors of the lung are uncommon pathologic findings and account for 1% of all pulmonary tumors. It is generally a benign histologic process, but can demonstrate characteristics that mimic malignant lesions, such as invasion of pleural, mediastinal, hilar, and vertebral structures. Recurrence and metastasis have also been reported. At the Mayo Clinic, 56,400 thoracic procedures were performed during more than 47 years, of which 23 (0.04%) were for an inflammatory pseudotumor of the lung

15. Pseudotumors are categorized into three subtypes: 1) Organizing pneumonia 2) Fibrous histiocytoma 3) Lymphoplasmacytic. Several features are common to all subtypes and include proliferation of spindle-shaped fibroblasts, granulomatous inflammation, lymphoid hyperplasia, fibrosis, and invasion of lymphocytes. Significant overlap among the three subtypes occurs. Inflammatory Pseudotumors

16. Cerfolio RJ, Allen MS, Nascimento AG, et al. Inflammatory pseudotumors of the lung. Ann Thorac Surg 1999; 67:933–936

17. Presentation: as belief that may arise from organizing pneumonia. 1/3 cases preceded by infective symptoms. Several reports linking infection to pseudotumors have included viral, bacterial, rickettsial, and fungal etiologies. Radiographic Appearances: Variable - Solitary peripheral nodule or Multiple - calcification & cavitation rare finding. - pleural thickening, adenopathy, endobronchial lesions. Inflammatory Pseudotumors

18. Surgical resection is the treatment of choice. Needle biopsy or transthoracic biopsy are likely to show fibrosis or inflammation and may not exclude a malignant lesion. Corticosteroids have been used for treatment with report of decreased size or regression. No randomized controlled trials have been reported. Overall mortality is low, with an overall 5-year survival of 91% in one surgical series of 23 patients. Inflammatory Pseudotumors

19. Clinical Pearls 1. Inflammatory pseudotumors are rare, benign histologic lesions that can demonstrate aggressive behavior clinically. 2. Patients may be asymptomatic or present with symptoms suggestive of a respiratory infection. 3. Radiographic findings are usually a solitary nodule or mass, but can also demonstrate a variety of abnormalities, including pleural or endobronchial disease. 4. Preoperative diagnosis is rarely made, and the treatment of choice is surgical resection. Prognosis is good. 5. Steroids may be useful when surgery is contraindicated.

20. References Agrons GA, Rosado-de-Christenson ML, Kirejczyk WM, et al. Pulmonary inflammatory pseudotumor: radiologic features. Radiology 1998; 206:511–518 Bando T, Fujimura M, Noda Y, et al. Pulmonary plasma cell granuloma improves with corticosteroid therapy. Chest 1994; 105:1574–1575. Cerfolio RJ, Allen MS, Nascimento AG, et al. Inflammatory pseudotumors of the lung. Ann Thorac Surg 1999; 67:933–936 Diez Pina JM, Fenandez VE, Saez RG, et al. Multifocal inflammatory pseudotumor of the lung with good response to corticoids. Arch Bronchoneumol 1998; 34:102–104 Giulio Melloni, MD, Angelo Carretta, MD, Paola Ciriaco, MD, Gianluigi Arrigoni, MD,Stefano Fieschi, MD, Nathalie Rizzo, MD, Edgardo Bonacina, MD. Inflammatory Pseudotumor of the Lung in Adults The Annals of Thoracic Surgery, Volume 79, Issue 2, February 2005, Pages 426-432 Ishioka S, Akihiro M, Yamasaki M, et al. Inflammatory pseudotumor of the lung with pleural thickening treated with corticosteroids [letter]. Chest 2000; 117:923 Juliette L. Wohlrab, MD; and Eric D. Anderson, MD, FCCP. Two of a Kind.Diverse Presentations of the Same Disease. Maier HC, Sommers SC. Recurrent and metastatic pulmonary fibrous histiocytoma/plasma cell granuloma in a child. Cancer 1987; 60:1073–1076. Matsubara O, Tan-Liu N, Kenney R, 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 Williams ME, Longmaid HE, Trey G, et al. Renal failure resulting from infiltration by inflammatory myofibroblastic tumor responsive to corticosteroid therapy. Am J Kidney Dis 1998; 31:E5.