Diagnostic and Therapeutic Challenges of a Large Pleural Inflammatory Myofibroblastic Tumor

Abstract

We report a 48-year-old woman with a pleural pseudoneoplasm requiring different diagnostic and therapeutic strategies. After initial presentation with increasing dyspnoea, temperature, dry cough, and interscapular pain diagnostic processing showed a large mediastinal mass with marked pleural effusion and high metabolic activity in the 18F-FDG-PET/CT. Extensive CT-guided biopsy of the tumor reaching from the visceral pleura into the right upper lobe revealed no malignancy, but a marked inflammatory tissue reaction containing foam cells. Initial empiric antibiotic therapy was temporarily successful. However, in the further course the mass relapsed and was resistant to antibiotics and a corticosteroid trial. With the working hypothesis of an inflammatory myofibroblastic tumor the patient underwent surgical tumor resection, finally confirming the suspected diagnosis. Due to residual disease intravenous immunoglobulins were administered leading to sustained response. This case with a pleural localisation of a large inflammatory pseudotumor with responsiveness to immunomodulation after incomplete resection extends the reported spectrum of thoracopulmonary manifestations of this rare entity.

Common Presentation of Uncommon Disease: Inflammatory myofibroblastic tumor of the lung, case report

“…The clinical and radiological manifestations are varied and non-specific. Therefore, it is difficult to establish the diagnosis unless surgical resection is performed [ 7 ]. They are usually asymptomatic and discovered incidentally as an abnormal chest radiograph, perhaps with nonspecific respiratory symptoms such as cough, chest pain, dyspnea, hemoptysis, and nonspecific inflammatory symptoms such as fever, malaise, and weight loss [ 1 , 2 , 6 ].…”

Inoperable inflammatory myofibroblastic tumour of the para-nasal sinuses and orbit with recurrence responding to methotrexate and prednisolone: a case report

“…Non-steroidal anti-inflammatory drugs used alone has also demonstrated success [ 26 ]. Immunomodulation with intravenous immunoglobulin therapy has been tried with success for post-surgical residual tumour with successful long term remission [ 27 ]. Another novel therapy under evaluation is the ALK inhibitor crizotinib, which showed long term partial response in those who were ALK positive [ 28 ].…”

Inflammatory Myofibroblastic Tumour: Report of a Rare Form with Exclusive Pleural Involvement

“…Pleural involvement has been described but occurs as extension of the pulmonary IMT. One case of possible exclusive pleural involvement has been recently described [ 5 ]. But, in contrast to our case, where the mass is strictly pleural, in Loeffler-Ragg's report there was a mediastinal mass with extension to the pleura.…”

“…Chromossomic clonal anomalies, histological transformation, and metastasis have been described in case reports, and a recurrence rate as high as 25% has been observed [ 3 ]. We report a form of IMT with exclusive pleural involvement that illustrates the complex differential diagnosis of this entity [ 4 , 5 ].…”