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Pulmonary inflammatory pseudotumor causing lung collapse responding to corticosteroid therapy.

ABSTRACT: Pulmonary inflammatory pseudotumor (PIP) is a rare benign tumor that represents less than one percent of all tumors found in the lungs. Despite the benign etiology, PIP can cause significant clinical problems due to its growth rate and potential to compromise adjacent pulmonary and thoracic structures. Complete surgical resection is the preferred therapy for PIP to prevent recurrence, however, this is not possible in some patients due to the size or location of the tumor. We present the case of an 18 year-old male presenting with PIP in the proximal left mainstem bronchus causing complete left lung collapse. Surgical resection was not possible due to tumor location, and therefore the patient was treated with corticosteroids with marked response. Corticosteroid use has for PIP has been described in few other situations, and this case demonstrates the potential for this therapeutic option in patients with PIP who have a contraindication to surgical resection.

SUBMITTER: Reddy RZ

PROVIDER: S-EPMC6010639 | biostudies-literature | 2018

REPOSITORIES: biostudies-literature

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Pulmonary inflammatory pseudotumor causing lung collapse responding to corticosteroid therapy.

Reddy Radhika Z RZ Carter Yvonne M YM Hsia David W DW

Respiratory medicine case reports 20180503

Pulmonary inflammatory pseudotumor (PIP) is a rare benign tumor that represents less than one percent of all tumors found in the lungs. Despite the benign etiology, PIP can cause significant clinical problems due to its growth rate and potential to compromise adjacent pulmonary and thoracic structures. Complete surgical resection is the preferred therapy for PIP to prevent recurrence, however, this is not possible in some patients due to the size or location of the tumor. We present the case of ...[more]

PMID: 29977776

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Project description:BackgroundResistin-like molecule alpha or found in inflammatory zone protein (Fizz1) is increased in pulmonary epithelial cells and also in limited amounts by other lung cells during various lung injuries and fibrosis. However, the direct role of Fizz1 produced in the pulmonary epithelium has not been determined.MethodsFizz1 Transgenic mice (CCSP/Fizz1) were generated that overexpress Fizz1 in the lung epithelium under the control of a doxycycline (Dox) inducible lung epithelial cell specific promoter Scgb1a1 (Clara cell secretory protein, CCSP). Histology and FACS analysis of lung cells were used to identify the direct effects of Fizz1 in the transgenic mice (Dox treated) when compared with control (CCSP/-) mice. Intratracheal bleomycin sulfate or silica in saline and saline alone were used to study the role of Fizz1 during bleomycin- and silica-induced pulmonary fibrosis in CCSP/Fizz1 and CCSP/- mice. Weight change, pulmonary inflammation, and fibrosis were assessed 10 days post bleomycin or 28 days post silica challenge.ResultsWhen CCSP/Fizz1 mice were fed Dox food, elevated Fizz1 protein was detected in lung homogenates by western blot. Lungs of mice in which Fizz1 was induced in the epithelium contained increased lung cells staining for CD11c and F4/80 by FACS analysis consistent with increased dendritic cells however, no changes were observed in the percentage of interstitial macrophages compared to CCSP/- controls. No significant changes were found in the lung histology of CCSP/Fizz1 mice after up to 8 weeks of overexpression compared to CCSP/- controls. Overexpression of Fizz1 prior to challenge or following challenge with bleomycin or silica did not significantly alter airway inflammation or fibrosis compared to control mice.ConclusionsThe current study demonstrates that epithelial cell derived Fizz1 is sufficient to increase the bone-marrow derived dendritic cells in the lungs, but it is not sufficient to cause lung fibrosis or alter chemical or particle-induced fibrosis.

Dataset Information

Pulmonary inflammatory pseudotumor causing lung collapse responding to corticosteroid therapy.

Publications

Pulmonary inflammatory pseudotumor causing lung collapse responding to corticosteroid therapy.

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