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[Inflammatory pseudotumors of the lung with severe course]
F Girard1, M Kambouchner, S Maugendre
1Fédération des maladies respiratoires et thoraciques, Hôpital Avicenne, CHU Bobigny, 93009 Bobigny. frederic.girard@avc.ap-hop-paris.fr
Abstract:
Pulmonary inflammatory pseudotumors are usually unique lesions of unknown etiology with good prognosis. We report two severe cases with mediastinal invasion, local recurrence, extrathoracic locations, one of them with a fatal evolution. Certain microscopic features, which were present in our cases (increased cellularity, nuclear pleomorphism, mitotic activity, focal necrosis, bizarre giant cells, vascular invasion), may have prognostic relevance in determining an aggressive behavior of these tumors. Surgical resection is the recommended treatment, and incomplete resection, as in our cases, seems to be a risk factor for developing recurrent inflammatory pseudotumor. Immunosuppressive therapy was ineffective as well as radiotherapy in one of our cases. Only high doses of corticosteroids seemed to slow the evolution of the disease.
Insights
Pulmonary inflammatory pseudotumors, typically benign, can exhibit aggressive behavior. Microscopic features like increased cellularity and bizarre giant cells may indicate poor prognosis, necessitating aggressive surgical management.
Area of Science:
- Pulmonology
- Pathology
- Oncology
Background:
- Pulmonary inflammatory pseudotumors (PIPs) are rare, typically solitary lesions of uncertain origin with a generally favorable prognosis.
- This study investigates two severe PIP cases exhibiting aggressive characteristics.
Observation:
- Cases presented with mediastinal invasion, local recurrence, and extrathoracic spread.
- One patient experienced a fatal outcome, highlighting the potential for severe disease progression.
Findings:
- Specific microscopic features, including increased cellularity, nuclear pleomorphism, mitotic activity, necrosis, giant cells, and vascular invasion, were noted.
- These features may serve as prognostic indicators for aggressive PIP behavior.
Implications:
- Incomplete surgical resection appears to be a significant risk factor for recurrence.
- While immunosuppressive therapy and radiotherapy were ineffective, high-dose corticosteroids showed potential in slowing disease progression.