Inflammatory Myofibroblastic Tumor of the Lung: A Report of a Rare Case

Abstract

The uncommon and mysterious pulmonary inflammatory myofibroblastic tumor (PIMT) primarily affects children and young people. PIMT is characterized by the proliferation of myofibroblastic spindle cells mixed with inflammatory cells. It can resemble both benign and malignant disorders, both radiographically and clinically. PIMT typically manifests as a solitary lung tumor. The genesis of the tumor is linked to genetic anomalies, including those related to the ALK gene (anaplastic lymphoma kinase); nonetheless, some cases are not ALK-positive, indicating genetic variability. Clinically, patients may have non-specific symptoms such as cough, chest pain, or hemoptysis, or they may not exhibit any symptoms at all. In these cases, imaging tests may unintentionally reveal unrelated conditions. From a histopathological perspective, PIMT is characterized by a heterogeneous cellular makeup, encompassing lymphocytes, myofibroblasts, plasma cells, and histiocytes, which generally exhibit a fascicular or storiform pattern. The diagnosis is verified using immunohistochemical labeling, molecular research, and histological examination. The cornerstone of treatment is still surgical resection, which has a good prognosis and a low recurrence rate. On the other hand, specific treatments, such as ALK inhibitors, have shown promise for incurable or recurring instances. Even though PIMT usually has a benign history, it is important to comprehend its biological behavior and molecular foundations for precise diagnosis and efficient management. This underscores the need for additional study into the pathophysiology and potential treatments of PIMT. This report presents a case of a 53-year-old female who presented with complaints of breathlessness and chest pain and was diagnosed with the condition accidentally.

Keywords: alk-1; imt; pleural; pseudotumor; pulmonary neoplasm.

Figure 1. MRI image showing evidence of a well-defined T2 hyperintense lesion (blue arrow) in the upper lobe of the left lung.

Figure 2. Photomicrograph (10x magnification) showing spindle cells arranged in fascicular arrangement with a surrounding population of lymphocytes and plasma cells on hematoxylin and eosin stain.

Figure 3. Photomicrograph (40x magnification) showing inflammatory cells with enlarged cells having spindle-shaped nuclei on hematoxylin and eosin stain.