Bhawani Jayashree, Shukla Samarth, Acharya Sourya
Department of Pathology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND.
Department of Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND.
Cureus. 2024 Jul 5;16(7):e63892. doi: 10.7759/cureus.63892. eCollection 2024 Jul.
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 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.
罕见且神秘的肺部炎性肌纤维母细胞瘤（PIMT）主要影响儿童和年轻人。PIMT的特征是肌纤维母细胞梭形细胞与炎性细胞混合增生。在影像学和临床上，它既可能类似良性疾病，也可能类似恶性疾病。PIMT通常表现为孤立性肺肿瘤。肿瘤的发生与基因异常有关，包括与间变性淋巴瘤激酶（ALK）基因相关的异常；然而，一些病例并非ALK阳性，这表明存在基因变异性。临床上，患者可能有咳嗽、胸痛或咯血等非特异性症状，也可能根本没有任何症状。在这些情况下，影像学检查可能会意外发现无关的病症。从组织病理学角度来看，PIMT的特征是细胞组成异质性，包括淋巴细胞、肌成纤维细胞、浆细胞和组织细胞，通常呈现束状或席纹状模式。通过免疫组化标记、分子研究和组织学检查来确诊。治疗的基石仍然是手术切除，其预后良好且复发率低。另一方面，针对无法治愈或复发的病例，特定治疗方法，如ALK抑制剂，已显示出前景。尽管PIMT通常病程良性，但了解其生物学行为和分子基础对于准确诊断和有效管理很重要。这凸显了对PIMT病理生理学和潜在治疗方法进行更多研究的必要性。本报告介绍了一例53岁女性病例，该患者因呼吸急促和胸痛就诊，意外被诊断为此病。
