We reported an IMT located in the right lung and mediastinum, which was treated with X-beam irradiation and cisplatin after subtotal resection.
IMT is a rare tumor and primarily occurs in soft tissues. Usually, IMT presents as a nodular circumscribed mass, occupying lesion, multinodular lesions in the soft tissue of chest, abdominopelvic or retroperitoneal region . The definitive diagnosis of IMT is mainly based on the histopathological report. In this case, the presence of CD163, CD68 SMA, CD34, and Vimentin and the absence of ALK, S-100, Desmin, CD23, STAT6, PAS, PASM, and acid-fast staining associated with the mix of spindle cells and inflammatory cells helped the histopathological diagnosis.
Because IMT always presents local invasiveness, thus local therapy plays an important role, especially surgical resection . Besides, complete surgical resection is the standard treatment for localized IMT. However, local recurrences are common with incomplete resection, which usually occurs in the presence of involved surgical margins . Typically, about 23–25% of IMT recurred after surgery [12, 13]. Nonetheless, a second surgery, radiotherapy, or chemotherapy regimen can be effective in recurrent IMT [14, 15].
Because of the inflammatory features of IMT, anti-inflammatory drugs, such as steroids and non-steroidal anti-inflammatory drugs (NSAIDs), were the first applied in the treatment. However, different cases reported opposite results of anti-inflammatory drugs [16, 17]. Therefore, the effect of anti-inflammatory drugs in IMT might depend on individual patient differences and tumor heterogeneity.
Chemotherapy is reportedly effective in IMT. Moreover, it is used in neoadjuvant therapy and postoperative adjuvant therapy for unresectable, progressive, or metastatic disease. Multiple chemotherapy regimens have been described as functional, including ifosfamide, carboplatin, vincristine and dactinomycin (IVA), paclitaxel, vincristine, methotrexate and vinblastine (MTX/VBL), and vinorelbine (MTX/VNB) [2, 3]. The development of targeted drugs shows a good therapeutic effect in the IMT treatment directed towards ALK, PDGFR β, ROS1, NTRK, RET, and other molecular targets [2–4]. Thus, molecular landscape targeted therapeutics might play a more critical role in IMT.
Radiotherapy also showed effectiveness in IMT [18–21]. It may be a choice for localized IMT patients who cannot tolerate surgery or could be used as postoperative adjuvant therapy in unresectable, progressive, or metastatic cases. However, there is no standardized radiation dose and combined drug therapy regimen.
It was reported that Low-dose radiation therapy (2000cGy) combined with steroid therapy treatment for IMT in the skull base showed a high recurrence rate . Nevertheless, low-dose radiation therapy (2000cGy) treatment for IMT in orbit showed good effect . It seems that the radiation dose might vary based on the tumor's location. Reference the radiotherapy dose for common tumors in the same position might be suitable. However, it remains unknown whether radiotherapy should combine chemotherapy or steroid treatment. The last is usually offered as a combined therapy [19, 20, 23]. Chemotherapy, especially cisplatin, might sensitize the patient for the radiation and improve the radiotherapy effect . For high-risk patients, chemotherapy combined with radiotherapy might be an intensive treatment.
In our case, surgery and radiochemotherapy played an essential role in treating this patient. Despite the local progress and subtotal resection, the progression-free survival (PFS) was more than 48 months. We first reported surgery and high-dose radiotherapy combined with chemotherapy treatment for local advanced IMT. In conclusion, surgery is the first choice for IMT, concurrent radiochemotherapy may be considered for local progress, local recurrence, and nonresectable IMT patients.