A Rare Case of Inflammatory Myofibroblastic Tumor Appearing Left Lower Lung
Location
D.P. Culp Center Ballroom
Start Date
4-5-2024 9:00 AM
End Date
4-5-2024 11:30 AM
Poster Number
69
Name of Project's Faculty Sponsor
Devapiran Jaishankar
Faculty Sponsor's Department
Internal Medicine
Competition Type
Competitive
Type
Poster Presentation
Presentation Category
Health
Abstract or Artist's Statement
Inflammatory myofibroblastic tumor (IMT) was first described in 1939 by Bunn. Umiker et al. coined the term “IMT." IMT manifests in various anatomical sites, spanning the lungs, bladder, spleen, breast, pancreas, liver, colon, spermatic cord, prostate, peripheral nerves, soft tissues, and orbit. A 36-year-old female patient with prior granulomatous inflammation and fibrosis of the left lung noted on wedge resection 5 years ago presented with left chest discomfort. CT chest revealed postoperative changes left lower lobe (LLL) lung and a new lesion measuring 4.1x1.8 cm. PET-CT scan confirmed a hyper metabolic mass (SUV max 6.02) in the LLL Lung CT guided needle biopsy demonstrated spindle cell and inflammatory proliferation, favoring inflammatory myofibroblastic tumor (Immunostaining revealing SMA positive, Desmin negative and ALK negative). Patient underwent a left thoracoscopic lower lobectomy with mediastinal lymph node dissection (levels 5, 7, 9, 10, 11), pathology indicated a 4 cm granulomatous inflammatory mass with abundant fibrosis, no evidence of neoplastic changes and multifocal distribution of granulomas. The differential included rheumatological disorders, infections, allergic disorders, and aspiration. Lingular findings included multifocal patchy interstitial fibrosis. All lymph nodes were reactive and negative for malignancy. Level 11 lymph node replaced by old, calcified granuloma-negative for AFB, GMS/Fungal gram stains. Patient recovered well from surgery and is under active surveillance. IMT predominantly described in children, constituting the most frequent primary lung lesion in pediatric patients is seldom reported in adults. IMTs represent from 0.04% to 0.1% of all pulmonary tumors. They can be asymptomatic or present with cough, hemoptysis, dyspnea, and pleuritic pain. Approximately 15-30% of patients may experience systemic symptoms like fever, weight loss, malaise, and fatigue. Some patients may have recurrent pneumonias. Pulmonary IMTs are more commonly found in the lower lobes. The exact cause of IMT remains uncertain. Recent research indicates IMT is linked to rearrangements in the anaplastic lymphoma kinase (ALK) gene, along with increased expression of ALK protein. Additionally, the condition involves gene fusions such as ROS1, NTRK3, and RET. Immunohistochemistry stains often reveal Vimentin, smooth muscle actin (SMA), and ALK (40% of the time) positive on the spindle tumor cells. Complete surgical resection is the most important treatment for IMT, and long-term follow-up is necessary to detect local recurrence. The prognosis of PIMT patients who undergo early surgical treatment is usually excellent, and adjuvant chemotherapy treatments are not required. Reoperation/resection is the mainstay of therapy for local recurrence. Chemotherapy proves beneficial in managing advanced IMT with sarcomatoid features. Reports indicate the utilization of gene-targeted medications in IMT treatment. The US National Comprehensive Cancer Network (NCCN) advocates for crizotinib as the gold standard for ALK-positive IMT care. Additionally, ceritinib, a second-generation ALK inhibitor, has demonstrated efficacy in IMT management. Pulmonary IMT is a rare tumor. The primary treatment method is surgery, with reoperation recommended for local recurrence. Chemotherapy may be an effective option for advanced IMT with sarcomatoid features. Targeted therapy can be applied for ALK-positive IMT.
A Rare Case of Inflammatory Myofibroblastic Tumor Appearing Left Lower Lung
D.P. Culp Center Ballroom
Inflammatory myofibroblastic tumor (IMT) was first described in 1939 by Bunn. Umiker et al. coined the term “IMT." IMT manifests in various anatomical sites, spanning the lungs, bladder, spleen, breast, pancreas, liver, colon, spermatic cord, prostate, peripheral nerves, soft tissues, and orbit. A 36-year-old female patient with prior granulomatous inflammation and fibrosis of the left lung noted on wedge resection 5 years ago presented with left chest discomfort. CT chest revealed postoperative changes left lower lobe (LLL) lung and a new lesion measuring 4.1x1.8 cm. PET-CT scan confirmed a hyper metabolic mass (SUV max 6.02) in the LLL Lung CT guided needle biopsy demonstrated spindle cell and inflammatory proliferation, favoring inflammatory myofibroblastic tumor (Immunostaining revealing SMA positive, Desmin negative and ALK negative). Patient underwent a left thoracoscopic lower lobectomy with mediastinal lymph node dissection (levels 5, 7, 9, 10, 11), pathology indicated a 4 cm granulomatous inflammatory mass with abundant fibrosis, no evidence of neoplastic changes and multifocal distribution of granulomas. The differential included rheumatological disorders, infections, allergic disorders, and aspiration. Lingular findings included multifocal patchy interstitial fibrosis. All lymph nodes were reactive and negative for malignancy. Level 11 lymph node replaced by old, calcified granuloma-negative for AFB, GMS/Fungal gram stains. Patient recovered well from surgery and is under active surveillance. IMT predominantly described in children, constituting the most frequent primary lung lesion in pediatric patients is seldom reported in adults. IMTs represent from 0.04% to 0.1% of all pulmonary tumors. They can be asymptomatic or present with cough, hemoptysis, dyspnea, and pleuritic pain. Approximately 15-30% of patients may experience systemic symptoms like fever, weight loss, malaise, and fatigue. Some patients may have recurrent pneumonias. Pulmonary IMTs are more commonly found in the lower lobes. The exact cause of IMT remains uncertain. Recent research indicates IMT is linked to rearrangements in the anaplastic lymphoma kinase (ALK) gene, along with increased expression of ALK protein. Additionally, the condition involves gene fusions such as ROS1, NTRK3, and RET. Immunohistochemistry stains often reveal Vimentin, smooth muscle actin (SMA), and ALK (40% of the time) positive on the spindle tumor cells. Complete surgical resection is the most important treatment for IMT, and long-term follow-up is necessary to detect local recurrence. The prognosis of PIMT patients who undergo early surgical treatment is usually excellent, and adjuvant chemotherapy treatments are not required. Reoperation/resection is the mainstay of therapy for local recurrence. Chemotherapy proves beneficial in managing advanced IMT with sarcomatoid features. Reports indicate the utilization of gene-targeted medications in IMT treatment. The US National Comprehensive Cancer Network (NCCN) advocates for crizotinib as the gold standard for ALK-positive IMT care. Additionally, ceritinib, a second-generation ALK inhibitor, has demonstrated efficacy in IMT management. Pulmonary IMT is a rare tumor. The primary treatment method is surgery, with reoperation recommended for local recurrence. Chemotherapy may be an effective option for advanced IMT with sarcomatoid features. Targeted therapy can be applied for ALK-positive IMT.