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Yang S, Tang Y, Yuan Z, Zhang J. Inflammatory myofibroblastic tumor in the liver after bone marrow transplantation: case report and literature review. Front Med (Lausanne) 2025; 12:1489399. [PMID: 40224630 PMCID: PMC11986994 DOI: 10.3389/fmed.2025.1489399] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/01/2024] [Accepted: 02/17/2025] [Indexed: 04/15/2025] Open
Abstract
Introduction Inflammatory myofibroblastic tumor (IMT) is a rare low-grade malignant neoplasm in the liver. Timely diagnosis and treatment of IMT are challenging due to its atypical symptoms and imaging results. Case report We report a 46-year-old woman who presented to our hospital with persistent hyperpyrexia and discomfort in the right upper abdomen for 2 months post bone marrow transplantation. Radiological findings revealed a space-occupying lesion of uncertain nature in the liver. Since the histological examination of the biopsy specimen indicated IMT, she underwent surgical resection. Subsequently, the postoperative pathology confirmed the diagnosis of IMT. The patient's febrile condition subsided after the surgery. A magnetic resonance imaging (MRI) scan performed 8 months later showed no signs of recurrence. Conclusion IMTs are caused by genetic rearrangements. Diagnosing IMT can be challenging especially in this case as we had to differentiate the tumor from inflammatory diseases associated with bone marrow transplantation. Hence, a thorough pathological immunohistochemical examination is required to confirm its diagnosis. Local IMTs should be treated with radical surgical resection. In cases of distant metastasis or incomplete resection cases, chemotherapy, targeted therapy, or immunotherapy can be utilized. Regular follow-up is crucial for improving the patient's survival rate.
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Affiliation(s)
| | | | | | - Jianwen Zhang
- Department of Hepatic Surgery and Liver Transplantation Center, Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China
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Leivaditis V, Baltagianni M, Liolis E, Baltayiannis N, Stanc G, Souka E, Batika P, Grapatsas K, Tchabashvili L, Tasios K, Antzoulas A, Litsas D, Beltsios E, Dahm M, Papatriantafyllou A, Koletsis E, Mulita F. Inflammatory myofibroblastic tumor of the lung: a comprehensive narrative review of clinical and therapeutic insights. KARDIOCHIRURGIA I TORAKOCHIRURGIA POLSKA = POLISH JOURNAL OF CARDIO-THORACIC SURGERY 2025; 22:32-43. [PMID: 40290717 PMCID: PMC12019978 DOI: 10.5114/kitp.2025.148514] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 08/11/2024] [Accepted: 09/11/2024] [Indexed: 04/30/2025]
Abstract
Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal neoplasm classified by the World Health Organization as an intermediate malignancy with less than a 5% chance of metastasis. IMTs consist of myofibroblastic and spindle fibroblastic cells accompanied by inflammatory infiltration, primarily affecting patients under 16 years old, though they can also occur in adults. The etiology and pathogenesis of IMTs remain unclear, with possible contributing factors including inflammation, trauma, autoimmune diseases, prior surgery, viral infections, and uncontrolled myofibroblast proliferation. The primary treatment is complete surgical resection, which is associated with long-term survival and a significantly reduced recurrence rate of 2%, compared to 60% for incomplete resections. Chemotherapy is generally not recommended but may be necessary for unresectable tumors. Advances in histopathological diagnosis provide deeper insights into IMT biology, aiding in the selection of appropriate treatments. This paper presents a comprehensive review of the literature on this rare clinical entity.
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Affiliation(s)
- Vasileios Leivaditis
- Department of Cardiothoracic and Vascular Surgery, Westpfalz Klinikum, Kaiserslautern, Germany
| | - Marianthi Baltagianni
- Department of Nursing, School of Health Sciences and Welfare, University of Western Attica, Athens, Greece
| | - Elias Liolis
- Department of Oncology, General University Hospital of Patras, Patras, Greece
| | | | - Gabriela Stanc
- Department of Pathology. ’Metaxa’ Anticancer Hospital, Piraeus, Greece
| | - Efthymia Souka
- Department of Pathology. ’Metaxa’ Anticancer Hospital, Piraeus, Greece
| | - Pella Batika
- Department of Cardiothoracic and Vascular Surgery, Westpfalz Klinikum, Kaiserslautern, Germany
| | - Konstantinos Grapatsas
- Department of Thoracic Surgery and Thoracic Endoscopy, Ruhrlandklinik, West German Lung Center, University Hospital Essen, University Duisburg-Essen Essen, Germany
| | - Levan Tchabashvili
- Department of General Surgery, General University Hospital of Patras, Patras, Greece
| | - Konstantinos Tasios
- Department of General Surgery, General University Hospital of Patras, Patras, Greece
| | - Andreas Antzoulas
- Department of General Surgery, General University Hospital of Patras, Patras, Greece
| | - Dimitrios Litsas
- Department of General Surgery, General Hospital of Lamia, Lamia, Greece
| | - Eleftherios Beltsios
- Department of Anesthesiology and Intensive Care, Hannover Medical School, Germany
| | - Manfred Dahm
- Department of Cardiothoracic and Vascular Surgery, Westpfalz Klinikum, Kaiserslautern, Germany
| | | | - Efstratios Koletsis
- Department of Cardiothoracic Surgery, General University Hospital of Patras, Patras, Greece
| | - Francesk Mulita
- Department of General Surgery, General University Hospital of Patras, Patras, Greece
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Si X, Wu S, Feng R, Wang X, Guan H, He J, Zeng X, Song W, Li S, Hu K, Liang Z, Zhang F, Huo L, Cheng X, Zhuo M, Liu W, Liu Y, Zhou X, Wang T, Zhang Y, Ni J, Wang X, Zhao J, Xu Y, Zhang X, Wang H, Liu X, Wang M, Cui X, Zhang L, Xu K. Chinese Expert Consensus on the Diagnosis and Treatment of Inflammatory Myofibroblastic Tumor. Thorac Cancer 2025; 16:e70027. [PMID: 40070347 PMCID: PMC11897609 DOI: 10.1111/1759-7714.70027] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2025] [Accepted: 02/16/2025] [Indexed: 03/15/2025] Open
Abstract
Inflammatory myofibroblastic tumor (IMT) is a rare spindle-cell neoplasm. IMT currently suffers from a paucity of standardized diagnostic and therapeutic guidelines. The Chinese expert consensus committee on the diagnosis and treatment of IMT formed an "Expert consensus on the diagnosis and treatment of inflammatory myofibroblastic tumor". This consensus was developed through a comprehensive synthesis of expert opinions, an extensive review of the literature, and a series of offline and online deliberations. The committee aspires that this consensus will enhance the therapeutic outcomes and prognosis for patients with IMT in the future.
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Affiliation(s)
- Xiaoyan Si
- Department of Pulmonary and Critical Care MedicinePeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Shafei Wu
- Department of PathologyPeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Rui'e Feng
- Department of PathologyPeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Xuan Wang
- Department of RadiologyPeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Hui Guan
- Department of Radiation OncologyPeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Jia He
- Department of Thoracic SurgeryPeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Xuan Zeng
- Department of PathologyPeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Wei Song
- Department of RadiologyPeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Shanqing Li
- Department of Thoracic SurgeryPeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Ke Hu
- Department of Radiation OncologyPeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Zhiyong Liang
- Department of PathologyPeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Fuquan Zhang
- Department of Radiation OncologyPeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Li Huo
- Department of Nuclear MedicineBeijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Xin Cheng
- Department of Nuclear MedicineBeijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Minglei Zhuo
- Department of Thoracic OncologyPeking University Cancer Hospital and InstituteBeijingChina
| | - Wei Liu
- Department of General SurgeryPeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Yong Liu
- Department of OrthopedicsPeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Xi Zhou
- Department of OrthopedicsPeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Tao Wang
- National Clinical Research Center for Obstetric & Gynecologic Diseases, Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Chinese Academy of Medical SciencesPeking Union Medical CollegeBeijingChina
| | - Yuhui Zhang
- Department of Respiratory and Critical Care MedicineBeijing Institute of Respiratory Medicine and Beijing Chao‐Yang Hospital, Capital Medical UniversityBeijingChina
| | - Jun Ni
- Department of Respiratory and Critical Care MedicineBeijing Institute of Respiratory Medicine and Beijing Chao‐Yang Hospital, Capital Medical UniversityBeijingChina
| | - Xiaojun Wang
- Department of Respiratory MedicineGansu Provincial HospitalLanzhouChina
| | - Jing Zhao
- Department of Pulmonary and Critical Care MedicinePeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Yan Xu
- Department of Pulmonary and Critical Care MedicinePeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Xiaotong Zhang
- Department of Pulmonary and Critical Care MedicinePeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Hanping Wang
- Department of Pulmonary and Critical Care MedicinePeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Xiaoyan Liu
- Department of Pulmonary and Critical Care MedicinePeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Mengzhao Wang
- Department of Pulmonary and Critical Care MedicinePeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Xiaoxia Cui
- Department of Pulmonary and Critical Care MedicinePeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Li Zhang
- Department of Pulmonary and Critical Care MedicinePeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
| | - Kaifeng Xu
- Department of Pulmonary and Critical Care MedicinePeking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical CollegeBeijingChina
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Qian X, Ning W, Dunmall LC, Qu Y, Wang Y, Zhang H. Treatment of intracranial inflammatory myofibroblastic tumor with PD-L1 inhibitor and novel oncolytic adenovirus Ad-TD-nsIL12: a case report and literature review. Front Immunol 2024; 15:1427554. [PMID: 39114662 PMCID: PMC11303231 DOI: 10.3389/fimmu.2024.1427554] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/07/2024] [Accepted: 07/01/2024] [Indexed: 08/10/2024] Open
Abstract
Inflammatory myofibroblastic tumor (IMT) is a rare pathological entity first described in 1939. This lesion is most commonly found in the lungs, but cases involving other systems, such as the central nervous system known as intracranial IMT (IIMT), have also been reported. Diagnosis currently relies on pathological results due to the lack of characteristic imaging changes. Surgical resection is an effective treatment, though the disease is invasive and may recur. Previous literature has reported a high level of programmed death 1 (PD-1) expression in IMT tissues, suggesting that immunotherapy may be effective for this condition. In this case report, we present a middle-aged male who received PD-1 inhibitor and oncolytic adenovirus (Ad-TD-nsIL12) treatment after IIMT resection surgery. This successful approach provides a new direction for the treatment of IIMT.
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Affiliation(s)
- Xiao Qian
- Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China
| | - Weihai Ning
- Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China
| | - Louisa Chard Dunmall
- Centre for Cancer Biomarkers and Biotherapeutics, Barts Cancer Institute, Queen Mary University of London, London, United Kingdom
| | - Yanming Qu
- Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China
| | - Yaohe Wang
- Centre for Cancer Biomarkers and Biotherapeutics, Barts Cancer Institute, Queen Mary University of London, London, United Kingdom
| | - Hongwei Zhang
- Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China
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CHMIEL PAULINA, SłOWIKOWSKA ALEKSANDRA, BANASZEK ŁUKASZ, SZUMERA-CIEćKIEWICZ ANNA, SZOSTAKOWSKI BART, SPAłEK MATEUSZJ, ŚWITAJ TOMASZ, RUTKOWSKI PIOTR, CZARNECKA ANNAM. Inflammatory myofibroblastic tumor from molecular diagnostics to current treatment. Oncol Res 2024; 32:1141-1162. [PMID: 38948020 PMCID: PMC11209743 DOI: 10.32604/or.2024.050350] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/02/2024] [Accepted: 04/09/2024] [Indexed: 07/02/2024] Open
Abstract
Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm with intermediate malignancy characterized by a propensity for recurrence but a low metastatic rate. Diagnostic challenges arise from the diverse pathological presentation, variable symptomatology, and lack of different imaging features. However, IMT is identified by the fusion of the anaplastic lymphoma kinase (ALK) gene, which is present in approximately 70% of cases, with various fusion partners, including ran-binding protein 2 (RANBP2), which allows confirmation of the diagnosis. While surgery is the preferred approach for localized tumors, the optimal long-term treatment for advanced or metastatic disease is difficult to define. Targeted therapies are crucial for achieving sustained response to treatment within the context of genetic alteration in IMT. Crizotinib, an ALK tyrosine kinase inhibitor (TKI), was officially approved by the US Food and Drug Administration (FDA) in 2020 to treat IMT with ALK rearrangement. However, most patients face resistance and disease progression, requiring consideration of sequential treatments. Combining radiotherapy with targeted therapy appears to be beneficial in this indication. Early promising results have also been achieved with immunotherapy, indicating potential for combined therapy approaches. However, defined recommendations are still lacking. This review analyzes the available research on IMT, including genetic disorders and their impact on the course of the disease, data on the latest targeted therapy regimens and the possibility of developing immunotherapy in this indication, as well as summarizing general knowledge about prognostic and predictive factors, also in terms of resistance to systemic therapy.
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Affiliation(s)
- PAULINA CHMIEL
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, 02-781, Poland
- Faculty of Medicine, Medical University of Warsaw, Warsaw, 02-091, Poland
| | - ALEKSANDRA SłOWIKOWSKA
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, 02-781, Poland
- Faculty of Medicine, Medical University of Warsaw, Warsaw, 02-091, Poland
| | - ŁUKASZ BANASZEK
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, 02-781, Poland
- Faculty of Medicine, Medical University of Warsaw, Warsaw, 02-091, Poland
| | - ANNA SZUMERA-CIEćKIEWICZ
- Department of Pathology, Maria Sklodowska Curie National Research Institute of Oncology, Warsaw, 02-781, Poland
| | - BARTłOMIEJ SZOSTAKOWSKI
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, 02-781, Poland
| | - MATEUSZ J. SPAłEK
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, 02-781, Poland
- Department of Radiotherapy, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, 02-781, Poland
| | - TOMASZ ŚWITAJ
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, 02-781, Poland
| | - PIOTR RUTKOWSKI
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, 02-781, Poland
| | - ANNA M. CZARNECKA
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, 02-781, Poland
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Vernemmen AIP, Samarska IV, Speel EJM, Riedl RG, Goudkade D, de Bruïne AP, Wouda S, van Marion AM, Verlinden IV, van Lijnschoten I, Friederich P, Winnepenninckx VJL, Zur Hausen A, Sciot RME, van den Hout MFCM. Abdominal inflammatory myofibroblastic tumour: Clinicopathological and molecular analysis of 20 cases, highlighting potential therapeutic targets. Histopathology 2024; 84:794-809. [PMID: 38155480 DOI: 10.1111/his.15122] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/11/2023] [Revised: 11/30/2023] [Accepted: 12/02/2023] [Indexed: 12/30/2023]
Abstract
AIMS Inflammatory myofibroblastic tumour (IMT) is a rare mesenchymal neoplasm of intermediate malignant potential, occurring at any age and at multiple sites. Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is an aggressive subtype of IMT, typically involving the abdomen. Most IMTs harbour kinase gene fusions, especially involving ALK and ROS1, but 20-30% of IMTs show no detectable translocations. The aim of this study is to further delineate clinicopathological and molecular characteristics of abdominal IMT and discover potential new therapeutic targets. METHODS AND RESULTS In 20 IMTs, including four EIMS, RNA fusion analysis was performed, followed by multiplex DNA analysis if no ALK or ROS1 fusion was detected. Fourteen IMTs (70.0%) had an ALK translocation and the fusion partner was identified in 11, including a RRBP1::ALK fusion, not previously described in classical (non-EIMS) IMT. RANBP2::ALK fusion was demonstrated in all EIMS. One IMT had a ROS1 fusion. In all ALK/ROS1 translocation-negative IMTs mutations or fusions - as yet unreported in primary IMT - were found in genes related to the receptor tyrosine kinase (RTK)/PI3K/AKT pathway. Three of four patients with EIMS died of disease [mean survival 8 months (4-15 months)], whereas only one of 14 classical IMT patients succumbed to disease [mean follow-up time 52 months (2-204 months); P < 0.01]. CONCLUSION This study shows the wide clinical spectrum of abdominal IMTs and affirms the poor prognosis of EIMS, raising discussion about its status as IMT subtype. Furthermore, the newly detected alterations of the RTK/PI3K/AKT pathway expand the molecular landscape of IMTs and provide potential therapeutic targets.
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Affiliation(s)
- Astrid I P Vernemmen
- Department of Pathology, School for Oncology and Reproduction (GROW), Maastricht University Medical Center, Maastricht, The Netherlands
| | - Iryna V Samarska
- Department of Pathology, School for Oncology and Reproduction (GROW), Maastricht University Medical Center, Maastricht, The Netherlands
| | - Ernst-Jan M Speel
- Department of Pathology, School for Oncology and Reproduction (GROW), Maastricht University Medical Center, Maastricht, The Netherlands
| | - Robert G Riedl
- Department of Pathology, Zuyderland Medical Center, Geleen, The Netherlands
| | - Danny Goudkade
- Department of Pathology, Zuyderland Medical Center, Geleen, The Netherlands
| | | | - Siep Wouda
- Department of Pathology, VieCuri Medical Center, Venlo, The Netherlands
| | | | - Ivana V Verlinden
- Department of Pathology, Laurentius Hospital, Roermond, The Netherlands
| | - Ineke van Lijnschoten
- Department of Pathology, PAMM Laboratory for Pathology and Medical Microbiology, Eindhoven, The Netherlands
| | - Pieter Friederich
- Department of Gastroenterology and Hepatology, Catharina Hospital, Eindhoven, The Netherlands
| | - Véronique J L Winnepenninckx
- Department of Pathology, School for Oncology and Reproduction (GROW), Maastricht University Medical Center, Maastricht, The Netherlands
| | - Axel Zur Hausen
- Department of Pathology, School for Oncology and Reproduction (GROW), Maastricht University Medical Center, Maastricht, The Netherlands
| | - Raf M E Sciot
- Department of Pathology, University Hospitals Leuven, KU Leuven, Leuven, Belgium
| | - Mari F C M van den Hout
- Department of Pathology, School for Oncology and Reproduction (GROW), Maastricht University Medical Center, Maastricht, The Netherlands
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Diao L, Li W, Jiang Q, Huang H, Zhou E, Peng B, Chen X, Zeng Z, He C. Inflammatory myofibroblastic tumor of the submandibular gland Harboring MSN-ALK gene fusion: A case report and literature review. Heliyon 2023; 9:e22928. [PMID: 38144359 PMCID: PMC10746421 DOI: 10.1016/j.heliyon.2023.e22928] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/23/2023] [Revised: 11/20/2023] [Accepted: 11/22/2023] [Indexed: 12/26/2023] Open
Abstract
Inflammatory myofibroblastic tumors (IMTs) are rare lesions with distinct clinical, pathological, and molecular characteristics. IMTs typically arise in the abdominal soft tissues, including the mesentery, omentum, and retroperitoneum, followed by the lungs and mediastinum, and usually affect both children and young adults. Herein, we present a rare case of an IMT in the submandibular gland of a 47-year-old male patient. Microscopically, the tumor displayed an infiltrative growth pattern with diffuse glandular tissue destruction. Their backgrounds revealed characteristic spindles and inflammatory cells. Immunohistochemistry revealed positivity for anaplastic lymphoma kinase (ALK), smooth muscle actin, and calponin in neoplastic cells. The inflammatory cells and some neoplastic cells were positive for CD68. In contrast, negative staining for cytokeratin, desmin, and CD30 was observed. Furthermore, fluorescence in situ hybridization revealed ALK gene rearrangements, and next-generation sequencing detected a moesin (MSN)-ALK gene fusion. This case highlights a rare and unique occurrence of IMT originating from the submandibular gland, which exhibited an MSN-ALK gene fusion.
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Affiliation(s)
- Limei Diao
- Department of Pathology, Chongqing University Cancer Hospital, Chongqing, 400030, China
- Chongqing Cancer Multi-omics Big Data Application Engineering Research Center, Chongqing University Cancer Hospital, Chongqing, 400030, China
| | - Wen Li
- Department of Pathology, Chongqing University Cancer Hospital, Chongqing, 400030, China
- Chongqing Key Laboratory of Translational Research for Cancer Metastasis and Individualized Treatment, Chongqing University Cancer Hospital, Chongqing, 400030, China
| | - Qingming Jiang
- Department of Pathology, Chongqing University Cancer Hospital, Chongqing, 400030, China
- Chongqing Cancer Multi-omics Big Data Application Engineering Research Center, Chongqing University Cancer Hospital, Chongqing, 400030, China
| | - Haiping Huang
- Department of Pathology, Chongqing University Cancer Hospital, Chongqing, 400030, China
- Chongqing Cancer Multi-omics Big Data Application Engineering Research Center, Chongqing University Cancer Hospital, Chongqing, 400030, China
| | - Enle Zhou
- Department of Pathology, Chongqing University Cancer Hospital, Chongqing, 400030, China
- Chongqing Key Laboratory of Translational Research for Cancer Metastasis and Individualized Treatment, Chongqing University Cancer Hospital, Chongqing, 400030, China
| | - Bingjie Peng
- Department of Pathology, Chongqing University Cancer Hospital, Chongqing, 400030, China
- Chongqing Key Laboratory of Translational Research for Cancer Metastasis and Individualized Treatment, Chongqing University Cancer Hospital, Chongqing, 400030, China
| | - Xiaoling Chen
- Department of pharmacy, Chongqing University Jiangjin Hospital, Chongqing, 402260, China
| | - Zhen Zeng
- Department of Pathology, Chongqing University Cancer Hospital, Chongqing, 400030, China
- Chongqing Cancer Multi-omics Big Data Application Engineering Research Center, Chongqing University Cancer Hospital, Chongqing, 400030, China
| | - Changqing He
- Department of Pathology, Chongqing University Cancer Hospital, Chongqing, 400030, China
- Chongqing Key Laboratory of Translational Research for Cancer Metastasis and Individualized Treatment, Chongqing University Cancer Hospital, Chongqing, 400030, China
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8
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Uterine inflammatory myofibroblastic tumor. Pathol Res Pract 2023; 242:154335. [PMID: 36706588 DOI: 10.1016/j.prp.2023.154335] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/07/2022] [Revised: 01/19/2023] [Accepted: 01/21/2023] [Indexed: 01/24/2023]
Abstract
Inflammatory myofibroblastic tumor (IMT) is recognized as a true neoplasm of unknown etiology, but its pathogenesis is related to abnormalities in the ALK gene. This is an uncommon tumor with a wide anatomic distribution and often constitutes a challenging diagnosis owing to its histological similarities with other tumors. Uterine IMTs are rare and their detailed characteristics should be described based on case reports and small case series. Thus, we performed a comprehensive review of the literature showing that uterine IMTs show a wide range of age at diagnosis (median, 39 years), and a symptomatology similar to that of common leiomyomas, only rarely presenting with inflammatory manifestations. IMTs represent 0.1% of "leiomyomas," an estimate that increases to 10% for pregnant women and to 14% for the smooth muscle tumors of uncertain malignant potential (STUMP) category of tumors, implying that tumors excised during pregnancy, STUMPs, and leiomyosarcomas should be systematically screened with ALK immunohistochemistry, as this is a targetable abnormality. Most reported cases are ALK-positive; the fusion partners vary, but in pregnancy-associated tumors, TIMP3 prevails. Almost 25% of the patients will show an aggressive course, and this is associated with older age, non-pregnancy-associated tumors, larger tumors, infiltrative tumor border, absence of abundant inflammation, atypia, important mitotic activity, and necrosis.
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9
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Su ZJ, Guo ZS, Wan HT, Hong XY. Inflammatory myofibroblastic tumor of the central nervous system: A case report. World J Clin Cases 2022; 10:12637-12647. [PMID: 36579095 PMCID: PMC9791513 DOI: 10.12998/wjcc.v10.i34.12637] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/30/2022] [Revised: 09/16/2022] [Accepted: 11/07/2022] [Indexed: 12/02/2022] Open
Abstract
BACKGROUND An inflammatory myofibroblastic tumor (IMT) occurring in the central nervous system is very rare, and thus its pathogenesis is unknown. This case report and literature review aimed to explore the pathogenesis, clinical features, imaging findings, pathological characteristics, immunohistochemical characteristics, diagnoses, treatments, and risks of postoperative recurrence of IMT in the central nervous system.
CASE SUMMARY A 67-year-old woman was admitted to the hospital with an exophthalmic protrusion and double vision in the left eye that had persisted for 3 mo. Magnetic resonance imaging (MRI) showed a 2.4 cm × 1.3 cm heterogeneous large mass in the bottom of the left anterior cranial fossa, which was closely related to the dura mater. Before surgery, we suspected the mass to be meningioma. The entire mass was successfully removed under neuronavigation and electrophysiological monitoring, and postoperative pathology indicated an IMT with extensive infiltration of chronic inflammatory cells and scattered multinucleated giant cells. Head MRI at the 3-mo follow-up showed that the tumor at the bottom of left anterior cranial fossa had been completely resected without recurrence.
CONCLUSION From the histological, immunohistochemical, and genetic analyses, the present case suggests that the pathogenesis of IMT-CNS is related to autoimmunity.
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Affiliation(s)
- Zhen-Jin Su
- Department of Neurosurgical Oncology, The First Hospital of Jilin University, Changchun 130000, Jilin Province, China
| | - Ze-Shang Guo
- Department of Neurosurgical Oncology, The First Hospital of Jilin University, Changchun 130000, Jilin Province, China
| | - Heng-Tong Wan
- Department of Neurosurgical Oncology, The First Hospital of Jilin University, Changchun 130000, Jilin Province, China
| | - Xin-Yu Hong
- Department of Neurosurgical Oncology, The First Hospital of Jilin University, Changchun 130000, Jilin Province, China
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Gros L, Dei Tos AP, Jones RL, Digklia A. Inflammatory Myofibroblastic Tumour: State of the Art. Cancers (Basel) 2022; 14:cancers14153662. [PMID: 35954326 PMCID: PMC9367282 DOI: 10.3390/cancers14153662] [Citation(s) in RCA: 31] [Impact Index Per Article: 10.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/24/2022] [Revised: 07/21/2022] [Accepted: 07/25/2022] [Indexed: 11/16/2022] Open
Abstract
Simple Summary Among sarcomas, which are rare cancers, inflammatory myofibroblastic tumors are extremely rare. Unlike other subtypes, this is a largely oncogene-driven neoplasia, and early gene rearrangement identification is important for accurate advanced stage treatment. In this manuscript, we review the clinicopathologic characteristics of this ultra-rare entity, as well as the current treatment landscape, with a particular focus on opportunities provided by tyrosine kinase inhibitors (TKIs). Abstract An inflammatory myofibroblastic tumor (IMT) is a neoplasm composed of myofibroblastic and fibroblastic spindle cells accompanied by inflammatory cells, including lymphocytes and eosinophils. It is an ultra-rare tumor, the optimal management of which remains to be defined. Surgery is the treatment of choice for localized tumors. The treatment of advanced disease is not precisely defined. Chemotherapy regimens result in an overall response rate of approximately 50% based on retrospective data. The latest pathophysiological data highlight the role played by tyrosine kinase fusion genes in IMT proliferation. Anaplast lymphoma kinase (ALK) oncogenic activation mechanisms have been characterized in approximately 80% of IMTs. In this context, data regarding targeted therapies are most important. The aims of this article are to review the latest published data on the use of systematic therapy, particularly the use of molecular targeted therapy, and to publish an additional case of an IMT with Ran-binding protein 2 (RANPB2)-ALK fusion showing a long response to a tyrosine kinase inhibitor.
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Affiliation(s)
- Louis Gros
- Department of Oncology, Lausanne University Hospital, University of Lausanne, 1011 Lausanne, Switzerland;
| | - Angelo Paolo Dei Tos
- Department of Pathology, Azienda Ospedale Università Padova, 35128 Padua, Italy;
- Department of Medicine, University of Padua School of Medicine, 35128 Padua, Italy
| | - Robin L. Jones
- Sarcoma Unit, The Royal Marsden NHS Foundation Trust, London SW3 6JJ, UK;
- Division of Clinical Sciences, Institute of Cancer Research, Royal Marsden Hospital, London SW3 6JJ, UK
| | - Antonia Digklia
- Department of Oncology, Lausanne University Hospital, University of Lausanne, 1011 Lausanne, Switzerland;
- Center of Sarcoma, Department of Oncology, Lausanne University Hospital, University of Lausanne, 1011 Lausanne, Switzerland
- Correspondence:
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Meng X, Zhang L, Wang Q, Chen J, Zhang C, Tao R, Wang Y. Genetic Testing and Immunotherapy for Intracranial Inflammatory Myofibroblastic Tumor: A Case Report. Onco Targets Ther 2022; 15:313-321. [PMID: 35401006 PMCID: PMC8985701 DOI: 10.2147/ott.s343562] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/14/2021] [Accepted: 03/15/2022] [Indexed: 12/25/2022] Open
Abstract
Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor that can develop in numerous organs, most commonly in the lungs and rarely in the brain. Here, we reported a 55-year-old patient with nasopharyngeal IMT and the recurrence in the skull base, slope and pterygoid sinus who underwent cranial base and slope tumor resection. Postoperative magnetic resonance imaging (MRI) and multiplex immunohistochemistry (mIHC) showed tumor recurrence and metastasis to the intracalvarium. While genetic testing revealed no significant related gene mutations, tertiary mutations in NSD1 and SOX9 genes were identified in the tumor tissues. The patient achieved partial remission after receiving 7 cycles of immunotherapy (toripalimab 240 mg for 1 cycle followed by 6 cycles of sintilimab 200 mg), and MRI examination indicated an almost complete remission of intracranial IMT after 16 cycles of immunotherapy. In summary, the novel class of immune-targeted agents may be effective in clinical management of rare intracranial IMT.
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Affiliation(s)
- Xiangji Meng
- Department of Neurosurgery, Shandong Cancer Hospital and Institute, Shandong First Medical University and Shandong Academy of Medical Sciences, Jinan, Shandong, 250117, People’s Republic of China
| | - Lei Zhang
- Department of Translational Medicine, Genecast Biotechnology Co., Ltd, Wuxi, Jiangsu, 214104, People’s Republic of China
| | - Qi Wang
- Department of Paediatrics, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, 250021, People’s Republic of China
| | - Jimin Chen
- Department of Translational Medicine, Genecast Biotechnology Co., Ltd, Wuxi, Jiangsu, 214104, People’s Republic of China
| | - Chunmei Zhang
- Department of Translational Medicine, Genecast Biotechnology Co., Ltd, Wuxi, Jiangsu, 214104, People’s Republic of China
| | - Rongjie Tao
- Department of Medical Oncology, Shandong Cancer Hospital and Institute, Shandong First Medical University and Shandong Academy of Medical Sciences, Jinan, Shandong, 250117, People’s Republic of China
- Rongjie Tao, Department of Medical Oncology, Shandong Cancer Hospital and Institute, Shandong First Medical University and Shandong Academy of Medical Sciences, Jinan, Shandong, 250117, People’s Republic of China, Email
| | - Yong Wang
- Shandong Cancer Hospital, Cheeloo College of Medicine, Shandong University, Jinan, Shandong, 250117, People's Republic of China
- Shandong Cancer Hospital and Institute, Shandong First Medical University and Shandong Academy of Medical Sciences, Jinan, Shandong, 250117, Peoples' Republic of China
- Correspondence: Yong Wang, Shandong Cancer Hospital and Institute, Shandong First Medical University and Shandong Academy of Medical Sciences, No. 440, Jiyan Road, Jinan, Shandong, 250117, People’s Republic of China, Email
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Mahajan P, Casanova M, Ferrari A, Fordham A, Trahair T, Venkatramani R. Inflammatory myofibroblastic tumor: molecular landscape, targeted therapeutics, and remaining challenges. Curr Probl Cancer 2021; 45:100768. [PMID: 34244015 DOI: 10.1016/j.currproblcancer.2021.100768] [Citation(s) in RCA: 29] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/13/2021] [Accepted: 06/21/2021] [Indexed: 12/29/2022]
Abstract
Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor of intermediate malignant potential that predominantly affects children, adolescents and young adults. IMT has a predilection for the lung, abdomen, pelvis, and retroperitoneum, however, can affect any part of the body. IMT is typically localized, and multifocal or metastatic disease is uncommon. Complete surgical resection is the treatment of choice when feasible. There is no established standard of care for unresectable and advanced IMT. Approximately half of IMTs harbor anaplastic lymphoma kinase (ALK) gene rearrangements, and fusions involving ROS1, PDGFRβ, RET and NTRK have also been described. Given the molecular landscape of IMT, management of these tumors has evolved to include tyrosine kinase inhibitors and novel targeted therapeutics. This review highlights the molecular characteristics, evolution of targeted therapies and the remaining challenges in the management of IMT.
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Affiliation(s)
- Priya Mahajan
- Department of Pediatrics, Texas Children's Cancer and Hematology Centers, Baylor College of Medicine, Houston, Texas
| | - Michela Casanova
- Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy
| | - Andrea Ferrari
- Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy
| | - Ashleigh Fordham
- Children's Cancer Institute, C25 Lowy Cancer Research Centre, UNSW Sydney New South Wales, Australia
| | - Toby Trahair
- Children's Cancer Institute, C25 Lowy Cancer Research Centre, UNSW Sydney New South Wales, Australia; Kids Cancer Centre, Sydney Children's Hospital, Randwick, New South Wales, Australia; School of Women's and Children's Health, UNSW Medicine, New South Wales, Australia
| | - Rajkumar Venkatramani
- Department of Pediatrics, Texas Children's Cancer and Hematology Centers, Baylor College of Medicine, Houston, Texas.
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Byers J, Yin H, Rytting H, Logan S, He M, Yu Z, Wang D, Warren M, Mangray S, Dehner LP, Zhou S. PD-L1 expression in angiomatoid fibrous histiocytoma. Sci Rep 2021; 11:2183. [PMID: 33500467 PMCID: PMC7838166 DOI: 10.1038/s41598-021-81746-y] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/25/2020] [Accepted: 01/11/2021] [Indexed: 01/22/2023] Open
Abstract
Angiomatoid fibrous histiocytoma (AFH) is a rare tumor of intermediate malignancy. Treatment options for unresectable and/or metastatic tumors are very limited. Immunotherapy with PD-1/PD-L1 inhibitors may be worth exploring. The aim of this study was to evaluate the expression of PD-L1 in AFHs. PD-L1 expression was assessed on 36 AFHs from 36 pediatric patients by immunohistochemical staining of PD-L1 (clone 22C3). Positivity was defined as membranous expression in ≥ 1% of either tumor or immune cells. The correlations between PD-L1 expression and clinicopathologic features were assessed. Two patients had lymph node metastasis. All patients underwent surgical resection; three of them also had systemic chemotherapy. Three patients had recurrence after initial resection; all patients were alive with a median follow-up of 2.5 years. Overall, twenty-two (61%) tumors were positively stained for PD-L1 and positivity was seen on both tumor and immune cells in eighteen of the 22 tumors. A positive correlation was found between tumor cell PD-L1 expression and CD8+ T-cell infiltration. There were no statistically significant differences between the status of PD-L1 expression and the clinicopathological features assessed. PD-L1 expression was identified in 61% of AFHs with a predominantly adaptive pattern. Our findings provide a rationale for future studies evaluating the potential of checkpoint immunotherapy for patients with unresectable and/or metastatic tumor.
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Affiliation(s)
- Joshua Byers
- Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, MS 43, 4650 Sunset Boulevard, Los Angeles, CA, 90027, USA
| | - Hong Yin
- Children's Healthcare of Atlanta, Atlanta, GA, USA
| | | | | | - Mai He
- Washington University in Saint Louis, Saint Louis, MO, USA
| | - Zhongxin Yu
- University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA
| | - Dehua Wang
- Cincinnati Children's Hospital, Cincinnati, OH, USA
| | - Mikako Warren
- Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, MS 43, 4650 Sunset Boulevard, Los Angeles, CA, 90027, USA
| | | | - Louis P Dehner
- Washington University in Saint Louis, Saint Louis, MO, USA
| | - Shengmei Zhou
- Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, MS 43, 4650 Sunset Boulevard, Los Angeles, CA, 90027, USA.
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Quiroga D, Liebner DA, Philippon JS, Hoffman S, Tan Y, Chen JL, Lenobel S, Wakely PE, Pollock R, Tinoco G. Activity of PD1 inhibitor therapy in advanced sarcoma: a single-center retrospective analysis. BMC Cancer 2020; 20:527. [PMID: 32503455 PMCID: PMC7275332 DOI: 10.1186/s12885-020-07021-x] [Citation(s) in RCA: 16] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/17/2020] [Accepted: 05/28/2020] [Indexed: 12/31/2022] Open
Abstract
BACKGROUND Sarcomas constitute a heterogeneous group of tumors with different clinical behaviors and variable responses to systemic therapies. Recent immunotherapy studies with PD1 inhibitors (PD1i) show promising results with use in certain soft-tissue sarcomas; however, the clinical and molecular features that best predict response to PD1i remain unclear. METHODS Demographic, imaging, histologic, and genetic sequencing data was collected for sarcoma patients who received nivolumab or pembrolizumab (PD1i) treatment at our institution between January 1st 2015 and April 23rd 2018. The primary objective was to determine progression-free survival (PFS) in patients with advanced sarcomas receiving PD1i. Secondary objectives included determining overall survival (OS) and assessment of characteristics associated with response to PD1i. Fifty-six patients who were treated with PD1i therapy met inclusion criteria for this study. RESULTS Partial response towards PD1i treatment was seen in 3 in 26 evaluable patients, but no complete responses were observed (overall response rate 11.5%). Within this group of patients, the 90 day PFS was found to be 48.8%. In patients in whom PD1 expression was known, there was a statistically significant positive correlation between expression of PD1 and longer PFS and OS rates. Patients that were treated with more than four cycles of PD1i therapy were also more likely to have a greater OS. CONCLUSIONS This study suggests activity of PD1i in a pretreated cohort of advanced sarcoma patients, particularly for the subset of patients with PD1 positive tumors. Our results highlight the importance of further research to better target the optimal patient population and markers of response.
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Affiliation(s)
- Dionisia Quiroga
- The Ohio State University Comprehensive Cancer Center, The Ohio State University, 410 W 12th Avenue, Columbus, OH, 43210, USA
- Department of Internal Medicine, Division of Medical Oncology, The Ohio State University, Starling Loving Hall, 320 W 10th Ave, Columbus, OH, 43210, USA
| | - David A Liebner
- The Ohio State University Comprehensive Cancer Center, The Ohio State University, 410 W 12th Avenue, Columbus, OH, 43210, USA
- Department of Internal Medicine, Division of Medical Oncology, The Ohio State University, Starling Loving Hall, 320 W 10th Ave, Columbus, OH, 43210, USA
- Department of Biomedical Informatics, The Ohio State University, 250 Lincoln Tower, 1800 Cannon Dr, Columbus, OH, 43210, USA
| | - Jennifer S Philippon
- The Ohio State University Comprehensive Cancer Center, The Ohio State University, 410 W 12th Avenue, Columbus, OH, 43210, USA
| | - Sarah Hoffman
- The Ohio State University Comprehensive Cancer Center, The Ohio State University, 410 W 12th Avenue, Columbus, OH, 43210, USA
| | - Yubo Tan
- Center for Biostatistics, The Ohio State University, 2012 Kenny Rd, Columbus, OH, 43221, USA
| | - James L Chen
- The Ohio State University Comprehensive Cancer Center, The Ohio State University, 410 W 12th Avenue, Columbus, OH, 43210, USA
- Department of Internal Medicine, Division of Medical Oncology, The Ohio State University, Starling Loving Hall, 320 W 10th Ave, Columbus, OH, 43210, USA
- Center for Biostatistics, The Ohio State University, 2012 Kenny Rd, Columbus, OH, 43221, USA
| | - Scott Lenobel
- Department of Radiology, The Ohio State University, 410 W 12th Avenue, Columbus, OH, 43210, USA
| | - Paul E Wakely
- Department of Pathology, The Ohio State University, 410 W 10th Ave, Columbus, OH, 43210, USA
| | - Raphael Pollock
- The Ohio State University Comprehensive Cancer Center, The Ohio State University, 410 W 12th Avenue, Columbus, OH, 43210, USA
- Department of Surgery, The Ohio State University, 410 W 10th Ave, Columbus, OH, 43210, USA
| | - Gabriel Tinoco
- The Ohio State University Comprehensive Cancer Center, The Ohio State University, 410 W 12th Avenue, Columbus, OH, 43210, USA.
- Department of Internal Medicine, Division of Medical Oncology, The Ohio State University, Starling Loving Hall, 320 W 10th Ave, Columbus, OH, 43210, USA.
- The Ohio State University Comprehensive Cancer Center, 320 W 10th Ave, A444 Starling Loving Hall, Columbus, OH, 43210, USA.
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PD-L1 Detection-Pearls and Pitfalls Associated With Current Methodologies Focusing on Entities Relevant to Dermatopathology. Am J Dermatopathol 2020; 41:539-565. [PMID: 31335407 DOI: 10.1097/dad.0000000000001287] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/22/2023]
Abstract
PD-L1 is a transmembrane glycoprotein with an extracellular as well as an intracellular cytoplasmic domain. Physiologically, it plays a pivotal role in regulating T-cell activation and tolerance. Many tumor cells have exploited this regulatory mechanism by overexpressing PD-L1 in an effort to escape immunologic surveillance. In this review, we parse the literature regarding the prognostic value of tumoral PD-L1 expression before discussing the various methodologies as well as the pearls and pitfalls associated with each for predicting response to anti-PD-1/PD-L1 therapies. Special attention is given to cutaneous entities in which PD-L1 expression has been documented with an emphasis on cutaneous malignancies that have seen the broadest applications of anti-PD-L1/PD-1 therapies. Currently, immunohistochemistry is the method that is most commonly used for detection of PD-L1. However, with the wide array of immunohistochemistry protocols and staining platforms available in the market, there seems to be different cutoffs not just for different entities but also for the same entity. This review is an attempt to address the need for standardization and validation of existing protocols for PD-L1 detection.
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Liu Q, Wei J, Liu X, Wang J. Anaplastic lymphoma kinase-negative pulmonary inflammatory myofibroblastic tumor with multiple metastases and its treatment by Apatinib: A case report. Medicine (Baltimore) 2019; 98:e18414. [PMID: 31876714 PMCID: PMC6946427 DOI: 10.1097/md.0000000000018414] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/30/2022] Open
Abstract
RATIONALE Primary pulmonary inflammatory myofibroblastic tumor (IMT) with distant metastasis is extremely rare. Moreover, metastasis of pulmonary IMT to bone marrow has never been reported in previous studies. Therapeutic approaches for anaplastic lymphoma kinase (ALK)-negative pulmonary IMT with metastasis are limited. Yet there is no report on the treatment of advanced IMT cases with anti-angiogenesis drugs. PATIENT CONCERNS We described a patient with a complaint of fatigue, with the chest computed tomography (CT) scan revealing 2 masses in bilateral lung. DIAGNOSES The CT-guided lung biopsy examined 1 lesion in the right lung, and the post-operative pathological diagnosis of ALK-negative pulmonary IMT was recommended. However, the lung lesions were found significantly enlarged during the subsequent visit 8 months later, along with multiple metastases to the bone and abdominal cavity. A bone marrow biopsy revealed bone marrow infiltration by spindle cells. INTERVENTIONS The patient began to take Celecoxib due to the rapid progression of IMT, however, resulting in the aggravated gastric ulcer. He stopped taking the medicine 1 month later, with no remarkable change in the lesions by CT. Apatinib was administrated instead of Celecoxib. OUTCOMES After the 5-month treatment of Apatinib, the mass in the abdominal cavity significantly shrank and the lung lesions slightly decreased in size. With the 9-month administration of Apatinib, the lung lesions and the abdominal mass kept stable, compared with the situation in the 5-month follow-up. LESSONS Although pulmonary IMT shows the potential of metastasis, its metastasizing to bone marrow is a highly unusual event. Apatinib is effective for pulmonary IMT, and should be taken into consideration for the treatment of inoperable pulmonary IMT patients who lack ALK rearrangement.
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Affiliation(s)
| | - Jianguo Wei
- Department of Pathology, Shaoxing People's Hospital, Shaoxing Hospital of Zhejiang University, Zhejiang
| | - Xizhong Liu
- Institute of Nonlinear Science, Shaoxing University, Shaoxing, China
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Inflammatory Myofibroblastic Tumors in Paranasal Sinus and Nasopharynx: A Clinical Retrospective Study of 13 Cases. BIOMED RESEARCH INTERNATIONAL 2018; 2018:7928241. [PMID: 30410939 PMCID: PMC6205320 DOI: 10.1155/2018/7928241] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 05/26/2018] [Accepted: 09/09/2018] [Indexed: 11/17/2022]
Abstract
Background Inflammatory myofibroblastic tumor (IMT), as a mesenchymal tumor, is common in the lung and abdomen but rare in the paranasal sinus and nasopharynx. Objective This study aimed to summarize the clinical characteristics of IMT in the paranasal sinus and nasopharynx and analyze the relationship between the treatment and the overall survival (OS). Method The clinical features, treatment, and follow-up data of patients diagnosed with IMT of the paranasal sinus or nasopharynx from 2006 to 2017 were retrospectively analyzed, and the previous literature was reviewed. Results IMT often presents as an ill-defined soft-tissue mass with bone destruction and invasion of surrounding structures. The treatment methods used in this study were different combinations of surgery, prednisone, radiotherapy, and chemotherapy or observation alone. Three of the 13 patients were lost and the follow-up time of the remaining 10 cases ranged from 2 to 87 months (median, 39 months). Two patients died of the disease; the other eight patients were stable. The 5-year survival rate was 72%. Among the four methods of treatment, only treatment with prednisone was significantly correlated with better OS (P = 0.046). Conclusions IMT is an intermediate tumor that often mimics malignancy. We are not sure if IMTs in the nasal cavity are more aggressive because of the biology or if the location and local therapy in the head region is more complicated. Radiologic findings help know the extent of the lesion. For unresectable nasal IMT, combined therapy with glucocorticoids, chemotherapy, and radiotherapy is sometimes a better choice. Glucocorticoids are especially recommended as a basic part of the integrated therapy. However, the standard treatment needs further research.
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Patel A, Kocoglu MH, Kaul A. Therapeutic strategies for durable response in plasma cell granulomas in the central nervous system. Ann Hematol 2018; 98:1027-1029. [PMID: 30178192 DOI: 10.1007/s00277-018-3490-7] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2018] [Accepted: 08/27/2018] [Indexed: 12/12/2022]
Affiliation(s)
- Ameet Patel
- Department of Medicine, University of Maryland Medical Center, 22 S Greene St, Baltimore, MD, 21201, USA.
| | - Mehmet H Kocoglu
- Division of Hematology/Oncology, Marlene and Stewart Greenebaum Comprehensive Cancer Center, University of Maryland Medical Center, Baltimore, MD, USA
| | - Akash Kaul
- American University Integrative Sciences, Tucker, GA, USA
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