ABSTRACT
BACKGROUND: Hepatic inflammatory myofibroblastic tumours (HIMTs) are rare and poorly described in the literature. Most publications are single patient case reports and lack detailed reporting on characteristics, management, and outcomes. This systematic review aimed to assess the demography, clinical presentation, typical imaging features, histopathology, treatment, and outcomes of patients presenting with HIMTs. METHODS: A systematic literature search was performed in MEDLINE (PubMed), EMBASE (Scopus), JSTOR, Cochrane CENTRAL (Cochrane Library), and the databases included in the Web of Science for studies published between 1940 and 2023 on HIMTs, including its reported synonyms. Case series or cohort studies that reported on the management and outcomes of at least four patients with histologically confirmed HIMTs were included in the analysis. RESULTS: After screening 4553 publications, 22 articles including a total of 440 patients with confirmed HIMTs were eligible for inclusion. The average age was 53.4 years (range 42.0-65.0) with a male to female ratio of 1.7:1. Abdominal pain, discomfort, fever, and loss of weight were the most common presenting symptoms. Surgical resection is the standard of care for HIMTs and is associated with low mortality of 3.4% and low disease recurrence. CONCLUSION: HIMT is a disease more often affecting middle-aged males. The lesions are typically solitary with low recurrence after treatment. The relative roles of surgical versus medical treatment remain unclear. Differences in clinical presentation, histopathology, and treatment of HIMTs compared to inflammatory myofibroblastic tumour (IMT) at extrahepatic sites could challenge the current view of IMT as a single pathological entity.
Subject(s)
Liver Neoplasms , Humans , Liver Neoplasms/pathology , Liver Neoplasms/therapy , Liver Neoplasms/surgery , Granuloma, Plasma Cell/surgery , Granuloma, Plasma Cell/pathology , Granuloma, Plasma Cell/diagnosis , Male , Neoplasms, Muscle Tissue/surgery , Neoplasms, Muscle Tissue/pathology , Neoplasms, Muscle Tissue/diagnosis , Female , Middle AgedABSTRACT
INTRODUCTION: Inflammatory myofibroblastic tumor (IMT) is a rare invasive soft tissue tumor. Many IMTs are positive for anaplastic lymphoma kinase (ALK) with ALK gene fusion; other gene mutations have also been reported, which indicates a key role for genetic testing and the development of target therapy to optimize treatment strategies. PATIENT CONCERNS: We report 2 patients who obtained clinical benefits following targeted treatment with ensartinib. DIAGNOSIS: The first patient was diagnosed as IMT, with TFG-ROS1 fusion gene mutation. The second patient was IMT harboring the ALK-STRN fusion gene mutation. INTERVENTIONS: We performed gene testing for these 2 patients. According to the test result, both patients received ensartinib 225 mg QD as targeted therapy for a 30-day cycle. OUTCOMES: The first patient achieved partial remission and maintained a stable state for 14.7 months. The second patient was treated for 10 months and reached complete remission after 5 months and is currently still benefiting from treatment. Treatment-related side effects were mild in both patients. CONCLUSION: Our cases provided some new insights and approaches for the clinical diagnosis and treatment of IMT.
Subject(s)
Neoplasms, Muscle Tissue , Humans , Female , Male , Neoplasms, Muscle Tissue/drug therapy , Neoplasms, Muscle Tissue/genetics , Neoplasms, Muscle Tissue/pathology , Adult , Anaplastic Lymphoma Kinase/genetics , Middle Aged , Soft Tissue Neoplasms/drug therapy , Soft Tissue Neoplasms/genetics , Soft Tissue Neoplasms/pathology , Antineoplastic Agents/therapeutic useSubject(s)
Lung Neoplasms , Female , Humans , Bronchoscopy/methods , Diagnosis, Differential , Lung Neoplasms/pathology , Lung Neoplasms/diagnostic imaging , Neoplasms, Muscle Tissue/pathology , Neoplasms, Muscle Tissue/surgery , Neoplasms, Muscle Tissue/diagnostic imaging , Neoplasms, Muscle Tissue/diagnosis , Plasma Cell Granuloma, Pulmonary/pathology , Plasma Cell Granuloma, Pulmonary/diagnostic imaging , Plasma Cell Granuloma, Pulmonary/surgery , Plasma Cell Granuloma, Pulmonary/diagnosis , Tomography, X-Ray Computed , AgedABSTRACT
ABSTRACT: Inflammatory myofibroblastic tumour in paediatric patients present with a diagnostic dilemma because of its clinical, radiological and histopathological features overlapping with other mesenchymal tumours common in this age. Because of its rarity, the exact features are still unclear. Here, we are reporting clinical, radiological and histopathological appearances of two such cases. In both cases, the exact diagnosis was confirmed only after immunohistochemistry. There is a need for further detailed study to exactly determine the natural course and prognosis of these tumours.
Subject(s)
Neoplasms, Muscle Tissue , Child , Humans , Neoplasms, Muscle Tissue/diagnosisSubject(s)
Lung Neoplasms , Neoplasms, Multiple Primary , Humans , Child , Lung Neoplasms/surgery , Lung Neoplasms/pathology , Lung Neoplasms/diagnostic imaging , Lung Neoplasms/diagnosis , Neoplasms, Multiple Primary/surgery , Neoplasms, Multiple Primary/pathology , Male , Adenocarcinoma/surgery , Adenocarcinoma/pathology , Neoplasms, Muscle Tissue/surgery , Neoplasms, Muscle Tissue/pathology , Pneumonectomy/methods , Tomography, X-Ray ComputedABSTRACT
BACKGROUND: Tumor-like lesions of the trachea are rare and challenging in diagnosis and management. Inflammatory myofibroblastoma, also known as Inflammatory pseudo tumors (IPTs), as well as Rosai Dorfman Disease (RDD) are inflammatory lesions that may involve the central airways with variable non-specific clinical features mimicking tumors. CASE PRESENTATION: In this study 2 cases with tumor-like lesions are presented. One case with an inflammatory pseudotumor and the other one with Rosai-Dorfman disease affecting the upper trachea. Both cases were successfully managed with tracheal resection anastomosis. CONCLUSION: Tracheal Inflammatory myofibroblastoma, and Rosai-Dorfman diseases are rare tumor like lesions that present with upper airway obstruction. Despite being benign, these lesions may have features suggestive of malignancy, requiring prompt management. Complete surgical excision by segmental resection and primary anastomosis (if feasible) is the treatment of choice with an optimum outcome.
Subject(s)
Histiocytosis, Sinus , Neoplasms, Muscle Tissue , Neoplasms , Humans , Histiocytosis, Sinus/diagnosis , Trachea/surgery , Trachea/pathology , Anastomosis, SurgicalABSTRACT
INTRODUCTION: Lymphadenopathies are a major cause of consultation in internal medicine, with various causes of diagnosis. Unexplained persistent lymphadenopathy must be biopsied to rule out malignant tumor. CASE REPORT: We report the case of a 53-year-old man, with inguinal lymphadenopathy evolving for more than one year. The patient had no associated symptoms and his blood tests were unremarkable. Due to the progression of the adenopathy and its hypermetabolism on PET-CT, an excisional biopsy was performed. Histological analysis revealed an intranodal proliferation of spindle cells with a palisading pattern. ß-catenine and smooth muscle actin labelling were positive, leading to the diagnosis of intranodal palisaded myofibroblastoma, a benign tumour. CONCLUSION: Intranodal palisaded myofibroblastoma is a rare benign cause of adenopathy, with often inguinal lymph node localization and slow growth and without risk of recurrence after surgical removal.
Subject(s)
Lymphadenopathy , Neoplasms, Muscle Tissue , Male , Humans , Middle Aged , Positron Emission Tomography Computed Tomography , Lymph Nodes/pathology , Lymphadenopathy/diagnosis , Lymphadenopathy/etiology , Neoplasms, Muscle Tissue/complications , Neoplasms, Muscle Tissue/diagnosis , Neoplasms, Muscle Tissue/pathology , BiopsyABSTRACT
A 37-year-old female patient was admitted to the hospital with a large liver mass, diagnosed as hepatic inflammatory myofibroblastic tumour (HIMT), characterized by unique radiographic features and predominantly occurring in adults. HIMT consists of myofibroblast spindle cells infiltrated by plasma cells and/or lymphocytes, with an unclear aetiology linked to factors like infection and immune response. Treatment typically involves surgical resection, with chemotherapy or targeted therapy options for cases of incomplete resection or metastasis, emphasizing the need for precise diagnosis and tailored treatment strategies.
Subject(s)
Hemangioma, Cavernous , Liver Neoplasms , Neoplasms, Muscle Tissue , Adult , Female , Humans , Diagnosis, Differential , Hemangioma, Cavernous/diagnosis , Hemangioma, Cavernous/pathology , Neoplasms, Muscle Tissue/diagnosis , Neoplasms, Muscle Tissue/surgery , Liver Neoplasms/pathologyABSTRACT
The authors present a case of a proliferative nodule located beneath an infant's lower lip that was initially discovered on prenatal ultrasound and fetal magnetic resonance imaging (MRI). Biopsy revealed a smooth muscle actin-positive spindled cell proliferation with hemangiopericytoma-like vessels consistent with infantile myofibromatosis (IM). Since the location prevented surgical management, the clinicians opted to observe the lesion. Ultimately, the lesion fully regressed on its own confirming conservative management is an option for isolated IM.
Subject(s)
Magnetic Resonance Imaging , Humans , Female , Infant , Myofibroma/pathology , Myofibroma/diagnosis , Pregnancy , Myofibromatosis/congenital , Myofibromatosis/pathology , Myofibromatosis/diagnosis , Ultrasonography, Prenatal , Prenatal Diagnosis , Lip Neoplasms/pathology , Lip Neoplasms/surgery , Lip Neoplasms/diagnosis , Neoplasms, Muscle Tissue/pathology , Neoplasms, Muscle Tissue/diagnosis , Neoplasms, Muscle Tissue/surgery , MaleSubject(s)
Humans , Female , Child , Inpatients , Physical Examination , Pediatrics , Neoplasms, Muscle Tissue , Lung Injury , Radiography, ThoracicABSTRACT
Inflammatory myofibroblastic tumor ï¼IMTï¼ is a rare mesenchymal soft tissue tumor characterized by borderline or low-grade malignancy. It is rare childhood tumor with an average age of onset of 10 years old. It is even rarer in infants and toddlers, and the etiology and pathogenesis of this tumor are still unclear. The clinical presentation of IMT is non-specific and are related to the location of the tumor. When the tumor compresses adjacent organs, it can cause pain and functional impairment. According to the current literature, IMT is most commonly found in the digestive and respiratory systems, but also occasionally occur in the genitourinary system, head and neck, and limbs. At present, there have been no reports of nasopharyngeal IMT involving nasal cavity of infants and toddlers at home and abroad.This article reports a case of a massive inflammatory myofibroblastic tumor involving the nasal cavity and nasopharynx in an infant. Plasma-assisted minimally invasive surgery was performed through multiple surgical approaches and achieved satisfactory therapeutic results. This case report may provide valuable reference for the treatment of similar diseases.
Subject(s)
Granuloma, Plasma Cell , Neoplasms, Muscle Tissue , Soft Tissue Neoplasms , Humans , Infant , Granuloma, Plasma Cell/pathology , Nasopharynx/pathologyABSTRACT
Intranodal palisaded myofibroblastoma (IPM) is a rare stroma-derived spindle-cell neoplasm of the lymph node with myofibroblastic differentiation and CTNNB1 (β-catenin gene) somatic mutations. We present a case of IPM found incidentally in the staging of lung adenocarcinoma. We describe the major histopathological and phenotypic features, including a palisaded bland spindle cell proliferation with myofibroblastic differentiation and Wnt pathway activation by immunohistochemistry, including β-catenin expression. Production of osteoid-like collagen directly from tumor cells was observed. We confirmed p.Gly34Arg CTNNB1 mutation by direct sequencing. We also reviewed the literature for similar cases.(AU)
El miofibroblastoma en empalizada intraganglionar linfático (MEIG) es una neoplasia infrecuente de células fusiformes del estroma del ganglio linfático con diferenciación miofibroblástica y mutaciones en CTNNB1 (gen de la β-catenina). Aquí mostramos el caso de un paciente con MEIG encontrado incidentalmente en la estadificación por un adenocarcinoma de pulmón. Se describen las características histopatológicas principales de la entidad, incluyendo una proliferación de células fusiformes con escasa atipia, empalizadas celulares y diferenciación miofibroblástica con activación de la vía Wnt, incluyendo expresión inmunohistoquímica de β-catenina. Se observó producción de colágeno de tipo osteoide por parte de las células tumorales. Se confirmó la presencia de la mutación p.Gly34Arg de CTNNB1 mediante secuenciación directa. Se recogen adicionalmente publicaciones de casos similares al nuestro.(AU)
Subject(s)
Humans , Female , Aged , Mutation , Neoplasms, Muscle Tissue , Stromal Cells , Wnt Signaling Pathway , beta Catenin , Lung Neoplasms , Pathology, Clinical , Inpatients , Physical Examination , Symptom AssessmentABSTRACT
We present the case of a vulval superficial myofibroblastoma with a lymphocytic and eosinophilic rim in a woman in her late 20s. The tumour presented in pregnancy as a cystic lesion with pain and increasing size. While the histopathology of superficial myofibroblastomas has been well defined in the literature, to our knowledge, there has been no documentation of the presence of an inflammatory infiltrate of lymphocytes and eosinophils surrounding and within the tumour. This may potentially act as a diagnostic or prognostic reference.
Subject(s)
Eosinophilia , Neoplasms, Muscle Tissue , Vulvar Neoplasms , Female , Humans , Eosinophilia/pathology , Eosinophils/pathology , Lymphocytes/pathology , Neoplasms, Muscle Tissue/diagnosis , Neoplasms, Muscle Tissue/surgery , Neoplasms, Muscle Tissue/pathology , Vulvar Neoplasms/diagnosis , Vulvar Neoplasms/surgery , Vulvar Neoplasms/pathology , AdultABSTRACT
Intranodal palisaded myofibroblastoma (IPM) is a rare stroma-derived spindle-cell neoplasm of the lymph node with myofibroblastic differentiation and CTNNB1 (ß-catenin gene) somatic mutations. We present a case of IPM found incidentally in the staging of lung adenocarcinoma. We describe the major histopathological and phenotypic features, including a palisaded bland spindle cell proliferation with myofibroblastic differentiation and Wnt pathway activation by immunohistochemistry, including ß-catenin expression. Production of osteoid-like collagen directly from tumor cells was observed. We confirmed p.Gly34Arg CTNNB1 mutation by direct sequencing. We also reviewed the literature for similar cases.
Subject(s)
Neoplasms, Muscle Tissue , beta Catenin , Humans , beta Catenin/genetics , beta Catenin/metabolism , Wnt Signaling Pathway/genetics , Lymph Nodes/pathology , Neoplasms, Muscle Tissue/genetics , Neoplasms, Muscle Tissue/metabolism , Neoplasms, Muscle Tissue/pathology , MutationABSTRACT
ABSTRACT: A 31-year-old man with multiple intrahepatic inflammatory myofibroblastoma tumor was referred to nuclear medicine department to assess its malignant potential. Multiple lesions in the liver exhibited 68 Ga-FAPI uptake at different degrees. Instead, there was no abnormal 18 F-FDG activity in the other hepatic lesions under the normal liver background except for the puncture site. This case reflects tumor heterogeneity of the disease and shows the potential value of 68 Ga-FAPI PET/CT for the evaluation of hepatic inflammatory myofibroblastoma tumor.
Subject(s)
Liver Neoplasms , Neoplasms, Muscle Tissue , Male , Humans , Adult , Fluorodeoxyglucose F18 , Positron Emission Tomography Computed Tomography , Positron-Emission TomographyABSTRACT
This report describes a 14-year-old male with a rare paratesticular inflammatory myofibroblastic tumor that presented atypically with acute unilateral scrotal pain and swelling. This presentation, which raised suspicion for testicular torsion, contrasts with the typical presentation of a slow-growing scrotal mass. Scrotal exploration revealed an infarcted right testis, demonstrating this locally aggressive tumor can undergo vascular invasion and occlude testicular blood supply. Thus, inflammatory myofibroblastic tumor should be considered in the differential diagnosis when evaluating patients with acute scrotal pain suspicious for testicular infarction.
