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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.
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Hemangioma Cavernoso , Neoplasias Hepáticas , Neoplasias de Tecido Muscular , Adulto , Feminino , Humanos , Diagnóstico Diferencial , Hemangioma Cavernoso/diagnóstico , Hemangioma Cavernoso/patologia , Neoplasias de Tecido Muscular/diagnóstico , Neoplasias de Tecido Muscular/cirurgia , Neoplasias Hepáticas/patologiaRESUMO
BACKGROUND: Inflammatory Myofibroblastoma Tumors (IMTs) are extremely tumour rare in the intraocular. CASE PRESENTATION: A ciliary body tumor was found under slit lamp biomicroscopy in a 55-year-old male first diagnosed with cataract. Then this patient underwent trans-sclera resection via partial lamellar sclerouvectomy and par plans vitrectomy to remove the mass. Hematoxylin and eosin (HE) staining and immunohistochemistry findings showed that the characteristics of the tumor were consistent with IMT. CONCLUSIONS: We reported a rare case of intraocular IMT, which is confirmed by H&E staining, and IHC positive staining for Vimentin, Desmin and ALK, while negative staining for SMA, S-100, ki-67, CK, CD68, and calponin.
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Neoplasias de Tecido Muscular , Neoplasias Uveais , Corpo Ciliar/cirurgia , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Neoplasias de Tecido Muscular/diagnóstico , Neoplasias de Tecido Muscular/patologia , Neoplasias de Tecido Muscular/cirurgia , Neoplasias Uveais/diagnóstico , Neoplasias Uveais/patologiaRESUMO
Inflammatory myofibroblastoma (IMT) is a rare solid tumor, and its etiology and pathogenesis are unclear. Crohn's disease is a non-specific intestinal inflammatory disease. The clinical manifestations, laboratory examinations, and imaging examinations of IMT are not specific, making diagnosis difficult. A case of Crohn's disease combined with IMT of abdominal wall was admitted to the Department of Gastroenterology at the Third Xiangya Hospital, Central South University, on Nov. 21, 2017. This patient was admitted to our hospital because of repeated right lower abdominal pain for 4 years. A 6 cm×5 cm mass was palpated in the right lower abdomen. After completing the transanal double-balloon enteroscopy and computed tomographic enterography for the small intestinal, the cause was still unidentified. The patient underwent surgery due to an abdominal wall mass with intestinal fistula on Sept. 12, 2018 and recovered well currently. According to histopathology and immunohistochemistry, he was diagnosed with Crohn's disease combined with IMT. Up to July 2020, the patients still took azathioprine regularly, without abdominal pain, abdominal distension, and other discomfort, and the quality of his life was good.
Assuntos
Parede Abdominal , Doença de Crohn , Neoplasias de Tecido Muscular , Dor Abdominal , Parede Abdominal/cirurgia , Doença de Crohn/complicações , Humanos , Intestino Delgado , Masculino , Neoplasias de Tecido Muscular/complicações , Neoplasias de Tecido Muscular/diagnóstico por imagem , Neoplasias de Tecido Muscular/cirurgiaRESUMO
Inflammatory myofibroblastic tumor is considered one of the rarest benign tumors constituting 0.7% of all lung neoplasms. It was first described in 1939. We report a case of a 10-year-old child who presented with recurrent cough and fever. Chest radiography and computed tomography demonstrated complete involvement of right lung by the tumor. The tumor along with the affected lung was meticulously dissected from the surrounding structures and was delivered outside. The histopathology of the specimen revealed it to be inflammatory fibroblastoma.
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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.
Assuntos
Histiocitose Sinusal , Neoplasias de Tecido Muscular , Neoplasias , Humanos , Histiocitose Sinusal/diagnóstico , Traqueia/cirurgia , Traqueia/patologia , Anastomose CirúrgicaRESUMO
Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a highly aggressive malignant subtype of inflammatory myofibroblastoma (IMT) associated with poor prognosis. IMT can occur in various parts of the body, most frequently in the lungs, followed by the mesentery, omentum, retroperitoneum, and pelvis, among other areas; however, it is exceptionally rare in the stomach. Anaplastic lymphoma kinase (ALK) is a critical driver of lung cancer development and is currently the "gold standard" target for non-small cell lung cancer treatment. However, there are few reports on the use of ALK inhibitors for EIMS, necessitating further investigation. A male patient with postoperative inflammatory myofibroblastic sarcoma of the stomach received postoperative chemotherapy and had a stable outcome. However, a repeat CT scan performed 11 months later revealed disease progression. The patient later underwent immunohistochemistry testing that indicated ALK positivity, and next-generation sequencing revealed STRN-ALK fusion. Ensartinib 225 mg qd was administered as recommended, and the patient experienced only mild pruritus and no adverse effects such as rash. Eight months after CT follow-up, the patient's subseptal soft tissue nodules had decreased, and the outcome was assessed as a partial response. The findings of this case report introduce a novel strategy for treating ALK-positive EIMS that utilizes ensartinib, a drug with previously demonstrated success in the treatment of ALK-positive cancer.
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Background Inflammatory pseudotumor (IPT) of the skull base is a rare, locally destructive lesion managed with a variety of treatments. We explore the impact of treatment on outcome and assess the prognosis of IPT. Methods This is a retrospective review of IPT of the skull base at a tertiary academic medical center. The primary outcome was radiographic progression after treatment. Outcome versus tumor location was also examined and a prognostic model was developed using a logistic regression. Results The demographics of 21 patients with IPT are reported. Treatment consisted of corticosteroids (in 80.1% of patients), disease modifying antirheumatic drugs (DMARDs; 33.3%), surgical resection (28.6%), radiation (23.8%), antibiotics (14.3%), chemotherapy (rituximab; 9.5%), and antivirals (4.8%). At 50.7 months, 50.8% had radiographic progression. Local therapy trended toward having a better response than systemic therapy ( p = 0.60). IPT of the orbit required 2.4 treatment modalities, compared with 2.0 for pharyngeal IPT, and 1.3 for posterior skull base masses ( p = 0.14). A total of 75% orbital IPT underwent radiographic progression, compared with 71% of pharyngeal IPT and 50% of posterior skull base masses ( p = 0.62). Sixteen patients were used to create the logistic model of radiographic progression. The Cox-Snell R 2 was 0.71 ( p = 0.03). No individual variables were statistically significant. Conclusion To our knowledge, this is among the largest sample of cases describing the presentation, treatment, and prognosis of IPT of the skull base. Our data suggest that there may be an improved response with local therapy over systemic therapy and better prognosis among posterolateral skull base masses.
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Inflammatory myofibroblastic tumor (IMT) is a rare tumor with low-grade malignant risk mainly occurring in soft tissues and lungs, and it is extremely rare in the breast. Meanwhile, imaging findings of the tumor often present with non-specific features that lead to misdiagnosis and delayed treatment. Here, we report a case of inflammatory myofibroblastic tumor in the breast with the imaging findings of mammography, magnetic resonance imaging (MRI), and pathologic findings to improve the understanding of the disease. The patient was treated by surgical operation, and was followed up for 44 months, no local recurrence and distant metastasis.
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Inflammatory myofibroblastic tumors (IMTs), also known as inflammatory pseudotumor are rarely reported in the trachea. Although initially considered benign, with the recent evidence of local recurrence after surgical resection, cases of metastatic spread, and advance in cytogenetic analysis, they are now categorized as tumors with malignant potential. Correct histological diagnosis is of utmost importance, as they closely resemble other benign and malignant spindle cell tumors with myofibroblasts predominance. Treatment remains challenging taking into account the location involving the airway. Here, we describe a case of tracheal IMF in an apparently healthy young adult presenting with recurrent hemoptysis.
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Inflammatory myofibroblastoma is a distinctive pseudosarcomatous lesion that occurs primarily in the viscera and soft tissue of children and young adults. It is composed of myofibroblastic spindle cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils. We report a 41-year-old man who presented with progressive dyspnea and pain in the right hypochondrium for 6 months. USG abdomen revealed a well-defined hypoechoic rounded mass in the subdiaphragmatic region. CT thorax and abdomen revealed a large hypodense mass in posterosuperior aspect of the right lobe of liver, right pleural effusion and basal consolidation of the right lung. Right thoracotomy was performed. The tumor mass was arising from the right side of diaphragm. Total surgical excision was done. On histomorphology and immunohistochemical analysis, a diagnosis of inflammatory myofibroblastoma was done. Following excision the patient was completely relieved of dyspnea and pain. There was no recurrence on follow-up for 2 years.