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This report discusses a rare case of a soft palate deformity in a young girl due to lipofibromatosis (LPF). This rare benign pediatric soft tissue tumour usually arises in the distal extremities. We believe this case represents the first report of lipofibromatosis involving only the maxillary bone.
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Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular lesion of the spleen that was first described in 2004. SANT is associated with other concurrent diseases, mostly malignancies. Calcifying fibrous pseudotumor (CFPT) is a tumor-like lesion usually arising from soft tissue or peritoneal sub-serosa. Both SANT and CFPT are considered to be variants of the inflammatory myofibroblastic tumor. We report the rare case of a 24-year-old female presenting with a left abdominal mass that was clinically diagnosed as an extraintestinal gastrointestinal stromal tumor (GIST). Histopathological examination revealed SANT of spleen and CFPT. We report this case due to its rarity of occurrence and unusual association of SANT with CFPT.
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Inflammatory myofibroblastic tumor （IMT） 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.
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Humanos , Lactante , Granuloma de Células Plasmáticas/patología , Nasofaringe/patología , Neoplasias de Tejido Muscular , Neoplasias de los Tejidos BlandosRESUMEN
Inflammatory myofibroblastic tumor is a rare tumor of mesenchymal origin. A case of intratracheal inflammatory myofibroblastic tumor in a male child was reported. The clinical characteristics, diagnosis, treatment and prognosis of the disease were reviewed based on the literature, and a differential diagnosis between inflammatory myofibroblastic tumor and hamartoma was performed to ultimately confirm the nature of the tumor in the child.
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Humanos , Niño , Masculino , Tráquea/patología , Granuloma de Células Plasmáticas/diagnóstico , Pronóstico , Diagnóstico Diferencial , Tomografía Computarizada por Rayos XRESUMEN
El tumor miofibroblástico inflamatorio primario gástrico es una neoplasia rara desarrollada de células madre mesenquimales, e infrecuentemente discutido en la literatura científica. El diagnóstico clínico a través de endoscopia y patología es desafiante para el equipo. Nosotros reportamos el caso de una paciente mujer con síndrome de obstrucción gástrica por un tumor de 10 cm diagnosticado con esta enfermedad usando histología e inmunohistoquímica.
Primary gastric inflammatory myofibroblastic tumor is a rare neoplasm developed from mesenchymal stem cells, infrequently discussed in the scientific literature. Clinical diagnosis through endoscopy and pathology is challenging for the medical team. We report the case of a female patient with gastric obstruction syndrome due to a 10 cm tumor diagnosed with this disease by histology and immunohistochemistry.
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Inflammatory myofibroblastic tumors (IMTs) are uncommon; intermediate grade soft tissue tumors occurring in young individuals with an uncertain behaviour. The incidence of pulmonary lymphangitis carcinomatosis (PLC) is around 6-8% of all pulmonary metastases. However, PLC due to papillary thyroid carcinoma (PTC) is very uncommon. We present a case of a 26-year-old male, who presented with a solitary left lung nodule on radiological scans. There was also a past history of thyroid surgery done two years back for PTC. Histology revealed a soft tissue tumor reminiscent of IMT. The periphery of the IMT nodule showed metastatic PTC in the form of extensive PLC. In view of this unusual histology, a diagnosis of PTC with nodular fasciitis-like stroma (PTC-NFS) was initially considered. However, molecular studies for anaplastic lymphoma kinase (ALK) gene rearrangement confirmed the diagnosis of IMT. This case highlights the unusual occurrence of tumor-to-tumor metastasis causing diagnostic challenges and also the importance of molecular testing.
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According to the WHO classification, mesenchymal tumors of the kidney are divided into mesenchymal tumors occurring mainly in adults and children. Accumulating evidence suggests that renal mesenchymal tumors represent a group of histologically heterogeneous diseases. We are reporting the case of a 58-year-old man with a history of nephron-sparing surgery due to renal cell carcinoma in 2014 in his left kidney. He was urgently admitted to the hospital due to left-sided nephrotoxic and anuria. Computed tomography (CT) imaging was performed and revealed irregular tumor mass. He underwent left-sided nephrectomy. Microscopic appearance and immunoprofile allowed to diagnose the tumor as an inflammatory myofibroblastic tumor (IMT) of the kidney. According to the 2016 World Health Organization (WHO) classification of tumors of the urinary system and male genital organs, IMT is a benign mesenchymal tumor with malignant potential. Typical locations are the liver and biliary tract, lung, and gastrointestinal tract. In the urogenital system, IMT occurs mainly in the bladder. So far, 48 cases have been reported in the kidney.
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Mediastinal masses span a wide histopathological spectrum. Inflammatory pseudotumors are rare and most commonly described in the lungs but these are reported in almost all the organs in the body. Mediastinal involvement is rare and difficult to diagnose. Clinical manifestations and laboratory investigations and radiology are non-specific. Histomorphology and Immunohistochemistry provide a valuable aid. Complete resection usually provides definitive diagnosis and is treatment of choice.
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@#Anterior mediastinal inflammatory myofibroblastoma is a rare tumor with insidious onset and easy misdiagnosis. In this report, we presented a case of anterior mediastinal inflammatory myofibroblastoma with thymoma. The mediastinal tumor was found by physical examination, and the prognosis was good after surgical treatment. For this disease, operation is an effective method for definite diagnosis and treatment, and complete excision can achieve good outcomes.
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The incidence of inflammatory myofibroblastic sarcoma is low, and bladder origin is more rare. We reported a 58-year-old patient with painless gross hematuria for one week. Total abdominal CT examination showed soft tissue mass in the anterior wall of the bladder, which was considered as bladder cancer, and bladder tumor resection was performed. Postoperative pathology showed inflammatory myofibroblastic sarcoma. Therefore, radical cystectomy was performed because of the high degree of malignancy. There was no recurrence during 3 years follow-up.
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Inflammatory myofibroblastic tumor (IMT)is a potentially or low-grade malignant mesenchymal neoplasm, which is rare in clinic. Renomedullary interstitial cell tumor(RICT) is a clinically rare benign renal tumor. The combination of these two diseases in one patient has not been reported. A 25-year-old female patient was admitted to the hospital due to left back pain for 12 days and hematuria for 1 week. MRI of kidneys showed a mass in the left renal pelvis, which was considered as renal pelvic carcinoma. Urine cytopathological examination was negative. Robot-assisted laparoscopic radical left nephroureterectomy was performed. There was no tumor recurrence or metastasis during the follow-up for more than 6 months after operation.
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Objective:To investigate the efficacy and safety of targeted therapy with Crizotinib for children with ALK gene mutation positive inflammatory myofibroblastic tumor (IMT). Methods:A retrospective analysis was performed on 4 children with ALK gene mutation positive IMT admitted to Shanghai Children′s Hospital from January 2019 to June 2021.Among them, 3 cases were given the targeted drug Crizotinib[280 mg/(m 2· time), q12h] orally, and 1 case was observed after complete tumor resection to analyze the efficacy and adverse drug reactions. Results:All 4 cases were male, aged from 2 years and 3 months to 11 years and 3 months.The tumors originated from the abdominal cavity in 2 cases, the right orbit in 1 case, and the right lung in 1 case.Pathological immunohistochemistry and fluorescence in situ hybridization were both positive for ALK gene mutation, and complete remission was achieved after comprehensive treatment.Among them, 3 patients were treated with oral Crizotinib, and 2 patients were tried to stop taking the drug for 1 year, relapsed 1 month later, and still achieved complete remission after the second treatment.The 4 cases were followed up for 8-30 months, and all survived.All the cases showed no abnormalities in blood image, liver and kidney function, myocardial enzyme profile, cardiac function, hearing and vision, and 2 cases showed prolonged Q-T interval in the course of Crizotinib treatment, which could be recovered by temporary withdrawal of drug, and no abnormality in electrocardiogram was found in continued drug use. Conclusions:Crizotinib was used to treat ALK mutation positive IMT, shrink tumor and consolidate postoperative treatment, which is a good choice for IMT in children with difficult surgical resection and refractory recurrence.
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Inflammatory myofibroblastic tumor (IMT) of gastrointestinal tract is a rare and low malignant mesenchymal tumor, which is composed of differentiated myofibroblastoid spindle cells and often accompanied by numerous inflammatory cells such as plasma cells, lymphocytes, and eosinophils. Surgical resection is the preferred treatment for IMT of gastrointestinal tract, and patients can achieve a good prognosis after surgery. In recent years, with the progress of imaging examination and pathological diagnosis technology, the detection rate of IMT of gastrointes-tinal tract has been greatly improved, but its pathogenesis and mechanism have not been completely investigated, requiring further research results to confirm. At the same time, due to the lack of standardized strategies for diagnosis and treatment of IMT of gastrointestinal tract, it was inevitable to miss diagnosis or treatment in clinical diagnosis and treatment. By reviewing relevant literatures and combined with the team's practical experience, the authors summarize the research progress of the diagnosis and treatment of IMT of gastrointestinal tract from the aspects of pathogenesis, clinical diagnosis and treatment strategies of IMT, aiming to provide references for the clinical treatment of surgeons.
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Objective:To investigate the efficacy of anaplastic lymphoma kinase-tyrosine kinase inhibitors (ALK-TKI) in treatment of inflammatory myofibroblastic tumor (IMT).Methods:The clinicopathological data of one recurrent abdominal IMT patient in Renmin Hospital of Wuhan University in 2018 were retrospectively analyzed. The clinicopathological and molecular characteristics, ALK-TKI treatment efficacy and prognosis of 41 patients with IMT reported in the literature from January 2010 to August 2020 were systematically reviewed.Results:This patient with abdominal IMT in Renmin Hospital of Wuhan University was a 27-year-old female who relapsed 2 months after surgery. Chemotherapy combined with bevacizumab was ineffective. After oral administration of crizotinib, the condition resolved after 1 month, and complete remission (CR) was achieved after 29 months. The median age of onset of 41 IMT cases reported in the literature was 22 years old (0-61 years old), of which 32 cases (78.0%) had multiple organ involvement, all of which had recurrence or metastasis. There were 38 cases of ALK mutation and 3 cases of TFG-ROS1 fusion gene-positive. Thirty-four patients treated with crizotinib in the first-line treatment of ALK-TKI, and the median resistance time of crizotinib was 8 months (2-48 months). The total clinical benefit rate of ALK-TKI was 85.3% (29/34), and 20 patients achieved CR. The median time for the first CR was 11 months (4-36 months), and the median duration time of medication for CR patients was 19.5 months (2-60 months). The median progression-free survival (PFS) time of 24 patients who underwent surgery and/or chemotherapy and radiotherapy was 4 months (1-45 months); after progression, ALK-TKI treatment was performed, and the median PFS time was 14 months (3-62 months).Conclusions:IMT is a true neoplasm with characteristics of recurrence and metastasis. Reasonable combination of ALK-TKI with surgery, radiotherapy and chemotherapy can improve the prognosis of IMT patients.
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It is a rare mesenchymal tumour of intermediate biologic potential (according to World Health Organization), with unknown aetiology. It is benign tumour with malignant potential. It frequently recurs and rarely metastasizes. Abdominopelvic inflammatory myofibroblastic tumours have the recurrence rate of 85% so meticulous follow up is necessary. Complete surgical excision is the main stay treatment.
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Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare entity and a novel variant of inflammatory myofibroblastic tumor (IMT), usually seen in children and nonsmoking young adults. Their occurrence in a posttransplant setting is still rare. These tumors are characterized by prominent epithelioid morphology, large histiocytoid “Reed Sternberg”-like cell, unique pattern of ALK immuno-reactivity, and aggressive clinical behavior. Their etiology and metastatic potential is controversial. In a post-transplant setting, many factors such as trauma, infections with EBV, HIV, Hepatitis C, mycobacteria, fungus, and chemotherapy-induced immunosuppression have been implicated in their etiology. We present the case of a 2-year-old female child who developed multiple omental and mesenteric tumor nodules, 8 months post liver transplant for progressive familial intrahepatic cholestasis (PFIC). Following a histopathological diagnosis of “mesenchymal neoplasm of possible malignant nature” on a trucut biopsy and frozen section, tumor debulking was performed. A final histological diagnosis of EMIS was made on the completely resected tumor. The patient remains in remission nearly 7 months after presentation, without any follow-up systemic chemotherapy. IMT after a solid organ transplant is rare, only 5 cases have been reported in the literature until now. Similar phenomenon has also been noted with hematopoietic stem cell transplant. However, to our knowledge, this case of EMIS in a post liver transplant patient is first of its kind.
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INTRODUCCIÓN: El tumor miofibroblástico inflamatorio (TMI) es una neoplasia benigna infrecuente, de comportamiento clínico impredecible. OBJETIVOS: describir 3 casos de TMI diagnosticados entre marzo 2014 y enero 2018 en Hospital Clinico San Borja Arriaran, y realizar una revisión actualizada de la literatura. CASO 1: Adolescente de género masculino de 14 años de edad, hospitalizado por dolor abdominal, diagnosticado de invaginación yeyunoyeyunal secundaria a un tumor de pared intestinal. La histología fue compatible con un tumor miofibroblástico inflamatorio. CASO 2: Adolescente de género femenino, edad 12 años, hospitalizada por neumonía y dolor lumbar en estudio asociado a pérdida de peso. Se diagnosticó una masa retroperitoneal que comprometía el músculo psoas derecho, músculos paravertebrales, vértebras, riñón derecho y diafragma ipsilateral. Se efectuó biopsia por punción cuyo resultado fue compatible con un tumor miofibroblástico inflamatorio. CASO 3: Preadolescente de género femenino de 11 años de edad, hospitalizada para estudio de infección del tracto urinario a repetición. Se identificó un tumor vesical y la biopsia mostró ser compatible con tumor miofibroblástico inflamatorio. CONCLUSIÓN: Debido al comportamiento variable del tumor miofibroblástico inflamatorio, el manejo de este dependerá de la localización, la expresión del anaplasic like lymphoma (ALK), el comportamiento del tumor y la posibilidad de resección.
INTRODUCTION: The inflammatory myofibroblastic tumor is an infrequent benign neoplasm with unpredictable cli nical behavior. OBJECTIVES: to describe three clinical cases at the San Borja Arriarán Clinical Hospital between March 2014 and January 2018 and to carry out an updated review of the literature. CASE 1: 14-year-old male adolescent, hospitalized due to abdominal pain, diagnosed with jejunojejunal intus susception secondary to an intestinal wall tumor. The histology was compatible with an inflamma tory myofibroblastic tumor. CASE 2: 12-year-old female adolescent, hospitalized due to pneumonia and low-back pain under study associated with weight loss. A retroperitoneal mass was diagnosed involving the right psoas muscle, paravertebral muscles, vertebrae, right kidney, and ipsilateral dia phragm. A puncture biopsy was performed and the result was compatible with an inflammatory myofibroblastic tumor. CASE 3: 11-year-old female pre-adolescent, hospitalized to study recurrent urinary tract infection. A bladder tumor was identified, and the biopsy showed compatibility with inflammatory myofibroblastic tumor. CONCLUSION: Due to the variable behavior of the inflammatory myofibroblastic tumor, its management will depend on the location, expression of the anaplastic lymphoma kinase (ALK), tumor behavior, and the resection possibility.
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Humanos , Masculino , Femenino , Niño , Adolescente , Neoplasias Retroperitoneales/diagnóstico , Neoplasias de la Vejiga Urinaria/diagnóstico , Neoplasias Intestinales/diagnóstico , Neoplasias Retroperitoneales/patología , Neoplasias de la Vejiga Urinaria/patología , Miofibroblastos/patología , Inflamación/diagnóstico , Inflamación/patología , Neoplasias Intestinales/patologíaRESUMEN
Inflammatory myofibroblastic tumor (IMT) is a rare tumor of unknown etiology and pathogenesis. The lesion has been recognized to occur at various sites but rarely affects the head and neck region. A 29-year-old male presented with a 13 months' history of a slow growing, painless growth in maxillary left posterior gingiva. An excisional biopsy was performed under local anesthesia. Microscopic examination revealed a compact cellular spindle cell proliferation with collagenous stroma having storiform architecture. Immunohistochemistry revealed that the tumor cells were positive for smooth muscle actin, CD-68 and negative for anaplastic lymphoma kinase. Oral IMT should be included in the differential diagnosis of localized gingival enlargement mimicking oral hyperplastic/reactive lesions
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Objective To investigate clinical, pathological and radiographic features of inflammatory myofibroblastic tumor (IMT) of the nasal cavity. Methods Four cases of pathologically confirmed IMT in the Shengjing hospital of China Medical University from April 2015 to April 2017 were retrospectively analyzed. Three dimensional CT and enhanced MRI was performed in all patients. Results The main symptoms were local obstruction, oppression or infringement. In 4 cases, IMT was single, with 2 cases of primary maxillary sinus, 1 case of nasal cavity, and 1 case of nasal septum;Pathological morphology presented diversity, consisting mainly of spindle-shaped cells which distributed in bundles or whirlpools, and showed inflammatory cell infiltration. Immunohistochemical results showed SMA (+) in 4 cases, Desmin (-) in 4 cases , and ALK (-) in 3 cases. CT plain tumors showed mostly uniform soft tissue densities. MR showed more enhancement of tumors, 1 case with necrotic cystic changes in the maxillary sinus IMT tumors, showed a significant ring after enhancement. Conclusions The IMT of nasal cavity and paranasal sinuses is rare and the clinical symptoms are non-specific. The diagnosis mainly depends on pathological examination and immunohistochemical staining. Preoperative CT and MRI examinations can not only provide information on the lesion location, extent, and adjacent tissue invasion, but also on the blood supply of the tumor and the internal components of the tissue, which will facilitate the choice of treatment options.