[Intranodal palisaded myofibroblastome: A rare cause of inguinal lymphadenopathy]. / Le myofibroblastome palissadique intraganglionnaire : une cause rare d'adénopathie inguinale.

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.

A case of Crohn's disease combined with inflammatory myofibroblastoma of abdominal wall. / å ‹ç½—æ©ç— åˆå¹¶è ¹å£ç‚Žæ€§è‚Œçº¤ç»´æ¯ç»†èƒžç˜¤1例.

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.

A lesson in clinicopathological correlation: Plaque-like myofibroblastic tumour presenting as a pruritic plaque in childhood.

Plaque-like myofibroblastic tumour (PLMT) is a rare skin condition which presents in childhood and infancy as a nodular fibrous plaque. Including our case, there are currently only 14 cases reported in the literature. Although it represents a well-defined clinicopathological diagnosis, there is significant under-reporting of this condition secondary to under-recognition and potential misdiagnosis as dermatofibroma.

Laryngeal Inflammatory Myofibroblastic Tumor With Anemia and Thrombocytosis.

Inflammatory myofibroblastic tumor (IMT) of the larynx is a rare benign lesion that commonly occurs in the soft tissues. We present the first case with systemic manifestations of laryngeal IMT that was associated with hypochromic, microcytic-type anemia and thrombocytosis.

Anaplastic lymphoma receptor tyrosine kinase-negative inflammatory myofibroblastic tumor of triceps brachii: Case report.

Inflammatory myofibroblastic tumor (IMT) is a non-neoplastic benign lesion comprising various inflammatory cells, including myofibroblasts and vascular tissues. It is a rare tumor that sometimes shows similar signs and progression as malignant tumors. The anatomical sites of IMTs include the lungs, liver, orbit, skin, mesentery, and maxillary sinus, but they rarely occur in the limb musculoskeletal system. To our knowledge, no case of neurological symptoms caused by the tumor in the triceps brachii muscle has been reported. In this article, we report the case of a 42-year-old male patient with an IMT that grew rapidly in the triceps brachii muscle and consequently caused symptoms of ulnar nerve lesion owing to its increasing size. The patient showed no ulnar nerve lesion symptoms after undergoing wide excision and was diagnosed with anaplastic lymphoma receptor tyrosine kinase- negative IMT.

Intrapericardial inflammatory myofibroblastic tumour in a 3-month-old infant associated with Coronavirus OC43 presenting with pericardial tamponade.

Inflammatory myofibroblastic tumour usually has a benign course and is very rarely associated with the heart. It can have life-threatening consequences, depending on its position or the presence of aggressive and metastatic complications. A 3-month-old boy presented with pericardial tamponade and was diagnosed with intrapericardial inflammatory myofibroblastic tumour associated with Coronavirus OC43. A large tumour attached to the left ventricle was completely removed by surgical resection and he made a full recovery. ABBREVIATIONS: ALK: anaplastic lymphoma kinase; CMV: cytomegalovirus; CRP: C-reactive protein; EB: Epstein-Barr virus; ESR: erythrocyte sedimentation rate; IM: inflammatory myofibroblastic tumour; NSAI: non-steroidal anti-inflammatory drugs; PTFE: polytetrafluoroethylene; SMA: smooth muscle actin.

Acute Intestinal Obstruction Due to Ileocolic Intussusception in an Adult; A Rare Presentation of Inflammatory Myofibroblastic Tumor.

BACKGROUND Intussusception is not very common in adults, and acute intestinal obstruction with intussusception due to inflammatory myofibroblastic tumor (IMT) is extremely rare. IMT is an uncommon lesion and has no single defined cause. It predominantly affects the pediatric age group and commonly involves the lungs. Here we present a case of IMT causing ileocolic intussusception leading to acute intestinal obstruction in an adult. CASE REPORT A 40-year-old female came to the emergency department with severe colicky pain in her abdomen, and reported 6 to 7 episodes of vomiting with bilious contents, along with an inability to pass feces and flatus for 3 days. An x-ray of her abdomen in erect posture revealed multiple air-fluid levels. Because she had a previous history of tuberculosis, a possible tubercular stricture as the cause of her acute obstruction was considered; an exploratory laparotomy was performed showing her bowel loops were dilated with ileocolic intussusception. The lead point of intussusception (a well-defined 4×4×3.5 cm solid mass), was found at 15 cm proximal to the ileocecal junction. A right hemicolectomy with ileo-transverse anastomosis was performed. The histopathological examination confirmed the presence of IMT. CONCLUSIONS IMT causing ileocolic intussusception with acute intestinal obstruction is an extremely rare presentation of an uncommon entity in adults. High index of suspicion, and appropriate investigations (x-ray abdomen, ultrasound, computed tomography, and colonoscopy) depending on presentation and clinical condition of the patient can result in prompt diagnosis and early management.

Unusual Nasal Insufficiency in an Infant: What's Behind the Nostril?

OBJECTIVES: Inflammatory myofibroblastic tumor (IMT) is a rare benign lesion, especially in the pediatric age. There are several cases described in pulmonary, digestive and renal localizations, but involvement in head and neck area is infrequent. METHODS: Case report and review of the literature. RESULTS: A 1 year and 11 months old child, during 2 months had clinical signs of nasal respiratory insufficiency and epistaxis subsequently developing a purulent rhinorrhea and a sleep apnea. His pediatrician previously requested a sinus and cavum X-ray with the finding of an image compatible with an intranasal mass. Endoscopic resection was performed of the mass with further immunohistochemical analysis showing the result of a lesion compatible with IMT. CONCLUSIONS: Even though there are very few cases in scientific literature of a tumor with these characteristics in infants, IMT must be present as a differential diagnosis of intranasal masses. The role of the pathologist is essential to reach the definitive diagnosis and the performance of an early surgical treatment decreases aesthetic consequences in this pathology.

Periadrenal inflammatory myofibroblastic tumour: half a decade before cure.

A 30-year-old ex-smoker with a background history of childhood asthma presented with worsening shortness of breath despite receiving high doses of oral corticosteroid for pemphigus vulgaris which was diagnosed 5 years earlier. A high-resolution CT examination of the thorax reported non-specific bronchiectatic changes and revealed an incidental suprarenal mass. A subsequent CT scan confirmed a large adrenal mass with areas of necrosis and calcification. Serum renin and aldosterone, urinary catecholamine and 5-hydroxyindoleacetic acid were within normal limits. Surgical intervention was delayed due to difficulty in optimising preoperative respiratory functions. He finally underwent a midline laparotomy for removal of the tumour. Histopathological examinations revealed extrapulmonary inflammatory myofibroblastic tumour arising from the periadrenal soft-tissue, with presence of normal adrenal gland. He showed immediate improvements of his asthmatic symptoms and pemphigus vulgaris following the surgery. His oral steroid was rapidly reduced and he achieved complete remission 2 months later.

A rare oesophageal inflammatory myofibroblastic tumour treated by endoscopy.

An inflammatory myofibroblastic tumour is a mesenchymal neoplasm that mostly involves the lung and rarely involves the oesophagus. Surgery has been most commonly used for the treatment of oesophageal inflammatory myofibroblastic tumours but there are no definite guidelines for their diagnosis and treatment. We describe the case of a 60-year-old woman presenting with dysphagia and poor appetite who was diagnosed with a submucosal oesophageal tumour by contrast enhanced computed tomography and ultrasonography endoscopy. She was treated successfully by endoscopic submucosal dissection with no complications. The final diagnosis was confirmed by pathological examination.

Inflammatory myofibroblastic tumor of the lung.

Inflammatory myofibroblastic tumors (IMT) of the lung, first reported in 1939, are considered a subset of inflammatory pseudo -tumors. They are a distinctive lesions composed of myofibroblastic spindle cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils. IMTs may be benign, invade surrounding structures, undergo malignant transformation, recur or may even metastasize. They can occur due to a genetic mutation or can occur secondary to infectious or autoimmune diseases. Patients may be asymptomatic, or present with cough, hemoptysis, dyspnea, pleuritic pain, constitutional symptoms or pneumonia. In this article we review the pathophysiology, genetics, clinical presentation, imaging findings of IMT of the lung. We also discuss the various surgical and non-surgical treatment options and the prognosis associated with this disease.

Inflammatory myofibroblastic tumors-A retrospective analysis of the Cooperative Weichteilsarkom Studiengruppe.

BACKGROUND: Inflammatory myofibroblastic tumors (IMTs) are a rare subgroup of soft tissue tumors. The outcome of patients with IMT has been reported as favorable when the tumor is completely resected. If surgical resection is not possible, systemic therapy has to be considered. However, the best systemic treatment and response rates are currently unclear. METHODS: Thirty-eight patients under the age of 21, who were registered between 2000 and 2014 with a primary diagnosis of IMT, were analyzed. RESULTS: IMT was typically localized intra-abdominally or in the pelvis. In 20 patients, the tumor was resected without further therapy; 17 patients were in complete remission at last evaluation and two patients were in partial remission. Eighteen patients received systemic therapy, 15 of whom had macroscopically incomplete resection. Systemic therapy most commonly consisted of regimens with dactinomycin, ifosfamide or cyclophosphamide, and vincristine, with or without doxorubicin, and it seemed to reduce tumor extension in individual cases. Five-year event-free survival was 74 ± 14% and 5-year overall survival was 91 ± 10% for all patients. The patients who died due to the disease were those with incomplete resection (n = 3). CONCLUSIONS: Surgery without further systemic therapy was a feasible and acceptable therapeutic option for every second patient with IMT. Standard chemotherapy for pediatric soft tissue sarcoma produced favorable results in individual cases and was able to shrink the tumor enough to enable resection. Superior efficacy of new targeted therapies such as anaplastic lymphoma kinase-inhibitors compared to standard chemotherapy has to be proven in the future.

Giant Intracardiac Smooth-Muscle Cell Tumor Presenting as Superior Vena Cava Syndrome.

We report the case of a 53-year-old man presenting with a superior vena cava syndrome secondary to a giant intracardiac mass occupying the majority of the right-side cavities of the heart. A mass measuring 10.5 × 9.5 × 4.0 cm originating from the superior vena cava and occupying most of the right atrium extended through the tricuspid valve into the right ventricle. The mass was resected. The patient was discharged on postoperative day 11. The pathology report revealed the mass to be a smooth-muscle cell tumor of unknown malignant potential.

Inflammatory myofibroblastic tumor of kidney together with ipsilateral perinephric and periureteric fibrosis: A case report and literature review.

RATIONALE: Both inflammatory myofibroblastic tumor (IMT) and retroperitoneal fibrosis are rare lesions, but kidney involvement is more rare. It is the first study about IMT of the kidney in a patient with perinephric and periureteric fibrosis and we hold that fibroblast proliferation may be an intermediate status in oncogenesis of IMT. But further investigation is necessary in order to better clarify the relationship between fibroblast proliferation and IMT. PATIENT CONCERNS: A 54-year-old female presented no positive signs except dull back pain after overwork. DIAGNOSES: On the basis of the urinary ultrasonography and computed tomography (CT) scan, we strongly suspected a renal cell carcinoma. INTERVENTIONS: Considering the little remaining function of the right kidney and the possibility of malignancy, we performed a laparoscopic right radical nephrectomy. OUTCOMES: According to the analysis of the postoperative paraffin section and immunohistochemistry assay, a final diagnosis of IMT and retroperitoneal fibrosis nodules was made. LESSONS: Both IMTs are rare lesions and its etiology and pathogeny are unclear. It is the first study about IMT of the kidney in a patient with perinephric and periureteric fibrosis. This report suggested that fibroblast proliferation may be an intermediate status in oncogenesis of IMT, but further investigation is necessary in order to better clarify the relationship between fibroblast proliferation and IMT. The preoperative diagnosis of renal IMT remains difficult. Preoperative fine-needle aspiration or percutaneous biopsy and intraoperative frozen section were applied to confirm the diagnosis to avoid unnecessary nephrectomy, especially in patients with renal insufficiency, bilateral masses, or a solitary kidney.

An inflammatory myofibroblastic tumor in the transplanted liver displaying quick wash-in and wash-out on contrast-enhanced ultrasound: A case report.

RATIONALE: Inflammatory myofibroblastic tumor (IMT) is an uncommon mesenchymal neoplasm, and its presence in a grafted liver is exceedingly rare. PATIENT CONCERNS: A 54-year-old woman was admitted to our hospital with a half-month history of intermittent melena. She had undergone deceased-donor liver transplantation (LT) for hepatitis B virus related liver cirrhosis without hepatocellular carcinoma 5 months previously. DIAGNOSIS: Laboratory examination showed impaired liver and renal functions and Epstein-Barr virus (EBV) infection, but tumor markers within normal ranges. Gastroscopy showed esophageal varices. Ultrasound and computed tomography angiography revealed an ill-defined and irregular solitary lesion in the porta hepatis, encasing both the portal vein and the hepatic artery. The lesion was characterized by arterial hyper-enhancement and hypo-enhancement in the remaining phases with contrast-enhanced ultrasound (CEUS). The lesion was finally confirmed as an IMT by ultrasound-guided biopsy. INTERVENTION: The patient received conservative treatment, including immunosuppression, endoscopic variceal ligation, antibiotics, steroids, and antiviral agents. OUTCOME: The patient's gastrointestinal bleeding was controlled, but the symptoms associated with portal hypertension worsened. Attempts to perform a transjugular intrahepatic portosystemic shunt were unsuccessful, and she unfortunately died soon after. LESSONS: A differential diagnosis of IMT should be considered in LT recipients presenting with EBV infection, normal tumor markers, and a de novo hepatic lesion with quick wash-in and wash-out on CEUS. Ultrasound is associated with the advantages of convenience and nonionizing radiation, and should thus be the priority approach for monitoring transplanted liver.

Pulmonary inflammatory Myofibroblastic tumor indistinguishable from tuberculosis: a case report in a five-year-old child with hemoptysis.

BACKGROUND: Pulmonary inflammatory myofibroblastic tumor (PIMT) is a rare disease in China and its incidence is much lower than that of tuberculosis. PIMT accounts for only 0.04-1.2% of all lung tumors. PIMT can occurs in any age and nearly every part of the body. The clinical symptoms and radiological features of PIMT are nonspecific. Diagnosis is only made on the basis of histopathologic or immunohistochemical evaluation of the postoperation resected tissue. The therapeutic approach to PIMT should rely mainly on complete surgical resection. CASE PRESENTATION: We report a case of PIMT with hemoptysis. The girl was misdiagnosed with tuberculosis and treated with anti-tuberculous drugs for a long period of time. A right upper and middle lobectomy was performed and further assessment of the tissue demonstrated a pathologic diagnosis of PIMT. CONCLUSIONS: Despite a high incidence of tuberculosis, we must consider the possibility of PIMTs in such cases to prevent misdiagnosis and mistreatment.