Inflammatory myofibroblastic tumor (IMT) is an intermediate tumor composed of differentiated myofibroblastic spindle cells with inflammatory cell infiltration. It can occur in all parts of the body, with the lungs being the most common, while the tissues outside the lungs, including the sigmoid colon, are rare. Herein, we present a case of a 10-year-old girl with sigmoid IMT who presented to our hospital with abdominal pain. An abdominal computed tomography (CT) revealed a well-defined, slightly low-density mass in her lower abdomen that was not clearly demarcated from the sigmoid colon. The mass showed significant uneven enhancement on contrast-enhanced CT and increased fluorine-18 fluorodeoxyglucose (18F-FDG) uptake on positron emission tomography (PET). Moreover, a systematic review of the published literature on sigmoid IMT was conducted and its clinical and radiographic features were summarized to increase the understanding of this rare disease.

The occurrence of rectosigmoid junction inflammatory myofibroblastic tumor (IMT) is uncommon in children. This is a rare form of mesenchymal tumor, belonging to the category of soft tissue tumors, and can be found at any anatomical site from the central nervous system to the gastrointestinal tract. Our patient was a 10-year-old male subject complaining of lack of defecation and constipation. The patient had decreased the frequency of defecation and constipation about two weeks before his referral and had not improved despite the use of laxatives. The abdomen was completely distended and there was no tenderness or guarding in the examination. Several airfluid levels are shown on the abdominal X-ray. In the ultrasound, free fluid was reported in the interlobular and pelvic spaces. The patient was transferred into the operating room. A tumor of the rectosigmoid junction was detected. Histopathologic studies showed evidence of IMT. IMT is a rare neoplasm of unknown origin, which may occur in various sites of the body. Complete surgical removal is usually curative, but early detection of recurrence is required. Treatment options include chemotherapy, radiation therapy, and immunotherapy. Further investigations are needed to improve the understanding and management of this rare tumor.

An aggressive subtype of inflammatory myofibroblastic tumor, epithelioid inflammatory myofibroblastic sarcoma occurs primarily inside the abdominal cavity, followed by a pulmonary localization. Most harbor anaplastic lymphoma kinase (ALK) gene rearrangements, with RANBP2 and RRBP1 among the well-documented fusion partners. We report the second case of primary epithelioid inflammatory myofibroblastic sarcoma of the brain, with a well-known EML4::ALK fusion. The case is notable for its intra-axial presentation that clinico-radiologically mimicked glioma.

Inflammatory myofibroblastic tumor (IMT) is a soft tissue neoplasm which can be locally invasive, recur, or in rare cases metastasize. Often originating from the abdomen or thorax, IMT most commonly affects children and young adults. Due to its rarity comprehensive reports detailing clinical management and outcome(s) are sparse and often based on limited index case numbers. This study systematically analyzes outcome metrics of pediatric IMT and identifies risk factors for mortality. Medline/Embase databases were searched in accordance with PRISMA guidelines. Final analysis included 57 studies with 673 IMT patients (355 males, 53 %). Individual patient data was available for 405 cases with a median follow-up period of 36 months. Tumor sites included abdomen/pelvis (n = 233, 58 %), thorax (n = 125, 31 %), head/neck (n = 34, 8 %), and extremities (n = 13, 3 %). Surgical tumor resection was the mainstay of treatment, while only 20 patients (5 %) were treated non-operatively. Recurrence(s) were reported in 80 patients (20 %) with 34 (12 %) requiring reoperation. Positive tumor margins were a significant risk factor for tumor recurrence (p < 0.0001). Chemo/radiotherapy was reported in 98 patients (25 %). Most patients (94 %) survived; 81 % (n = 237) with no evidence of recurrent disease, 14 % (n = 41) were alive with disease, and 25 (6 %) died of disease. Positive margins at primary operation, and metastatic disease were associated with mortality (p < 0.0001 for both). IMT is a rare tumor with favorable outcome for the majority of patients. Whilst most patients will present with benign tumors, complete surgical resection (R0) is crucial, as positive surgical margins are a significant risk factor for tumor recurrence and mortality.

An inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal neoplasm of borderline malignant potential. Nearly half of all IMTs have rearrangement of anaplastic lymphoma kinase (ALK) locus on chromosome 2p23 which can be treated with targeted therapy. Herein, we describe an unusual presentation of IMT involving an anatomical region rarely implicated in this disease process. A 15-year-old male patient came to the ER with dysphagia and coffee ground emesis. On esophagogastroscopy, a nodular luminal obstructing 30 × 50 mm mass in the lower esophagus was found, which was continuous with a large, partially circumferential gastric mass extending from the mid-body to the proximal antrum. Biopsies from esophageal and gastric masses revealed submucosal lesions composed of cytologically bland spindle and epithelioid cells, intermingled with inflammatory infiltrate, for which several immunohistochemical (IHC) stains were performed. The molecular study demonstrated ATIC::ALK fusion. Based on morphological, IHC, and molecular study findings, the diagnosis of ALK-positive IMT was rendered. Because surgical excision was deemed infeasible, the patient was started on ALK-inhibiting therapy with crizotinib. The patient responded well with no evidence of residual or recurrent disease on follow-up imaging or surveillance esophagogastroduodenoscopy. Crizotinib was ultimately discontinued after 10 months of therapy, and the patient continues to undergo surveillance imaging for monitoring of disease burden.

An inflammatory myofibroblastic tumor (IMT), frequently misdiagnosed as a malignant neoplasm, is a rare tumor characterized by the presence of myofibroblastic spindle cells and infiltration of inflammatory cells. In the current study, a 49-year-old female patient with a huge abdominal mass in the left abdominal wall was examined. Diagnostic procedures included blood tests, as well as ultrasound, Doppler, and computed tomography (CT) scans, which revealed the presence of a huge complex multiloculated cystic lesion measuring 30 x 37 x 20 cm. The patient underwent complete excision of the mass. Histopathological examination confirmed the benign nature of the tumor and revealed no evidence of malignancy. A comprehensive review of the available literature shows that the current case is one of the few documented cases. The report concluded by emphasizing the importance of surgical intervention as the primary therapeutic strategy and the crucial role of histopathology in the diagnostic process.

UNASSIGNED: Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm with malignant potential. Bladder IMT is even rarer and mainly treated by surgical resection However, partial or radical cystectomy would affect the quality of life of patients due to major surgical trauma, and classical TURBT is hard to avoid intraoperative complications including obturator nerve reflex and bleeding etc. Therefore, the safe and effective better choice of surgical approaches become critical to bladder IMT.
UNASSIGNED: A 42-year-old male patient was admitted to the department of urology with persistent painless gross hematuria for more than 10 days without the presentation of hypertension. Preoperative routine urine examination of red blood cells was 7738.9/HPF (normal range ≤ 3/HPF). CTU indicated a space occupying lesion (6.0 cm×5.0 cm) in the left posterior wall of the bladder with heterogeneous enhancement in the excretory phase. MRI also indicated bladder tumor with slightly equal SI on T1WI and mixed high SI on T2WI (6.0 cm×5.1cm×3.5cm) in the left posterior wall of the bladder. En bloc resection of bladder IMT with 1470 nm diode laser in combination of removing the enucleated tumor by the morcellator system was performed. Postoperative pathological examination revealed bladder IMT, with IHC positive for Ki-67 (15-20%), CK AE1/AE3, SMA, and Desmin of bladder IMT and negative for ALK of bladder IMT as well as FISH negative for ALK gene rearrangement. Second TUR with 1470 nm diode laser was performed within 6 weeks to reduce postoperative risk of recurrence due to highly malignant potential for the high expression of Ki-67 (15-20%) and negative ALK in IHC staining. The second postoperative pathology report showed chronic inflammation concomitant with edema of the bladder mucosa without bladder IMT, furthermore no tumor was observed in muscularis propria layer of bladder. No recurrence occurred during the period of 24-month follow-up.
UNASSIGNED: En bloc resection of bladder IMT in combination of the following second transurethral resection with 1470 nm diode laser is a safe and effective surgical approach for the huge bladder IMT with highly malignant potential.

Inflammatory myofibroblastic tumors (IMTs) of the heart are rarely observed in the eldly. We report a case involving an elderly woman with an IMT situated on the right atrial wall. The tumor was fully excised. The patient had a smooth recovery post-surgery and remained free of recurrence for three years.

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.

Liver tumors in children are uncommon and show remarkable morphologic heterogeneity. Pediatric tumors may arise from either the epithelial or mesenchymal component of the liver and rarely may also show both lines of differentiation. Both benign and malignant liver tumors have been reported in children. The most common pediatric liver tumors by age are benign hepatic infantile hemangiomas in neonates and infants, malignant hepatoblastoma in infants and toddlers, and malignant hepatocellular carcinoma in teenagers. Here, we provide an up-to-date review of pediatric liver tumors. We discuss the clinical presentation, imaging findings, pathology, and relevant molecular features that can help in the correct identification of these tumors, which is important in managing these children.