Pulmonary Calcifying Fibrous Tumor in a Pediatric Patient: A Case Report.

A calcifying fibrous tumor (CFT), also known as calcifying fibrous pseudotumor, is an uncommon non-cancerous neoplasm usually located in the gastrointestinal tract. Its location in the lung is extremely rare, and only a few case reports have been published. This case report describes our diagnostic approach in a 9-year-old male patient with an incidental pulmonary mass. The mass was initially misdiagnosed, requiring multiple imaging tests and interventions to obtain the definitive diagnosis of pulmonary CFT. This paper aims to contribute to the limited information available on pulmonary CFT by presenting detailed findings from computed tomography and magnetic resonance imaging.

Inflammatory myofibroblastic tumor of the adrenal gland: A case report.

Inflammatory Myofibroblastic Tumor (IMT) occurring in the adrenal gland is extremely rare, and pathologic examination is the gold standard for confirming the diagnosis. We report a case of IMT of adrenal origin in a patient whose diagnosis was confirmed by pathological examination after surgical resection of the tumor. Although previous studies have reported an overall favorable prognosis for IMT, regular and long-term follow-up is necessary.

Inflammatory myofibroblastic tumor from molecular diagnostics to current treatment.

Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm with intermediate malignancy characterized by a propensity for recurrence but a low metastatic rate. Diagnostic challenges arise from the diverse pathological presentation, variable symptomatology, and lack of different imaging features. However, IMT is identified by the fusion of the anaplastic lymphoma kinase (ALK) gene, which is present in approximately 70% of cases, with various fusion partners, including ran-binding protein 2 (RANBP2), which allows confirmation of the diagnosis. While surgery is the preferred approach for localized tumors, the optimal long-term treatment for advanced or metastatic disease is difficult to define. Targeted therapies are crucial for achieving sustained response to treatment within the context of genetic alteration in IMT. Crizotinib, an ALK tyrosine kinase inhibitor (TKI), was officially approved by the US Food and Drug Administration (FDA) in 2020 to treat IMT with ALK rearrangement. However, most patients face resistance and disease progression, requiring consideration of sequential treatments. Combining radiotherapy with targeted therapy appears to be beneficial in this indication. Early promising results have also been achieved with immunotherapy, indicating potential for combined therapy approaches. However, defined recommendations are still lacking. This review analyzes the available research on IMT, including genetic disorders and their impact on the course of the disease, data on the latest targeted therapy regimens and the possibility of developing immunotherapy in this indication, as well as summarizing general knowledge about prognostic and predictive factors, also in terms of resistance to systemic therapy.

Inflammatory myofibroblastic tumor in a patient with X-Linked hypophosphatemia: A case of Occam's razor or Hickam's dictum?

We present the case of a patient with X-Linked Hypophosphatemia (XLH) and an inflammatory myofibroblastic tumor (IMT) of the bladder which prompted further investigation into the possible relationship between XLH and IMT i.e. a case of Occam's Razor or Hickam's Dictum?

Imaging findings of primary lung tumors in children.

PURPOSE: Pediatric lung tumors are primarily discussed in the surgical literature. However, limited research has been reported on their imaging findings, and only a few tumor types have been documented. Therefore, the aim of this article is to describe the imaging features of primary lung tumors in children. METHODS: The archives of the pediatric radiology unit were reviewed for primary lung tumors documented between 2007 and 2023. In total, 24 patients (9 girls and 15 boys; aged 5 months to 16 years) were included in the study. Their demographic characteristics, clinical presentation, and histopathologic results were obtained. All imaging studies were reviewed by two radiologists for various findings (e.g., lymphadenopathy, atelectasis, pleural effusion, calcification, multiplicity, pneumothorax, axial and lobar location, laterality, tumor margin, mediastinal shift, contrast enhancement pattern, signal intensity on T1- and T2-weighted images, and diffusion pattern), and a final decision was made by consensus. The mean tumor size was compared between the benign and malignant groups using a t-test. RESULTS: There were 15 (62.5%) benign tumors, as follows: inflammatory myofibroblastic tumor (IMT; n = 10, 41%), hemangioma (n = 2, 8%), pneumocytoma (n = 2, 8%), and mature cystic teratoma (n = 1, 4%). Moreover, there were 9 (37.5%) malignant tumors, as follows: pleuropulmonary blastoma (PPB; n = 6, 25%), adenocarcinoma (n = 2, 8%), and lymphoepithelioma-like carcinoma (LELC) (n = 1, 4%). The most frequently reported symptoms were cough, fever, dyspnea, chest pain, and recurrent infection; six patients reported no clinical symptoms. Fifteen tumors (62%) were located in the right lung. The mean tumor diameter at the time of diagnosis was 6.4 ± 3 cm (benign group: 6.7 ± 3.4 cm; malignant group: 6 ± 2.3 cm, P > 0.050). Calcification was present in 80% of the patients with IMT. At the time of diagnosis, two (8.3%) patients were found to have metastasis: one was diagnosed with adenocarcinoma and the other with LELC. Tumors were located peripherally in 18 (75%) patients. CONCLUSION: The symptoms associated with lung masses are non-specific. There is no correlation between tumor size and malignancy. The most common tumors observed in this study were IMT and PPB, respectively. IMT is highly associated with calcification. CLINICAL SIGNIFICANCE: Primary lung tumors are rarely seen in children, and they have different histopathological types. Calcification might be an important radiological clue for the diagnosis of IMT, which is the most common lung tumor in children.

Inflammatory myofibroblastic tumor in the liver: a case report.

Background: Hepatic inflammatory myofibroblastic tumor (IMT) is an infrequent tumor with potential malignancy. However, it lacks specific clinical symptoms and usual imaging features. Case presentation: A 34-year-old woman had a six-month history of fever and on-and-off pain in the upper right part of her abdomen that lasted for two weeks. Imaging tests revealed a liver mass initially thought to be liver malignancy, but subsequent histopathological examination after liver removal confirmed the diagnosis as hepatocellular inflammatory myofibroblastic tumor (HIMT). Conclusion: Hepatic inflammatory myofibroblastic tumor (IMT) is an uncommon growth with vague clinical symptoms and lab results. Surgical removal remains the primary treatment method, resulting in favorable prognostic outcomes.

Pediatric Inflammatory Myofibroblastic Tumor of Rectosigmoid Junction: A Case Report and Review of the Literature.

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.

Inflammatory Myofibroblastic Tumor of the Sciatic Nerve Mimicking Lumbar Disc Herniation: A Diagnostic Challenge.

Inflammatory myofibroblastic tumors (IMTs), which involve the proliferation of fibroblastic-myofibroblastic cells mixed with inflammatory infiltrates, are exceedingly rare in the extremities. There are no reported IMTs involving the sciatic nerve. This type of involvement may cause entrapment of the sciatic nerve, whose symptoms may mimic lumbar disc herniation (LDH), especially when it occurs in patients with lumbar degenerative disc disease. We describe the case of a 40-year-old male with lumbar degenerative disc disease accompanied by IMT involving the sciatic nerve whose symptoms mimicked LDH and posed a diagnostic challenge. We showed the course of the disease as well as the systematic imaging manifestations of IMTs involving the sciatic nerve and discussed their therapeutic management.

Endobronchial Inflammatory Myofibroblastic Tumour Masquerading as a Ruptured Hydatid Cyst.

Inflammatory myofibroblastic tumours (IMTs) represent a rare group of neoplastic lesions characterized by a diverse clinical presentation. Endobronchial involvement is infrequently reported, and its manifestation mimicking the symptoms of a ruptured hydatid cyst adds an additional layer of complexity to the diagnostic challenge. This case report delves into an exceptional clinical scenario where an endobronchial IMT masqueraded as a ruptured hydatid cyst, initially confounding the diagnostic team. Through a detailed examination of the patient's clinical history, radiological imaging, bronchoscopy findings and subsequent histopathological analysis, we aim to contribute to the existing medical literature and shed light on the nuances encountered in accurately identifying and differentiating these two entities.

Inflammatory Myofibroblastic Tumor After Receiving Treatment for Non-small Cell Carcinoma.

Inflammatory pseudotumor encompasses a spectrum of both neoplastic and non-neoplastic conditions characterized by a histological pattern featuring a proliferation of cytologically bland spindle cells, accompanied by a prominent chronic inflammatory infiltrate. Within this spectrum, inflammatory myofibroblastic tumor (IMT) has emerged as a distinct entity over the past two decades, marked by unique clinical, pathological, and molecular characteristics. Typically affecting the visceral soft tissues of children and adolescents, IMT exhibits a propensity for local recurrence while posing a minimal risk of distant metastasis. They are extremely rare in adults, constituting less than 1% of adult lung tumors. Our patient, a 63-year-old female, has an intricate medical background, encompassing chronic obstructive pulmonary disease (COPD), a previous history of smoking (35 pack-years, quit a year before admission), coronary artery disease, non-obstructive hypertrophic cardiomyopathy, and obstructive sleep apnea. Presenting with a diagnostic dilemma, she recently received treatment for non-small cell carcinoma with radiation therapy, which has evolved into a swiftly advancing case of IMT.

A Case Report of Inflammatory Myofibroblastic Tumor: A Rare Benign Lung Tumor.

Inflammatory myofibroblastic tumors (IMTs) of the lung are a rare type of mesenchymal tumors that tend to occur more in the lungs of children. They are extremely rare in adults. IMTs require extensive pulmonary resection because they are commonly locally invasive. The key to preventing recurrence is complete resection, and the prognosis is excellent after surgery. We report a case of a patient with an inflammatory pseudotumor of the lung. The patient is a 27-year-old female who presented with a dry cough. A chest radiograph and computed tomography showed a lesion in the left main bronchus and near-total left lung collapse. As surgery was necessary to establish the diagnosis, left pneumonectomy was performed followed by a histological examination of the surgical specimen which confirmed inflammatory pseudotumor.

Inflammatory myofibroblastic tumor of the liver after adrenal neuroblastoma surgery: a case report.

A boy aged 55 months was diagnosed with stage IV Neuroblastoma (NB) of the right adrenal gland 2 years ago. Preoperative chemotherapy was given and he was then treated with retroperitoneal tumor resection and lymph node dissection. After surgery, the children were transferred to the Hemato-Oncology Department for chemotherapy according to the high-risk group NB, with outpatient follow-up every 6 months. In the second postoperative year, abdominal computed tomography (CT) scan revealed a rounded hypodense area in the upper part of the right posterior lobe of the liver, with marked inhomogeneous enhancement in the venous phase after enhancement, which was surgically resected, and postoperative pathology confirmed inflammatory myofibroblastic tumor (IMT) of liver. The patient was not given any special treatment after surgery. In this study, whole transcriptome sequencing was performed on the postoperative specimen of adrenal NB and the specimen of IMT of liver. This unusual case emphasizes the need for close monitoring of second tumor development in NB survivors even in the absence of known predisposing factors.

Inflammatory myofibroblastic tumor of the cecum presenting as acute abdomen, a rare pathology with a rarer presentation.

Inflammatory myofibroblastic tumor is an extremely rare neoplastic lesion with a predilection for aggressive local and recurrent behavior. The tumor tends to occur in the lungs of children and young adults, and although it can develop in older patients and other organs, this is extremely rare. Symptoms are nonspecific and depend on the location and size of the tumor. The gastrointestinal tract is rarely this mass's primary site of origin, and the cecum is an even rarer location. We present the case of an otherwise healthy 55-year-old female who presented with an acute abdomen and a mass in her abdomen; after successful surgery, she fully recovered. Inflammatory myofibroblastic tumor causing acute abdomen was the final diagnosis.

Primary epithelioid inflammatory myofibroblastic sarcoma of the brain with EML4::ALK fusion mimicking intra-axial glioma: a case report and brief literature review.

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.

Treatment and outcomes in pediatric inflammatory myofibroblastic tumors - A systematic review of published studies.

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.

A Rare Inflammatory Myofibroblastic Tumor of the Spleen: A Case Report.

BACKGROUND/AIM: Inflammatory myofibroblastic tumors (IMTs) are rare, solid, potentially malignant lesions of uncertain etiology. Histologically, IMTs exhibit a combination of lymphocytes and inflammatory cells within a fibroblastic myxoid layer. The diagnosis of IMTs poses a challenge for various medical specialties, including surgeons, pathologists, and oncologists, due to their non-specific clinical presentation. Furthermore, radiologists face difficulties in interpreting computed tomography (CT) or magnetic resonance imaging (MRI) results, which often yield polymorphic and inconclusive findings. Ultimately, histopathologists play a crucial role in reaching a definitive diagnosis based on the tumor's histological characteristics. They are detected in every system of the human body, most commonly in the lungs. Here, we report an uncommon occurrence of IMT in the spleen of a patient with nonspecific abdominal pain. CASE REPORT: A 56-year-old Caucasian female presented to Konstantopouleio General Hospital of Nea Ionia, Athens, Greece, with abdominal pain and discomfort. The patient had no significant medical history and normal laboratory tests. An abdominal CT revealed a large mass in the spleen. A splenectomy was performed. Histopathological analysis of the tumor revealed IMTS. CONCLUSION: Splenic IMT is a rare benign tumor with moderate malignant potential. It lacks a distinct clinical presentation and is typically identified either incidentally or during the examination of abdominal pain.

Inflammatory Myofibroblastic Tumor of the Esophagus and Stomach Successfully Treated With ALK Inhibitor in a Pediatric Patient: A Case Report and Concise Review of Literature.

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.

Reclassification of a spindle cell sarcoma after identification of a TFG-ROS1 fusion: A case demonstrating the clinical benefit of next-generation sequencing in sarcoma.

BACKGROUND: Inflammatory myofibroblastic tumors (IMTs) are rare mesenchymal soft tissue sarcomas that often present diagnostic challenges due to their wide and varied morphology. A subset of IMTs have fusions involving ALK or ROS1. The role of next-generation sequencing (NGS) for classification of unselected sarcomas remains controversial. METHODS AND RESULTS: We report a case of a metastatic sarcoma in a 34-year-old female originally diagnosed as an unclassified spindle cell sarcoma with myofibroblastic differentiation and later reclassified as IMT after NGS revealed a TFG-ROS1 rearrangement. Histologically, the neoplasm had spindle cell morphology with a lobulated to focally infiltrative growth pattern with scant inflammatory cell infiltrate. Immunohistochemistry demonstrated focal desmin and variable smooth muscle actin staining but was negative for SOX10, S100, and CD34. Fluorescence in situ hybridization was negative for USP6 or ALK gene rearrangements. NGS revealed a TFG-ROS1 rearrangement and the patient was treated with crizotinib with clinical benefit. CONCLUSIONS: We discuss the role of NGS as well as its potential benefit in patients with unresectable, ALK-negative metastatic disease. Considering this case and previous literature, we support the use of NGS for patients requiring systemic treatment.

Advances in uterine inflammatory myofibroblastic tumours: Diagnostic challenges and risk stratification.

The current understanding of inflammatory myofibroblastic tumours (IMTs) of the gynaecological tract has recently been enhanced by their increased recognition. This increase is largely due to greater accessibility to RNA-based molecular assays used to identify their defining ALK rearrangements. This review summarises the clinical characteristics, morphological spectrum, immunohistochemical profile and molecular underpinnings of uterine IMT. Additionally, this review discusses practical diagnostic considerations including overlap between uterine IMT and smooth muscle tumours as well as pregnancy-associated uterine IMT. Finally, we highlight recent literature demonstrating the potential for aggressive behaviour in uterine IMT, including a novel risk stratification model for identifying high-risk IMT.