Inflammatory myofibroblastoma mimicking cavernous hemangioma in the liver.

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.

Vulval superficial myofibroblastoma with lymphocytic and eosinophilic infiltrate.

We present the case of a vulval superficial myofibroblastoma with a lymphocytic and eosinophilic rim in a woman in her late 20s. The tumour presented in pregnancy as a cystic lesion with pain and increasing size. While the histopathology of superficial myofibroblastomas has been well defined in the literature, to our knowledge, there has been no documentation of the presence of an inflammatory infiltrate of lymphocytes and eosinophils surrounding and within the tumour. This may potentially act as a diagnostic or prognostic reference.

Myofibroblastoma in the Liver: A Case Report and Review of Literature.

Myofibroblastoma is a rare benign mesenchymal tumor first described in the breast. It is also known as mammary-type myofibroblastoma outside of the breast, more frequently located along the embryonic milk line. Exceptionally, myofibroblastoma can occur at visceral locations. We present a case of myofibroblastoma detected incidentally in the liver. A well-circumscribed mass, grossly measuring 6.2 cm in the liver parenchyma, was found on imaging studies. Histologically, the lesion is characterized by benign spindle cells in a hyalinized collagenous stroma, with positive staining for SMA and ER, focal positivity for CD34, negative for desmin, and loss of RB1. This rare tumor at such an unusual location makes it diagnostically challenging, especially on core biopsy of the lesion. To our knowledge, this is the second case of myofibroblastoma in the liver reported in the English literature and the first such case with a detailed pathology description.

Inflammatory Myofibroblastic Tumor of the Sigmoid Colon in an Infant: A Case Report and Literature Review.

Inflammatory myofibroblastic tumor (IMT) infrequently involves the sigmoid colon, and has not previously been described in an infant sigmoid colon.An inflammatory myofibroblastic tumor arose from the sigmoid colon of an 11-month-old boy, confirmed by anaplastic lymphoma kinase (ALK), smooth muscle actin (SMA) and desmin immunohistochemical staining. The patient recovered well after complete resection of the tumor.Sigmoid IMT can occur in infancy. This eighth case is the youngest so far. The child did well after surgical resection.

Inflammatory Myofibroblastic Tumor (IMT) of the Trachea Excised by Transtracheal Surgery: Case Report.

This report describes an extremely rare case of a primary inflammatory myofibroblastic tumor of the trachea. The patient underwent surgical resection by a transtracheal approach and reconstruction with esophageal tracheoplasty. This case report highlights the rarity of such tumors and a minimally invasive and safe surgical technique for tumors around the central neck structures.

Intraocular myofibroblastoma tumour of the ciliary body: a case report and literature review.

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.

Diagnostic Challenges of Intra-operative Frozen Consultation for Mammary Epithelioid Myofibroblastoma.

Myofibroblastoma (MFB) of the breast is a rare benign neoplasm that exhibits several morphologic variants and presents diagnostic challenges for pathologists, especially in recognizing intra-operative frozen sections. In order to raise awareness of this tumor and avoid misdiagnosis, we describe a case of a 38-year-old female patient diagnosed as epithelioid MFB. This painless tumor was well-circumscribed, found in the left breast and was physically examined over a period of six months. Histologically, this tumor was predominantly composed of epithelioid cells, which arranged as single cells, small clusters or nests. Tumor stroma was collagenized with spindle cells, adipose and focal myxoid areas. This case was misinterpreted as invasive carcinoma in the frozen section. The immunohistochemical profile demonstrated positivity for Vimentin, desmin, SMA, calponin, CD34, ER, PR and AR, whereas pan-keratin, keratin 7, keratin 34ßE12, keratin 5/6, EMA, p63 and S100 were negative. RB1 was abnormally negative, confirming the diagnosis of epithelioid MFB. Making a correct diagnosis is primarily dependent on awareness by the pathologist of this unusual variant of MFB and careful integration of clinicopathologic findings to avoid potential diagnostic pitfalls.

Inflammatory myofibroblastic tumor of the kidney in patient with nephron-sparing surgery. Case report and review of the literature.

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.

Mammary-type myofibroblastoma of the thigh mimicking liposarcoma.

Myofibroblastoma is a rare tumor that was originally described in the breast but has since been found in extra-mammary locations, where it been renamed mammary-type myofibroblastoma (MTM). We describe a case of MTM occurring in the anterior compartment of the thigh, which was initially interpreted as a possible a liposarcoma on MRI. Two subsequent biopsy samples and eventual excision revealed the tumor to have the characteristic pathology of MTM. This benign tumor, albeit rare, needs to be considered in the imaging differential diagnosis of fat containing deep soft tissue masses of the extremity.

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.

Molecular Characterization of Inflammatory Tumors Facilitates Initiation of Effective Therapy.

Inflammatory myofibroblastic tumor (IMT) is a rare, mesenchymal tumor that has an increased incidence in childhood. Tumors are usually isolated to the chest, abdomen, and retroperitoneum, but metastatic presentations can be seen. Presenting symptoms are nonspecific and include fever, weight loss, pain, shortness of breath, and cough. Approximately 85% of IMTs harbor actionable kinase fusions. The diagnosis can be delayed because of overlapping features with inflammatory disorders, such as elevated inflammatory markers, increased immunoglobin G levels, fever, weight loss, and morphologic similarity with nonmalignant conditions. We present a girl aged 11 years with a TFG-ROS1 fusion-positive tumor of the lung that was initially diagnosed as an immunoglobin G4-related inflammatory pseudotumor. She underwent complete left-sided pneumonectomy and later recurred with widely metastatic disease. We then report the case of a boy aged 9 years with widely metastatic TFG-ROS1 fusion-positive IMT with rapid molecular diagnosis. In both children, there was an excellent response to oral targeted therapy. These cases reveal that rapid molecular testing of inflammatory tumors is not only important for diagnosis but also reveals therapeutic opportunities. Targeted inhibitors produce significant radiologic responses, enabling potentially curative treatment approaches for metastatic ROS1 fusion IMT with previously limited treatment options. Primary care pediatricians and pediatric subspecialists have a crucial role in the early consultation of a pediatric oncology center experienced in molecular diagnostics to facilitate a comprehensive evaluation for children with inflammatory tumors.

Myofibroblastic tumor of the esophagus - a case report of long-term follow-up and literature review.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal neoplasm with intermediate malignant potential. Although most often seen in the lungs, it can occur at multiple anatomical locations, including the gastrointestinal tract. An esophageal lesion is extremely rare, however. IMTs present most commonly in children and young adults. The main therapeutic approach is surgical resection. CASE REPORT: We report on the follow-up of a case in a 13-year-old boy with IMT in the esophagus. He underwent surgical resection in 2013 and is free of disease to date. CONCLUSION: Surgical resection is the most preferred therapy. If the resection is complete, the risk of recurrence is low. Nevertheless, every patient should be carefully followed up after the resection.

First report of an adult female patient with endobronchial inflammatory myofibroblastic tumor in Taiwan: A case report.

An inflammatory myofibroblastic tumor (IMT) of the respiratory system is an uncommon disease. In Taiwan, there is a lack of previous studies on tracheobronchial IMT. The tumor is characterized by overexpression of anaplastic lymphoma receptor tyrosine kinase (ALK)-1. Surgical resection is the standard treatment of choice nowadays.

Rectal inflammatory myofibroblastic tumor: Case report and literature review.

RATIONALE: Rectal inflammatory myofibroblastic tumor (IMT) is an extremely rare mesenchymal tumor characterized by a mixture of spindle-shaped myofibroblasts or fibroblasts and inflammatory infiltration of lymphocytes and plasma cells. To date, only 8 cases of rectal IMT have been reported. Herein, we report an additional case of rectal IMT in a 28-year-old woman. PATIENT CONCERNS: A 28-year-old woman presented with abdominal pain and hematochezia. DIAGNOSES: Colonoscopy showed a 3.0-cm subepithelial tumor with central ulceration, covered by white exudate in the rectum. Rectal magnetic resonance imaging revealed a 4.0 × 3.0-cm-sized well-defined subepithelial tumor in the right wall of the rectum, with suspicious right perirectal fat infiltration. INTERVENTIONS: Laparoscopic anterior resection was performed. Microscopic examination of the surgical specimen revealed bland-looking spindle cells intermingled with lymphoplasma cells. Immunohistochemistry and fluorescence in situ hybridization showed anaplastic lymphoma kinase positivity and anaplastic lymphoma kinase positivity rearrangement. Rectal IMT was confirmed based on histological, immunohistochemical, and fluorescence in situ hybridization findings. The patient was doing well without evidence of tumor recurrence 1 year after the surgery. LESSONS: Rectal IMT, despite its rarity, should be considered in the differential diagnosis of rectal cancer. Second, an accurate histopathologic diagnosis and complete surgical resection can be the most important approaches to offer a chance for the cure of rectal IMT.

Inflammatory myofibroblastic tumor: molecular landscape, targeted therapeutics, and remaining challenges.

Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor of intermediate malignant potential that predominantly affects children, adolescents and young adults. IMT has a predilection for the lung, abdomen, pelvis, and retroperitoneum, however, can affect any part of the body. IMT is typically localized, and multifocal or metastatic disease is uncommon. Complete surgical resection is the treatment of choice when feasible. There is no established standard of care for unresectable and advanced IMT. Approximately half of IMTs harbor anaplastic lymphoma kinase (ALK) gene rearrangements, and fusions involving ROS1, PDGFRß, RET and NTRK have also been described. Given the molecular landscape of IMT, management of these tumors has evolved to include tyrosine kinase inhibitors and novel targeted therapeutics. This review highlights the molecular characteristics, evolution of targeted therapies and the remaining challenges in the management of IMT.