Massive pericardial effusion caused by "inflammatory myofibroblastic tumour" in a 3-month-old child.

Tumours originating from cardiac tissues are rarely encountered during childhood, and fortunately most of these tumours are benign in nature. Inflammatory myofibroblastic tumour, which has unique clinical, pathological, and molecular characteristics, is a relatively new entity compared with previously mentioned tumoural processes originating from the heart. Most of the cardiac intima-media thickness patients are in the age group of 4 months to 17 years. This rarely seen tumoural process has not been subject of any specific research and the prognosis is not well known. Here we present the case of a 3-month-old child who was admitted to our outpatient clinic with massive pericardial effusion and who has shown excellent progress after surgical resection of over 1 year.

Inflammatory myofibroblastic tumour causing intestinal obstruction in a patient with neurofibromatosis type 1.

Inflammatory myofibroblastic tumours (IMTs) are rare tumours with unpredictable biological behaviour ranging from benign to locally invasive and rarely, distant metastasis. While neurofibromatosis type 1 (NF1) may manifest with gastrointestinal soft tissue tumours, this is the first report in the literature that describes an IMT occurring in a NF1 patient who presented with intestinal obstruction. Our patient presented with intestinal obstruction secondary to an obstructing terminal ileum mesenteric tumour. En bloc bowel resection was performed, with histology revealing an IMT and an adjacent neurofibroma. The resection margins were clear and the patient was free of recurrence at six months.

ALK-negative lung inflammatory myofibroblastic tumor in a young adult: A case report and literature review of molecular alterations.

RATIONALE: Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor that is prevalent among children and adolescents. Surgery is the most important therapeutic approach for IMT and complete resection is recommended. Although 50% of IMTs show anaplastic lymphoma kinase (ALK) rearrangements, crizotinib has proven an effective therapeutic approach. However, the genetic landscape of this tumor is still not fully understood and treatment options are limited, especially in the majority of ALK-negative tumors. PATIENT CONCERNS: We describe the clinical case of a healthy 18-year-old female in whom a pulmonary nodule was incidentally detected. DIAGNOSES: Following a small increase in the size of the nodule, the patient underwent both 18FDG-PET/CT and 68Ga-PET/CT, resulting in a suspicion of bronchial hamartoma. INTERVENTIONS: The patient underwent surgery and a salivary gland-like lung tumor was diagnosed. OUTCOMES: After surgery, the patient was referred to our cancer center, where a review of the histology slides gave a final diagnosis of ALK-negative lung IMT. Given the histology, it was decided not to administer adjuvant therapy and the patient was placed in a 3-monthly follow-up program. The patient is still disease-free 2 years post-surgery. LESSONS: Although there is no standard of care for the treatment of IMT, identifying genomic alterations could help to redefine the management of patients with negative-ALK disease. Our review of the literature on IMT and other kinase fusions revealed, in addition to ALK rearrangements, the potential association of ROS1, NTRK, RET, or PDGFR beta alterations with the tumor.

Perianal mammary-type myofibroblastoma.

We report a unique case of an extramammary mammary-type myofibroblastoma of the perianal region. The patient was a 40-year old female with no significant past history, who presented with a right side painless perianal mass. Gross examination of the excised mass showed a well-circumscribed, apparently encapsulated, nodular mass weighing 30 g and measuring 5 cm. in the greatest dimension. The cut surface showed solid, yellow homogenous tissue. Microscopically, the neoplasm was composed of bland spindled cells generally arranged into short fascicles with abundant myxoid stroma. The cells stained strongly for desmin and CD34. To our knowledge, a review of the literature discloses only ten cases of extramammary, mammary-type myofibroblastoma.

[Role of myofibroblast in inflammatory bowel disease and tumor genesis]. / A myofibroblastok szerepe a vastagbél gyulladásos és daganatos folyamataiban.

Stroma cells with the microenvironment around them have primary role in the regulation of inflammation processes, conformation of tumors and development of metastasis. Myofibroblasts have essential role in inflammation processes and in the regeneration of encompassed tissue. Molecules produced by them operate the cells of the immune system and effect the proliferation of epithelium. Tumors can activate myofibroblasts which can lead to uncontrolled epithelium proliferation across production of changed and increased regulation ligands (such as cytokines, chemokines, chemotactic and other growth factors) and activation of stem cell. This process could lead piling of uncontrolled epithelial cells and can impact later on conformation of tumors. In this study we present an overview about of myofibroblasts and their roles in inflammation and neoplastic processes.

Time lag between the increase of IL-6 with fever and NF-kappaB activation in the peripheral blood in inflammatory myofibroblastic tumor.

We describe a case of inflammatory myofibroblastic tumor (IMT) that occurred in the retroperitoneum. The patient manifested systemic symptoms, such as intermittent fever, anemia, thrombocytosis, and hypergammaglobulinemia. In order to elucidate the mechanism of intermittent fever in IMT, we analyzed nuclear factor-kappa B (NF-kappaB) activation in peripheral blood mononuclear cells (PBMCs) using flow cytometry, and serum cytokine levels. NF-kappaB activation was observed in the peripheral blood T cells and monocytes/macrophages. Among the measured cytokines, only interleukin (IL)-6 levels were elevated. IL-6 levels during pyrexia in the afternoon were higher than those during apyrexia in the morning. In contrast to IL-6, NF-kappaB activation in PBMCs was lower during pyrexia than during apyrexia; this is considered to be because the activation is subject to negative feedback. The time lag between the increase of IL-6 in the serum and NF-kappaB activation in the PBMCs at the onset of intermittent fever in IMT may provide further insight into the role of cytokines and NF-kappaB activation in febrile inflammatory diseases.

Locally aggressive granular cell tumor causing priapism of the crus of the clitoris. A light and ultrastructural study, with observations concerning the pathogenesis of fibrosis of the corpus cavernosum in priapism.

A case of focal priapism of the clitoris caused by a microscopic granular cell tumor (GCT) is described. This neoplasm is considered locally aggressive because it invades the lumens of peripheral cavernous sinuses of the crus of the clitoris. Caverns adjacent to those invaded by tumor exhibit stasis, telangiectasia, and necrosis of the smooth muscle of the trabecular wall. These alterations lead to telescoping collapse and compression of the cavernous spaces and culminate in fibrosis. Ultrastructurally, replicated basal lamina is found surrounding clusters of granular cells. We suspect that the multilayered lamina, in addition to being produced by tumor cells, is derived from the trabecular endothelium surrounding the caverns invaded by the GCT. The replication of the basal lamina may be provoked by cycles of injury and repair to these vessels caused by repeated episodes of prolonged vascular stasis. A peculiar large vein with perforating branches was observed in the center of the cavernous spaces of the crus. This vein is not found in normal crura and, therefore, represents a morphologic adaptation created to drain the cavernous spaces.

Malignant granular cell tumor. Report of a case with special reference to carcinoembryonic antigen.

A case of malignant granular cell tumor and its histochemical and electron-microscopic characteristics are reported. This case showed, in addition to the well-known distribution of this type of tumor in subcutaneous fat, mediastinum, retroperitoneum and lungs, multiple foci in the myocardium. Contrary to recent studies reporting the presence of carcinoembryonic antigen (CEA) in benign and malignant granular cell tumors, this case is CEA-negative. We suggest that the reported CEA-reactivity in this type of tumor is probably due to cross-reacting antibodies against antigens, presumably associated with lysosomes.

Granular cell myoblastoma: positive staining for carcinoembryonic antigen.

An immunoperoxidase technique for the detection of carcinoembryonic antigen was applied to 10 cases of granular cell myoblastoma. Consistent, strong, intracytoplasmic granular staining, which can be easily interpreted, was obtained in all cases. Schwannomas, neurofibromas, dermatofibromas, and leiomyomas were negative. The test is helpful in confirming doubtful cases. The results tend to support the suggestion that granular cell myoblastoma is derived from perineural rather than endoneural cells.

Granular cell tumors of the breast. Steroid receptor analysis and localization of carcinoembryonic antigen, myoglobin, and S100 protein.

Granular cell tumors occur in a variety of sites, including the breast (6%). Origins from histiocytic, myogenic, fibroblastic, and neurogenic elements have been proposed. Female predominance suggests that estrogenic hormones are involved. Four granular cell tumors of the breast and one in an axillary lymph node were studied for sex steroid receptor content, myoglobin, carcinoembryonic antigen, and S100 protein localization. Antimyoglobin antibody did not localize in these tumors. Carcinoembryonic antigen and S100 protein localized in the cytoplasm of these tumors. Neither estrogen nor progesterone receptor protein were present in these tumors in detectable amounts. Ultrastructural features of these granular cell tumors in the breast are similar to those described for extramammary granular cell tumors. These studies agree with previous data that suggest a neurogenic origin for granular cell tumors.

[Granular-cell tumor (Abrikosov). Immunohistological study of 4 cases with review of the literature]. / Tumeur à cellules granuleuses (Abrikossoff). Etude immunohistologique de 4 cas avec revue de la littérature.

Granular-cell tumour (GCT) is a benign neoplastic proliferation first described by Abrikossoff (1), who considered it to be of muscular origin. Since then, however, the histogenesis of GCT has been a matter of serious controversy, and various cell types have been considered as being the origin of GCT. In this work we investigated the immunohistochemical profile of four cases of GCT using antibodies to several neural differentiation markers: S-100 protein, neurofilaments (NF), glial fibrillary acidic protein (GFAP). The results were compared to those obtained on three cases of schwannomas (S) and to those already reported in literature. Four cases of GCT were retrieved from the files of the Laboratory of Histopathology of the Clinic of Dermatology, Hôp. E.-Herriot. The tumours had been observed during a two-year-period (1983-1984), fixed in Bouin's fixative and embedded in paraffin. In parallel, three cases of S that were treated in the same way were also studied. This was performed on 3 mu-thick paraffin sections using the avidin-biotin-peroxidase complex method (kit Vectastain, Vector Lab., Burlingame, USA). The following antibodies were used: a) antiserum to protein S-100 (Dakopatts, Denmark) (working dilution 1: 50); b) monoclonal antibody to 200 Kd NF (Labsystem, Helsinki) (working dilution 1: 40): C) monoclonal antibody to GFAP (Biosoft, Paris) (working dilution 1: 10).(ABSTRACT TRUNCATED AT 250 WORDS)

Inflammatory myofibroblastic tumor of the bladder.

We illustrate a case of an inflammatory myofibroblastic tumor (IMT) involving the bladder in a woman with dysuria and review the literature and differential diagnosis. Inflammatory myofibroblastic tumor, also referred to as pseudosarcomatous myofibroblastic proliferation, is a rare lesion that can arise in the genitourinary system and is characterized by a fascicular arrangement of myofibroblasts with admixed inflammatory cells and slitlike vessels. Urinary bladder IMT can be a diagnostic pitfall because its histologic features (brisk mitoses, invasion into muscularis propria, and prominent nucleoli) can mimic malignancy. The differential diagnosis of urinary bladder IMT includes sarcomatoid carcinoma and leiomyosarcoma. Diagnostic features such as bland nuclear chromatin, ganglion-like cells, pale eosinophilic cytoplasm with long processes, overexpression of anaplastic lymphoma kinase (immunohistochemistry or gene rearrangement studies), and the absence of atypical mitoses help distinguish IMT from its malignant mimics. Current controversies regarding postoperative spindle cell nodule and IMT are discussed.

Inflammatory myofibroblastic tumor of the maxillary sinus related with pulp necrosis of maxillary teeth: case report.

Inflammatory myofibroblastic tumor (IMT) is a benign lesion composed of myofibroblasts accompanied by varying numbers of inflammatory cells. Various pathogenetic factors have been proposed, but the etiology of most IMTs remains unknown. This article presents a case of IMT occurring in the left maxillary sinus. A 24-year-old man complained of throbbing pain in the maxillary left molars and swelling of the left cheek. His maxillary left second molar was diagnosed as pulp necrosis and root canal treatment performed. After that, his symptoms continued and he was referred to the Department of Otolaryngology. Computerized tomography disclosed compact soft tissue masses in the left maxillary sinus with obstruction of maxillary ostium. Under general anesthesia, the lesions were fully excised. Histopathologically, the lesions were composed of plump or spindled myofibroblasts. Cells were immunoreactive for smooth muscle actin and ß-catenin, and were negative for ALK1, CD34, and EMA. The diagnosis was IMT of left maxillary sinus. Although it is very rare, IMT should be included as a differential diagnosis in patients with compact masses in maxillary sinus.

Inflammatory myofibroblastic tumor presenting as an abdominal wall mass in an adult patient.

Inflammatory myofibroblastic tumor of the abdominal wall is a rare soft-tissue tumor presentation in adults. A 50-year-old woman was referred with abdominal pain and a palpable mass in the left lower quadrant. Computed tomography scan and magnetic resonance investigation revealed an 8-cm heterogeneous abdominal wall mass. Tumor markers were within normal limits. Fine-needle aspiration cytology and tru-cut biopsies yielded necrotic material. A preoperative diagnosis of a resectable rhabdomyosarcoma was suggested. On exploration a tumor measuring 8 x 8 x 6 cm was resected along with the involved structures. Histopathologic examination of specimen revealed an inflammatory myofibroblastic tumor of the abdominal wall. The patient has been followed up for the last 12 months without clinical or radiographic evidence of recurrence. Inflammatory myofibroblastic tumor arising from the anterior abdominal wall in adults is an unusual manifestation of soft-tissue tumors, which can be managed by a multidisciplinary team of surgeons, oncologists, radiologists and pathologists.