Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A cardiac pseudosarcomatous fibromyxoid tumor (PFT) is described in a previously healthy 35-year-old man, together with a review of the literature. Pseudosarcomatous fibromyxoid tumor is within the spectrum of inflammatory (myofibroblastic) pseudotumors. It has previously been described predominantly within the genitourinary tract and respiratory tract. Inflammatory pseudotumor is rare as a cardiac tumor, and cardiac PFT is not previously reported. No recurrence or metastatic disease has been reported after resection of PFTs elsewhere in the body, and this tumor seems to have a benign clinical course.
 ...
PMID:Cardiac pseudosarcomatous fibromyxoid tumor: a review of the literature. 1627 45

Mesenteric fibromatosis, sclerosing mesenteritis, inflammatory pseudotumor, and extrapleural solitary fibrous tumor constitute a loosely associated group of benign fibrous tumors and tumorlike lesions of the mesentery. These lesions are linked histologically by the presence of fibroblasts or fibrosis and anatomically by their location within the mesentery. Although rare, and distinctly different in pathogenesis and biologic behavior, these fibrous lesions have pathologic and radiologic features that overlap with one another and with more common neoplastic and nonneoplastic lesions of the mesentery. Mesenteric fibromatosis is a locally aggressive, benign proliferative process that may occur sporadically or in association with familial adenomatous polyposis. It most frequently manifests as a focal mesenteric mass and may simulate lymphoma, metastatic disease, or a soft-tissue sarcoma. Sclerosing mesenteritis is a rare idiopathic disorder that most commonly produces a stellate mass within the mesentery and should be differentiated from metastatic disease, specifically metastatic carcinoid, because it frequently responds to conservative or medical management. Inflammatory pseudotumor (inflammatory myofibroblastic tumor) is a benign, chronic inflammatory disorder of unknown cause that manifests as a solid mesenteric mass, indistinguishable from malignancy. Extrapleural solitary fibrous tumor is a tumor of submesothelial origin that is identical to the solitary fibrous tumor of the pleura. When located in the mesentery or peritoneal cavity, extrapleural solitary fibrous tumor has an imaging pattern that must be differentiated from metastatic disease, soft-tissue sarcomas, and other benign and malignant neoplasms of the mesentery and peritoneum. Knowledge of this group of benign fibrous tumors and tumorlike lesions of the mesentery is important in the preoperative evaluation of a mesenteric mass.
 ...
PMID:From the archives of the AFIP: benign fibrous tumors and tumorlike lesions of the mesentery: radiologic-pathologic correlation. 1641 55

Inflammatory pseudotumor or pseudosarcomatous fibromyxoid tumor and postoperative spindle cell nodule of the bladder are unusual lesions of uncertain pathogenesis which share overlapping, if not identical, histologic features. We present our experience with 42 cases, the largest series to date, to study the etio-pathogenesis, histologic features, biologic behavior and relationship to "inflammatory myofibroblastic tumor" of childhood. Patients ranged in age from 7 to 77 years (mean 47 y) and males predominated (3.2:1). Most patients presented with hematuria (31/42). Common associations were smoking (10/30) and previous instrumentation or surgery (9/42). The clinicopathologic features of patients having or not having prior instrumentation were identical. Grossly the lesions were polypoid or nodular and involved any portion of bladder wall, most commonly the dome (9/27) and measured 1 to 10 cm (mean 4 cm). They were composed of spindled and stellate cells arranged in a myxoid background with numerous inflammatory cells. Myxoid hypocellular areas were more pronounced near the mucosal surface with greater cellularity and a fascicular arrangement in the deep aspect of the lesion. "Atypical" features included mitotic activity (0 to 20/10 HPF; mean 2/10 HPF; median 1/10 HPF; none atypical), necrosis (22/42), and extension into muscularis propria (28/32) or perivesicular fat (3/8). Lesions were positive for cytokeratin (31/33), SMA (23/34), desmin (21/35), and Alk-1 protein (12/26). FISH confirmed the Alk-1 translocation in 4/6 cases. Treatment included transurethral resection (30/42), partial cystectomy (9/42), and total cystectomy (3/42). Initial diagnostic error resulted in radiotherapy and chemotherapy in 3 patients. Follow-up was available in 28 patients. (range 3 to 93 mo; median 25 mo). Three patients developed recurrences, but none had metastases. Because the clinicopathologic features of lesions associated with and without instrumentation were similar and inseparable, we believe they are essentially the same entity, and propose the term pseudosarcomatous myofibroblastic proliferation. The preponderance of evidence which includes the extravesical growth, local recurrence, and Alk-1 gene translocation in some cases suggests perhaps a neoplastic process with limited growth potential. Even in the face of atypical histologic features (muscle invasion and necrosis) the prognosis is excellent. Despite the Alk-1 gene translocation, there continues to be sufficient evidence for regarding these as distinct from the so-called inflammatory myofibroblastic tumor of childhood.
 ...
PMID:Pseudosarcomatous myofibroblastic proliferations of the bladder: a clinicopathologic study of 42 cases. 1741 15

Inflammatory myofibroblastic tumor (IMT) of the urinary tract, also termed postoperative spindle cell nodule, inflammatory pseudotumor, and pseudosarcomatous fibromyxoid tumor, is rare and in the past was believed to reflect diverse entities. We reviewed a series of 46 IMTs arising in the ureter, bladder, and prostate, derived primarily from a large consultation practice. There were 30 male and 16 females aged 3 to 89 years (mean 53.6). Lesions were 1.2 to 12 cm (mean 4.2). There was a history of recent prior instrumentation in 8 cases. Morphology was similar to that previously described for IMT occurring in this region, with the exception of 1 case that focally appeared sarcomatous. Polypoid cystitis coexisted in 5 patients (11%). Mitoses were typically scant (0 to 20/10 hpf, mean 1). Necrosis was seen in 14 (30%) cases. Invasion of the muscularis propria was documented in 19 (41%). By immunohistochemistry (IHC), lesions at least focally expressed anaplastic lymphoma kinase (ALK) (20/35, 57%), AE1/3 (25/34, 73%), CAM5.2 (10/15, 67%), CK18 (6/6, 100%), actin (23/25, 92%), desmin (15/19, 79%), calponin (6/7, 86%), caldesmon (4/7, 57%, rare cells), p53 (10/13, 77%), and most lacked S100 (0/14), CD34 (0/13), CD117 (2/13, 15%), CD21 (0/5), and CD23 (0/3). ALK gene alterations were detected by fluorescence in situ hybridization (FISH) in 13/18 (72%) tested cases, including 2 with prior instrumentation; 13/18 (72%) showed agreement between FISH ALK results and ALK protein results by IHC. Most bladder IMTs were managed locally, but partial cystectomy was performed as the initial management in 7 cases and cystectomy in 1 (1 IMT was initially misinterpreted as carcinoma, 1 IMT was found incidentally as a separate lesion in a cystectomy specimen performed for urothelial carcinoma). Follow-up was available in 32 cases (range 3 to 120 mo; mean 33; median 24). There were 10 patients with recurrences (2 with 2 recurrences). Recurrences were unassociated with muscle invasion or with ALK alterations. In 2 cases, tumors of the urinary tract (TURs) showing IMT preceded (1 and 2 mo, respectively) TURs showing sarcomatoid carcinoma with high-grade invasive urothelial carcinoma accompanied with separate fragments of IMT. Even on re-review the IMT in these 2 cases were morphologically indistinguishable from other cases of IMT, with FISH demonstrating ALK alterations in the IMT areas in one of them. Both these patients died of their carcinomas. Lastly, there was 1 tumor with many morphological features of IMT and an ALK rearrangement, yet overtly sarcomatous. This case arose postirradiation for prostate cancer 4 years before the development of the lesion, with tumor recurrence at 4 months and death from intra-abdominal metastatic disease at 9 months. In summary, urinary tract IMTs are rare and share many features with counterparts in other sites, displaying similar morphology and immunogenotypic features whether de novo or postinstrumentation. Typical IMTs can be locally aggressive, sometimes requiring radical surgical resection, but none of our typical cases metastasized, although they can rarely arise contemporaneously with sarcomatoid urothelial carcinomas. For these reasons, close follow-up is warranted.
 ...
PMID:Inflammatory myofibroblastic tumors of the urinary tract: a clinicopathologic study of 46 cases, including a malignant example inflammatory fibrosarcoma and a subset associated with high-grade urothelial carcinoma. 1712 5

Inflammatory myofibroblastic tumor (IMT) is a neoplasm of intermediate biologic potential. In this study, we report a subset of IMTs with histologic atypia and/or clinical aggressiveness that were analyzed for clinicopathologic features, outcome, and immunohistochemical expression of anaplastic lymphoma kinase (ALK) and other markers to identify potential pathologic prognostic features. Fifty-nine IMTs with classic morphology (5 cases), atypical histologic features (21 cases), local recurrence (27 cases), and/or metastasis (6 cases) were studied. Immunohistochemistry was performed for ALK1 and other markers (Mib-1, c-Myc, cyclin D1, caspase 3, Bcl-2, Mcl-1, survivin, p27, CD56, p53, MDM-2) using standard techniques. The 59 IMTs had an age at diagnosis ranging from 3 weeks to 74 years (mean 13.2 y, median 11 y, 44% in the first decade). The mean tumor size was 7.8 cm. Sites included the abdomen or pelvis in 64%, lung in 22%, head and neck in 8%, and extremities in 5%. The follow-up ranged from 3 months to 11 years, with a mean of 3.6 years and a median of 3 years. Thirty-three patients had local recurrences, including 13 with multiple local recurrences and 6 patients with both local recurrences and distant metastases. Six patients died of disease, 5 with local recurrences, and 1 with distant metastases. Histologic evolution to a more pleomorphic cellular, spindled, polygonal, or round cell morphologic pattern was observed in 7 cases. Abdominal and pelvic IMTs had a recurrence rate of 85%. Recurrent and metastatic IMTs were larger, with mean diameters of 8.7 and 11 cm, respectively. Cytoplasmic ALK reactivity was seen in 56%. ALK-negative IMTs occurred in older patients (mean age 20.1) years and had greater nuclear pleomorphism, atypia, and atypical mitoses. All 6 metastatic IMTs were ALK-negative. Nuclear expression of p53 was detected in 80% of IMTs overall, but in only 25% of the metastatic subset. There were no significant differences among the subgroups for c-Myc, cyclin D1, MDM-2, Mcl-1, Bcl-2, CD56, p27, caspase 3, or survivin expression. In conclusion, among these 59 IMTs, ALK reactivity was associated with local recurrence, but not distant metastasis, which was confined to ALK-negative lesions. Absent ALK expression was associated with a higher age overall, subtle histologic differences, and death from disease or distant metastases (in a younger subset). Other proliferative, apoptotic, and prognostic markers did not correlate well with morphology or outcome. Thus, ALK reactivity may be a favorable prognostic indicator in IMT and abdominopelvic IMTs recur more frequently.
 ...
PMID:Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. 1741 97

Inflammatory pseudotumor (IPT) is a rare tumor that occurs in various organs and tissues. The clinical picture varies from the more frequent benign lesions to the rare malignant tumors with distant metastases. IPT associated with hematopoietic stem cell transplantation (HSCT) is rarely reported. In this article, we review the reports of IPT after HSCT and describe the first case of bladder IPT. We also review the possible factors involved in the pathogenesis. IPT might be rare but it is a potentially serious complication of HSCT. It should be considered in patients with otherwise unexplained inflammatory symptoms or signs or with any mass lesion in the post-HSCT period. A knowledge of this entity and insistence on a definitive biopsy of mass lesions in the post-HSCT period can avoid unnecessary treatment such as radical surgery, chemotherapy or radiotherapy.
 ...
PMID:Inflammatory pseudotumor following hematopoietic stem cell transplantation: a new case and review of the literature. 1766 Aug 42

Inflammatory myofibroblastic tumor (IMT) of the lung is a rare tumor but it should be considered when dealing with primary lung tumors in children, adolescents, and nonsmoking adults. It is, from a pathologic point of view, a benign tumor composed of a spindle cell proliferation and inflammatory cells. Its clinical behavior, however, is variable with a benign evolution at one, and a malignant evolution with recurrent and metastatic disease at the other end of the spectrum. Diagnosis is very difficult and often only possible after resection of the tumor. We present a case of pulmonary IMT in a 15-year-old male with malignant features on radiographic and F-Fluoro-deoxyglucose positron emission tomography imaging. Pathogenesis, pathology findings, clinical behavior, and imaging of pulmonary IMT are briefly discussed.
 ...
PMID:Invasive inflammatory myofibroblastic tumor of the lung. 1955 Feb 47

We report a case of a 28-year-old man with a dry cough and chest pain. Chest X-ray film showed a huge mass in the right lung field. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a huge mass which occupied most of the right thoracic cavity and invaded the superior vena cava, heart atrium and right pulmonary vein. Positron emission tomography (PET) showed metastasis of bone, right adrenal grand and lymph nodes. A tumor specimen was biopsied percutaneously, and the diagnosis was pathologically confirmed as an inflammatory myofibroblastic tumor. Immunohistochemical staining also showed an overexpression of ALK in the tumor. He was treated with a non-steroid anti-inflammation drug and steroid, but they were ineffective. He underwent chemotherapy with bleomicin, etoposide and cisplatin. After two cycles of chemotherapy, the tumor slightly reduced in size, but was eventually refractory to the regimen finally. He also underwent with paclitaxel and carboplatin. At present, if operative extirpation is not possible, there is no way to treat an inflammatory myofibroblastic tumor. In the future, new therapy incorporating ALK inhibitors would be expected to treat those cases of IMT in which local recurrences and distant metastases occur.
 ...
PMID:[A case of rapidly growing inflammatory myofibroblastic tumor in the lung]. 2005 97

Inflammatory myofibroblastic tumor is a rare lesion that is usually located in the lung. The most commonly reported extrapulmonary locations are the mesentery and the retroperitoneum. We report the case of an 8-year-old girl with an inflammatory myofibroblastic tumor in an unusual location. The patient was referred to our center for suspected complicated appendicitis. Abdominal ultrasonography detected ileoileal intussusception with a mass in the distal portion. Emergency laparotomy confirmed ileoileal intussusception secondary to an intraluminal tumor. Histologic study revealed the mass was an inflammatory myofibroblastic tumor. Inflammatory myofibroblastic tumors are rare mesenchymal masses usually found in children. When located in the abdomen, they can mimic malignant lesions like lymphomas, sarcomas, or metastases. The cause and prognosis of inflammatory myofibroblastic tumors are unknown.
 ...
PMID:[Unusual location of an inflammatory myofibroblastic tumor: a case report]. 2054 83

Inflammatory Myofibroblastic Tumor (IMT) is a rare spindle cell neoplasm with a relatively indolent course. Its morphology may be confused with both reactive processes and/or malignant neoplasms on FNA specimens. Herein we discuss the cytologic features and IHC studies of IMT. The archives of the Department of Pathology at the Johns Hopkins Hospital were searched for IMT. A total of 257 cases were identified over a period of 11 years. Among them, 20 cases had cytology material. The patients' ages ranged from 7 to 81 years old with a median age of 54 years. The locations of the tumor in descending order were: liver (9/20, 45%), lung (8/20, 40%), abdomen (1/20, 5%), pelvis (1/20, 5%), and kidney (1/20, 5%). On FNA, the majority of tumors consisted of bland spindle cells with oval nuclei and small prominent nucleoli in a background of lymphocytes and plasma cells. Focal cytological atypia and "ganglion-like" cells were identified in 7 cases, likely related to the risk of metastases and malignant transformation. The lesional cells expressed ALK (8/17, 47.1%) and actin (10/10, 100%), but with variable expression of cytokeratin. Ki-67 showed low proliferative indices. ALK gene rearrangement was detected by FISH in three out of three cases and correlated with ALK protein expression by IHC. The cytologic diagnosis of IMT is challenging. When encountering a spindle cell lesion with prominent inflammatory component, a high index of suspicion in combination with the use of ancillary studies increases the diagnostic yield of IMT.
 ...
PMID:Cytology of fine-needle aspiration of inflammatory myofibroblastic tumor. 2073 Aug 98


<< Previous 1 2 3 4 5 Next >>