Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Inflammatory pseudotumor is a reactive benign lesion which can be very difficult to distinguish from some malignant bladder tumors like sarcomas or sarcomatoid carcinoma. Inflammatory pseudotumour (IPT) of the urinary bladder is a benign proliferate lesion which can simulate clinically and histologically a sarcoma. A case of an inflammatory pseudotumor of the urinary bladder in a 18-years-old man is presented. This patient presented with sudden onset of gross painless hematurial related to large polypoid and ulcerated bladder masses found on endoscopy. Initial pathological analysis was interpreted as rhabdomyosarcoma but subsequent reviews were consistent with a benign process resembling nodular fasciitis. This rare, benign and presumed non-neoplastic, reactive lesion must be differentiated from sarcomas of the urinary bladder. Immunohistochemistry seems to be the method of election in differentiating inflammatory pseudo-tumor from other spindle cell proliferations of the bladder. When diagnosis is certain, complete transurethral resection is the treatment of choice. However, if there is no total pathologic confirmation, if it is a very wide lesion or if it is recurring after endoscopic resection, a partial cystectomy is suggested.
 ...
PMID:[Inflammatory pseudotumor of the bladder: a new case report]. 1287 98

Inflammatory myofibroblastic tumor of the urinary bladder is an unusual spindle cell neoplasm that displays cytologic atypia, infiltrative growth and mitotic activity mimicking malignant tumors, such as leiomyosarcoma, rhabdomyosarcoma and sarcomatoid carcinoma. The objective of this study was to determine if anaplastic lymphoma kinase (ALK-1) protein expression detected by immunohistochemistry and ALK rearrangements detected by fluorescence in situ hybridization (FISH) were useful in distinguishing inflammatory myofibroblastic tumor from malignant spindle cell tumors of the urinary bladder. In inflammatory myofibroblastic tumor, ALK-1 expression was identified in 13 of 21 cases (62%) and ALK rearrangements in 14 of 21 cases (67%). All cases of inflammatory myofibroblastic tumor demonstrating ALK-1 expression, carried ALK rearrangements. One case negative for ALK-1 expression exhibited ALK rearrangement. ALK rearrangements were more common in women (P=0.0032). Leiomyosarcoma, sarcomatoid carcinoma, embryonal rhabdomyosarcoma and reactive myofibroblastic proliferations were negative for ALK-1 protein and ALK rearrangements. Immunohistochemistry using markers of muscle, epithelial, neural, and follicular dendritic cell differentiation showed overlap between inflammatory myofibroblastic tumor with and without ALK gene rearrangements, and between inflammatory myofibroblastic tumor and spindle cell malignancies. However, coexpression of cytokeratin and muscle-specific antigens was unique to inflammatory myofibroblastic tumor, observed in approximately half the tumors. This study indicates that detection of ALK protein and ALK gene rearrangements are useful in distinguishing inflammatory myofibroblastic tumor from spindle cell malignancies in the urinary bladder. Additionally, our findings suggest that ALK rearrangement is the primary mechanism for ALK activation and that inflammatory myofibroblastic tumor likely represents a heterogeneous group of spindle cell proliferations with the majority associated with ALK translocations, and the remaining associated with other etiologies.
 ...
PMID:Utility of ALK-1 protein expression and ALK rearrangements in distinguishing inflammatory myofibroblastic tumor from malignant spindle cell lesions of the urinary bladder. 1739 40

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.
 ...
PMID:Inflammatory myofibroblastic tumor presenting as an abdominal wall mass in an adult patient. 2062 74

Inflammatory myofibroblastic tumor (IMFT) of the urinary bladder is an unusual spindle cell lesion that exhibits cytologic atypia, infiltrative growth, and mitotic activity mimicking malignant tumors, such as leiomyosarcoma, rhabdomyosarcoma, and sarcomatoid carcinoma. Recently, anaplastic lymphoma kinase (ALK) gene translocations or ALK protein expression in IMFT has been reported, especially in patients of children and young adults. This lesion has been described in numerous locations in addition to the urinary bladder. The detection of ALK protein and ALK gene rearrangements are useful in distinguishing IMFT from spindle cell malignancies in the urinary bladder.
 ...
PMID:Inflammatory myofibroblastic tumor of the urinary bladder diagnosed by anaplastic lymphoma kinase immunostaining. 2262 12

Intraperitoneal solid tumors are far less common in children than in adults, and the histologic spectrum of neoplasms of the peritoneum and its specialized folds in young patients differs from that in older patients. Localized masses may be caused by inflammatory myofibroblastic tumor, Castleman disease, mesenteric fibromatosis, or other mesenchymal masses. Inflammatory myofibroblastic tumor is a mesenchymal tumor of borderline biologic potential that appears as a solitary circumscribed mass, possibly with central calcification. Castleman disease is an idiopathic lymphoproliferative disorder that appears as a circumscribed, intensely enhancing mass in the mesentery. Mesenteric fibromatosis, or intra-abdominal desmoid tumor, is a benign tumor of mesenchymal origin associated with familial adenomatous polyposis. Mesenteric fibromatosis appears as a mildly enhancing, circumscribed solitary mass without metastases. Diffuse peritoneal disease may be due to desmoplastic small round cell tumor (DSRCT), non-Hodgkin lymphoma, or rhabdomyosarcoma. DSRCT is a rare member of the small round blue cell tumor family that causes diffuse peritoneal masses without a visible primary tumor. A dominant mass is typically found in the retrovesical space. Burkitt lymphoma is a pediatric tumor that manifests with extensive disease because of its short doubling time. The bowel and adjacent mesentery are commonly involved. Rhabdomyosarcoma may arise as a primary tumor of the omentum or may spread from a primary tumor in the bladder, prostate, or scrotum. Knowledge of this spectrum of disease allows the radiologist to provide an appropriate differential diagnosis and suggest proper patient management.
...
PMID:Solid tumors of the peritoneum, omentum, and mesentery in children: radiologic-pathologic correlation: from the radiologic pathology archives. 2576 37

The onset of malignant solid tumors in infants is insidious and difficult to diagnose on time. The purpose of our study is to provide a theoretical basis for clinical diagnosis by retrospective analysis of the data in the past 14 years. Here, we retrospectively collected the clinical data of infants aged 0-12 months with malignant solid tumors in Beijing Tongren Hospital Affiliated to Capital Medical University from May 2005 to May 2019. The epidemiology, clinical characteristics, treatments and prognosis were statistically analyzed. A total of 496 infants (294 males and 202 females) with malignant solid tumors were analyzed. The main period of onset was 1-11 months. The most common tumor was retinoblastoma (RB, 51.8%), followed by hepatoblastoma (HB, 26.6%), neuroblastoma (NB, 10.5%), rhabdomyosarcoma (RMS, 3.4%), malignant renal tumors (3.2%), infantile fibrosarcoma (IFS, 1.6%), malignant teratoma (1.2%), Ewing's sarcoma (ES, 0.8%), medulloblastoma (MB, 0.4%) and inflammatory myofibroblastic tumor (IMT, 0.4%). The median follow-up time was 32 months (range 2-162 months). The 1-year, 3-year, and 5-year overall survival of all patients were 97.3%, 89.2%, and 81.1%, respectively, and event-free survival was 94.7%, 84.8%, and 75.8%, respectively. In conclusion, as a special group, malignant solid tumors in infants are complex, heterogeneous, and relatively rare. The prognosis of RB, HB, NB, RMS, malignant renal tumors, IFS, malignant teratoma, ES, MB, and IMT, were excellent duo to timely diagnosis and rational treatment.
 ...
PMID:Prevalence, clinical features and prognosis of malignant solid tumors in infants: a 14-year study. 3325 78