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LeiomyomaUterine NeoplasmsMyometriumTumor Markers, BiologicalLeiomyoma, EpithelioidTumor BurdenLeiomyomatosisAngiomyomaCell Line, TumorTumor Necrosis Factor-alphaUterine MyomectomyLeiomyosarcomaNeoplasms, ExperimentalWilms TumorGenes, Tumor SuppressorHysterectomyCarcinoid TumorTumor Suppressor Protein p53Cell ProliferationBrain NeoplasmsSmooth Muscle TumorImmunohistochemistryNeoplasmsNeuroendocrine TumorsTumor Suppressor ProteinsAdenocarcinomaNeoplasm MetastasisNorpregnadienesNeovascularization, PathologicGastrointestinal Stromal TumorsMammary Neoplasms, ExperimentalTumor MicroenvironmentRNA, MessengerTomography, X-Ray ComputedCell DivisionGene Expression Regulation, NeoplasticUterine Artery EmbolizationNeoplasm ProteinsReverse Transcriptase Polymerase Chain ReactionSkin NeoplasmsLiver NeoplasmsOvarian NeoplasmsMuscle NeoplasmsSignal TransductionTransplantation, HeterologousMenorrhagiaAntigens, NeoplasmCell Transformation, NeoplasticSoft Tissue NeoplasmsMice, Inbred BALB CTreatment OutcomeColonic NeoplasmsFood, PreservedReceptors, ProgesteroneSarcomaXenograft Model Antitumor AssaysBone NeoplasmsKidney NeoplasmsVascular NeoplasmsNeoplasm StagingMagnetic Resonance ImagingCarcinoma, Squamous CellNose NeoplasmsBlotting, WesternBronchial NeoplasmsFibrosarcomaTime FactorsRetrospective StudiesMice, SCIDGliomaNeoplasms, Multiple PrimaryPancreatic NeoplasmsMammary Neoplasms, AnimalDNA, NeoplasmGenital Neoplasms, MaleLung NeoplasmsMice, Inbred C57BLTransforming Growth Factor beta3Mice, NudeKi-67 AntigenSarcoma, ExperimentalMutationSelenium-Binding ProteinsCarcinoma, Ehrlich TumorBreast NeoplasmsGene Expression ProfilingDown-RegulationOsteosarcomaUterusEsophageal NeoplasmsCell SurvivalDisease Progression
Mesenchymal tumorsBenign and malignantAtypical LeiomyomaNeoplasmsLeiomyosarcomasDifferential diagnosisUterineCD117UterusNeoplasmSpindleHistologicClinicalFibrosarcomaGISTHeterogeneousFibrousLesionsPrognosisTractInflammatoryVariantsPeripheralDiagnosisSoft-tissueSuggestsMolecularRareRepresentCancerSmooth muscleFeaturesHigh riskMassIncreaseSizeSolidApproximatelyStageFinalGradePresenceLarge
Mesenchymal tumors4
  • Accurate diagnosis of GIST typically relies on a combination of cytologic and immunohistochemical characteristics to distinguish them from other GI mesenchymal tumors. (rgare.com)
  • GISTs are a subset of mesenchymal tumors and represent the most common mesenchymal neoplasms of GI tract. (biomedcentral.com)
  • Gastrointestinal stromal tumors are KIT-expressing and KIT (tyrosine kinase receptor - CD117)-signaling driven mesenchymal tumors. (biomedcentral.com)
  • Dr. Howitt is a gynecologic and sarcoma pathologist, with academic interests in gynecologic mesenchymal tumors and morphologic and clinical correlates of molecular alterations in gynecologic neoplasia. (stanford.edu)
Benign and malignant1
  • The differential diagnosis of a subepithelial tumor arising in the GI tract is broad, including GISTs and other benign and malignant tumours. (rgare.com)
Atypical Leiomyoma2
  • 15. Coexistence of Conventional Leiomyoma, Fumarate Hydratase-deficient Atypical Leiomyoma, and Perivascular Epithelioid Cell Tumor in a Uterus: A Case Study. (nih.gov)
  • The histologic distinction between atypical leiomyoma and low grade leiomyosarcoma is difficult and is based on the following criteria: number of mitosis, cellular atypia and pattern of necrosis 5,6 . (bvsalud.org)
Neoplasms3
  • 6. Uterine perivascular epithelioid cell neoplasms (PEComas): report of two cases and literature review. (nih.gov)
  • 14. [Clinicopathological features of uterine neoplasms with perivascular epithelioid cell differentiation]. (nih.gov)
  • Soft-tissue tumors constitute a large and heterogeneous group of neoplasms. (medscape.com)
Leiomyosarcomas3
  • By light microscopy alone the distinction among GISTs and other tumours in the differential diagnosis (particularly leiomyomas, true leiomyosarcomas, and GI tract schwannomas) can be difficult, because the histologic findings do not reliably or specifically relate to the immunophenotype or the molecular genetics of the lesions. (rgare.com)
  • There were 49 cases of smooth muscle tumors: 27 leiomyomas and 22 leiomyosarcomas. (bvsalud.org)
  • In the past, these tumors were presumed to have elements of smooth muscle (smooth muscle origin), so they were classified as leiomyomas, leiomyosarcomas and leiomyoblastomas [ 3 ]. (biomedcentral.com)
Differential diagnosis1
Uterine9
  • 1. Five cases of uterine perivascular epithelioid cell tumors (PEComas) and review of literature. (nih.gov)
  • 3. Perivascular Epithelioid Cell Tumor (PEComa) of the Uterine Cervix: A Case Report of a 43-Yr-Old Woman With Abnormal Uterine Bleeding Treated With Hysterectomy. (nih.gov)
  • 4. Perivascular Epithelioid Cell Tumor (PEComa) of the Uterine Cervix in a Patient with Tuberous Sclerosis Complex: A Literature Review. (nih.gov)
  • 5. Microscopic endometrial perivascular epithelioid cell nodules: a case report with the earliest presentation of a uterine perivascular epithelioid cell tumor. (nih.gov)
  • 9. Uterine Perivascular Epithelioid Cell Tumor (PEComa) in A 56-year-Old Woman. (nih.gov)
  • 10. Perivascular epithelioid cell tumors of the uterine cervix. (nih.gov)
  • 12. [Endometrial stromal sarcoma complicating uterine perivascular epithelioid cell tumor: report of a case]. (nih.gov)
  • 19. Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa). (nih.gov)
  • ULMS are rare smooth muscle tumors accounting for approximately 1% of patients with uterine cancer 1 with an estimated annual incidence of 0.64 per 100,000 women. (sarcomahelp.org)
CD1171
  • Greater than 90% of GISTs express the CD117 antigen as evidence of the KIT mutation, which helps distinguish this tumour from other bowel wall tumors such as leiomyomas and other spindle cell tumors (which are CD117 negative). (rgare.com)
Uterus10
  • 2. Malignant perivascular epithelioid cell tumor (PEComa) of the uterus. (nih.gov)
  • 7. Perivascular epithelioid cell tumor (PEComa) of the uterus with aggressive behavior at presentation. (nih.gov)
  • 8. Two cases of perivascular epithelioid cell tumor of the uterus: clinical, radiological and pathological diagnostic challenge. (nih.gov)
  • 11. Perivascular epithelioid cell neoplasm (PEComa) of the uterus: A systematic review. (nih.gov)
  • 17. Perivascular Epithelioid Cell Tumor of the Uterus with Ovarian Involvement: A Case Report and Review of the Literature. (nih.gov)
  • 18. [Perivascular epithelioid cell tumours of the uterus]. (nih.gov)
  • 20. [Perivascular epithelioid cell tumor (PEComa) of uterus : report of a case]. (nih.gov)
  • 5-7 On pelvic examination, the uterus is often enlarged, and in some cases part of the tumor may prolapse through the cervical os and into the vaginal canal. (sarcomahelp.org)
  • Benign leiomyomas ( fibroids ) and ULMS often coexist in the same uterus, but are genetically distinct entities. (sarcomahelp.org)
  • The belief that the risk of ULMS is elevated among women with a "rapidly growing" uterus or leiomyoma was proven false in a study of 1322 women admitted to two community hospitals for hysterectomy or myomectomy . (sarcomahelp.org)
Neoplasm3
  • Malignant C4258 Pleomorphic Rhabdomyosarcoma An aggressive malignant mesenchymal neoplasm with skeletal muscle differentiation, occurring in adults and rarely in children. (nih.gov)
  • Malignant C27273 Poroma Eccrine Poroma A benign, well circumscribed sweat gland neoplasm with eccrine or apocrine differentiation. (nih.gov)
  • Hereditary Wilms' Tumor Familial embryonal neoplasm derived from nephrogenic blastemal cells. (nih.gov)
Spindle2
  • Those with spindle cell GISTs have a slightly better survival rate compared to epithelioid or mixed histology GIST tumours. (rgare.com)
  • The tumor is characterized by the presence of bizarre round, spindle, and polygonal cells. (nih.gov)
Histologic1
  • 12] There have also been reports of nerve sheath tumors exhibiting histologic hybrid features of schwannoma and soft-tissue perineurioma. (medscape.com)
Clinical2
  • The aim of this retrospective study is to evaluate and compare clinical features, like gender, age, location and symptomatology of leiomyoma and leiomyosarcoma, and review the literature from 1970 to 2010, since there isn't any recent comparative article. (bvsalud.org)
  • The database of the German Clinical Center of Competence for Genital Sarcomas and Mixed Tumors in Greifswald (DKSM) was reviewed between the years 2010 and 2020. (amjcaserep.com)
Fibrosarcoma2
  • Fibrosarcoma , for example, is categorized as a tumor arising from fibroblasts. (medscape.com)
  • Patients with the inherited form appear to be at increased risk for secondary nonocular malignancies such as osteosarcoma, malignant fibrous histiocytoma, and fibrosarcoma. (nih.gov)
GIST10
  • Malignant GIST tumors represent less than 1% of GI cancers. (rgare.com)
  • Incidentally found microscopic GIST tumors can be more common. (rgare.com)
  • Small GIST tumors have been co-incidentally found in 35% of patients when their stomach was removed due to underlying gastric adenocarcinoma. (rgare.com)
  • With increased screening and use of imaging techniques, it is expected that we will see a further increase in GIST tumor incidence rates in the future. (rgare.com)
  • In terms of staging, 53% of GIST tumours stage as localized, 19% as regional, 23% as distant, and 5% as unstaged. (rgare.com)
  • GIST tumors have also been stratified by risk into four levels, tagged I to IV, also based on tumor size and mitotic index. (rgare.com)
  • Critical Illness Claims Issue # 1 - Is the tumour a GIST? (rgare.com)
  • Another tyrosine kinase mutation that is occasionally seen in KIT mutation-negative GIST tumours is the platelet-derived growth factor receptor alpha (PDGFRA). (rgare.com)
  • Critical Illness Claims Issue # 2 - Is a GIST malignant? (rgare.com)
  • However, GIST is a recently recognized tumor entity and the literature on these stromal tumors has rapidly expanded. (biomedcentral.com)
Heterogeneous1
  • By the early 1990s, it became apparent that there were inconsistencies and ambiguities in the heterogeneous collection of tumours classified as GISTs. (rgare.com)
Fibrous3
  • Generally, soft-tissue tumors grow centripetally, though some benign tumors (eg, fibrous lesions) may grow longitudinally along tissue planes. (medscape.com)
  • This results in the formation of a relatively well-defined zone of compressed fibrous tissue that may contain scattered tumor cells. (medscape.com)
  • The patient presented for consultation with the result of an incisional biopsy that reported a proliferative lesion combining fibrous elements of fascicular or swirling arrangement with collagenized streaks with dense, lymphoplasmacytic infiltrate and some macrophages, with negative staining for ALK 1, with a diagnosis of inflammatory myofibroblastic tumour.Due to suspicion of IgG4-related disease (IgG4-RD) the biopsy was sent for review and pertinent complementary studies were requested. (bvsalud.org)
Lesions1
  • These tumors represented 0,092% of all oral lesions, with similar distribution between genders. (bvsalud.org)
Prognosis1
  • Prior to the 1970s, surgery was the primary therapy for malignant soft tissue tumors, and most patients with high-grade tumors had a poor prognosis and a significant mortality. (medscape.com)
Tract1
  • These tumours can also be found incidentally when investigating a patient for some other gastrointestinal disease or on routine imaging of the GI tract. (rgare.com)
Inflammatory1
  • Objective: To describe the case of a patient attended for neurological involvement due to hypertrophic pachymeningitis with initial diagnosis of inflammatory myofibroblastic tumour and final diagnosis of IgG4-related disease. (bvsalud.org)
Variants1
  • These tumors were previously considered variants of alveolar rhabdomyosarcoma. (nih.gov)
Peripheral3
  • Along with neurofibroma, schwannoma constitutes one of the two most common benign peripheral nerve sheath tumors. (medscape.com)
  • The presence of a noninvasive tumor next to a peripheral nerve suggests the diagnosis of neurilemmoma. (medscape.com)
  • The peripheral portion of the tumor compresses surrounding, normal soft tissue because of centripetal expansile growth. (medscape.com)
Diagnosis1
  • Current achievements in the field of soft-tissue tumors are the result of advances in molecular biology, oncogenetics, imaging techniques, immunochemistry, diagnosis by fine-needle aspiration (FNA), surgical reconstruction, radiation therapy, and tissue banking. (medscape.com)
Soft-tissue4
  • Although most soft-tissue tumors of various histogenetic types are classified as either benign or malignant, many are of an intermediate nature, which typically implies aggressive local behavior with a low-to-moderate propensity for metastasis. (medscape.com)
  • Benign soft-tissue tumors are fairly common and are treated with surgery alone. (medscape.com)
  • [ 2 , 3 ] See Soft-Tissue Sarcomas: What You Need to Know , a Critical Images slideshow, to help identify and treat some of these malignant tumors of mesenchymal origin. (medscape.com)
  • Most soft-tissue tumors respect fascial boundaries, remaining confined to the compartment of origin until the later stages of development. (medscape.com)
Suggests1
  • The lack of PAX3-FOXO1 fusions in most of these tumors suggests that are biologically and clinically related to embryonal rhabdomyosarcoma. (nih.gov)
Molecular2
  • Pathogenic alterations, molecular signatures, tumor mutational burden (TMB), and copy number alterations (CNA) were correlated with pattern of invasion.45 HPVA (11 pattern A, 17 pattern B and 17 pattern C tumors) were included. (stanford.edu)
  • CNA burden increased from pattern A to C.Our findings further indicate that key molecular events in HPVA correlate with the morphologic invasive properties of the tumor and their aggressiveness. (stanford.edu)
Rare1
  • Smooth muscle tumors are very rare in the oral cavity due to the scarcity of smooth muscle in this region, and arise mainly from tunnica media of blood vessels. (bvsalud.org)
Represent1
  • Malignant Gastrointestinal Stromal Tumours (GISTs) represent less than 1% of GI cancers. (rgare.com)
Cancer1
  • The origin of these tumors explicates their resistance to cancer chemotherapy. (biomedcentral.com)
Smooth muscle2
  • Cases of smooth muscle tumors of the oral cavity were also compiled from 1970 to 2010. (bvsalud.org)
  • Nine thousand seven hundred and seventy histopathologic exams were accomplished during this period and only 64 (0,092%) were diagnosed as smooth muscle tumors, that corresponded to 49 cases, being 27 (55%) leiomyomas e 22 (45%) leiomyossarcomas. (bvsalud.org)
Features2
  • Of note, the benign nerve sheath tumors are classified as World Health Organization (WHO) grade I on the basis of their benign cytologic features, in contrast to the malignant counterparts, which are WHO grade III or IV. (medscape.com)
  • Traditionally, tumors have been classified according to histogenetic features. (medscape.com)
High risk2
  • APOBEC mutational signature was detected only in Silva pattern C tumors (5/17), and pathogenic PIK3CA changes were detected only in destructively invasive HPVA (patterns B and C). When cases were grouped as low-risk (pattern A and pattern B without LVI) and high-risk (pattern B with LVI and pattern C), high-risk tumors were enriched in mutations in PIK3CA, ATRX, and ERBB2. (stanford.edu)
  • There was a statistically significant difference in TMB between low-risk and high-risk pattern tumors (p=0.006), as well as between Pattern C tumors with and without an APOBEC signature (p=0.002). (stanford.edu)
Mass1
  • The tumors are composed entirely of the supporting Schwann cells and peripherally displaced nerve fibers, resulting in a globoid eccentric tumor mass. (medscape.com)
Increase1
  • The larger tumors increase the size of the parent nerve and become separated from surrounding fascicles by a capsule derived from perineurium and epineurium, as demonstrated in the image. (medscape.com)
Size2
  • This means that simple endoscopy can miss these tumors or underestimate their size. (rgare.com)
  • Endoscopic ultrasound better estimates the size of these tumors. (rgare.com)
Solid2
  • From 27 leiomyomas, 21 (78%) were vascular type and only 6 (22%) were solid, no epithelioid was found. (bvsalud.org)
  • Final pathology revealed a solid tumor consistent with a moderately differentiated leiomyosarcoma of the vulva. (amjcaserep.com)
Approximately1
  • Comprises approximately 1% of Wilms tumors. (nih.gov)
Stage1
  • The pattern-based (Silva) classification of invasive HPV-associated endocervical adenocarcinomas (HPVA) is an established and reproducible method to predict outcomes for this otherwise stage-dependent group of tumors. (stanford.edu)
Final1
  • Objetivo: Describir el caso de una paciente asistida en nuestro servicio por compromiso neurológico por PH con diagnóstico inicial de tumor miofibroblástico inflamatorio (TMI) y diagnóstico final de enfermedad relacionada con IgG4. (bvsalud.org)
Grade1
  • A thin layer of tissue called the reactive zone surrounds the compression zone, especially in higher-grade tumors. (medscape.com)
Presence1
  • Endocervical Carcinoma An adenocarcinoma characterized by the presence of malignant glandular epithelium resembling the endocervical epithelium. (nih.gov)
Large1
  • The rarity of these tumors has prevented the performance of large epidemiological studies to identify risk factors. (sarcomahelp.org)