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Seminars in Reproductive Medicine May 2010Leiomyomas are believed to derive from the transformation of myometrial smooth muscle cells/connective tissue fibroblasts. Although the identity of the molecule(s) that... (Review)
Review
Leiomyomas are believed to derive from the transformation of myometrial smooth muscle cells/connective tissue fibroblasts. Although the identity of the molecule(s) that initiate such cellular transformation and orchestrate subsequent growth is still unknown, conventional evidence indicates that ovarian steroids are essential for leiomyoma growth. Ovarian steroid action in their target cell/tissue is mediated in part through local expression of various growth factors, cytokines, and chemokines. These autocrine/paracrine molecules with proinflammatory and profibrotic activities serve as major contributing factors in regulating cellular transformation, cell growth and apoptosis, angiogenesis, cellular hypertrophy, and excess tissue turnover, events central to leiomyoma growth. This review addresses the key regulatory functions of proinflammatory and profibrotic mediators and their molecular mechanisms, downstream signaling that regulates cellular events that result in transformation, and commitments of specific cells into forming a cellular environment with a possible role in development and subsequent growth of leiomyomas.
Topics: Female; Humans; Inflammation Mediators; Leiomyoma; MicroRNAs; Phenotype; Transforming Growth Factor beta; Uterine Neoplasms
PubMed: 20414842
DOI: 10.1055/s-0030-1251476 -
Modern Pathology : An Official Journal... Jan 2014Smooth muscle tumors are here considered an essentially dichotomous group composed of benign leiomyomas and malignant leiomyosarcomas. Soft tissue smooth muscle tumors... (Review)
Review
Smooth muscle tumors are here considered an essentially dichotomous group composed of benign leiomyomas and malignant leiomyosarcomas. Soft tissue smooth muscle tumors with both atypia and mitotic activity are generally diagnosed leiomyosarcomas acknowledging potential for metastasis. However, lesions exist that cannot be comfortably placed in either category, and in such cases the designation 'smooth muscle tumor of uncertain biologic potential' is appropriate. The use of this category is often necessary with limited sampling, such as needle core biopsies. Benign smooth muscle tumors include smooth muscle hamartoma and angioleiomyoma. A specific category of leiomyomas are estrogen-receptor positive ones in women. These are similar to uterine leiomyomas and can occur anywhere in the abdomen and abdominal wall. Leiomyosarcomas can occur at any site, although are more frequent in the retroperitoneum and proximal extremities. They are recognized by likeness to smooth muscle cells but can undergo pleomorphic evolution ('dedifferentiation'). Presence of smooth muscle actin is nearly uniform and desmin-positivity usual. This and the lack of KIT expression separate leiomyosarcoma from GIST, an important problem in abdominal soft tissues. EBV-associated smooth muscle tumors are a specific subcategory occurring in AIDS or post-transplant patients. These tumors can have incomplete smooth muscle differentiation but show nuclear EBER as a diagnostic feature. In contrast to many other soft tissue tumors, genetics of smooth muscle tumors are poorly understood and such diagnostic testing is not yet generally applicable in this histogenetic group. Leiomyosarcomas are known to be genetically complex, often showing 'chaotic' karyotypes including aneuploidy or polyploidy, and no recurrent tumor-specific translocations have been detected.
Topics: Angiomyoma; Biomarkers, Tumor; Cell Differentiation; Hamartoma; Herpesvirus 4, Human; Humans; Leiomyoma; Leiomyosarcoma; Neoplasms, Muscle Tissue; Prognosis; Soft Tissue Neoplasms; Terminology as Topic
PubMed: 24384850
DOI: 10.1038/modpathol.2013.178 -
Archives of Pathology & Laboratory... Aug 2013Myofibroblastic proliferations of the urinary bladder, which share some similarities with nodular fasciitis, were first reported in 1980. Since then, they have had... (Review)
Review
CONTEXT
Myofibroblastic proliferations of the urinary bladder, which share some similarities with nodular fasciitis, were first reported in 1980. Since then, they have had several designations, the most frequently used being inflammatory myofibroblastic tumor. Based on both histopathologic and prognostic grounds, some authors prefer the term pseudosarcomatous myofibroblastic proliferation, at least for some of the proliferations. These same scientists also assimilate the so-called postoperative spindle cell nodules with the pseudosarcomatous myofibroblastic proliferations. Little is known about these low-grade myofibroblastic proliferations.
OBJECTIVES
To review the literature about low-grade myofibroblastic proliferations occurring in the urinary bladder.
DATA SOURCES
Textbooks and literature review. We obtained most of the clinicopathologic peculiarities from a patient population composed of the most-relevant, previously reported cases.
CONCLUSIONS
The low-grade myofibroblastic proliferations of the urinary bladder are rare lesions affecting males more often than they do females. The most-common signs and symptoms are hematuria and dysuria. Histopathologically, they are spindle cell proliferations in a loose myxoid stroma, even though compact proliferations or hypocellular fibrous patterns can be found. Immunohistochemistry is quite nonspecific, except for ALK-1 positivity (20%-89%). Fluorescence in situ hybridization has demonstrated clonal genetic aberrations involving the ALK gene in 50% to 60% of cases. After surgery, only 6% of patients experience local recurrence, without metastases or deaths from the disease. Malignant transformation has been reported exceptionally. These myofibroblastic proliferations are probably part of a continuum with, at one end, benign pseudosarcomatous proliferations and, at the opposite end, more-aggressive lesions. Because of the frequently indolent clinical course, aggressive treatment would be unjustified.
Topics: Anaplastic Lymphoma Kinase; Cell Proliferation; Female; Humans; Immunohistochemistry; Inflammation; Male; Myofibroblasts; Neoplasms, Muscle Tissue; Receptor Protein-Tyrosine Kinases; Urinary Bladder; Urinary Bladder Neoplasms
PubMed: 23899070
DOI: 10.5858/arpa.2012-0326-RA -
Medical Science Monitor : International... May 2020BACKGROUND The aim of this study was to investigate STMN1 and MKI67 expression in uterine leiomyosarcoma and their potential roles as biomarkers for diagnosis. MATERIAL...
BACKGROUND The aim of this study was to investigate STMN1 and MKI67 expression in uterine leiomyosarcoma and their potential roles as biomarkers for diagnosis. MATERIAL AND METHODS The expression of STMN1 and MKI67 mRNA in uterine leiomyosarcoma were investigated in TCGA database. The overall survival (OS) and disease-free survival (DFS) were compared between high and low expression groups. Seventy-two patients who received hysterectomy were included and divided into 4 groups: uterine normal smooth muscle tissue (UNSM=30), uterine leiomyoma (UL=30), uterine cellular leiomyoma (UCL=24), and uterine leiomyosarcoma (ULS=18). The STMN1 and MKI67 protein expression of the 4 groups were examined by immunohistochemistry (IHC) assay. RESULTS The expression level of STMN1 mRNA in cancer tissue was significantly higher than those of normal uterine smooth muscle tissue. The high and low expression of STMN1 and mki67 gene mRNA was not related to the patients' OS and DFS (P>0.05). The positive rate of STMN1 protein in uterine leiomyosarcoma was 100.00%, which was significantly higher than that of the other 3 groups (χ²=11.72, P=0.008). And the positive rate of KIM67 protein in uterine leiomyosarcoma was 77.78%, which was also significantly higher than that of the other 3 groups (χ²=48.89, P=0.000). The diagnostic sensitivity and specificity were 77.78%, 90.74% for STMN1 combined MKI67 with the positive predictive value and negative predictive value of 73.68% and 92.45%, respectively. CONCLUSIONS STMN1 and MKI67 were upregulated in uterine leiomyosarcoma and act as potential biomarkers for uterine leiomyosarcoma diagnosis.
Topics: Biomarkers, Tumor; Databases, Genetic; Diagnosis, Differential; Disease-Free Survival; Female; Gene Expression; Gene Expression Regulation, Neoplastic; Humans; Immunohistochemistry; Ki-67 Antigen; Leiomyoma; Leiomyosarcoma; Stathmin; Uterine Neoplasms
PubMed: 32425177
DOI: 10.12659/MSM.923749 -
The Journal of International Medical... 2011Primary pancreatic leiomyosarcoma is a rare mesenchymal tumour that is believed to arise from the walls of the pancreatic blood vessels or the pancreatic duct. A...
Primary pancreatic leiomyosarcoma is a rare mesenchymal tumour that is believed to arise from the walls of the pancreatic blood vessels or the pancreatic duct. A 56-year-old female was referred with epigastric pain and abdominal mass. Preoperative computed tomography showed a large soft tissue mass in the pancreatic body and tail. Fine needle aspiration biopsy indicated a spindle cell type tumour. The patient received distal pancreatectomy with no adjuvant treatment. Histology revealed a pleomorphic spindle cell neoplasm with an immunoprofile suggestive of smooth muscle origin. The absence of other lesions in the body was consistent with the diagnosis of primary pleomorphic leiomyosarcoma. The patient was well and tumour-free 14 months after surgery. Detailed immunohistochemical analyses are necessary in the diagnosis of this highly malignant tumour. Radical resection offers the only chance of long-term survival.
Topics: Female; Humans; Leiomyosarcoma; Middle Aged; Pancreatectomy; Pancreatic Neoplasms; Prognosis; Tomography, X-Ray Computed
PubMed: 21986161
DOI: 10.1177/147323001103900447 -
In Vivo (Athens, Greece) 2019Power morcellation remains one of the most significant developments in minimal access surgery over the past decade, allowing many more patients to benefit from the least... (Review)
Review
Power morcellation remains one of the most significant developments in minimal access surgery over the past decade, allowing many more patients to benefit from the least invasive surgical route. However, its use is not without controversy, particularly with regards to the risks of an undiagnosed leiomyosarcoma. Increased media and, in particular, on-going social media coverage since events in 2014 have only served to intensify the debate, culminating in the Food and Drug Administration essentially 'banning' its use in the USA. Practice however continues to vary and this technique remains widely used in Europe and in particular the UK. The aim of this article was to review the development of power morcellation in gynaecology and the underlying risks, including that of undiagnosed leiomyosarcoma, as well as appraise the evolving literature on patient awareness and informed consent and the wider implications of morcellation restriction.
Topics: Animals; Disease Management; Evidence-Based Practice; Female; Humans; Incidence; Leiomyoma; Leiomyosarcoma; Minimally Invasive Surgical Procedures; Morcellation; Quality Improvement; Undiagnosed Diseases
PubMed: 31471384
DOI: 10.21873/invivo.11616 -
The Journal of Hand Surgery Mar 1994Vascular leiomyomas or angioleiomyomas are benign solitary smooth muscle tumors that occur uncommonly in the hand. The peak incidence is in the third to fifth decades of... (Review)
Review
Vascular leiomyomas or angioleiomyomas are benign solitary smooth muscle tumors that occur uncommonly in the hand. The peak incidence is in the third to fifth decades of life, and men are more often affected than women. This tumor is rarely diagnosed before surgery. The usual treatment is simple excision of the mass and ligation of feeder vessels. Although the tumors occur anywhere in the hand, there are only two previous cases of vascular leiomyoma involving the digital artery. A recent case of this tumor involving a digital artery documented by arteriography and treated by excision of the mass and end-to-end anastomosis of the artery is presented. The authors review their experience with vascular leiomyomas in the hand and present four cases along with a review of 105 cases found in the English literature.
Topics: Adult; Angiomyoma; Female; Fingers; Hand; Humans; Male; Middle Aged; Soft Tissue Neoplasms; Vascular Diseases
PubMed: 8201195
DOI: 10.1016/0363-5023(94)90020-5 -
Cancer Science May 2021Uterine leiomyosarcoma (LMS) is a rare but deadly disease. Due to poor understanding of the molecular and genetic causes of the disease, the diagnosis of LMS has been...
Uterine leiomyosarcoma (LMS) is a rare but deadly disease. Due to poor understanding of the molecular and genetic causes of the disease, the diagnosis of LMS has been based primarily on histology. Nuclear atypia is one of hallmarks in LMS, however, it also occurs in 2 clinically benign variants, including smooth muscle tumors with fumarate hydratase alteration (SMT-FH) and leiomyoma with bizarre nuclei (LM-BN). In addition to nuclear atypia, many well recognized biomarkers used for LMS are also frequently overexpressed in LM-BN, and the histogenesis and molecular natures for LM-BN and LMS remain largely unknown. To characterize the molecular profiling of LMS, SMT-FH, and LM-BN, we performed integrated comprehensive genomic profiling including whole-genome sequencing (WGS) and RNA sequencing and genomic microarray analyses to assess genome-wide copy number alterations (CNAs) and immunohistochemistry (IHC) in all 3 tumor types. We found that both LM-BN and LMS showed genomic instability and harbored extensive CNAs throughout the whole genome. By contrast, the SMT-FH presented its characteristic 1q43-44 deletions in all cases tested, with minimal CNAs in the rest of genomic regions. Further analyses revealed that LMS and LM-BN groups showed similar patterns of CNAs that are tended to cluster together and separated from the SMT-FH group. The integrated molecular profiling enabled the detection of novel and traditional biomarkers and showed excellent discrimination between LM-BN and LMS. Our study suggests that LM-BN, despite having similar nuclear atypia to SMT-FH, showed similar genomic instability but distinct genomic alterations with its malignant counterpart of LMS. The integrated molecular profiling is of clinical importance in characterizing these rare uterine smooth muscle tumors.
Topics: Adult; Biomarkers, Tumor; Cell Nucleus; Female; Fumarate Hydratase; Gene Deletion; Gene Dosage; Gene Expression Profiling; Humans; Leiomyoma; Leiomyosarcoma; Middle Aged; Muscle, Smooth; Necrosis; Principal Component Analysis; Sequence Analysis, RNA; Tissue Array Analysis; Uterine Neoplasms
PubMed: 33338329
DOI: 10.1111/cas.14775 -
The British Journal of Radiology Apr 2021The purpose of this study was to describe the imaging appearance, diagnosis, and management of mammary and extramammary myofibroblastoma (MFB) in a series of 23 patients. (Review)
Review
OBJECTIVE
The purpose of this study was to describe the imaging appearance, diagnosis, and management of mammary and extramammary myofibroblastoma (MFB) in a series of 23 patients.
METHODS AND MATERIALS
Following institutional review board approval, cases were identified by searching for "myofibroblastoma" in radiology reports. Multimodality imaging and pathological features were assessed.
RESULTS
23 cases of myofibroblastoma were identified in 15 males and 8 females. Most cases were in the breast (20/23, 87%), presenting as a palpable mass or discovered incidentally on mammography in females or chest CT in males. Extramammary MFB lesions (3/23, 13%) presented with symptoms related to mass effect. At imaging, MFB most often demonstrated an oval or irregular mass that was hypoechoic or heterogeneously echogenic with variable margins. MRI showed T2 hyperintensity, diffusion restriction, and plateau kinetics. Extramammary MFB appeared as an enhancing mass with variable fat content and T2 intensity.
CONCLUSION
Here we describe imaging and clinicopathological features of mammary and extramammary myofibroblastoma.
ADVANCES IN KNOWLEDGE
Imaging description of this rare tumor is limited in the literature, and to date this is the largest case series describing the imaging findings.
Topics: Aged; Aged, 80 and over; Breast; Breast Neoplasms; Diagnosis, Differential; Diagnostic Imaging; Female; Humans; Magnetic Resonance Imaging; Male; Mammography; Middle Aged; Multimodal Imaging; Neoplasms, Muscle Tissue; Positron Emission Tomography Computed Tomography; Radiography, Thoracic; Tomography, X-Ray Computed; Ultrasonography
PubMed: 33332985
DOI: 10.1259/bjr.20201019 -
Pathologica Dec 2019The diagnosis of bland-looking spindle cell lesions of the breast is often challenging because there is a close morphological and immunohistochemical overlap among the... (Review)
Review
The diagnosis of bland-looking spindle cell lesions of the breast is often challenging because there is a close morphological and immunohistochemical overlap among the different entities. The present review will discuss reactive spindle cell nodule/exuberant scar, nodular fasciitis, inflammatory pseudotumor, myofibroblastoma (classic type), lipomatous myofibroblastoma, palisaded myofibroblastoma, benign fibroblastic spindle cell tumor, spindle cell lipoma, fibroma, leiomyoma, solitary fibrous tumor, myxoma, schwannoma/neurofibroma, desmoid-type fibromatosis, dermatofibrosarcoma protuberans, low-grade fibromatosis-like spindle cell carcinoma, inflammatory myofibroblastic tumor and low-grade myofibroblastic sarcoma arising in the breast parenchyma. The pathologist should be aware of each single lesion to achieve a correct diagnosis to ensure patient a correct prognostic information and therapy. Accordingly representative illustrations and morphological/immunohistochemical diagnostic clues will be provided.
Topics: Breast; Breast Neoplasms; Carcinoma; Dermatofibrosarcoma; Diagnosis, Differential; Fasciitis; Female; Fibrosarcoma; Humans; Neoplasms, Muscle Tissue; Skin Neoplasms
PubMed: 31965112
DOI: 10.32074/1591-951X-31-19