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Smooth Muscle Hamartoma

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21. 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy Full Text available with Trip Pro

= one-PDZ and one-LIM domain protein; MLP/CRP3 = muscle LIM protein/cysteine-rich protein 3; FHL2 = four-and-a-half LIM protein 2; MAPRs = muscle ankyrin repeat proteins; MURFs = muscle-specific ring-finger proteins. Modified with permission of the American Physiological Society. x 38 Hoshijima, M. Mechanical stress-strain sensors embedded in cardiac cytoskeleton: Z disk, titin, and associated structures. Am J Physiology Heart Circ Physiol . 2006 ; 290 : H1313–H1325 | | | ---- | ---- Figure 6 (...) external peer review by reviewers appointed by HRS and each of the collaborating societies, and revisions were made by the chairs. Section 2 Arrhythmogenic cardiomyopathy 2.1. Arrhythmogenic cardiomyopathy ACM is defined as an arrhythmogenic heart muscle disorder not explained by ischemic, hypertensive, or valvular heart disease. ACM may present clinically as symptoms or documentation of atrial fibrillation, conduction disease, and/or right ventricular (RV) and/or left ventricular (LV) arrhythmia

2019 International Society for Heart and Lung Transplantation

23. Guideline on the Diagnosis, Treatment, and Follow-up of Patients with Endometrial Cancer

, Treatment, and Follow-up of Patients with Endometrial Cancer | April 2018 13 Abbreviation Explanation MCP metoclopramide MELF microcystic, elongated, fragmented (glandular pattern) MGA megestrol acetate MMMT malignant müllerian mixed tumor MPA medroxyprogesterone acetate MRI magnetic resonance imaging MSI-H microsatellite instability–high NK 1 RA neurokinin-1 receptor antagonist PCOS polycystic ovary syndrome PET-CT positron emission tomography–computed tomography PHTS PTEN hamartoma tumor syndrome PMB

2018 German Guideline Program in Oncology

25. Management of Uterine Fibroids

, Intervention, Comparator, Outcomes, Timing, and Setting Appendix C. Screening Forms Appendix D. Reasons for Exclusion Appendix E. Risk of Bias Form and Summary Appendix F. Registered Study Protocols Appendix G. Study Outcome Data Appendix H. Estimates of Subsequent Treatment Appendix I. Summary of Existing Systematic Reviews xiii Evidence Summary Introduction Uterine fibroids (i.e., leiomyomata) are common benign smooth muscle tumors of the uterus. Most women will develop one or more uterine fibroids (...) concern affecting surgical treatment is the risk of discovering a leiomyosarcoma, a cancer of the uterine muscle, rather than a fibroid at surgery. These are rare but ominous: an average of 1,600 new cases occur in the United States each year. They have poor outcomes with an average 5-year survival of 36 percent if cancer is not isolated to the uterus. Scope To inform clinical decisions about care we focused on evidence from randomized controlled trials (RCTs) that assess currently available

2017 Effective Health Care Program (AHRQ)

26. The UK guidelines for management and surveillance of Tuberous Sclerosis Complex Full Text available with Trip Pro

, recurrent pneumothoraces, and deterioration in lung function. LAM is characterized by abnormal proliferation or infiltration of smooth muscle cells, lymphatic vessels and cystic destruction of the lung. These cells are thought to express a melanogenisis//-associated antigen recognized by HMB45 and they may express estrogen and progesterone receptor proteins. Mutations in TSC2 gene occur in both TSC-associated and sporadic LAM smooth muscle cells and lead to overactivation of the mTOR pathway. mTOR (...) with antiarrhythmic medications, surgery or mTOR inhibitors depending on the situation. Eye Approximately half of patients with TSC have retinal or optic nerve hamartomas; in half of these patients, the hamartomas occur bilaterally. Three morphological types of retinal hamartomas have been reported in literature: flat, smooth, non-calcified, grey, translucent lesions; elevated, multinodular, calcified, opaque lesions, resembling mulberries; and transitional lesions, which have morphological features of both

2018 Tuberous Sclerosis Association

28. Genetics of Skin Cancer (PDQ®): Health Professional Version

different anatomic locations can have moderately differing aggressiveness; for example, SCC from glabrous (smooth, hairless) skin has a lower metastatic rate than SCC arising from the vermillion border of the lip or from scars.[ ] Additionally, in the epidermal compartment, melanocytes distribute singly along the basement membrane and can undergo malignant transformation into melanoma. Melanocytes are derived from neural crest cells and migrate to the epidermal compartment near the eighth week (...) development. These can form a large variety of benign or malignant tumors with diverse biological behaviors. Several of these tumors are associated with familial syndromes. Overall, there are dozens of different histological subtypes of these tumors associated with individual components of the adnexal structures.[ ] Finally, the subcutis is a layer that extends below the dermis with varying depth, depending on the anatomic location. This deeper boundary can include muscle, fascia, bone, or cartilage

2018 PDQ - NCI's Comprehensive Cancer Database

30. Childhood Vascular Tumors Treatment (PDQ®): Health Professional Version

with congestive heart failure have been mistaken for infantile hemangiomas. Other tumors in the differential diagnosis include angiosarcoma, metastatic neuroblastoma, and mesenchymal hamartomas. If there is any question about the diagnosis, a biopsy is recommended, although bleeding is a risk of the procedure.[ ] Spindle Cell Hemangioma Clinical presentation Spindle cell hemangiomas, initially called spindle cell hemangioendotheliomas, often occur as superficial (skin and subcutis), painful lesions involving (...) can arise spontaneously, in sites of trauma, or within capillary and arteriovenous malformations. Pyogenic granulomas have also been associated with medications including oral contraceptives and retinoids. Most occur as solitary growths, but multiple (grouped) or rarely disseminated lesions have been described. These lesions appear as small or large, smooth or lobulated vascular nodules that can grow rapidly, sometimes over weeks to months and have a tendency to bleed profusely. These lesions

2018 PDQ - NCI's Comprehensive Cancer Database

31. Genetics of Kidney Cancer (Renal Cell Cancer) (PDQ®): Health Professional Version

hemangioblastomas appear as a bright orange spherical tumor supplied by a tortuous vascular supply. Nearly 50% of patients have bilateral retinal hemangioblastomas.[ ] The median number of lesions per affected eye is approximately six.[ ] Other retinal lesions in VHL can include retinal vascular hamartomas, flat vascular tumors located in the superficial aspect of the retina.[ ] Longitudinal studies are important for the understanding of the natural history of these tumors. Left untreated, retinal

2018 PDQ - NCI's Comprehensive Cancer Database

32. Myoid (Muscular) Hamartoma of the Breast: Case Report and Review of the Literature Full Text available with Trip Pro

Myoid (Muscular) Hamartoma of the Breast: Case Report and Review of the Literature BACKGROUND: Myoid (muscular) hamartoma (MH) of the breast is a rare benign tumour-forming lesion composed of differentiated mammary glandular and stromal structures, fatty tissue, and areas of smooth muscle. It is considered to be a variant of mammary hamartoma. CASE REPORT: We report the case of a 46-year-old woman with MH, and provide a literature review explaining the origin of smooth muscle cells (...) . Histologically, the tumour consisted of fibrolipomatous stroma containing ductal and lobular structures of the mammary gland located mainly at the tumour borders. The glandular structures showed signs of micro- and macrocystic changes, apocrine metaplasia, and adenosis. The dominant feature was the presence of a fascicular formation of spindle cells, predominantly in central parts, with incursion between glandular structures. Immunohistochemically, foci of smooth muscle tissue were positive for desmin

2010 Breast Care

33. Gastric Adenomyoma: The Unexpected Mimicker Full Text available with Trip Pro

Gastric Adenomyoma: The Unexpected Mimicker Gastric adenomyoma is a rare benign tumor composed of epithelial structures and smooth muscle stroma. Here, we report an unusual case of gastric adenomyoma mostly composed of smooth muscle that was incidentally found during a laparoscopic intervention. On radiology, it mimicked an acquired hypertrophic pyloric stenosis in an adult patient, and pathologically it resembled a pure smooth muscle hamartoma. Complete submission of the lesion for histology

2017 GE Portuguese journal of gastroenterology

34. Renal Cell Carcinoma

occurs in tuberous sclerosis (TS). It accounts for approximately 1% of surgically removed tumours. Ultrasound, CT, and MRI often lead to diagnosis due to the presence of adipose tissue. Biopsy is rarely useful. Pre-operatively, it may be difficult to differentiate between smooth muscle cell tumours and epithelial tumours. AML can be found in TS in LNs, but is not metastases, and has a multicentric genesis. AML can be due to angiotrophic-type growth in the renal vein or the IVC. AML with LN (...) ), tuberous sclerosis complex, germline succinate dehydrogenase (SDH) mutation, nonpolyposis colorectal cancer syndrome, hyperparathyroidism-jaw tumour syndrome, PTEN hamartoma syndrome, constitutional chromosome 3 translocation, and familial nonsyndromic ccRCC. RMC can be included because of its association with hereditary haemoglobinopathies [4, 5, 36, 47]. 3.3.4 Angiomyolipoma (AML) Angiomyolipoma is a benign mesenchymal tumour, can occur sporadically, and is four times more likely in women. It also

2015 European Association of Urology

35. A Practice Guideline from the American College of Medical Genetics and Genomics and the National Society of Genetic Counselors: Referral Indications for Cancer Predisposition Assessment

%), and small intestinal (13%) cancers. In addition, there are increased risks for breast cancer (54%), ovar- ian sex cord tumors with annular tubules (21%), and adenoma malignum of the cervix (10%) and the testes, especially Sertoli cell tumors (9%). 162 PJ polyps are hamartomatous with glandu- lar epithelium supported by smooth muscle cells contiguous with the muscularis mucosa. Referral should be considered for any individual with a per- sonal history of or first-degree relative with (i) two or more his (...) or periungual fibroma • Hypomelanotic macules (=3) • Shagreen patch (connective tissue nevus) • Cortical tuber in the brain • Subependymal glial nodule • Subependymal giant cell astrocytoma • Multiple retinal nodular hamartomas • Cardiac rhabdomyomas, single or multiple • Lymphangiomyomatosis • Renal angiomyolipoma Minor criteria • Multiple, randomly distributed pits in dental enamel • Hamartomatous rectal polyps • Bone cysts • “Confetti” skin lesions • Multiple renal cysts • Nonrenal hamartoma • Cerebral

2015 American College of Medical Genetics and Genomics

36. Genetic Testing and Management of Hereditary Gastrointestinal Cancer Syndromes

of the GI tract should undergo evaluation for JPS. Genetic evaluation of a patient with possible JPS should include testing for SMAD4 and BMPR1A mutations. Cowden syndrome (PTEN hamartoma tumor syndrome) Individuals with multiple gastrointestinal hamartomas or ganglioneuromas should be evaluated for Cowden syndrome and related conditions. Genetic evaluation of a patient with possible Cowden syndrome should include testing for PTEN mutations. Serrated/hyperplastic polyposis syndrome Individuals who meet (...) quality of evidence). 17. Cardiovascular examination for and evaluation for hereditary hemorrhagic telangiectasia should be considered for SMAD4 mutation carriers (conditional recommendation, very low quality of evidence). Cowden syndrome (PTEN hamartoma tumor syndrome) 18. Surveillance in affected or at-risk Cowden syndrome patients should include screening for colon, stomach, small bowel, thyroid, breast, uterine, kidney, and skin (melanoma) cancers (conditional recommendation, low quality

2015 American College of Gastroenterology

37. 2015 American Thyroid Association Management Guidelines for Adult Patients with Thyroid Nodules and Differentiated Thyroid Cancer

to stratify the risk of malignancy in thyroid nodules, and aid decision-making about whether FNA is indicated. Studies consistently report that several US gray scale features in multivariate analyses are associated with thyroid cancer, the majority of which are PTC. These include the presence of microcalci?cations, nodule hypoechogenicity compared with the surrounding thyroid or strap muscles, irregular margins (de?ned as either in?ltrative, microlobulated, or spiculated), and a shape taller than wide (...) ). FTC ex- hibits other differences in sonographic features compared to PTC. These tumors are more likely to be iso- to hyperechoic, noncalci?ed, round (width greater than anterioposterior di- mension) nodules with regular smooth margins (82). Similarly, the follicular variant of papillary cancer (FVPTC) is also more likely than conventional PTC to have this same appearance as FTC (79). Distant metastases are rarely observed arising from follicular cancers 70–90 a Recommend FNA at ‡1cm Intermediate

2015 Pediatric Endocrine Society

38. Laser treatment of epidermal nevi: A multicenter retrospective study with long-term follow-up. (Abstract)

patients treated with a laser for an epidermal nevus with more than a one-year follow-up.Seventy patients were treated for different types of epidermal nevi, mostly with ablative lasers: 23 verrucous epidermal nevi, 16 nevi sebaceous, 26 Becker nevi, two inflammatory linear verrucous epidermal nevi, one smooth-muscle hamartoma, one rounded and velvety epidermal nevus, and one nevus lipomatosus superficialis. The follow-up period ranged between 12 and 127 months (median 37 months). Better results, fewer

2019 Journal of American Academy of Dermatology

39. Childhood Soft Tissue Sarcoma Treatment (PDQ®): Health Professional Version

peripheral nerve sheath tumor). Smooth muscle (e.g., leiomyosarcoma). Vascular tissue—blood and lymphatic vessels (e.g., angiosarcoma). (Refer to the PDQ summary on for more information about childhood vascular tumors.) Distribution of Soft Tissue Sarcoma by Age and Histology Pediatric soft tissue sarcomas are a heterogenous group of malignant tumors that originate from primitive mesenchymal tissue and account for 7% of all childhood tumors (rhabdomyosarcomas, 4%; other soft tissue sarcomas, 3 (...) for children and adolescents with cancer. Between 1975 and 2010, childhood cancer mortality decreased by more than 50%.[ ] Childhood and adolescent cancer survivors require close monitoring because cancer therapy side effects may persist or develop months or years after treatment. (Refer to the PDQ summary on for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.) Rhabdomyosarcoma, a tumor of striated muscle, is the most common soft

2017 PDQ - NCI's Comprehensive Cancer Database

40. Clinicopathologic characteristics of early-onset Becker's nevus in Korean children and adolescents. (Abstract)

Clinicopathologic characteristics of early-onset Becker's nevus in Korean children and adolescents. Becker's nevus (BN) presents as a hairy patch or plaque with or without proliferation of the dermal smooth muscles. BN has been described as acquired as found in a similar entity, congenital smooth muscle hamartoma (CSMH). This study was aimed at evaluating the clinicopathological aspects of BN in Korean cases in differential diagnosis with CSMH.We performed a retrospective study of 103 patients (...) involvement tended to make severe cosmetic concerns. In contrast, hypertrichosis was more frequent in CSMH. Either skin-colored lesion or pseudo-Darier's sign was not seen in early-onset BN. BN showed less dermal smooth muscle than CSMH.Androgens themselves do not seem to be related to the development of BN but play only an aggravating role especially in male patients. Considering high occurrence in exposed areas, BN may distress patients severely. As early laser treatment may be helpful in some patients

2017 International Journal of Dermatology

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