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Smooth Muscle Hamartoma

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121. Disorders of the Breast (Diagnosis)

bilaterally or unilaterally. This is a rare condition and results when the mammary ridges fail to develop or completely disappear. Athelia or amastia is sometimes associated with (ie, absent chest wall muscles, absence of ribs 2-5, deformities of the hands or vertebrae). Amastia in girls can be treated with augmentation mammoplasty. An extra breast (ie, polymastia) or extra nipple (ie, polythelia) occurs in approximately 1% of the population. It may be an inheritable condition. [ ] Supernumerary nipples (...) -guided needle aspiration is significally less than that for open incision and drainage; thus, it may be the preferred treatment. [ ] Mastitis in nonlactating adolescents may occur. [ ] S aureus is the usual cause in cases that require drainage; however, most patients respond to oral antibiotics without additional intervention. Fibroadenoma Breast masses in adolescent girls are usually benign. The most common discrete breast mass is a (70%). Upon examination, these masses are smooth, mobile, and round

2014 eMedicine Pediatrics

122. Mesenteric Tumors (Overview)

, Kovatich AJ, Carr NJ, Emory TS, et al. Gastrointestinal stromal tumors/smooth muscle tumors (GISTs) primary in the omentum and mesentery: clinicopathologic and immunohistochemical study of 26 cases. Am J Surg Pathol . 1999 Sep. 23 (9):1109-18. . Seow-Choen F. The management of desmoids in patients with familial adenomatous polyposis (FAP). Acta Chir Iugosl . 2008. 55 (3):83-7. . Clark SK, Phillips RK. Desmoids in familial adenomatous polyposis. Br J Surg . 1996 Nov. 83 (11):1494-504. . Caspari R (...) is palpated on physical examination. It is very amenable to surgical excision, though local recurrence has been described. Other benign neoplasms of the mesentery are exceedingly rare, but tumors of neural origin, hamartomas, [ ] and stromal tumors of small size with nonaggressive clinical behavior have been described. Malignant mesenteric tumors A relatively few tumor types account for the vast majority of mesenteric malignancies; in these malignancies, the small-bowel mesentery is almost exclusively

2014 eMedicine Surgery

123. Benign Neoplasm of the Small Intestine (Follow-up)

manifestations. Gastrointest Radiol . 1991 Spring. 16(2):104-8. . Rüfenacht H, Kasper M, Heitz PU, et al. "Brunneroma": hamartoma or tumor?. Pathol Res Pract . 1986 Mar. 181(1):107-11. . Bremer EH, Battaile WG, Bulle PH. Villous tumors of the upper gastrointestinal tract. Clinical review and report of a case. Am J Gastroenterol . 1968 Aug. 50(2):135-43. . Ludwig DJ, Traverso LW. Gut stromal tumors and their clinical behavior. Am J Surg . 1997 May. 173(5):390-4. . He LJ, Wang BS, Chen CC. Smooth muscle (...) tumours of the digestive tract: report of 160 cases. Br J Surg . 1988 Feb. 75(2):184-6. . Morgan BK, Compton C, Talbert M, et al. Benign smooth muscle tumors of the gastrointestinal tract. A 24-year experience. Ann Surg . 1990 Jan. 211(1):63-6. . Amin MB, Ma CK, Linden MD, et al. Prognostic value of proliferating cell nuclear antigen index in gastric stromal tumors. Correlation with mitotic count and clinical outcome. Am J Clin Pathol . 1993 Oct. 100(4):428-32. . Nincheri Kunz M, Evaristi L, Spadoni R

2014 eMedicine Surgery

124. Mesenteric Tumors (Follow-up)

, Ali KP, Tej PR. Leiomyosarcoma of the mesentry, a rare mesentric tumour. Int J Surg Case Rep . 2015. 7C:58-60. . Varghese M, Bruland O, Wiedswang AM, Lobmaier I, Røsok B, Benjamin RS, et al. Metastatic mesenteric dedifferentiated leiomyosarcoma: a case report and a review of literature. Clin Sarcoma Res . 2016. 6:2. . Miettinen M, Monihan JM, Sarlomo-Rikala M, Kovatich AJ, Carr NJ, Emory TS, et al. Gastrointestinal stromal tumors/smooth muscle tumors (GISTs) primary in the omentum and mesentery (...) cystic lymphangioma of the small bowel in an adult: A case presentation and literature review. Acta Gastroenterol Belg . 2016 Sep-Dec. 79 (3):491-493. . Gonzalez-Crussi F, deMello DE, Sotelo-Avila C. Omental-mesenteric myxoid hamartomas. Infantile lesions simulating malignant tumors. Am J Surg Pathol . 1983 Sep. 7 (6):567-78. . Goh BK, Chow PK, Kesavan SM, Yap WM, Chung YF, Wong WK. A single-institution experience with eight CD117-positive primary extragastrointestinal stromal tumors: critical

2014 eMedicine Surgery

125. Asthma (Overview)

in the United States. The pathophysiology of asthma is complex and involves airway inflammation, intermittent airflow obstruction, and bronchial hyperresponsiveness. The mechanism of inflammation in asthma may be acute, subacute, or chronic, and the presence of airway edema and mucus secretion also contributes to airflow obstruction and bronchial reactivity. Varying degrees of mononuclear cell and eosinophil infiltration, mucus hypersecretion, desquamation of the epithelium, smooth muscle hyperplasia (...) , and airway remodeling are present. [ , ] Airway hyperresponsiveness or bronchial hyperreactivity in asthma is an exaggerated response to numerous exogenous and endogenous stimuli. The mechanisms involved include direct stimulation of airway smooth muscle and indirect stimulation by pharmacologically active substances from mediator-secreting cells such as mast cells or nonmyelinated sensory neurons. The degree of airway hyperresponsiveness generally correlates with the clinical severity of asthma

2014 eMedicine Emergency Medicine

126. An Incidentally Discovered Asymptomatic Para-Aortic Paraganglioma with Peutz-Jeghers Syndrome Full Text available with Trip Pro

and pedunculated polyp in duodenum. Colonoscopy revealed multiple colonic polyps. Pathological examination of the polyps confirmed hamartomas with smooth muscle arborization, compatible with Peutz-Jeghers polyps. CT scan guided left para-aortic lymph node biopsy revealed the characteristic features of extra-adrenal para-aortic paraganglioma. Although cases of various GI and extra GI malignancies in PJS patients has been reported, the present case appears to be the first in literature in which the PJS syndrome

2012 Saudi journal of gastroenterology : official journal of the Saudi Gastroenterology Association

127. Muscular tumors of the stomach: clinical and pathological study of 113 cases. Full Text available with Trip Pro

surgery Male Middle Aged Muscle, Smooth pathology Neoplasms, Muscle Tissue diagnosis pathology surgery Stomach Neoplasms diagnosis pathology surgery 1973 8 1 2001 3 28 10 1 1973 8 1 0 0 ppublish 4353198 PMC1355623 Cancer. 1970 Jan;25(1):176-85 5410309 Acta Pathol Microbiol Scand. 1967;71(2):194-202 5582844 Cancer. 1955 Jan-Feb;8(1):101-11 13231039 Cancer. 1962 Mar-Apr;15:400-9 13917556 (...) Muscular tumors of the stomach: clinical and pathological study of 113 cases. 4353198 1973 10 11 2018 11 13 0003-4932 178 2 1973 Aug Annals of surgery Ann. Surg. Muscular tumors of the stomach: clinical and pathological study of 113 cases. 148-55 Morrissey K K Cho E S ES Gray G F GF Jr Thorbjarnarson B B eng Journal Article United States Ann Surg 0372354 0003-4932 AIM IM Aged Female Hamartoma diagnosis pathology surgery Humans Leiomyoma diagnosis pathology surgery Leiomyosarcoma pathology

1973 Annals of Surgery

128. Lymphangioleiomyomatosis

of lymphangioleiomyomatosis is unknown. The tempting hypothesis that female sex hormones play a role in pathogenesis remains unproved. The disease usually arises spontaneously, but LAM bears many similarities to the pulmonary findings of (TS); LAM occurs in some patients with TS and is thought by some to be a forme fruste of TS. Mutations in the tuberous sclerosis complex-2 gene ( TSC-2 ) have been described in LAM cells and angiomyolipomas (benign renal hamartomas made of smooth muscle, blood vessels, and adipose (...) have increased accumulations of tau in the entorhinal cortex (ERC), according to a study scheduled... 3D Model Pneumothorax Video How to do Needle Thoracostomy SOCIAL MEDIA Add to Any Platform Loading , MD, MAS, University of Colorado Denver Click here for Patient Education NOTE: This is the Professional Version. CONSUMERS: Lymphangioleiomyomatosis (LAM) is an indolent, progressive growth of smooth muscle cells throughout the lungs, pulmonary blood vessels, lymphatics, and pleurae. It is rare

2013 Merck Manual (19th Edition)

129. Evaluation and Care of the Normal Neonate

in the birth by the mother and her partner helps them adapt to parenting. The First Few Hours Immediately at delivery, the neonate’s respiratory effort, heart rate, color, tone, and reflex irritability should be assessed; all are key components of the Apgar score assigned at 1 min and 5 min after birth (see Table: ). Apgar scores between 8 and 10 indicate that the neonate is making a smooth transition to extrauterine life; scores ≤ 7 at 5 min (particularly if sustained beyond 10 min) are linked to higher (...) , pale Pink body, blue extremities All pink Heart rate P ulse Absent 100 beats/min > 100 beats/min Reflex response to nasal catheter/tactile stimulation G rimace None Grimace Sneeze, cough Muscle tone A ctivity Limp Some flexion of extremities Active Respiration R espiration Absent Irregular, slow Good, crying *A total score of 7–10 at 5 min is considered normal; 4–6, intermediate; and 0–3, low. Preventive interventions include administration into both eyes of an antimicrobial agent (eg, 0.5

2013 Merck Manual (19th Edition)

130. Tuberous sclerosis

they are commonly multiple and bilateral. About 20-30% of people with TSC have renal cysts, causing few problems. However, 2% may also have . Very rare (< 1%) problems include and ( ). Lungs [ ] Patients with TSC can develop progressive replacement of the lung parenchyma with multiple cysts, known as (LAM). Recent genetic analysis has shown that the proliferative bronchiolar smooth muscle in TSC-related lymphangioleiomyomatosis is monoclonal metastasis from a coexisting renal angiomyolipoma. Cases of TSC (...) of the neck, or scattered across the trunk or thighs. The frequency of these lesions rises with age. pits are found in almost all adults with TSC. Intraoral are small surface-tumours found in the gums, inside the cheeks or tongue. Gum (gingival) fibromas are found in about 20-50% of people with TSC, more commonly in adults. Eyes [ ] Retinal lesions, called astrocytic hamartomas (or "phakomas"), which appear as a greyish or yellowish-white lesion in the back of the globe on the ophthalmic examination

2012 Wikipedia

131. A case of diffuse pilar leiomyoma or acquired smooth muscle hamartoma? (Abstract)

A case of diffuse pilar leiomyoma or acquired smooth muscle hamartoma? A 62-year-old woman presented with a skin-coloured indurated asymptomatic plaque, 150 mm in diameter on the knee. Histological analysis of a skin biopsy taken from the lesion showed haphazardly arranged bundles of smooth muscle in the deep dermis, characteristic of a pilar leiomyoma. Cutaneous pilar leiomyomas are rare, benign smooth muscle tumours arising from the arrector pili muscle, which usually appear as red or brown (...) papules < 15 mm in diameter. This case is unusual in its clinical appearance and size, being at least 10 times larger than the largest previously documented cutaneous pilar leiomyoma. Similar lesions have also been previously reported under the term 'acquired smooth muscle hamartoma' (ASMH). These cases are also rare and have no known consistent clinical features. Histologically it is difficult to differentiate between ASMH and leiomyomas, and some authors would consider this case as a new report

2009 Clinical & Experimental Dermatology

133. Effect of Fasting on the Size of Abdominal Lymphatic Tumors in Women

disease of women that is characterized by a proliferation of abnormal smooth muscle-like cells (LAM cells) in the lungs, which leads to cystic destruction of the lung parenchyma, in the axial lymphatics, resulting in lymphangioleiomyomas, and in abdominal angiomyolipomas, primarily in the kidneys. Lymphangioleiomyomas may cause abdominal distension and compress abdominal organs, producing obstipation, bladder obstruction and neurological deficits. Leakage of chyle may be responsible for ascites (...) MeSH terms: Layout table for MeSH terms Sclerosis Tuberous Sclerosis Lymphangioleiomyomatosis Lymphangiomyoma Pathologic Processes Hamartoma Neoplasms Neoplasms, Multiple Primary Neoplastic Syndromes, Hereditary Malformations of Cortical Development, Group I Malformations of Cortical Development Nervous System Malformations Nervous System Diseases Neurocutaneous Syndromes Heredodegenerative Disorders, Nervous System Neurodegenerative Diseases Congenital Abnormalities Genetic Diseases, Inborn

2007 Clinical Trials

134. Survivin Expression in Tuberous Sclerosis Complex Cells Full Text available with Trip Pro

Survivin Expression in Tuberous Sclerosis Complex Cells Tuberous Sclerosis Complex (TSC) is a tumor suppressor gene disorder with mutations of TSC1/TSC2 genes. This leads to the development of hamartomas that most frequently affect central nervous system, kidney, and skin. Angiomyolipomas are abdominal masses made up of muscle vessels and adipose tissues that grow mostly in proximity to kidneys and liver. Bleeding and kidney failure are the major justification for surgery. This study shows (...) that angiomyolipoma-derived human smooth muscle TSC2-/- cells express the apoptosis inhibitor protein survivin when exposed to IGF-1. Survivin expression is also triggered whenever culture conditions perturb normal TSC2-/- cell function, such as the omission of EGF from the growth medium, the supplementation of anti-EGFR, blockade of PI3K and ERK, or inhibition of mTOR. Interestingly, single or simultaneous inhibition of PI3K by LY294002 and ERK by PD98059 does not prevent IGF-1-mediated survivin expression

2007 Molecular Medicine

135. Adenomyoma of the stomach mimicking infantile hypertrophic pyloric stenosis. (Abstract)

Adenomyoma of the stomach mimicking infantile hypertrophic pyloric stenosis. We report a case of gastric adenomyoma in a 1-month-old girl. This patient presented with gastric outlet obstruction simulating infantile hypertrophic pyloric stenosis. Histologic examination of the excised specimen showed irregularly arranged glands and interlacing smooth muscle bundles surrounding the glandular elements. We suggest that this lesion is not just heterotopia, but hamartoma composed of abnormal mixture (...) of endoderm-derived epithelial component and mesoderm-derived smooth muscle.

2007 Journal of Pediatric Surgery

136. Adenomyomatous polyp of the endometrium: a case report. (Abstract)

and was found to have a large, sessile, polypoid mass completely filling the endometrial cavity, consisting of multiple, thick-walled vessels; scant smooth muscle; and foci of endometrial glands and stroma. This lesion was diagnosed as an adenomyomatous polyp. No history of tamoxifen use was present in this case. The diagnosis of perivascular epithelioid cell tumor was ruled out.We found 1 other report of a similar lesion, which may have represented a hamartoma. (...) Adenomyomatous polyp of the endometrium: a case report. Adenomyomatous polyps of the endometrium are rare, pedunculated lesions that contain smooth muscle in addition to the usual components of an endometrial polyp. Grossly, adenomyomatous polyps have the same appearance as an ordinary endometrial polyp and are characterized histologically by the intertwining of smooth muscle, endometrial glands and endometrial stroma.A 32-year-old woman underwent a hysterectomy for refractory menometrorrhagia

2008 Journal of Reproductive Medicine

137. Hamartomatous tongue lesions in children. (Abstract)

). Interestingly, hamartomatous lesions (18/135) were the third most common lesion category identified. Lingual hamartomas were predominantly submucosal in location and were classified histologically by tissue composition as follows: neurovascular (2/18), smooth muscle predominant (5/18), fat predominant (1/18), and smooth muscle and fat containing (10/18). All 5 smooth muscle predominant hamartomas also contained vasculature, and 1 case additionally contained salivary gland tissue. The single fat predominant (...) hamartoma additionally contained vessels and salivary gland. The final 10 hamartomas contained varying amounts of both smooth muscle and fat, and also admixed combinations of vessels, nerves, and salivary glands. Two of these 10 cases additionally contained foci of choristomatous elements, including cutaneous adnexal structures and cartilage. Most patients with hamartomatous lesions were young, 2 years or less. Eight cases were congenital in origin. Females outnumbered males by 2:1. The majority

2007 American Journal of Surgical Pathology

138. Epithelial and mesenchymal hamartomatous changes in a mature port-wine stain: Morphologic evidence for a multiple germ layer field defect. (Abstract)

histologic observations that may elucidate this phenomenon. An extensive PWS on the face of a 75-year-old man exhibited gross thickening with cobblestoning and nodularity. Histologic examination revealed not only the expected vascular abnormalities, but also a number of widely distributed epithelial, neural, and mesenchymal hamartomatous changes. Epithelial changes included epidermal nevus, sebaceous trichofolliculoma, and basaloid follicular hamartoma. Changes of connective tissue nevus, smooth-muscle (...) hamartoma, neural hamartoma, and subcuticular hamartoma were also noted. The complex hamartomatous changes observed in the PWS of this patient involved multiple germ lines and were distributed in a widespread pattern. These changes not only offer an explanation for the skin thickening and nodularity of this patient, but also suggest a genetically determined, multilineage developmental field defect in the pathogenesis of this lesion. Further studies of other patients are necessary to understand the full

2004 Journal of American Academy of Dermatology

139. Primary cutaneous diffuse leiomyosarcoma with desmoplasia. (Abstract)

Primary cutaneous diffuse leiomyosarcoma with desmoplasia. A 65-year-old man presented with an indurated plaque over his left cheek and left neck. An initial punch biopsy of skin showing increased smooth muscle bundles was consistent with a diagnosis of smooth muscle hamartoma. A second incisional skin biopsy revealed a well-differentiated smooth muscle proliferation invading into the dermis and subcutaneous fat in a diffusely infiltrative pattern and with a desmoplastic component, suggesting

2006 Australasian Journal of Dermatology

140. Clinical and molecular insights into lymphangioleiomyomatosis. (Abstract)

Clinical and molecular insights into lymphangioleiomyomatosis. Lymphangioleiomyomatosis (LAM) is a rare disease of women that is characterized by a proliferation of abnormal smooth muscle-like cells (LAM cells), which leads to cystic lung lesions, lymphatic abnormalities, and abdominal tumors (e.g., angiomyolipomas). LAM occurs sporadically or in association with tuberous sclerosis complex, an autosomal dominant syndrome characterized by hamartoma-like tumor growths. The tumor suppressor genes

2005 Sarcoidosis, Vasculitis, and Diffuse Lung Diseases

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