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Smooth Muscle Hamartoma

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101. Glomus Tumor (Diagnosis)

, with most cases involving subungual sites. These tumors are characteristically painful, often causing paroxysmal pain in response to temperature changes (especially cold) or pressure. Note the images below. Glomus tumor. Multiple glomus tumors. Glomus tumors are thought to arise from the glomus body or Sucquet-Hoyer canal, a thermoregulatory arteriovenous shunt composed of modified smooth muscle cells. [ , ] Glomus tumors most frequently occur in areas with high concentrations of glomus bodies (...) canal), primary collecting vein, intraglomerular reticulum, and a capsular portion. These specialized arteriovenous anastomoses are particularly concentrated in the reticular dermis of the fingers. Glomus tumors are thought to represent hamartomatous proliferations of modified smooth muscle cells originating from preexisting normal glomus cell populations. The three components of most glomus tumors include glomus cells, vasculature, and smooth muscle cells. The most common type of glomus tumor

2014 eMedicine.com

102. Benign Vulvar Lesions (Diagnosis)

. Different epithelia, from keratinized squamous epithelium to squamous mucosa, cover the vulva. The labia minora are rich with sebaceous glands but have few sweat glands and no hair follicles. The epithelium of the vestibule is neither pigmented nor keratinized and contains eccrine glands. Benign vulvar disorders are a significant issue for patients. These disorders include vulvar atrophy, benign tumors, hamartomas and cysts, infectious disorders, and nonneoplastic epithelial disorders. [ ] Infectious (...) vulvar melanosis Postinflammatory hypopigmentation Vulvar melanosis [ ] Benign tumors, hamartomas, and cysts involving the vulvar region include the following [ ] : Bartholin and Skene duct cysts (fibroepithelial polyp) Fibroma, fibromyoma, and dermatofibroma Hidradenoma Syringoma Hemangioma Angiokeratoma Heterotopic sebaceous glands and sebaceous gland hyperplasia Papillomatosis (papillary vulvar hirsutism) Schwannoma [ ] Angiomyxoma: a benign and aggressive mesenchymal tumor. [ ] Congenital

2014 eMedicine.com

103. Asthma (Diagnosis)

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.com

104. Pilar Cyst (Diagnosis)

aggressive. Recurrences and metastases have been observed, with rare malignant transformation. [ , ] Next: Pathophysiology Erroneously but often called sebaceous cysts, trichilemmal cysts are lined by stratified squamous epithelium similar to that in the isthmus of the hair follicle. [ ] This is the segment between the insertion of the erector pili muscle and the sebaceous gland duct, where no inner root sheath exists. The keratinization is similar to that which occurs in the outer root sheath (...) , Swanson NA. Benign Epithelial Tumors, Hamartomas, and Hyperplasias. Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K, eds. Fitzpatrick's Dermatology in General Medicine . 8th ed. New York, NY: McGraw-Hill; 2012. 1334. James WD, Berger TG, Elston DM. Andrews’ Diseases of the Skin: Clinical Dermatology . 12th ed. Philadelphia, Pa: Saunders Elsevier; 2015. 673. Al-Khateeb TH, Al-Masri NM, Al-Zoubi F. Cutaneous cysts of the head and neck. J Oral Maxillofac Surg . Jan 2009. 67(1):52-7

2014 eMedicine.com

105. Intestinal Polyposis Syndromes (Follow-up)

to the arborizing frond-like growth with delicate finger-like projections of the stroma. (Hematoxylin and eosin stain, 1X magnification). Hamartomatous (Peutz-Jeghers) polyp, small bowel. Closer view of the fingerlike projections of the stroma demonstrates prominent smooth muscle fascicles between the glandular elements. (Hematoxylin and eosin stain, 4X magnification). Hamartomatous polyp, as seen in the stomach. (Endoscopic image). Colon in familial adenomatous polyposis (FAP). (Endoscopic image). Small bowel (...) , Greer KE. Multiple hamartoma syndrome (Cowden's disease). Arch Dermatol . 1972 Nov. 106(5):682-90. . Padberg GW, Schot JD, Vielvoye GJ, Bots GT, de Beer FC. Lhermitte-Duclos disease and Cowden disease: a single phakomatosis. Ann Neurol . 1991 May. 29(5):517-23. . HD Riley WS. Macrocephaly, pseudopapilledema and multiple hemangiomata: a previously undescribed heredofamilial syndrome. Pediatrics . 1960. 26:293-300. Bannayan GA. Lipomatosis, angiomatosis, and macrencephalia. A previously undescribed

2014 eMedicine Pediatrics

106. Disorders of the Breast (Follow-up)

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

107. Intestinal Polyposis Syndromes (Overview)

magnification). Hamartomatous (Peutz-Jeghers) polyp, small bowel. This pedunculated polyp has a cerebriform appearance due to the arborizing frond-like growth with delicate finger-like projections of the stroma. (Hematoxylin and eosin stain, 1X magnification). Hamartomatous (Peutz-Jeghers) polyp, small bowel. Closer view of the fingerlike projections of the stroma demonstrates prominent smooth muscle fascicles between the glandular elements. (Hematoxylin and eosin stain, 4X magnification). Hamartomatous (...) it as a multiple hamartomatous syndrome with autosomal dominant inheritance. [ ] In 1991, Padberg et al suggested that the disorder known as cerebelloparenchymal disorder VI (Lhermitte-Duclos disease) is part of the multiple hamartoma syndrome. [ ] Individuals with Cowden disease present at age 10-30 years with hyperplastic hamartomatous polyps throughout the GI tract (including the esophagus), glycogenic acanthosis of the esophagus, orocutaneous hamartomas of the face, pulmonary hamartomas, and neoplasia

2014 eMedicine Pediatrics

108. Intestinal Polyposis Syndromes (Treatment)

a cerebriform appearance due to the arborizing frond-like growth with delicate finger-like projections of the stroma. (Hematoxylin and eosin stain, 1X magnification). Hamartomatous (Peutz-Jeghers) polyp, small bowel. Closer view of the fingerlike projections of the stroma demonstrates prominent smooth muscle fascicles between the glandular elements. (Hematoxylin and eosin stain, 4X magnification). Hamartomatous polyp, as seen in the stomach. (Endoscopic image). Colon in familial adenomatous polyposis (FAP (...) . . Weary PE, Gorlin RJ, Gentry WC Jr, Comer JE, Greer KE. Multiple hamartoma syndrome (Cowden's disease). Arch Dermatol . 1972 Nov. 106(5):682-90. . Padberg GW, Schot JD, Vielvoye GJ, Bots GT, de Beer FC. Lhermitte-Duclos disease and Cowden disease: a single phakomatosis. Ann Neurol . 1991 May. 29(5):517-23. . HD Riley WS. Macrocephaly, pseudopapilledema and multiple hemangiomata: a previously undescribed heredofamilial syndrome. Pediatrics . 1960. 26:293-300. Bannayan GA. Lipomatosis, angiomatosis

2014 eMedicine Pediatrics

109. Gastrointestinal Neoplasms (Follow-up)

of the GI tract in children, in addition to information gleaned from fairly sparse literature. Next: Esophageal Neoplasms Benign and malignant smooth-muscle tumors of the esophagus in children account for fewer than 5% of all leiomyomas and leiomyosarcomas. Tumors in the pediatric population, in contrast to those in adults, are multiple; localized tumors represent only 9% of cases. In children, these tumors are more commonly found in girls than in boys. Presenting symptoms include dysphagia, retained (...) of neoplasm in the GI tract, accounting for 80% of polyps in children. Grossly, the polyps appear beefy red and range in diameter from several millimeters to several centimeters. Histologically, these lesions represent benign hamartomas and have no malignant potential. Operative view of a pedunculated juvenile polyp found in the mid jejunum. Juvenile polyps commonly manifest as painless rectal bleeding in toddlers but may manifest with prolapse (4%) or abdominal pain (10%). [ ] Diagnosis is made through

2014 eMedicine Pediatrics

110. Disorders of the Breast (Treatment)

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

111. Mesenteric Tumors (Treatment)

, 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

112. Benign Neoplasm of the Small Intestine (Treatment)

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

113. Asthma (Diagnosis)

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

114. Tuberous Sclerosis (Overview)

and renal cysts. Angiomyolipomas, found in 70-80% of patients with tuberous sclerosis complex, are composed of blood vessels, smooth muscle, adipose tissue, and connective tissue. The gene for (PKD), PKD1, is contiguous with the TSC2 gene on chromosome 16, and patients with tuberous sclerosis complex occasionally have symptoms of PKD. Cardiac involvement is in the form of hamartomas, namely rhabdomyomas. Pulmonary lesions are lymphangioleiomyomatosis (LAM) and pulmonary cysts. These lesions are composed (...) of blood vessels, adipose tissue, and smooth muscle in abnormal arrangements. Ocular involvement includes retinal hamartomas or that may calcify but rarely lead to decreased visual acuity or other symptoms. Phalangeal cysts may develop in the hands and feet, and sclerotic lesions may develop in the pelvis or the spine. The genes responsible for tuberous sclerosis complex have been identified. In 1993, TSC2, located on chromosome 16, was the first gene discovered to be involved in tuberous sclerosis

2014 eMedicine Pediatrics

115. Tuberous Sclerosis (Diagnosis)

and renal cysts. Angiomyolipomas, found in 70-80% of patients with tuberous sclerosis complex, are composed of blood vessels, smooth muscle, adipose tissue, and connective tissue. The gene for (PKD), PKD1, is contiguous with the TSC2 gene on chromosome 16, and patients with tuberous sclerosis complex occasionally have symptoms of PKD. Cardiac involvement is in the form of hamartomas, namely rhabdomyomas. Pulmonary lesions are lymphangioleiomyomatosis (LAM) and pulmonary cysts. These lesions are composed (...) of blood vessels, adipose tissue, and smooth muscle in abnormal arrangements. Ocular involvement includes retinal hamartomas or that may calcify but rarely lead to decreased visual acuity or other symptoms. Phalangeal cysts may develop in the hands and feet, and sclerotic lesions may develop in the pelvis or the spine. The genes responsible for tuberous sclerosis complex have been identified. In 1993, TSC2, located on chromosome 16, was the first gene discovered to be involved in tuberous sclerosis

2014 eMedicine Pediatrics

116. Gastrointestinal Neoplasms (Treatment)

of the GI tract in children, in addition to information gleaned from fairly sparse literature. Next: Esophageal Neoplasms Benign and malignant smooth-muscle tumors of the esophagus in children account for fewer than 5% of all leiomyomas and leiomyosarcomas. Tumors in the pediatric population, in contrast to those in adults, are multiple; localized tumors represent only 9% of cases. In children, these tumors are more commonly found in girls than in boys. Presenting symptoms include dysphagia, retained (...) of neoplasm in the GI tract, accounting for 80% of polyps in children. Grossly, the polyps appear beefy red and range in diameter from several millimeters to several centimeters. Histologically, these lesions represent benign hamartomas and have no malignant potential. Operative view of a pedunculated juvenile polyp found in the mid jejunum. Juvenile polyps commonly manifest as painless rectal bleeding in toddlers but may manifest with prolapse (4%) or abdominal pain (10%). [ ] Diagnosis is made through

2014 eMedicine Pediatrics

117. Gastrointestinal Neoplasms (Overview)

of the GI tract in children, in addition to information gleaned from fairly sparse literature. Next: Esophageal Neoplasms Benign and malignant smooth-muscle tumors of the esophagus in children account for fewer than 5% of all leiomyomas and leiomyosarcomas. Tumors in the pediatric population, in contrast to those in adults, are multiple; localized tumors represent only 9% of cases. In children, these tumors are more commonly found in girls than in boys. Presenting symptoms include dysphagia, retained (...) of neoplasm in the GI tract, accounting for 80% of polyps in children. Grossly, the polyps appear beefy red and range in diameter from several millimeters to several centimeters. Histologically, these lesions represent benign hamartomas and have no malignant potential. Operative view of a pedunculated juvenile polyp found in the mid jejunum. Juvenile polyps commonly manifest as painless rectal bleeding in toddlers but may manifest with prolapse (4%) or abdominal pain (10%). [ ] Diagnosis is made through

2014 eMedicine Pediatrics

118. Disorders of the Breast (Overview)

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

119. Gastrointestinal Neoplasms (Diagnosis)

of the GI tract in children, in addition to information gleaned from fairly sparse literature. Next: Esophageal Neoplasms Benign and malignant smooth-muscle tumors of the esophagus in children account for fewer than 5% of all leiomyomas and leiomyosarcomas. Tumors in the pediatric population, in contrast to those in adults, are multiple; localized tumors represent only 9% of cases. In children, these tumors are more commonly found in girls than in boys. Presenting symptoms include dysphagia, retained (...) of neoplasm in the GI tract, accounting for 80% of polyps in children. Grossly, the polyps appear beefy red and range in diameter from several millimeters to several centimeters. Histologically, these lesions represent benign hamartomas and have no malignant potential. Operative view of a pedunculated juvenile polyp found in the mid jejunum. Juvenile polyps commonly manifest as painless rectal bleeding in toddlers but may manifest with prolapse (4%) or abdominal pain (10%). [ ] Diagnosis is made through

2014 eMedicine Pediatrics

120. Intestinal Polyposis Syndromes (Diagnosis)

magnification). Hamartomatous (Peutz-Jeghers) polyp, small bowel. This pedunculated polyp has a cerebriform appearance due to the arborizing frond-like growth with delicate finger-like projections of the stroma. (Hematoxylin and eosin stain, 1X magnification). Hamartomatous (Peutz-Jeghers) polyp, small bowel. Closer view of the fingerlike projections of the stroma demonstrates prominent smooth muscle fascicles between the glandular elements. (Hematoxylin and eosin stain, 4X magnification). Hamartomatous (...) it as a multiple hamartomatous syndrome with autosomal dominant inheritance. [ ] In 1991, Padberg et al suggested that the disorder known as cerebelloparenchymal disorder VI (Lhermitte-Duclos disease) is part of the multiple hamartoma syndrome. [ ] Individuals with Cowden disease present at age 10-30 years with hyperplastic hamartomatous polyps throughout the GI tract (including the esophagus), glycogenic acanthosis of the esophagus, orocutaneous hamartomas of the face, pulmonary hamartomas, and neoplasia

2014 eMedicine Pediatrics

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