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Smooth Muscle Hamartoma

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41. A case of adult undifferentiated embryonal sarcoma of the liver successfully treated with right trisectionectomy: a case report Full Text available with Trip Pro

trisectionectomy. Histopathological studies revealed that the tumor was composed of pleomorphic and polynuclear dyskaryotic cells with eosinophilic globules in the cytoplasm. Mesenchymal hamartoma-like tissue was observed in the peripheral part of the tumor. Immunohistochemical analyses showed the tumor stained with vimentin, α-smooth muscle actin, desmin, α1-antitrypsin, and α1-antichymotripsin. Therefore, a histological diagnosis of UESL was made. Eighteen months following treatment, two recurrent tumors

2017 Surgical Case Reports

42. Gastrointestinal disorders in Curry–Jones syndrome: Clinical and molecular insights from an affected newborn Full Text available with Trip Pro

in addition to an apparent intestinal duplication that seemed to originate posterior to the pancreas. Histopathology of serosal nodules revealed bundles of smooth muscle with associated ganglion cells. Molecular analysis demonstrated the SMO c.1234 C>T mutation in varying amounts in affected skin (up to 35%) and intestinal hamartoma (26%). Gastrointestinal features including structural malformations, motility disorders, and upper GI bleeding are major causes of morbidity in CJS. Smooth muscle hamartomas (...) are a recognized feature of children with CJS typically presenting with abdominal obstruction requiring surgical intervention. A somatic mutation in SMO likely accounts for the structural malformations and predisposition to form bowel hamartomas and myofibromas. The mutation burden in the involved tissues likely accounts for the variable manifestations.© 2017 Wiley Periodicals, Inc.

2017 American journal of medical genetics. Part A

43. 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

2016 PDQ - NCI's Comprehensive Cancer Database

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

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%).[ ] The distribution (...) 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 tissue sarcoma

2016 PDQ - NCI's Comprehensive Cancer Database

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

give rise to SCC, and this cancer has somewhat differing behavior depending on its source. Even in cancer derived from the skin, SCC from 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 (...) the dermis with varying depth, depending on the anatomic location. This deeper boundary can include muscle, fascia, bone, or cartilage. The subcutis can be affected by inflammatory conditions such as panniculitis and malignancies such as liposarcoma.[ ] These compartments give rise to their own malignancies but are also the region of immediate adjacent spread of localized skin cancers from other compartments. The boundaries of each skin compartment are used to define the staging of skin cancers

2016 PDQ - NCI's Comprehensive Cancer Database

46. 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

2016 PDQ - NCI's Comprehensive Cancer Database

48. Neck lump

at the lateral edge of the thyroid cartilage [ ]. Thyroglossal cysts may become infected and can present as inflammatory swellings [ ]. Branchial cyst Branchial cysts most commonly present in early adult life as painless, slow-growing, smooth , fluctuant swellings in the lateral neck (but the first presentation may be when they become infected). They usually occur along the line of the deep cervical lymph nodes at the junction of the upper third and lower two thirds of the anterior border of sternomastoid (...) [ ; ; ; ; ]. Laryngocele A laryngocele arises from the laryngeal saccule and can extend through the thyrohyoid membrane. It usually presents with intermittent neck swelling, which becomes palpable when the person performs the valsalva manoeuvre [ ]. Other types of congenital and developmental lumps include: Dermoid cyst — inclusion cyst occurring along lines of fusion (for example under the tongue, on the palate) [ ]. Lymphangioma (cystic hygroma) — soft, fluctuant masses (lymphatic hamartomas) under the skin; usually

2016 NICE Clinical Knowledge Summaries

50. Un cas d'hamartome musculaire cervical retrouvé à Antananarivo Full Text available with Trip Pro

Universitaire Joseph Ravoahangy Andrianavalona, Antananarivo, Madagascar. fre Case Reports Journal Article Un cas d'hamartome musculaire cervical retrouvé à Antananarivo. 2015 11 23 Uganda Pan Afr Med J 101517926 IM Child Female Hamartoma diagnosis pathology surgery Humans Madagascar Muscle, Smooth pathology Neck pathology cervical muscle hematoma cervicotomy latero-cervical swelling 2015 10 26 2015 11 12 2016 3 12 6 0 2015 1 1 0 0 2016 12 15 6 0 epublish 26966480 10.11604/pamj.2015.22.283.8284 PAMJ-22-283 (...) Un cas d'hamartome musculaire cervical retrouvé à Antananarivo 26966480 2016 12 13 2016 12 30 1937-8688 22 2015 The Pan African medical journal Pan Afr Med J [A case of cervical muscle hematoma found in Antananarivo]. 283 10.11604/pamj.2015.22.283.8284 Razafindrakoto Rex Mario RM Service d'Oto-rhino-laryngologie et de Chirurgie Cervico-faciale, Centre Hospitalier Universitaire d'Andohatapenaka, Antananarivo, Madagascar. Laza Odilon O Département d'Anatomie Pathologique, Centre Hospitalier

2015 The Pan African medical journal

51. Genetics of Skin Cancer

, these cancers are likely derived from epidermal stem cells associated with the hair follicle.[ ] A variety of tissues, such as lung and uterine cervix, can give rise to SCC, and this cancer has somewhat differing behavior depending on its source. Even in cancer derived from the skin, SCC from 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 (...) 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. The subcutis can be affected by inflammatory conditions such as panniculitis and malignancies such as liposarcoma.[ ] These compartments give rise to their own malignancies but are also the region

2012 PDQ - NCI's Comprehensive Cancer Database

53. Sarcoma, Childhood Soft Tissue

to as nonrhabdomyosarcomatous soft tissue sarcomas and account for approximately 3% of all childhood tumors.[ ] This heterogeneous group of tumors includes the following neoplasms:[ ] Connective tissue (e.g., desmoid-type fibromatosis). Peripheral nervous system (e.g., malignant 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 (...) 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 tissue sarcoma in children aged 0 to 14 years and accounts for 50% of tumors in this age group.[ ] (Refer to the PDQ summary on for more information.) In pediatrics, the remaining soft tissue sarcomas are commonly referred

2012 PDQ - NCI's Comprehensive Cancer Database

54. Renal angiomyolipoma: a radiological classification and update on recent developments in diagnosis and management Full Text available with Trip Pro

features, and clinical behavior. The importance of understanding this group of neoplasms is emphasized by the fact that many types of angiomyolipoma contain little to no fat, and despite being benign, sometimes escape a pre-operative diagnosis. These types of angiomyolipomas can all be considered when encountering a renal mass that is both hyperattenuating relative to renal parenchyma on unenhanced CT and T2-hypointense, features that reflect their predominant smooth muscle component. We review recent (...) Renal angiomyolipoma: a radiological classification and update on recent developments in diagnosis and management Angiomyolipoma is the most common benign solid renal neoplasm observed in clinical practice. Once thought to be a hamartoma and almost always diagnosed by the imaged-based detection of fat, angiomyolipomas are now known to consist of a heterogeneous group of neoplasms. Although all are considered perivascular epithelioid cell tumors, many display different pathology, imaging

2014 Abdominal Imaging

55. Benign Vulvar Lesions (Follow-up)

. 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

56. Urologic Imaging Without X-rays: Ultrasound, MRI, and Nuclear Medicine (Diagnosis)

some or all of the TZ. Such calcifications have been correlated with a history of prostatitis but are often seen in healthy young men with no history of prostatic inflammation. The preprostatic sphincter, composed of the bladder neck and periurethral tissue, is located between the 2 lobes of the TZ. This structure demonstrates the dramatic hypoechogenicity typical of muscle due to the concentration of smooth muscle fibers. These muscles appear as an inverted Y, which has been termed the Eiffel (...) , this variant gives the appearance of bilateral basilar hypoechogenicity. In 12% of patients, abnormal penetration of the seminal vesicles and vas deferens was present at the rectal surface of the prostate, characterized by a lack of CZ tissue posterior to these structures, which sonographically appeared as midline hypoechogenicity at the base of the prostate. In 6% of cases, abnormally large (>2 mm in diameter) smooth muscle bundles were present within the ejaculatory duct sheath, which also produced

2014 eMedicine.com

57. Peutz-Jeghers Syndrome (Diagnosis)

syndrome are typical hamartomas. Their histology is characterized by extensive smooth muscle arborization throughout the polyp. [ , ] This may give the lesion the appearance of pseudoinvasion, because some of the epithelial cells, usually from benign glands, are surrounded by the smooth muscle (the lack of dysplasia in the polyps help to differentiate pseudoinvasion from malignancy). [ , ] Cancer develops in the gastrointestinal tract of patients with Peutz-Jeghers syndrome with a higher frequency than (...) of a “clean-sweep” strategy for disease surveillance or because they are resulting in symptoms, including bleeding. Previous Next: Histologic Findings Polyps in Peutz-Jeghers syndrome (PJS) can reliably be differentiated from other types of polyps only by histopathology, [ ] and surgical pathologists can have a critical role in recommending genetic counseling and surveillance of the patient and/or family members when the diagnosis is suspected. [ ] Smooth-muscle hyperplasia, with an elongated, arborized

2014 eMedicine.com

58. Nonneoplastic Epithelial Disorders of the Vulva (Diagnosis)

, and Skene ducts. 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 (...) : , lentiginosis, and benign 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

2014 eMedicine.com

59. Nevi, Melanocytic (Diagnosis)

such as hair follicles, within the subcutis, and sometimes within skeletal muscle, smooth muscle bundles, nerves, or sebaceous glands. Errant embryological migration is also believed to be the source of melanocytic nevus cell "rests," which can be observed in the capsules of lymph nodes. Occasionally, rests of melanocytes can also be found in the subcapsular space or within lymph node trabecula. The importance of melanocytic nevus rests is that they can sometimes be mistaken for metastatic deposits because (...) Author: Timothy McCalmont, MD; Chief Editor: Dirk M Elston, MD Share Email Print Feedback Close Sections Sections Melanocytic Nevi Overview Background Melanocytic nevi are benign neoplasms or hamartomas composed of melanocytes, the pigment-producing cells that constitutively colonize the epidermis. Melanocytes are derived from the neural crest and migrate during embryogenesis to selected ectodermal sites (primarily the skin and the CNS), but also to the eyes and the ears. Ectopic melanocytes have

2014 eMedicine.com

60. Sebaceous Adenoma (Diagnosis)

differentiation. The following terms are recognized within this category: sebaceous adenoma, sebaceous epithelioma (sebaceoma), sebaceous carcinoma, basal cell carcinoma with sebaceous differentiation, sebocrine adenoma, and sebomatricoma. Skin lesions containing benign sebaceous gland proliferation (eg, sebaceous hyperplasia), congenital hamartomas (eg, nevus sebaceus), and lesions with ectopic sebaceous structures (eg, Fordyce spot, Montgomery tubercles) are generally not considered to be true sebaceous (...) be identified that synthesize melanin-containing organelles (melanin granules) in the ducts and acini of human sebaceous glands. [ ] As one of the skin's adnexal structures, the sebaceous gland is intimately associated with hair (pilar) and arrector pili muscle. Because of the intimate association of sebaceous glands with hair and apocrine ducts, many sebaceous neoplasms show complex histopathologic features with various elements of pilar and sweat gland differentiation. Pilar and sebaceous neoplasms share

2014 eMedicine.com

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