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the lesions. [ ] For acne in the lesion, use standard acne treatments. If there is doubt about the diagnosis (eg possible melanoma), urgent referral is appropriate. Prognosis The naevus remains indefinitely. [ ] Becker naevus syndrome [ , , ] This is a Becker's naevus with additional abnormalities of underlying tissues derived from ectoderm. It is a type of epidermal naevus syndrome. There may be underdevelopment or overdevelopment of underlying structures, including: Smoothmusclehamartoma. Hypoplasia (...) , with elongation and fusion of adjacent rete ridges and variable hyperkeratosis. Dermis: hyperplasia of the dermal smoothmuscle and melanophages. Management [ ] No treatment is necessary except for cosmetic reasons; usually reassurance and a diagnosis are enough. For cosmetic treatment of the lesion: or laser treatment may help with hair removal. Laser treatment can sometimes reduce the pigmentation, but may be ineffective or may worsen the lesion. Fractional resurfacing has also been used to lighten
Haemangioleiomyomatous tumour of the lung. A case of haemangioleiomyomatous tumour of the lung, occurring as a peripheral, solitary nodule in an asymptomatic 54-year-old man is presented. The tumour was well-demarcated and microscopically it was characterised by the presence of vascular spaces with endothelial, pericytic, and, predominantly, smoothmuscle proliferation. Islands of cartilage and slit-like spaces lined by bronchial epithelium make this a hamartomatous lesion of a quite (...) distinctive and unusual variety, which does not fit any of the well-recognised patterns of hamartomas previously described. The long-term prognosis after limited excision is considered to be favourable.
Muscle Proliferation Bronchoscopy Female Pneumothorax Tuberous Sclerosis Additional relevant MeSH terms: Layout table for MeSH terms Sclerosis Lung Diseases Pneumothorax Tuberous Sclerosis Lymphangioleiomyomatosis Pathologic Processes Respiratory Tract Diseases Pleural Diseases Hamartoma Neoplasms Neoplasms, Multiple Primary Neoplastic Syndromes, Hereditary Malformations of Cortical Development, Group I Malformations of Cortical Development Nervous System Malformations Nervous System Diseases (...) Responsible Party: National Heart, Lung, and Blood Institute (NHLBI) ClinicalTrials.gov Identifier: Other Study ID Numbers: 950186 95-H-0186 First Posted: November 4, 1999 Last Update Posted: December 19, 2018 Last Verified: November 2, 2018 Layout table for additional information Studies a U.S. FDA-regulated Drug Product: No Studies a U.S. FDA-regulated Device Product: No Keywords provided by National Institutes of Health Clinical Center (CC) ( National Heart, Lung, and Blood Institute (NHLBI) ): Smooth
antigen, claudin-1, CD34, smoothmuscle actin, and KIT was performed. The findings were compared with those in mucosal biopsies of 5 submucosal neurofibromas from NF1 patients. All 26 polyps were sessile, ranging from 1 to 6 mm in size (mean, 2.5 mm). Most arose in the distal colon (15 rectosigmoid, 7 descending, 2 transverse, and 2 ascending), and were incidentally found at screening colonoscopy. After a mean follow-up of 6.5 years (range, 3 mo to 17.5 y), none of the patients developed other neural (...) Mucosal Schwann Cell "Hamartoma": Clinicopathologic Study of 26 Neural Colorectal Polyps Distinct From Neurofibromas and Mucosal Neuromas. Colorectal polyps containing S-100-positive neural proliferations in the lamina propria that lack ganglion cells have been variously referred to as "neuromas" or "neurofibromas." However, these lesions have not been systematically examined, and whether they are associated with type 1 neurofibromatosis (NF1) or other inherited syndromes is unknown. The aim
were predominantly young women. Their pulmonary neoplasm(s) were usually asymptomatic, often multiple, well circumscribed, medium-sized (mean diameter=2.8 cm), and composed almost exclusively of cartilage and bone surrounded by a fibrous pseudocapsule. The cartilage was usually myxoid, less frequently hyaline, and commonly calcified, ossified, or both. They showed no fat, smoothmuscle or entrapped respiratory epithelium, tissues that were common in pulmonary hamartoma (P<0.0001). None (...) Pulmonary Chondroma: A Tumor Associated With Carney Triad and Different From Pulmonary Hamartoma. The Carney triad is the clinical association of gastric stromal sarcomas, pulmonary cartilaginous tumors, and extra-adrenal paragangliomas. The pulmonary tumors are its second commonest component and have been misinterpreted clinically and pathologically as metastases from the gastric tumors and pulmonary cartilaginous hamartomas, respectively. They have not been previously described in detail
protein, glial fibrillary acid protein, smoothmuscle actin, and calponin but lack of staining for cytokeratins. This profile is consistent with an incomplete myoepithelial differentiation of the receptor-expressing mesenchymal cells. In conclusion, sex steroid hormone receptor expression is a nonrandom event in pulmonary hamartoma, and may be related to the development and growth of this tumor. (...) Immunoreactivity for sex steroid hormone receptors in pulmonary hamartomas. Sex steroid hormone [ie, estrogen (ER), progesterone (PgR), and androgen (AR)] receptors have been identified previously in normal salivary glands and, more variably, in salivary gland and salivary gland-type tumors. No data are available, however, on their expression in pulmonary hamartoma, a benign biphasic tumor consisting of reactive epithelial cells and neoplastic fibromyxoid stroma, cartilage and fat, which shares
Anti-EGFR Antibody Efficiently and Specifically Inhibits Human TSC2âˆ’/âˆ’ SmoothMuscle Cell Proliferation. Possible Treatment Options for TSC and LAM Tuberous sclerosis complex (TSC), a tumor syndrome caused by mutations in TSC1 or TSC2 genes, is characterized by the development of hamartomas. We previously isolated, from an angiomyolipoma of a TSC2 patient, a homogenous population of smoothmuscle-like cells (TSC2(-/-) ASM cells) that have a mutation in the TSC2 gene as well as TSC2 loss
Neuromuscular and vascular hamartoma of small bowel presenting as inflammatory bowel disease. We present the case of a rare hamartomatous condition of the small intestine clinically mimicking inflammatory bowel disease. Disorganised fascicles of smoothmuscle derived from both muscularis mucosae and propria, bundles of non-myelinated nerve fibres with groups of abnormal ganglion cells, and haemangiomatous vessels were present within the submucosa of a long segment of small intestine causing
Neuromuscular and vascular hamartoma of small bowel Two examples of a hitherto undescribed hamartomatous condition of the small intestine are presented. They consist of fascicles of smoothmuscle derived from the muscularis mucosae, bundles of unmyelinated nerve fibres with scattered ganglion cells, and haemangiomatous vessels, occurring focally within a segment of the intestine, and causing stenosis.
Center: 1) gynecologic mesenchymal neoplasms with striking smoothmuscle or fibroblastic differentiation that did not meet the clinical or histologic criteria for leiomyosarcoma or other established neoplasms containing smoothmuscle; and 2) metastatic lesions showing ovoid and spindle cell morphology, involving lung, originally diagnosed as low-grade leiomyosarcoma, low-grade smoothmuscle neoplasm, intravenous leiomyomatosis, fibrous hamartoma, and benign metastasizing leiomyoma. We identified 12 (...) Endometrial stromal sarcomas with unusual histologic features: a report of 24 primary and metastatic tumors emphasizing fibroblastic and smoothmuscle differentiation. We report the clinicopathologic features of 24 uterine primary and metastatic endometrial stromal sarcomas with fibromyxoid features (ESS-F) and smoothmuscle differentiation (ESS-SM) (endometrial stromal sarcoma variants). Two groups of tumors were retrieved from the surgical pathology files at Memorial Sloan-Kettering Cancer
Folliculosebaceous smoothmusclehamartoma. Cutaneous hamartomas are a group of heterogenous benign skin conditions demonstrating epithelial and mesenchymal components in varying proportions. Folliculosebaceous (cystic) hamartomas comprise a distinct group of uncommon cutaneous tumor-like malformations. Histologically, these lesions demonstrate varying proportions of adnexal and stromal tissues. We review the literature on folliculosebaceous hamartoma and report a case with smoothmuscle as its
A case of acquired smoothmusclehamartoma of the scrotum. We report a case of acquired smoothmusclehamartoma (ASMH) of tunica dartos, believed to be only the fifth so far reported. A 24-year-old man had a 6-month history of an asymptomatic tight and thickened scrotum. The skin was difficult to pinch. A biopsy specimen from the skin showed increased and proliferated smooth-muscle bundles composed of well-differentiated and uniform spindle cells that showed typical features of acquired smooth (...) musclehamartoma. Interestingly, dilatations of the lymph vessels were noted in the upper dermis above the proliferated smoothmuscles. It has been reported recently that long-standing severe lymphoedema may cause histological features mimicking ASMH. As the present case was not preceded by oedema of the scrotum, we consider this case to be true ASMH.