1/10. Desmoplastic neuroepithelial tumor of infancy in the nevus sebaceus syndrome: report of a unique constellation and review of the literature.The nevus sebaceus syndrome (NSS) is a neurocutaneous disorder characterized by unilateral hyperplasia of skin appendages and skeletal hemihypertrophy, hemimegalencephaly, or hemiatrophy along with disabling seizures. Despite the proneness of the dermal stigmata to eventually undergo neoplastic transformation, the malformative lesions of the central nervous system rarely evolve into frank tumors. We present the case of a 10-year-old girl with left-sided sebaceus nevi, ipsilateral enlargement of the skull, and a desmoplastic neuroepithelial tumor (DNET) in the right fronto-parietal area of the brain. The tumor was removed by surgery. Histologically, it corresponded to a mitotically active small-cell anaplastic astrocytoma with genuine desmoplasia. Investigative methods included immunohistochemical positivity for glial fibrillary acidic protein, lack of expression of neuronal markers, and ultrastructural documentation of sheaths of basal lamina and collagen around tumor cells. A survey of the literature of brain tumors associated with NSS revealed two cases of histologically verified pilocytic astrocytomas, and one each of a choroid plexus papilloma, a mixed glioma, and a meningioma, as well as a subependymal giant cell astrocytoma--the latter possibly in an overlap syndrome of NSS and tuberous sclerosis. We hypothesize that the tumor described herein, one involving both atypical differentiation and enhanced growth potential, is paradigmatic of neuropathological events to be expected in the NSS.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
2/10. Plate-like sebaceous hyperplasia overlying dermatofibroma.Epidermal hyperplasia, sometimes with primitive hair follicle-like differentiation, is a characteristic finding overlying dermatofibroma. We here report plate-like sebaceous hyperplasia overlying a dermatofibroma. Such changes are interpreted as a result of inductive epithelial effect by the dermatofibroma on the overlying epidermis.- - - - - - - - - - ranking = 6keywords = hyperplasia (Clic here for more details about this article) |
3/10. muir-torre syndrome: role of the dermatopathologist in diagnosis.muir-torre syndrome (MTS) is an autosomal dominantly inherited disorder characterized by sebaceous lesions and visceral malignancies. The defect is thought to be the result of a mutation in mismatch repair genes and associated with microsatellite instability. Two cases whose diagnoses were suggested first by the dermatopathologist are discussed. The first is a 47-year-old white man who over the past 6 years developed multiple sebaceous lesions. Due to the number of sebaceous lesions and their morphology, the possible diagnosis of MTS was suggested by the dermatopathologist. Subsequently, a lesion in the right colon was found during colonoscopy that proved to be a poorly differentiated cecal adenocarcinoma. A pedigree analysis revealed other family members afflicted with multiple malignancies. genetic testing of the colonic adenocarcinoma showed microsatellite instability. The second patient is a 50-year-old white man who underwent biopsy of a skin lesion that showed features of both a sebaceous hyperplasia and sebaceous adenoma. Because of the mixed, unusual features of the lesion, the dermatopathologist suggested the diagnosis of MTS. It was later confirmed that the patient had a history of malignancies of the colon and kidney as well as a family history significant for multiple malignant neoplasms. These cases demonstrate the important role of the dermatopathologist in alerting the clinician to the possibility of muir-torre syndrome when the diagnosis of a sebaceous neoplasm is made, especially when unusual histologic features are observed.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
4/10. An illustrative case of muir-torre syndrome: contribution of immunohistochemical analysis in identifying indicator sebaceous lesions.BACKGROUND: muir-torre syndrome (MTS) is an autosomal dominant genodermatosis characterized by the association of at least 1 cutaneous sebaceous tumor and 1 internal malignancy, often arising in the gastrointestinal tract. It is secondary to germline mutations in dna mismatch repair genes, mainly MLH-1 and MSH-2. OBSERVATIONS: We report the case of a 54-year-old man with a 2-year history of skin-colored papules clinically reminiscent of large sebaceous hyperplasias on the nose and back, but histologically diagnosed as sebaceous adenomas and epitheliomas. His family history was positive for colon cancer in the mother and 2 brothers. A colonoscopy done during the hospitalization revealed 2 sessile polyps in the left colon, both showing a low-grade dysplasia on the biopsy specimen. Immunohistochemical staining performed on the cutaneous and colic biopsy specimens revealed a lack of expression of MSH-2 and MSH-6. genetic testing revealed microsatellite instability in the colon and cutaneous tumors. CONCLUSION: The immunohistochemical testing for MSH-2, MSH-6, and MLH-1 is useful for rapid identification of an underlying mismatch repair defect and early diagnosis of MTS.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
5/10. Mucous cell hyperplasia in an odontogenic cyst from a patient with muir-torre syndrome.Mucous cell proliferation in a periapical radicular cyst from a patient with a family history of colonic malignancies and multiple sebaceous neoplasms of the skin, so-called muir-torre syndrome, is reported. Such goblet cell hyperplasia has not been previously reported to be associated with any known syndrome. We believe the finding of mucous cell hyperplasia in an odontogenic cyst may not simply be coincidental, but should raise suspicion of paraneoplastic potential and warrant further evaluation for possible occult neoplastic disease.- - - - - - - - - - ranking = 6keywords = hyperplasia (Clic here for more details about this article) |
6/10. Sebaceous adenoma of the oral cavity.The solitary sebaceous adenoma is uncommon both on the skin and in the oral mucosa. According to certain criteria, two cases have shown the features of this neoplasm. The differential diagnosis of this lesion should include Fordyce's granules and also adenomatoid sebaceous hyperplasia.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
7/10. Sebaceous trichofolliculoma.Sebaceous trichofolliculoma, a hitherto unrecognized variant of trichofolliculoma, is a clinically and histologically easily recognizable tumor of higher than usual differentiation. Clinical features, histology, differential diagnosis and therapy are presented, using as a basis three patients (all male, ages 21, 36, and 51). Clinically, sebaceous trichofolliculomas occur in sebaceous follicle-rich areas (in all three cases here, the nose). They are skin-colored, centrally depressed lesions of 4--12 mm in diameter. Lateral borders and depth of hamartoma cannot be sharply delineated. Thick terminal hairs, vellus-hairs and occasional trichoids protrude from a fistula-like opening. No connecting tract to paranasal sinus and no bony destruction were found using radiopaque techniques. Histologically, the sebaceous trichofolliculoma has a characteristic pattern. It is a hamartomatous growth, with a rather large central cavity or sinus and with secondary branches, all keratinizing. The cavity is filled with loose epidermoid corneocytes, trichilemmal-like cellular debris, and hairs. Many strikingly large sebaceous follicles (unlike the picture in sebaceous ducts, follicular infundibula, and hair-follicle units. Terminal hair-follicles and vellus hair-follicles are found in various stages of the hair growth-cycle. There is usually no inflammation. Differential clinical diagnosis includes dermoid cysts and congenital fistulas (which may have widespread cords, sacs or ducts to the paranasal structures). Histological differentiation should include trichofolliculoma, pilar sheath acanthoma, dilated pore, circumscribed sebaceous gland hyperplasia, and steatocystoma multiplex. Therapy consists of surgical excision.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
8/10. Sebaceous gland adenoma of the palpebral conjunctiva in a patient with Muir-Torre-syndrome: a case report.BACKGROUND: muir-torre syndrome (MTS) is a hereditary genodermatosis associated with internal malignancies. methods: We report the histological case of an atypical sebaceous gland adenoma of the palpebral conjunctiva in a 42-year-old female patient. RESULTS: The combination of this adenoma with atypical adenomatous hyperplasia of the endometrium and a strong family history of gastrointestinal and urogenital cancers led to the diagnosis of MTS. CONCLUSION: We present a case where the diagnosis of an atypical solitary sebaceous gland adenoma of the palpebral conjunctiva combined with a conspicuous personal and family history led to the diagnosis of MTS.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
9/10. muir-torre syndrome: clinical features and molecular genetic analysis.We report a 62-year-old man with rectal cancer, two keratoacanthomas and multiple sebaceous adenomas, epitheliomas and sebaceous hyperplasia. His brother and father died from colorectal cancer. A subgroup of patients with the muir-torre syndrome (MTS) is allelic to the cancer family syndrome. This genetic disorder is caused by an autosomal dominant inherited germline mutation in one of the dna mismatch repair genes. It is thought that a somatic mutation of the other allele leads to a genomic instability responsible for tumorigenesis. In the patient presented here the instability was detected in two characteristic skin lesions; sebaceous adenoma and epithelioma. The search for a causal germline mutation revealed a frameshift mutation in the mismatch repair gene hMSH2 leading to a truncated protein. A presymptomatic molecular diagnosis can be offered to the children of the patient.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
10/10. muir-torre syndrome.muir-torre syndrome is a rare autosomal dominant disorder characterized by unusual sebaceous neoplasms and visceral malignancy. Excluding sebaceous hyperplasia and sebaceous nevus of Jadassohn, sebaceous neoplasms occur so rarely that the presence of a sebaceous neoplasm mandates consideration of muir-torre syndrome. Sebaceous neoplasms precede or are synchronous with visceral malignancies in up to 40% or more of patients with muir-torre syndrome, and the presence of such a lesion warrants an evaluation for visceral malignancy.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
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