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The Muir-Torre syndrome: a disease of sebaceous and colonic neoplasms.

The Muir-Torre syndrome of sebaceous neoplasms of the skin, with or without keratoacanthomas, and multiple low-grade visceral malignancies with prolonged survival is a rare disorder. Colonic polyps are frequently present, and the syndrome appears to be familial. We report 2 unrelated patients with the Muir-Torre syndrome. Each case exhibited sebaceous adenomas. Gastrointestinal findings included colonic adenocarcinomas and a tubulovillous adenoma. Although an unusual disease, the Muir-Torre syndrome requires recognition because these patients are at risk for multiple primary malignancies and may have family members also at risk.

Adenocarcinoma↗

Superficial epithelioma with sebaceous differentiation.

In 1980, Rothko, Farmer, and Zeligman first described the disease entity "Superficial epithelioma with sebaceous differentiation" as a histologically distinct, rare, benign lesion. We report here the first Japanese case fitting this description. The patient is a 38-year-old Japanese woman, who had a small, yellow, flat-topped, well-defined plaque on her right upper eyelid. Histologically, the lesion was a multilobular, superficial, dermal tumor with numerous broad attachments to the overlying epidermis. The overall configuration was a plate-like proliferation of tumor cells with well-defined borders. The tumor cells were basically uniform basaloid cells with single or clustered sebocytes in the upper part of the tumor and multiple well differentiated sebaceous lobules in the middle and lower parts of the tumor. Multiple cystic spaces were formed in the lobules. Differential diagnosis of several tumors with sebaceous differentiation is discussed.

Adult↗

[Fordyce's spots: disease, heterotopia or adenoma? Histological and ultrastructural study].

Fordyce spots are sebaceous glands without hair follicles, most often located under the epithelium of the cheek and free lip border. These glands, present in 80 to 95% of adults, according to the localization, cannot be considered as ectopic. Along the same lines, these spots are neither a disease, a lesion or an adenoma. These glands are acino-tubular and distributed in clusters. They are connected to the covering epithelium by a duct with a keratinizing stratified epithelium. By analog to what has been done in the skin, the acinous cells can be artificially classified in three groups: basal germinative cells partially differentiated intermediary cells and totally differentiated preductal cells. With PAS-Mac Manus, the peripheral cells are characterized by a reduced cytoplasm, the scarcity of organelles, as well as the presence of tonofilaments and desmosomes. The intermediary cells have a low nucleus-cytoplasm ratio. While keeping their epidermal characters, they become rich in lipid droplets of variable sizes and are closely related to numerous mitochondria. The cytoplasm of completely differentiated cells, with unrecognizable organelles, are invaded by large lipid globules. In the periacinous connective tissues, fenestrated capillaries are observed.

Adenoma↗

Superficial epithelioma with sebaceous differentiation.

Five cases of superficial epithelioma with sebaceous differentiation (SESD) are reported. They occurred as solitary papules on the face of 5 patients, aged 57 to 72. The tumor is characterized by a superficial platelike proliferation of basaloid to squamoid cells with broad attachments to the overlying epidermis. Clusters of mature sebaceous cells are present within the tumors. None of the tumors have recurred or spread following simple excision. SESD is a non-aggressive tumor of uncertain histogenesis with a tendency toward sebaceous differentiation.

Aged↗

Muir-Torre syndrome: a case report and review of the literature.

The presence of sebaceous skin tumors with visceral neoplasms is known as Muir-Torre syndrome (MTS). It is a phenotypic subset of hereditary nonpolyposis colorectal cancer and is caused by mutations in genes encoding for mismatch repair (MMR) proteins. The presence of a sebaceous gland neoplasm should raise concern for a potential diagnosis of MTS. Immunohistochemical analysis of the sebaceous skin tumors can be helpful in screening for an MMR defect and preselecting patients who are at increased risk of a visceral malignancy. We report a case of MTS and show immunohistochemical analysis of the sebaceous neoplasm. We also review the literature on MTS and the effectiveness of immunohistochemical analysis in screening patients at risk for MTS.

Colorectal Neoplasms, Hereditary Nonpolyposis↗

Spontaneous skin neoplasms in aged Sprague-Dawley rats.

A total of 93 tumors of the epidermis, its appendages, and dermis were observed in 1,433 (717 males, 716 females) rats employed in oncogenicity studies over a 2-yr period. Mammary gland neoplasms will be reported separately. Fifty-seven (61.3%) were epithelial with 49 in males and 8 in females. Keratoacanthoma was the most frequent epithelial neoplasm in males (22) followed by squamous cell carcinoma (11) and papilloma (5). Sebaceous gland neoplasms seen in males (5) included both adenomas (3) and carcinomas (2). In males, there were also 3 trichoepitheliomas, 1 pilomatricoma, 1 basal cell tumor, and 1 malignant melanoma. Of the 8 epithelial neoplasms in females, there were 3 squamous cell carcinomas, 2 keratoacanthomas, and 1 each basal cell tumor, malignant melanoma, and trichoepithelioma. There were 21 mesenchymal neoplasms in males and 15 in females. The most frequent neoplasm was fibroma (7 males, 8 females) followed by lipoma (7 males, 4 females) and fibrosarcoma (4 males, 3 females). One male had a liposarcoma and 2 males each had hemangioma. The total neoplasm incidence of 70/717 (9.8%) in males and 23/716 (3.2%) in females showed that skin neoplasms were 3 time more common in males than in females. Epithelial neoplasms of the skin were 6 times more common in males than in females. Males were more than twice as likely to have epithelial rather than mesenchymal skin neoplasms whereas the reverse was seen in females.

Aging↗

Caruncle lesions: the use of histological evaluation in determining a definitive diagnosis.

Caruncle tumors are rare and difficult to definitively diagnose using standard clinical examination techniques. Because some of these lesions may be cancerous, eventually leading to local invasion of tissue, metastasis, and even death, biopsy with histologic evaluation may be indicated. A brief discussion of caruncle tumors and a case report is presented.

Adenocarcinoma↗

[Keratoacanthoma in co-existence with nevus sebaceus (author's transl)].

A keratoacanthoma developed on a nevus sebaceus on the cheek of a 7 years old girl within three weeks. The nevus revealed additional organoid and suborganoid hamartomas. The occurrence of keroacanthoma or even squamous cell epithelioma is a very rare event in nevus sebaceus.

Cell Transformation, Neoplastic↗

Thomsen-Friedenreich and its precursor (Tn) antigen expression in normal skin and in benign cutaneous tumours: a marker for sebaceous differentiation.

The Thomsen-Friedenreich (T) antigen is the core disaccharide of cancer-associated carbohydrates, whose expression allegedly correlates with the prognosis of some carcinomas. We studied the expression of the T antigen and its precursor (Tn) with monoclonal antibodies in formalin-fixed specimens of normal skin and various benign cutaneous tumours and inflammatory lesions (n: 105). In normal skin, both antigens were consistently expressed within the cytoplasm of mature sebocytes and rarely over the luminal surface of secretory sweat gland cells. All (21/21) sebaceous tumours showed strong T/Tn positivity; several (9/16) sweat-gland tumours were also immunoreactive, although more weakly. Pilar (n = 11), non-adnexal tumours (n = 45) and inflammatory lesions (n = 12) were as a rule unreactive. These results suggest that the T antigen is a sensitive marker of sebaceous differentiation that can be used for the study of adnexal skin tumours in routinely processed tissue specimens.

Antigens, Neoplasm↗

Circumscribed sebaceous gland hyperplasia: autoradiographic and histoplanimetric studies.

Circumscribed sebaceous gland hyperplasia (sebaceous hyperplasia) is a common benign facial tumor found in adults and aged. Seventeen biopsies of sebaceous hyperplasia from 10 patients, females and males aged 57-87 years were secured which were injected 45 min or up to 15 days prior with tritiated thymidine, and prepared for autoradiography. Specimens were evaluated by cell counts (labeling index, labeled total cells), and histoplanimetric techniques (sagittal section areas and circumference of sebaceous hyperplasia, of sebocytes, and of sebocyte nuclei). Controls were uninvolved sebaceous follicles from the same subjects, from an age-matched group, and from young male adults. In sebaceous hyperplasia the labeling index of basal cells is low with 5.7% +/- 2.3 vs uninvolved sebaceous follicles from the same subject (8.6% +/- 2.4) and the younger subjects with a value of 10.1% +/- 2.0). Labeled cells are retained in the acini much longer than in young adults (slowed down transit and turnover time). The glands of sebaceous hyperplasia are very large, their sebocytes are small, and many more basal cells are found per unit basement membrane length.

Adenoma↗