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PubMed2006

The importance of functional testing in the genetic assessment of Muir-Torre syndrome, a clinical subphenotype of HNPCC.

A majority of families with hereditary nonpolyposis colorectal cancer (HNPCC) are attributable to germline mutations in three DNA mismatch repair (MMR) genes, MLH1, MSH2 and MSH6. However, the clinical phenotype appears to reflect a complex interplay between the predisposing mutation and putative constitutional and somatic modifiers. Certain MMR gene mutations predispose to combined occurrence of cutaneous sebaceous gland neoplasms and visceral malignancies, which is known as Muir-Torre syndrome (MTS) and regarded as a phenotypic variant of HNPCC. The sebaceous tumors associated with MTS appear in many patients before visceral malignancies providing important predictability of HNPCC-related integral cancers in mutation carriers. Since most sebaceous skin tumors are, however, sporadic, the contribution of non-truncating mutations found in skin cancer patients is difficult to interpret and genetic assessment of MTS requires a functional test. Here, we studied the repair efficiency of the two MSH2 missense mutations, L187P and C697F, found in HNPCC families including a few mutation carriers with sebaceous skin tumors. Both mutations were completely deficient in an MMR assay, which together with tumor findings suggested their predisposing role in both internal and skin malignancies in the families.

Colorectal Neoplasms, Hereditary Nonpolyposis↗
PubMed1986

Sebaceous carcinoma of the face following irradiation.

Malignant neoplasms of sebaceous glands are uncommon but generally occur about the head and neck, where these glands are concentrated. The sites of most frequent occurrence are the ocular adnexae, where sebaceous carcinoma presents as an infiltrating malignancy with a poor prognosis. Cutaneous sebaceous carcinoma is rare at other sites but has a better prognosis. We have located eight cases in the literature of sebaceous carcinoma arising in a previously irradiated field: five in the ocular adnexae and three elsewhere on the face. We here report a fourth case of recurrent sebaceous carcinoma with metastases that occurred on the face of an 82-year-old woman who had received radiation to the area for cosmetic epilation 35 years previously.

Adenocarcinoma↗
PubMed1995

Sebaceous carcinoma.

Sebaceous carcinoma is a rare, aggressive, malignant tumor derived from the adnexal epithelium of sebaceous glands. It may arise in ocular or extraocular sites and exhibits such a variety of histologic growth patterns and diverse clinical presentations that the diagnosis is often delayed for months to years. We discuss incidence as well as clinical, histologic, diagnostic, prognostic, and management issues of this aggressive neoplasm.

Adenocarcinoma, Sebaceous↗
PubMed1989

Sebaceous carcinoma of the parotid gland. An immunohistochemical and ultrastructural study.

Sebaceous carcinoma of salivary gland origin is extremely rare and, because of its rarity, the clinicopathological characteristics and the histogenesis are not fully understood. We present a case of sebaceous carcinoma of the parotid gland which brings the total number of reported cases to 22. The tumor showed epithelial cell nests which were mainly composed of sebaceous cells with marked cellular atypia. In most of the nests, glandular spaces lined by ductal epithelium were present. Scattered mucous cells and flattened eosinophilic cells at the periphery of the nests were also seen. Ultrastructural and immunohistochemical observations of the tumour revealed coexistence of sebaceous and glandular differentiations in some tumour cells. Tumour cells with lipid granules often participated in the formation of glandular structures or exhibited intracytoplasmic lumina, and immunohistochemical localization of lactoferrin and secretory component, the functional markers of ductal epithelium of salivary gland, was demonstrated not only in duct-forming tumour cells but also in many sebaceous tumour cells. It seems likely that sebaceous carcinoma originates from pluripotential duct cells which can differentiate into sebaceous, ductal and mucous cells.

Adenocarcinoma↗
PubMed1978

Bioassay of cupferron for possible carcinogenicity (CAS No. 135-20-6).

A bioassay of cupferron for possible carcinogenicity was conducted using Fischer 344 rats and B6C3F1 mice. Cupferron was administered in the feed, at either of two concentrations, to groups of 49 or 50 male and 50 female animals of each species. The time-weighted average high and low dietary concentrations of cupferron were, respectively, 0.30 and 0.15 percent for male and female rats, and 0.4 and 0.2 percent for male and female mice. After a 78-week period of compound administration, observation of the rats continued for an additional period of up to 28 weeks and observation of the mice continued for an additional period of up to 18 weeks. For each species, 50 animals of each sex were placed on test as controls and fed only the basal diet. Among both sexes of rats and mice there was a significant positive association between the dose of cupferron administered and mortality; however, in all groups of animals sufficient numbers survived long enough to establish the carcinogenicity of this compound. There were significant positive associations between the concentrations of cupferron administered to male and female rats and the incidences of: squamous-cell carcinomas of the forestomach, hepatocellular carcinomas and neoplastic nodules, and hemangiosarcomas. When a binomial distribution and a spontaneous incidence rate corresponding to the appropriate historical control incidence were assumed, the incidences of auditory sebaceous gland neoplasms in female rats and female mice were significant. There were significant positive associations between the concentrations administered and the incidences of hepatocellular carcinomas in female mice, the incidences of hemangiosarcomas in both sexes of mice, and the incidence of Harderian gland adenomas in both sexes of mice. Under the conditions of this bioassay cupferron was carcinogenic in Fischer 344 rats, causing hemangiosarcomas, hepatocellular carcinomas, and squamous-cell carcinomas of the forestomach in males and females as well as carcinomas of the auditory sebaceous gland in females. The chemical was also carcinogenic in B6C3F1 mice, causing hemangiosarcomas in males and hepatocellular carcinomas, carcinomas of the auditory sebaceous gland, a combination of hemangiosarcomas and hemangiomas, and adenomas of the Harderian gland in females.

Journal Article↗
PubMed1982

[Sebaceous trichofolliculoma].

A 20 year old male patient is reported who has, since the childhood, two adjacent, slightly depressed, orifices on the midline of the dorsum of the nose through which a few hairs came out. The histology of the excised lesion revealed a central cystic cavity, open to the surface, in which converge multiple mature hair follicles, either of vellus and terminal types. There are conspicuous, well developed sebaceous glands. No secondary or tertiary hair follicles could be found and hair shafts are often thick, long and pigmented. The differential diagnosis is discussed, particularly with the trichofolliculoma and the fistula of the dorsum of the nose. The lesion shares most of the features of this last lesion and probably is a superficial variety of fistula of the dorsum of the nose. The lesion is quite different from the classical type of trichofolliculoma and the name sebaceous trichofolliculoma is considered to be an inadequate one.

Adult↗
PubMed1977

Wax ester synthesis in the mouse preputial gland tumour.

The microsomal fraction from the mouse preputial gland tumour contains an acyltransferase which catalyzes the synthesis of wax esters. The enzyme is inhibited by moderate concentrations of free fatty acids (40 muM or more) but the inhibition is relieved by the addition of bovine serum albumin. The specific activity of the enzyme increases markedly between the 20th and 30th days of tumor growth. A number of other lipid synthesizing enzymes show similar trends for specific activity as related to tumour age.

Acyltransferases↗
PubMedDate not supplied

Needle aspiration cytology of sebaceous carcinoma.

Sebaceous carcinoma is uncommon and usually presents in the ocular adnexae as cancer of the meibomian glands. That cutaneous sebaceous carcinoma generally occurs along with other epidermal elements has caused some nosologic confusion; tumors in which sebaceous elements predominate are encountered rarely. They are aggressive tumors in any location but particularly around the ocular adnexae, necessitating wide excision and careful follow-up to detect metastases. At our institution two such tumors were excised recently, one from the eyelid and one from previously irradiated skin. Subsequent metastases were detected without difficulty on needle aspiration cytology, which produced characteristic epithelial cells with bubbly , lipid-rich cytoplasm. The cytologic, histologic and ultrastructural appearances of these tumors that are suited to diagnosis on fine needle aspirates are reported.

Adenocarcinoma↗
PubMed1977

Culture of cloned cells from the mouse preputial gland tumor ESR 586.

Cells from the mouse preputial gland tumor ESR 586 have been cultured and cloned. Trypsin-ethylenediaminetetraacetate was used to obtain single cells. The cells are grown in a modified CMRL-1415 medium supplemented with 10% fetal calf serum. Clones tend to fall into two classes: Class 1, those that undergo morphological differentiation in liquid medium to form round bodies filled with lipid vaculoes; and Class 2, those that do not differentiate. Preliminary studies on the control of differentiation in Class 1 clones suggest that a minimum cell density is required before differentiation takes place. Analysis of the lipids from differentiating and nondifferentiating clones reveals the presence of sebaceous-type lipids in differentiating clones only. No requirement for testosterone was found for these cells.

Animals↗
PubMed1986

[Pathological studies on epidermic and appendage tumors and tumor-like lesions--analysis of 3,425 cases].

This paper analyses 3,425 cases of epidermic and appendage tumors and tumor-like lesions. There were 2,137 cases (62.4%) of benign tumors and tumor-like lesions and 1,288 (37.6%) of malignant tumors. The incidence of benign tumors and tumor-like lesions in the epidermis (1,742 cases, 81.5%) was higher than that in the appendage (395 cases, 18.5%). The most common malignant tumor was squamous cell carcinoma (958, 74.4%) and the basal cell carcinoma (296, 23.0%) ranked second, while the malignant tumor in the appendage was very rare (34, 2.6%). The ratio of squamous cell carcinoma and basal cell carcinoma was 3.2:1. For the sex difference in general, male was slightly common than the female (1.5:1 in the benign tumors and 2.1:1 in the malignant), but in basal cell carcinoma and benign tumors of the hair follicle and sweat glands, there were more female than the male. For the age difference, the incidence of the benign tumor and tumor-like lesion were higher in the adolescence and adult, while the malignant tumors, were more prevalent in the adult and the elderly. The basal cell carcinoma was frequently seen in the old ages. For the site difference, most cases of skin tumors were located in the head and neck, but more than half of squamous cell carcinomas occurred in the penis.

Adolescent↗
PubMed2005

Neoadjuvant chemotherapy in the management of sebaceous gland carcinoma of the eyelid with regional lymph node metastasis.

A 55-year-old Asian Indian woman who had recurrent sebaceous gland carcinoma of the left lower eyelid with orbital extension and regional lymph node metastasis was treated with neoadjuvant chemotherapy, using a combination of carboplatin and 5-fluorouracil. Eyelid-sparing orbital exenteration was performed after 3 cycles of chemotherapy, followed by radiotherapy to the regional lymph nodes. Subsequently, 3 cycles of adjuvant chemotherapy were administered. Significant eyelid and orbital tumor volume reduction was achieved with neoadjuvant chemotherapy, making eyelid-sparing orbital exenteration possible. Chemotherapy also spared the patient from radical neck dissection. The patient had limited morbidity and was free of local, regional, and systemic disease at 26 months of follow-up.

Adenocarcinoma, Sebaceous↗
PubMed1985

Adnexal carcinomas of the skin. II. Extraocular sebaceous carcinomas.

Extraocular sebaceous carcinomas are uncommonly seen neoplasms, and have been confused in the past with basal cell carcinomas showing sebaceous differentiation. In contrast to the latter tumors, however, sebaceous carcinomas have a distinct risk of aggressive behavior. This study presents clinicopathologic data on five cases of sebaceous carcinoma arising in cutaneous locations outside of the ocular adnexae. Four of five patients were men, and the average age at diagnosis was 63 years. Three tumors occurred on the face, one arose in the skin of the neck, and another occurred on the penis, an anatomic site that is extremely rare for sebaceous carcinoma. Three tumors metastasized, and two patients died of tumor or with residual tumor growth. In light of this behavior, the premise that extraocular sebaceous carcinomas rarely spread to distant sites may need reexamination.

Adenocarcinoma↗
PubMed1991

Fine-needle aspiration biopsy of orbital and periorbital masses.

We present the cytologic findings of 18 fine-needle aspirates performed on orbital and periorbital masses. An accurate specific diagnosis was confirmed by histology in ten cases. In eight cases the diagnostic evaluation and/or therapeutic regimen was significantly altered by the results of the fine-needle biopsy. In three cases the cytologic diagnoses obviated the need for open biopsy. Two specimens were insufficient for diagnosis. No false positive diagnosis of malignancy occurred. Electron microscopy was useful in three cases to confirm the correct cytologic diagnosis. Immunocytochemistry helped to define a lymphoid proliferation as monoclonal. The cytologic findings from an unusual case of sebaceous carcinoma are presented.

Adenocarcinoma↗
PubMed1991

Intraoral sebaceous carcinoma.

An instance of sebaceous carcinoma that presented as a mass in the left buccal mucosa is described. This is the first known report in the English-language literature of a sebaceous carcinoma presenting as an intraoral tumor. The light and electron microscopic findings are described. The histologic differential diagnosis, clinical behavior, and appropriate therapy are discussed. Previously reported intraoral sebaceous neoplasms and sebaceous carcinomas of the parotid are reviewed.

Adenocarcinoma↗
PubMed1992

Histopathology of malignant salivary gland tumours.

This report is based upon the Salivary Gland Register in Hamburg and on the second revised edition of the WHO Histological Typing of Salivary Gland Tumours. The group of malignant salivary gland tumours contains carcinomas, malignant non-epithelial tumours, malignant lymphomas and secondary tumours. The various carcinomas are classified in a continuous separate listing because the different types are distinguished not only by histopathology, but also by differences in prognosis and treatment. The term "tumour" is replaced by "carcinoma" in two entities: acinic cell carcinoma and mucoepidermoid carcinoma. New entities are: polymorphous low-grade adenocarcinoma, basal cell adenocarcinoma, salivary duct carcinoma and malignant myoepithelioma. Carcinoma in pleomorphic adenoma can be distinguished as non-invasive and invasive carcinoma, and carcinosarcoma. Malignant non-epithelial tumours are mostly malignant fibrous histiocytoma, malignant schwannoma and rhabdomyosarcoma. The large majority of malignant lymphomas are non-Hodgkin-lymphomas with high differentiation. Many lymphomas are associated with chronic immunosialadenitis (Sjögren's syndrome). Secondary tumours are mostly metastases from primary squamous cell carcinomas or from melanomas of the skin (head and neck area). Haematogeneous metastases are very rare (mainly from lung, kidney or breast).

Adenocarcinoma↗
PubMed1976

Multiple sebaceous adenomas and internal malignant disease: a case report with chromosomal analysis.

A case of multiple sebaceous adenomas associated with internal malignant disease is further documented. Thirteen similar cases reported in the literature are also reviewed. We support the thesis that this association represents a unique syndrome. It can be seen in both sexes and usually is manifested during the fifth and sixth decades. A family history of malignant disease was revealed among seven of the nine cases mentioned. Of the other skin lesions, keratoacanthoma is also frequently seen. The internal malignant tumors most commonly afflict the gastrointestinal tract, especially the large intestine. With proper treatment the internal tumors appear to be of a low degree of malignancy. However, these patients have a propensity to develop muliple visceral tumors and require periodic follow-up. Some patients develop skin tumors prior to the discovery of internal malignant disease, an event that offers some prognostic value. Chromosomal study of the cultured blood lymphocytes by the Giemsa banding technique failed to reveal any abnormality.

Adenocarcinoma↗