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Sebaceous carcinoma is a rare and aggressive cutaneous carcinoma. It is believed that this malignancy predominates in the periocular region and occurs more frequently in Asian populations and in women. The objective of the current study was to analyze demographic characteristics and outcomes for patients with this malignancy from a large United States-based population registry.An analysis of the National Cancer Institute's Surveillance, Epidemiology, and End Results database from 1973 through 2004 was performed.Of 1349 patients who were identified, 54% were men, 86.2% were white, and 5.5% were of Asian/Pacific Islander ancestry. The median age at diagnosis was 73 years. The most frequent site of disease was the eyelid (38.7%). The population-matched 5- and 10-year age-matched relative survival rate was 91.9% (standard error [SE], 1.9%) and 79.2% (SE, 3.7%), respectively. Cause of death was attributable to cancer in 31% of patients. Orbital involvement did not predict for worsened survival compared with nonorbital involvement (5-year overall survival, 75.2% vs 68%, respectively; P=.66). The overall population-matched rate of sebaceous carcinoma was highest in whites (2.03 cases per 1000,000; SE, 0.08) versus Asian/Pacific Islanders (1.07 per 1000,000; SE, 0.18; P=.0001) versus blacks (0.48 per 1,000,000; SE, 0.11; P.0001).The current results support the finding of a predominance of men among patients with sebaceous carcinoma, and no difference was observed in the prognosis for orbital and periorbital involvement. This retrospective analysis also corroborated previous case reports of a higher incidence among patients with advanced age and the highest incidence for sites in the eyelid and skin of the face. The results also established that Asian/Pacific Islander ancestry is not a risk factor for developing sebaceous carcinoma.

The association of multiple tumors of the sebaceous glands with primary visceral carcinomas was described for the first time by Torre in 1967. Another 26 cases with similar features have been subsequently reported in the literature. In 12 of these patients isolated or multiple keratoacanthomas and in 8 intestinal polyps were also found. The authors add the report of a personal case. The patient is a 54-year-old man who, during a period of 8 years, has shown multiple sebaceous tumors, 1 keratoacanthoma, and 3 primary adenocarcinomas of the colon. The outline and nosologic position of this pathologic conditions are discussed.

Sebaceous lymphadenoma is a rare, benign neoplasm of the parotid gland. In the 18 years since the initial report seven additional cases have been published. None includes ultrastructural studies or lipid analysis. Electron microscopy shows that the epithelial cells undergo a progressive maturation from basal to the typical lipid-laden mature sebaceous cells as in the cutaneous sebaceous glands. No morphologic differences are found between cutaneous sebaceous glands and the sebaceous glands in this parotid neoplasm. Thin-layer chromatography demonstrates the same type of lipids in the subaceous lymphadenoma as well as in cutaneous sebaceous glands. These findings suggest that the parotid salivary gland may be ectodermally rather than endodermally derived.

Primary sebaceous tumors of salivary glands are extremely rare, although sebaceous glands are commonly present in parotid and submandibular glands. A review of the files of the Armed Forces Institute of Pathology yielded 21 cases of primary salivary gland sebaceous tumors. Five were sebaceous adenomas, 9 sebaceous lymphadenomas, 5 sebaceous carcinomas and 2 sebaceous lymphadenocarcinomas. Seventeen tumors were located in the parotid gland and one each in the submandibular gland, the minor salivary glands of buccal mucosa and in ectopic salivary gland tissue in a periparotid lymph node. Thirteen tumors occurred in males and six in females. The peak incidence for all the sebaceous tumors, occurred in the sixth and seventh decades. The influence of age, sex, race, clinical symptoms, and pathology on survival is reviewed for each tumor group. Our histopathologic observations strongly suggest that sebaceous lymphadenoma and sebaceous lymphadenocarcinoma arise from sebaceous glandular rests in a lymph node in a fashion similar to that of a Warthin tumor.

Three cases of Torre-Muir syndrome are described in which isolated sebaceous carcinomas were associated with internal malignancy. The first was a 66-year-old woman who had a sebaceous carcinoma in association with carcinoma of the uterus, two adenocarcinomas of colon, carcinoma of renal pelvis and bladder, and a squamous cell carcinoma of the skin. The second was a 78-year-old woman who had a sebaceous carcinoma associated with carcinomas of the colon, ovary, and pancreas. The third patient had a sebaceous carcinoma associated with breast cancer. The subject is discussed and the previously reported cases reviewed.

The first reported case of sebaceous differentiation in adenoid cystic carcinoma is presented. The tumor arose in the superficial lobe of the left parotid gland of a 48-year-old white man, had a predominantly solid histologic pattern, and manifested focal sebaceous differentiation in each of three recurrences over a two-year period. Review of the medical literature suggests that sebaceous differentiation is a normal property of some salivary ducts and may be found in any type of salivary gland neoplasm which has a ductal component. The differential diagnosis of sebaceous and clear cell lesions of salivary glands is discussed.

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.

A patient with tuberous sclerosis and biopsy-proven lymphangiomyoma is presented. Lymphangiomyoma is a hamartomatous proliferation of predominantly smooth muscle that involves the lung and lymph nodes. It gives a syndrome of chylothorax, diffuse pulmonary reticular pattern, and frequent spontaneous pneumothorax. That the pulmonary involvement of tuberous sclerosis is practically identical, both grossly and microscopically, to that of lymphangiomyoma and that both syndromes occur in females between 20 to 50 years of age, suggests a common pathologic basis. The concept that lymphangiomyoma is a hamartoma makes this association an attractive one. The presence of chylothorax has been emphasized as a possible differential point between these two entities. However, 2 cases of tuberous sclerosis with chylothorax have now been reported. The presence or absence of chylothorax may depend on whether the thoracic duct is involved.