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The Muri-Torre syndrome is an autosomal-dominant genodermatosis. The criteria of the diagnosis is the occurrence of at least one sebaceous skin tumour and at least one malignant internal tumour. Skin tumours must be considered as very important signs because they may precede visceral malignancies. The authors present the case of a 62 years old man. Three tumours were excised from three different locations of his trunk in the Plastic Surgery Ward of our hospital. The tumours proved to be sebaceous adenoma, sebaceous epithelioma and sebaceous carcinoma by histology. Because of this result, we had conducted a detailed tumour screening which found a non-symptomatic malformation of the proximal colon tract. Histology of the resected colon part revealed adenocarcinoma. With the presentation of this case the authors call attention on the fact, that in case of the diagnosis of a sebaceous skin tumour, careful tumour screening could be lifesaving.

Sebaceous carcinoma in the oral cavity is extremely rare, and we have found only four previously reported cases. We describe a fifth case. A wide surgical excision seems to be the correct treatment and estimation of serum carcinoembryonic antigen (CEA) may be a useful tumour marker in the follow-up of intraoral sebaceous carcinomas.

Sebaceous carcinoma of salivary gland origin is an extremely rare malignancy. It occurs mainly in the parotid gland. This is a case report of a sebaceous carcinoma in a 57-year-old woman who had a lump over the right parotid region for 8-9 months. The tumour was composed of small basaloid cells and large foamy cells. Sebaceous differentiation was evident in some tumour islands. This is the first case reported in the Department of Oral Pathology, Faculty of Dental Sciences, University of Peradeniya.

Sebaceous carcinoma is a rare malignant tumor derived from the epithelium of sebaceous glands. It potentially may develop from any sebaceous gland, but most commonly occurs in the periorbital area. We report a case of sebaceous carcinoma in a 14-year-old girl who was first seen with an asymptomatic 2.5 x 2.3-cm firm, multilobulated cutaneous nodule on the anterior thorax, which had been present for 1 year. She was adopted, and no family history is available. A skin biopsy specimen revealed a poorly differentiated infiltrative carcinoma involving the dermis and subcutaneous fat and focally involving the epidermis. Tumor cells had sebaceous and squamous differentiation. A diagnosis of sebaceous carcinoma was made. She was treated by surgical excision with a 2-cm margin. Further work-up showed no evidence of Muir-Torre syndrome.

Purpose: To evaluate the efficacy of topical mitomycin-C for pagetoid invasion of the conjunctiva by sebaceous gland carcinoma., Design: Prospective noncomparative consecutive interventional case series., Intervention: All patients received topical 0.04% mitomycin-C four times daily for 1 week followed by 1 week off medication. The treatment cycles were repeated until resolution of the conjunctival malignancy was clinically evident., Participants: Four patients with histopathologically proven intraepithelial (pagetoid) invasion of the conjunctiva by sebaceous gland carcinoma were managed with this regimen., Results: The mean patient age was 78 years. Before treatment, the main tumor site included the upper eyelid in two cases and the lower eyelid in two cases. Previous resection of the tumor had been performed elsewhere in three cases over the prior 6 years. At the time of our examination, map biopsies confirmed pagetoid invasion involving 25% to 90% of the conjunctival surface, with bulbar, forniceal, and tarsal conjunctival involvement in all four cases and corneal extension in one case. There was no evidence of deep tumor within the conjunctival stroma or tarsus in any case. After treatment, medication intolerance and early discontinuation occurred in one patient, and continued tumor progression was documented. Of the remaining three patients, chemotherapy was used for a mean of four cycles with complete resolution of the pagetoid invasion, confirmed histopathologically in two cases, and without recurrence in all three cases over 12 months (mean) follow-up. The medication caused moderate temporary local irritation but no serious intraocular or extraocular complications., Conclusions: Preliminary evidence suggests that topical mitomycin-C is effective treatment for pagetoid invasion of the conjunctiva by sebaceous gland carcinoma. Longer follow-up is necessary to assess the duration of tumor control.

Fine-needle aspiration (FNA) plays a pivotal role as the initial diagnostic modality of choice when dealing with salivary gland (SG) lesions. Due to the heterogeneous nature of most SG neoplasms, cytologic interpretation on FNA can be quite challenging. This is particularly valid when dealing with rare tumor types, such as the papillary-cystic variant of acinic-cell carcinoma (ACC-PCV), resulting in a high rate of false-negative cytologic diagnoses. Seven cases of ACC-PCV diagnosed on tissue resection with a prior FNA performed in cytology were studied. Material consisted of Diff-Quik and Papanicolaou stained cytologic smears, as well as hematoxylin-eosin stained cell block and histopathologic sections. In no case did the FNA performed prior to the surgical resection eventuate in a cytopathologic diagnosis of cancer. A retrospective morphologic review of the smears disclosed several characteristics common to all 7 cases, i.e., mostly tightly cohesive fragments of neoplastic epithelium seen as monolayered sheets or with a prominent papillary architecture, high nuclear:cytoplasmic ratio ductal-type epithelium, cystic material and degenerated cellular debris, histiocytes, cells with squamoid and metaplastic oncocytic changes, vacuolated and pigmented histiocyte-like tumor cells, and lack of a predominant single-cell component or naked neoplastic cell nuclei. ACC-PCV represents a rare yet significant cause of false-negative results for cancer on SG aspirations. An erroneous interpretation may occur due to lack of experience in dealing with this tumor type, the rarity of published literature on ACC-PCV, and a predominantly cystic, somewhat variegated appearance of the tumor mimicking other benign and malignant SG lesions., (Copyright 2002 Wiley-Liss, Inc.)

Sebaceous neoplasms arising in dermoid cysts of the ovary are exceedingly rare. We report a well-differentiated sebaceous carcinoma arising in a dermoid cyst in a 39-year-old female, the fourth such case in the literature.

The classification of benign sebaceous neoplasms has been challenged both by the assertion that sebaceous adenomas are really carcinomas and by difficulties in drawing the boundaries between sebaceomas and other lesions. We performed a clinicopathologic study of 30 cases of basaloid neoplasms with sebaceous differentiation, excluding cases of definite sebaceous carcinoma with severe nuclear atypia invading deep within the subcutaneous tissue and those of ocular sebaceous carcinoma. We tried to classify sebaceous neoplasms in six categories with defined histopathologic criteria. All the neoplasms were characterized by aggregations of basaloid cells admixed with sebocytes and sebaceous duct-like structures located in the dermis with or without connection to the epidermis. The categories were 1) sebaceoma (14 cases); 2) trichoblastoma with sebaceous differentiation (3 cases); 3) apocrine poroma with sebaceous differentiation (2 cases); 4) low-grade sebaceous carcinoma (6 cases); 5) sebaceous carcinoma (4 cases); and 6) basal cell carcinoma with sebaceous differentiation (1 case). The sebaceoma was further subclassified as classic type (12 cases) or verruca/seborrheic keratosis type (2 cases). Although most sebaceomas can be distinguished from other lesions, there are problematic cases. We discuss the histopathologic diagnostic problems associated with sebaceoma and also argue in favor of the concept of sebaceous adenoma.

Background: Ocular sebaceous carcinoma (SC) is a rare tumor of the eyelids. Clinically it frequently involves the upper lid in older women. Microscopically it tends to extend far beyond its assessed clinical margins. SC is characterized by a variety of tissue invasion mechanisms. It may spread by direct extension, be multifocal in advanced cases, and develop "skip areas" after trauma. Despite its ability to develop discontinuity, over the past two decades there have been several favorable case reports of SC treated by Mohs micrographic surgery (MMS)., Objective: To illustrate by case report the clinical presentation and management of patients with SC and to document our series of SC patients treated by MMS over the last 14 years., Methods: A retrospective study was conducted of all cases of sebaceous carcinoma involving the ocular adnexa treated at the University of Wisconsin Mohs Surgery Clinic from 1987 to 2001. We also reviewed the accumulated medical literature of SC treated by MMS., Results: In our series, there were nine cases of periocular SC. Five cases originated on the upper lid and four on the lower lid. Five of nine patients (55%) showed epithelial invasion. One patient developed a local recurrence 1.5 years later and was treated by orbital exenteration. This patient has had no evidence of disease for 5 years. Eight of nine patients (88%) had no evidence of local recurrence with a follow-up of 1-14 years. In our literature review we found 40 additional cases of orbital SC treated by MMS. Intraepithelial spread of SC was found in 50% of patients (24 of 48). Six patients developed local recurrence. The overall local cure rate following MMS is 87.8% (43 of 49), with a mean follow-up of 3.1 years. The regional metastatic rate was 8% (4 of 49). There were no deaths reported., Conclusion: We present nine new cases of SC. The age, sex, and site distribution are compatible with other SC cases reported in the literature. We reviewed the medical literature and compiled 49 cases of SC treated by Mohs surgery. Intraepithelial spread was discovered in 50% of the cases. Multifocal disease or discontinuity was present in 6% (3 of 49). Mohs surgery appears to be an effective method for excising the microscopic ramifications of primary SC. When feasible, we recommend in SC cases where intraepithelial pagetoid spread has been observed, that removal of another Mohs layer should be considered in order to provide an additional assurance layer against local tumor recurrence.

Sebaceous neoplasms, including carcinomas, are tumors most often seen around the eye and eyelid. Cases of extraorbital sebaceous tumors associated with skin adnexa are less common. The parotid gland may also be a source for sebaceous neoplasms, and cases associated with minor salivary gland tissue have also been reported. Once beyond the oropharynx, individual cases of pharyngeal and laryngeal sebaceous tumors are reported. The case of a patient with primary sebaceous carcinoma of the bronchus is presented. Our case is that of an endobronchial mass that has histopathologic features of a sebaceous carcinoma, with predominance of areas with morphologically classic sebaceous differentiation, confirmed by oil red O stain for lipid, analyzed by immunohistochemistry and evaluated ultrastructurally. This unique tumor extends the spectrum of extraocular, extracutaneous neoplasms with sebaceous differentiation from existing single reports in the hypopharyx and larynx to now include bronchus.

Purpose: To elucidate the relation between infiltrating cytotoxic T lymphocytes and the expression of MUC1 and sialoglycans in malignant eyelid tumors., Methods: The distribution of MUC1, Maackia amurensis lectin-II (MAL-II)-recognized sialoglycan, and CD8-positive T lymphocytes was examined histochemically in 14 patients with malignant eyelid tumors: three squamous cell carcinomas, six sebaceous gland carcinomas, and five basal cell carcinomas. The density of CD8-positive cells was examined and correlated with MUC1 and sialoglycan expression., Results: MUC1 was identified in squamous cell carcinoma and sebaceous gland carcinoma, but not in basal cell carcinoma. CD8-positive cells were more densely distributed in those squamous cell and sebaceous gland carcinoma cases that were more intense in MUC1 expression and weaker in MAL-II binding. Tumors with a strong expression of both MUC1 and MAL-II-bound sialoglycans showed few CD8-positive cells., Conclusions: MUC1 with few sialoglycans is likely to induce an intense infiltration of CD8-positive cytotoxic T lymphocytes in eyelid cancers.

Purpose: To provide evidence-based clinical recommendations for treatment options and future prospects for the management of common malignant eyelid tumors, including global ratings for the strength of published evidence supporting them., Clinical Relevance: Approximately 5% to 10% of all skin cancers occur in the eyelid. Incidence studies indicate that basal cell carcinoma is the most frequent malignant eyelid tumor, followed by squamous cell carcinoma, sebaceous gland carcinoma, and malignant melanoma. Many therapeutic methods have been suggested to combat the morbidity and mortality associated with these lesions., Literature Reviewed: A MEDLINE and PubMed literature search (1966-1999) was conducted for English language abstracts and appropriate (selected) full-text references retrieved regarding treatment of malignant eyelid tumors. These sources then were used to prepare recommendations for patient care. Each recommendation was rated according to: (1) its importance in the care process and (2) the strength of evidence supporting the given recommendation., Results: All recommendations were rated as level A (very important to patient-care outcome). For basal cell carcinoma, squamous cell carcinoma, and sebaceous gland carcinoma, the published evidence supporting two recommendations (Mohs' micrographic surgery or excision with frozen-section control) were graded as I (providing strong evidence in support of a recommendation). For sebaceous gland carcinoma, the recommendations also included conjunctival map biopsies. The published evidence supporting all other recommendations for these three eyelid tumors were graded II (substantial evidence in support of a recommendation), primarily because of the small numbers of patients in each clinical study. For malignant melanoma, the recommendation for therapy (i.e., excision with variable margins depending on tumor thickness) was based on published papers individually variably rated as I, II, and III, reflecting ongoing debate as to the best method of therapy., Conclusions: Published reports regarding the treatment of malignant eyelid tumors include a myriad of treatment options. The strongest evidence favors complete surgical removal using histologic controls for verifying tumor-free margins of excision.

Background: Sebaceous carcinomas are relatively rare cutaneous tumors; there are fewer than 600 cases reported. They most commonly arise within the meibomian gland of the eyelid; fewer than 150 of the reported cases were extraocular. These tumors have a high incidence of local recurrence and regional metastasis. The relationship of sebaceous tumors and visceral malignancy is well established in the literature., Objective: We describe a 44-year-old white female with chronic progressive multiple sclerosis who developed multiple scalp sebaceous adenomas and a solitary sebaceous carcinoma., Conclusion: Extraocular sebaceous carcinomas are rare tumors with high incidence of local recurrence and regional metastasis. Surgery with wide surgical margins is the standard treatment. We report the first case of multiple sebaceous tumors in a patient with multiple sclerosis. The presence of sebaceous tumors warrants a search for an internal malignancy. Literature on sebaceous tumors and their relationship with visceral malignancies and immunologic disorders is discussed. Literature on sebaceous carcinoma with special attention to extraocular sebaceous carcinoma is also discussed.

Purpose: To describe a technique for sentinel node mapping and biopsy in patients with orbital or adnexal tumors., Methods: Five patients with orbital and adnexal tumors were studied. Two patients had malignant eyelid melanomas (one of the skin and one of the conjunctiva), one with orbital invasion. Two patients had sebaceous gland carcinoma, and one patient had a mucoepidermoid carcinoma of the conjunctiva; 500 microCi of Technetium-99m sulfur nanocolloid (Nycomed Amersham, Princeton, NJ) diluted to 1.0 mL was injected intradermally at the lateral canthus. The patients were positioned as they would be during surgery. Lymphoscintigraphy was performed by means of anterior, lateral, and oblique views. The tracer was followed to the first lymphatic basin, and the sentinel node was identified. Cutaneous markers were placed to denote the site. During surgery, lymphoscintigraphy scans and a hand-held gamma probe were used to locate the sentinel node. Once excised, the sentinel node was sent for histopathology. Frozen sectioning confirmed the presence of lymphoid tissue. Permanent sections with immunohistochemical markers were performed to examine for metastatic disease., Results: The sentinel node biopsy technique was applied to 5 patients with orbital and adnexal tumors. All lymph nodes were free of tumor on histopathologic examination., Conclusions: Sentinel node mapping and biopsy are possible for orbital and adnexal tumors. The morbidity of elective lymph node dissection and adjuvant radiotherapy can be avoided. Our results are preliminary, and further work must be done to identify the lymphatic basins of the orbit and ocular adnexa.

Objective: To analyze the association between retinoblastoma (Rb) and sebaceous carcinoma (SC) of the eyelid to improve surveillance of survivors of RB:, Design: Case report and systematic literature review., Methods: Ten patients who had SC develop after Rb were identified by systematic literature review, and a child who died with lymph node, lung, and liver metastases 7 years after irradiation for Rb is described. The data were analyzed by univariate statistics, including cumulative frequency distribution plots and Kaplan-Meier analysis., Results: Of 11 children with SC of the eyelid who all had hereditary RB, 9 (82%; 95% confidence interval, 48-98) received a median of 46 Gy (range, 21-89) of radiotherapy at a median age of 16 months (range, 0.5-15 years) and had SC develop within the field of radiation. Their median age at diagnosis of SC was 14 years (range, 8-30 years), median diagnostic delay 12 months (range, 6 months-3 years), and median interval from irradiation 11 years (range, 5-26 years); 7 of them (78%; 95% confidence interval, 40-97) were diagnosed between 5 and 15 years after radiotherapy. SC also developed at the age of 32 and 54 years in two nonirradiated Rb patients. Five patients had regional lymph node metastases after a median time of 12 months (range, 1 month-24 years). The 5-year cumulative probability of survival was 87%., Conclusions: SC of the eyelid may occur in patients with hereditary Rb regardless of primary treatment, especially within the field 5 to 15 years after radiotherapy.

We report a case of ocular sebaceous carcinoma, involving incidental discovery in an elderly woman and its recurrence following inadequate eradication. We review the clinical presentation and treatment options with emphasis on potential errors in clinical judgment leading to recurrence.

Background: Sebaceous carcinoma is an aggressive neoplasm that commonly arises from the meibomian glands of the eyelids and other sebaceous glands of the ocular adnexa. Historic data indicate a nearly 30% local recurrence rate with standard surgical excision. Excision by means of Mohs micrographic surgery may be more efficacious. However, reports documenting the effectiveness of this technique for the treatment of eyelid sebaceous carcinoma have been limited to a few cases., Objective: We report our experience in the treatment of ocular sebaceous carcinoma with the Mohs fresh tissue technique., Methods: Eighteen patients with a diagnosis of sebaceous carcinoma of the eyelid who underwent resection by means of the Mohs fresh tissue technique during the years 1988-1998 were reviewed., Results: Sixteen of the 18 patients were free of disease after an average follow-up of 37 months (11.1% recurrence rate). One patient who experienced local recurrence also had metastatic disease of the parotid lymph nodes (5.6% metastatic rate). The recurrence and metastasis were noted 9 months after excision. The other patient experienced a local recurrence 19 months postoperatively. Both patients exhibited pagetoid spread and involvement of both the upper and lower eyelid at the time of Mohs excision., Conclusion: Mohs surgery offers excellent results when used as the primary treatment modality for sebaceous carcinoma of the eyelid. When compared with historic series of standard surgical excision, Mohs micrographic surgery has a significantly lower recurrence rate and metastatic rate.

Purpose: Demonstration of the importance of surgical excision and histological examination in presence of an apparently harmless tumoral alteration of the eyelids., Case Presentation: We report the case of a 65 year old patient suffering from systemic lupus erythematosus who noted a tumoral lesion on his left lower eyelid. Suspecting a chalazion, his dermatologist simply performed a cauterization. Six weeks later, a recurrence of the tumor appeared at the same location, and again, cauterization was done. A few weeks later, the patient consulted our clinic with a polycyclic tumor of 5 mm in diameter, involving the lid margin of the temporal part of the lower left eyelid. The patient had only moderate signs of blepharitis. There were no palpable preauricular and cervical lymph nodes. Suspecting a malignant tumor, the entire tumoral lesion was removed surgically., Histopathology: The histopathologic examination showed a highly differentiated sebaceous gland carcinoma, most probably originating from a meibomian gland. The margins of the excision were found to be tumor-free., Discussion: Sebaceous cell carcinoma is a rare entity. Depending on its histological differentiation it can be highly malignant. Infiltrative, and can metastasize. The mortality may reach 30% if low differentiation is present. As illustrated in the present case, the lesion may masquerade a chalazion. Therefore, in case of atypical lesion of the eyelid region complete surgical removal followed by a histopathological examination should be performed.

A 54-year-old male had a dome-shaped and skin-colored nodule on his nose. Histopathologically, we diagnosed this neoplasm as a low-grade sebaceous carcinoma rather than a sebaceoma based on the scanning magnification and cytology. This low-grade sebaceous carcinoma was associated with glandular structures. We regarded the glandular structures as those of apocrine glandular differentiation based on 1) the histopathologic features of the glandular structures formed by columnar luminal cells with evidence of decapitation secretion; 2) the expression of cytokeratin (CK) 19, CK8, CK8/18, and CK7 in the luminal cells; 3) the positive reaction of carcinoembryonic antigen and epithelial membrane antigen on the luminal surface and in the cytoplasm of the luminal cells; and 4) the common embryologic origin of the folliculosebaceous-apocrine unit. We found CK15 expression in undifferentiated cells within the mantles of normal hair follicles, suggesting that sebaceous stem cells might exist in mantles as follicular stem cells exist in bulge areas. Pluripotent stem cells in the folliculosebaceous-apocrine unit can give rise to follicular stem cells, sebaceous stem cells, and apocrine stem cells. Our patient's neoplasm showed apocrine glandular differentiation and partial immunohistochemical positivity for CK15 in the neoplastic aggregations. We believe this neoplasm originated from pluripotent stem cells destined to become sebaceous stem cells or from sebaceous stem cells, which also have the ability to differentiate within apocrine glands.

We report two cases of sebaceous carcinoma arising on actinic keratosis. The first patient, a 75-year-old female, had a granuloma pyogenicum-like tumor on her left temple, and the second patient, an 81-year-old female, developed a tumor with erythema on her left cheek. In both cases, histopathological examination revealed findings typical of sebaceous carcinoma in the center of the tumors, and in the periphery, actinic elastosis and intraepidermal proliferation of squamoid atypical cells without vacuolation was observed. Immunohistochemical examinations using six antibodies also revealed that neoplastic cells of both cases demonstrated sebaceous differentiation. These cases suggest that extraocular sebaceous carcinoma may arise from actinic keratosis.

Purpose: Because of previous reports of increased rate of recurrence and mortality after radiation therapy, eyelid sebaceous cell carcinoma has been considered radioresistant. Recent reports of success with primary radiation therapy have been attributed to advancements in irradiating technology and technique. Two cases of eyelid sebaceous cell carcinoma successfully treated with radiation therapy are reported. The techniques used are compared with the techniques described in previous reports, and factors favoring successful treatment are reevaluated., Methods: Case series and review of the literature. Two cases of eyelid sebaceous cell carcinoma that underwent radiation therapy are described. Both patients were offered, but refused, surgical excision. One patient received 69 Gy combined superficial and megavoltage x-ray irradiation to the left lower eyelid. The second patient received 59 Gy megavoltage electron beam irradiation to the right upper eyelid. A review of the literature was performed, and Fisher's exact test analysis was used to compare the results of all reported cases treated with < or =55 Gy with those treated with >55 Gy., Results: In both cases, the tumor responded to radiation therapy. One patient died 39 months after treatment, of myocardial infarction. The second patient is without clinical evidence of tumor recurrence 46 months after treatment. Fisher's exact test showed an advantage to patients treated with >55 Gy radiation (p = 0.05)., Conclusion: Radiation therapy with an appropriate delivery system is effective as a curative treatment for eyelid sebaceous cell carcinoma when >55 Gy of radiation dose is delivered. It should be considered for patients seeking an alternative to surgical excision.

Purpose: Patients infected with the human immunodeficiency virus (HIV) are at increased risk for developing malignancies, which are acquired at a younger age and are more aggressive. Sebaceous cell carcinoma is a rare eyelid tumor typically occurring in the seventh decade of life. We report two cases of sebaceous cell carcinoma in young HIV-infected patients., Methods: Case series and review of the literature. We describe two HIV-infected patients with sebaceous cell carcinoma of the eyelid and caruncle. The first patient was a 36-year-old woman with a 9-month history of an enlarging right lower eyelid mass. The second patient was a 34-year-old man with a 6-month history of an enlarging right caruncular mass., Results: Biopsy showed both masses to be sebaceous cell carcinoma. The first patient underwent Mohs' micrographic excision of the lesion followed by reconstruction of the full-thickness eyelid defect with a combination of tarsoconjunctival and myocutaneous advancement flaps. The second patient underwent exenteration because of orbital involvement., Conclusion: Sebaceous cell carcinoma should be considered for any suspicious eyelid lesion in young HIV-infected patients.

We report on a rare distinctive variant of infiltrating ductal carcinoma characterized by sebaceous differentiation of tumor cells. The neoplasm was identified in a lumpectomy specimen from a 45-year-old woman with extensive metastatic disease. In addition to conventional in situ and invasive ductal components, approximately half of the tumor cells exhibited a phenotype resembling tumors of the sebaceous skin appendage with coarsely vacuolated cytoplasm and peripherally displaced nuclei. The sebaceous moiety was also present in the distant metastatic deposits. There was no evidence of mucin production by tumor cells. Ultrastructurally, empty-appearing non-membrane bound vacuoles attested to the sebaceous cells' lipid content. The immunoprofile of the lesion included positivity for cytokeratin and epithelial membrane antigen. Vimentin, S100 protein and carcinoembryonic antigen were not expressed. Most tumor cell nuclei reacted with antibodies to oestrogen and progesterone receptors but failed to show overexpression of the HER2/neu protein. The MIB-1 labeling index averaged 16%. At variance with sebaceous breast carcinomas on record, the present case is notable for its prolonged clinical course.

The cytologic findings of two cases of metastatic sebaceous carcinoma are described and compared to three cases of locally recurrent basal cell carcinoma. Morphological findings for sebaceous carcinoma in fine-needle aspiration biopsy (FNAB) smears included cellular, loosely cohesive cell clusters with central necrosis, squamous pearl formation, and adjacent keratin debris. The tumor cells had moderate amounts of vacuolated cytoplasm, round to oval vesicular nuclei with clumped chromatin, nucleoli, some nuclear overlap, and numerous mitotic figures. An interesting finding was the presence of numerous multinucleated giant cells, probably responding to extravasated lipid or keratin material. In contrast, the FNAB smears of basal cell carcinoma typically were less cellular, with more tightly cohesive and smaller clusters of uniform hyperchromatic basaloid cells with high nuclear to cytoplasmic ratios, and a narrow rim of cytoplasm without vacuolization. The morphologic features of sebaceous carcinoma in FNAB smears appear to be distinct from those of basal cell carcinoma. FNAB can be a useful preoperative diagnostic technique to distinguish these two cutaneous malignancies., (Copyright 1999 Wiley-Liss, Inc.)

Background: Diagnosis of sebaceous carcinoma of the periorbital region is often delayed. Clinically, this lesion can mimic several inflammatory disorders. Histopathologically, it can mimic either squamous cell or basal cell carcinoma., Objective: To identify an immunohistochemical approach to assist in the diagnosis of periorbital sebaceous carcinoma., Method: The immunohistochemical profiles of several cases of periorbital sebaceous, basal cell, and squamous cell carcinoma were examined., Results: Although at least focal epithelial membrane antigen (EMA) staining can effectively distinguish sebaceous carcinoma (10 of 11 were positive) from basal cell carcinoma (1 of 16 were positive), most squamous cell carcinomas examined were also focally EMA positive (11 of 14). However, Cam 5.2 reactivity was seen in most sebaceous carcinomas (8 of 11) but no squamous cell carcinomas (0 of 14). In addition, at least focal BRST-1 reactivity was also seen in most sebaceous carcinomas (7 of 11) but no basal cell carcinomas (0 of 16)., Conclusions: Periorbital sebaceous, basal cell, and squamous cell carcinomas have different immunohistochemical staining profiles; a panel of commonly available antibodies, including anti-EMA, BRST-1, and Cam 5.2, may help distinguish these diseases from each other when that distinction cannot be clearly made by light microscopy alone.

A very rare case of a sebaceous carcinoma of the external auditory canal with basal cell differentiation is presented. Fewer than 400 cases affecting any part of the body have so far been reported and of that only seven cases have been known to involve the external auditory canal. The clinical features, pathology and treatment are described and the relevant literature has been reviewed.

Purpose: To describe a previously unreported presentation of sebaceous carcinoma, an aggressive tumor that often presents insidiously with minimal symptoms and nonspecific signs., Methods: We report a 71-year-old man who presented with unilateral ocular irritation and ipsilateral, idiopathic, papillary changes of the superior palpebral conjunctiva. The patient underwent incisional biopsy of the palpebral conjunctiva followed by full-thickness excision of the involved eyelid., Result: Histopathologic examination established the diagnosis of sebaceous carcinoma., Conclusion: Unexplained asymmetric, papillary changes of the palpebral conjunctiva should arouse suspicion of sebaceous carcinoma.

Sebaceous gland tumor is a rare disease that is a sign of Muir-Torre syndrome, an autosomal, dominantly inherited genodermatosis characterized by the presence of at least one sebaceous gland tumor and a minimum of one internal malignancy. Recent studies have indicated that defective DNA mismatch repair occurs in Muir-Torre syndrome. Cutaneous lesions may occur before diagnosis of the internal cancer. We describe a 64-year-old male patient with multiple sebaceous epitheliomas with no evident internal malignancy. Microsatellite instability, determined by examining dinucleotide CA repeats at the microsatellite loci, was observed in DNA from one sebaceous epithelioma but not from the other two sebaceous epitheliomas or from one basal cell epithelioma with sebaceous differentiation, suggesting that this condition is unlikely to be due to germ-line mutation of mismatch repair genes.

Background: Sebaceous cell carcinoma is a distinctive tumor of the eyelid associated with a high rate of metastasis and mortality. Involvement of both upper and lower lids has been described; however, involvement of all 4 eyelids is extremely rare., Objective: To describe the evaluation and diagnosis of the clinicopathologic features in a 74-year-old patient with 4-eyelid sebaceous carcinoma and a history of whole face irradiation for eczema., Methods: Bilateral eyelid, conjunctiva, map, and fine needle biopsy of enlarged, submandibular lymph node specimens were stained with hematoxylin-eosin and oil-red-O for light microscopy and fresh-frozen sections were evaluated by electron microscopy., Results: The biopsy specimens from the eyelids and conjunctiva showed sebaceous carcinoma cells in the meibomian glands and scattered within the conjunctival epithelium. The cells were moderately well differentiated. Similar cells stained with oil-red-O were found in the submandibular lymph nodes., Conclusions: The appearance of bilateral sebaceous carcinoma supports the concept of simultaneous occurrence of multiple primaries in specific patients, especially after radiation therapy. Patients with a history of facial irradiation and atypical eyelid lesions should be evaluated for malignant tumors such as sebaceous carcinoma.

Background: Sebaceous carcinoma of the eyelid is rare. The diagnosis might be difficult because of its ability to masquerade as other periocular lesions. Prognosis is still regarded as being poor compared with most other malignant eyelid tumours with a mortality second only to malignant melanoma. The present study retrospectively analyses clinical and histopathological findings and outcome in a series of patients with sebaceous carcinoma of the eyelid in Britain., Methods: 43 patients with histologically confirmed sebaceous carcinoma treated at Moorfields Eye Hospital between 1976 and 1992 were subjected to retrospective analysis. Clinical data of all patients were reviewed from the charts; all surviving patients except four cases lost for follow up were re-examined. Histological specimens were reviewed in 41 cases., Results: 23 females and 20 males, mean age 63 years (range 37-79), were treated. Primary therapy was surgery in 37 and radiotherapy in six cases. After a median follow up of 40 months (range 1-148) 30 patients were alive without recurrences, four patients had died from the tumour, and one was alive with local recurrence and distant metastases. Four patients had died of non-tumour related causes. Histologically, unfavourable outcome was correlated with poor tumour differentiation and extensive invasion., Conclusion: Early diagnosis and consequent surgical therapy of sebaceous carcinoma of the eyelid leads to a better outcome and higher survival rates than generally assumed. Even local recurrences can be treated successfully. However, sebaceous carcinoma remains a threatening disease, which leads to death in 9% and to mutilating exenteration in 23% of our patients.

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.

In addition to Paget's disease, a heterogeneous group of processes with diverse histogeneses can give rise to intraepidermal pagetoid cells. These diseases share as their common denominator the presence of discrete non-Malpighian or abnormal Malpighian cells occurring singly or in nests within the epidermis. Either Pagetoid cells can represent the only histologic change, as in pagetoid squamous cell carcinoma in situ or superficial spreading malignant melanoma in situ, or they can be an expression of an associated dermal or internal malignancy, as in sebaceous carcinoma or breast carcinoma. The histologic appearance of the pagetoid cells in these diverse disorders can be quite similar, rendering the differential diagnosis difficult. A review of the entities that enter into the differential diagnosis of intraepidermal pagetoid cells is presented, emphasizing their distinguishing histologic and immunophenotypic features and differential diagnosis.

The distributional patterns of MUC 1 (the mucin whose cDNA was first cloned) and Keratin 14 (K14) in the invasive regions of malignant eyelid tumors were immunohistochemically examined by comparing with other histochemical markers. The MUC 1-positive tumor cells were detected in several serial, small, invasive tumor masses in the deep subepithelial region of the low differentiated carcinoma. They were also continuously detected in the border region between accumulated lymphocytes including T cells and tumor masses of the sebaceous carcinoma. On the other hand, K14-positive tumor cells were detected in the marginal regions of large tumor masses or those with smooth edges, some of which overlapped the distribution of MUC 1-positive cells in the tissues of undifferentiated carcinoma, squamous cell carcinoma, and sebaceous carcinoma. In general, MUC 1 may be expressed in the invasive tumor cells, whereas K14 may be expressed in the marginal cells of the stable, proliferating tumor masses.

Sebaceous carcinoma of the Zeis gland of the cilia is a rare malignant tumor. A 90-year-old Japanese man with a Zeis gland carcinoma on the left upper eyelid is reported. The tumor was a 12 x 3-mm, reddish, partially yellow, papillomatous nodule accompanied by loss of several lashes. Histologically, two types of tumor cells, a dark basaloid cell and a larger pale cell, showed lobular and comedo-acinar growth patterns. The carcinoma did not recur during the 8 months following surgery; however, the patient died of gastric carcinoma.

A case of sebaceous carcinoma arising in the buccal mucosa is described.

Sebaceous carcinoma is an infrequent tumor of tardy increase, locally aggressive, potentially recurrent, with natural propensity for metastasize, both loco-regional or remote (lymphogenous or hematogenus metastases). Principal sitting are the annexed glands to ocular sense (Zeis glands and specially Meibomian's). Its origin in salivary glands is exceedingly uncommon, only those arising in the parotid have been reported. Our contribution are 2 cases which source were the sublingual gland and a minor palatine salivary gland, respectively. The perusal of the literature has been negative: no one previous case of sebaceous carcinoma could be discovered in the literature.

We retrospectively studied 14 patients confirmed histopathologically to have eyelid sebaceous carcinoma during 1986-1994. We removed all tumors totally with frozen section controls and performed reconstruction either by primary apposition or transposition flaps. We observed recurrence in 5 patients (35.7%) in whom a surgical margin of either 1 or 3 mm was taken but none in those with a 5-mm surgical margin. At least 5 mm of normal tissue around the tumor should be removed considering the metastatic potential of these tumors.

A case of cervical spine metastasis from an extraocular sebaceous carcinoma of the scalp is presented. Anterior decompression and fusion were performed and resulted in complete relief of symptoms. Postoperatively the primary tumor behaved in a very aggressive manner, with visceral metastases leading to the death of the patient in a few weeks.

Although sebaceous glands are prominent on the vulva, sebaceous carcinomas of the vulva rarely occur. In fact, there have been only two cases of sebaceous carcinomas of the vulva reported in the literature. Eighty percent of vulvar cancers are squamous in origin with human papillomavirus (HPV) DNA detected in approximately 60% of these cancers. We present a third patient with sebaceous carcinoma of the vulva and the first to our knowledge that has been analyzed for HPV DNA. The case report and a review of the literature are presented.

Background: Since sebaceous carcinomas with a poor prognosis arising from the cutaneous adnexae of the eyelid are sometimes difficult to differentiate from squamous cell carcinoma or basal cell carcinoma on clinical and histopathological findings, definite differentiation between these tumors by additional methods is necessary., Objective and Methods: To clarify the usefulness of histochemical and immunohistochemical examinations in diagnosing ocular sebaceous carcinoma, histochemical and immunohistochemical studies were carried out on the 2 patients with unusual ocular sebaceous carcinoma., Results: The findings of ocular sebaceous carcinoma, at least in our 2 cases, were almost identical to those of extraocular sebaceous carcinoma., Conclusion: Immunohistochemical detection of human milk fat globules subclass 1, human milk fat globules subclass 2, and breast carcinoma-associated antigen 225 was useful for diagnosing ocular sebaceous carcinomas as well as extraocular ones.

A report of an 87-year-old Caucasian female with an extensive sebaceous carcinoma of the parotid gland is presented. The computed tomographic characteristics of this rare neoplasm are reported for the first time.

Sebaceous adenomas and squamous cell carcinoma developed in a male patient in addition to viral, mycotic and bacterial infections, several years after the removal of three malignant tumors from his lower gastrointestinal and urinary tract. Skin tests with trichophytin, candidin, and mixed bacteria were negative. Various aspects regarding cutaneous changes associated with colorectal and bladder carcinomas are discussed.

We examined a neoplasm of > 10 years' duration on the head of a woman. Light microscopically, the neoplasm was composed of multiple epithelial lobules in the dermis. The islands were mainly made up of basaloid cells and sebaceous cells; the latter cells were focally grouped in the lobules. Neither a palisade arrangement of peripheral cells nor the formation of peritumoral clefts were seen. Ductal spaces were scattered in the lobules and their luminal surfaces showed keratinization. Furthermore, in the peripheral parts of some lobules, there were sweat-gland-like structures. Immunohistochemically, most of the lobules were shown to have stratified squamous epithelium-type keratins, while the sweat-gland-like structures were shown to have simple epithelium-type keratins and stained positive for carcinoembryonic antigen. Ultrastructurally, many cells had lipid formation in their cytoplasm, and some cells formed luminal walls keratinizing without microvilli and keratohyaline granules. Although most of this neoplasm showed sebaceous differentiation, it partially differentiated to secretory cells of sweat gland apparatus.

Extraocular sebaceous carcinoma (SC) is a rare tumor usually found on the head and neck. A 78 year old Japanese female who had an asymptomatic vulvar tumor is reported here. The excised specimen showed SC with metastasis to the inguinal lymph nodes. This is the fourth reported case of SC arising from female genitalia, and the second case that apparently arose from the labia minora. Contrary to the previously reported cases, tumor cells in the present case had abundant glycogen. Thus, differential diagnosis of SC from metastatic renal cell carcinoma is difficult morphologically because both of them have glycogen and lipid. Intraepidermal invasion of tumor cells has been reported in SC, but a suspected lesion of this phenomenon in the present case was proved to be histiocytic infiltration by immunohistochemistry using anti-CD 68 antibody.