Sebaceous Epithelioma

An 83-year-old woman had developed an asymptomatic, yellowish, dome-shaped skin tumor on her scalp which had enlarged for a period of 60 years to 10 times 10 times 10 mm in size. Histopathologically, the tumor consisted of undifferentiated basaloid cells, differentiated sebaceous cells and transitional cells. Although the basaloid cells resembled those of basal cell epithelioma (BCE), the tumor was distinct from BCE in the following points; existence of cystic spaces, no peripheral palisading, no proliferation of connective tissue stroma, and no tendency toward local invasion. Immunohistochemical studies using antikeratin monoclonal antibodies revealed that the tumor contained both the keratin types of BCE and of sebaceous glands. Electron microscopically, the tumor cells contained lipid droplets and keratohyaline granules in their cytoplasm. It is suggested that “sebaceous epithelioma” is a benign skin tumor which may be distinguished cytologically from BCE.     

Basal cell carcinoma with sebaceous differentiation

Some authors have used sebaceous epithelioma as a synonym for basal cell carcinoma (BCC) with sebaceous differentiation. However, our review of the literature revealed that definite cases of BCC with sebaceous differentiation that provide adequate clinical and histopathologic information are scarce. We present the case of a 72-year-old woman with a pigmented nodular lesion on her right ala nasi region, clinically diagnosed as pigmented BCC. Histopathologically, this nodular lesion, which was completely excised, showed typical features of BCC. It was noteworthy that within one aggregation of the presented BCC, tiny and small duct-like structures lined by cornified layers with a crenulated inner surface were seen. Vacuolated cells were scattered within a few aggregations, and they had foamy, bubbly cytoplasm and starry nuclei. The vacuolated cells were immunohistochemically positive for epithelial membrane antigen (EMA). These histopathologic findings demonstrated unquestionable sebaceous differentiation in this BCC, namely BCC with sebaceous differentiation, which should be distinguishable from both sebaceoma and sebaceous carcinoma. The small duct-like structures lined by eosinophilic cuticle, indicating apocrine differentiation, were also observed in this BCC.     

A Case of Superficial Epithelioma with Sebaceous Differentiation

Superficial epithelioma with sebaceous differentiation developed on the left cheek of a 58-year-old man over a three-year period. Biopsy of the lesion demonstrated plate-like lobules of basophilic basaloid cells with broad attachments to the overlying epidermis. Clusters of or solitary sebaceous cells were present within the lobules. Three tumor types were considered; a subtype of sebaceoma growing in the epidermis, an acanthotic seborrheic keratosis subtype with sebaceous differentiation, or a tumor of the follicular infundibulum with sebaceous differentiation.     

Alopecia areata associated with regression of cutaneous melanoma

A 41-year-old white female with a past medical history of hypothyroidism and alopecia universalis presented on January 24, 2002 with a recently changing mole. She indicated changes in size and color of the superior aspect of a mole that had been present for more than 8 years. She had approximately 20 lifetime peeling sunburns due to being a lifeguard. No family or previous personal history of skin cancers, including melanoma or atypical nevi, was reported. Her history of alopecia universalis began 12 years previously and has partially resolved with remaining patchy alopecia of the scalp and eyebrows. On diagnosis of alopecia universalis, she was initially treated with oral prednisone for 1 year and topical minoxidil for 3 months. Currently, she is not being treated for this condition. She denied other previous skin conditions. She had a surgical history of tonsillectomy at the age of 7 years. Her current medication includes levothyroxine (0.015 microg) for hypothyroidism diagnosed 12 years previously. She reported no known drug allergies. During the initial physical examination, she presented with phototype II skin with two adjacent pigmented lesions on her left foot within a 1.3 cm square. The first lesion on the left posterior distal heel was an irregular, brown-black, 0.5 x 0.6 cm macule. The second lesion, on the left posterior proximal heel, was an irregular, brown, speckled, 0.3 x 0.4 cm macule (Fig. 1). The patient had ophiasis of the scalp and total alopecia of the bilateral eyebrows. In keeping with the patient's wishes, alopecia lesions were not biopsied and clinical photographs of the alopecia are not included in this article. Two 3 mm punch biopsies were performed within each lesion. The left posterior proximal lesion showed malignant melanoma, with a Breslow depth of 0.4 mm, anatomic level II, marked lymphocytic response and partial regression (Fig. 2). The left posterior distal lesion showed malignant melanoma in situ, arising in a lentiginous compound nevus, with architectural disorder and cytological atypia. These two lesions were concluded to be one lesion with clinical regression. She underwent local excision with 1-cm margins and sentinel lymph node biopsy owing to the presence of regression, which showed no evidence of metastatic melanoma. Lactate dehydrogenase and chest X-ray were within normal limits. The alopecia areas were not biopsied previously or at that time.     

Electron microscopic study of sebaceous epithelioma.

A case of sebaceous epithelioma on the scalp of a 50 year old female was reported. Using an electron microscope, cells from the tumor were classified into 5 stages according to their development. Furtheremore, our investigation indicate that lipid synthesis was activated in sebaceous epithelioma cells rather than in normal sebaceous cells, and can apparently be distinguished from lipid-droplet formation by degradation. These sebaceous epithelioma cells also possessed the ability to secrete which suggests that these tumor cells originated from germinative cells of the sebaceous gland rather than epidermal basal cells.     

Metastasizing adenocarcinoma and multiple neoplastic proliferations arising in a nevus sebaceus

Nevus sebaceus of Jadassohn is a hamartoma of multiple skin structures. Many neoplasms have been reported to arise in association with nevus sebaceus, most commonly trichoblastoma/basal cell carcinoma and syringocystadenoma papilliferum. We report a case of a 66-year-old woman with an adenocarcinoma as well as multiple neoplastic proliferations arising in a long standing nevus sebaceus on the scalp, with subsequent occipital neck metastatic disease. On histologic evaluation, the epidermis showed changes reminiscent of tumor of the follicular infundibulum as well as basaloid proliferations resembling superficial trichoblastoma. A focus suggestive of syringofibroadenoma was also present. A small dermal collection of basaloid and more mature sebocytes was consistent with a sebaceoma/sebaceous epithelioma. Most of the lesion was composed of an adenocarcinoma with areas showing ductal differentiation with decapitation secretion, well-formed papillae and focal cribriform structures. Other portions demonstrated a high-grade neoplasm with prominent nuclear atypia and a solid pattern of growth resembling high-grade breast carcinoma. Anti-epithelial membrane antigen strongly labeled tumor cells and highlighted ductal structures. Less than 1% of cells expressed progesterone or estrogen receptors. Her2/neu reactivity was focally present, showing 1+ membranous reactivity in 10% of cells. Anti-p63 labeled basaloid cells surrounding the tumor lobules. A breast primary was ruled out by clinical and radiologic examination. This report illustrates an extraordinary case of adnexal neoplasia displaying various lines of differentiation arising in association with nevus sebaceus.     

Complex adnexal tumor of the primary epithelial germ with distinct patterns of superficial epithelioma with sebaceous differentiation, immature trichoepithelioma, and apocrine adenocarcinoma.

A 60-year-old man came for treatment of a sharply outlined erythematous plaque on the gluteal area (45 x 20 mm) of 20 years' duration. Eccentrically located on the plaque was a nodule, 20 mm in diameter. Histological study of the plaque showed a superficial platelike tumor with basaloid bland cytology and sebaceous gland differentiation. Histologic study of the nodule found an undifferentiated adenocarcinoma whose ductlike glandular structures opened to the skin surface and infiltrated the whole depth of the dermis. Study of other areas of the lesion detected two more neoplasms. A nodule of squamous cell carcinoma was found within the superficial band of the benign sebaceous tumor. The fourth neoplastic pattern consisted of epithelial islands composed of basaloid cells within a fibroblastic stroma. There was prominent palisading of epithelial cell nuclei at the periphery of the islands, which usually were surrounded by a sheath of mesenchymal cells. In this complex adnexal tumor of the primary epithelial germ, sebaceous and follicular differentiation both simulate neoplastic patterns recently described as separate entities: superficial epithelioma with sebaceous differentiation and immature trichoepithelioma. The undifferentiated adenocarcinoma may represent differentiation toward the third component of the germ, that is, the apocrine gland.     

AGGRESSIVE TRICHOEPITHELIOMA VERSUS KERATOTIC BASAL CELL CARCINOMA

A 43-year-old white man presented with an infiltrative le-sion on the left nasolabial fold, which had grown slowly over 15 years. There was a history of moderate sun expo-sure. Prior history was negative for cutaneous carcinomas. Family history was negative for trichoepithelioma. Physical examination revealed a patient with a fair complexion, blue eyes, light skin, and light brown hair. There was evidence of severe actinic damage manifested by marked solar elas-tosis of the face. A 1.5 × 1.3 cm infiltrative, depressed, skin-colored plaque, with elevated pearly borders was noted on the left nasolabial fold. There was nodule formation with superficial ulceration in the center of the lesion (Fig. 1). No other skin tumors were present.
A punch biopsy showed a deeply infiltrating tumor. Sub-sequently, the tumor was excised by Mohs micrographic surgery in six stages, revealing deep and wide clinically un-detected invasion into the orbicularis oris, zygomaticus minor, and the levator labii superioris muscles. The final size of the defect was 5.0 × 5.0 cm (Fig. 2). Reconstruction was later performed by otolaryngology with satisfactory results.
Pathological Study : The tumor was composed of multiple islands of basaloid cells embedded in a dense fibrotic stro-ma. Some islands showed peripheral palisading of their cells (Fig. 3). Other areas contain numerous horn cysts with abrupt and complete keratinization surrounded by basaloid cells (Fig. 4). Areas of calcification and foreign body reac-tion are present secondary to ruptured cysts. Stromal-stro-mal clefting characteristic of trichoepithelioma is seen adjacent to some tumor islands (Fig. 5). The tumor infil-trates into the underlying muscle (Fig. 6). Stromal muco-polysaccharide deposition and stromal-tumor separation as seen in basal cell carcinoma (BCC) were not present.     

Lacrimal caruncle primary basal cell carcinoma: case report and review

BACKGROUND: Basal cell carcinoma (BCC) is a common malignant skin neoplasm. The surface of the caruncle contains sebaceous glands, hair follicles, and lacrimal and sweat gland elements. Consequently, the caruncle may spawn any neoplasm that occurs in the conjunctiva, skin, or lacrimal gland. We report a patient with a primary BCC located on the lacrimal caruncle. METHODS: An 80-year-old man with a 5-month history of a gradually enlarging left caruncular neoplasm was seen. The lesion was nodular and irregularly brown-black colored, with no involvement of adjacent conjunctiva or skin. He had a history of sun exposure, but no personal or family history of other malignant neoplasms. RESULTS: The mass was excised completely. Histopathologic examination revealed a BCC originating in the basal layer of the conjunctival epithelium. No tumor recurrence has been noted after 7 years of follow-up. Primary BCC of the caruncle is unusual, and only four cases have been described in the PubMed. BCC should be considered in the differential diagnosis of caruncle and adjacent skin lesions.     

The spectrum of organoid nevi

Two patients in whom tumors developed in organoid nevi are reported. The first patient, a 50-year-old man, had a trichilemmoma arising from an organoid nevus on the scalp. The second patient, a 68-year-old woman, had a basal cell epithelioma, sebaceous epithelioma, syringocystadenoma papilliferum, and a trichilemmoma arising from an organoid nevus on the face.     

Basaloid squamous cell carcinoma with 'monster' cells: a mimic of pleomorphic basal cell carcinoma

Pleomorphic giant or 'monster' cells represent a well-recognized yet uncommon finding associated with basal cell carcinoma (BCC), usually of nodular type. We present a case of basaloid squamous cell carcinoma (basaloid SCC) with 'monster' cells that closely mimicked those described in pleomorphic nodular BCC. Clinically, the lesion presented as a fleshy, hyperkeratotic nodule in an 82-year-old woman. Histopathology revealed a basaloid lesion with lobulated borders and focal retraction artifact but a lack of prominent palisading or stromal mucin. There were areas of necrosis and small foci of keratinization. Striking bizarre monstrous pleomorphic nuclei were widely scattered throughout the lesion. Ber-EP4 immunohistochemistry proved to be negative and epithelial membrane antigen (EMA) expression was moderate to strong in 70% of the basaloid epithelium. Monster cells have not previously been highlighted in cutaneous SCC or in its uncommon cutaneous basaloid variant. The prognostic significance of monster cells is unknown but, given the relative paucity of keratinization in basaloid SCC, these lesions should probably be regarded as poorly differentiated. We have not previously encountered an SCC that so closely resembles nodular BCC with pleomorphic monster cells and believe that this is the first such report in the literature.     

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.     

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.     

Systemic sarcoidosis presenting with alopecia of the scalp

A 39‐year‐old African‐American woman was referred to the department of dermatology, Mount Sinal Hospital, New York for evaluation of a skin lesion on her scalp which had been present for 3 years and had recently changed in appearance. On examination, she was found to have erythematous plaques with hair loss extending from the frontal hairline on the right side in a “C‐shaped” distribution, terminating behind the left ear. Two years later the lesion had rapidly extended to the vertex of the scalp and left temporal area ( Fig. 1 ). There was a 10 cm × 12 cm area of alopecia at the vertex with slight erythema, but there was no evidence of scarring, inflammation or nodularity of the scalp ( Fig. 2 ). A skin biopsy taken from the forehead revealed granulomatous dermatitis showing noncaseating granuloma with negative acid fast bacilli (AFBC) and ammonical‐silverstain for fungus (GMS) ( Fig. 3 ).

Figure 1 Open in figure viewer PowerPoint Erythematous plaque with hair loss on the left temporal area     

线状皮脂腺痣综合征1例

报告1例线性皮脂腺痣综合征，患者男，23岁，头皮中线，右前额至鼻尖和左面颊，下颌至颈部两片大面积皮脂腺痣，双眼角结膜胬肉样改变及高度近视眼底，X线片示蝶鞍增大，提示鞍区占位性病变。     

Basal cell carcinomas in association with basaloid follicular hamartoma

We describe a unique case of various types of basal cell carcinoma (BCC) associated with basaloid follicular hamartoma (BFH) in a 56-year-old female patient. The lesion consisted of a dark brown and elastic soft nodule and papules within the area of a birthmark on the neck. The lesion was surgically excised. Histological examination of the nodular region revealed aggregations of neoplastic basaloid cells. We diagnosed the nodule as BCC with a racemiform or reticular pattern. In addition, a specimen taken from brownish black papules within the birthmark was found to be composed of anastomosing cords of basaloid cells accompanied by infundibular cystic structures. These features were consistent with an infundibulocystic BCC. In contrast, specimens from a hamartomatous plaque showed distinctive branching strands of basaloid cells that are suggestive of BFH. Therefore, our findings indicate that several types of BCC may develop within a BFH.     

A case of multiple sebaceous epithelioma: analysis of microsatellite instability

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.     

Squamous cell carcinoma in a patient with Netherton's syndrome

A 29-year-old white woman with a history of Netherton's syndrome presented with two squamous cell carcinomas on the right dorsal hand and the left upper arm. She reported a 2-year history of these lesions, which were originally treated as warts. She denied excessive sun exposure, immunosuppressive therapy, or a previous history of skin cancer. Her past medical history included acute renal failure, multiple urinary tract infections, meningitis, and recurrent otitis media as a child. In addition, she had an ovarian abscess at 4 years of age with resulting salpingo-oophorectomy. She also reported a history of severe myopia, glaucoma, and multiple ocular infections with a resulting corneal scar. In addition to atopic dermatitis, she had a 10-year history of psoriasis. Her medications included topical steroids and emollients for atopic dermatitis and psoriasis, in addition to Timolol ophthalmic drops for glaucoma. Her family history was significant for a 22-year-old sister with Netherton's syndrome (Fig. 1). She denied any history of skin cancer in her sister or other members of her family. On physical examination, she had an exfoliative erythroderma, madarosis, and diffuse patchy alopecia. In the bilateral axilla, she had well-defined pink scaly plaques which were confirmed as psoriasis by biopsy. On the right dorsal hand, she had a 1.5 x 1.0 cm pink verrucous plaque (Fig. 2). On the left upper arm, she had a 1.5 x 0.8 cm pink scaly plaque. Biopsies of both sites confirmed squamous cell carcinomas. Both lesions were completely excised with 4 mm margins.