Clinical and Experimental Dermatology 1990; 15: 222-224.
Sclerosing-sweat-duct (microcystic adnexal) carcinoma—a tumour from a single eccrine origin L.REQUENA, A.MARQUINA,* V.ALEGRE,* A.ALIAGA* AND E.SANCHEZ YUS Departments of Dermatology, Hospital Clinico San Carlos, Madrid and * Valencia General Hospital, Valencia, Spain Accepted for publication 18 October 1989
Summary A patient with a sclerosing-sweat-duct carcinoma of the upper Hp is reported. Histologically the tumour showed solid islands and strands of squamous cells and sparse small ductal structures, some of them containing central cores of dense eosinophilic keratin. All this was embedded in a sclerotic stroma. These features, in addition to positive immunoreactivity for carcinoembryonic antigen (CEA) in the lumina of small ducts, and the presence of S100 protein-positive cells in some cords and ducts, are consistent with the notion that this tumour exhibits differentiation toward eccrine sweat structures. Microcystic adnexal carcinoma, first described by Goldstein et al.^^ refers to tumours with both follicular and sweat-gland differentiation. It is often located in the upper lip of middle-aged or elderly patients and is characterized histologically by the presence of small horny cysts, cords and ducts embedded in a sclerotic stroma. The presence of keratin-filled horny cysts lined by squamous epithelium in the upper portion of the tumour—these closely resemble follicular structures—and the pre.sence of ductal structures in the deeper areas, have suggested a dual differentiation for this tumour; both follicular and eccrine. We report a patient with sclerosing carcinoma of the sweat ducts involving the upper lip. Immunohistopathological studies showed positive immunoreactivity for carcinoembryonic antigen (CEA) and S-100 protein in some cells of ducts and solid islands. In our opinion, these findings support differentiation of this tumour solely towards eccrine structures. Case report An 81-year-old man had a slowly enlarging plaque of 2 Correspondence: Luis Requena, C/Leopoldo Alas Clarin 4-6 F, 28035-Madrid, Spain. 222
Figure 1. Clinical appearance ofthe lesion: an induniicti upper lip.
years' duration on his upper lip. On physical examination there was an erythematous and indurated plaque on the central area of the upper lip, 3 0 x 4 0 cm in size, extending from the vermiHion border to the nasal columnella (Fig. 1). The lesion had telangiectases on its surface and a striking induration on palpation. There was no apparent involvement of underlying labial mueosa nor submental or cervical adenopathy. A biopsy was performed. Histopathological examination showed the presence, in the mid- and lower dermis, of numerous solid squamouscell nests of varying sizes (Fig. 2). Some of these nests contained central cores of eosinophilic keratin (Fig. 3). Other areas ofthe tumour exhibited ductal differentiation with isolated or grouped cytoplasmic vacuoles within the squamous nests and comma-like strands of epithelial cells (Fig. 4). This biopsy specimen was interpreted as a microcystic adnexal carcinoma and the tumour was excised using permanent section control for margin evaluation. Histopathological study of complete specimen showed identical findings in the upper and middermis. In the lower dermis, there were numerous solid cords or nests of basaloid cells, without evident lumen
SCLEROSING-SWEAT-DUCT CARCINOMA
223
[•"igure 4. An epithelial nest with small dticts and a tail-like protrusion (H&K x288).
Figure 2. Numerous small epithelial nests are in the middle and the lower dermis surrounded by a sclenrtic stroma {H&l.^ x 10).
Figure i. Fpitheliat nests with central cores of dense keraiin embedded in a sclerotic stroma (H&R x29).
Figure 5. Perineural invasion. A nerve tihre is surroiindid h\ a row of basaloid cells (H&E x 288).
Figure 6. CFA-positiveluminal structure. Thesolid islandsareCI'.Anegative (diaminoben/idine haematoxylin x 154).
formation, surrounded by a sclerotic stroma. In these bundles, closely resembhng the histopathological pattern deeper portions of the neoplasm it was not unusual for of cutaneous metastatic scirrhous carcinoma. In addition fields to contain only tiny strands and clusters of basaloid to widespread dermal involvement, the neoplasm infilepithelial cells embedded between sclerotic collagen trated subcutaneous tissue, and isolated nests of basaloid
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L.REQUENA FT AL.
Figure 7. Isolated cells are positive for S-100 protein (diaminobenzidine-haematoxilin x 154).
epithelial cells could be seen between the orbicularis oris muscle fibres. Perineural invasion was identified in some areas (Fig. 5). Immunoperoxidase staining for CEA revealed, mainly in the upper ducts, tbcally scanty staining in the lumina of small ducts or on the ductal lining surface of tumour cells (Fig. 6). There were also isolated cells of solid islands or ducts positive for S-100 protein (Fig. 7). Discussion Sclerosing-sweat-duct carcinoma u.sually aff^t'cts the face of middle-aged or elderly patients. The tumour is slowgrowing and appears as a firm or indurated plaque.' Typically this neoplasm invades deep structures, involving skeletal muscle and perineural spaces and these factors lead to a high recurrence rate unless the tumour is adequally excised. Therefore, microscopically controlled excision using Moh's technique to insure clear margins before closure has been proposed as the best therapy.^ However, in spite of this method of excision, there are reported cases^ in which a subsequent biopsy has demonstrated a recurrence. It is quite likely that in these recurrent cases after Moh's surgery, one could obtain a specimen in which the margins were completely free of tumour and yet tumour remained behind in the patient. Therefore, a wide local excision of the tumour with careful examination of all the margins of the specimen may be the treatment of choice. Histopathologically this tumour had two components. The first component consists of numerous keratin-filled horny cysts lined by squamous epithelium and the second comprises small ducts lined by two or three layers of epithelial cells, containing amorphous eosinophilic material and solid basaloid strands of eccrine-type epithelium. These findings support the view, initially proposed by Goldstein et al.^ of dual differentiation for this neoplasm: the first component would involve ex-
pression of follicular differentiation and the second would represent sweat-gland differentiation. The CEA results were also consistent with dual differentiation, because the secretory and ductal cells in eccrine and apocrine glands contain and secrete CEA, whereas foUicular structures do not.' Thus, positive islands for CEA represented eccrine differentiation and negative ones were follicle-derived structures. Nevertheless, keratinization of the upper portion ofthe intradermal eccrine duct is usually seen in syringomata' and retention milia cysts.•* The finding of S100 protein-positive cells in some cords and ducts ofthe tumour also supports an eccrine differentiation theory, because this protein is found in eccrine coil cells.^ Thus, these findings would mean that the three segments of normal eccrine sweat glands (the secretory portion, the intradermal duct and acrosyringium) are represented in the tumour. The islands negative for CEA must be interpreted bearing in mind that this antigen can be absent in purely squamous foci. In this regard, threedimensional reconstructions of these neoplasms have been proposed by Cooper** as an aid for the better understanding their histogenesis. Five of the six early cases of microcystic adnexal carcinoma' were located on the upper lip and all six cases contained horny cysts histologically, as well as duct- and gland-like structures. Later, it was obvious that these tumours might also be found in other locations and might lack horny cysts. They were then termed *sclerosingsweat-duct (syringomatous) carcinomas'.^ The case reported here has the clinical features of microcystic adnexal carcinoma and the histopathological characteristics of sclerosing-sweat-duct syringomatous carcinoma, confirming that these terms describe the same entity. References 1. Goldstein DJ, Barr RJ, Santa Cruz DJ. Microcystic adnexal carcinoma. A distinct clinicopathologic entity. Cancer 1982; 50: 566 572. 2. Cooper PH, Mills SE, Leonard DD, Santa Cruz DJ, Headington JT, Barr RJ, Katz DA. Sclcrosing sweat duct (syringomatous) carcinoma. .American Journal of Surgical Pathulngy 1985; 9: 422433. 3. i,ever WF, Schaumburg-Lever G. In: Histopathology ofthe Skin. 6th tdition. Philadelphia: JB Lippincott Company, 1983: 551. 4. Pinkus H, Mchregan AH. In: A Guide to Dermatahistopathology. 3rd edition. New York: Appleton Century, 1981: 436. 5. Flcischmann HE, Roth RJ, Wood C, Nickolofl BJ. Microcystic adnexal carcinoma treated by microscopically controlled excision. Journal of Dermatologie Surgery and Oncology 1984; 10: 873-875. 6. Cooper PH, Mills SE. Microcystic adnexal carcinoma. Journal of the American Academy of Dermatology 1984; 10: 908-914. 7. Penneys NS, Nadji M, Morales A. Carcinoembryonic antigen in benign sweat gland tumors. Archives of Dermatology 1982; 118: 225-227. 8. Penneys NS. Immunohistochemistry of adnexal neoplasms, ^owrnal of Cutaneous Pathology 1984; I I : .^57-364. 9. Cooper PH. Sclerosing carcinomas of sweat ducts (Microcystic adnexal carcinoma). Archives of Dermatology 1986; 122: 261-264.
Sclerosing-sweat-duct (microcystic adnexal) carcinoma—a tumour from a single eccrine origin L.REQUENA, A.MARQUINA,* V.ALEGRE,* A.ALIAGA* AND E.SANCHEZ YUS Departments of Dermatology, Hospital Clinico San Carlos, Madrid and * Valencia General Hospital, Valencia, Spain Accepted for publication 18 October 1989
Summary A patient with a sclerosing-sweat-duct carcinoma of the upper Hp is reported. Histologically the tumour showed solid islands and strands of squamous cells and sparse small ductal structures, some of them containing central cores of dense eosinophilic keratin. All this was embedded in a sclerotic stroma. These features, in addition to positive immunoreactivity for carcinoembryonic antigen (CEA) in the lumina of small ducts, and the presence of S100 protein-positive cells in some cords and ducts, are consistent with the notion that this tumour exhibits differentiation toward eccrine sweat structures. Microcystic adnexal carcinoma, first described by Goldstein et al.^^ refers to tumours with both follicular and sweat-gland differentiation. It is often located in the upper lip of middle-aged or elderly patients and is characterized histologically by the presence of small horny cysts, cords and ducts embedded in a sclerotic stroma. The presence of keratin-filled horny cysts lined by squamous epithelium in the upper portion of the tumour—these closely resemble follicular structures—and the pre.sence of ductal structures in the deeper areas, have suggested a dual differentiation for this tumour; both follicular and eccrine. We report a patient with sclerosing carcinoma of the sweat ducts involving the upper lip. Immunohistopathological studies showed positive immunoreactivity for carcinoembryonic antigen (CEA) and S-100 protein in some cells of ducts and solid islands. In our opinion, these findings support differentiation of this tumour solely towards eccrine structures. Case report An 81-year-old man had a slowly enlarging plaque of 2 Correspondence: Luis Requena, C/Leopoldo Alas Clarin 4-6 F, 28035-Madrid, Spain. 222
Figure 1. Clinical appearance ofthe lesion: an induniicti upper lip.
years' duration on his upper lip. On physical examination there was an erythematous and indurated plaque on the central area of the upper lip, 3 0 x 4 0 cm in size, extending from the vermiHion border to the nasal columnella (Fig. 1). The lesion had telangiectases on its surface and a striking induration on palpation. There was no apparent involvement of underlying labial mueosa nor submental or cervical adenopathy. A biopsy was performed. Histopathological examination showed the presence, in the mid- and lower dermis, of numerous solid squamouscell nests of varying sizes (Fig. 2). Some of these nests contained central cores of eosinophilic keratin (Fig. 3). Other areas ofthe tumour exhibited ductal differentiation with isolated or grouped cytoplasmic vacuoles within the squamous nests and comma-like strands of epithelial cells (Fig. 4). This biopsy specimen was interpreted as a microcystic adnexal carcinoma and the tumour was excised using permanent section control for margin evaluation. Histopathological study of complete specimen showed identical findings in the upper and middermis. In the lower dermis, there were numerous solid cords or nests of basaloid cells, without evident lumen
SCLEROSING-SWEAT-DUCT CARCINOMA
223
[•"igure 4. An epithelial nest with small dticts and a tail-like protrusion (H&K x288).
Figure 2. Numerous small epithelial nests are in the middle and the lower dermis surrounded by a sclenrtic stroma {H&l.^ x 10).
Figure i. Fpitheliat nests with central cores of dense keraiin embedded in a sclerotic stroma (H&R x29).
Figure 5. Perineural invasion. A nerve tihre is surroiindid h\ a row of basaloid cells (H&E x 288).
Figure 6. CFA-positiveluminal structure. Thesolid islandsareCI'.Anegative (diaminoben/idine haematoxylin x 154).
formation, surrounded by a sclerotic stroma. In these bundles, closely resembhng the histopathological pattern deeper portions of the neoplasm it was not unusual for of cutaneous metastatic scirrhous carcinoma. In addition fields to contain only tiny strands and clusters of basaloid to widespread dermal involvement, the neoplasm infilepithelial cells embedded between sclerotic collagen trated subcutaneous tissue, and isolated nests of basaloid
224
L.REQUENA FT AL.
Figure 7. Isolated cells are positive for S-100 protein (diaminobenzidine-haematoxilin x 154).
epithelial cells could be seen between the orbicularis oris muscle fibres. Perineural invasion was identified in some areas (Fig. 5). Immunoperoxidase staining for CEA revealed, mainly in the upper ducts, tbcally scanty staining in the lumina of small ducts or on the ductal lining surface of tumour cells (Fig. 6). There were also isolated cells of solid islands or ducts positive for S-100 protein (Fig. 7). Discussion Sclerosing-sweat-duct carcinoma u.sually aff^t'cts the face of middle-aged or elderly patients. The tumour is slowgrowing and appears as a firm or indurated plaque.' Typically this neoplasm invades deep structures, involving skeletal muscle and perineural spaces and these factors lead to a high recurrence rate unless the tumour is adequally excised. Therefore, microscopically controlled excision using Moh's technique to insure clear margins before closure has been proposed as the best therapy.^ However, in spite of this method of excision, there are reported cases^ in which a subsequent biopsy has demonstrated a recurrence. It is quite likely that in these recurrent cases after Moh's surgery, one could obtain a specimen in which the margins were completely free of tumour and yet tumour remained behind in the patient. Therefore, a wide local excision of the tumour with careful examination of all the margins of the specimen may be the treatment of choice. Histopathologically this tumour had two components. The first component consists of numerous keratin-filled horny cysts lined by squamous epithelium and the second comprises small ducts lined by two or three layers of epithelial cells, containing amorphous eosinophilic material and solid basaloid strands of eccrine-type epithelium. These findings support the view, initially proposed by Goldstein et al.^ of dual differentiation for this neoplasm: the first component would involve ex-
pression of follicular differentiation and the second would represent sweat-gland differentiation. The CEA results were also consistent with dual differentiation, because the secretory and ductal cells in eccrine and apocrine glands contain and secrete CEA, whereas foUicular structures do not.' Thus, positive islands for CEA represented eccrine differentiation and negative ones were follicle-derived structures. Nevertheless, keratinization of the upper portion ofthe intradermal eccrine duct is usually seen in syringomata' and retention milia cysts.•* The finding of S100 protein-positive cells in some cords and ducts ofthe tumour also supports an eccrine differentiation theory, because this protein is found in eccrine coil cells.^ Thus, these findings would mean that the three segments of normal eccrine sweat glands (the secretory portion, the intradermal duct and acrosyringium) are represented in the tumour. The islands negative for CEA must be interpreted bearing in mind that this antigen can be absent in purely squamous foci. In this regard, threedimensional reconstructions of these neoplasms have been proposed by Cooper** as an aid for the better understanding their histogenesis. Five of the six early cases of microcystic adnexal carcinoma' were located on the upper lip and all six cases contained horny cysts histologically, as well as duct- and gland-like structures. Later, it was obvious that these tumours might also be found in other locations and might lack horny cysts. They were then termed *sclerosingsweat-duct (syringomatous) carcinomas'.^ The case reported here has the clinical features of microcystic adnexal carcinoma and the histopathological characteristics of sclerosing-sweat-duct syringomatous carcinoma, confirming that these terms describe the same entity. References 1. Goldstein DJ, Barr RJ, Santa Cruz DJ. Microcystic adnexal carcinoma. A distinct clinicopathologic entity. Cancer 1982; 50: 566 572. 2. Cooper PH, Mills SE, Leonard DD, Santa Cruz DJ, Headington JT, Barr RJ, Katz DA. Sclcrosing sweat duct (syringomatous) carcinoma. .American Journal of Surgical Pathulngy 1985; 9: 422433. 3. i,ever WF, Schaumburg-Lever G. In: Histopathology ofthe Skin. 6th tdition. Philadelphia: JB Lippincott Company, 1983: 551. 4. Pinkus H, Mchregan AH. In: A Guide to Dermatahistopathology. 3rd edition. New York: Appleton Century, 1981: 436. 5. Flcischmann HE, Roth RJ, Wood C, Nickolofl BJ. Microcystic adnexal carcinoma treated by microscopically controlled excision. Journal of Dermatologie Surgery and Oncology 1984; 10: 873-875. 6. Cooper PH, Mills SE. Microcystic adnexal carcinoma. Journal of the American Academy of Dermatology 1984; 10: 908-914. 7. Penneys NS, Nadji M, Morales A. Carcinoembryonic antigen in benign sweat gland tumors. Archives of Dermatology 1982; 118: 225-227. 8. Penneys NS. Immunohistochemistry of adnexal neoplasms, ^owrnal of Cutaneous Pathology 1984; I I : .^57-364. 9. Cooper PH. Sclerosing carcinomas of sweat ducts (Microcystic adnexal carcinoma). Archives of Dermatology 1986; 122: 261-264.
