CASE REPORTS PAUL A. LEVINE, MD Case Report Editor
Microcystic adnexal carcinoma of the lower lip NEAL D. FUTRAN, MD. DMD. VITO C. QUATELA, MD. STEPHEN E. PRESSER, MD. and JAN E. MUHLBAUER, MD.
Rochester, New York
Basal cell carcinoma is the most frequently seen cutaneous malignancy seen on the face. Though rare, neoplasms of eccrine derivation may also be seen. Microcystic adnexal carcinoma (MAC) was first described by Goldstein et al.' in 1982. It is an unusual cutaneous neoplasm thought to be derived from both pilar and eccrine sweat structures. This locally aggressive tumor has a prediliction for the face-particularly the upper lip. It may also be seen on other areas of the face, axilla, and breast. Of the 41 cases of this neoplasm described in the literature, 1~18 only one was manifested on the lower lip. 12 This report was initially published in 1975 as an unusual basal cell carcinoma, but in retrospect, was thought to be MAC. This neoplasm can be clinically and histologically confused with other malignant and benign cutaneous neoplasms, which may lead to inadequate initial treatment with subsequent recurrence. We present another rare case of MAC of the lower lip. CASE REPORT
S.M. was an 84-year-oldman who had a T1NoMo indurated painless lesion of the lower lip. This lesion had been present for several months, and its persistent, slow growth initiated the patient's desire for treatment. The patient's medical history was significant for severe congestive heart failure and hypertension, which were medically controlled.
From the Departments of Otolaryngology-Head and Neck Surgery (Drs. Futran and Quatela), Dermatology (Dr. Presser), and Pathology (Dr. Muhlbauer), University of Rochester Medical Center. Presented at the Annual Meeting of the American Academy of Otolaryngology-Head and Neck Surgery, Kansas City, Mo., Sept. 2226. 1991. Received for publication Sept. 22, 1991; revision received Feb. 2, 1992; accepted Feb. 12, 1992. Reprint requests: Neal D. Futran, MD, DMD, 7885 Hidden Oaks, Pittsford, NY 14534.
23/4/37319
The lesion was initially treated at another institution by wedge resection and primary closure. Pathologicevaluation, however, revealed MAC and a positive deep margin. Performance of three stages of Mohs surgery necessary to completely excise the neoplasm resulted in a full-thickness loss of 75% of the lower lip. Reconstruction was achieved by use of bilateral Karapandzic flaps and a midline lip switch procedure from the upper lip to the lower lip." All flaps healed well, and microstomia was minimal. Histologic sections showed an atypical epithelial proliferation widely infiltratingthe reticular dermis beneath a large ulceration. The tumor exhibited prominent ductal differentiation with "tadpole-like" structures in some areas, whereas other portions of the tumor displayed hom cysts and dyskeratosis (Fig. I). Tumor cells exhibited both skeletal and perineural invasion. The cytologic characteristicswere relatively bland, with mild nuclear pleomorphism and only a few mitoses. The stromawas largely sclerotic, with a patchyinfiltrate of lymphocytes and plasma cells. Though the patient's wounds healed well, within a month of the surgery, severe congestive heart failure developed. His condition was refractory to maximal medical therapy and the patient died 6 weeks after tumor removal and reconstruction, free of disease. DISCUSSION
MAC was first described as a distinct entity by Goldstein et al. 1 in 1982. The tumor appeared to exhibit both follicular and sweat gland differentiation, and the cell of origin might be a pluripotential adnexal keratinocyte. Forty-one cases of this entity have been described in the literature. 118 All have similar clinical and pathologic features of MAC, but additional synonyms such as scle-
rosing sweat duct carcinoma, syringomatous carcinoma, and sweat gland carcinoma have been used. Fifteen cases are defined as MAC,I-9 eleven cases are defined as sclerosing sweat duct carcinoma," and the other case reports are described by different names,":" but with similar findings to MAC.
457
458
OfolaryngologyHead and Neck Surgery
Case Reports
Fig.i. Photomicrograph of infiltrating tumor cells, with focal ductal differentiation and sclerotic stroma. (Hematoxylin-eosin stain; original magnification x 125.)
Patients have manifested MAC from the second to the eighth decade, but most patients are located in the older age groups. Though originally thought to have a prediliction for women, MAC appears equally in both sexes. The large majority of MACs appear on the face, with most lesions occurring on the upper lip and nasolabial area. This neoplasm appears as a solitary, slowly growing, smooth-surfaced, indurated nodule or plaque. Cooper et al. 10 described one patient whose lesion was present for 17 years before medical consultation was sought. The innocuous clinical appearance, however, belies the infiltrative and locally aggressive nature of MAC. MAC exhibits distinct histopathological findings.l.2.6.7.10 Cords and islands of squamous and basaloid cells are seen throughout the dermis and are embedded in hyalinized stroma. Ductal elements vary from tumor to tumor, but are usually lined by two layers of cuboidal cells. Cornification of cysts in the superficial portions of the tumor is present. A desmoplastic stroma and sclerosis are seen, but cells exhibit few mitotic figures and little nuclear atypia. Some authors':' have used carcinoembryonic antigen (CEA) and S-100 protein to support the diagnosis of MAC. Positive immunoreactivity for CEA present in the lumina of small ducts, and the presence of S-100
protein-positive cells in some cords and ducts of the tumor, support an eccrine origin of MAC. Of great significance is the invasion of neoplastic cells into subcutaneous tissues, skeletal muscle, fat, perineural spaces, and nerve fasicles. Though locally aggressive, only one case of MAC has invaded bone," and one case spread to a regional lymph node" thought to be the result of direct perineural invasion. The mainstay of treatment for MAC has been surgical excision. Primary radiation therapy has been used with little benefit."? Because MAC exhibits deep invasion into skeletal muscle and neural structures, Mohs micrographically controlled surgery has been the recommended treatment for total extirpation of tumor. 3.6.7,9 The use of postoperative radiotherapy to prevent recurrence is inconclusive. 2,9 Despite all efforts, however, the recurrence rate of MAC is substantial at 40%. Late recurrence (more than 3 years after initial treatment) is not uncommon. Lupton and Mclvlarlin' even reported a recurrence after 30 years offollowup. Though recurrence is significant, no deaths have been attributed to MAC. The case reported here exhibits classic features of MAC, except in its location on the lower lip. The locally aggressive nature of MAC leads to a larger surgical defect than originally anticipated, and can create a for-
Volume 107 Number 3 september 1992
midable reconstructive effort, as seen in this patient. Though rare, MAC should be included in the differential diagnosis for cutaneous lesions on the face. Even after surgical extirpation and reconstruction, the high recurrence rate of MAC indicates the need for lifetime followup. REFERENCES
I. Goldstein DJ, Barr RJ, Santa Cruz DJ. Microcystic adnexal carcinoma: a distinct clinicopathologic entity. Cancer 1982;50:566-72. 2. Cooper PH, Mills SE. Microcystic adnexal carcinoma. J Am Acad Dermatol 1984;10:908-14. 3. Fleischmann HE, Roth RJ, Wood C, Nickoloff BJ. Microcystic adnexal carcinoma treated by microscopically controlled excision. J Dermatol Surg Oncol 1984;10:873-5. 4. Rongioletti F, Grosshans E, Rebora A. Microcystic adnexal carcinoma. BrJ DermatoI1986;115:101-4. 5. Lupton GP, MeMarlin SL. Microcystic adnexal carcinoma: report of a case with 30-year followup. Arch Dermatol 1986;122:286-9. 6. Nickoloff BJ, Fleischmann HE, Carmel J, et al. Microcystic adnexal carcinoma: Immunohistologic observations suggesting dual (pilar and eccrine) differentiation. Arch Dermatol 1986;122:290-4. 7. Mayer MH, Winton GB, Smith AC, Lupton GP, Parry EL, Shagets FW. Microcystic adnexal carcinoma (sclerosing sweat duct carcinoma). Plast Reconstr Surg 1989;84:970-5.
Case Reports
459
8. Requina L, Marquina A, Alegre V, Aliaga A, Sanchez Yus E. Sclerosing-sweat-duct (microcystic adnexal) carcinoma-a tumor from a single eccrine origin. Clin Exper Dermatol 1990;15:222-4. 9. Birkby CS, Argenyi ZB, Whitaker DC. Microcystic adnexal carcinoma with mandibular invasion and bone marrow replacement. J Dermatol Surg Oncol 1989;15:308-12. 10. Cooper PH, Mills SE, Leonard DD, et al. Sclerosing sweat duct (syringomatous) carcinoma. Am J Surg Pathol 1985;9:422-33. II. Gulmen S, Pullon PA. Sweat gland carcinoma of the lips. Oral Surg Oral Pathol Oral Med 1976;41:643-9. 12. Kelly DE, Klein KM, Harrigan WF. Lip reconstruction following resection for an unusual basal cell carcinoma. Oral Surg Oral Pathol Oral Med 1975;40:19-26. 13. Lipper S, Peiper Sc. Sweat gland carcinoma with syringomatous feature: A light microscopic and ultrastructural study. Cancer 1979;44:157-63. 14. Stem lB, Stout DA. Trichofolliculma showing perineural invasion: trichofolliculocarcinoma? Arch Dermatol 1979;115: 1003-4. 15. Werthheimer FW, Mintz SM. Solitary trichoepithelioma of the upper lip: report of case. J Oral Surg 1972;30:215-6. 16. Apisamthanaraz P, Bovenmyer DA, Mehregan AH. Combined adnexal tumor of the skin. Arch Dermatol 1984;120:231-3. 17. Stout AP, Cooley SGE. Carcinoma of sweat glands. Cancer 1951;4:521-36. 18. Cottel WI. Eccrine epithelioma: Case report. J Dermatol Surg Oncol 1982;8:610-1. 19. Jackson I. Facial flaps in head and neck reconstruction. 1st ed. St. Louis: C.Y. Mosby Co., 1985:357-60,380-2.
Microcystic adnexal carcinoma of the lower lip NEAL D. FUTRAN, MD. DMD. VITO C. QUATELA, MD. STEPHEN E. PRESSER, MD. and JAN E. MUHLBAUER, MD.
Rochester, New York
Basal cell carcinoma is the most frequently seen cutaneous malignancy seen on the face. Though rare, neoplasms of eccrine derivation may also be seen. Microcystic adnexal carcinoma (MAC) was first described by Goldstein et al.' in 1982. It is an unusual cutaneous neoplasm thought to be derived from both pilar and eccrine sweat structures. This locally aggressive tumor has a prediliction for the face-particularly the upper lip. It may also be seen on other areas of the face, axilla, and breast. Of the 41 cases of this neoplasm described in the literature, 1~18 only one was manifested on the lower lip. 12 This report was initially published in 1975 as an unusual basal cell carcinoma, but in retrospect, was thought to be MAC. This neoplasm can be clinically and histologically confused with other malignant and benign cutaneous neoplasms, which may lead to inadequate initial treatment with subsequent recurrence. We present another rare case of MAC of the lower lip. CASE REPORT
S.M. was an 84-year-oldman who had a T1NoMo indurated painless lesion of the lower lip. This lesion had been present for several months, and its persistent, slow growth initiated the patient's desire for treatment. The patient's medical history was significant for severe congestive heart failure and hypertension, which were medically controlled.
From the Departments of Otolaryngology-Head and Neck Surgery (Drs. Futran and Quatela), Dermatology (Dr. Presser), and Pathology (Dr. Muhlbauer), University of Rochester Medical Center. Presented at the Annual Meeting of the American Academy of Otolaryngology-Head and Neck Surgery, Kansas City, Mo., Sept. 2226. 1991. Received for publication Sept. 22, 1991; revision received Feb. 2, 1992; accepted Feb. 12, 1992. Reprint requests: Neal D. Futran, MD, DMD, 7885 Hidden Oaks, Pittsford, NY 14534.
23/4/37319
The lesion was initially treated at another institution by wedge resection and primary closure. Pathologicevaluation, however, revealed MAC and a positive deep margin. Performance of three stages of Mohs surgery necessary to completely excise the neoplasm resulted in a full-thickness loss of 75% of the lower lip. Reconstruction was achieved by use of bilateral Karapandzic flaps and a midline lip switch procedure from the upper lip to the lower lip." All flaps healed well, and microstomia was minimal. Histologic sections showed an atypical epithelial proliferation widely infiltratingthe reticular dermis beneath a large ulceration. The tumor exhibited prominent ductal differentiation with "tadpole-like" structures in some areas, whereas other portions of the tumor displayed hom cysts and dyskeratosis (Fig. I). Tumor cells exhibited both skeletal and perineural invasion. The cytologic characteristicswere relatively bland, with mild nuclear pleomorphism and only a few mitoses. The stromawas largely sclerotic, with a patchyinfiltrate of lymphocytes and plasma cells. Though the patient's wounds healed well, within a month of the surgery, severe congestive heart failure developed. His condition was refractory to maximal medical therapy and the patient died 6 weeks after tumor removal and reconstruction, free of disease. DISCUSSION
MAC was first described as a distinct entity by Goldstein et al. 1 in 1982. The tumor appeared to exhibit both follicular and sweat gland differentiation, and the cell of origin might be a pluripotential adnexal keratinocyte. Forty-one cases of this entity have been described in the literature. 118 All have similar clinical and pathologic features of MAC, but additional synonyms such as scle-
rosing sweat duct carcinoma, syringomatous carcinoma, and sweat gland carcinoma have been used. Fifteen cases are defined as MAC,I-9 eleven cases are defined as sclerosing sweat duct carcinoma," and the other case reports are described by different names,":" but with similar findings to MAC.
457
458
OfolaryngologyHead and Neck Surgery
Case Reports
Fig.i. Photomicrograph of infiltrating tumor cells, with focal ductal differentiation and sclerotic stroma. (Hematoxylin-eosin stain; original magnification x 125.)
Patients have manifested MAC from the second to the eighth decade, but most patients are located in the older age groups. Though originally thought to have a prediliction for women, MAC appears equally in both sexes. The large majority of MACs appear on the face, with most lesions occurring on the upper lip and nasolabial area. This neoplasm appears as a solitary, slowly growing, smooth-surfaced, indurated nodule or plaque. Cooper et al. 10 described one patient whose lesion was present for 17 years before medical consultation was sought. The innocuous clinical appearance, however, belies the infiltrative and locally aggressive nature of MAC. MAC exhibits distinct histopathological findings.l.2.6.7.10 Cords and islands of squamous and basaloid cells are seen throughout the dermis and are embedded in hyalinized stroma. Ductal elements vary from tumor to tumor, but are usually lined by two layers of cuboidal cells. Cornification of cysts in the superficial portions of the tumor is present. A desmoplastic stroma and sclerosis are seen, but cells exhibit few mitotic figures and little nuclear atypia. Some authors':' have used carcinoembryonic antigen (CEA) and S-100 protein to support the diagnosis of MAC. Positive immunoreactivity for CEA present in the lumina of small ducts, and the presence of S-100
protein-positive cells in some cords and ducts of the tumor, support an eccrine origin of MAC. Of great significance is the invasion of neoplastic cells into subcutaneous tissues, skeletal muscle, fat, perineural spaces, and nerve fasicles. Though locally aggressive, only one case of MAC has invaded bone," and one case spread to a regional lymph node" thought to be the result of direct perineural invasion. The mainstay of treatment for MAC has been surgical excision. Primary radiation therapy has been used with little benefit."? Because MAC exhibits deep invasion into skeletal muscle and neural structures, Mohs micrographically controlled surgery has been the recommended treatment for total extirpation of tumor. 3.6.7,9 The use of postoperative radiotherapy to prevent recurrence is inconclusive. 2,9 Despite all efforts, however, the recurrence rate of MAC is substantial at 40%. Late recurrence (more than 3 years after initial treatment) is not uncommon. Lupton and Mclvlarlin' even reported a recurrence after 30 years offollowup. Though recurrence is significant, no deaths have been attributed to MAC. The case reported here exhibits classic features of MAC, except in its location on the lower lip. The locally aggressive nature of MAC leads to a larger surgical defect than originally anticipated, and can create a for-
Volume 107 Number 3 september 1992
midable reconstructive effort, as seen in this patient. Though rare, MAC should be included in the differential diagnosis for cutaneous lesions on the face. Even after surgical extirpation and reconstruction, the high recurrence rate of MAC indicates the need for lifetime followup. REFERENCES
I. Goldstein DJ, Barr RJ, Santa Cruz DJ. Microcystic adnexal carcinoma: a distinct clinicopathologic entity. Cancer 1982;50:566-72. 2. Cooper PH, Mills SE. Microcystic adnexal carcinoma. J Am Acad Dermatol 1984;10:908-14. 3. Fleischmann HE, Roth RJ, Wood C, Nickoloff BJ. Microcystic adnexal carcinoma treated by microscopically controlled excision. J Dermatol Surg Oncol 1984;10:873-5. 4. Rongioletti F, Grosshans E, Rebora A. Microcystic adnexal carcinoma. BrJ DermatoI1986;115:101-4. 5. Lupton GP, MeMarlin SL. Microcystic adnexal carcinoma: report of a case with 30-year followup. Arch Dermatol 1986;122:286-9. 6. Nickoloff BJ, Fleischmann HE, Carmel J, et al. Microcystic adnexal carcinoma: Immunohistologic observations suggesting dual (pilar and eccrine) differentiation. Arch Dermatol 1986;122:290-4. 7. Mayer MH, Winton GB, Smith AC, Lupton GP, Parry EL, Shagets FW. Microcystic adnexal carcinoma (sclerosing sweat duct carcinoma). Plast Reconstr Surg 1989;84:970-5.
Case Reports
459
8. Requina L, Marquina A, Alegre V, Aliaga A, Sanchez Yus E. Sclerosing-sweat-duct (microcystic adnexal) carcinoma-a tumor from a single eccrine origin. Clin Exper Dermatol 1990;15:222-4. 9. Birkby CS, Argenyi ZB, Whitaker DC. Microcystic adnexal carcinoma with mandibular invasion and bone marrow replacement. J Dermatol Surg Oncol 1989;15:308-12. 10. Cooper PH, Mills SE, Leonard DD, et al. Sclerosing sweat duct (syringomatous) carcinoma. Am J Surg Pathol 1985;9:422-33. II. Gulmen S, Pullon PA. Sweat gland carcinoma of the lips. Oral Surg Oral Pathol Oral Med 1976;41:643-9. 12. Kelly DE, Klein KM, Harrigan WF. Lip reconstruction following resection for an unusual basal cell carcinoma. Oral Surg Oral Pathol Oral Med 1975;40:19-26. 13. Lipper S, Peiper Sc. Sweat gland carcinoma with syringomatous feature: A light microscopic and ultrastructural study. Cancer 1979;44:157-63. 14. Stem lB, Stout DA. Trichofolliculma showing perineural invasion: trichofolliculocarcinoma? Arch Dermatol 1979;115: 1003-4. 15. Werthheimer FW, Mintz SM. Solitary trichoepithelioma of the upper lip: report of case. J Oral Surg 1972;30:215-6. 16. Apisamthanaraz P, Bovenmyer DA, Mehregan AH. Combined adnexal tumor of the skin. Arch Dermatol 1984;120:231-3. 17. Stout AP, Cooley SGE. Carcinoma of sweat glands. Cancer 1951;4:521-36. 18. Cottel WI. Eccrine epithelioma: Case report. J Dermatol Surg Oncol 1982;8:610-1. 19. Jackson I. Facial flaps in head and neck reconstruction. 1st ed. St. Louis: C.Y. Mosby Co., 1985:357-60,380-2.
