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Clear cell hidradenoma in a young girl
J AM ACAD DERMATOL VOLUME 42, NUMBER 4
Brief reports 693
Clear cell hidradenoma in a young girl Dorothea Faulhaber, MD,a,b Birgit Wörle, MD,a Beata Trautner, MD,a and Christian A. Sander, MDa Munich, Germany, and New York, New York Clear cell hidradenoma was diagnosed in a girl at 31⁄2 years of age. Only one case of hidradenoma has been documented in the first decade of life, although it remains unclear whether it was clear cell hidradenoma. This case demonstrates that clear cell hidradenoma is a rare differential diagnosis of cutaneous tumors even in young children, which is of special interest, because malignant transformations can occur. (J Am Acad Dermatol 2000;42:693-5.)
H
idradenomas are mostly benign neoplasms. Clear cell hidradenomas are by far the most frequent subtype of hidradenomas.1,2 Following the classification of Abenoza and Ackerman1 from 1990 and observations in the recent literature, they are mainly characterized by polygonal cells, round nuclei, clear cytoplasm, and apocrine differentiation.2,3 Usually they are diagnosed between the fourth and eighth decade of life, with a peak incidence in the sixth decade.1 We describe a young girl, in whom a clear cell hidradenoma developed at 31⁄2 years of age. Hence this case demonstrates that clear cell hidradenomas can even develop in children and should therefore be considered as a rare differential diagnosis of neoplasms in young children.
CASE REPORT A 5-year-old Turkish girl was admitted with an asymptomatic brownish-red tumor on the chest about 12 cm below the right mamilla. The lesion first appeared at the age of 31⁄2 years and had developed slowly. Examination revealed a firm and well-circumscribed tumor, partly atrophic with a shiny surface and extensive surface vascularization (Fig 1). It measured 2.5 × 2.0 cm and rested on a well-mobilized plaque (3.5 × 2.0 cm) that exhibited telangiectasia. Duplex sonography at 7.5 MHz showed a partly cystic lesion with well-vascularized areas. There was no Fig 1. Clinical appearance: Tumor with shiny and well-vascularized surface on the chest. From the Department of Dermatology, Ludwig-MaximiliansUniversity, Munich,a and the Department of Dermatology, Cornell University Medical College, New York.b Reprint requests: Dorothea Faulhaber, MD, Department of Dermatology, Cornell University Medical College, 525 E 68th St, New York, NY 10021. E-mail: [email protected]. Copyright © 2000 by the American Academy of Dermatology, Inc. 0190-9622/2000/$12.00 + 0 16/54/103274 doi:10.1067/mjd.2000.103274
evidence for abdominal angioma at 3.5 MHz sonography. The patient underwent excision of the tumor followed by plastic wound closure. Histopathologic investigation revealed a tumor in the mid and deep layers of the dermis that exhibited typical characteristics of a clear cell hidradenoma (Fig 2). The post-
694 Brief reports
J AM ACAD DERMATOL APRIL 2000
Fig 2. Histopathologically typical characteristics of a clear cell hidradenoma. Multilobulated tumor, focal large pale to clear cells with round or plump oval, often eccentrically located nuclei, surrounded by smaller cells with eosinophilic cytoplasm. Focal tubular structures. (Hematoxylin-eosin stain; original magnification ×100.)
operative history was unremarkable and no signs of recurrence were found after 12 months of follow-up.
DISCUSSION Clear cell hidradenomas are usually benign, asymptomatic, nodular, intradermal tumors and account for 95% of all hidradenomas. They have apocrine differentiation following the classification of Abenoza and Ackerman1 from 1990. The well-circumscribed and multilobulated tumors may be confined to the dermis, or they may be present in both the dermis and the subcutaneous tissue. Apocrine hidradenomas show mostly endophytic growth, in rare cases both endophytic and exophytic growth can be seen. Solid and cystic components are found in varying portions; the predominant pattern is mostly solid, the other is both solid and cystic, and in some cases tubulocystic structures are found. The main histopathologic characteristic of clear cell hidradenomas is the presence of large pale to clear cells with small monomorphous round or plump oval and often eccentrically located nuclei. The cells appear clear because of large glycogen deposits within the cytoplasm. In addition, a marked variability in the composition of squamoid and mucinous cells and tubular structures with “lining” cells showing evidence of “decapitation” secretion are findings that indicate a hidradenoma with apocrine differentiation.1-3 Usually, clear cell hidrade-
nomas grow slowly and are present for many years before diagnosis. Signs of erosion or ulceration are possible. In rare cases malignant transformation of clear cell hidradenomas with metastatic spread has been reported.4-12 To our knowledge, only one case of hidradenoma in a patient in the first decade of life has been mentioned in the literature by Abenoza and Ackerman,1 who reported an age range from 3 to 93 years in a study with 219 hidradenomas. However, in the same study a zero prevalence of hidradenomas is reported for the first decade of life. In addition, if there was a patient at the age of 3 years, it is unclear whether this child had the clear cell hidradenoma subtype. It is important to consider clear cell hidradenomas as a rare differential diagnosis of cutaneous tumors even in young children, especially since in some cases a malignant transformation has been observed. The differential diagnosis includes eccrine hidradenoma, hemangioma, glomus tumor, cutaneous lymphoma, dermatofibrosarcoma protuberans, leiomyoma, follicular cyst, tricholemmoma, and a sweat gland tumor. Clear cell hidradenomas should be surgically removed. Because they have a high local recurrence rate and the potential of malignant transformation, safe margins should be applied during resection.5,11,13
Brief reports 695
J AM ACAD DERMATOL VOLUME 42, NUMBER 4
REFERENCES 1. Abenoza P, Ackerman AB. Hidradenomas. In: Abenoza P, Ackerman AB, editors. Neoplasms with eccrine differentiation. Philadelphia: Lea & Febiger; 1990. p. 311-50. 2. Gianotti R, Alessi E. Clear cell hidradenoma associated with the folliculo-sebaceous-apocrine unit. Am J Dermatopathol 1997; 19:351-7. 3. Requena L, Kiryu H, Ackerman AB. Apocrine hidradenoma. In: Requena L, Kiryu H, Ackerman AB, editors. Neoplasms with apocrine differentiation. Philadelphia: Lippincott-Raven; 1997. p. 243-6. 4. Biddlestone LR, McLaren KM, Tidman MJ. Malignant hidradenoma: a case report demonstrating insidious histological and clinical progression. Clin Exp Dermatol 1991;16:474-7. 5. Cooper PH. Carcinomas of sweat glands. Pathol Annu 1987;22:83-124. 6. Gartmann H, Tritsh H. Nodulares Hidradenom mit krebsiger Umwandlung. Z Hautkr 1971;46:163-8. 7. Kazakis A, Wood C, Anderson R. Metastatic malignant clear cell hidradenoma associated with bullous pemphigoid. Br J Dermatol 1988;118:819-26.
8. Lopez-Burbano LF, Cimorra GA, Gonzalez-Peirona E, Alfaro J. Malignant clear-cell hidradenoma. Plast Reconstr Surg 1987;80: 300-3. 9. Schroeder WA, Hosler MW. Malignant clear cell hidradenoma of the lip. Mil Med 1989;154:508-11. 10. Schweitzer WJ, Goldin HM, Bronson DM. Ulcerated tumor on the scalp: clear-cell hidradenoma. Arch Dermatol 1989;125:9856. 11. Stratigos AJ, Olbricht S, Kwan TH, Bowers KE. Nodular hidradenoma: a report of three cases and review of the literature. Dermatol Surg 1998;24:387-91. 12. Touma D, Laporte M, Goossens A, Ledoux M. Malignant clear cell hidradenoma. Dermatology 1993;186:284-6. 13. Kato N, Ueno H. Clear cell hidradenoma: a tumor with basaliomatous changes in the overlying epidermis and follicular infundibula of surrounding skin. J Dermatol 1992;19:436-42.
AMERICAN BOARD OF DERMATOLOGY EXAMINATION DATES In 2000, the Certifying Examination of the American Board of Dermatology (ABD) will be held at the Holiday Inn O’Hare International in Rosemont, Illinois, on Oct 29 and 30, 2000. The deadline for receipt of applications is May 1, 2000. The next examination for subspecialty certification in Dermatopathology will be held in Tampa, Florida, on Friday, Nov 10, 2000. The deadline for receipt of applications is July 1, 2000. The date of the next examination for subspecialty certification in Clinical and Laboratory Dermatological Immunology has not been determined. The next Recertification Examination of the ABD will be held in 2001. The deadline for receipt of applications is March 1, 2001. For further information about these examinations, please contact: Harry J. Hurley, MD Executive Director American Board of Dermatology Henry Ford Hospital 1 Ford Place Detroit, MI 48202-3450 Telephone: 313-874-1088 Fax: 313-872-3221
Brief reports 693
Clear cell hidradenoma in a young girl Dorothea Faulhaber, MD,a,b Birgit Wörle, MD,a Beata Trautner, MD,a and Christian A. Sander, MDa Munich, Germany, and New York, New York Clear cell hidradenoma was diagnosed in a girl at 31⁄2 years of age. Only one case of hidradenoma has been documented in the first decade of life, although it remains unclear whether it was clear cell hidradenoma. This case demonstrates that clear cell hidradenoma is a rare differential diagnosis of cutaneous tumors even in young children, which is of special interest, because malignant transformations can occur. (J Am Acad Dermatol 2000;42:693-5.)
H
idradenomas are mostly benign neoplasms. Clear cell hidradenomas are by far the most frequent subtype of hidradenomas.1,2 Following the classification of Abenoza and Ackerman1 from 1990 and observations in the recent literature, they are mainly characterized by polygonal cells, round nuclei, clear cytoplasm, and apocrine differentiation.2,3 Usually they are diagnosed between the fourth and eighth decade of life, with a peak incidence in the sixth decade.1 We describe a young girl, in whom a clear cell hidradenoma developed at 31⁄2 years of age. Hence this case demonstrates that clear cell hidradenomas can even develop in children and should therefore be considered as a rare differential diagnosis of neoplasms in young children.
CASE REPORT A 5-year-old Turkish girl was admitted with an asymptomatic brownish-red tumor on the chest about 12 cm below the right mamilla. The lesion first appeared at the age of 31⁄2 years and had developed slowly. Examination revealed a firm and well-circumscribed tumor, partly atrophic with a shiny surface and extensive surface vascularization (Fig 1). It measured 2.5 × 2.0 cm and rested on a well-mobilized plaque (3.5 × 2.0 cm) that exhibited telangiectasia. Duplex sonography at 7.5 MHz showed a partly cystic lesion with well-vascularized areas. There was no Fig 1. Clinical appearance: Tumor with shiny and well-vascularized surface on the chest. From the Department of Dermatology, Ludwig-MaximiliansUniversity, Munich,a and the Department of Dermatology, Cornell University Medical College, New York.b Reprint requests: Dorothea Faulhaber, MD, Department of Dermatology, Cornell University Medical College, 525 E 68th St, New York, NY 10021. E-mail: [email protected]. Copyright © 2000 by the American Academy of Dermatology, Inc. 0190-9622/2000/$12.00 + 0 16/54/103274 doi:10.1067/mjd.2000.103274
evidence for abdominal angioma at 3.5 MHz sonography. The patient underwent excision of the tumor followed by plastic wound closure. Histopathologic investigation revealed a tumor in the mid and deep layers of the dermis that exhibited typical characteristics of a clear cell hidradenoma (Fig 2). The post-
694 Brief reports
J AM ACAD DERMATOL APRIL 2000
Fig 2. Histopathologically typical characteristics of a clear cell hidradenoma. Multilobulated tumor, focal large pale to clear cells with round or plump oval, often eccentrically located nuclei, surrounded by smaller cells with eosinophilic cytoplasm. Focal tubular structures. (Hematoxylin-eosin stain; original magnification ×100.)
operative history was unremarkable and no signs of recurrence were found after 12 months of follow-up.
DISCUSSION Clear cell hidradenomas are usually benign, asymptomatic, nodular, intradermal tumors and account for 95% of all hidradenomas. They have apocrine differentiation following the classification of Abenoza and Ackerman1 from 1990. The well-circumscribed and multilobulated tumors may be confined to the dermis, or they may be present in both the dermis and the subcutaneous tissue. Apocrine hidradenomas show mostly endophytic growth, in rare cases both endophytic and exophytic growth can be seen. Solid and cystic components are found in varying portions; the predominant pattern is mostly solid, the other is both solid and cystic, and in some cases tubulocystic structures are found. The main histopathologic characteristic of clear cell hidradenomas is the presence of large pale to clear cells with small monomorphous round or plump oval and often eccentrically located nuclei. The cells appear clear because of large glycogen deposits within the cytoplasm. In addition, a marked variability in the composition of squamoid and mucinous cells and tubular structures with “lining” cells showing evidence of “decapitation” secretion are findings that indicate a hidradenoma with apocrine differentiation.1-3 Usually, clear cell hidrade-
nomas grow slowly and are present for many years before diagnosis. Signs of erosion or ulceration are possible. In rare cases malignant transformation of clear cell hidradenomas with metastatic spread has been reported.4-12 To our knowledge, only one case of hidradenoma in a patient in the first decade of life has been mentioned in the literature by Abenoza and Ackerman,1 who reported an age range from 3 to 93 years in a study with 219 hidradenomas. However, in the same study a zero prevalence of hidradenomas is reported for the first decade of life. In addition, if there was a patient at the age of 3 years, it is unclear whether this child had the clear cell hidradenoma subtype. It is important to consider clear cell hidradenomas as a rare differential diagnosis of cutaneous tumors even in young children, especially since in some cases a malignant transformation has been observed. The differential diagnosis includes eccrine hidradenoma, hemangioma, glomus tumor, cutaneous lymphoma, dermatofibrosarcoma protuberans, leiomyoma, follicular cyst, tricholemmoma, and a sweat gland tumor. Clear cell hidradenomas should be surgically removed. Because they have a high local recurrence rate and the potential of malignant transformation, safe margins should be applied during resection.5,11,13
Brief reports 695
J AM ACAD DERMATOL VOLUME 42, NUMBER 4
REFERENCES 1. Abenoza P, Ackerman AB. Hidradenomas. In: Abenoza P, Ackerman AB, editors. Neoplasms with eccrine differentiation. Philadelphia: Lea & Febiger; 1990. p. 311-50. 2. Gianotti R, Alessi E. Clear cell hidradenoma associated with the folliculo-sebaceous-apocrine unit. Am J Dermatopathol 1997; 19:351-7. 3. Requena L, Kiryu H, Ackerman AB. Apocrine hidradenoma. In: Requena L, Kiryu H, Ackerman AB, editors. Neoplasms with apocrine differentiation. Philadelphia: Lippincott-Raven; 1997. p. 243-6. 4. Biddlestone LR, McLaren KM, Tidman MJ. Malignant hidradenoma: a case report demonstrating insidious histological and clinical progression. Clin Exp Dermatol 1991;16:474-7. 5. Cooper PH. Carcinomas of sweat glands. Pathol Annu 1987;22:83-124. 6. Gartmann H, Tritsh H. Nodulares Hidradenom mit krebsiger Umwandlung. Z Hautkr 1971;46:163-8. 7. Kazakis A, Wood C, Anderson R. Metastatic malignant clear cell hidradenoma associated with bullous pemphigoid. Br J Dermatol 1988;118:819-26.
8. Lopez-Burbano LF, Cimorra GA, Gonzalez-Peirona E, Alfaro J. Malignant clear-cell hidradenoma. Plast Reconstr Surg 1987;80: 300-3. 9. Schroeder WA, Hosler MW. Malignant clear cell hidradenoma of the lip. Mil Med 1989;154:508-11. 10. Schweitzer WJ, Goldin HM, Bronson DM. Ulcerated tumor on the scalp: clear-cell hidradenoma. Arch Dermatol 1989;125:9856. 11. Stratigos AJ, Olbricht S, Kwan TH, Bowers KE. Nodular hidradenoma: a report of three cases and review of the literature. Dermatol Surg 1998;24:387-91. 12. Touma D, Laporte M, Goossens A, Ledoux M. Malignant clear cell hidradenoma. Dermatology 1993;186:284-6. 13. Kato N, Ueno H. Clear cell hidradenoma: a tumor with basaliomatous changes in the overlying epidermis and follicular infundibula of surrounding skin. J Dermatol 1992;19:436-42.
AMERICAN BOARD OF DERMATOLOGY EXAMINATION DATES In 2000, the Certifying Examination of the American Board of Dermatology (ABD) will be held at the Holiday Inn O’Hare International in Rosemont, Illinois, on Oct 29 and 30, 2000. The deadline for receipt of applications is May 1, 2000. The next examination for subspecialty certification in Dermatopathology will be held in Tampa, Florida, on Friday, Nov 10, 2000. The deadline for receipt of applications is July 1, 2000. The date of the next examination for subspecialty certification in Clinical and Laboratory Dermatological Immunology has not been determined. The next Recertification Examination of the ABD will be held in 2001. The deadline for receipt of applications is March 1, 2001. For further information about these examinations, please contact: Harry J. Hurley, MD Executive Director American Board of Dermatology Henry Ford Hospital 1 Ford Place Detroit, MI 48202-3450 Telephone: 313-874-1088 Fax: 313-872-3221