BRIEF REPORT
Eccrine Poroma and Eccrine Porocarcinoma in Linear Epidermal Nevus Jiehyun Jeon, MD, PhD,* Joo Ha Kim, MD,* Yoo Sang Baek, MD,* Aeree Kim, MD, PhD,† Soo Hong Seo, MD, PhD,* and Chil Hwan Oh, MD, PhD*
(Am J Dermatopathol 2014;36:430–432)
INTRODUCTION Epidermal nevi arise from pluripotential germinative cells in the basal layer of the embryonic epidermis. These cells give rise to keratinocytes and skin appendages such as sebaceous glands, hair follicles, and apocrine glands. Therefore, epidermal nevi can be classified according to their predominant components, and terms such as nevus verrucosus, nevus sebaceus, nevus comedonicus, and nevus syringocystadenosus papilliferus are given to appropriate lesions.1 However, the division is not absolute, and overlap may be seen both clinically and histologically between classifications, forming a continuous spectrum.2 There are occasional reports of benign or malignant tumors developing in the preexisting verrucous, solitary, or linear epidermal nevi. However, these secondary neoplasms seem to be much rarer in epidermal nevi than in sebaceous or apocrine nevi.2 The majority of neoplasms that originate in epidermal nevi are epithelial tumors such as basal cell carcinoma, keratoacanthoma, Bowen disease, and squamous cell carcinoma. Skin appendageal secondary tumors are rare, and to the best of our knowledge, only 1 case report of eccrine poroma and eccrine porocarcinoma that developed within a linear epidermal nevus has been published.3
The initial biopsy specimen was obtained from the nodular lesion and from the brownish patch. Histopathology of the biopsy taken from the protruding nodule showed an ulcerated surface and tumor mass, which was continuous with the surface epithelium. The tumorous lesion consisted of anaplastic cells with relatively pale and large nuclei intermingled with cuboidal cells in an anastomosing band-like arrangement, accompanied by occasional mitosis and a gray-to-violaceous mucinous substance, which suggested a malignant lesion of sweat gland origin. The whole lesion was infiltrated with heavy inflammatory cells including plasma cells (Fig. 2). The other biopsy specimen from the patch lesion had features of seborrheic keratosis with pigmentation, acanthosis, and horn pseudocysts, which were consistent with epidermal nevus (Fig. 3). Later, wide excisions of the malignant nodule and a small, erythematous, friable-looking plaque located on the lower border of the posterior part of epidermal nevus were performed. The dermatopathologic findings of the excised nodule were similar to the findings of the previously biopsied lesion, and a diagnosis of eccrine porocarcinoma was made. In addition, the excised erythematous plaque lesion demonstrated characteristics of eccrine poroma, as it was made up of monomorphous cuboidal and basaloid cells downgrowing from the multifocal attachment to the epidermis, forming occasional ductal structures (Fig. 4). With multiple tumor formation at different loci on the existing epidermal nevus, we suspected the possibility that the patient would develop another malignant lesion remained relatively high. We recommended prophylactic complete excision of the epidermal nevus, but he refused further surgical treatment. Instead, the patient
CASE REPORT A 74-year-old man visited our clinic with a linear epidermal nevus after the lines of Blaschko on his right lower flank area. The linear epidermal nevus was discontinuous near the frontal one-third of the lesion and slightly verrucous on the surface with several darkly pigmented spots. The patient’s chief complaint was an oozing protruding erythematous nodule on the forefront marginal area of the epidermal nevus (Fig. 1), which had been present for 1 year. He had suffered from comorbidities including abdominal aortic dissection, unstable angina, hypertension, and usual interstitial pneumonia. Examination of the skin revealed no remarkable findings except for the previously described lesions. No palpable lymph nodes were detected. From the Departments of *Dermatology, and †Pathology, Korea University College of Medicine, Seoul, Korea. The authors declare no conflicts of interest. Reprints: Chil Hwan Oh, MD, PhD, Department of Dermatology, Korea University College of Medicine, 148 Gurodong-ro, Guro-dong, Guro-gu, Seoul 152-703, Korea (e-mail: [email protected]). © 2014 Lippincott Williams & Wilkins
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| www.amjdermatopathology.com
FIGURE 1. Linear epidermal nevi after the lines of Blaschko on patient’s right lower flank area with a protruding erythematous nodule on the forefront marginal area and a small erythematous plaque on the lower border of the posterior part of epidermal nevi. Am J Dermatopathol Volume 36, Number 5, May 2014
Am J Dermatopathol Volume 36, Number 5, May 2014
Eccrine Neoplasms in Linear Epidermal Nevus
FIGURE 2. A. The tumor nodule with an ulcerated surface is continuous with the surface epithelium. Tumor nests extend into the dermis and are accompanied by heavy inflammation (H&E, ·15). B. High power magnification of the demarcated area. Tumor nests originating in the epidermis extend into the dermis with some central necrosis and cells that consist of the tumor nests show nuclear atypia and mitosis (arrows) (H&E, ·200). consented to regular follow-up for the surveillance of any nodule, erosion, or ulceration on the epidermal nevus.
DISCUSSION Most epidermal nevi are small and asymptomatic and do not cause serious problems throughout life.2 The incidence of secondary tumors developing in epidermal nevi is low, and malignancies associated with epidermal nevi are rare. However, epidermal nevi can be regarded as the precursors of epidermal and appendageal neoplasm formations. The term “epidermal nevus” is designated for nevi composed of keratinocytes.4 However, Solomon and Esterly pointed out that very few epidermal nevi are exclusively of 1 type,5 and
FIGURE 3. Histopathologic features of seborrheic keratosis with pigmentation, acanthosis, and horn pseudocysts from a biopsy specimen harvested from the patch lesion of the epidermal nevi (H&E, ·40). 2014 Lippincott Williams & Wilkins
various types of tumor growth have been reported sporadically on epidermal nevi such as squamous cell carcinoma,2,6 Bowen disease,7 basal cell epithelioma,8–10 keratoacanthoma,11 eccrine poroma,3 and eccrine porocarcinoma.3 However, such tumors seem to be much more rare than in sebaceous or apocrine nevi.2 Eccrine poroma is derived from cells of the outer layer of the acrosyringium and upper dermal eccrine duct.12 The tumor replaces the epidermis and grows down into the dermis.12 Eccrine porocarcinoma is reported to coexist with benign eccrine poroma in up to 11% of cases,13 and some authors suggest that eccrine porocarcinoma results from the malignant transformation of the previously benign longstanding eccrine poroma.14 Squamous cell and basal cell carcinomas are most likely derived from keratinocytes or multipotential epidermal germ cells in the epidermal nevus, which harbors abnormal clones of cells, reflecting the genetic mosaicism from somatic mutation.4 As for eccrine neoplasms which arise from nonorganoid epidermal nevus, keratinocyte genetic derangement does not fully explain tumor formation. It is possible that adnexal tumors directly develop from epidermal nevi, because epidermal nevus tissue contains appendageal anomalies such as immature hair follicles and sebaceous, eccrine or apocrine glands.4 Benign and malignant adnexal tumors in epidermal nevi may originate from these abnormal structures, possibly due to the genetic instability because of distorted physiologic relationships,15 and thus disorganized proliferations in the lesion will be accentuated and accumulate. Another hypothesis is that tumors developing on epidermal nevi represent collision tumors.10 A previously reported case of eccrine poroma and eccrine porocarcinoma that developed in a linear epidermal nevus3 and the present case share similarities, including preexisting congenital epidermal nevi, multiple eccrine neoplasms grown at unrelated distant loci concurrently from the congenital epidermal nevi, and separate benign and malignant www.amjdermatopathology.com |
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Am J Dermatopathol Volume 36, Number 5, May 2014
Jeon et al
FIGURE 4. A. Relatively well-demarcated tumor showing acanthosis and papillomatosis with varying thicknesses of tumor mass demonstrating ductal structures and heavy inflammatory cell infiltration (H&E, ·40). B. Section through the eccrine poroma showing monomorphous cuboidal and basaloid cells downgrowing from the multifocal attachment to the epidermis, forming occasional ductal structures (H&E, ·100).
lesions that were found and diagnosed at the same time. Considering the similar findings of these 2 cases, the former hypothesis concerning adnexal tumor formation in epidermal nevi is more plausible. The development of concurrent eccrine poroma and eccrine porocarcinoma at unrelated distant loci in a linear epidermal nevus is a rare event. This is the second report of eccrine poroma and eccrine porocarcinoma developed within a linear epidermal nevus. REFERENCES 1. Rogers M, McCrossin I, Commens C. Epidermal nevi and the epidermal nevus syndrome. A review of 131 cases. J Am Acad Dermatol. 1989;20: 476–488. 2. Affleck AG, Leach IH, Varma S. Two squamous cell carcinomas arising in a linear epidermal naevus in a 28-year-old female. Clin Exp Dermatol. 2005;30:382–384. 3. Hamanaka S, Otsuka F. Multiple malignant eccrine poroma and a linear epidermal nevus. J Dermatol. 1996;23:469–471. 4. Burns T, Breathnach S, Cox N, et al. Rook’s Textbook of Dermatology. West Sussex, UK: Wiley-Blackwell; 2010. 5. Solomon LM, Esterly NB. Epidermal and other congenital organoid nevi. Curr Probl Pediatr. 1975;6:1–56.
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| www.amjdermatopathology.com
6. Levin A, Amazon K, Rywlin AM. A squamous cell carcinoma that developed in an epidermal nevus. Report of a case and a review of the literature. Am J Dermatopathol. 1984;6:51–55. 7. Swint RB, Klaus SN. Malignant degeneration of an epithelial nevus. Arch Dermatol. 1970;101:56–58. 8. Horn MS, Sausker WF, Pierson DL. Basal cell epithelioma arising in a linear epidermal nevus. Arch Dermatol. 1981;117:247. 9. Joshi A, Sah SP, Agarwalla A, et al. Basal cell carcinoma arising in a localized linear verrucous epidermal naevus. Acta Derm Venereol. 2000;80:227–228. 10. Hafner C, Klein A, Landthaler M, et al. Clonality of basal cell carcinoma arising in an epidermal nevus. New insights provided by molecular analysis. Dermatology. 2009;218:278–281. 11. Braunstein BL, Mackel SE, Cooper PH. Keratoacanthoma arising in a linear epidermal nevus. Arch Dermatol. 1982;118:362–363. 12. McKee PH, Calonje E, Granter SR. Pathology of the Skin With Clinical Correlations. Philadelphia, PA: Elsevier; 2005. 13. Spencer DM, Bigler LR, Hearne DW, et al. Pedal papule. Eccrine porocarcinoma (EPC) in association with poroma. Arch Dermatol. 1995;131: 211–214. 14. Robson A, Greene J, Ansari N, et al. Eccrine porocarcinoma (malignant eccrine poroma): a clinicopathologic study of 69 cases. Am J Surg Pathol. 2001;25:710–720. 15. Martin PC, Smith JL, Pulitzer DR, et al. Compound (primordial) adnexal carcinoma arising in a systematized compound epithelial nevus. Am J Surg Pathol. 1992;16:417–425.
2014 Lippincott Williams & Wilkins
Eccrine Poroma and Eccrine Porocarcinoma in Linear Epidermal Nevus Jiehyun Jeon, MD, PhD,* Joo Ha Kim, MD,* Yoo Sang Baek, MD,* Aeree Kim, MD, PhD,† Soo Hong Seo, MD, PhD,* and Chil Hwan Oh, MD, PhD*
(Am J Dermatopathol 2014;36:430–432)
INTRODUCTION Epidermal nevi arise from pluripotential germinative cells in the basal layer of the embryonic epidermis. These cells give rise to keratinocytes and skin appendages such as sebaceous glands, hair follicles, and apocrine glands. Therefore, epidermal nevi can be classified according to their predominant components, and terms such as nevus verrucosus, nevus sebaceus, nevus comedonicus, and nevus syringocystadenosus papilliferus are given to appropriate lesions.1 However, the division is not absolute, and overlap may be seen both clinically and histologically between classifications, forming a continuous spectrum.2 There are occasional reports of benign or malignant tumors developing in the preexisting verrucous, solitary, or linear epidermal nevi. However, these secondary neoplasms seem to be much rarer in epidermal nevi than in sebaceous or apocrine nevi.2 The majority of neoplasms that originate in epidermal nevi are epithelial tumors such as basal cell carcinoma, keratoacanthoma, Bowen disease, and squamous cell carcinoma. Skin appendageal secondary tumors are rare, and to the best of our knowledge, only 1 case report of eccrine poroma and eccrine porocarcinoma that developed within a linear epidermal nevus has been published.3
The initial biopsy specimen was obtained from the nodular lesion and from the brownish patch. Histopathology of the biopsy taken from the protruding nodule showed an ulcerated surface and tumor mass, which was continuous with the surface epithelium. The tumorous lesion consisted of anaplastic cells with relatively pale and large nuclei intermingled with cuboidal cells in an anastomosing band-like arrangement, accompanied by occasional mitosis and a gray-to-violaceous mucinous substance, which suggested a malignant lesion of sweat gland origin. The whole lesion was infiltrated with heavy inflammatory cells including plasma cells (Fig. 2). The other biopsy specimen from the patch lesion had features of seborrheic keratosis with pigmentation, acanthosis, and horn pseudocysts, which were consistent with epidermal nevus (Fig. 3). Later, wide excisions of the malignant nodule and a small, erythematous, friable-looking plaque located on the lower border of the posterior part of epidermal nevus were performed. The dermatopathologic findings of the excised nodule were similar to the findings of the previously biopsied lesion, and a diagnosis of eccrine porocarcinoma was made. In addition, the excised erythematous plaque lesion demonstrated characteristics of eccrine poroma, as it was made up of monomorphous cuboidal and basaloid cells downgrowing from the multifocal attachment to the epidermis, forming occasional ductal structures (Fig. 4). With multiple tumor formation at different loci on the existing epidermal nevus, we suspected the possibility that the patient would develop another malignant lesion remained relatively high. We recommended prophylactic complete excision of the epidermal nevus, but he refused further surgical treatment. Instead, the patient
CASE REPORT A 74-year-old man visited our clinic with a linear epidermal nevus after the lines of Blaschko on his right lower flank area. The linear epidermal nevus was discontinuous near the frontal one-third of the lesion and slightly verrucous on the surface with several darkly pigmented spots. The patient’s chief complaint was an oozing protruding erythematous nodule on the forefront marginal area of the epidermal nevus (Fig. 1), which had been present for 1 year. He had suffered from comorbidities including abdominal aortic dissection, unstable angina, hypertension, and usual interstitial pneumonia. Examination of the skin revealed no remarkable findings except for the previously described lesions. No palpable lymph nodes were detected. From the Departments of *Dermatology, and †Pathology, Korea University College of Medicine, Seoul, Korea. The authors declare no conflicts of interest. Reprints: Chil Hwan Oh, MD, PhD, Department of Dermatology, Korea University College of Medicine, 148 Gurodong-ro, Guro-dong, Guro-gu, Seoul 152-703, Korea (e-mail: [email protected]). © 2014 Lippincott Williams & Wilkins
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| www.amjdermatopathology.com
FIGURE 1. Linear epidermal nevi after the lines of Blaschko on patient’s right lower flank area with a protruding erythematous nodule on the forefront marginal area and a small erythematous plaque on the lower border of the posterior part of epidermal nevi. Am J Dermatopathol Volume 36, Number 5, May 2014
Am J Dermatopathol Volume 36, Number 5, May 2014
Eccrine Neoplasms in Linear Epidermal Nevus
FIGURE 2. A. The tumor nodule with an ulcerated surface is continuous with the surface epithelium. Tumor nests extend into the dermis and are accompanied by heavy inflammation (H&E, ·15). B. High power magnification of the demarcated area. Tumor nests originating in the epidermis extend into the dermis with some central necrosis and cells that consist of the tumor nests show nuclear atypia and mitosis (arrows) (H&E, ·200). consented to regular follow-up for the surveillance of any nodule, erosion, or ulceration on the epidermal nevus.
DISCUSSION Most epidermal nevi are small and asymptomatic and do not cause serious problems throughout life.2 The incidence of secondary tumors developing in epidermal nevi is low, and malignancies associated with epidermal nevi are rare. However, epidermal nevi can be regarded as the precursors of epidermal and appendageal neoplasm formations. The term “epidermal nevus” is designated for nevi composed of keratinocytes.4 However, Solomon and Esterly pointed out that very few epidermal nevi are exclusively of 1 type,5 and
FIGURE 3. Histopathologic features of seborrheic keratosis with pigmentation, acanthosis, and horn pseudocysts from a biopsy specimen harvested from the patch lesion of the epidermal nevi (H&E, ·40). 2014 Lippincott Williams & Wilkins
various types of tumor growth have been reported sporadically on epidermal nevi such as squamous cell carcinoma,2,6 Bowen disease,7 basal cell epithelioma,8–10 keratoacanthoma,11 eccrine poroma,3 and eccrine porocarcinoma.3 However, such tumors seem to be much more rare than in sebaceous or apocrine nevi.2 Eccrine poroma is derived from cells of the outer layer of the acrosyringium and upper dermal eccrine duct.12 The tumor replaces the epidermis and grows down into the dermis.12 Eccrine porocarcinoma is reported to coexist with benign eccrine poroma in up to 11% of cases,13 and some authors suggest that eccrine porocarcinoma results from the malignant transformation of the previously benign longstanding eccrine poroma.14 Squamous cell and basal cell carcinomas are most likely derived from keratinocytes or multipotential epidermal germ cells in the epidermal nevus, which harbors abnormal clones of cells, reflecting the genetic mosaicism from somatic mutation.4 As for eccrine neoplasms which arise from nonorganoid epidermal nevus, keratinocyte genetic derangement does not fully explain tumor formation. It is possible that adnexal tumors directly develop from epidermal nevi, because epidermal nevus tissue contains appendageal anomalies such as immature hair follicles and sebaceous, eccrine or apocrine glands.4 Benign and malignant adnexal tumors in epidermal nevi may originate from these abnormal structures, possibly due to the genetic instability because of distorted physiologic relationships,15 and thus disorganized proliferations in the lesion will be accentuated and accumulate. Another hypothesis is that tumors developing on epidermal nevi represent collision tumors.10 A previously reported case of eccrine poroma and eccrine porocarcinoma that developed in a linear epidermal nevus3 and the present case share similarities, including preexisting congenital epidermal nevi, multiple eccrine neoplasms grown at unrelated distant loci concurrently from the congenital epidermal nevi, and separate benign and malignant www.amjdermatopathology.com |
431
Am J Dermatopathol Volume 36, Number 5, May 2014
Jeon et al
FIGURE 4. A. Relatively well-demarcated tumor showing acanthosis and papillomatosis with varying thicknesses of tumor mass demonstrating ductal structures and heavy inflammatory cell infiltration (H&E, ·40). B. Section through the eccrine poroma showing monomorphous cuboidal and basaloid cells downgrowing from the multifocal attachment to the epidermis, forming occasional ductal structures (H&E, ·100).
lesions that were found and diagnosed at the same time. Considering the similar findings of these 2 cases, the former hypothesis concerning adnexal tumor formation in epidermal nevi is more plausible. The development of concurrent eccrine poroma and eccrine porocarcinoma at unrelated distant loci in a linear epidermal nevus is a rare event. This is the second report of eccrine poroma and eccrine porocarcinoma developed within a linear epidermal nevus. REFERENCES 1. Rogers M, McCrossin I, Commens C. Epidermal nevi and the epidermal nevus syndrome. A review of 131 cases. J Am Acad Dermatol. 1989;20: 476–488. 2. Affleck AG, Leach IH, Varma S. Two squamous cell carcinomas arising in a linear epidermal naevus in a 28-year-old female. Clin Exp Dermatol. 2005;30:382–384. 3. Hamanaka S, Otsuka F. Multiple malignant eccrine poroma and a linear epidermal nevus. J Dermatol. 1996;23:469–471. 4. Burns T, Breathnach S, Cox N, et al. Rook’s Textbook of Dermatology. West Sussex, UK: Wiley-Blackwell; 2010. 5. Solomon LM, Esterly NB. Epidermal and other congenital organoid nevi. Curr Probl Pediatr. 1975;6:1–56.
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| www.amjdermatopathology.com
6. Levin A, Amazon K, Rywlin AM. A squamous cell carcinoma that developed in an epidermal nevus. Report of a case and a review of the literature. Am J Dermatopathol. 1984;6:51–55. 7. Swint RB, Klaus SN. Malignant degeneration of an epithelial nevus. Arch Dermatol. 1970;101:56–58. 8. Horn MS, Sausker WF, Pierson DL. Basal cell epithelioma arising in a linear epidermal nevus. Arch Dermatol. 1981;117:247. 9. Joshi A, Sah SP, Agarwalla A, et al. Basal cell carcinoma arising in a localized linear verrucous epidermal naevus. Acta Derm Venereol. 2000;80:227–228. 10. Hafner C, Klein A, Landthaler M, et al. Clonality of basal cell carcinoma arising in an epidermal nevus. New insights provided by molecular analysis. Dermatology. 2009;218:278–281. 11. Braunstein BL, Mackel SE, Cooper PH. Keratoacanthoma arising in a linear epidermal nevus. Arch Dermatol. 1982;118:362–363. 12. McKee PH, Calonje E, Granter SR. Pathology of the Skin With Clinical Correlations. Philadelphia, PA: Elsevier; 2005. 13. Spencer DM, Bigler LR, Hearne DW, et al. Pedal papule. Eccrine porocarcinoma (EPC) in association with poroma. Arch Dermatol. 1995;131: 211–214. 14. Robson A, Greene J, Ansari N, et al. Eccrine porocarcinoma (malignant eccrine poroma): a clinicopathologic study of 69 cases. Am J Surg Pathol. 2001;25:710–720. 15. Martin PC, Smith JL, Pulitzer DR, et al. Compound (primordial) adnexal carcinoma arising in a systematized compound epithelial nevus. Am J Surg Pathol. 1992;16:417–425.
2014 Lippincott Williams & Wilkins
