Seminars in Ophthalmology
ISSN: 0882-0538 (Print) 1744-5205 (Online) Journal homepage: http://www.tandfonline.com/loi/isio20
A Rare Case of Eccrine Porocarcinoma of the Eyelid Paul Yihseng Chua, Kurt Spiteri Cornish, Grant Stenhouse & Christopher W. Barras To cite this article: Paul Yihseng Chua, Kurt Spiteri Cornish, Grant Stenhouse & Christopher W. Barras (2014): A Rare Case of Eccrine Porocarcinoma of the Eyelid, Seminars in Ophthalmology, DOI: 10.3109/08820538.2013.874475 To link to this article: http://dx.doi.org/10.3109/08820538.2013.874475
Published online: 27 Feb 2014.
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Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=isio20 Download by: [University of California Santa Barbara]
Date: 05 November 2015, At: 19:27
Seminars in Ophthalmology, Early Online, 1–3, 2014 ! Informa Healthcare USA, Inc. ISSN: 0882-0538 print / 1744-5205 online DOI: 10.3109/08820538.2013.874475
C ASE REPORT
A Rare Case of Eccrine Porocarcinoma of the Eyelid Paul Yihseng Chua1, Kurt Spiteri Cornish1, Grant Stenhouse2, and Christopher W. Barras1
Downloaded by [University of California Santa Barbara] at 19:27 05 November 2015
1
Department of Ophthalmology, Aberdeen University Hospital, Aberdeen, Scotland and 2Department of Pathology, Raigmore Hospital, Inverness, Scotland
ABSTRACT Background: To report a rare case of eyelid eccrine porocarcinoma and compare this to previous documented cases in the literature. Main Observations: We report a case of an 86-year-old man who presented with three months’ history of irritation in the right eye, who was found to have an irregular nodule on lower eyelid, which was later diagnosed as eccrine porocarcinoma (EPC). The lesion was excised and the defect repaired with Hughes flap. A computerized tomography (CT) scan of the head, neck, and chest showed no metastasis and no lymphadenopathy. There was no evidence of recurrence after 18 months of follow-up. Conclusion: To date, there have been only six cases of eyelid EPC reported in the literature. EPC has significant risk of recurrence and metastases after local excision. It is therefore important to consider it in the differential diagnosis of malignant eyelid tumour. A histological diagnosis should prompt wide margin excision, assessment of the patient for regional lymph node involvement, and imaging for metastatic disease. Keywords: Cancer, eccrine porocarcinoma, eyelid, malignancy, tumour
INTRODUCTION
The specimen measured 22 mm horizontally by 6 mm height and 7 mm depth. Microscopic examination revealed an invasive epithelial tumor arising at the junction of the skin and conjunctival mucosa. The tumor showed varying sized islands of tumor, some showing squamous differentiation and staining positively with immunohistochemical markers of cytokeratin 5 (CK5) and epithelial membrane antigen (EMA), along with zones of more poorly differentiated basaloid areas (Figure 1). There was evidence of ductular differentiation highlighted with carcinoembryonic antigen staining (CEA) and easily identifiable perineural and lymphovascular space invasion. The lesion invaded to a depth of 2.5 mm and was clear of all the excision margins by at least 2 mm. A histological diagnosis of eccrine porocarcinoma with infiltrative architecture was confirmed when the slides were sent for review at a tertiary referral center because of the rare nature of the disease. A further 2 mm margin was excised from the lower lid and the defect was repaired with a Hughes flap. Subsequently, a computerized
Eccrine porocarcinoma (EPC) is a rare, malignant, sweat gland tumor which arises from the intraepidermal ductal portion of the eccrine gland. It can be difficult to differentiate clinically from other skin tumors such as sebaceous cell carcinoma. We report a case of EPC of the eyelid. To date, only six such cases have ever been reported in the literature.1–6
CASE REPORT A previously healthy 86 year-old man presented with a three-month history of right eye discomfort, epiphora, and gritty sensation. He had no previous ocular history and was on no medication. On examination, there was an irregular nodule within a distinct margin on the right lower eyelid. The lesion measured 15 mm horizontally and 5 mm vertically. It was associated with loss of eyelashes and was suspicious of malignancy. An excisional biopsy was performed.
Received 25 June 2013; accepted 8 December 2013; published online 27 February 2014 Correspondence: Mr. Kurt Spiteri Cornish, MD, FRCOphth, Ophthalmology Department, Aberdeen Royal Infirmary, Aberdeen AB25 2ZN, Scotland. E-mail: [email protected]
1
Downloaded by [University of California Santa Barbara] at 19:27 05 November 2015
2
P. Y. Chua et al.
FIGURE 1. Haematoxylin and Eosin stained photomicrographs of the eccrine porocarcinoma. (A) The whole lesion at the junction of the conjunctival mucosa and skin (original magnification x20). (B) The tumor shows zones of basaloid (*) and squamous (arrow) morphology (original magnification x40). (C) Ductular elements present in the tumor (**) (original magnification x 200). (D) Lymphatic invasion (arrowhead) by an island of tumor (original magnification x 400).
TABLE 1. Summary of cases of EPC involving the eyelids. Age/gender Boynton and Markowich1 Orella et al.2 D’Abrosia et al.3 Kim et al.4 Greco et al.5 Jain et al.6 Present case (2011)
68/F 37/M 71/M 75/M 70/M 70/M 86/M
Site Lower Lower Lower Upper Lower Upper Lower
eyelid eyelid eyelid eyelid eyelid eyelid eyelid
Treatment
Follow-up
Outcome
Full thickness excision Wide excision Mohs micrographic surgery Full thickness excision Full thickness excision Mohs micrographic surgery Wide excision
3 years N/A N/A 6 months 2 years 6 months 18 months
No recurrence No recurrence N/A Exenteration of affected eye No recurrence No recurrence No recurrence
tomography (CT) scan of the head, neck, and chest showed no metastasis and no clinically significant lymphadenopathy. The patient was followed up for 18 months and showed no sign of recurrence.
DISCUSSION Eccrine porocarcinoma (EPC) is a rare, malignant, sweat gland tumor that arises from the acrosyringium, the intra-epidermal ductal portion of the eccrine sweat gland. It is usually seen on the trunk, extremities, and head and neck region. It rarely presents on the eyelids and only six such cases have been documented to date.1–6 All previous cases are
documented in Table 1, and a review of these cases revealed that eyelid EPC does not possess any distinguishing clinical features and can affect both upper and lower lids. However, EPC is known for its potential metastatic spread. It has been suggested that approximately 12–20% of cases metastasize to local lymph nodes.7 Histopathologically, eccrine porocarcinoma demonstrates features suggestive of its development from the acrosyringium, the intra-epidermal spiral duct of the sweat gland. It is usually characterized by islands of atypical poromatous basaloid cells and the presence of intracellular lumina and/or true ducts. EPC can be differentiated from squamous and sebaceous cell carcinoma by the presence of ductal Seminars in Ophthalmology
Eccrine Porocarcinoma of the Eyelid
Downloaded by [University of California Santa Barbara] at 19:27 05 November 2015
differentiation (either intra-cystoplasmic or actual duct formation). The ducts are usually visible on microscopy and can be outlined with CEA and EMA immunohistochemical markers. It can be differentiated from adenosquamous carcinoma as the latter is typically poorly differentiated, the glandular differentiation tends to be seen toward the deeper aspect of the lesion, and there is no spatial orientation to the acrosyringea. Poor prognostic indicators in EPC include the presence of lymphovascular and perineural invasion, necrosis, and a tumor depth of greater than 7 mm.7
CONCLUSION We report a rare sweat gland neoplasm in the lower eyelid. Despite being rare, it is important that EPC is included in the differential diagnosis of eyelid tumors, especially because of its metastatic potential and unknown natural history. A histological diagnosis of EPC should prompt complete surgical removal, assessment of regional lymph nodes, and search for metastatic spread.
!
2014 Informa Healthcare USA, Inc.
3
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
REFERENCES 1. Boynton JR, Markowitch Jr W. Porocarcinoma of the eyelid. Ophthalmology 1997;104:1626–1628. 2. Lozano Orella JA, Valcayo Penalba A, San Juan CC, et al. Eccrine porocarcinoma: Report of nine cases. Dermatol Surg 1997;23:925–928. 3. D’Ambrosia RA, Ward H, Parry E. Eccrine porocarcinoma of the eyelid treated with Mohs micrographic surgery. Dermatol Surg 2004;30:570–571. 4. Kim Y, Scolyer RA, Chia E-M, et al. Eccrine porocarcinoma of the upper eyelid. Australas J Dermatol 2005;46:278–281. 5. Greco M, Amorosi A, Vitagliano T, Bottoni U. Eccrine porocarcinoma of the face involving eyelids: A rare case report. Acta Chir Plast 2006;48:115–118. 6. Jain R, Prabhakaran VC, Huilgol SC, et al. Eccrine porocarcinoma of the upper eyelid. Ophthal Plast Reconstr Surg 2008;24:221–223. 7. Snow SN, Reizner GT. Eccrine porocarcinoma of the face. J Am Acad Dermatol 1992;27:306–311.
ISSN: 0882-0538 (Print) 1744-5205 (Online) Journal homepage: http://www.tandfonline.com/loi/isio20
A Rare Case of Eccrine Porocarcinoma of the Eyelid Paul Yihseng Chua, Kurt Spiteri Cornish, Grant Stenhouse & Christopher W. Barras To cite this article: Paul Yihseng Chua, Kurt Spiteri Cornish, Grant Stenhouse & Christopher W. Barras (2014): A Rare Case of Eccrine Porocarcinoma of the Eyelid, Seminars in Ophthalmology, DOI: 10.3109/08820538.2013.874475 To link to this article: http://dx.doi.org/10.3109/08820538.2013.874475
Published online: 27 Feb 2014.
Submit your article to this journal
Article views: 24
View related articles
View Crossmark data
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=isio20 Download by: [University of California Santa Barbara]
Date: 05 November 2015, At: 19:27
Seminars in Ophthalmology, Early Online, 1–3, 2014 ! Informa Healthcare USA, Inc. ISSN: 0882-0538 print / 1744-5205 online DOI: 10.3109/08820538.2013.874475
C ASE REPORT
A Rare Case of Eccrine Porocarcinoma of the Eyelid Paul Yihseng Chua1, Kurt Spiteri Cornish1, Grant Stenhouse2, and Christopher W. Barras1
Downloaded by [University of California Santa Barbara] at 19:27 05 November 2015
1
Department of Ophthalmology, Aberdeen University Hospital, Aberdeen, Scotland and 2Department of Pathology, Raigmore Hospital, Inverness, Scotland
ABSTRACT Background: To report a rare case of eyelid eccrine porocarcinoma and compare this to previous documented cases in the literature. Main Observations: We report a case of an 86-year-old man who presented with three months’ history of irritation in the right eye, who was found to have an irregular nodule on lower eyelid, which was later diagnosed as eccrine porocarcinoma (EPC). The lesion was excised and the defect repaired with Hughes flap. A computerized tomography (CT) scan of the head, neck, and chest showed no metastasis and no lymphadenopathy. There was no evidence of recurrence after 18 months of follow-up. Conclusion: To date, there have been only six cases of eyelid EPC reported in the literature. EPC has significant risk of recurrence and metastases after local excision. It is therefore important to consider it in the differential diagnosis of malignant eyelid tumour. A histological diagnosis should prompt wide margin excision, assessment of the patient for regional lymph node involvement, and imaging for metastatic disease. Keywords: Cancer, eccrine porocarcinoma, eyelid, malignancy, tumour
INTRODUCTION
The specimen measured 22 mm horizontally by 6 mm height and 7 mm depth. Microscopic examination revealed an invasive epithelial tumor arising at the junction of the skin and conjunctival mucosa. The tumor showed varying sized islands of tumor, some showing squamous differentiation and staining positively with immunohistochemical markers of cytokeratin 5 (CK5) and epithelial membrane antigen (EMA), along with zones of more poorly differentiated basaloid areas (Figure 1). There was evidence of ductular differentiation highlighted with carcinoembryonic antigen staining (CEA) and easily identifiable perineural and lymphovascular space invasion. The lesion invaded to a depth of 2.5 mm and was clear of all the excision margins by at least 2 mm. A histological diagnosis of eccrine porocarcinoma with infiltrative architecture was confirmed when the slides were sent for review at a tertiary referral center because of the rare nature of the disease. A further 2 mm margin was excised from the lower lid and the defect was repaired with a Hughes flap. Subsequently, a computerized
Eccrine porocarcinoma (EPC) is a rare, malignant, sweat gland tumor which arises from the intraepidermal ductal portion of the eccrine gland. It can be difficult to differentiate clinically from other skin tumors such as sebaceous cell carcinoma. We report a case of EPC of the eyelid. To date, only six such cases have ever been reported in the literature.1–6
CASE REPORT A previously healthy 86 year-old man presented with a three-month history of right eye discomfort, epiphora, and gritty sensation. He had no previous ocular history and was on no medication. On examination, there was an irregular nodule within a distinct margin on the right lower eyelid. The lesion measured 15 mm horizontally and 5 mm vertically. It was associated with loss of eyelashes and was suspicious of malignancy. An excisional biopsy was performed.
Received 25 June 2013; accepted 8 December 2013; published online 27 February 2014 Correspondence: Mr. Kurt Spiteri Cornish, MD, FRCOphth, Ophthalmology Department, Aberdeen Royal Infirmary, Aberdeen AB25 2ZN, Scotland. E-mail: [email protected]
1
Downloaded by [University of California Santa Barbara] at 19:27 05 November 2015
2
P. Y. Chua et al.
FIGURE 1. Haematoxylin and Eosin stained photomicrographs of the eccrine porocarcinoma. (A) The whole lesion at the junction of the conjunctival mucosa and skin (original magnification x20). (B) The tumor shows zones of basaloid (*) and squamous (arrow) morphology (original magnification x40). (C) Ductular elements present in the tumor (**) (original magnification x 200). (D) Lymphatic invasion (arrowhead) by an island of tumor (original magnification x 400).
TABLE 1. Summary of cases of EPC involving the eyelids. Age/gender Boynton and Markowich1 Orella et al.2 D’Abrosia et al.3 Kim et al.4 Greco et al.5 Jain et al.6 Present case (2011)
68/F 37/M 71/M 75/M 70/M 70/M 86/M
Site Lower Lower Lower Upper Lower Upper Lower
eyelid eyelid eyelid eyelid eyelid eyelid eyelid
Treatment
Follow-up
Outcome
Full thickness excision Wide excision Mohs micrographic surgery Full thickness excision Full thickness excision Mohs micrographic surgery Wide excision
3 years N/A N/A 6 months 2 years 6 months 18 months
No recurrence No recurrence N/A Exenteration of affected eye No recurrence No recurrence No recurrence
tomography (CT) scan of the head, neck, and chest showed no metastasis and no clinically significant lymphadenopathy. The patient was followed up for 18 months and showed no sign of recurrence.
DISCUSSION Eccrine porocarcinoma (EPC) is a rare, malignant, sweat gland tumor that arises from the acrosyringium, the intra-epidermal ductal portion of the eccrine sweat gland. It is usually seen on the trunk, extremities, and head and neck region. It rarely presents on the eyelids and only six such cases have been documented to date.1–6 All previous cases are
documented in Table 1, and a review of these cases revealed that eyelid EPC does not possess any distinguishing clinical features and can affect both upper and lower lids. However, EPC is known for its potential metastatic spread. It has been suggested that approximately 12–20% of cases metastasize to local lymph nodes.7 Histopathologically, eccrine porocarcinoma demonstrates features suggestive of its development from the acrosyringium, the intra-epidermal spiral duct of the sweat gland. It is usually characterized by islands of atypical poromatous basaloid cells and the presence of intracellular lumina and/or true ducts. EPC can be differentiated from squamous and sebaceous cell carcinoma by the presence of ductal Seminars in Ophthalmology
Eccrine Porocarcinoma of the Eyelid
Downloaded by [University of California Santa Barbara] at 19:27 05 November 2015
differentiation (either intra-cystoplasmic or actual duct formation). The ducts are usually visible on microscopy and can be outlined with CEA and EMA immunohistochemical markers. It can be differentiated from adenosquamous carcinoma as the latter is typically poorly differentiated, the glandular differentiation tends to be seen toward the deeper aspect of the lesion, and there is no spatial orientation to the acrosyringea. Poor prognostic indicators in EPC include the presence of lymphovascular and perineural invasion, necrosis, and a tumor depth of greater than 7 mm.7
CONCLUSION We report a rare sweat gland neoplasm in the lower eyelid. Despite being rare, it is important that EPC is included in the differential diagnosis of eyelid tumors, especially because of its metastatic potential and unknown natural history. A histological diagnosis of EPC should prompt complete surgical removal, assessment of regional lymph nodes, and search for metastatic spread.
!
2014 Informa Healthcare USA, Inc.
3
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
REFERENCES 1. Boynton JR, Markowitch Jr W. Porocarcinoma of the eyelid. Ophthalmology 1997;104:1626–1628. 2. Lozano Orella JA, Valcayo Penalba A, San Juan CC, et al. Eccrine porocarcinoma: Report of nine cases. Dermatol Surg 1997;23:925–928. 3. D’Ambrosia RA, Ward H, Parry E. Eccrine porocarcinoma of the eyelid treated with Mohs micrographic surgery. Dermatol Surg 2004;30:570–571. 4. Kim Y, Scolyer RA, Chia E-M, et al. Eccrine porocarcinoma of the upper eyelid. Australas J Dermatol 2005;46:278–281. 5. Greco M, Amorosi A, Vitagliano T, Bottoni U. Eccrine porocarcinoma of the face involving eyelids: A rare case report. Acta Chir Plast 2006;48:115–118. 6. Jain R, Prabhakaran VC, Huilgol SC, et al. Eccrine porocarcinoma of the upper eyelid. Ophthal Plast Reconstr Surg 2008;24:221–223. 7. Snow SN, Reizner GT. Eccrine porocarcinoma of the face. J Am Acad Dermatol 1992;27:306–311.
