Orbit, 2015; 34(4): 229–231 ! Informa Healthcare USA, Inc. ISSN: 0167-6830 print / 1744-5108 online DOI: 10.3109/01676830.2015.1014514
RESEARCH REPORT
A Cyst You Can’t Miss: A Rare Presentation of an Orbital Apocrine Hidrocystoma Soroosh Behshad1, Natalie C. Weil1, Son T. Ho1, Greg K. Mayhall2, and Alejandra A. Valenzuela1 1
Department of Ophthalmology, Tulane University School of Medicine, New Orleans, Louisiana, USA and Department of Pathology and Laboratory Medicine, Tulane University School of Medicine, New Orleans, Louisiana, USA
Orbit Downloaded from informahealthcare.com by University New South Wales on 08/17/15 For personal use only.
2
ABSTRACT Apocrine hidrocystomas are benign cystic lesions derived from the sweat glands of Moll and seldom found in the orbit. The authors present a case of a 41-year-old healthy man, with no prior medical history, referred for a painless enlarging mass, medial to his right upper eyelid for the past 3 months. Computed tomography showed a well-defined cystic lesion localized in the supero-medial anterior orbit. Following complete excision of the lesion, histopathology revealed an apocrine hidrocystoma. Although rare, apocrine hidrocystomas should be considered in the differential diagnosis for cystic mass of the orbit at any age group. Keywords: Cystadenoma, cyst in the orbit, orbital apocrine hidrocystoma
INTRODUCTION
The overlying skin was intact and showed no evidence of inflammation or infection. Exophthalmometry measurements were 15 mm bilaterally. Extraocular movements were normal and no changes were noted with Valsalva. The remainder of his ocular exam, including optic nerve function and fundoscopy were normal. Computed tomography (CT) identified a 2 2 cm well-defined, homogeneous, cystic lesion in the right supero-medial orbit extending anterior to the globe and posteriorly in close relation to the trochlea with no bone involvement (Figure 1b). The patient underwent a right anterior orbitotomy, through a gull-wing incision, which allowed a complete excision of the cystic mass (Figure 1c). Histopathology analysis revealed a fibrous tissue wall-lined by epithelium composed of two columnar cell layers, with variable areas of thickening. Further analysis showed intracytoplasmic granules in the inner lining cells that were demonstrable by diastase resistant periodic acid-Schiff (PAS) staining. On high power microscopy, the epithelial lining showed characteristic ‘‘decapitation secretions.’’ Under these
Apocrine hidrocystomas are benign cystic lesions that arise from the apocrine secretory glands of Moll. They are typically solitary and are found most often on the head, neck, along the eyelid margins, and very rarely in the orbit. The purpose of this paper is to report the unique occurrence of an orbital apocrine hidrocystoma and discuss histopathological characteristics at diagnosis.
CASE REPORT A 45-year-old man presented with right eyelid swelling for the past three months. He stated that the mass had gradually increased in size and was causing mild pressure and occasional pain. He denied any changes in vision, recent upper respiratory infection, and/or history of ocular adnexal trauma. On examination, he had a well-defined, soft, nontender, and fixed right supero-medial mass in the anterior orbit (Figure 1b). First callout Figure 1.
Received 24 August 2014; Accepted 28 January 2015; Published online 7 May 2015 Correspondence: Alejandra A. Valenzuela, 1430 Tulane Ave., SL-60, New Orleans, LA 70112, USA. E-mail: [email protected]
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Orbit Downloaded from informahealthcare.com by University New South Wales on 08/17/15 For personal use only.
FIGURE 1. (a) Clinical photograph of right soft, non-tender, and cystic supero-medial mass in the anterior orbit. (b) CT showing a well-defined, homogeneous, cystic lesion in the right supero-medial orbit extending anterior to the globe. (c) Intra-operative photograph showing the well-defined cystic lesions, measuring 2 2 cm removed in toto.
FIGURE 2. (a) Low power view of cystic spaces in apocrine hidrocystoma. (b) Note the fibrous pseudo-capsule surrounding the epithelial lining. Cystic spaces are lined by a double layer of epithelial cells; an inner layer of cuboidal to columnar cells with eosinophillic cytoplasm and an outer layer of flattened myoepithelial cells. (c) High power view shows the epithelial lining with characteristic ‘‘decapitation secretion.’’
findings the diagnosis of orbital apocrine hidrocystoma was suggested as the diagnosis (Figure 2a–2c). First callout Figure 2. The patient had complete resolution of symptoms after surgery and he has shown no signs of recurrence at 2 years follow-up.
DISCUSSION Hidrocystomas are benign tumors typically found in the head and neck, particularly the eyelid, however occurrence within the orbit is extremely rare, as indicated by only a few cases described.1,2 There are two types of hidrocystomas: eccrine and apocrine. Apocrine hidrocystoma are benign adenomatous cystic proliferations of secretory cells of Moll. They tend to be translucent or bluish with transillumination and typically occur on the eyelid margin.1,3 These cysts can be multiple and require complete excision of the cyst wall to prevent reoccurrence.1,2
Eccrine hidrocystomas originate from the sweat glands, are typically one to three millimeters in diameter, and cluster around the lower eyelids.2 These ductal retention cysts increase in prevalence in conditions such as heat and humidity.4 Clinically eccrine and apocrine hidrocystomas are characteristically similar and occur in the same locations. Therefore, histopathology is essential to differentiate between the two. On histology, eccrine hidrocystomas appear as unilocular cysts, which typically contain a single cystic cavity composed of one or two layers of cuboidal cells.5 On the other hand, apocrine hidrocystomas can be unilocular or multilocular dermal cysts, with a fibrous pseudocapsule often present. The cystic spaces are lined by a double layer of epithelial cells with the outer layer of the cysts lining appearing as flattened myoepitherlial cells and the inner layer consisting of cuboidal to tall columnar cells with basally Orbit
Orbit Downloaded from informahealthcare.com by University New South Wales on 08/17/15 For personal use only.
Orbital Apocrine Hidrocystoma located round vesicular nuclei and eosinophillic cytoplasm (Figure 2b).5 Characteristic decapitation secretion from the apocrine cells is usually present (Figure 2c). Approximately 50% of these lesions will show numerous small papillary projections growing into the central cavity.5,6 Chronic inflammation is often present within the interstitium. Intracytoplasmic granules in the inner lining cells may be demonstrable by distase resistant periodic acid-Schiff (PAS) staining. The myoepithelial layer can be highlighted by with smooth muscle actin (SMA) and p63 immunohistochemistry.5,6 Apocrine cystadenomas stain distinctly with immunoreactive stains for cytokeratins and human milk fat globulin.7 The abundant secretory granules and other features characteristic of mature apocrine secretory epithelium help establish the diagnosis. The pathophysiology of hidrocystomas is unclear, though many theories exist, such as; traumatic implantation of epithelial cells into the orbit, bone remodeling, and orbital enlargement in long-standing lesions of the orbit.3,8 The sequestration of epithelial cells at the embryonic stage could lead to this condition, as several cases have been described in the pediatric population. The above features or conditions were absent in our patient and with his history and short clinical course, the cause can likely idiopathic in nature. Occurrence of an intraorbital apocrine hidrocystoma is extremely rare. Case literature evaluation shows only a few cases with idiopathic origin, unrelated to previous trauma or childhood
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2015 Informa Healthcare USA, Inc.
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onset.1–3,8 This study emphasizes the importance of considering this diagnosis when evaluating a cystic orbital mass in an adult.
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. Valenzuela AA, Heathcote JG. Apocrine hidrocystoma of the orbit. Orbit 2011;2:316–317. 2. Ssi-Yan-Kai IC, Pearson AR. Recurrent giant orbital apocrine hidrocystoma. Eye (Lond) 2012;26(6):895–896. 3. Fateme E, Farhad F. Giant eccrine hidrocystoma of the orbit. J Res Med Sci 2007;12(3):150–152. 4. In reference 3, is there a volume number for the reference?. 5. Sarabi K, Khachemoune A. Hidrocystomas—a brief review. Med Gen Med 2006;8:57. 6. Calonje III E, Brenn T, Lazar AJ, McKee PH. McKee’s Pathology of Skin, 4th Edition. Philadelphia/Canada: WB Saunders/Elsevier 2012;1508–1510. 7. In reference 5, please confirm the correct publication city and publisher name for the volume. Your information said Philadelphia Saunders but online indicates Canada for Elsevier. 8. Vani D, Dayananda TR, Shashidhar HB, et al. Multiple apocrine hidrocystomas: A case report. J Clin Diagn Res 2013;7(1):171–172.
RESEARCH REPORT
A Cyst You Can’t Miss: A Rare Presentation of an Orbital Apocrine Hidrocystoma Soroosh Behshad1, Natalie C. Weil1, Son T. Ho1, Greg K. Mayhall2, and Alejandra A. Valenzuela1 1
Department of Ophthalmology, Tulane University School of Medicine, New Orleans, Louisiana, USA and Department of Pathology and Laboratory Medicine, Tulane University School of Medicine, New Orleans, Louisiana, USA
Orbit Downloaded from informahealthcare.com by University New South Wales on 08/17/15 For personal use only.
2
ABSTRACT Apocrine hidrocystomas are benign cystic lesions derived from the sweat glands of Moll and seldom found in the orbit. The authors present a case of a 41-year-old healthy man, with no prior medical history, referred for a painless enlarging mass, medial to his right upper eyelid for the past 3 months. Computed tomography showed a well-defined cystic lesion localized in the supero-medial anterior orbit. Following complete excision of the lesion, histopathology revealed an apocrine hidrocystoma. Although rare, apocrine hidrocystomas should be considered in the differential diagnosis for cystic mass of the orbit at any age group. Keywords: Cystadenoma, cyst in the orbit, orbital apocrine hidrocystoma
INTRODUCTION
The overlying skin was intact and showed no evidence of inflammation or infection. Exophthalmometry measurements were 15 mm bilaterally. Extraocular movements were normal and no changes were noted with Valsalva. The remainder of his ocular exam, including optic nerve function and fundoscopy were normal. Computed tomography (CT) identified a 2 2 cm well-defined, homogeneous, cystic lesion in the right supero-medial orbit extending anterior to the globe and posteriorly in close relation to the trochlea with no bone involvement (Figure 1b). The patient underwent a right anterior orbitotomy, through a gull-wing incision, which allowed a complete excision of the cystic mass (Figure 1c). Histopathology analysis revealed a fibrous tissue wall-lined by epithelium composed of two columnar cell layers, with variable areas of thickening. Further analysis showed intracytoplasmic granules in the inner lining cells that were demonstrable by diastase resistant periodic acid-Schiff (PAS) staining. On high power microscopy, the epithelial lining showed characteristic ‘‘decapitation secretions.’’ Under these
Apocrine hidrocystomas are benign cystic lesions that arise from the apocrine secretory glands of Moll. They are typically solitary and are found most often on the head, neck, along the eyelid margins, and very rarely in the orbit. The purpose of this paper is to report the unique occurrence of an orbital apocrine hidrocystoma and discuss histopathological characteristics at diagnosis.
CASE REPORT A 45-year-old man presented with right eyelid swelling for the past three months. He stated that the mass had gradually increased in size and was causing mild pressure and occasional pain. He denied any changes in vision, recent upper respiratory infection, and/or history of ocular adnexal trauma. On examination, he had a well-defined, soft, nontender, and fixed right supero-medial mass in the anterior orbit (Figure 1b). First callout Figure 1.
Received 24 August 2014; Accepted 28 January 2015; Published online 7 May 2015 Correspondence: Alejandra A. Valenzuela, 1430 Tulane Ave., SL-60, New Orleans, LA 70112, USA. E-mail: [email protected]
229
230 S. Behshad et al.
Orbit Downloaded from informahealthcare.com by University New South Wales on 08/17/15 For personal use only.
FIGURE 1. (a) Clinical photograph of right soft, non-tender, and cystic supero-medial mass in the anterior orbit. (b) CT showing a well-defined, homogeneous, cystic lesion in the right supero-medial orbit extending anterior to the globe. (c) Intra-operative photograph showing the well-defined cystic lesions, measuring 2 2 cm removed in toto.
FIGURE 2. (a) Low power view of cystic spaces in apocrine hidrocystoma. (b) Note the fibrous pseudo-capsule surrounding the epithelial lining. Cystic spaces are lined by a double layer of epithelial cells; an inner layer of cuboidal to columnar cells with eosinophillic cytoplasm and an outer layer of flattened myoepithelial cells. (c) High power view shows the epithelial lining with characteristic ‘‘decapitation secretion.’’
findings the diagnosis of orbital apocrine hidrocystoma was suggested as the diagnosis (Figure 2a–2c). First callout Figure 2. The patient had complete resolution of symptoms after surgery and he has shown no signs of recurrence at 2 years follow-up.
DISCUSSION Hidrocystomas are benign tumors typically found in the head and neck, particularly the eyelid, however occurrence within the orbit is extremely rare, as indicated by only a few cases described.1,2 There are two types of hidrocystomas: eccrine and apocrine. Apocrine hidrocystoma are benign adenomatous cystic proliferations of secretory cells of Moll. They tend to be translucent or bluish with transillumination and typically occur on the eyelid margin.1,3 These cysts can be multiple and require complete excision of the cyst wall to prevent reoccurrence.1,2
Eccrine hidrocystomas originate from the sweat glands, are typically one to three millimeters in diameter, and cluster around the lower eyelids.2 These ductal retention cysts increase in prevalence in conditions such as heat and humidity.4 Clinically eccrine and apocrine hidrocystomas are characteristically similar and occur in the same locations. Therefore, histopathology is essential to differentiate between the two. On histology, eccrine hidrocystomas appear as unilocular cysts, which typically contain a single cystic cavity composed of one or two layers of cuboidal cells.5 On the other hand, apocrine hidrocystomas can be unilocular or multilocular dermal cysts, with a fibrous pseudocapsule often present. The cystic spaces are lined by a double layer of epithelial cells with the outer layer of the cysts lining appearing as flattened myoepitherlial cells and the inner layer consisting of cuboidal to tall columnar cells with basally Orbit
Orbit Downloaded from informahealthcare.com by University New South Wales on 08/17/15 For personal use only.
Orbital Apocrine Hidrocystoma located round vesicular nuclei and eosinophillic cytoplasm (Figure 2b).5 Characteristic decapitation secretion from the apocrine cells is usually present (Figure 2c). Approximately 50% of these lesions will show numerous small papillary projections growing into the central cavity.5,6 Chronic inflammation is often present within the interstitium. Intracytoplasmic granules in the inner lining cells may be demonstrable by distase resistant periodic acid-Schiff (PAS) staining. The myoepithelial layer can be highlighted by with smooth muscle actin (SMA) and p63 immunohistochemistry.5,6 Apocrine cystadenomas stain distinctly with immunoreactive stains for cytokeratins and human milk fat globulin.7 The abundant secretory granules and other features characteristic of mature apocrine secretory epithelium help establish the diagnosis. The pathophysiology of hidrocystomas is unclear, though many theories exist, such as; traumatic implantation of epithelial cells into the orbit, bone remodeling, and orbital enlargement in long-standing lesions of the orbit.3,8 The sequestration of epithelial cells at the embryonic stage could lead to this condition, as several cases have been described in the pediatric population. The above features or conditions were absent in our patient and with his history and short clinical course, the cause can likely idiopathic in nature. Occurrence of an intraorbital apocrine hidrocystoma is extremely rare. Case literature evaluation shows only a few cases with idiopathic origin, unrelated to previous trauma or childhood
!
2015 Informa Healthcare USA, Inc.
231
onset.1–3,8 This study emphasizes the importance of considering this diagnosis when evaluating a cystic orbital mass in an adult.
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. Valenzuela AA, Heathcote JG. Apocrine hidrocystoma of the orbit. Orbit 2011;2:316–317. 2. Ssi-Yan-Kai IC, Pearson AR. Recurrent giant orbital apocrine hidrocystoma. Eye (Lond) 2012;26(6):895–896. 3. Fateme E, Farhad F. Giant eccrine hidrocystoma of the orbit. J Res Med Sci 2007;12(3):150–152. 4. In reference 3, is there a volume number for the reference?. 5. Sarabi K, Khachemoune A. Hidrocystomas—a brief review. Med Gen Med 2006;8:57. 6. Calonje III E, Brenn T, Lazar AJ, McKee PH. McKee’s Pathology of Skin, 4th Edition. Philadelphia/Canada: WB Saunders/Elsevier 2012;1508–1510. 7. In reference 5, please confirm the correct publication city and publisher name for the volume. Your information said Philadelphia Saunders but online indicates Canada for Elsevier. 8. Vani D, Dayananda TR, Shashidhar HB, et al. Multiple apocrine hidrocystomas: A case report. J Clin Diagn Res 2013;7(1):171–172.
