Orbit, 2015; 34(4): 220–222 ! Informa Healthcare USA, Inc. ISSN: 0167-6830 print / 1744-5108 online DOI: 10.3109/01676830.2015.1049370
ORIGINAL ARTICLE
P63 Positive Mucoepidermoid Tumor of the Lacrimal Sac with Associated Papilloma Yiannis Iordanous1,2, Jillian C. Belrose1, Dani C. Cadieux1, Subrata Chakrabarti3, James P. Farmer4,5, and Larry H. Allen1,2
Orbit Downloaded from informahealthcare.com by University New South Wales on 08/17/15 For personal use only.
1
Schulich School of Medicine and Dentistry, University of Western Ontario, London, Ontario, Canada, 2Ivey Eye Institute, University of Western Ontario, London, Ontario, Canada, 3Department of Pathology, University of Western Ontario, London, Ontario, Canada, 4Department of Pathology and Molecular Medicine, Queen’s University, Kingston, Ontario, Canada, and 5Department of Ophthalmology, Queen’s University, Kingston, Ontario, Canada
ABSTRACT We report a case of a 44-year-old man who presented with a left medial canthal mass and epiphora. Imaging was suggestive of a mass continuous with the nasolacrimal sac. Subsequent surgical exploration revealed a mass adherent to bone with invasion of the lacrimal system. Histological examination revealed a squamous/ transitional cell papilloma overlying a low-grade mucoepidermoid carcinoma (MEC). Complete surgical resection was completed and pathology confirmed the diagnosis. This is the first case in which a MEC has been reported concurrently with an overlying papilloma, providing support for the hypothesis that MECs arise from papillomas in the lacrimal sac. Additionally, the tissue stained positive for p63, which is congruent with MEC immunoreactivity in the salivary gland. The description of these unique histopathological findings may assist in definitive diagnosis and improve our understanding of the pathophysiology underlying lacrimal sac MEC tumors. Keywords: Histopathology, lacrimal sac, mucoepidermoid carcinoma, p63, papilloma
INTRODUCTION
with a 1-year history of an enlarging left medial canthal mass and symptoms of epiphora. No other symptoms were reported. Examination revealed a visual acuity of 20/20 bilaterally, IOP of 20 mm HG OD and 18 mm HG OS. Ocular motility, anterior segment, and posterior segment assessments were unremarkable. A CT of head and orbits with IV contrast identified a 1.2 1.8 1.2 cm mass approaching the nasolacrimal duct. On imaging, the mass appeared continuous with the nasolacrimal sac, but did not demonstrate significant extension into the lacrimal apparatus (Figure 1). No bone, globe, muscle, or fat involvement was observed on CT. After a discussion with the patient, surgical exploration and removal of the mass was attempted. Intraoperatively, a mass was noted under the medial canthal tendon which was adherent to bone along the medial and inferior orbital rim. Additionally, the mass
Mucoepidermoid carcinomas (MEC) are defined by the presence of epidermoid, intermediate, and mucinsecreting cells.1 This tumor subtype has been reported in a variety of tissues; however, only 16 cases have been described in the lacrimal sac.2–11 MECs typically present as a medial canthal mass, and behave in a locally aggressive manner. We report the first case of a mucoepidermoid carcinoma of the lacrimal sac that presented within an overlying papilloma, and immunoreactivity for p63 protein.
CASE REPORT A 44-year-old male presented to the Oculoplastics service at the University of Western Ontario clinic
Received 8 February 2015; Accepted 2 May 2015; Published online 29 June 2015 Correspondence: Yiannis Iordanous, Ivey Eye Institute, St. Joseph’s Hospital, 268 Grosvenor Street, London, Ontario N6A 4V2, Canada. Tel: 519 646 6100. Fax: 519-646-6313. E-mail: [email protected]
220
Lacrimal Sac Mucoepidermoid Tumor
Orbit Downloaded from informahealthcare.com by University New South Wales on 08/17/15 For personal use only.
FIGURE 1. Axial CT scan at baseline demonstrating a mass in the medial canthal area of the left orbit. No bony erosion is seen.
221
FIGURE 3. Photomicrograph of the tumor demonstrating positive p63 staining.
tumor. Given the nature of the tumor and complete resection, no further intervention was deemed necessary. At his most recent follow-up, approximately 1 month after surgery, the patient was healing well, with no clinical evidence of local recurrence.
DISCUSSION
FIGURE 2. Photomicrograph of the tumor showing papilloma (left side) overlying a mucinous tumor (right side) (H and E stain, original magnification 50). Inset on the left shows magnified view of the papilloma; inset on the right shows magnified view of the mucinous neoplasm.
invaded into the lacrimal duct system. Tissue biopsy was sent to pathology, and histological examination identified a squamous/transitional cell papilloma overlying a low-grade mucoepidermoid carcinoma (Figure 2). The tissue stained positive for CK 5/6, CK7, and high molecular weight cytokeratin (data not shown). Importantly, the tissue also stained positive for p63 (Figure 3). The cells were negative for PSA, BRST2, CDX2 and CK20 markers (data not shown). Post-operative imaging revealed enhancing tissue in the left medial canthal area. After further discussion with the patient, a more extensive surgical resection was planned. This included lateral rhinectomy, left medial maxillectomy, left nasal lacrimal sac tumor excision, and medial canthal tendon repair. The removed tissue measured 2.5 1.5 0.4 cm, including the lacrimal sac (1.2 0.6 0.4 cm) and lacrimal duct (1.1 0.2 cm). Pathology report confirmed that the lesion was removed with clear borders, and confirmed the diagnosis of a low-grade mucoepidermoid !
2015 Informa Healthcare USA, Inc.
Two previous publications have introduced cases in which a papilloma diagnosis preceded a diagnosis of a mucoepidermoid carcinoma of the lacrimal apparatus.7,10 Importantly, this is the first case in which a MEC has been reported concurrently with a papilloma, providing support for the hypothesis that MECs may arise from papillomas in the lacrimal sac. Further research will be required to clarify whether MECs do indeed transition from papillomas, and whether any risk factors or triggers are implicated. The rarity of a MEC presentation in the lacrimal sac can also create some degree of difficulty and uncertainty with diagnosis. Therefore, identification of sensitive and specific markers for these tumors is necessary to assist in a timely diagnosis and to guide management. To this end, p63 immunoreactivity may provide important information. A recent publication by Sams and colleagues demonstrated that 100 percent of mucoepidermoid carcinomas of the salivary gland exhibit a strong positive nuclear staining pattern for p63, whereas none of the acinic cell carcinomas stained positively for p63.12 To our knowledge, this is the first case of a mucoepidermoid carcinoma of the lacrimal sac, which was assessed for p63 immunoreactivity. Based on the positive staining pattern identified in this case, we propose that immunohistochemical p63 assessment should be considered in subsequent mucin-containing tumors of the lacrimal sac. Further research into the sensitivity and specificity of this marker in various tumors may
222 Y. Iordanous et al. provide valuable information, which could assist in the differential diagnosis of lacrimal sac tumors.
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
Orbit Downloaded from informahealthcare.com by University New South Wales on 08/17/15 For personal use only.
REFERENCES 1. Chaudry AP, Vickers RA, Gorlin RJ. Intraoral minor salivary gland tumors. an analysis of 1,414 cases. Oral Surg Oral Med Oral Pathol 1961;14:1194–1226. 2. Yuksel D, Kosker M, Saribas F, Simsek S. Surgical treatment of mucoepidermoid carcinoma of the lacrimal sac. Semin Ophthalmol 2014;29(2):70–72. 3. Bianchi FA, Tosco P, Campisi P, et al. Mucoepidermoid carcinoma of the lacrimal sac masquerading as dacryocystitis. J Craniofac Surg 2010;21(3):797–800. 4. Stefanyszyn MA, Hidayat AA, Pe’er JJ, Flanagan JC. Lacrimal sac tumors. Ophthal Plast Reconstr Surg 1994;10(3):169–184.
5. Blake J, Mullaney J, Gillan J. Lacrimal sac mucoepidermoid carcinoma. Br J Ophthalmol 1986;70(9): 681–685. 6. Bambirra EA, Miranda D, Rayes A. Mucoepidermoid tumor of the lacrimal sac. Arch Ophthalmol 1981;99(12):2149–2150. 7. Lee SB, Kim KN, Lee SR, Bernardino CR. Mucoepidermoid carcinoma of the lacrimal sac after dacryocystectomy for squamous papilloma. Ophthal Plast Reconstr Surg 2011;27(2):e44–46. 8. Williams JD, Agrawal A, Wakely Jr PE,.Mucoepidermoid carcinoma of the lacrimal sac. Ann Diagn Pathol 2003;7(1):31–34. 9. Khan JA, Sutula FC, Pilch BZ, Joseph MP. Mucoepidermoid carcinoma involving the lacrimal sac. Ophthal Plast Reconstr Surg 1988;4(3):153–157. 10. Ni C, Wagoner MD, Wang WJ, et al. Mucoepidermoid carcinomas of the lacrimal sac. Arch Ophthalmol 1983;101(10):1572–1574. 11. Parmar DN, Rose GE. Management of lacrimal sac tumours. Eye (Lond) 2003;17(5):599–606. 12. Sams RN, Gnepp DR. P63 expression can be used in differential diagnosis of salivary gland acinic cell and mucoepidermoid carcinomas. Head Neck Pathol 2013;7(1):64068.
Orbit
ORIGINAL ARTICLE
P63 Positive Mucoepidermoid Tumor of the Lacrimal Sac with Associated Papilloma Yiannis Iordanous1,2, Jillian C. Belrose1, Dani C. Cadieux1, Subrata Chakrabarti3, James P. Farmer4,5, and Larry H. Allen1,2
Orbit Downloaded from informahealthcare.com by University New South Wales on 08/17/15 For personal use only.
1
Schulich School of Medicine and Dentistry, University of Western Ontario, London, Ontario, Canada, 2Ivey Eye Institute, University of Western Ontario, London, Ontario, Canada, 3Department of Pathology, University of Western Ontario, London, Ontario, Canada, 4Department of Pathology and Molecular Medicine, Queen’s University, Kingston, Ontario, Canada, and 5Department of Ophthalmology, Queen’s University, Kingston, Ontario, Canada
ABSTRACT We report a case of a 44-year-old man who presented with a left medial canthal mass and epiphora. Imaging was suggestive of a mass continuous with the nasolacrimal sac. Subsequent surgical exploration revealed a mass adherent to bone with invasion of the lacrimal system. Histological examination revealed a squamous/ transitional cell papilloma overlying a low-grade mucoepidermoid carcinoma (MEC). Complete surgical resection was completed and pathology confirmed the diagnosis. This is the first case in which a MEC has been reported concurrently with an overlying papilloma, providing support for the hypothesis that MECs arise from papillomas in the lacrimal sac. Additionally, the tissue stained positive for p63, which is congruent with MEC immunoreactivity in the salivary gland. The description of these unique histopathological findings may assist in definitive diagnosis and improve our understanding of the pathophysiology underlying lacrimal sac MEC tumors. Keywords: Histopathology, lacrimal sac, mucoepidermoid carcinoma, p63, papilloma
INTRODUCTION
with a 1-year history of an enlarging left medial canthal mass and symptoms of epiphora. No other symptoms were reported. Examination revealed a visual acuity of 20/20 bilaterally, IOP of 20 mm HG OD and 18 mm HG OS. Ocular motility, anterior segment, and posterior segment assessments were unremarkable. A CT of head and orbits with IV contrast identified a 1.2 1.8 1.2 cm mass approaching the nasolacrimal duct. On imaging, the mass appeared continuous with the nasolacrimal sac, but did not demonstrate significant extension into the lacrimal apparatus (Figure 1). No bone, globe, muscle, or fat involvement was observed on CT. After a discussion with the patient, surgical exploration and removal of the mass was attempted. Intraoperatively, a mass was noted under the medial canthal tendon which was adherent to bone along the medial and inferior orbital rim. Additionally, the mass
Mucoepidermoid carcinomas (MEC) are defined by the presence of epidermoid, intermediate, and mucinsecreting cells.1 This tumor subtype has been reported in a variety of tissues; however, only 16 cases have been described in the lacrimal sac.2–11 MECs typically present as a medial canthal mass, and behave in a locally aggressive manner. We report the first case of a mucoepidermoid carcinoma of the lacrimal sac that presented within an overlying papilloma, and immunoreactivity for p63 protein.
CASE REPORT A 44-year-old male presented to the Oculoplastics service at the University of Western Ontario clinic
Received 8 February 2015; Accepted 2 May 2015; Published online 29 June 2015 Correspondence: Yiannis Iordanous, Ivey Eye Institute, St. Joseph’s Hospital, 268 Grosvenor Street, London, Ontario N6A 4V2, Canada. Tel: 519 646 6100. Fax: 519-646-6313. E-mail: [email protected]
220
Lacrimal Sac Mucoepidermoid Tumor
Orbit Downloaded from informahealthcare.com by University New South Wales on 08/17/15 For personal use only.
FIGURE 1. Axial CT scan at baseline demonstrating a mass in the medial canthal area of the left orbit. No bony erosion is seen.
221
FIGURE 3. Photomicrograph of the tumor demonstrating positive p63 staining.
tumor. Given the nature of the tumor and complete resection, no further intervention was deemed necessary. At his most recent follow-up, approximately 1 month after surgery, the patient was healing well, with no clinical evidence of local recurrence.
DISCUSSION
FIGURE 2. Photomicrograph of the tumor showing papilloma (left side) overlying a mucinous tumor (right side) (H and E stain, original magnification 50). Inset on the left shows magnified view of the papilloma; inset on the right shows magnified view of the mucinous neoplasm.
invaded into the lacrimal duct system. Tissue biopsy was sent to pathology, and histological examination identified a squamous/transitional cell papilloma overlying a low-grade mucoepidermoid carcinoma (Figure 2). The tissue stained positive for CK 5/6, CK7, and high molecular weight cytokeratin (data not shown). Importantly, the tissue also stained positive for p63 (Figure 3). The cells were negative for PSA, BRST2, CDX2 and CK20 markers (data not shown). Post-operative imaging revealed enhancing tissue in the left medial canthal area. After further discussion with the patient, a more extensive surgical resection was planned. This included lateral rhinectomy, left medial maxillectomy, left nasal lacrimal sac tumor excision, and medial canthal tendon repair. The removed tissue measured 2.5 1.5 0.4 cm, including the lacrimal sac (1.2 0.6 0.4 cm) and lacrimal duct (1.1 0.2 cm). Pathology report confirmed that the lesion was removed with clear borders, and confirmed the diagnosis of a low-grade mucoepidermoid !
2015 Informa Healthcare USA, Inc.
Two previous publications have introduced cases in which a papilloma diagnosis preceded a diagnosis of a mucoepidermoid carcinoma of the lacrimal apparatus.7,10 Importantly, this is the first case in which a MEC has been reported concurrently with a papilloma, providing support for the hypothesis that MECs may arise from papillomas in the lacrimal sac. Further research will be required to clarify whether MECs do indeed transition from papillomas, and whether any risk factors or triggers are implicated. The rarity of a MEC presentation in the lacrimal sac can also create some degree of difficulty and uncertainty with diagnosis. Therefore, identification of sensitive and specific markers for these tumors is necessary to assist in a timely diagnosis and to guide management. To this end, p63 immunoreactivity may provide important information. A recent publication by Sams and colleagues demonstrated that 100 percent of mucoepidermoid carcinomas of the salivary gland exhibit a strong positive nuclear staining pattern for p63, whereas none of the acinic cell carcinomas stained positively for p63.12 To our knowledge, this is the first case of a mucoepidermoid carcinoma of the lacrimal sac, which was assessed for p63 immunoreactivity. Based on the positive staining pattern identified in this case, we propose that immunohistochemical p63 assessment should be considered in subsequent mucin-containing tumors of the lacrimal sac. Further research into the sensitivity and specificity of this marker in various tumors may
222 Y. Iordanous et al. provide valuable information, which could assist in the differential diagnosis of lacrimal sac tumors.
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
Orbit Downloaded from informahealthcare.com by University New South Wales on 08/17/15 For personal use only.
REFERENCES 1. Chaudry AP, Vickers RA, Gorlin RJ. Intraoral minor salivary gland tumors. an analysis of 1,414 cases. Oral Surg Oral Med Oral Pathol 1961;14:1194–1226. 2. Yuksel D, Kosker M, Saribas F, Simsek S. Surgical treatment of mucoepidermoid carcinoma of the lacrimal sac. Semin Ophthalmol 2014;29(2):70–72. 3. Bianchi FA, Tosco P, Campisi P, et al. Mucoepidermoid carcinoma of the lacrimal sac masquerading as dacryocystitis. J Craniofac Surg 2010;21(3):797–800. 4. Stefanyszyn MA, Hidayat AA, Pe’er JJ, Flanagan JC. Lacrimal sac tumors. Ophthal Plast Reconstr Surg 1994;10(3):169–184.
5. Blake J, Mullaney J, Gillan J. Lacrimal sac mucoepidermoid carcinoma. Br J Ophthalmol 1986;70(9): 681–685. 6. Bambirra EA, Miranda D, Rayes A. Mucoepidermoid tumor of the lacrimal sac. Arch Ophthalmol 1981;99(12):2149–2150. 7. Lee SB, Kim KN, Lee SR, Bernardino CR. Mucoepidermoid carcinoma of the lacrimal sac after dacryocystectomy for squamous papilloma. Ophthal Plast Reconstr Surg 2011;27(2):e44–46. 8. Williams JD, Agrawal A, Wakely Jr PE,.Mucoepidermoid carcinoma of the lacrimal sac. Ann Diagn Pathol 2003;7(1):31–34. 9. Khan JA, Sutula FC, Pilch BZ, Joseph MP. Mucoepidermoid carcinoma involving the lacrimal sac. Ophthal Plast Reconstr Surg 1988;4(3):153–157. 10. Ni C, Wagoner MD, Wang WJ, et al. Mucoepidermoid carcinomas of the lacrimal sac. Arch Ophthalmol 1983;101(10):1572–1574. 11. Parmar DN, Rose GE. Management of lacrimal sac tumours. Eye (Lond) 2003;17(5):599–606. 12. Sams RN, Gnepp DR. P63 expression can be used in differential diagnosis of salivary gland acinic cell and mucoepidermoid carcinomas. Head Neck Pathol 2013;7(1):64068.
Orbit
