Ophthal Plast Reconstr Surg, Vol. 30, No. 3, 2014
neck, chest, and abdomen, failed to disclose any regional lymph nodes or distant metastases. The tumor was then classified as T2/N0/M0 according to the latest classification of malignant lacrimal gland tumors.4 The parents were informed of the malignant nature of the tumor and the unpredictable evolution of the disease. After a thorough discussion with the parents, informed consent was obtained for a radical removal of the lesion. Orbital exenteration was performed 1 month after the incisional biopsy.
DISCUSSION In the periocular area, sebaceous carcinomas originate from the sebaceous cells that comprise the Meibomian and Zeiss glands. They are generally aggressive, often metastasizing to the regional lymph nodes.5 However, adenocarcinomas with sebaceous features may occur at extraocular sites devoid of sebaceous cells such as the parotid gland.6 In this location, the tumor likely arises from pluripotent cells that have differentiated in sebaceous cells. As lacrimal gland malignancies are similar to tumors of the major salivary gland,4–7 it was believed that the presence of sebaceous cells in adenocarcinomas of the lacrimal gland represents abnormal differentiation. These cells show a characteristic microvesicular or “bubbly” cytoplasmic profile, with EMA positivity similar to mature sebocytes. Notably, sebaceous differentiation is extremely rare in PETLG. The literature search indicated only 11 cases, all in adults.8–15 Three of the cases originated from pleomorphic adenomas.8,9 Generally, PETLG are rare in children. The few cases that have been published are adenoid cystic carcinomas.16,17 Rarely, eyelid sebaceous cell carcinomas have been described in children after radiotherapy.18,19 To the authors’ knowledge, their case report is the first to document a true PETLG with sebaceous differentiation. Hence, the progression of the tumor is difficult to predict. The management of a child with a large lacrimal gland mass remains contentious. Primary malignant tumors of the lacrimal gland are rare; therefore, there are no established criteria for therapy. Immunohistochemistry (expression of p53 and Ki67) may provide aid in evaluating the prognosis of lacrimal gland tumors. Some experts disagree with an incisional biopsy as the primary approach for a well-delineated lacrimal mass. However, experience with adult cases indicates that a variety of lesions may mimic lacrimal tumors.20 Hence, they opted for a conservative approach and first elected to biopsy the mass. Treatment options include an orbital-sparing tumor resection followed by chemotherapy and radiotherapy. However, after discussing the alternatives with the parents, partial exenteration was preferred as the initial surgical procedure. To date, the patient remains under the care of a pediatric oncologist.
REFERENCES 1. Shields JA, Shields CL, Epstein JA, et al. Review: primary epithelial malignancies of the lacrimal gland: the 2003 Ramon L. Font lecture. Ophthal Plast Reconstr Surg 2004;20:10–21. 2. Lacrimal Gland Tumor Study G. An epidemiological survey of lacrimal fossa lesions in Japan: number of patients and their sex ratio by pathological diagnosis. Jpn J Ophthalmol 2005;49:343–8. 3. Skinner HD, Garden AS, Rosenthal DI, et al. Outcomes of malignant tumors of the lacrimal apparatus: the University of Texas MD Anderson Cancer Center experience. Cancer 2011;117:2801–10. 4. Rootman J, White VA. Changes in the 7th edition of the AJCC TNM classification and recommendations for pathologic analysis of lacrimal gland tumors. Arch Pathol Lab Med 2009;133:1268–71. 5. Shields JA, Demirci H, Marr BP, et al. Sebaceous carcinoma of the ocular region: a review. Surv Ophthalmol 2005;50:103–22. 6. Erovic BM, Goldstein DP, Kim D, et al. Sebaceous gland carcinoma of the head and neck: the Princess Margaret Hospital experience. Head Neck 2013;35:316–20.
Case Reports
7. Bernardini FP, Devoto MH, Croxatto JO. Epithelial tumors of the lacrimal gland: an update. Curr Opin Ophthalmol 2008;19:409–13. 8. Witschel H, Zimmerman LE. Malignant mixed tumor of the lacrimal gland. A clinicopathologic report of two unusual cases. Albrecht Von Graefes Arch Klin Exp Ophthalmol 1981;216:327–37. 9. Konrad EA, Thiel HJ. Adenocarcinoma of the lacrimal gland with sebaceous differentiation. A clinical study using light and electronmicroscopy. Graefes Arch Clin Exp Ophthalmol 1983;221:81–5. 10. Rodgers IR, Jakobiec FA, Gingold MP, et al. Anaplastic carcinoma of the lacrimal gland presenting with recurrent subconjunctival hemorrhages and displaying incipient sebaceous differentiation. Ophthal Plast Reconstr Surg 1991;7:229–37. 11. Harvey PA, Parsons MA, Rennie IG. Primary sebaceous carcinoma of lacrimal gland: a previously unreported primary neoplasm. Eye (Lond) 1994;8 (pt 5):592–5. 12. Briscoe D, Mahmood S, Bonshek R, et al. Primary sebaceous carcinoma of the lacrimal gland. Br J Ophthalmol 2001;85:625–6. 13. Yamamoto N, Mizoe JE, Hasegawa A, et al. Primary sebaceous carcinoma of the lacrimal gland treated by carbon ion radiotherapy. Int J Clin Oncol 2003;8:386–90. 14. Yoon JS, Kim SH, Lee CS, et al. Clinicopathological analy sis of periocular sebaceous gland carcinoma. Ophthalmologica 2007;221:331–9. 15. Kiratli H, Tarlan B, Fırat P. Primary sebaceous carcinoma of the lacrimal gland. Orbit 2012;31:352–4. 16. Galliani CA, Faught PR, Ellis FD. Adenoid cystic carcinoma of the lacrimal gland in a six-year-old girl. Pediatr Pathol 1993;13:559–65. 17. Thavaraj V, Sridhar MR, Sethi A, et al. Adenoid cystic carcinoma of the lacrimal gland. Indian J Pediatr 2003;70:751–3. 18. Lemos LB, Santa Cruz DJ, Baba N. Sebaceous carcinoma of the eyelid following radiation therapy. Am J Surg Pathol 1978;2:305–11. 19. Boniuk M, Zimmerman LE. Sebaceous carcinoma of the eye lid, eyebrow, caruncle, and orbit. Trans Am Acad Ophthalmol Otolaryngol 1968;72:619–42. 20. Prabhakaran VC, Cannon PS, McNab A, et al. Lesions mimicking lacrimal gland pleomorphic adenoma. Br J Ophthalmol 2010;94:1509–12.
Papillary Cystadenocarcinoma of the Lacrimal Gland After Radiation for Bilateral Retinoblastoma Shivali A. Menda, M.D., H. Jane Kim, M.D., Michele M. Bloomer, M.D., J. Brooks Crawford, M.D., M. Reza Vagefi, M.D., and Robert C. Kersten, M.D. Abstract: The authors report a case of papillary cystadenocarcinoma of the lacrimal gland after irradiation for bilateral retinoblastoma. A 32-year-old man with a history of bilateral retinoblastoma, diagnosed shortly after birth, was treated with enucleation of the OS and a single session of radiation to the OD. Over 30 years later, he presented with an orbital mass of the right lacrimal gland that on biopsy demonstrated papillary cystadenocarcinoma.
Department of Ophthalmology, University of California, San Francisco, California, U.S.A. Accepted for publication April 21, 2013. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Robert C. Kersten, M.D., Department of Ophthalmology, University of California, 10 Koret Way, San Francisco, CA 94143. E-mail: [email protected] DOI: 10.1097/IOP.0b013e31829bb43e
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
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Ophthal Plast Reconstr Surg, Vol. 30, No. 3, 2014
Case Reports
CASE REPORT A 32-year-old African American man presented with a palpable mass in the right superotemporal orbit and increasing painless proptosis over 2 months. The ocular history is significant for bilateral retinoblastoma diagnosed shortly after birth that required enucleation of the OS in 1979. The patient received 1 dose of radiation therapy on the right side. He did not receive chemotherapy. On presentation, visual acuity was 20/25 OD. External examination revealed an hourglass facial deformity. The right upper eyelid had an S-shaped configuration. Extraocular motility was full. Dilated examination was significant for choroidal folds and flat scar without calcification that likely represented treated retinoblastoma. MRI demonstrated an extraconal, heterogenous, and multilobulated mass involving the right lacrimal gland extending in the orbital apex with a small area of orbital roof erosion (Fig. 1). The patient underwent an anterior orbitotomy with gross surgical excision via an eyelid crease approach. The posterior extent of mass and capsule were incompletely excised to preserve vision. Intraoperatively, the mass appeared multicystic with chocolate-colored fluid. On microscopic examination, a cystic neoplasm with a papillary epithelial lining was observed (Fig. 2). Higher magnification revealed mild-to-moderate grade atypical epithelial cells with occasional mitotic figures (Fig. 2, inset). The tumor cells stained positively with EMA and keratin stains. The patient underwent a metastatic workup that included PET CT, and no evidence of extraorbital tumor was found. Postoperative MRI showed residual tumor in the right lacrimal fossa. The head and neck tumor board recommended a complete resection of the tumor with margins as patient was not a candidate for further radiation therapy, and there is no proven chemotherapy. Despite multiple discussions with the patient and his family, he refused any additional intervention.
DISCUSSION Lacrimal gland neoplasms represent 10% of primary orbital neoplasms with tumors of epithelial origin accounting for 20%.1–3 Of these, primary adenocarcinoma represents only 2% to 5% of epithelial neoplasms of the lacrimal gland.2,3 This tumor is subdivided into mucinous adenocarcinoma, ductal adenocarcinoma, and papillary cystadenocarcinoma types.1 Currently, there are only 2 case reports of cystadenocarcinoma of the lacrimal gland in the literature.4,5 To the authors’ knowledge, this rare neoplasm has not been previously described in the setting of orbital radiation for retinoblastoma. Hereditary retinoblastoma is associated with an increased risk of secondary malignancies.6,7 A familial history of retinoblastoma and of external beam radiation may portend an even higher risk.6,7 Most patients develop either bone or soft tissue malignancies.6 There are only 2 case reports of patients with secondary lacrimal gland neoplasms after treatment for retinoblastoma. One describes a 4-month-old girl with a history of bilateral retinoblastoma treated with external beam radiation, systemic chemotherapy, and enucleation of the OD, who developed a lacrimal gland pleomorphic adenoma 17 years after original treatment.8 Bubella et al.9 recently reported a case of lacrimal adenocarcinoma arising from a pleomorphic adenoma in a patient with bilateral retinoblastoma treated with radiation occurring 20 years later. Given epithelial neoplasms of the lacrimal gland are histologically similar to tumors of the major salivary glands and the rarity of papillary cystadenocarcinoma of the orbit, the head and neck literature can provide a reference point for treatment
e58
FIG. 1. A 32-year-old man presenting with painless proptosis of the OD with a history bilateral retinoblastoma treated previously with enucleation OS and orbital radiation OD. MRI demonstrates a multicystic tumor arising from the right lacrimal gland extending in the muscle cone with erosion of the orbital roof.
FIG. 2. Histopathology of the orbital specimen from a 32-year-old man with a lacrimal gland mass. The tumor is composed of a papillary epithelium lining cystic spaces (hematoxylin-eosin; ×10). On higher magnification, there is a squamous epithelium with prominent nucleoli and occasional mitotic figures (inset) (hematoxylin-eosin; ×40).
paradigms and outcomes of this tumor type.2 In 1 large series of 40 patients with salivary gland cystadenocarcinoma, treatment consisted of local excision of intraoral lesions with parotidectomy or lobectomy for parotid gland tumors.10 Ten percent of patients demonstrated metastatic disease and required regional lymphadenectomy followed by radiation therapy. Ninety percent of patients were either alive or had died of unrelated causes during a mean follow up of 59 months after initial excision. Three tumors recurred locally between 18 and 122 months. In only 2 cases of cystadenocarcinoma of the lacrimal gland, both patients were alive at 7 and 9 years after treatment with en bloc excision and no radiotherapy or chemotherapy.1,4,5 The patient’s monocular status placed him in a particularly difficult situation when contemplating further surgery after incomplete excision which he eventually deferred. In conclusion, a lacrimal gland neoplasm may occur rarely years after external beam radiation treatment for retinoblastoma. A tumor of lacrimal origin should be considered in the differential for new-onset proptosis in this patient subpopulation. To date, a pleomorphic adenoma, an adenocarcinoma arising from a pleomorphic adenoma and papillary cystadenocarcinoma, has been reported. Treatment of papillary cystadenocarcinoma is largely surgical with a guarded prognosis.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 30, No. 3, 2014
REFERENCES 1. Bernardini FP, Devoto MH, Croxatto JO. Epithelial tumors of the lacrimal gland: an update. Curr Opin Ophthalmol 2008;19:409–13. 2. Weis E, Rootman J, Joly TJ, et al. Epithelial lacrimal gland tumors: pathologic classification and current understanding. Arch Ophthalmol 2009;127:1016–28. 3. Shields CL, Shields JA, Eagle RC, et al. Clinicopathologic review of 142 cases of lacrimal gland lesions. Ophthalmology 1989;96:431–5. 4. Devoto MH, Croxatto JO. Primary cystadenocarcinoma of the lacrimal gland. Ophthalmology 2003;110:2006–10. 5. Peng X, Li G, Wang J, et al. Papillary cystadenocarcinoma of the lacrimal gland. Ann Ophthalmol (Skokie) 2010;42 Spec No:15–9. 6. Mohney BG, Robertson DM, Schomberg PJ, et al. Second nonocular tumors in survivors of heritable retinoblastoma and prior radiation therapy. Am J Ophthalmol 1998;126:269–77. 7. Moll AC, Imhof SM, Schouten-Van Meeteren AY, et al. Second primary tumors in hereditary retinoblastoma: a register-based study, 1945-1997: is there an age effect on radiation-related risk? Ophthalmology 2001;108:1109–14. 8. Hadjistilianou T, de Francesco S, Signori C, et al. Pleomorphic adenoma of the lacrimal gland in an 18-year-old girl irradiated for bilateral retinoblastoma. Orbit 2006;25:51–3. 9. Bubella DM, Bubella RM, Carità S, et al. Adenocarcinoma of the lacrimal gland in bilateral retinoblastoma treated with external radiotherapy: a case report. J Fr Ophtalmol 2011;34:647–50. 10. Foss RD, Ellis GL, Auclair PL. Salivary gland cystadenocarcinomas. A clinicopathologic study of 57 cases. Am J Surg Pathol 1996;20:1440–7.
Bilateral Dacryocystoceles in a Pregnant Woman Kristin E. Hirabayashi, B.A., Elizabeth Yang, M.D., Julio Echegoyen, M.D., Ph.D., Steven J. Yoon, M.D., and Jeremiah P. Tao, M.D., F.A.C.S. Abstract: The authors describe, for the first time, bilateral, sequential large dacryocystoceles during pregnancy and review the literature for this presentation. A 26-year-old, 15-week pregnant woman presented with OD epiphora, diplopia, and pain in the setting of an inferomedial orbital mass. Surgical exploration and histopathology were consistent with a dacryocystocele, and a dacryocystorhinostomy was curative. She returned at 34-week gestation, with an identical presentation on the left side. Review of the literature reveals that dacryocystoceles occasionally present in adults; however, bilateral involvement may be unusual. Bilateral dacryocystoceles have not been previously reported in a pregnant woman.
Case Reports
on the right side and fullness of the inferonasal orbit. There was mild tenderness to the medial lower eyelid and medial canthus but no erythema. Extraocular motility testing revealed reduced OD supraduction and adduction. Visual acuity was 20/200 OD (poor effort due to discomfort) and 20/20 OS. There was no afferent pupillary defect to corroborate an optic nerve etiology for the low vision. Intraocular pressures were 20 and 21 mm Hg. A dilated fundus examination was normal, but some inferonasal contour abnormalities consistent with external globe compression (but no ischemia or atrophy) was evident. The eye examination was otherwise normal. Lacrimal probing and irrigation revealed a complete distal obstruction of nasolacrimal outflow with all irrigated fluid regurgitating from the opposite, ipsilateral punctum. CT scans of the orbital region revealed a well-circumscribed mass in the inferomedial orbit, compressing the globe (Fig. 1). Oral clindamycin was initiated to treat presumptive dacryocystitis. At 10 days, medical management was unsuccessful, and the symptoms progressed. Surgical intervention for decompression or removal of the mass was recommended and accepted by the patient due to the nonresolving orbital signs. The intraoperative findings were consistent with a dacryocystocele, with the intralesional contents consisting of clear fluid and no mucopurulent, infectious material. Histopathologic analysis of a sac specimen demonstrated mild inflammation of otherwise normalappearing nasolacrimal sac tissue that was also negative for granulomas or malignancy. An external dacryocystorhinostomy was performed, and a Crawford silicone tube (JEDMED Instrument Company, St. Louis, MO, U.S.A.) was placed after draining the sac contents. Extraocular motility returned to normal, and the symptoms resolved. At 34-week gestation, the patient returned, complaining of diplopia, left epiphora, and OS pain with extraocular movements. External examination demonstrated fullness of the inferonasal left orbit. Extraocular motility testing showed reduced adduction and supraduction of the OS. Visual acuity was 20/20 OD and 20/25 OS. Intraocular pressures were 18 and 19 mm Hg. The eye examination, including dilated fundus examination, was normal. Some clear fluid, but no mucopurulent material was expressed, form the punctae with pressure over the lacrimal sac region. CT scans of the orbital region demonstrated a 1.31 × 1.37 cm well-circumscribed mass in the inferomedial orbit (Fig. 2). Given her difficulty with pain control and some decelerations on fetal heart tracings, the patient was admitted for monitoring, bed rest, and empirical treatment of a presumptive
CASE REPORT A 26-year-old woman at 15-week gestation presented with symptoms of diplopia, right epiphora, and OD pain with extraocular movements. She was otherwise healthy with no prior ocular history. External examination demonstrated an increased tear leak *Department of Ophthalmology, Gavin Herbert Eye Institute, University of California, Irvine, California; †Department of Ophthalmology, Yale Eye Center, Yale University, New Haven, Connecticut; ‡Department of Oculofacial Plastic and Orbital Surgery, Maryland Eye Associates, Annapolis, Maryland, U.S.A. Accepted for publication April 24, 2013. The authors have no financial or conflicts of interest to disclose. Supported, in part, by an institutional grant from research to prevent blindness. Address correspondence and reprint requests to Kristin E. Hirabayashi, Department of Ophthalmology, Gavin Herbert Eye Institute, University of California, Irvine, CA 92697. E-mail: [email protected] DOI: 10.1097/IOP.0b013e31829bb45c
FIG. 1. Orbitofacial CT of patient at 15-week gestation demonstrating a mass in the right orbital region.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
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neck, chest, and abdomen, failed to disclose any regional lymph nodes or distant metastases. The tumor was then classified as T2/N0/M0 according to the latest classification of malignant lacrimal gland tumors.4 The parents were informed of the malignant nature of the tumor and the unpredictable evolution of the disease. After a thorough discussion with the parents, informed consent was obtained for a radical removal of the lesion. Orbital exenteration was performed 1 month after the incisional biopsy.
DISCUSSION In the periocular area, sebaceous carcinomas originate from the sebaceous cells that comprise the Meibomian and Zeiss glands. They are generally aggressive, often metastasizing to the regional lymph nodes.5 However, adenocarcinomas with sebaceous features may occur at extraocular sites devoid of sebaceous cells such as the parotid gland.6 In this location, the tumor likely arises from pluripotent cells that have differentiated in sebaceous cells. As lacrimal gland malignancies are similar to tumors of the major salivary gland,4–7 it was believed that the presence of sebaceous cells in adenocarcinomas of the lacrimal gland represents abnormal differentiation. These cells show a characteristic microvesicular or “bubbly” cytoplasmic profile, with EMA positivity similar to mature sebocytes. Notably, sebaceous differentiation is extremely rare in PETLG. The literature search indicated only 11 cases, all in adults.8–15 Three of the cases originated from pleomorphic adenomas.8,9 Generally, PETLG are rare in children. The few cases that have been published are adenoid cystic carcinomas.16,17 Rarely, eyelid sebaceous cell carcinomas have been described in children after radiotherapy.18,19 To the authors’ knowledge, their case report is the first to document a true PETLG with sebaceous differentiation. Hence, the progression of the tumor is difficult to predict. The management of a child with a large lacrimal gland mass remains contentious. Primary malignant tumors of the lacrimal gland are rare; therefore, there are no established criteria for therapy. Immunohistochemistry (expression of p53 and Ki67) may provide aid in evaluating the prognosis of lacrimal gland tumors. Some experts disagree with an incisional biopsy as the primary approach for a well-delineated lacrimal mass. However, experience with adult cases indicates that a variety of lesions may mimic lacrimal tumors.20 Hence, they opted for a conservative approach and first elected to biopsy the mass. Treatment options include an orbital-sparing tumor resection followed by chemotherapy and radiotherapy. However, after discussing the alternatives with the parents, partial exenteration was preferred as the initial surgical procedure. To date, the patient remains under the care of a pediatric oncologist.
REFERENCES 1. Shields JA, Shields CL, Epstein JA, et al. Review: primary epithelial malignancies of the lacrimal gland: the 2003 Ramon L. Font lecture. Ophthal Plast Reconstr Surg 2004;20:10–21. 2. Lacrimal Gland Tumor Study G. An epidemiological survey of lacrimal fossa lesions in Japan: number of patients and their sex ratio by pathological diagnosis. Jpn J Ophthalmol 2005;49:343–8. 3. Skinner HD, Garden AS, Rosenthal DI, et al. Outcomes of malignant tumors of the lacrimal apparatus: the University of Texas MD Anderson Cancer Center experience. Cancer 2011;117:2801–10. 4. Rootman J, White VA. Changes in the 7th edition of the AJCC TNM classification and recommendations for pathologic analysis of lacrimal gland tumors. Arch Pathol Lab Med 2009;133:1268–71. 5. Shields JA, Demirci H, Marr BP, et al. Sebaceous carcinoma of the ocular region: a review. Surv Ophthalmol 2005;50:103–22. 6. Erovic BM, Goldstein DP, Kim D, et al. Sebaceous gland carcinoma of the head and neck: the Princess Margaret Hospital experience. Head Neck 2013;35:316–20.
Case Reports
7. Bernardini FP, Devoto MH, Croxatto JO. Epithelial tumors of the lacrimal gland: an update. Curr Opin Ophthalmol 2008;19:409–13. 8. Witschel H, Zimmerman LE. Malignant mixed tumor of the lacrimal gland. A clinicopathologic report of two unusual cases. Albrecht Von Graefes Arch Klin Exp Ophthalmol 1981;216:327–37. 9. Konrad EA, Thiel HJ. Adenocarcinoma of the lacrimal gland with sebaceous differentiation. A clinical study using light and electronmicroscopy. Graefes Arch Clin Exp Ophthalmol 1983;221:81–5. 10. Rodgers IR, Jakobiec FA, Gingold MP, et al. Anaplastic carcinoma of the lacrimal gland presenting with recurrent subconjunctival hemorrhages and displaying incipient sebaceous differentiation. Ophthal Plast Reconstr Surg 1991;7:229–37. 11. Harvey PA, Parsons MA, Rennie IG. Primary sebaceous carcinoma of lacrimal gland: a previously unreported primary neoplasm. Eye (Lond) 1994;8 (pt 5):592–5. 12. Briscoe D, Mahmood S, Bonshek R, et al. Primary sebaceous carcinoma of the lacrimal gland. Br J Ophthalmol 2001;85:625–6. 13. Yamamoto N, Mizoe JE, Hasegawa A, et al. Primary sebaceous carcinoma of the lacrimal gland treated by carbon ion radiotherapy. Int J Clin Oncol 2003;8:386–90. 14. Yoon JS, Kim SH, Lee CS, et al. Clinicopathological analy sis of periocular sebaceous gland carcinoma. Ophthalmologica 2007;221:331–9. 15. Kiratli H, Tarlan B, Fırat P. Primary sebaceous carcinoma of the lacrimal gland. Orbit 2012;31:352–4. 16. Galliani CA, Faught PR, Ellis FD. Adenoid cystic carcinoma of the lacrimal gland in a six-year-old girl. Pediatr Pathol 1993;13:559–65. 17. Thavaraj V, Sridhar MR, Sethi A, et al. Adenoid cystic carcinoma of the lacrimal gland. Indian J Pediatr 2003;70:751–3. 18. Lemos LB, Santa Cruz DJ, Baba N. Sebaceous carcinoma of the eyelid following radiation therapy. Am J Surg Pathol 1978;2:305–11. 19. Boniuk M, Zimmerman LE. Sebaceous carcinoma of the eye lid, eyebrow, caruncle, and orbit. Trans Am Acad Ophthalmol Otolaryngol 1968;72:619–42. 20. Prabhakaran VC, Cannon PS, McNab A, et al. Lesions mimicking lacrimal gland pleomorphic adenoma. Br J Ophthalmol 2010;94:1509–12.
Papillary Cystadenocarcinoma of the Lacrimal Gland After Radiation for Bilateral Retinoblastoma Shivali A. Menda, M.D., H. Jane Kim, M.D., Michele M. Bloomer, M.D., J. Brooks Crawford, M.D., M. Reza Vagefi, M.D., and Robert C. Kersten, M.D. Abstract: The authors report a case of papillary cystadenocarcinoma of the lacrimal gland after irradiation for bilateral retinoblastoma. A 32-year-old man with a history of bilateral retinoblastoma, diagnosed shortly after birth, was treated with enucleation of the OS and a single session of radiation to the OD. Over 30 years later, he presented with an orbital mass of the right lacrimal gland that on biopsy demonstrated papillary cystadenocarcinoma.
Department of Ophthalmology, University of California, San Francisco, California, U.S.A. Accepted for publication April 21, 2013. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Robert C. Kersten, M.D., Department of Ophthalmology, University of California, 10 Koret Way, San Francisco, CA 94143. E-mail: [email protected] DOI: 10.1097/IOP.0b013e31829bb43e
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
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Ophthal Plast Reconstr Surg, Vol. 30, No. 3, 2014
Case Reports
CASE REPORT A 32-year-old African American man presented with a palpable mass in the right superotemporal orbit and increasing painless proptosis over 2 months. The ocular history is significant for bilateral retinoblastoma diagnosed shortly after birth that required enucleation of the OS in 1979. The patient received 1 dose of radiation therapy on the right side. He did not receive chemotherapy. On presentation, visual acuity was 20/25 OD. External examination revealed an hourglass facial deformity. The right upper eyelid had an S-shaped configuration. Extraocular motility was full. Dilated examination was significant for choroidal folds and flat scar without calcification that likely represented treated retinoblastoma. MRI demonstrated an extraconal, heterogenous, and multilobulated mass involving the right lacrimal gland extending in the orbital apex with a small area of orbital roof erosion (Fig. 1). The patient underwent an anterior orbitotomy with gross surgical excision via an eyelid crease approach. The posterior extent of mass and capsule were incompletely excised to preserve vision. Intraoperatively, the mass appeared multicystic with chocolate-colored fluid. On microscopic examination, a cystic neoplasm with a papillary epithelial lining was observed (Fig. 2). Higher magnification revealed mild-to-moderate grade atypical epithelial cells with occasional mitotic figures (Fig. 2, inset). The tumor cells stained positively with EMA and keratin stains. The patient underwent a metastatic workup that included PET CT, and no evidence of extraorbital tumor was found. Postoperative MRI showed residual tumor in the right lacrimal fossa. The head and neck tumor board recommended a complete resection of the tumor with margins as patient was not a candidate for further radiation therapy, and there is no proven chemotherapy. Despite multiple discussions with the patient and his family, he refused any additional intervention.
DISCUSSION Lacrimal gland neoplasms represent 10% of primary orbital neoplasms with tumors of epithelial origin accounting for 20%.1–3 Of these, primary adenocarcinoma represents only 2% to 5% of epithelial neoplasms of the lacrimal gland.2,3 This tumor is subdivided into mucinous adenocarcinoma, ductal adenocarcinoma, and papillary cystadenocarcinoma types.1 Currently, there are only 2 case reports of cystadenocarcinoma of the lacrimal gland in the literature.4,5 To the authors’ knowledge, this rare neoplasm has not been previously described in the setting of orbital radiation for retinoblastoma. Hereditary retinoblastoma is associated with an increased risk of secondary malignancies.6,7 A familial history of retinoblastoma and of external beam radiation may portend an even higher risk.6,7 Most patients develop either bone or soft tissue malignancies.6 There are only 2 case reports of patients with secondary lacrimal gland neoplasms after treatment for retinoblastoma. One describes a 4-month-old girl with a history of bilateral retinoblastoma treated with external beam radiation, systemic chemotherapy, and enucleation of the OD, who developed a lacrimal gland pleomorphic adenoma 17 years after original treatment.8 Bubella et al.9 recently reported a case of lacrimal adenocarcinoma arising from a pleomorphic adenoma in a patient with bilateral retinoblastoma treated with radiation occurring 20 years later. Given epithelial neoplasms of the lacrimal gland are histologically similar to tumors of the major salivary glands and the rarity of papillary cystadenocarcinoma of the orbit, the head and neck literature can provide a reference point for treatment
e58
FIG. 1. A 32-year-old man presenting with painless proptosis of the OD with a history bilateral retinoblastoma treated previously with enucleation OS and orbital radiation OD. MRI demonstrates a multicystic tumor arising from the right lacrimal gland extending in the muscle cone with erosion of the orbital roof.
FIG. 2. Histopathology of the orbital specimen from a 32-year-old man with a lacrimal gland mass. The tumor is composed of a papillary epithelium lining cystic spaces (hematoxylin-eosin; ×10). On higher magnification, there is a squamous epithelium with prominent nucleoli and occasional mitotic figures (inset) (hematoxylin-eosin; ×40).
paradigms and outcomes of this tumor type.2 In 1 large series of 40 patients with salivary gland cystadenocarcinoma, treatment consisted of local excision of intraoral lesions with parotidectomy or lobectomy for parotid gland tumors.10 Ten percent of patients demonstrated metastatic disease and required regional lymphadenectomy followed by radiation therapy. Ninety percent of patients were either alive or had died of unrelated causes during a mean follow up of 59 months after initial excision. Three tumors recurred locally between 18 and 122 months. In only 2 cases of cystadenocarcinoma of the lacrimal gland, both patients were alive at 7 and 9 years after treatment with en bloc excision and no radiotherapy or chemotherapy.1,4,5 The patient’s monocular status placed him in a particularly difficult situation when contemplating further surgery after incomplete excision which he eventually deferred. In conclusion, a lacrimal gland neoplasm may occur rarely years after external beam radiation treatment for retinoblastoma. A tumor of lacrimal origin should be considered in the differential for new-onset proptosis in this patient subpopulation. To date, a pleomorphic adenoma, an adenocarcinoma arising from a pleomorphic adenoma and papillary cystadenocarcinoma, has been reported. Treatment of papillary cystadenocarcinoma is largely surgical with a guarded prognosis.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 30, No. 3, 2014
REFERENCES 1. Bernardini FP, Devoto MH, Croxatto JO. Epithelial tumors of the lacrimal gland: an update. Curr Opin Ophthalmol 2008;19:409–13. 2. Weis E, Rootman J, Joly TJ, et al. Epithelial lacrimal gland tumors: pathologic classification and current understanding. Arch Ophthalmol 2009;127:1016–28. 3. Shields CL, Shields JA, Eagle RC, et al. Clinicopathologic review of 142 cases of lacrimal gland lesions. Ophthalmology 1989;96:431–5. 4. Devoto MH, Croxatto JO. Primary cystadenocarcinoma of the lacrimal gland. Ophthalmology 2003;110:2006–10. 5. Peng X, Li G, Wang J, et al. Papillary cystadenocarcinoma of the lacrimal gland. Ann Ophthalmol (Skokie) 2010;42 Spec No:15–9. 6. Mohney BG, Robertson DM, Schomberg PJ, et al. Second nonocular tumors in survivors of heritable retinoblastoma and prior radiation therapy. Am J Ophthalmol 1998;126:269–77. 7. Moll AC, Imhof SM, Schouten-Van Meeteren AY, et al. Second primary tumors in hereditary retinoblastoma: a register-based study, 1945-1997: is there an age effect on radiation-related risk? Ophthalmology 2001;108:1109–14. 8. Hadjistilianou T, de Francesco S, Signori C, et al. Pleomorphic adenoma of the lacrimal gland in an 18-year-old girl irradiated for bilateral retinoblastoma. Orbit 2006;25:51–3. 9. Bubella DM, Bubella RM, Carità S, et al. Adenocarcinoma of the lacrimal gland in bilateral retinoblastoma treated with external radiotherapy: a case report. J Fr Ophtalmol 2011;34:647–50. 10. Foss RD, Ellis GL, Auclair PL. Salivary gland cystadenocarcinomas. A clinicopathologic study of 57 cases. Am J Surg Pathol 1996;20:1440–7.
Bilateral Dacryocystoceles in a Pregnant Woman Kristin E. Hirabayashi, B.A., Elizabeth Yang, M.D., Julio Echegoyen, M.D., Ph.D., Steven J. Yoon, M.D., and Jeremiah P. Tao, M.D., F.A.C.S. Abstract: The authors describe, for the first time, bilateral, sequential large dacryocystoceles during pregnancy and review the literature for this presentation. A 26-year-old, 15-week pregnant woman presented with OD epiphora, diplopia, and pain in the setting of an inferomedial orbital mass. Surgical exploration and histopathology were consistent with a dacryocystocele, and a dacryocystorhinostomy was curative. She returned at 34-week gestation, with an identical presentation on the left side. Review of the literature reveals that dacryocystoceles occasionally present in adults; however, bilateral involvement may be unusual. Bilateral dacryocystoceles have not been previously reported in a pregnant woman.
Case Reports
on the right side and fullness of the inferonasal orbit. There was mild tenderness to the medial lower eyelid and medial canthus but no erythema. Extraocular motility testing revealed reduced OD supraduction and adduction. Visual acuity was 20/200 OD (poor effort due to discomfort) and 20/20 OS. There was no afferent pupillary defect to corroborate an optic nerve etiology for the low vision. Intraocular pressures were 20 and 21 mm Hg. A dilated fundus examination was normal, but some inferonasal contour abnormalities consistent with external globe compression (but no ischemia or atrophy) was evident. The eye examination was otherwise normal. Lacrimal probing and irrigation revealed a complete distal obstruction of nasolacrimal outflow with all irrigated fluid regurgitating from the opposite, ipsilateral punctum. CT scans of the orbital region revealed a well-circumscribed mass in the inferomedial orbit, compressing the globe (Fig. 1). Oral clindamycin was initiated to treat presumptive dacryocystitis. At 10 days, medical management was unsuccessful, and the symptoms progressed. Surgical intervention for decompression or removal of the mass was recommended and accepted by the patient due to the nonresolving orbital signs. The intraoperative findings were consistent with a dacryocystocele, with the intralesional contents consisting of clear fluid and no mucopurulent, infectious material. Histopathologic analysis of a sac specimen demonstrated mild inflammation of otherwise normalappearing nasolacrimal sac tissue that was also negative for granulomas or malignancy. An external dacryocystorhinostomy was performed, and a Crawford silicone tube (JEDMED Instrument Company, St. Louis, MO, U.S.A.) was placed after draining the sac contents. Extraocular motility returned to normal, and the symptoms resolved. At 34-week gestation, the patient returned, complaining of diplopia, left epiphora, and OS pain with extraocular movements. External examination demonstrated fullness of the inferonasal left orbit. Extraocular motility testing showed reduced adduction and supraduction of the OS. Visual acuity was 20/20 OD and 20/25 OS. Intraocular pressures were 18 and 19 mm Hg. The eye examination, including dilated fundus examination, was normal. Some clear fluid, but no mucopurulent material was expressed, form the punctae with pressure over the lacrimal sac region. CT scans of the orbital region demonstrated a 1.31 × 1.37 cm well-circumscribed mass in the inferomedial orbit (Fig. 2). Given her difficulty with pain control and some decelerations on fetal heart tracings, the patient was admitted for monitoring, bed rest, and empirical treatment of a presumptive
CASE REPORT A 26-year-old woman at 15-week gestation presented with symptoms of diplopia, right epiphora, and OD pain with extraocular movements. She was otherwise healthy with no prior ocular history. External examination demonstrated an increased tear leak *Department of Ophthalmology, Gavin Herbert Eye Institute, University of California, Irvine, California; †Department of Ophthalmology, Yale Eye Center, Yale University, New Haven, Connecticut; ‡Department of Oculofacial Plastic and Orbital Surgery, Maryland Eye Associates, Annapolis, Maryland, U.S.A. Accepted for publication April 24, 2013. The authors have no financial or conflicts of interest to disclose. Supported, in part, by an institutional grant from research to prevent blindness. Address correspondence and reprint requests to Kristin E. Hirabayashi, Department of Ophthalmology, Gavin Herbert Eye Institute, University of California, Irvine, CA 92697. E-mail: [email protected] DOI: 10.1097/IOP.0b013e31829bb45c
FIG. 1. Orbitofacial CT of patient at 15-week gestation demonstrating a mass in the right orbital region.
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