Orbit, 2014; 33(5): 378–381 ! Informa Healthcare USA, Inc. ISSN: 0167-6830 print / 1744-5108 online DOI: 10.3109/01676830.2014.894541
C ASE REPORT
Transitional Cell-Type Papillary Carcinoma of the Lacrimal Sac Manifesting as a Medial Canthal Skin Lesion Benjamin P. Erickson1, Yasha S. Modi1, Marcus J. Ko1, Rehan M. Hussain1, Carmen Gomez-Fernandez2, and Thomas E. Johnson1 1
Bascom Palmer Eye Institute, Miami, Florida, USA and 2University of Miami Hospital, Department of Pathology, Miami, Florida, USA
ABSTRACT A 61-year-old man presented with a 2.5 cm fungating skin lesion above the right medial canthus, accompanied by fluctuance and subtle inferotemporal globe displacement. The tumor had erupted 2 months previously and grown rapidly. Computed tomography (CT) revealed a 4.2 2.8 2.1 cm exophytic mass of the right orbit, superimposed on chronic dacryocystitis. Incisional biopsy disclosed faulty epithelial maturation sequence with possible basement membrane invasion. The patient underwent wide excision with medial maxillectomy, inferior turbinectomy, ethmoidectomy, and partial rhinectomy. The orbital floor was then reconstructed; a paramedian forehead flap and myocutaneous cheek advancement flap were used to fill cutaneous defects. All surgical margins were negative on both frozen and permanent sections. Definitive histopathology was consistent with transitional cell type papillary carcinoma of the lacrimal sac. Adjuvant radiotherapy was recommended, but the patient has declined further treatment. He remains recurrence-free at one year. Keywords: Cutaneous lesion, lacrimal sac tumor, orbital tumor, transitional cell carcinoma
Primary epithelial malignancy of the lacrimal drainage system is rare, but rates of local recurrence and mortality are high.1 Approximately 400 cases have been reported, including 37 of transitional cell carcinomas.2,3 Epiphora, recurrent dacryocystitis, and palpable medial canthal masses are the most common initial symptoms.4,5,6 We present the management of an unusual case of transitional cell carcinoma, in which a fungating skin lesion in the medial canthal region was the main finding at presentation.
mass above the right medial canthus was found incidentally. Fluctuance was also present inferior to the medial canthal tendon. Further examination disclosed subtle inferotemporal globe displacement and mild deficits in adduction and supraduction (Figure 1A). The patient stated that symptoms began less than a year ago with facial swelling and intermittent mucoid discharge. He reported having received multiple courses of oral antibiotics with only partial resolution. The lesion itself erupted 2 months prior to presentation and had since grown rapidly. CT revealed a 4.2 2.8 2.1 cm exophytic mass of the right orbit, superimposed on chronic dacryocystitis. It was contiguous with both the skin lesion and lacrimal sac, and extended through the nasolacrimal duct, culminating in a 9 6 mm soft tissue element within the nasal cavity (Figure 1B). No regional or
Case Report A 61-year-old man presented to the emergency department intoxicated, with a urine drug screen positive for cocaine. A 2.5-cm diameter fungating
Received 3 November 2013; Revised 21 January 2014; Accepted 11 February 2014; Published online 28 April 2014 Correspondence: Thomas Johnson, M.D., Bascom Palmer Eye Institute, 900 NW 17th Street, Miami, FL 33131, USA. E-mail: [email protected]
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FIGURE 1. (A) 61-year-old male with a fungating lesion of the right medial canthus. (B) CT scan of the orbits demonstrating continuity between the skin lesion and ipsilateral lacrimal sac, accompanied by underlying chronic dacryocystitis.
FIGURE 2. (A) External appearance at the time of surgery; note the significant interval growth of the cutaneous lesion. (B) Resected specimen, including the entire lacrimal drainage system. (C) The medial orbital floor reconstructed with titanium mesh. (D) Appearance after canthal reconstruction and rotation of the paramedian forehead flap.
!
2014 Informa Healthcare USA, Inc.
380 B. P. Erickson et al. metastatic spread was apparent on scans of the brain, neck and chest. Bedside incisional biopsy of the skin lesion disclosed full-thickness faulty epithelial maturation sequence with areas of possible basement membrane invasion. Review by multiple pathologists yielded a differential that included squamous cell carcinoma, verrucus carcinoma, and transitional cell carcinoma of the lacrimal sac. The patient subsequently underwent wide excision with medial maxillectomy, inferior turbinectomy, ethmoidectomy, and partial rhinectomy.
Adequate tumor-free margins were obtained while sparing the globe. The orbital floor was rebuilt with titanium mesh and porous polyethylene. The medial canthus was reconstructed; a paramedian forehead flap and myocutaneous cheek advancement flap were then used to fill cutaneous defects (Figure 2). All surgical margins were negative on frozen and permanent sections. Staining was positive for cytokeratin 7 and negative for cytokeratin 20. Definitive histopathology was most consistent with transitional cell-type papillary carcinoma (Figure 3).
FIGURE 3. (A) A fungating mass composed of transitional cell-type epithelium overlying branching fibrovascular cores (hematoxylin & eosin, 100). (B) Full-thickness faulty epithelial maturational sequencing, with areas of possible basement membrane invasion (arrow)(hematoxylin & eosin, 200). Immunohistochemistry stains positively for cytokeratin 7 (C) and negatively for cytokeratin 20 (D) (100). Orbit
Lacrimal Sac Transitional Cell Carcinoma The forehead flap pedicle was divided after the first postoperative month. Adjuvant radiotherapy was strongly recommended, but the patient has declined further treatment. He remains recurrencefree at one year.
Comment The lacrimal sac contains goblet cells as well as stratified columnar and ciliated respiratory epithelium.1,7 Neoplasia therefore produces a variety of epithelial malignancies, including squamous cell, transitional cell, mucoepidermoid, adenoid cystic, and adenocarcinoma.1,2 The majority arise de novo, though transformation of benign papillomas is not uncommon.6 Transitional cell carcinoma is the second most common epithelial malignancy after squamous cell, accounting for 13% to 15% of tumors.4,8 Mean age at presentation is 50, while that for benign papillomas is nearly a decade younger.2,3,4 Lacrimal sac carcinoma typically manifests with a firm, subcutaneous mass arising above the medial canthal tendon. This corresponds with the anatomic location of the minimally distensible fundus of the lacrimal sac; by contrast dacryoceles and chronic dacryocystitis protrude below the canthal tendon. Tearing complaints are common and in some instances precede the appearance of a mass lesion – a fact that must be kept in mind when preforming routine dacryocystorhinostomy. In later stages, orbital invasion may produce non-axial displacement and/or proptosis of the globe.6 Although telangectasias, fistulae and ulceration of the overlying skin have been described previously, a full-blown cutaneous manifestation – as in our case – is extremely unusual.3,6 Histologic differentiation from squamous cell carcinoma may be difficult and is to some extent subjective.4 Lesions can, in fact, contain areas of squamous differentiation. It also is not uncommon for a diagnosis to change after definitive surgical resection. Preechawai and colleagues reported a case of papillary transitional cell carcinoma that initially was diagnosed as squamous papilloma with dysplasia on incisional biopsy.9 En-bloc excision of the tumor and lacrimal drainage system – along with variable excision of the nose, sinuses and turbinates – remains the standard of care for advanced lesions.5,7 Although evidence is limited, many authors advocate adjuvant radiotherapy even for cases with negative margins.1,2,10 Proton therapy in particular permits delivery of radiation
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2014 Informa Healthcare USA, Inc.
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with less potential toxicity to the visual apparatus.4 Careful pre-treatment review of histopathology is essential, however, as radiation can catalyze malignant transformation in benign papillomas. The estimated rates of local recurrence and mortality after recurrence are both nearly 50% and appear roughly comparable to those for squamous cell carcinoma.1,3 Metastatic spread, though rare, may affect the lungs, esophagus, and liver.4,5 The incidence of metastases in the Armed Forces Institute of Pathology cohort was 9.1%.3 Given the high risk of recurrence, lifetime follow-up is mandatory. This unusual case of transitional cell type papillary carcinoma of the lacrimal gland suggests that it must be included in the differential for fungating skin lesions of the medial canthus.
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. Islam S, Thomas A, Eisenberg RL, Hoffman GR. Surgical management of transitional cell carcinoma of the lacrimal sac: Is it time for a new treatment algorithm? J Plast Reconstr Aesthet Surg 2012;65(2):e33–e36 2. Azari AA, Kanavi MR, Saipe N, et al. Transitional cell carcinoma of the lacrimal sac presenting with bloody tears. JAMA Ophthalmol 2013;131(5):689–690. 3. Stefanyszyn MA, Hidayat AA, Pe’er JJ, Flanagan JC. Lacrimal sac tumors. Ophthal Plast Reconstr Surg 1994; 10(3):169–184. 4. Heathcote JG. Transitional neoplasms of the naso-lacrimal system: A review of the histopathology and histogenesis. Saudi J Ophthalmol 2012;26(2):125–131. 5. Montalban A, Lie´tin B, Louvrier C, et al. Malignant lacrimal sac tumors. Eur Ann Otorhinolaryngol Head Neck Dis 2010;127(5):165–172. 6. Parmar DN, Rose GE. Management of lacrimal sac tumours. Eye (Lond) 2003;17(5):599–606. 7. Fishman JR, Gladstone GJ, Jackson IT. Squamous cell carcinoma of the lacrimal sac. Plast Reconstr Surg 1993; 92(7):1375–1379. 8. Ni C, D’Amico DJ, Fan CQ, et al. Tumors of the lacrimal sac: a clinicopathological analysis of 82 cases. Int Ophthalmol Clin 1982;1:121–140. 9. Preechawai P, Della Roccad RC, Della Rocca D, et al. Transitional cell carcinoma of the lacrimal sac. J Med Assoc Thai 2005;88 Suppl 9:S138–S142. 10. Katircioglu YA, Altiparmak UE, Akmansu H, et al. Squamous cell carcinoma of the lacrimal sac. Orbit 2003; 22(3):151–153.
Copyright of Orbit is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
C ASE REPORT
Transitional Cell-Type Papillary Carcinoma of the Lacrimal Sac Manifesting as a Medial Canthal Skin Lesion Benjamin P. Erickson1, Yasha S. Modi1, Marcus J. Ko1, Rehan M. Hussain1, Carmen Gomez-Fernandez2, and Thomas E. Johnson1 1
Bascom Palmer Eye Institute, Miami, Florida, USA and 2University of Miami Hospital, Department of Pathology, Miami, Florida, USA
ABSTRACT A 61-year-old man presented with a 2.5 cm fungating skin lesion above the right medial canthus, accompanied by fluctuance and subtle inferotemporal globe displacement. The tumor had erupted 2 months previously and grown rapidly. Computed tomography (CT) revealed a 4.2 2.8 2.1 cm exophytic mass of the right orbit, superimposed on chronic dacryocystitis. Incisional biopsy disclosed faulty epithelial maturation sequence with possible basement membrane invasion. The patient underwent wide excision with medial maxillectomy, inferior turbinectomy, ethmoidectomy, and partial rhinectomy. The orbital floor was then reconstructed; a paramedian forehead flap and myocutaneous cheek advancement flap were used to fill cutaneous defects. All surgical margins were negative on both frozen and permanent sections. Definitive histopathology was consistent with transitional cell type papillary carcinoma of the lacrimal sac. Adjuvant radiotherapy was recommended, but the patient has declined further treatment. He remains recurrence-free at one year. Keywords: Cutaneous lesion, lacrimal sac tumor, orbital tumor, transitional cell carcinoma
Primary epithelial malignancy of the lacrimal drainage system is rare, but rates of local recurrence and mortality are high.1 Approximately 400 cases have been reported, including 37 of transitional cell carcinomas.2,3 Epiphora, recurrent dacryocystitis, and palpable medial canthal masses are the most common initial symptoms.4,5,6 We present the management of an unusual case of transitional cell carcinoma, in which a fungating skin lesion in the medial canthal region was the main finding at presentation.
mass above the right medial canthus was found incidentally. Fluctuance was also present inferior to the medial canthal tendon. Further examination disclosed subtle inferotemporal globe displacement and mild deficits in adduction and supraduction (Figure 1A). The patient stated that symptoms began less than a year ago with facial swelling and intermittent mucoid discharge. He reported having received multiple courses of oral antibiotics with only partial resolution. The lesion itself erupted 2 months prior to presentation and had since grown rapidly. CT revealed a 4.2 2.8 2.1 cm exophytic mass of the right orbit, superimposed on chronic dacryocystitis. It was contiguous with both the skin lesion and lacrimal sac, and extended through the nasolacrimal duct, culminating in a 9 6 mm soft tissue element within the nasal cavity (Figure 1B). No regional or
Case Report A 61-year-old man presented to the emergency department intoxicated, with a urine drug screen positive for cocaine. A 2.5-cm diameter fungating
Received 3 November 2013; Revised 21 January 2014; Accepted 11 February 2014; Published online 28 April 2014 Correspondence: Thomas Johnson, M.D., Bascom Palmer Eye Institute, 900 NW 17th Street, Miami, FL 33131, USA. E-mail: [email protected]
378
Lacrimal Sac Transitional Cell Carcinoma
379
FIGURE 1. (A) 61-year-old male with a fungating lesion of the right medial canthus. (B) CT scan of the orbits demonstrating continuity between the skin lesion and ipsilateral lacrimal sac, accompanied by underlying chronic dacryocystitis.
FIGURE 2. (A) External appearance at the time of surgery; note the significant interval growth of the cutaneous lesion. (B) Resected specimen, including the entire lacrimal drainage system. (C) The medial orbital floor reconstructed with titanium mesh. (D) Appearance after canthal reconstruction and rotation of the paramedian forehead flap.
!
2014 Informa Healthcare USA, Inc.
380 B. P. Erickson et al. metastatic spread was apparent on scans of the brain, neck and chest. Bedside incisional biopsy of the skin lesion disclosed full-thickness faulty epithelial maturation sequence with areas of possible basement membrane invasion. Review by multiple pathologists yielded a differential that included squamous cell carcinoma, verrucus carcinoma, and transitional cell carcinoma of the lacrimal sac. The patient subsequently underwent wide excision with medial maxillectomy, inferior turbinectomy, ethmoidectomy, and partial rhinectomy.
Adequate tumor-free margins were obtained while sparing the globe. The orbital floor was rebuilt with titanium mesh and porous polyethylene. The medial canthus was reconstructed; a paramedian forehead flap and myocutaneous cheek advancement flap were then used to fill cutaneous defects (Figure 2). All surgical margins were negative on frozen and permanent sections. Staining was positive for cytokeratin 7 and negative for cytokeratin 20. Definitive histopathology was most consistent with transitional cell-type papillary carcinoma (Figure 3).
FIGURE 3. (A) A fungating mass composed of transitional cell-type epithelium overlying branching fibrovascular cores (hematoxylin & eosin, 100). (B) Full-thickness faulty epithelial maturational sequencing, with areas of possible basement membrane invasion (arrow)(hematoxylin & eosin, 200). Immunohistochemistry stains positively for cytokeratin 7 (C) and negatively for cytokeratin 20 (D) (100). Orbit
Lacrimal Sac Transitional Cell Carcinoma The forehead flap pedicle was divided after the first postoperative month. Adjuvant radiotherapy was strongly recommended, but the patient has declined further treatment. He remains recurrencefree at one year.
Comment The lacrimal sac contains goblet cells as well as stratified columnar and ciliated respiratory epithelium.1,7 Neoplasia therefore produces a variety of epithelial malignancies, including squamous cell, transitional cell, mucoepidermoid, adenoid cystic, and adenocarcinoma.1,2 The majority arise de novo, though transformation of benign papillomas is not uncommon.6 Transitional cell carcinoma is the second most common epithelial malignancy after squamous cell, accounting for 13% to 15% of tumors.4,8 Mean age at presentation is 50, while that for benign papillomas is nearly a decade younger.2,3,4 Lacrimal sac carcinoma typically manifests with a firm, subcutaneous mass arising above the medial canthal tendon. This corresponds with the anatomic location of the minimally distensible fundus of the lacrimal sac; by contrast dacryoceles and chronic dacryocystitis protrude below the canthal tendon. Tearing complaints are common and in some instances precede the appearance of a mass lesion – a fact that must be kept in mind when preforming routine dacryocystorhinostomy. In later stages, orbital invasion may produce non-axial displacement and/or proptosis of the globe.6 Although telangectasias, fistulae and ulceration of the overlying skin have been described previously, a full-blown cutaneous manifestation – as in our case – is extremely unusual.3,6 Histologic differentiation from squamous cell carcinoma may be difficult and is to some extent subjective.4 Lesions can, in fact, contain areas of squamous differentiation. It also is not uncommon for a diagnosis to change after definitive surgical resection. Preechawai and colleagues reported a case of papillary transitional cell carcinoma that initially was diagnosed as squamous papilloma with dysplasia on incisional biopsy.9 En-bloc excision of the tumor and lacrimal drainage system – along with variable excision of the nose, sinuses and turbinates – remains the standard of care for advanced lesions.5,7 Although evidence is limited, many authors advocate adjuvant radiotherapy even for cases with negative margins.1,2,10 Proton therapy in particular permits delivery of radiation
!
2014 Informa Healthcare USA, Inc.
381
with less potential toxicity to the visual apparatus.4 Careful pre-treatment review of histopathology is essential, however, as radiation can catalyze malignant transformation in benign papillomas. The estimated rates of local recurrence and mortality after recurrence are both nearly 50% and appear roughly comparable to those for squamous cell carcinoma.1,3 Metastatic spread, though rare, may affect the lungs, esophagus, and liver.4,5 The incidence of metastases in the Armed Forces Institute of Pathology cohort was 9.1%.3 Given the high risk of recurrence, lifetime follow-up is mandatory. This unusual case of transitional cell type papillary carcinoma of the lacrimal gland suggests that it must be included in the differential for fungating skin lesions of the medial canthus.
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. Islam S, Thomas A, Eisenberg RL, Hoffman GR. Surgical management of transitional cell carcinoma of the lacrimal sac: Is it time for a new treatment algorithm? J Plast Reconstr Aesthet Surg 2012;65(2):e33–e36 2. Azari AA, Kanavi MR, Saipe N, et al. Transitional cell carcinoma of the lacrimal sac presenting with bloody tears. JAMA Ophthalmol 2013;131(5):689–690. 3. Stefanyszyn MA, Hidayat AA, Pe’er JJ, Flanagan JC. Lacrimal sac tumors. Ophthal Plast Reconstr Surg 1994; 10(3):169–184. 4. Heathcote JG. Transitional neoplasms of the naso-lacrimal system: A review of the histopathology and histogenesis. Saudi J Ophthalmol 2012;26(2):125–131. 5. Montalban A, Lie´tin B, Louvrier C, et al. Malignant lacrimal sac tumors. Eur Ann Otorhinolaryngol Head Neck Dis 2010;127(5):165–172. 6. Parmar DN, Rose GE. Management of lacrimal sac tumours. Eye (Lond) 2003;17(5):599–606. 7. Fishman JR, Gladstone GJ, Jackson IT. Squamous cell carcinoma of the lacrimal sac. Plast Reconstr Surg 1993; 92(7):1375–1379. 8. Ni C, D’Amico DJ, Fan CQ, et al. Tumors of the lacrimal sac: a clinicopathological analysis of 82 cases. Int Ophthalmol Clin 1982;1:121–140. 9. Preechawai P, Della Roccad RC, Della Rocca D, et al. Transitional cell carcinoma of the lacrimal sac. J Med Assoc Thai 2005;88 Suppl 9:S138–S142. 10. Katircioglu YA, Altiparmak UE, Akmansu H, et al. Squamous cell carcinoma of the lacrimal sac. Orbit 2003; 22(3):151–153.
Copyright of Orbit is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
