Ophthal Plast Reconstr Surg, Vol. 32, No. 2, 2016
10. Kersten RC, Kulwin DR. “One-stitch” canalicular repair. A simplified approach for repair of canalicular laceration. Ophthalmology 1996;103:785–9. 11. Loff HJ, Wobig JL, Dailey RA. The bubble test: an atraumatic method for canalicular laceration repair. Ophthal Plast Reconstr Surg 1996;12:61–4. 12. McLeish WM, Bowman B, Anderson RL. The pigtail probe protected by silicone intubation: a combined approach to canalicular reconstruction. Ophthalmic Surg 1992;23:281–3. 13. Liu B, Li Y, Long C, et al. Novel air-injection technique to locate the medial cut end of lacerated canaliculus. Br J Ophthalmol 2013;97:1508–9.
Orbital Epithelioid Sarcoma: A Case Report Lama L. Jurdy, M.D.*, Leo E. Blank, M.D.†, Johannes Bras, M.D., Ph.D.‡, and Peerooz Saeed, M.D., Ph.D.* Abstract: Epithelioid sarcoma is a rare but often aggressive malignancy of soft tissue that usually occurs in young adults as a superficial lesion in the distal upper limbs. To date, there are only 4 case reports of epithelioid sarcoma primarily occurring in the orbit. Two of these patients were treated with primary exenteration only one of whom was alive 3 years after diagnosis. Radical surgical excision is thus the first treatment of choice for primary orbital epithelioid sarcoma. The authors present a patient with primary orbital epithelioid sarcoma who refused exenteration. Surgical debulking followed by local brachytherapy was performed. The patient remains tumor free 5 years after diagnosis. The literature remains limited regarding treatment options for primary orbital epithelioid sarcoma. However, based on reported cases and this case, the authors conclude that surgical excision combined with local iridium radiation therapy is an acceptable treatment when treating primary orbital epithelioid sarcoma.
E
pithelioid sarcoma (ES) is a rare (1% of soft tissue sarcomas), high-grade soft tissue sarcoma first recognized by Enzinger in 1970.1,3 It is known to most commonly affect the tendon sheaths in distal upper extremities in young adults with a 2:1 male predominance.1–4 It is also known to have a high propensity for local recurrence and distant metastasis; hence, the prognosis is poor, and radical surgical excision is paramount for successful treatment.3 ES is a soft tissue tumor of unknown etiology, most likely originating from mesenchymal tissue. The tumor is clinically characterized by a slow-growing swelling, most commonly in the distal part of the extremities, especially the hand, with other possible sites like proximal upper extremities, the trunk, the perineum, penis, and vulva being much less likely.1,4 The classical Departments of *Ophthalmology, †Radiation Oncology, and ‡Pathology, Academic Medical Center, University of Amsterdam, The Netherlands Accepted for publication April 14, 2014. Presented as a Poster at the ESOPRS September 2013 meeting in Barcelona. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Peerooz Saeed, M.D., Ph.D., Department of Ophthalmology, Academic Medical Center, University of Amsterdam, Meibergdreef 9, Amsterdam, 1105 AZ, the Netherlands. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000227
Case Reports
form (distal extremity) usually occurs in young adults, and the more recently described proximal tumor usually develops in older adults and predominantly involves the pelvis, perineum, and genital tract.1 The prognosis is unfavorable, with a 77% local recurrence rate and a 45% metastatic rate.1 Complete surgical resection is hence the first choice for treatment of ES to the extent of amputation as the first operative procedure.3,4 However, it is now accepted that a local resection achieving clear margins, combined with adjuvant radiotherapy is equally adequate.4 Surgery is usually followed by radiation therapy. Anecdotes of subsequent treatment with chemotherapy are found in the literature.5 To date, there are only 4 reported cases of ES primarily occurring in the orbit.1,2,6 Two of these patients were primarily treated with an orbital exenteration.2 The other two patients were initially treated with an extensive surgical debulking followed by chemotherapy.1,2 One of these 2 patients required an exenteration after a secondary local recurrence of the tumor, and he died 2 months later because of intracranial extension of a recurrent tumor.1 The one who had the extensive debulking followed by chemotherapy and no exenteration also passed away 29 months after diagnosis. In one of the patients who had undergone primary exenteration, one developed osseous metastases during the course of the disease and died during palliative chemotherapy 14 months after the initial diagnosis6. Hence, only 1 of the 2 patients who underwent primary exenteration was alive 3 years after diagnosis.2 This demonstrates the relentless progression of this orbital disease and emphasizes the importance of early detection, proper histopathologic diagnosis, and adequate surgical excision. ES poses a diagnostic challenge since it has an unclear histogenesis.3 It most likely originates from mesenchymal tissue with metaplastic conversion to epithelia with a nodular pseudogranulomatous appearance.2,4 A distinctive microscopic characteristic of ES is a mixture of epithelioid and spindle-shaped cells with palisading areas of central necrosis in a nodular infiltrate that resembles a granuloma.4 Immunohistochemistry is crucial for differentiation, and special stains and tissue cultures are important to exclude a granulomatous infection.4 ES characteristically exhibits reactivity for epithelial as well as mesenchymal markers.4 It is positive for cytokeratin, epithelial membrane antigen (EMA), vimentin, and CD34 in 50% of cases.4 Cytokeratin subtyping is of diagnostic importance in differentiating ES from synovial sarcoma or metastatic carcinoma.4
CASE REPORT The present study reports a 39-year-old woman patient who initially presented in early June 2008 with a recurrent
FIG. 1. Preoperative MR: T1-weighted MR showing a left medial intraorbital tumor.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
e47
Ophthal Plast Reconstr Surg, Vol. 32, No. 2, 2016
Case Reports
FIG. 2. Pathology: hematoxylin-eosin stain; partially spindlecell, mainly epithelioid malignant tumor. Intense staining forepithelial markers (including CK 19). Tumor is negative forendothelial markers CD31/factor VIII (excluding epithelioidangiosarcoma) and negative for S100/HMB45 (excluding clearcell sarcoma/melanoma).
mitoses were found (5/10 high power field) along with areas of focal necrosis. There was pronounced deposition of collagenous material between tumor cells and numerous small blood vessels with extravasated erythrocytes and focal accumulations of iron pigment. In the background, there was a dense mixed inflammatory infiltrate. The tumor was seen to grow in patches with its edges predominantly sharply defined. The cells were strongly positive for CD34 and epithelial markers: pankeratine, CAM5.2 (a cytokeratin antibody), EMA, and cytokeratin19. They were weakly positive for CD31 and negative for Factor VIII, Desmin, Myogenic Differentiation Antigen (MYOD1), S-100, and melanoma marker HMB45. These findings fit best with a highgrade ES (Fig. 2). Exenteration was recommended based on the diagnosis of primary orbital ES with possible involvement of the medial rectus and bulbus but was categorically refused by the patient. The tumor was macroscopically radically excised. A lateral orbitotomy was performed followed by dissection of the tissue around the tumor. Note was made that the medial rectus muscle did not appear to be involved with the tumor. Frozen sections of excised medial rectus muscle, nerve and tendon, and tenon, conjunctiva, caruncle, fat around the tumor area and bulbus tissue were tumor free. Two iridium moulages with catheters for local radiation therapy were then inserted, and brachytherapy was administered for a total of 4 days. The patient is now tumor free 5 years after initial presentation (Figure 3). Postoperative complications were diplopia, ptosis, trichiasis, and a dense cataract secondary to the radiation therapy. Surgical strabismus correction and ptosis correction were successfully performed. Phacoemulsification of the radiation-induced cataract was also performed with a final visual acuity of 0.8.
CONCLUSIONS FIG. 3. Postoperative MR: contrast-enhanced T1-weighted MR showing no evidence of tumor recurrence.
subconjunctival hemorrhage in her left eye. The patient was healthy and had no ophthalmic history. The best corrected visual acuity of her left eye was 1.25. Ophthalmic examination revealed no abnormalities with the exception of a slightly thickened caruncle and conjunctival hemorrhage in regression. Her pupillary responses were intact, and her eye movements and visual field examination were normal. The patient was seen again a month later with a recurrent conjunctival hemorrhage, now with headache and left periorbital pain. On the MRI an intraorbital, extraconal tumor was seen, measuring 1.9 × 1.5 × 2.2 cm, and compressing the medial bulbus with possible involvement of the medial rectus (Fig. 1). Multiple biopsies were taken through a transcaruncular approach. Immunohistochemical staining analysis revealed fusiform and epithelioid cells, which were large with an eosinophilic cytoplasm and pleomorphic, enlarged nucleoli. Frequent
e48
Although the literature remains limited regarding treatment options for primary orbital ES, on the basis of earlier reports and this case the authors conclude that surgical excision combined with local iridium radiation therapy is an acceptable alternative treatment in select cases when treating primary orbital ES.
REFERENCES 1. Alkatan HM, Chaudhry I, Al-Qahtani A. Epithelioid sarcoma of the orbit. Ann Saudi Med 2011;31:187–9. 2. White VA, Heathcote JG, Hurwitz JJ, et al. Epithelioid sarcoma of the orbit. Ophthalmology 1994;101:1680–7. 3. de Visscher SA, van Ginkel RJ, Wobbes T, et al. Epithelioid sarcoma: still an only surgically curable disease. Cancer 2006;107:606–12. 4. Burgos AM, Chávez JG, Sánchez JL, et al. Epithelioid sarcoma: a diagnostic and surgical challenge. Dermatol Surg 2009;35:687–91. 5. Tariq Z, Ghose A, Bawany MZ, et al. A case report of complete remission of pulmonary metastases from epithelioid sarcoma to navelbine chemotherapy. Am J Ther 2012;19:5–97. 6. Thranitz M, Berg T, Kneifel C, et al. Epithelioid sarcoma of the orbit. HNO 2013. Advance online publication. doi: 10.1007/ s00106-013-2780-6.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
10. Kersten RC, Kulwin DR. “One-stitch” canalicular repair. A simplified approach for repair of canalicular laceration. Ophthalmology 1996;103:785–9. 11. Loff HJ, Wobig JL, Dailey RA. The bubble test: an atraumatic method for canalicular laceration repair. Ophthal Plast Reconstr Surg 1996;12:61–4. 12. McLeish WM, Bowman B, Anderson RL. The pigtail probe protected by silicone intubation: a combined approach to canalicular reconstruction. Ophthalmic Surg 1992;23:281–3. 13. Liu B, Li Y, Long C, et al. Novel air-injection technique to locate the medial cut end of lacerated canaliculus. Br J Ophthalmol 2013;97:1508–9.
Orbital Epithelioid Sarcoma: A Case Report Lama L. Jurdy, M.D.*, Leo E. Blank, M.D.†, Johannes Bras, M.D., Ph.D.‡, and Peerooz Saeed, M.D., Ph.D.* Abstract: Epithelioid sarcoma is a rare but often aggressive malignancy of soft tissue that usually occurs in young adults as a superficial lesion in the distal upper limbs. To date, there are only 4 case reports of epithelioid sarcoma primarily occurring in the orbit. Two of these patients were treated with primary exenteration only one of whom was alive 3 years after diagnosis. Radical surgical excision is thus the first treatment of choice for primary orbital epithelioid sarcoma. The authors present a patient with primary orbital epithelioid sarcoma who refused exenteration. Surgical debulking followed by local brachytherapy was performed. The patient remains tumor free 5 years after diagnosis. The literature remains limited regarding treatment options for primary orbital epithelioid sarcoma. However, based on reported cases and this case, the authors conclude that surgical excision combined with local iridium radiation therapy is an acceptable treatment when treating primary orbital epithelioid sarcoma.
E
pithelioid sarcoma (ES) is a rare (1% of soft tissue sarcomas), high-grade soft tissue sarcoma first recognized by Enzinger in 1970.1,3 It is known to most commonly affect the tendon sheaths in distal upper extremities in young adults with a 2:1 male predominance.1–4 It is also known to have a high propensity for local recurrence and distant metastasis; hence, the prognosis is poor, and radical surgical excision is paramount for successful treatment.3 ES is a soft tissue tumor of unknown etiology, most likely originating from mesenchymal tissue. The tumor is clinically characterized by a slow-growing swelling, most commonly in the distal part of the extremities, especially the hand, with other possible sites like proximal upper extremities, the trunk, the perineum, penis, and vulva being much less likely.1,4 The classical Departments of *Ophthalmology, †Radiation Oncology, and ‡Pathology, Academic Medical Center, University of Amsterdam, The Netherlands Accepted for publication April 14, 2014. Presented as a Poster at the ESOPRS September 2013 meeting in Barcelona. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Peerooz Saeed, M.D., Ph.D., Department of Ophthalmology, Academic Medical Center, University of Amsterdam, Meibergdreef 9, Amsterdam, 1105 AZ, the Netherlands. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000227
Case Reports
form (distal extremity) usually occurs in young adults, and the more recently described proximal tumor usually develops in older adults and predominantly involves the pelvis, perineum, and genital tract.1 The prognosis is unfavorable, with a 77% local recurrence rate and a 45% metastatic rate.1 Complete surgical resection is hence the first choice for treatment of ES to the extent of amputation as the first operative procedure.3,4 However, it is now accepted that a local resection achieving clear margins, combined with adjuvant radiotherapy is equally adequate.4 Surgery is usually followed by radiation therapy. Anecdotes of subsequent treatment with chemotherapy are found in the literature.5 To date, there are only 4 reported cases of ES primarily occurring in the orbit.1,2,6 Two of these patients were primarily treated with an orbital exenteration.2 The other two patients were initially treated with an extensive surgical debulking followed by chemotherapy.1,2 One of these 2 patients required an exenteration after a secondary local recurrence of the tumor, and he died 2 months later because of intracranial extension of a recurrent tumor.1 The one who had the extensive debulking followed by chemotherapy and no exenteration also passed away 29 months after diagnosis. In one of the patients who had undergone primary exenteration, one developed osseous metastases during the course of the disease and died during palliative chemotherapy 14 months after the initial diagnosis6. Hence, only 1 of the 2 patients who underwent primary exenteration was alive 3 years after diagnosis.2 This demonstrates the relentless progression of this orbital disease and emphasizes the importance of early detection, proper histopathologic diagnosis, and adequate surgical excision. ES poses a diagnostic challenge since it has an unclear histogenesis.3 It most likely originates from mesenchymal tissue with metaplastic conversion to epithelia with a nodular pseudogranulomatous appearance.2,4 A distinctive microscopic characteristic of ES is a mixture of epithelioid and spindle-shaped cells with palisading areas of central necrosis in a nodular infiltrate that resembles a granuloma.4 Immunohistochemistry is crucial for differentiation, and special stains and tissue cultures are important to exclude a granulomatous infection.4 ES characteristically exhibits reactivity for epithelial as well as mesenchymal markers.4 It is positive for cytokeratin, epithelial membrane antigen (EMA), vimentin, and CD34 in 50% of cases.4 Cytokeratin subtyping is of diagnostic importance in differentiating ES from synovial sarcoma or metastatic carcinoma.4
CASE REPORT The present study reports a 39-year-old woman patient who initially presented in early June 2008 with a recurrent
FIG. 1. Preoperative MR: T1-weighted MR showing a left medial intraorbital tumor.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
e47
Ophthal Plast Reconstr Surg, Vol. 32, No. 2, 2016
Case Reports
FIG. 2. Pathology: hematoxylin-eosin stain; partially spindlecell, mainly epithelioid malignant tumor. Intense staining forepithelial markers (including CK 19). Tumor is negative forendothelial markers CD31/factor VIII (excluding epithelioidangiosarcoma) and negative for S100/HMB45 (excluding clearcell sarcoma/melanoma).
mitoses were found (5/10 high power field) along with areas of focal necrosis. There was pronounced deposition of collagenous material between tumor cells and numerous small blood vessels with extravasated erythrocytes and focal accumulations of iron pigment. In the background, there was a dense mixed inflammatory infiltrate. The tumor was seen to grow in patches with its edges predominantly sharply defined. The cells were strongly positive for CD34 and epithelial markers: pankeratine, CAM5.2 (a cytokeratin antibody), EMA, and cytokeratin19. They were weakly positive for CD31 and negative for Factor VIII, Desmin, Myogenic Differentiation Antigen (MYOD1), S-100, and melanoma marker HMB45. These findings fit best with a highgrade ES (Fig. 2). Exenteration was recommended based on the diagnosis of primary orbital ES with possible involvement of the medial rectus and bulbus but was categorically refused by the patient. The tumor was macroscopically radically excised. A lateral orbitotomy was performed followed by dissection of the tissue around the tumor. Note was made that the medial rectus muscle did not appear to be involved with the tumor. Frozen sections of excised medial rectus muscle, nerve and tendon, and tenon, conjunctiva, caruncle, fat around the tumor area and bulbus tissue were tumor free. Two iridium moulages with catheters for local radiation therapy were then inserted, and brachytherapy was administered for a total of 4 days. The patient is now tumor free 5 years after initial presentation (Figure 3). Postoperative complications were diplopia, ptosis, trichiasis, and a dense cataract secondary to the radiation therapy. Surgical strabismus correction and ptosis correction were successfully performed. Phacoemulsification of the radiation-induced cataract was also performed with a final visual acuity of 0.8.
CONCLUSIONS FIG. 3. Postoperative MR: contrast-enhanced T1-weighted MR showing no evidence of tumor recurrence.
subconjunctival hemorrhage in her left eye. The patient was healthy and had no ophthalmic history. The best corrected visual acuity of her left eye was 1.25. Ophthalmic examination revealed no abnormalities with the exception of a slightly thickened caruncle and conjunctival hemorrhage in regression. Her pupillary responses were intact, and her eye movements and visual field examination were normal. The patient was seen again a month later with a recurrent conjunctival hemorrhage, now with headache and left periorbital pain. On the MRI an intraorbital, extraconal tumor was seen, measuring 1.9 × 1.5 × 2.2 cm, and compressing the medial bulbus with possible involvement of the medial rectus (Fig. 1). Multiple biopsies were taken through a transcaruncular approach. Immunohistochemical staining analysis revealed fusiform and epithelioid cells, which were large with an eosinophilic cytoplasm and pleomorphic, enlarged nucleoli. Frequent
e48
Although the literature remains limited regarding treatment options for primary orbital ES, on the basis of earlier reports and this case the authors conclude that surgical excision combined with local iridium radiation therapy is an acceptable alternative treatment in select cases when treating primary orbital ES.
REFERENCES 1. Alkatan HM, Chaudhry I, Al-Qahtani A. Epithelioid sarcoma of the orbit. Ann Saudi Med 2011;31:187–9. 2. White VA, Heathcote JG, Hurwitz JJ, et al. Epithelioid sarcoma of the orbit. Ophthalmology 1994;101:1680–7. 3. de Visscher SA, van Ginkel RJ, Wobbes T, et al. Epithelioid sarcoma: still an only surgically curable disease. Cancer 2006;107:606–12. 4. Burgos AM, Chávez JG, Sánchez JL, et al. Epithelioid sarcoma: a diagnostic and surgical challenge. Dermatol Surg 2009;35:687–91. 5. Tariq Z, Ghose A, Bawany MZ, et al. A case report of complete remission of pulmonary metastases from epithelioid sarcoma to navelbine chemotherapy. Am J Ther 2012;19:5–97. 6. Thranitz M, Berg T, Kneifel C, et al. Epithelioid sarcoma of the orbit. HNO 2013. Advance online publication. doi: 10.1007/ s00106-013-2780-6.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
![[Orbital epithelioid hemangioma: a case report].](/assets/img/hold.png)
