Case report
Malignant melanoma of the lacrimal sac: a case report Jiro Maegawa1, MD, Kazunori Yasumura1, MD, Toshinori Iwai2, DDS, Masaharu Hata3, MD, Yoshiaki Inayama4, MD, and Shinji Kobayashi5, MD
Departments of 1Plastic and Reconstructive Surgery, 2Maxillofacial Surgery, 3Radiology, and 4Diagnostic Pathology, Yokohama City University Hospital, Kanazawa-ku, and 5 Department of Plastic and Reconstructive Surgery, Kanagawa Children Medical Center, Yokohama, Japan Correspondence Jiro Maegawa, MD Department of Plastic and Reconstructive Surgery Yokohama City University Hospital 3-9 Fukuura, Kanazawa-ku Yokohama 236-0004 Japan E-mail: [email protected] Conflicts of interest: None.
Introduction Malignant melanoma of the lacrimal sac is rare.1–11 A case of lacrimal sac melanoma in a 61-year-old man is presented. Case report A 61-year-old patient had noticed a mass in the medial canthal region of the left palpebra for about one year. He visited an ophthalmologist because the mass gradually grew, and there were bloody clots from the left nasal cavity. The ophthalmologist referred the patient to our hospital suspecting malignancy. During the first visit, the patient complained of epiphora, a mass 34 · 23 mm in size, and slight pain in the medial canthal region (Fig. 1) without obvious metastases to regional lymph nodes and transit lesions in the canthal region and nasal mucosa. Computed tomography and magnetic resonance imaging (Fig. 2) revealed a clearly defined mass occupying the lacrimal sac and duct, expanding into the surrounding bone and soft tissue. A biopsy specimen revealed typical characteristics of malignant melanoma. ª 2013 The International Society of Dermatology
Carbon ion radiotherapy was then considered as the first choice of treatment for the head and neck region;12 this up-to-date radiotherapy was available at a nearby specialist institute. However, a positron emission tomography scan performed to identify distant metastases revealed multiple liver metastases, which were clinical stage IV in this patient. According to the protocol for
Figure 1 Closed frontal view of the patient. Black colored
mass in the left-side, medial canthal area (arrow) International Journal of Dermatology 2014, 53, 243–245
243
244
Case report
Malignant melanoma of the lacrimal sac
Maegawa et al.
Figure 2 T2-weighted coronal magnetic resonance imaging
demonstrating a hypointense mass lesion in the lacrimal sac and nasolacrimal duct
(a)
head and neck melanoma at the institute, which had a limited capacity for carbon ion radiotherapy, patients with melanoma but without metastasis had priority over those with metastasis for this treatment. Therefore, carbon ion radiotherapy was not indicated in this case. Two weeks after biopsy, the strategy for treating this head and neck melanoma was changed to include en bloc excision and flap reconstruction under general anesthesia to control local tumor invasion. Through a lateral rhinotomy, an en bloc excision, including the lacrimal apparatus, medial wall of the orbit, and medial upper and lower lids (Fig. 3a), and a medial maxillectomy were performed. A T-shaped, median forehead flap repaired the defect left by excision (Fig. 3b). Histopathological examination showed a 30 · 23 · 20 mm melanoma occupying the lacrimal sac and nasolacrimal duct with invasion of the maxilla. The resected specimen had a clear surgical margin. Hematoxylin and eosin staining revealed epithelioid cells in sheets containing a significant amount of melanin, and immunohistochemical analysis was positive for HMB45. The patient received three cycles of dacarbazine, nimustine, vincristine, and interferon-beta, but he subsequently developed other metastases in the gastric mucosa, lung, and brain 10 months after surgery. At the time of the patient’s death, there was no sign of local tumor recurrence.
(b)
Figure 3 Excised specimen including the lacrimal apparatus, medial wall of the orbit, and medial upper and lower lids and a
probe in the lacrimal tract (a). After excision of the tumor, the nasal cavity, the maxillary sinus, and the orbital contents on the left side are exposed (b). A T-shaped, median forehead flap repaired the defect left by excision
International Journal of Dermatology 2014, 53, 243–245
ª 2013 The International Society of Dermatology
Maegawa et al.
Malignant melanoma of the lacrimal sac
Case report
Discussion
References
Since Duguid’s paper,1 approximately 25–30 reports of lacrimal sac melanoma have been published.1–11,13 Bloody epiphora and a mass in the medial canthal region are the most common symptoms.1–9 Based on a review of available reports, the average age of patients with lacrimal melanoma was about 58 years, and 35% (six of 21) of patients developed metastasis more than six months after treatment.1–11,13 Magnetic resonance imaging has more recently been used to identify further characteristic features of melanoma of the lacrimal sac,13 but imaging studies do not confirm the diagnosis of melanoma; imaging shows the local extent of disease and is part of disease staging. Treatment of metastatic melanoma remains problematic. The efficacy of anti-CTLA4 (cytotoxic T-lymphocyte antigen-4) therapy with ipilimumab has been reported; the therapy improves overall survival in patients with advanced and metastatic melanoma,14 but it was not available at that time in Japan. Regarding genetic mutations with melanoma, it has been reported that mucosal and acral melanoma harbor more KIT-activating mutations than do other subtypes of melanoma, which suggests that KIT inhibitors may potentially be effective as therapy targeting this distinct subtype of melanoma.15 With technological advancement in the areas of head and neck high-technique radiotherapy, optically guided intensity modulated radiotherapy11 or carbon ion radiotherapy12 has been used, but wide excision is the standard treatment for the primary tumor. However, the functional and esthetic disadvantages of excision should always be considered in this region. Although carbon ion radiotherapy12 was not used in this case, it has the advantages of favorable dose localization with increasing biological effect with depth. It is considered more effective against locally advanced or pathologically radioresistant tumors at an acceptable level of morbidity compared with other external beams, megavoltage photons, or electrons.7 In cases of melanoma in the nasal cavity or paranasal sinuses, 3-year local control was achieved in >80%, but almost half of the patients died from distant metastasis.12 However, careful consideration of the indication for radiotherapy is required because melanoma is relatively radiotherapy resistant.
1 Duguid IM. Malignant melanoma of the lacrimal sac. Br J Ophthalmol 1964; 48: 394–398. 2 Lloyd WC III, Leone CR Jr. Malignant melanoma of the lacrimal sac. Arch Ophthalmol 1984; 102: 104–107. 3 Pe’er JJ, Stefanyszyn M, Hidayat AA. Nonepithelial tumors of the lacrimal sac. Am J Ophthalmol 1994; 118: 650–658. 4 Kuwabara H, Takeda J. Malignant melanoma of the lacrimal sac with surrounding melanosis. Arch Pathol Lab Med 1997; 121: 517–519. 5 Nam JH, Kim SM, Choi JH, et al. Primary malignant melanoma of the lacrimal sac: a case report. Korean J Intern Med 2006; 21: 248–251. 6 Sitole S, Zender CA, Ahmad AZ, et al. Lacrimal sac melanoma. Ophthal Plast Reconstr Surg 2007; 23: 417–419. 7 Heindl LM, Schick B, Kämpgen E, et al. Malignant melanoma of the lacrimal sac. Ophthalmologe 2008; 105: 1146–1149. 8 Lee HM, Kang HJ, Choi G, et al. Two cases of primary malignant melanoma of the lacrimal sac. Head Neck 2001; 23: 809–813. 9 Sendra TJ, Galindo CN, Rodriguez-Peralto JL, et al. Malignant melanoma of the lacrimal sac. Otolaryngol Head Neck Surg 2004; 131: 334–336. 10 Gleizal A, Kodjikian L, Lebreton F, et al. Early CT-scan for chronic lacrimal duct symptoms - case report of a malignant melanoma of the lacrimal sac and review of the literature. J Craniomaxillofac Surg 2005; 33: 201–204. 11 Lu H, Yao M, Anderson K, Buatti J. Optically guided stereotactic radiotherapy for lacrimal sac tumors: a report on two cases. Technol Cancer Res Treat 2008; 7: 35–40. 12 Tsujii H, Mizoe JE, Kamada T, et al. Overview of clinical experiences on carbon ion radiotherapy at NIRS. Radiother Oncol 2004; 73: S41–S49. 13 Billing K, Malhotra R, Selva D, et al. Magnetic resonance imaging findings in malignant melanoma of the lacrimal sac. Br J Ophthalmol 2003; 87: 1187–1188. 14 Hodi FS, O’Day SJ, McDermott DF, et al. Improved survival with ipilimumab in patients with metastatic melanoma. N Engl J Med 2010; 363: 711–723. 15 Handolias D, Hamilton AL, Salemi R, et al. Clinical responses observed with imatinib or sorafenib in melanoma patients expressing mutations in KIT. Br J Cancer 2010; 102: 1219–1223.
ª 2013 The International Society of Dermatology
International Journal of Dermatology 2014, 53, 243–245
245
Malignant melanoma of the lacrimal sac: a case report Jiro Maegawa1, MD, Kazunori Yasumura1, MD, Toshinori Iwai2, DDS, Masaharu Hata3, MD, Yoshiaki Inayama4, MD, and Shinji Kobayashi5, MD
Departments of 1Plastic and Reconstructive Surgery, 2Maxillofacial Surgery, 3Radiology, and 4Diagnostic Pathology, Yokohama City University Hospital, Kanazawa-ku, and 5 Department of Plastic and Reconstructive Surgery, Kanagawa Children Medical Center, Yokohama, Japan Correspondence Jiro Maegawa, MD Department of Plastic and Reconstructive Surgery Yokohama City University Hospital 3-9 Fukuura, Kanazawa-ku Yokohama 236-0004 Japan E-mail: [email protected] Conflicts of interest: None.
Introduction Malignant melanoma of the lacrimal sac is rare.1–11 A case of lacrimal sac melanoma in a 61-year-old man is presented. Case report A 61-year-old patient had noticed a mass in the medial canthal region of the left palpebra for about one year. He visited an ophthalmologist because the mass gradually grew, and there were bloody clots from the left nasal cavity. The ophthalmologist referred the patient to our hospital suspecting malignancy. During the first visit, the patient complained of epiphora, a mass 34 · 23 mm in size, and slight pain in the medial canthal region (Fig. 1) without obvious metastases to regional lymph nodes and transit lesions in the canthal region and nasal mucosa. Computed tomography and magnetic resonance imaging (Fig. 2) revealed a clearly defined mass occupying the lacrimal sac and duct, expanding into the surrounding bone and soft tissue. A biopsy specimen revealed typical characteristics of malignant melanoma. ª 2013 The International Society of Dermatology
Carbon ion radiotherapy was then considered as the first choice of treatment for the head and neck region;12 this up-to-date radiotherapy was available at a nearby specialist institute. However, a positron emission tomography scan performed to identify distant metastases revealed multiple liver metastases, which were clinical stage IV in this patient. According to the protocol for
Figure 1 Closed frontal view of the patient. Black colored
mass in the left-side, medial canthal area (arrow) International Journal of Dermatology 2014, 53, 243–245
243
244
Case report
Malignant melanoma of the lacrimal sac
Maegawa et al.
Figure 2 T2-weighted coronal magnetic resonance imaging
demonstrating a hypointense mass lesion in the lacrimal sac and nasolacrimal duct
(a)
head and neck melanoma at the institute, which had a limited capacity for carbon ion radiotherapy, patients with melanoma but without metastasis had priority over those with metastasis for this treatment. Therefore, carbon ion radiotherapy was not indicated in this case. Two weeks after biopsy, the strategy for treating this head and neck melanoma was changed to include en bloc excision and flap reconstruction under general anesthesia to control local tumor invasion. Through a lateral rhinotomy, an en bloc excision, including the lacrimal apparatus, medial wall of the orbit, and medial upper and lower lids (Fig. 3a), and a medial maxillectomy were performed. A T-shaped, median forehead flap repaired the defect left by excision (Fig. 3b). Histopathological examination showed a 30 · 23 · 20 mm melanoma occupying the lacrimal sac and nasolacrimal duct with invasion of the maxilla. The resected specimen had a clear surgical margin. Hematoxylin and eosin staining revealed epithelioid cells in sheets containing a significant amount of melanin, and immunohistochemical analysis was positive for HMB45. The patient received three cycles of dacarbazine, nimustine, vincristine, and interferon-beta, but he subsequently developed other metastases in the gastric mucosa, lung, and brain 10 months after surgery. At the time of the patient’s death, there was no sign of local tumor recurrence.
(b)
Figure 3 Excised specimen including the lacrimal apparatus, medial wall of the orbit, and medial upper and lower lids and a
probe in the lacrimal tract (a). After excision of the tumor, the nasal cavity, the maxillary sinus, and the orbital contents on the left side are exposed (b). A T-shaped, median forehead flap repaired the defect left by excision
International Journal of Dermatology 2014, 53, 243–245
ª 2013 The International Society of Dermatology
Maegawa et al.
Malignant melanoma of the lacrimal sac
Case report
Discussion
References
Since Duguid’s paper,1 approximately 25–30 reports of lacrimal sac melanoma have been published.1–11,13 Bloody epiphora and a mass in the medial canthal region are the most common symptoms.1–9 Based on a review of available reports, the average age of patients with lacrimal melanoma was about 58 years, and 35% (six of 21) of patients developed metastasis more than six months after treatment.1–11,13 Magnetic resonance imaging has more recently been used to identify further characteristic features of melanoma of the lacrimal sac,13 but imaging studies do not confirm the diagnosis of melanoma; imaging shows the local extent of disease and is part of disease staging. Treatment of metastatic melanoma remains problematic. The efficacy of anti-CTLA4 (cytotoxic T-lymphocyte antigen-4) therapy with ipilimumab has been reported; the therapy improves overall survival in patients with advanced and metastatic melanoma,14 but it was not available at that time in Japan. Regarding genetic mutations with melanoma, it has been reported that mucosal and acral melanoma harbor more KIT-activating mutations than do other subtypes of melanoma, which suggests that KIT inhibitors may potentially be effective as therapy targeting this distinct subtype of melanoma.15 With technological advancement in the areas of head and neck high-technique radiotherapy, optically guided intensity modulated radiotherapy11 or carbon ion radiotherapy12 has been used, but wide excision is the standard treatment for the primary tumor. However, the functional and esthetic disadvantages of excision should always be considered in this region. Although carbon ion radiotherapy12 was not used in this case, it has the advantages of favorable dose localization with increasing biological effect with depth. It is considered more effective against locally advanced or pathologically radioresistant tumors at an acceptable level of morbidity compared with other external beams, megavoltage photons, or electrons.7 In cases of melanoma in the nasal cavity or paranasal sinuses, 3-year local control was achieved in >80%, but almost half of the patients died from distant metastasis.12 However, careful consideration of the indication for radiotherapy is required because melanoma is relatively radiotherapy resistant.
1 Duguid IM. Malignant melanoma of the lacrimal sac. Br J Ophthalmol 1964; 48: 394–398. 2 Lloyd WC III, Leone CR Jr. Malignant melanoma of the lacrimal sac. Arch Ophthalmol 1984; 102: 104–107. 3 Pe’er JJ, Stefanyszyn M, Hidayat AA. Nonepithelial tumors of the lacrimal sac. Am J Ophthalmol 1994; 118: 650–658. 4 Kuwabara H, Takeda J. Malignant melanoma of the lacrimal sac with surrounding melanosis. Arch Pathol Lab Med 1997; 121: 517–519. 5 Nam JH, Kim SM, Choi JH, et al. Primary malignant melanoma of the lacrimal sac: a case report. Korean J Intern Med 2006; 21: 248–251. 6 Sitole S, Zender CA, Ahmad AZ, et al. Lacrimal sac melanoma. Ophthal Plast Reconstr Surg 2007; 23: 417–419. 7 Heindl LM, Schick B, Kämpgen E, et al. Malignant melanoma of the lacrimal sac. Ophthalmologe 2008; 105: 1146–1149. 8 Lee HM, Kang HJ, Choi G, et al. Two cases of primary malignant melanoma of the lacrimal sac. Head Neck 2001; 23: 809–813. 9 Sendra TJ, Galindo CN, Rodriguez-Peralto JL, et al. Malignant melanoma of the lacrimal sac. Otolaryngol Head Neck Surg 2004; 131: 334–336. 10 Gleizal A, Kodjikian L, Lebreton F, et al. Early CT-scan for chronic lacrimal duct symptoms - case report of a malignant melanoma of the lacrimal sac and review of the literature. J Craniomaxillofac Surg 2005; 33: 201–204. 11 Lu H, Yao M, Anderson K, Buatti J. Optically guided stereotactic radiotherapy for lacrimal sac tumors: a report on two cases. Technol Cancer Res Treat 2008; 7: 35–40. 12 Tsujii H, Mizoe JE, Kamada T, et al. Overview of clinical experiences on carbon ion radiotherapy at NIRS. Radiother Oncol 2004; 73: S41–S49. 13 Billing K, Malhotra R, Selva D, et al. Magnetic resonance imaging findings in malignant melanoma of the lacrimal sac. Br J Ophthalmol 2003; 87: 1187–1188. 14 Hodi FS, O’Day SJ, McDermott DF, et al. Improved survival with ipilimumab in patients with metastatic melanoma. N Engl J Med 2010; 363: 711–723. 15 Handolias D, Hamilton AL, Salemi R, et al. Clinical responses observed with imatinib or sorafenib in melanoma patients expressing mutations in KIT. Br J Cancer 2010; 102: 1219–1223.
ª 2013 The International Society of Dermatology
International Journal of Dermatology 2014, 53, 243–245
245
