Int J Clin Exp Pathol 2015;8(7):8636-8638 www.ijcep.com /ISSN:1936-2625/IJCEP0008315
Case Report Primary eyelid angiosarcoma in a Chinese patient Xiaoli Huang, Song Sun Department of Ophthalmology Wuxi No. 2 Hospital Affiliated to Nanjing Medical University, 68 Zhongshan Rd, Wuxi 214002, P. R. China Received March 22, 2015; Accepted May 22, 2015; Epub July 1, 2015; Published July 15, 2015 Abstract: Purpose: To describe a case of primary eyelid angiosarcoma and review the literature to emphasize aware of this rare disease. Case Report: We report a further case which is the first Chinese primary eyelid angiosarcoma in the literature. A 76-year-old woman presented with a 6-month history of a painless lesion on her left eyelid. The patient finally proved to be angiosarcoma on histopathologic and be treated with complete surgical excision with a frozen section margin control. With a 6 months follow-up, we haven’t found any symptoms of recurrence or metastasis. Conclusions: Cutaneous angiosarcoma with eyelid is a rare, soft-tissue sarcoma of endothelial cell origin that is aggressive malignancy and has a poor prognosis. In our case report, the patient was treated with complete surgical excision with a frozen section margin control, and a 6 months follow-up, we haven’t found any symptoms of recurrence or metastasis. Keywords: Eyelid, eye, angiosarcoma, tumor, surgical resection
Introduction Cutaneous angiosarcoma (AS) with eyelid is a rare, soft-tissue sarcoma of endothelial cell origin that be aggressive malignancy and has a poor prognosis. It’s first described by Livingston and Klemperer in 1926 [1]. Soft tissue sarcomas account for less than 1% of all cancers, and angiosarcoma account for 2% of them, and cutaneous angiosarcomas mostly occur at head and neck are as account for 59%, rarely involve periocular tissue and affect men twice as often as women [2], and results in a terrible 5-year survival rate of 12%~33% [3]. Case report A 76-year-old woman was referred to our department for a 6-month history of a lesion on her left face. Specialist examination showed an erythematous lesion involving the upper eyelid, inner canthus, lower eyelid, and bridge of the nose. The lesion was approximately 2 cm × 2 cm, without ulceration and without clear boundaries (Figure 1A). The patient had no history of skin cancer. The patient’s family history and past medical history were not significant. Laboratory examination including blood chemistry and blood count could not reveal any specific pathology.
An excisional skin biopsy from the center of the lesion was performed. Ocular examination showed serosanguinous drainage from the biopsy site. Histopathological examination demonstrated a cytologically atypical vascular neoplasm composed of spindle-shaped and epithelioid cells (Figure 2A). The tumour cells were intensely immunopositive for CD31 (Figure 2B) and CD34 (Figure 2C). A diagnosis of eyelid angiosarcoma (AS) was made. The patient underwent complete surgical excision and free flap skin graft under general anaesthesia to achieve complete surgical resection of the tumour. Twelve frozen sections were examined and found to have negative intraoperative margins. The survival of the graft flap was good even after 1 month (Figure 1B). After 12 months of follow-up, there was no evidence of recurrence or metastasis. Discussion Cutaneous angiosarcomais a rare vascular endothelial cells malignant tumour. Panta Rouhani et al [2] reviewed 12, 114 cases of skin and soft tissue sarcoma, and angiosarcoma was found to account for 1.6%. Angiosarcoma has a higher incidence among Caucasians, a male: female incidence ratio of 2.0, and rarely involves the eyelid tissue; in fact,
Primary eyelid angiosarcoma
Figure 1. A: Patient with an isolated unilateral angiosarcoma of left eyelid nasally; B: One month after the surgery.
Figure 2. A: Histopathological examination showed dermal tumor tissue flake, crack-like distribution, some fissures see obesity tumor cells, plasma rich red dye, Cutaneous (hematoxylin and eosin, × 200); B: Immunoreactivity was
8637
Int J Clin Exp Pathol 2015;8(7):8636-8638
Primary eyelid angiosarcoma intense for CD31 (original magnification × 100); C: Immunoreactivity was intense for CD34 (original magnification × 200).
the eyelids are involved in only 3% of cases. Most patients were elderly women approximately 70-years-old. Researchers [4] believe that a history of exposure to chemicals, ionizing radiation, chronic lymphedema, sunlight, and trauma, and the presence of other relevant mutations are related to the development of angiosarcoma. Angiosarcomas are rapidly progressing diseases that can spread not only to nearby lymph nodes, but also metastasize through the blood to the lungs, liver, bone, and other locations. Therefore, it is important to make an early diagnosis and to institute the correct treatment. The diagnosis of angiosarcoma is based on histopathological evaluation. Histologically, angiosarcoma is characterized by abnormal and pleomorphic malignant endothelial cells [5]. Neoplastic cells range from spindle to epithelioid cells with large irregular nuclei, coarse chromatin, and eosinophilic cytoplasm. In addition, angiosarcoma is associated with positive immunohistochemical markers, including CD31, CD34, and VIII factor related antigen. CD31 is one of the most specific and sensitive markers [6]. Our case was considered a well-differentiated angiosarcoma with the expression of immunohistochemical markers of vascular differentiation (CD31 and CD34). The mainstay treatment for cutaneous angiosarcoma includes surgical excision followed by external beam radiotherapy. Although recent case reports have described approaches for the management and about the outcome of angiosarcoma [6], specific management criteria are absent owing to the lack of large randomized controlled trials of patients with angiosarcoma. The prognosis of cutaneous angiosarcoma is very poor, with a 5-year survival rate of only 12-33% [7]. The most important factors for improving prognosis are small tumour size, younger age, isolated tumors, negative resection margin, and postoperative radiation therapy [7]. Our case is important for a number of reasons. First, angiosarcoma of the eyelids is rare. We
8638
describe the first case of primary eyelid angiosarcoma in a Chinese patient. In addition, successful treatment of this case can provide some suggestions for treatment. Finally, clinicians should be alert that angiosarcoma might present with a wide variety of clinical features. Disclosure of conflict of interest None. Address correspondence to: Dr. Song Sun, Department of Ophthalmology Wuxi No. 2 Hospital Affiliated to Nanjing Medical University, 68 Zhongshan Road, Wuxi 214002, P. R. China. Tel: 86 510 66681222; Fax: 86 510 82754933; E-mail: songsun2000@163. com
References [1] [2]
[3]
[4]
[5]
[6]
[7]
Panizzon R, Schneider BV, Schnyder UW. Rosacea-like angiosarcoma of the face. Dermatologica 1990; 181: 252-54. DRouhani P, Fletcher CD, Devesa SS, Toro JR. Cutaneous soft tissue sarcoma incidence patterns in the U.S.: an analysis of 12,114 cases. Cancer 2008; 113: 616-27. DeMartelaere SL, Roberts D, Burgess MA, Morrison WH, Pisters PW, Sturgis EM, Ho V, Esmaeli B. Neoadjuvant chemotherapy-specific and overall treatment outcomes in patients with cutaneous angiosarcoma of the face with periorbital involvement. Head Neck 2008; 30: 639-46. Hassane S, Fouad E, Said I, Karim R, Asmae N, Houcine C, Abdelbar O. Orbital metastatic angiosarcoma. Korean J Ophthalmol 2010; 24: 364-66. Papalas JA, Manavi CK, Woodward JA, Sangueza OP, Cummings TJ. Angiosarcoma of the eyelid: a clinicdopathologic comparison between isolated unilateral tumors and tumors demonstrating extrapalpebral involvement. Am J Dermatopathol 2010; 32: 694-99. Demirci H, Christanson MD. Eyelid angiosarcoma: a case report and review of the literature. Middle East Afr J Ophthalmol 2013; 20: 25962 Ettl T, Kleinheinz J, Mehrotra R, Schwarz S, Reichert TE, Driemel O. Infraorbital cutaneous angiosarcoma: a diagnostic and therapeutic dilemma. Head Face Med 2008; 4: 18.
Int J Clin Exp Pathol 2015;8(7):8636-8638
Case Report Primary eyelid angiosarcoma in a Chinese patient Xiaoli Huang, Song Sun Department of Ophthalmology Wuxi No. 2 Hospital Affiliated to Nanjing Medical University, 68 Zhongshan Rd, Wuxi 214002, P. R. China Received March 22, 2015; Accepted May 22, 2015; Epub July 1, 2015; Published July 15, 2015 Abstract: Purpose: To describe a case of primary eyelid angiosarcoma and review the literature to emphasize aware of this rare disease. Case Report: We report a further case which is the first Chinese primary eyelid angiosarcoma in the literature. A 76-year-old woman presented with a 6-month history of a painless lesion on her left eyelid. The patient finally proved to be angiosarcoma on histopathologic and be treated with complete surgical excision with a frozen section margin control. With a 6 months follow-up, we haven’t found any symptoms of recurrence or metastasis. Conclusions: Cutaneous angiosarcoma with eyelid is a rare, soft-tissue sarcoma of endothelial cell origin that is aggressive malignancy and has a poor prognosis. In our case report, the patient was treated with complete surgical excision with a frozen section margin control, and a 6 months follow-up, we haven’t found any symptoms of recurrence or metastasis. Keywords: Eyelid, eye, angiosarcoma, tumor, surgical resection
Introduction Cutaneous angiosarcoma (AS) with eyelid is a rare, soft-tissue sarcoma of endothelial cell origin that be aggressive malignancy and has a poor prognosis. It’s first described by Livingston and Klemperer in 1926 [1]. Soft tissue sarcomas account for less than 1% of all cancers, and angiosarcoma account for 2% of them, and cutaneous angiosarcomas mostly occur at head and neck are as account for 59%, rarely involve periocular tissue and affect men twice as often as women [2], and results in a terrible 5-year survival rate of 12%~33% [3]. Case report A 76-year-old woman was referred to our department for a 6-month history of a lesion on her left face. Specialist examination showed an erythematous lesion involving the upper eyelid, inner canthus, lower eyelid, and bridge of the nose. The lesion was approximately 2 cm × 2 cm, without ulceration and without clear boundaries (Figure 1A). The patient had no history of skin cancer. The patient’s family history and past medical history were not significant. Laboratory examination including blood chemistry and blood count could not reveal any specific pathology.
An excisional skin biopsy from the center of the lesion was performed. Ocular examination showed serosanguinous drainage from the biopsy site. Histopathological examination demonstrated a cytologically atypical vascular neoplasm composed of spindle-shaped and epithelioid cells (Figure 2A). The tumour cells were intensely immunopositive for CD31 (Figure 2B) and CD34 (Figure 2C). A diagnosis of eyelid angiosarcoma (AS) was made. The patient underwent complete surgical excision and free flap skin graft under general anaesthesia to achieve complete surgical resection of the tumour. Twelve frozen sections were examined and found to have negative intraoperative margins. The survival of the graft flap was good even after 1 month (Figure 1B). After 12 months of follow-up, there was no evidence of recurrence or metastasis. Discussion Cutaneous angiosarcomais a rare vascular endothelial cells malignant tumour. Panta Rouhani et al [2] reviewed 12, 114 cases of skin and soft tissue sarcoma, and angiosarcoma was found to account for 1.6%. Angiosarcoma has a higher incidence among Caucasians, a male: female incidence ratio of 2.0, and rarely involves the eyelid tissue; in fact,
Primary eyelid angiosarcoma
Figure 1. A: Patient with an isolated unilateral angiosarcoma of left eyelid nasally; B: One month after the surgery.
Figure 2. A: Histopathological examination showed dermal tumor tissue flake, crack-like distribution, some fissures see obesity tumor cells, plasma rich red dye, Cutaneous (hematoxylin and eosin, × 200); B: Immunoreactivity was
8637
Int J Clin Exp Pathol 2015;8(7):8636-8638
Primary eyelid angiosarcoma intense for CD31 (original magnification × 100); C: Immunoreactivity was intense for CD34 (original magnification × 200).
the eyelids are involved in only 3% of cases. Most patients were elderly women approximately 70-years-old. Researchers [4] believe that a history of exposure to chemicals, ionizing radiation, chronic lymphedema, sunlight, and trauma, and the presence of other relevant mutations are related to the development of angiosarcoma. Angiosarcomas are rapidly progressing diseases that can spread not only to nearby lymph nodes, but also metastasize through the blood to the lungs, liver, bone, and other locations. Therefore, it is important to make an early diagnosis and to institute the correct treatment. The diagnosis of angiosarcoma is based on histopathological evaluation. Histologically, angiosarcoma is characterized by abnormal and pleomorphic malignant endothelial cells [5]. Neoplastic cells range from spindle to epithelioid cells with large irregular nuclei, coarse chromatin, and eosinophilic cytoplasm. In addition, angiosarcoma is associated with positive immunohistochemical markers, including CD31, CD34, and VIII factor related antigen. CD31 is one of the most specific and sensitive markers [6]. Our case was considered a well-differentiated angiosarcoma with the expression of immunohistochemical markers of vascular differentiation (CD31 and CD34). The mainstay treatment for cutaneous angiosarcoma includes surgical excision followed by external beam radiotherapy. Although recent case reports have described approaches for the management and about the outcome of angiosarcoma [6], specific management criteria are absent owing to the lack of large randomized controlled trials of patients with angiosarcoma. The prognosis of cutaneous angiosarcoma is very poor, with a 5-year survival rate of only 12-33% [7]. The most important factors for improving prognosis are small tumour size, younger age, isolated tumors, negative resection margin, and postoperative radiation therapy [7]. Our case is important for a number of reasons. First, angiosarcoma of the eyelids is rare. We
8638
describe the first case of primary eyelid angiosarcoma in a Chinese patient. In addition, successful treatment of this case can provide some suggestions for treatment. Finally, clinicians should be alert that angiosarcoma might present with a wide variety of clinical features. Disclosure of conflict of interest None. Address correspondence to: Dr. Song Sun, Department of Ophthalmology Wuxi No. 2 Hospital Affiliated to Nanjing Medical University, 68 Zhongshan Road, Wuxi 214002, P. R. China. Tel: 86 510 66681222; Fax: 86 510 82754933; E-mail: songsun2000@163. com
References [1] [2]
[3]
[4]
[5]
[6]
[7]
Panizzon R, Schneider BV, Schnyder UW. Rosacea-like angiosarcoma of the face. Dermatologica 1990; 181: 252-54. DRouhani P, Fletcher CD, Devesa SS, Toro JR. Cutaneous soft tissue sarcoma incidence patterns in the U.S.: an analysis of 12,114 cases. Cancer 2008; 113: 616-27. DeMartelaere SL, Roberts D, Burgess MA, Morrison WH, Pisters PW, Sturgis EM, Ho V, Esmaeli B. Neoadjuvant chemotherapy-specific and overall treatment outcomes in patients with cutaneous angiosarcoma of the face with periorbital involvement. Head Neck 2008; 30: 639-46. Hassane S, Fouad E, Said I, Karim R, Asmae N, Houcine C, Abdelbar O. Orbital metastatic angiosarcoma. Korean J Ophthalmol 2010; 24: 364-66. Papalas JA, Manavi CK, Woodward JA, Sangueza OP, Cummings TJ. Angiosarcoma of the eyelid: a clinicdopathologic comparison between isolated unilateral tumors and tumors demonstrating extrapalpebral involvement. Am J Dermatopathol 2010; 32: 694-99. Demirci H, Christanson MD. Eyelid angiosarcoma: a case report and review of the literature. Middle East Afr J Ophthalmol 2013; 20: 25962 Ettl T, Kleinheinz J, Mehrotra R, Schwarz S, Reichert TE, Driemel O. Infraorbital cutaneous angiosarcoma: a diagnostic and therapeutic dilemma. Head Face Med 2008; 4: 18.
Int J Clin Exp Pathol 2015;8(7):8636-8638
