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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 727-730

Primary cutaneous angiosarcoma of the eyelid: Case report of a rare entity


1 Department of Ophthalmology, Armed Forces Medical College, Pune, Maharashtra, India
2 Department of Ophthalmology, Sanjay Gandhi Post Graduate Institute, Lucknow, India
3 Department of Pathology, Command Hospital, Pune, India

Date of Submission07-Feb-2021
Date of Acceptance14-May-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Rakesh Shetty
Department of Ophthalmology, Armed Forces Medical College, Wanowrie, Pune, Maharashtra - 411 040
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_176_21

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  Abstract 


A 67-year-old male, presented with the complaints of itchy, painless, rapidly enlarging black colored mass arising from his left upper eyelid. Ocular examination revealed a dark colored nodule with overlying crust on the temporal aspect of the left upper eyelid. An excisional biopsy revealed a well-circumscribed tumor composed of anastomosing vascular channels of varying sizes. Lining endothelial cells revealed focal areas of hypercellularity, mild to moderate pleiomorphism. Immunohistochemistry revealed that tumor cells are diffusely immunopositive for CD34. A diagnosis of primary cutaneous angiosarcoma of eyelid was made. Systemic oncological workup for metastatic disease was negative. The patient thereafter underwent wide surgical excision with frozen section control of margins followed by free flap skin graft under general anesthesia to achieve complete surgical resection of the tumor. Thus, although extremely rare, angiosarcoma of eyelid should be considered in the differential diagnosis of unusual or atypical eyelid lesions; the early diagnosis and treatment of which improves the survival rate.

Keywords: Cutaneous angiosarcoma, eyelid angiosarcoma, eyelid angiosarcoma in Indian patient, isolated eyelid angiosarcoma, nodular cutaneous angiosarcoma of eyelid


How to cite this article:
Singh A, Kaushik J, Shetty R, Jain VK, Kumar A, Mishra P. Primary cutaneous angiosarcoma of the eyelid: Case report of a rare entity. Indian J Ophthalmol Case Rep 2021;1:727-30

How to cite this URL:
Singh A, Kaushik J, Shetty R, Jain VK, Kumar A, Mishra P. Primary cutaneous angiosarcoma of the eyelid: Case report of a rare entity. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 21];1:727-30. Available from: https://www.ijoreports.in/text.asp?2021/1/4/727/327720



Angiosarcoma is a rare, aggressive, angiopoietic malignant tumor with a predilection for skin and superficial soft tissue.[1],[2] It constitutes one of the rarest of soft tissue neoplasms comprising less than 1% of all sarcomas. Primary ocular adnexal angiosarcomas are a further rarer entity and pose a clinical diagnostic challenge since mostly these are initially diagnosed as basal carcinoma or pyogenic granuloma.[3],[4],[5] Herein, we report a case of 67-year-old male who presented to us with a rapidly progressive eyelid lesion, which on clinical examination appeared to be a black colored, pedunculated, gyriform nodular mass and presumed to be keratoacanthoma or a hyperkeratotic nodular squamous cell carcinoma of the eyelid, but on histopathologic examination was revealed to be a low-grade angiosarcoma. Histologically, angiosarcoma consists of cells with anastomosing vascular channels with pleomorphic, mitotic neoplastic endothelial cells. Because of its aggressive nature with tendency to metastasize to liver, lung, and bone, early diagnosis and treatment is essential to provide best possible outcomes for the patient.


  Case Report Top


A 67 years old, otherwise healthy male, presented to the outpatient services of ophthalmology department of this tertiary care hospital with the complaints of itchy, painless, rapidly enlarging black colored mass arising from his left upper eyelid. He first noticed the mass 3 months back which was gradually increasing in size and extent. There was no history of similar complaint in the right eye or any significant history of ocular pathology or radiotherapy in the past. External ocular examination revealed a dark colored nodule with overlying crust on the temporal aspect of the left upper eyelid, measuring 2.5 cm × 1.5 cm [Figure 1]a and [Figure 1]b. The tarsal plate appeared to be spared, macroscopically. There was no palpable lymphadenopathy. The patient was pseudophakic in both eyes with the best-corrected visual acuity of 6/9 and 6/12 in the right and left eyes, respectively. Rest of the ocular examination was unremarkable. An excisional biopsy of the mass was performed and excised tissue was sent for histopathologic evaluation.
Figure 1: 1a and b Clinical presentation: dark colored nodular mass with overlying crust on the temporal aspect of the left upper eyelid

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Histopathological examination revealed a fairly circumscribed tumor composed of anastomosing vascular channels of varying sizes [Figure 2]a. Lining endothelial cells reveal focal area of hypercellularity, mild to moderate pleiomorphism, interspersed areas of hemorrhage and necrosis [Figure 2]b and [Figure 2]c, and mild increase in mitotic activity [Figure 2]d.
Figure 2: 2a, H and E, 40x: Fairly circumscribed tumor composed of anastomosing vascular channels of varying sizes. 2b, H and E, 100x: Anastomosing vascular channels lined by endothelial cells revealing focal areas of hypercellularity with moderate nuclear pleomorphism. 2c, H and E, 400x: Areas of hemorrhage. 2d, H and E, 400x: Mild increase in mitotic activity (Mitotic figures encircled)

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On immunohistochemistry, tumor cells were diffusely immunopositive for CD34 [Figure 3]a and [Figure 3]b while negative for PanCK (pancytokeratin, tumor stem cell marker for cancers from epithelial origin) and D2-40 (a monoclonal antibody used a lymphatic endothelial marker for tumors like Kaposi sarcoma, malignant mesothelioma). Mib-1 index was around 10-12% indicating a low grade tumor.
Figure 3: 3a, CD34, 100x: Tumor cells are diffusely positive for CD34. 3b, Mib-1 index, 100x: 10–12%

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A diagnosis of primary cutaneous angiosarcoma of eyelid was made based on immunohistochemistry and histology. Extensive systemic oncological workup for metastatic disease was carried out but was found to be negative. In view of the localized nature of the lesion, without evidence of regional or distant spread, the patient was treated with wide surgical excision with intraoperative frozen section control of margins, followed by free flap skin graft under general anesthesia to achieve complete surgical resection of the tumor. The survival of the graft flap was good postoperatively. After 1 year of follow-up, the patient was healthy and showed no evidence of recurrence or metastasis.


  Discussion Top


Angiosarcoma, first described by Livingston and Klemperer in 1926, is a rare malignant tumor of vascular endothelial cells origin, constituting <1% of all sarcomas.[1],[2],[6],[7] Cutaneous angiosarcoma has a predilection for face and scalp and rarely involves eyelid tissue; less than 30 cases have been reported till date.[1],[2],[3],[7],[8],[9] A systematic analysis of the data from Surveillance, Epidemiology and End Results (SEER) program by Albores-Saavedra et al.[10] revealed just 10 patients of cutaneous angiosarcoma involving the eyelid from 1973 to 2007.[11] Its incidence increases with age and is higher among Caucasians, with a greater preponderance for males than females.[1],[3],[12] Etiologically, angiosarcoma may be seen post radiation therapy or in association with chronic lymphoedema (Stewart-Treves syndrome) but can also arise sporadically, primarily affecting the head and neck region of elderly individuals. Clinically, it may present as a haematoma-like lesion, but can also resemble rosacea, eczema, haemangioma, violaceous plaques or nodules, xanthelasma and cellulitis.[1] Advanced lesions can mimic epithelial neoplasms including squamous cell carcinoma, keratoacanthoma, basal cell carcinoma, and malignant melanoma.[1],[2],[3],[5] Angiosarcoma is a rapidly progressing disease that can metastasize through the blood to the lungs, liver, bone. The SEER program data analysis also revealed that, when stratified by stage of disease, patients with localized cancer had relative survival rates of 62 and 54% at 5 and 10 years, whereas patients with distant cancer had a relative survival of just 6.2% at both 5 and 10 years.[10],[11] Papalas et al.[13] reported that patients with isolated, nodular eyelid involvement had a survival rate of 100% at a mean follow-up of 3 years, whereas patients with diffuse disease had a survival rate of 57% at a mean follow-up of 3 years.[3] It is, therefore, very crucial to make an early diagnosis, both by the ophthalmologist and pathologist, and impart prompt treatment to enable better survival rates. Important favorable prognostic factors associated with increased survival rates include tumors of small size, younger age (<50 years), localized tumors, and anatomical site (trunk). Histologically, morphology of cutaneous angiosarcoma is highly variable, ranging from well-differentiated lesions with irregular, dilated, anastomosing vascular structures with inconspicuous endothelial cells to poorly differentiated ones with ill-defined vascular structures consisting of sheets of pleomorphic, mitotically active, neoplastic endothelial cells. It is a great masquerader, as lesions with high-grade morphology and solid sheet-like epithelioid areas can mimic carcinomas and fibro-sarcomas while well differentiated, low-grade cutaneous angiosarcomas frequently mimic benign hemangiomas, reactive vascular lesions including papillary endothelial hyperplasia, exuberant granulation tissue (pyogenic granuloma), and intermediate vascular tumors like hemangioendotheliomas, presenting a diagnostic dilemma for the pathologist.[3],[5] Immunohistochemistry tumor markers of angiosarcoma include CD31, CD34, VIII factor-related antigen, Ulex europaeus agglutinin I and Vascular Endothelial Growth Factor; CD31 being the more specific and sensitive among them.[1] Paucity of randomized clinical trials for angiosarcoma means treatment protocols are guided based on guidelines of soft-tissue sarcoma. Modalities of the treatment for the cutaneous angiosarcoma includes surgical excision followed by radiotherapy (either external beam radiotherapy or plaque radiotherapy) if tumor is amenable to surgical resection or radiotherapy alone for nonresectable lesions. Although neoadjuvant chemotherapy and antiangiogenic molecules have also been used in the treatment of cutaneous angiosarcoma, mostly to obviate the need for major surgery and as eye-sparing option, they are associated with mixed results and higher rates of recurrence, as compared to surgery.[3],[6],[7]


  Conclusion Top


Cutaneous angiosarcoma is an aggressive tumor with a poor prognosis and a propensity for local recurrence and metastases. Isolated eyelid involvement is extremely rare but has better prognosis when compared with patients with secondary eyelid involvement and can be managed surgically along with either neoadjuvant chemotherapy or radiation. If other regions of the face are involved in addition to the eyelid, the prognosis is dismal. Thus, although extremely rare, angiosarcoma should be considered in the differential diagnosis of unusual or atypical eyelid lesions, as early diagnosis and treatment of this deadly masquerader improves the survival rate.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Shustef E, Kazlouskaya V, Prieto VG, Ivan D, Aung PP. Cutaneous angiosarcoma : A current update. J Clin Pathol 2017;70:917-25.  Back to cited text no. 1
    
2.
Bray LC, Sullivan TJ, Whitehead K. Angiosarcoma of the eyelid. Aust Newzeal J Ophthalmol 1995;23:69-72.  Back to cited text no. 2
    
3.
Demirci H, Christanson MD. Eyelid angiosarcoma : A case report and review of the literature. Middle East Afr J Ophthalmol 2013;20:259-62.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Lemanski N, Farber M, Carruth BP, Wladis EJ. Primary adnexal angiosarcoma masquerading asperiorbital hematoma. Surv Ophthalmol 2014;59:655-9.  Back to cited text no. 4
    
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Milman T, Shields JA, Brooks J, Eagle RC. Primary cutaneous angiosarcoma of the eyelid : A diagnostic and therapeutic challenge. Ocul Oncol Pathol 2018;19107:230-5.  Back to cited text no. 5
    
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Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ. Angiosarcoma. Lancet Oncol 2010;11:983-91.  Back to cited text no. 6
    
7.
DeMartelaere SL, Roberts D, Burgess MA, Morrison WH, Pisters PW, Sturgis EM, et al. Neoadjuvant chemotherapy-specific and overall treatment outcomes in patients with cutaneous angiosarcoma of the face with periorbital involvement. Head Neck. 2008;30:639-46. doi: 10.1002/hed.20757. PMID: 18213722.  Back to cited text no. 7
    
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Gündüz K, Shields JA, Shields CL, Eagle RC, Nathan F. Cutaneous angiosarcoma with eyelid involvement. Am J Ophthalmol 1998;125:870-1.  Back to cited text no. 8
    
9.
Huang X, Sun S. Primary eyelid angiosarcoma in a Chinese patient. Int J Clin Exp Pathol 2015;8:8636-8.  Back to cited text no. 9
    
10.
Albores-Saavedra J, Schwartz AM, Henson DE, Kostun L, Hart A, Angeles-Albores D, et al. Cutaneous angiosarcoma. Analysis of 434 cases from the surveillance, epidemiology, and end results program, 1973-2007. Ann Diagn Pathol 2011;15:93-7.  Back to cited text no. 10
    
11.
Surveillance Epidemiology and End Results. Overview of the SEER Program. (n.d.) http://seer.cancer.gov/about/.  Back to cited text no. 11
    
12.
Rouhani 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. doi: 10.1002/cncr.23571. PMID: 18618615.  Back to cited text no. 12
    
13.
Papalas JA, Manavi CK, Woodward JA. Angiosarcoma of the eyelid : A clinicopathologic comparison between isolated unilateral tumors and tumors demonstrating extrapalpebral involvement. Am J Dermatopathol 2010;32:694-9.  Back to cited text no. 13
    


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