Purpose
To provide a critical analysis of a series of periocular lobular capillary hemangiomas in adults, outlining characteristic clinical and histopathologic patterns in comparison with those of other vascular tumors of adults and children.
Design
Retrospective observational case series.
Methods
Review of clinical data, hematoxylin-eosin–stained sections, and immunohistochemical studies of smooth muscle actin (SMA), D2-40, CD34, and glucose transporter 1 (GLUT-1).
Results
The 7 female and 4 male patients were diagnosed with periocular lobular capillary hemangioma at a median age of 39 years (range, 17–82 years). The tumors were small (3–14 mm, median size 6 mm) and well circumscribed, arose over the course of weeks to months, and developed most commonly in the canthal region, followed by the upper eyelid skin. The tumors were all composed microscopically of repeating units of various sizes (lobules) consisting of CD34-postive, GLUT-1-negative endothelial cells and SMA-positive pericytes arranged in macro- or microlobules. Some foci also exhibited ectatic vessels or diffuse, nonlobular capillary proliferations. Excision was curative without recurrence.
Conclusion
Although capillary hemangiomas are more common in children, lobular capillary hemangiomas can also arise in the periocular region of adults. Some histopathologic features of these lesions are shared with those of infantile hemangioma and tufted angioma of children, but features of the clinical presentation and the results of immunohistochemical staining patterns are distinctive.
Most periocular capillary vascular tumors develop in infancy or early childhood and are not well characterized in adults. The various types of capillary proliferations that occur in adults can have overlapping cellular features with each other and some childhood tumors. Clinicopathologic correlations are essential for making the correct diagnosis.
Ophthalmologists are most familiar with the term “capillary hemangioma” in the context of an early infantile, red, periocular mass that can cause deprivational, anisometropic, or strabismic amblyopia. These lesions appear within the first weeks of life; they grow rapidly and then spontaneously regress in early childhood. In the eyelid, they are treated before advanced regression occurs to forestall the amblyopic concerns. In adults, cavernous hemangiomas are the most common benign orbital tumor. It has been argued that these lesions are probably venous malformations rather than true neoplasms and display no tendency for spontaneous involution.
Lobular capillary hemangioma was first recognized as an acquired periocular lesion of adults in 1996 but has since rarely been reported in the eyelids. Pyogenic granuloma has been subsumed into the category of lobular capillary hemangioma, although there are distinctive clinical and histomorphologic features that make retaining this separate diagnosis in ophthalmology useful. An angiomatous proliferation, tufted angioma, somewhat reminiscent histopathologically of lobular capillary hemangioma, has a unique clinical presentation and has recently been described in the eyelids of 2 adults and once in the orbit. It is unclear, however, whether these particular lesions truly represent the clinicopathologic entity of tufted angioma with its distinctive clinical appearance and behavior. In this report, we review the classification of a spectrum of vascular tumors of the ocular adnexa occurring across all age groups and provide a critical analysis of 11 acquired periocular lobular capillary vascular lesions in adults. We highlight the distinguishing clinical, histopathologic, and immunohistochemical characteristics of childhood and adult lesions.
Methods
This retrospective, observational case series was conducted under the auspices of the Massachusetts Eye and Ear Infirmary’s Institutional Review Board, in compliance with the rules and regulations of the Health Insurance Portability and Accountability Act and all applicable federal and state laws, and in adherence to the tenets of the Declaration of Helsinki.
Cases on file in the ophthalmic pathology laboratory at our institution from 2010 to 2015 were reviewed; 11 cases were identified that were considered acceptable for this study. Patients were aged 18 years and older at the time of the search. Cases selected for inclusion were clinically described as being located in the eyelids or “anterior orbit” and initially bore one of the following diagnoses: lobular capillary hemangioma, adult-type capillary hemangioma, tufted hemangioma, or tufted angioma. Lesions diagnosed as pyogenic granuloma, typically of the conjunctiva, were excluded. Patients’ medical and surgical records and clinical photographs or radiographic studies were examined if available. Histopathologic features displayed in formalin-fixed, paraffin-embedded, and hematoxylin-eosin–stained sections on glass slides were critically reexamined and the diagnoses were revised when necessary. Paraffin blocks were available for the preparation of additional sections for supplemental immunohistochemical staining. Biomarkers used included smooth muscle actin (SMA) for pericytes, glucose transporter 1 (GLUT-1) for pluripotent vascular endothelial cells, D2-40 for lymphatic endothelial cells, and CD34 for stable vascular endothelial cells.
Results
Clinical Findings
The general features of the 11 patients in this series are summarized in Table 1 . Four were male and 7 were female, with an average age of 42.4 years (range, 17–82 years; median, 39). The excised growths measured an average size of 7 mm in maximum dimension (range, 3–14 mm; median, 6 mm). Two lesions had some overlapping histopathologic features with tufted angioma and were initially given this diagnosis; the rest were diagnosed originally as “adult-type capillary hemangioma” or “capillary hemangioma.” Four of 11 lesions developed in the upper eyelid skin ( Figure 1 , Upper left), 5 in the deeper tissues of the medial or lateral canthi ( Figure 1 , Upper right, Bottom left, and Bottom right), and 2 originated from the tarsal conjunctival surface ( Figure 1 , Middle right) of the lower eyelid, 1 being at the margin ( Figure 1 , Middle left). Preoperatively, the deeper lesions were often diagnosed as “cysts” or otherwise as basal cell carcinoma, chalazion, pyogenic granuloma, and unspecified vascular malformation. No patient had a history of systemic vascular disease, trauma, or pain. Eighty percent of the tumors (8 of 10 lesions where the duration was known) developed within months, although 2 had been present for a year or more. No recurrences were noted after excision.
| Case | Preoperative Diagnosis | Patient Age/Sex | Location | Duration | Length of Follow-up |
|---|---|---|---|---|---|
| 1 | Cyst | 28/F | Right lateral canthus | 2 months | 4 years |
| 2 | Basal cell carcinoma or cyst | 45/F | Left lateral third of upper eyelid | 3 months | 4 years |
| 3 | Benign vascular lesion | 61/F | Left central third of upper eyelid | 6 weeks | 4 years |
| 4 | Chalazion | 39/F | Left lateral third of lower eyelid at margin | 6 weeks | 3.5 years |
| 5 | Pyogenic granuloma | 82/F | Left medial third of lower tarsal conjunctiva | 1 month | 2.5 years |
| 6 | Varix | 20/M | Unspecified side, deep medial third of upper eyelid | 6 months | 2 years |
| 7 | Cyst | 56/M | Left lateral canthus | 1 year | 2 years |
| 8 | Not specified | 27/F | Right medial canthus | Unknown | 1 year |
| 9 | Not specified | 17/M | Left upper eyelid | 4 months | 1 year |
| 10 | Vascular malformation (based on CT) | 34/F | Left medial canthus | 5 years | 9 months |
| 11 | Dermoid cyst | 57/M | Right medial canthus | 3 months | 3 months |
Histopathologic and Immunohistochemical Findings
All lesions were composed microscopically of various-sized lobules of plump or flattened endothelial cells with surrounding pericytes. At low power, several patterns were observed. Four tumors were composed of large lobules (macrolobular pattern) of superficial capillaries separated by fibrous bands immediately beneath the epithelium ( Figure 2 , Upper left and Upper right). Other examples were composed of smaller lobules (microlobular pattern) compressed by dense tarsal ( Figure 2 , Middle left) or deeper dermal ( Figure 2 , Bottom left) collagen, which created nearly bloodless capillary lumens and broader interlobular septa ( Figure 2 , Bottom right). The intratarsal tumor displaced a meibomian gland, a gland of Moll ( Figure 2 , Middle right), and a Zeiss gland at the eyelid margin. The deeper dermal and subcutaneous tumors had overlapping histopathologic features with tufted angioma with circumscribed “cannonball-like” foci of dermal capillaries ( Figure 2 , Bottom right). At high power, plump endothelial cells enclosing compressed lumens were seen ( Figure 3 , Upper left). Some tumors were composed of smaller microlobules with myxoid stromal foci ( Figure 3 , Upper right), while others displayed more diffuse, unorganized capillary proliferations ( Figure 3 , Middle right). In 4 lesions, ectatic vascular spaces within the lobules were reminiscent of the maturation phase of infantile hemangioma ( Figure 3 , Middle left). One lobular capillary hemangioma was superficially ulcerated ( Figure 3 , Bottom left). Dilated crescentic vessels were observed at the periphery of some of the capillary lobules ( Figure 3 , Bottom right). In 3 cases, an intravascular protrusion of the capillary proliferation was detected within the lobules ( Figure 4 , Upper left and Upper right).
Immunohistochemistry for CD34 was diffusely positive in the endothelial cells of all tumors, highlighting the overall patterns of large ( Figure 4 , Middle left) and small ( Figure 4 , Middle right, left panel) lobules. All tumors were GLUT-1 negative with respect to the endothelial cells. SMA highlighted an equally prominent population of bland pericytes ( Figure 4 , Middle right, right panel). D2-40 was negative in all tumors, including in crescentic vessels at the edge of the lobules ( Figure 4 , Bottom left). On the other hand, scattered lymphatic channels in the nearby dermis and the intervening fibrous septae stained positive for D2-40 ( Figure 4 , Bottom left and Bottom right).
Results
Clinical Findings
The general features of the 11 patients in this series are summarized in Table 1 . Four were male and 7 were female, with an average age of 42.4 years (range, 17–82 years; median, 39). The excised growths measured an average size of 7 mm in maximum dimension (range, 3–14 mm; median, 6 mm). Two lesions had some overlapping histopathologic features with tufted angioma and were initially given this diagnosis; the rest were diagnosed originally as “adult-type capillary hemangioma” or “capillary hemangioma.” Four of 11 lesions developed in the upper eyelid skin ( Figure 1 , Upper left), 5 in the deeper tissues of the medial or lateral canthi ( Figure 1 , Upper right, Bottom left, and Bottom right), and 2 originated from the tarsal conjunctival surface ( Figure 1 , Middle right) of the lower eyelid, 1 being at the margin ( Figure 1 , Middle left). Preoperatively, the deeper lesions were often diagnosed as “cysts” or otherwise as basal cell carcinoma, chalazion, pyogenic granuloma, and unspecified vascular malformation. No patient had a history of systemic vascular disease, trauma, or pain. Eighty percent of the tumors (8 of 10 lesions where the duration was known) developed within months, although 2 had been present for a year or more. No recurrences were noted after excision.
| Case | Preoperative Diagnosis | Patient Age/Sex | Location | Duration | Length of Follow-up |
|---|---|---|---|---|---|
| 1 | Cyst | 28/F | Right lateral canthus | 2 months | 4 years |
| 2 | Basal cell carcinoma or cyst | 45/F | Left lateral third of upper eyelid | 3 months | 4 years |
| 3 | Benign vascular lesion | 61/F | Left central third of upper eyelid | 6 weeks | 4 years |
| 4 | Chalazion | 39/F | Left lateral third of lower eyelid at margin | 6 weeks | 3.5 years |
| 5 | Pyogenic granuloma | 82/F | Left medial third of lower tarsal conjunctiva | 1 month | 2.5 years |
| 6 | Varix | 20/M | Unspecified side, deep medial third of upper eyelid | 6 months | 2 years |
| 7 | Cyst | 56/M | Left lateral canthus | 1 year | 2 years |
| 8 | Not specified | 27/F | Right medial canthus | Unknown | 1 year |
| 9 | Not specified | 17/M | Left upper eyelid | 4 months | 1 year |
| 10 | Vascular malformation (based on CT) | 34/F | Left medial canthus | 5 years | 9 months |
| 11 | Dermoid cyst | 57/M | Right medial canthus | 3 months | 3 months |
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