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| Year : 2013 | Volume
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| Issue : 4 | Page : 405-407 |
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| Angiolymphoid hyperplasia with eosinophilia: A rare benign vascular tumor of breast |
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Neelima Tirumalasetti
Department of Pathology, Katuri Medical College, Guntur, Andhra Pradesh, India
Click here for correspondence address and email
| Date of Web Publication | 18-Jan-2014 |
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 Abstract | | |
Angiolymphoid hyperplasia with eosinophilia typically involves head and neck with rare involvement of other sites like breast. A 27year old female presented with a painless swelling in the upper and outer quadrant of left breast. Fine needle aspiration cytology showed sheets of lymphoid cells along with numerous eosinophils. Subsequent histopathological examination showed eosinophilic plump and vacuolated endothelial cells in small groups and sheets within a fibrous background, typically accompanied by lymphoid aggregates and eosinophils, suggestive of angiolymphoid hyperplasia with eosinophilia. Vascular tumors of the breast are extremely rare. The goal of this case report is to advert to this unusual clinical picture, to review and discuss diagnostics, differential diagnoses and treatment options. Keywords: Angiolymphoid hyperplasia, breast, eosinophilia
How to cite this article:
Tirumalasetti N. Angiolymphoid hyperplasia with eosinophilia: A rare benign vascular tumor of breast. Indian J Pathol Microbiol 2013;56:405-7 |
| Introduction | |  |
Angiolymphoid hyperplasia with eosinophilia, also known as epithelioid hemangioma, most commonly occur in the subcutaneous tissue of the head and neck but may also be rarely seen in the breast. [1],[2] This entity occurs predominantly in patients in the third to fifth decade. [2]
| Case Report | | |
A 27-year-old female presented with a painless swelling in the upper and outer quadrant of left breast. On examination, there was a lump measuring 2 × 1.5 cm. The mass was firm, mobile, and non-tender. General examination was otherwise unremarkable; in particular, there were neither skin lesions nor palpable lymphadenopathy.
Ultrasonography of the breast revealed a mass in the left breast measuring 2 × 1.5 × 1 cm. Radiological differential diagnosis was either a fibroadenoma or an intramammary lymph node. Fine needle aspiration cytology showed sheets of lymphoid cells along with numerous eosinophils [Figure 1].
The mass was excised and sent for histopathological examination. | Figure 1: FNAC smear showing sheets of lymphoid cells along with eosinophils (H&E,400x)
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Histopathology showed eosinophilic plump and vacuolated endothelial cells in small groups and sheets within a fibrous background, typically accompanied by lymphoid aggregates and eosinophils [Figure 2].
The patient did not reveal any peripheral blood eosinophilia.
| Discussion | | |
Angiolymphoid hyperplasia with eosinophilia (ALHE), also known as epithelioid hemangioma, is a uncommon benign vaso-proliferative disease. It presents most commonly in patients aged 20-50 years, with a mean onset of 30-33 years. [3] ALHE affects women more often than men. [4] The condition is more commonly encountered in the Asian population followed by Caucasians. It is rare in the black population. [4]
ALHE lesions are most commonly seen in the peri-auricular area, forehead, and scalp. Rare sites of involvement include the orbit, oral mucosa, shoulders, hands, breasts, liver, spleen, heart, bone, and penis. [3],[5] The majority of patients present with a mass of a year or less in duration. [6]
Systemic eosinophilia of 6-34% is seen in 20% of patients with ALHE. [5] Serum hypereosinophilia is inconstant and is not required to make the diagnosis of ALHE. [7]
Despite the benign nature of ALHE, considerable controversy still exists as to the basic nature of these lesions. Some authors consider ALHE as a neoplasm developing from endothelial cells; others suggest that it is secondary to an inflammatory vascular reaction secondary to complex immunologic mechanisms. Many other hypotheses have been reported implicating environmental factors such as insect bite, trauma, and infections. Some authors consider that arterio-venous shunt is the main etiopathogenetic mechanism. The predominance of T lymphocytes and a rearrangement of TCR receptor in some cases made some authors suppose that ALHE is a low-grade neoplastic disease secondary to various stimuli. [8],[9]
More recently, Kempf et al. evaluated seven patients with ALHE and found that five of the seven patients showed a clonal T-cell population and proliferative T-cell activity, suggesting that a subset of these lesions might represent T-cell lymphoproliferative disorders of benign or low grade malignancy. Regardless of the anatomic site involved, there is not a single reported case of ALHE with aggressive behavior. Hence, it is fair to assume that these lesions are benign tumoral conditions. [10]
Grossly, these are usually circumscribed lesions of subcutis or dermis measuring 0.5-2.0 cm in size, with rare examples exceeding 5 cm. Less frequently they involve deep soft tissue, vessels, and parenchymal organs. Apart from the size, gross characteristics of this process are not well described. Many of them have a rather nonspecific nodular appearance and some with retained blood may have an appearance suggestive of a hemangioma. Occasionally, subcutaneous examples may resemble a lymph node because of its circumscription and a peripheral lymphoid reaction. [11]
Epithelioid hemangioma are characterized by a prominent proliferation of small, capillary-sized vessels lined by plump, epithelioid endothelial cells. The vessels typically have an immature appearance and they may lack a well-defined lumen, but they are well formed with single cell layering of the endothelium and an intact myopericytic/smooth muscle layer. The endothelial cells have amphophilic or eosinophilic cytoplasm that is sometimes vacuolated and they contain a single, relatively large nucleus with an open chromatin pattern and often, a central nucleolus. [11]
ALHE is usually well demarcated from the surrounding soft tissue, and commonly, it is associated with (sometimes centered around) a larger vessel, usually a muscular artery. An inflammatory milieu rich in eosinophils, plasma cells, and lymphocytes is present in the overwhelming majority of cases. Eosinophils usually account for 5-15% of the infiltrate, though in rare cases eosinophils may account for up to 50%. [3] Cytokines like interleukin-5 which interfere with the production and activation of eosinophils are supposed to play a key role in the pathogenesis of eosinophil rich inflammatory mileu in ALHE. [12]
Aggregation of lymphocytes is typical with a tendency to form lymphoid follicles. The lack of sinus structure indicates that these follicles arise in the subcutaneous tissue. [13]
It is also common to encounter epithelioid endothelial cells within the lumen of the larger vessel, either replacing part of the normal endothelial lining or "coating" fibrin fronds, as seen in papillary endothelial hyperplasia. [11]
Two types of ALHE lesion are described by Wells and Whimster. The early lesion demonstrates a predominance of rapidly proliferating atypical vasculature. However, late lesions illustrate maturation of these atypical blood vessels with thickened walls along with a high prevalence of lymphoid follicles seen towards the periphery of the lesion. [14]
Complete local excision and follow-up are optimal management for epithelioid hemangioma. Local recurrence is reported to occur in up to one-third of patients. [15] ALHE rarely regresses spontaneously; however, malignant transformation does not occur. [16]
| Conclusions | | |
Angiolymphoid hyperplasia with eosinophilia, a benign vascular tumor, commonly presents in third decade with a female preponderance. Surgical removal is the treatment of choice. There is no evidence of malignant transformation.
| Acknowledgement | | |
To management and technical staff of department of pathology,Katuri Medical College, Guntur.
| References | | |
| 1. | Chan JKC, Hui PK, Ng CS, Yuen NW, Kung IT, Gwi E. Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia) and Kimura's disease in Chinese. Histopathology 1989;15:557-74. 
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| 2. | Nair M, Aron M, Sharma MC. Angiolymphoid hyperplasia with eosinophilia (epithelioid hemangioma) of the breast: Report of a case. Surg Today 2000;30:747-9.
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| 3. | Meyerle J, Glusac E. Angiolymphoid hyperplasia with eosinophilia 2005. Available from: http://www.emedicine.com/derm/topic30.htm [Last accessed on 2006 Jan].
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| 4. | Weiss SW, Goldblum JR. Benign tumours and tumour-like lesions of blood vessels. In: Enzinger FM, Weiss SW, editors. Soft Tissue Tumours. 4 th ed. Philadelphia, PA: The CV Mosby Co; 2001. p. 856-64 .
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| 5. | Moran WJ, Dobleman TJ, Bostwick DG. Epithelioid hemangioendothelioma (histiocytoid hemangioma) of the palate. Laryngoscope 1987;97:1299-302 .
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| 6. | Fetsch JF, Weiss SW. Observations concerning the pathogenesis of epithelioid hemangioma (angiolymphoid hyperplasia). Mod Pathol 1991;4:449-55.
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| 7. | Doloi PK, Khanna S. Angiolymphoid Hyperplasia with Eosinophilia- A case report. IJOHNS International Journal of Otolaryngology and Head & Neck Surgery 2012;1:44-7.
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| 8. | Ramchandani PL, Sabesan T, Hussein K. Angiolymphoid Hyperplasia with Eosinophilia Masquerading as Kimura Disease. Br J Oral Maxillofac Surg 2005;43:249-52.
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| 9. | Demitsu T, Nagato H, Inoue T. Angiolymphoid Hyperplasia with Eosinophilia: Its Character and Therapy. Skin Surg 2000;9:8-16.
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| 10. | Kempf W, Haeffner AC, Zepter K, Sander CA, Flaig MJ, Mueller B, et al. Angiolymphoid hyperplasia with eosinophilia: Evidence for a T-cell lymphoproliferative origin. Hum Pathol 2002;33:1023-9.
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| 11. | Olsen TG, Helwig EB. Angiolymphoid hyperplasia with eosinophilia. A clinicopathologic study of 116 patients. J Am Acad Dermatol 1985;12:781-96.
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| 12. | Braun-Falco M, Fischer S, Plötz SG, Ring J. Angiolymphoid Hyperplasia with Eosinophilia treated with Anti-Interleukin-5 Antibody (Mepolizumab). Br J Dermatol 2004;151:1103-4.
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| 13. | Wright DH, Padley NR, Judd MA. Angiolymphoid hyperplasia with eosinophilia simulating lymphadenopathy. Histopathology 1981;5:127-40.
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| 14. | Wells GC, Whimster IW. Subcutaneous angiolymphoid hyperplasia with eosinophilia. Br J Dermatol 1969;81:1-14.
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| 15. | Reed RJ, Terazakis N. Subcutaneous angioblastic lymphoid hyperplasia with eosinophilia (Kimura's disease). Cancer 1972;29:489-97.
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| 16. | Kukreja N, Koslowski M, Insall R. Angiolymphoid hyperplasia with eosinophilia presenting as an axillary artery aneurysm. BMJ Case Reports 2011pii:bcr 0220113836.
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Correspondence Address:
Neelima Tirumalasetti
C/O Ch. Venkat Rao, Door No: 5-54-80, 6/18, Brodipet, Guntur - 522 002. Andhra Pradesh
India
 Source of Support: Indian Council of Medical Research, Conflict of Interest: None  | Check |
DOI: 10.4103/0377-4929.125352
[Figure 1], [Figure 2] |
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