Volume 5, Issue 1 , Pages 13-15, January 2010
Retroauricular intravascular lobular capillary haemangioma in a child
Article Outline
Summary
Lobular capillary haemangioma, formerly pyogenic granuloma, has an unclear etiopathogenesis and rarely occurs in an intravascular location.
A 9-year-male with a right retroauricular mass that was clinically mistaken for a typical dermoid but appeared unusually vascular at excision was finally diagnosed as an intravascular lobular capillary haemangioma arising in a haemangioma (composite haemangioma). The histolopathologic features of lobules of capillaries in a loose oedematous fibromyxoid stroma and “glomerulations” confirmed the diagnosis. At 2 years after excision, there is no recurrence and he is asymptomatic.
Keywords: Lobular capillary haemangioma, Intravascular, Child
1. Introduction
Lobular capillary haemangioma was formerly referred to as pyogenic granuloma—a misnomer since it is neither infectious nor a granuloma. The intravascular variant may rarely occur in pre-existing vascular lesions like haemangiomas and is termed a ‘composite haemangioma’. Although ultrasonographic features of moderate echogenicity and hypervascularity may be suggestive, the diagnosis is usually a histological surprise. This case is probably the youngest patient with the rare condition in published English literature.
2. Case report
A 9-year-male presented with a painless progressively enlarging retroauricular swelling of a year's duration. There was no antecedent history of trauma. On examination, the subcutaneous mass was well defined, hemispherical, firm, non-compressible and non-tender (Fig. 1). A clinical diagnosis of a post-auricular dermoid was made and no further imaging was contemplated. At excision, the 4
×3×3cm mass appeared unusually vascular and adherent to the overlying skin; numerous veins seemed to anchor the mass to a fibrofatty investment. The cut surface showed solid and cystic areas; the larger cysts had a smooth lining and contained clear fluid. Microscopically, a diffuse lesion with irregular proliferating vascular channels lay beneath the skin. These channels were lined by prominent endothelial cells forming “glomerulations”—intraluminal lobular masses covered with endothelium and attached to the vessel wall at one point (Fig. 2). Some glomerulations extended into the surrounding fibrofatty tissue (Fig. 3). Ectatic vascular channels with organising thrombi and cholesterol mural deposits were noted. A final diagnosis of an ‘intravascular lobular capillary haemangioma arising in a haemangioma’ was made. The post-operative period was uneventful. At 2 years follow up, he is asymptomatic and has had no recurrence.
Fig. 1.
Clinical photograph of retroauricular mass (incidentally meant to serve as teaching material for typical dermoid).
Fig. 2.
Photomicrograph (low power) of lesion showing “glomerulations”—intraluminal lobular masses covered by endothelium (H&E, ×250).
Fig. 3.
Photomicrograph (scanner) showing diffuse involvement of the surrounding fibrofatty tissue with the lesion (H&E, ×100).
3. Discussion
‘Pyogenic granulomas’, first described in 1897 by French surgeons, Poncet and Dor [1], were named so because of a presumed infectious etiology and associated granulomatous inflammation [2]. Since these lesions lack either of these and are angiomatous, they are now termed ‘lobular capillary haemangiomas’ [3]. Their exact pathogenesis is unknown; however a neoplastic origin is postulated [4]. The proposed etiologies include a hyperproliferative response at sites of trauma and wound healing, excessive production of tumour angiogenesis factor, viral oncogenesis, hormonal stimuli and drugs like indinavir, 5-fluorouracil, capecitabine and mitoxantrone [5].
Lobular capillary haemangioma affects the skin and mucous membranes of the aerodigestive tract; the intravascular variant occurs in the vessels. Cutaneous lesions occupy the superficial dermis and are bright red papules, nodules or polypoidal masses of varying sizes. They present as solitary, pedunculated/sessile swellings in the face, arms and hands; often with a history of antecedent trauma and sometimes at sites of cutaneous burns. Ulceration and bleeding are common; untreated lesions may undergo fibrosis and involute. The common mucosal lesion is the pregnancy eupulis—gingival lesions in early pregnancy. Mucosal lesions have been sporadically reported in children in various sites including the larynx [6] and colon [7].
The intravascular variant of lobular capillary haemangioma was described by Cooper et al. in 1979 and is reported to occur between 15 and 66 years [8]. It is commonly seen in relation to the veins of the head, neck and upper extremities and has a slight female preponderance [9]. 27 cases have been reported in the world literature till recently [10]. The first case in a child was reported from this institution by the senior author in 2001[11]. The intravascular variant may rarely occur in pre-existing vascular lesions like nevus flammeus, spider angiomas and haemangiomas [12]. In a detailed report of 128 cases of pyogenic granulomas in children, Pagliai et al. noted that 6 developed within a haemangioma [12]. The diagnosis of an intravascular capillary haemangioma is rarely clinical and usually a histological surprise. Previously reported cases in adults have occurred without a history of preceding trauma and the masses have been largely asymptomatic [13]. The described case was mistaken for a retroauricular dermoid while the other child reported from this institution earlier was thought to have a lipoma. In the head and neck region, they can mimic common congenital (e.g. dermoid, vascular malformation, branchial cyst) and acquired (e.g. enlarged lymph node, parasitic cyst, lipoma) conditions of childhood. An incidental ultrasound may detect the characteristic moderate echogenicity and hypervascularity and suggest the vascular nature [9], [10]. Rarely hypoechogenicity with minimal vascularity has been documented in large lesions [3]. An unusual vascularity at excision, as in our case, may be the first indication of a clinical misdiagnosis.
The diagnosis of a lobular capillary haemangioma is established at histopathological examination. It is characterised by lobules of capillaries in a loose oedematous fibromyxoid stroma. When an intravascular lobular capillary haemangioma arises in a haemangioma, it is termed a composite haemangioma. The intravenous/intravascular occurrence is attributed to the proliferating endothelium of vasa-vasorum in the parent vein with capillary formation and “glomerulations” are pathognomonic [11]. Histological differential diagnoses include other intravascular lesions such as angiosarcoma, intravascular papillary endothelial hyperplasia, intravenous atypical vascular proliferation, intravascularfasciitis, angiolymphoid hyperplasia and an organised thrombus [10].
Unlike cutaneous lesions where an array of therapeutic modalities is available, surgical excision is the treatment of choice for intravascular lobular capillary haemangioma. It is curative and has no risk of haematogenous spread or recurrence [8], [14].
4. Conclusion
Intravascular lobular capillary haemangioma (pyogenic granuloma) arising in haemangioma is a rare entity with a controversial etiopathogenesis. It may mimic common congenital and acquired neck masses in children; a careful ultrasonography may suggest the diagnosis. Surgical excision is curative and characteristic histological features confirm the diagnosis.
References
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PII: S1871-4048(09)00002-1
doi:10.1016/j.pedex.2008.12.007
© 2009 Elsevier Ireland Ltd. All rights reserved.
Volume 5, Issue 1 , Pages 13-15, January 2010
