A giant vascular hamartoma of the breast in a child.
H Deshmukh, S Prasad, T Patankar
Department of Radiology, King Edward Memorial Hospital, Mumbai, India., India
Department of Radiology, King Edward Memorial Hospital, Mumbai, India.
Vascular tumors of the breast are uncommon. Perilobular hemangiomas, the commonest histologic subtype of breast hemangiomas, constitute majority of microscopic, vascular mammary tumors. Macroscopic hemangiomas and vascular hamartomas of the breast are distinctly rare. We describe a giant, vascular hamartoma of the right breast in a five year old girl and discuss pertinent literature.
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Deshmukh H, Prasad S, Patankar T. A giant vascular hamartoma of the breast in a child. J Postgrad Med 1997;43:50-1
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Deshmukh H, Prasad S, Patankar T. A giant vascular hamartoma of the breast in a child. J Postgrad Med [serial online] 1997 [cited 2021 Apr 10 ];43:50-1
Available from: https://www.jpgmonline.com/text.asp?1997/43/2/50/407
A 5 year old girl presented with a painless, right breast mass gradually growing over a period of one year. An aspiration of the breast mass was attempted in a peripheral hospital which yielded blood. Clinical examination of the right breast revealed a large, well defined, non-tender, non-inflammatory swelling. Color doppler sonography showed blood flow within multiple tubular channels with both arterial and venous doppler signals. A right internal mammary angiogram showed hypertrophied vessels feeding a large, hypervascular mass [Figure:1]. There was no arterio-venous shunting. Prominent draining veins were seen emptying into the internal mammary vein [Figure:2]. Core biopsy of the lesion using 16G needle showed arrangement of the epithelial elements in the form of ducts and lobules with irregular, anastomosing slit-like spaces within a dense hyaline fibrous stroma. The channels lined by fibroblasts were filled with red blood cells. A histological diagnosis of a breast hamartoma associated with pseudo-angiomatous stromal hyperplasia (PASH) was made. The patient however, refused surgery.
Mammary hamartomas are well circumscribed tumors composed of a variable mixture of epithelial elements, fat and fibrous tissue. Breast hamartomas have been reported at all ages. They can grow to a large size and produce marked breast asymmetry. Mammography of hamartoma typically demonstrates a sharply circumsribed density which may have a mottled appearance due to tissue heterogeneity. A lucent zone separates the lesion from the adjacent breast parenchyma. The diagnosis of the hamartoma is based on a combination of clinical, radiologic and histopathologic findings.
Myoid hamartomas, fibroadenolipoma and chondrolipoma are histologic variants of hamartomas. Hamartomas with PASH may show marked vascularity. PASH is characterised histologically by proliferation of spindle cells of fibroblastic or myofibroblastic nature that line irregular, anastomosing slit-like spaces. PASH represents a localised form of stromal overgrowth with a hormonal (primarily progestogenic) pathogenesis.
Other vascular tumors of the breast include hemangiomas, lymphangiomas and lesions with both hemangio-lymphangiomatous components such as angiomatosis. Perilobular hemangiomas comprise the commonest microscopic vascular tumors, often incidentally found in mastectomy and biopsy specimen performed for breast disease. They are composed of meshwork of capillary sized blood vessels. Cavernous hemangiomas, venous hemangiomas and arteriovenous hemangiomas derive their nomenclature based on the type of vascular channels and constitute the macroscopic hemangiomas. They usually range in size from 0.2 to 2.5 cm in greatest diameter. Angiomatosis are unencapsulated lesions exhibiting non-circumscribed growth pattern as opposed to the circumscribed growth pattern of hemangiomas. Benign vascular tumors that can exceptionally involve the breast are hemangiopericytoma and cystic lymphangioma. Angiosarcoma, the malignant counterpart of hemangioma, constitutes the most common clinically palpable vascular lesion in the breast. It occurs in young females, metastazises extensively via bloodstream and consequently has poor prognosis. Hemangiomas and vascular hamartomas may resolve spontaneously. They may be associated with mammary hypoplasia if the underlying breast bud is involved in the neoplastic process.
In conclusion, hemangiomas and hamartomas constitute common vascular tumors of the pediatric breast and may show identical angiographic findings. Biopsy of the lesion is essential to establish the definitive diagnosis.
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