(Radiographics. 1999;19:1086-1088.)
© RSNA, 1999
Breast Imaging Case of the Day1
Dvora Cyrlak, MD and
Philip M. Carpenter, MD
1 From the Departments of Radiological Sciences and Pathology, University of California, Irvine Medical Center, 101 The City Drive, Orange, CA 92868-3298. From the 1998 RSNA scientific assembly. Received January 4, 1999; revision requested January 12 and received January 22; accepted January 22. Address reprint requests to D.C.
Index Terms: Breast neoplasms, 01.31 • Breast neoplasms, diagnosis, 01.31
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HISTORY
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A 40-year-old woman underwent mammography and ultrasonography (US) for a 3-cm palpable mass in the upper outer quadrant of the right breast.
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FINDINGS
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The right breast mass was mostly obscured at craniocaudal mammography (Fig 1a), but its borders appeared predominantly circumscribed in the mediolateral projection (Fig 1b). There were no associated calcifications. US demonstrated a circumscribed, heterogeneous, hypoechoic solid mass containing cystic spaces (Fig 2). Excisional biopsy of the mass was performed.
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Figure 1a. Craniocaudal (a) and mediolateral (b) mammograms of the right breast show a 3 x 4-cm mass in the upper outer quadrant. The mass was palpable at clinical examination (note overlying skin BB). The margins of the mass are mostly obscured in a but are predominantly circumscribed in b.
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Figure 1b. Craniocaudal (a) and mediolateral (b) mammograms of the right breast show a 3 x 4-cm mass in the upper outer quadrant. The mass was palpable at clinical examination (note overlying skin BB). The margins of the mass are mostly obscured in a but are predominantly circumscribed in b.
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DIAGNOSIS: Pseudoangiomatous stromal hyperplasia.
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DISCUSSION
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Pseudoangiomatous stromal hyperplasia is a benign proliferative lesion of mammary stroma containing complex anastomosing spaces that may be confused with angiosarcoma at histologic analysis (1). It represents a clinicopathologic spectrum ranging from focal, incidental microscopic findings to clinically and mammographically evident breast masses (2). Pseudoangiomatous stromal hyperplasia is a relatively common incidental finding at breast biopsy. In a study by Ibrahim et al (2) of 200 consecutive breast specimens, 23% (n = 46) demonstrated this finding. In contrast, the tumoral form of pseudoangiomatous stromal hyperplasia is rare, with only 71 previously reported cases in the literature (1–7).
The tumoral form of pseudoangiomatous stromal hyperplasia most commonly manifests as a single, circumscribed, palpable mass in a premenopausal female (1,3–6). The mass is usually large (5–6 cm in diameter), with reported diameters ranging from 1 to 12 cm (1,4,6). The reported age range of patients with pseudoangiomatous stromal hyperplasia is 14–67 years, although most patients are in their forties or late thirties (1,3–6). The masses may grow over time and may recur after excision, and contralateral pseudoangiomatous stromal hyperplasia has been reported in two patients (1,4,6). Tumoral pseudoangiomatous stromal hyperplasia has frequently been misdiagnosed at clinical examination as a fibroadenoma (6). Only one case of pseudoangiomatous stromal hyperplasia in a male (with axillary gynecomastia) has been reported (7).
At mammography, masses due to pseudoangiomatous stromal hyperplasia are usually non-calcified and appear well circumscribed or (as in this case) partially circumscribed (Fig 1) (3,4,6). Indistinct or obscured borders have also been reported (6), as has a single mass with a spiculated border (3). The masses are seen at US as hypoechoic solid masses (3,4). Echotexture may be slightly heterogeneous, and a small cystic component has been reported in one case (4). In our patient, a cystic component that was evident at US (Fig 2) was confirmed at pathologic analysis. Posterior sound quality varies from moderate enhancement to mild shadowing (3).
Although mass lesions in pseudoangiomatous stromal hyperplasia often grow over time and may recur after excisional biopsy, they are neither associated with malignancy nor considered to be premalignant lesions (4). At histologic analysis, this benign tumor of breast stroma has a "characteristic pattern of proliferating myofibroblasts that creates slit-like spaces" (1) (Fig 3). The myofibroblasts are believed to have aberrant reactivity to hormones (particularly progesterone) that starts as a focal accentuation of mammary physiologic changes during the menstrual cycle (1,5,6). According to Powell et al (6), "Foci that eventually create discrete masses probably escape normal physiological control mechanisms, cease cycling with the remaining breast, and acquire the capacity for independent myofibroblastic proliferation."
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Figure 3a. (a) Low-power photomicrograph (original magnification, x40; hematoxylin-eosin stain) shows cellular fibrous stroma surrounding small glands. (b) Higher-power photomicrograph (original magnification, x400; hematoxylin-eosin stain) demonstrates separation of collagen bundles from stromal cells, which simulates multiple small vascular spaces. These spaces do not contain red blood cells.
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Figure 3b. (a) Low-power photomicrograph (original magnification, x40; hematoxylin-eosin stain) shows cellular fibrous stroma surrounding small glands. (b) Higher-power photomicrograph (original magnification, x400; hematoxylin-eosin stain) demonstrates separation of collagen bundles from stromal cells, which simulates multiple small vascular spaces. These spaces do not contain red blood cells.
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The pathologist may confirm the presence of myofibroblastic proliferation in pseudoangiomatous stromal hyperplasia with immunohistochemical testing. The tumor will test negative for factor VIII, an endothelium-specific marker, and positive for CD34 and muscle actin (1,6). Biochemical testing has demonstrated progesterone receptors but not estrogen receptors (5). The most important differential diagnostic consideration for the pathologist is low-grade angiosarcoma. The stromal cells of pseudoangiomatous hyperplasia usually have a benign nuclear appearance in contrast to the highly atypical appearance of the endothelial cells of an angiosarcoma. Because of its stromal cellularity, pseudoangiomatous stromal hyperplasia may also be mistaken for phyllodes tumor, although it lacks the typical abnormal glandular configuration of the latter. Finally, pseudoangiomatous stromal hyperplasia may be mistaken for a fibroadenoma if the pseudovascular spaces are not recognized.
Pseudoangiomatous stromal hyperplasia is a rare cause of a dominant, circumscribed or partially circumscribed solid breast mass, particularly in premenopausal females (4). If the results of core biopsy indicate pseudoangiomatous stromal hyperplasia in a lesion that is consistent with this diagnosis, confirmation with open biopsy is not necessary (3). Mammographic follow-up is an option, and if there is further tumor growth, open biopsy is performed (3). In our patient, there was no evidence of recurrence at follow-up mammography performed 6 months after surgical excision. It is important for the radiologist to be aware of pseudoangiomatous stromal hyperplasia so that he or she can suggest the diagnosis in the appropriate clinical and mammographic setting and understand its implications for patient management.
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References
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Vuitch MF, Rosen PP, Erlandson RA. Pseudoangiomatous hyperplasia of mammary stroma. Hum Pathol 1986; 17:185-191.[Medline]
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Ibrahim RE, Sciotto CG, Weidner N. Pseudoangiomatous hyperplasia of mammary stroma: some observations regarding its clinicopathological spectrum. Cancer 1989; 63:1154-1160.[Medline]
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Cohen MA, Morris EA, Rosen PP, Dershaw DD, Liberman L, Abramson AF. Pseudoangiomatous stromal hyperplasia: mammographic, sonographic and clinical patterns. Radiology 1996; 198:117-120.[Abstract/Free Full Text]
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Polger MR, Denison CM, Lester S, Meyer JE. Pseudoangiomatous stromal hyperplasia: mammographic and sonographic appearances. AJR 1996; 166:349-352.[Abstract/Free Full Text]
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Anderson C, Ricci A, Jr, Pedersen CA, Cartun RW. Immunocytochemical analysis of estrogen and progesterone receptors in benign stromal lesions of the breast: evidence for hormonal etiology in pseudoangiomatous hyperplasia of mammary stroma. Am J Surg Pathol 1991; 15:145-149.[Medline]
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Powell CM, Cranor ML, Rosen PP. Pseudoangiomatous stromal hyperplasia (PASH): a mammary stromal tumor with myofibroblastic differentiation. Am J Surg Pathol 1995; 19:270-277.[Medline]
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Seidman JD, Borkowski A, Aisner SC, Chen-Chih JS. Rapid growth of pseudoangiomatous hyperplasia of mammary stroma in axillary gynecomastia in an immunosuppressed patient. Arch Pathol Lab Med 1993; 117:736-738.[Medline]
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HISTORY
FINDINGS
