Pseudoangiomatous Hyperplasia Research Articles

English

Gigantomastia is a rare breast disease with massive bilateral enlargement of breast causing physical and psychosocial disability for the patient. Diffuse pseudoangiomatous stromal hyperplasia causing gigantomastia is a very rare presentation. We present a case of 45-year-old female with bilateral gigantomastia and cyclical mastalgia, who underwent bilateral mastectomy, postoperatively diagnosed with pseudoangiomatous hyperplasia. Gigantomastia is a rare condition characterised by excessive breast tissue growth resulting in massive enlargement of both breast. An arbitrary definition for gigantomastia is breast size more than 1.5 kg or when the breast tissue comprises 3 % or more of patients total body weight. Gigantomastia is accompanied by skin ulceration, postural problems, back pain, traction nerve injury and social stigmata. It is physically and psychosocially disabling for the patient. The aetiopathogenesis of gigantomastia is poorly understood, but generally classified as idiopathic, drug induced (penicillamine, cyclosporine) and due to hormonal imbalance (gestational and pubertal).1 Pseudoangiomatous stromal hyperplasia (PASH) is a benign mesenchymal proliferative disease of the breast which is usually an incidental finding accompanying other breast pathology like fibroadenoma, fibrocystic disease, breast cancer, gynaecomastia etc. It was first described by Vuitch, Erlandson and rosen (1986). Ibrahim et al. reported the occurrence of incidental microscopic PASH in breast specimens as around 23 %.2 Around 200 cases of PASH presenting as a tumoral palpable mass is there in literature. Another very rare and under recognised presentation is as diffuse PASH with bilateral or unilateral massive breast enlargement. Here we report a case of diffuse PASH with gigantomastia and a review of literature.

Rapidly-growing pseudoangiomatous stroma hyperplasia (PASH) causing bilateral gigantomastia in a 15-year-old patient

A 15-year-old female patient presented to our clinic with rapidly-growing breasts, causing her back and neck pain, mastalgia, and unwanted attention at school. Clinical evaluation showed bilateral enlarged breasts with multiple palpable tumors. Her height and body weight were 1.76 m and 54 kg, respectively. Both ultrasound and magnetic resonance imaging (MRI) were conducted. Histological biopsies showed pseudoangiomatous stroma hyperplasia (PASH). Bilateral diffuse multiple tumors leading to massive bilateral breast enlargement are rare, especially in pediatric patients. To the best of our knowledge, all previous reported cases were patients of 29 years old or older. We present the evaluation and treatment in a rare case of multiple PASH causing bilateral gigantomastia in an adolescent patient.Level of evidence: Level V, therapeutic study.

PASH: stromal and pseudoangiomatous hyperplasia — surgical treatment using oncoplastic techniques — Thorek and dermal flap

PASH: stromal and pseudoangiomatous hyperplasia — surgical treatment using oncoplastic techniques — Thorek and dermal flap

Case Report: a rare case of PASH mimicking a lactational adenoma

Pseudoangiomatous stromal hyperplasia (PASH) is a rare, benign breast lesion that is usually discovered as an incidental finding in breast biopsies. We present a case report of a twenty three-year-old female who presented with a large central mass in the left breast 24 weeks into her pregnancy. An ultrasound-guided core biopsy was performed which was reported as a lactational adenoma and due to the significant size of the mass it was excised as a suspected giant lactational adenoma. The ultrasound appearance was of a mass with well-defined superficial and radial margins with multiple large gentle lobulations, and a thin echogenic pseudocapsule pointing towards a benign diagnosis. Multiple prominent internal vessels were visualised on doppler imaging; PASH lesions do not commonly have internal blood flow which therefore pointed away from the diagnosis in this case. It is likely the imaging features were confounded by the pregnant state. Macroscopically, the lesion consisted of a large lobulated red mass measuring 170 x 170 x 75 mm and weighing 838 g with a central area containing yellow cream-like material measuring 25 x 20 mm. Microscopically, the breast tissue showed prominent gynaecomastoid-like lobules with intervening oedematous stroma showing florid pseudoangiomatous hyperplasia. There was prominent but only patchy lactational change. PASH can often be an incidental finding and is commonly found in combination with other diagnoses. It is therefore possible for PASH to be overlooked in biopsy specimens, as in this case, and it is important to analyse the breast stroma carefully for evidence of PASH, even if the biopsy contains an alternative lesion that could account for the mass seen clinically. We feel this case highlights the potential for PASH to be overlooked in core biopsy specimens when a concurrent lesion is present and therefore not appropriately treated.

Review of cases with pseudoangiomatous hyperplasia (PASH) diagnosed in breast cores referred from breastscreen

Review of cases with pseudoangiomatous hyperplasia (PASH) diagnosed in breast cores referred from breastscreen

Double Independent Pedicle Oncoplastic Mammaplasty: A New Technique for Breast Preservation

INTRODUCTION: This study describes a novel means of oncoplastic mammaplasty named the double independent pedicle method. PATIENTS AND METHODS: Between October 2008 and August 2011, eight patients with upper- quadrant tumors of unfavorable proportions were included after signing an informed consent statement. The tumors were excised with a macroscopic margin of 2 cm. The areola was kept vascularized by means of a medial or lateral pedicle. An extensive inferocentral pedicle was constructed to fill the defect. Metal clips were emplaced to guide radiotherapy. The contralateral breast symmetry was corrected. RESULTS: The patients’ mean age was 49.00 (± 12.59) years. The mean tumor size was 44.25 mm (± 18.25 mm). The median preoperative ptosis was grade 2 (range: 1-3), and this was corrected in all cases. The physician’s assessment (from 0 to 10), for breast shape and symmetry, was 9.13 (± 1.46). All the patients said that they were more satisfied with breast shape after the surgery. A medial pedicle was chosen in half of the cases. Invasive ductal carcinoma was the commonest tumor. There was one benign case of pseudoangiomatous hyperplasia. One patient presented a voluminous hematoma, and evolved with partial dehiscence of the suture. There was one case of mild partial areolar necrosis. All the tumors were resected with free margins, but two patients (25.00%) underwent mastectomy and reconstruction after postoperative discovery of multicenter tumors. There was no fatty necrosis or recurrence over a 26.13-month (± 11.01) follow-up. CONCLUSIONS: The technique enabled breast conservation in unfavorable situations, with ptosis correction.

Pseudoangiomatous Hyperplasia of Mammary Stroma in a Male Breast Presenting as a Discrete Nodule

Pseudoangiomatous Hyperplasia of Mammary Stroma in a Male Breast Presenting as a Discrete Nodule

L’hyperplasie pseudo-angiomateuse du stroma mammaire: À propos d’un cas compliqué de gigantomastie

Pseudo-angiomatous hyperplasia of mammary stroma (PASH) is a histopathological entity which is a microscopic fortuitous finding in mammary biopsies performed for different reasons. It may be symptomatic and appears then as a palpable lump. The term pseudo-angiomatous emphasizes the characteristic aspect of the stroma simulating a vascular tumor. We report a case of PASH in a 71 year-old woman who presented a recurring breast mass with rapid swelling of the mammary gland (70 x 60 x 20 cm) treated by mastectomy. PASH must be distinguished from a well-differentiated angiosarcoma. It is ruled out by immunohistochemistry.

Aggressive Giant Fibroepithelial Lesion with Unusual Vascular Stroma—A Case Report

The stroma of fibroadenoma and phyllodes tumor usually consists of fibroblastic proliferation. Rarely the stroma contains bundles of smooth muscle. Pseudoangiomatous hyperplasia of the mammary stroma has been described in fibroadenomas. However, true benign vascular stroma has not been reported. We report a case of a 34-year-old Chinese woman who presented with a large mass occupying the entire left breast. Left mastectomy was performed and showed a large, well-circumscribed, lobulated, rubbery-firm tumor measuring 13 × 10 × 6 cm. Microscopic examination revealed a fibroepithelial tumor formed by an organoid pattern of ductal structures with a very striking stromal appearance composed of extensive vascular proliferation and that demonstrated strong immunoreactivity for CD31, CD34, and Factor VIII. Ultrastructural examination revealed intercellular junctions, basal lamina, pinocytotic vesicles, and Weibel-Palade bodies in the cells lining the vascular spaces, confirming their endothelial nature. These findings rule out the diagnosis of pseudoangiomatous hyperplasia. The patient developed local recurrence a year later, and the resection showed malignant phyllodes tumor with ductal carcinoma in situ. The extensive vascular stroma noted in the primary tumor may have played a role in the malignant transformation of the epithelial and stromal components in this tumor.

Expression of CD34 and bcl‐2 in phyllodes tumours, fibroadenomas and spindle cell lesions of the breast

Strong expression of CD34 and bcl-2 has been described in solitary fibrous tumours. It has been proposed that these lesions arise from long-lived mesenchymal cells. We tested the hypothesis that spindle cell lesions of the breast arise from similar mesenchymal cells in the mammary stroma. Sections of phyllodes tumours (26), fibroadenomas (15), myofibroblastomas (two), pseudoangiomatous hyperplasia (five) and myoid hamartoma (one) were stained immunohistochemically for CD34 and bcl-2. Conventional mammary carcinoma is known to be CD34-negative: we therefore stained 11 spindle cell carcinomas and one adenosquamous carcinoma. The mammary stroma, particularly around lobules, stained for CD34. All the lesions (except the carcinomas) showed spindle cell staining for CD34. There was more staining in fibroadenomas than in phyllodes tumours (especially malignant tumours). The staining in phyllodes tumours was typically patchy. In some there was increased or decreased staining adjacent to the epithelium. There were occasional spindle cells positive for bcl-2 in the normal perilobular stroma. bcl-2 was frequently expressed in spindle cells in fibroadenomas, phyllodes tumours and pseudoangiomatous hyperplasia, and rarely in the other lesions. The combined expression of CD34 and bcl-2 suggests that fibroadenomas, phyllodes tumours and pseudoangiomatous hyperplasia may arise from long-lived bcl-2-positive mesenchymal cells in the breast in a manner similar to that proposed for solitary fibrous tumours. The absence of CD34 staining in spindle cell carcinomas is of potential diagnostic value in the distinction from malignant phyllodes tumours in difficult cases.

Imaging Appearances of Pseudoangiomatous Hyperplasia of Mammary Stroma: Case Reports

A 34-year-old pre-menopausal women complained of ‘tingling’ in the outer half of the right breast which was associated with enlargement of the breast. There were no other symptoms. On examination the right breast was noted to be larger than the left breast. A discrete mass was not palpated. Mammography showed a uniform increase in the density of the breast but a discrete mass was difficult to identify (Fig. 1). Subsequent ultrasound demonstrated a large predominantly echogenic lesion with a well-defined wall and occasional cyst-like areas at its periphery. It was so large that it was not possible to measure the size of the lesion accurately, although using the cursors on the ultrasound image enabled an antero-posterior size estimate to be made (Fig. 2). Fine needle aspiration cytology (FNAC) was inadequate. Trucut biopsy showed scanty fragments of fibrocollagenous tissue with a few ductal clusters but a definitive diagnosis remained elusive. Magnetic resonance imaging (MRI) of the breast was undertaken on a Siemens Open 0.2T system using a shoulder coil with the patient in a semisupine position. T1-weighted spin-echo images (TR 420, TE 15 ms) were obtained before and after intravenous administration of 20 ml (9.4 g) dimeglumine gadopentetate (Gd-DTPA, Magnevist, Schering Health Care). Repeated T1W sequences were undertaken immediately following administration of the contrast agent but a dynamic sequence was not employed in this instance. These images confirmed the presence of a well-encapsulated lesion measuring 10 cm in diameter with signal characteristics strikingly lower than the adjacent fat. The lesion had a mixed signal pattern with areas of intermediate signal interspersed with islands of low intensity. The cystic lesions shown on ultrasound were difficult to identify on the non-enhanced images but were clearly identified after GdDTPA by the presence of an enhancing margin (Fig. 3). T2-weighted turbo spin-echo images (TR 3879, TE 102 ms) showed a low signal capsule encasing a lesion which was predominantly of high signal but within which there were areas of low signal (Fig. 4). The fluid nature of the cystic lesions noted on ultrasound was confirmed with low signal on T1W and high signal on T2W. An encapsulated mass measuring 13 × 11 × 3 cm was excised from the breast. The cut surface was mainly solid but with a few cysts at the periphery (Fig. 5). On microscopy the mass had a normal mixture of stromal and epithelial elements but the epithelium showed extensive microcystic change and occasional macroscopic cysts separated by abundant stoma containing a network of spaces lined by myofibroblasts (Fig. 6); this was confirmed by using S100 and factor VIII staining. A diagnosis of pseudoangiomatous hyperplasia was made.

Mammary Hamartomas: An Immunohistochemical Study of Ten Cases

Ten cases of breast hamartomas were reviewed; the patients' age ranged from 31 to 55 (mean 40.4, median 39). All cases presented with a palpable, sometimes tender, lump. The typical mammographic feature was a well defined, round to lens shaped, variable dense mass, occasionally surrounded by a thin radiolucent zone. All hamartomas were unilateral (4 in the right and 6 in the left breast, respectively) and no recurrence occurred after local excision. The tumor size ranged from 5 to 150 mm (mean 54 mm). Histologically all hamartomas were composed of a typical fibrous, adipose and glandular tissue combination. Immunohistochemically there was a strong positivity for cytokeratin and epithelial membrane antigen in the epithelial cells, a positive finding for vimentin and muscle-specific actin in stromal and myoepithelial cells, and for S-100 protein in myoepithelial cells. Vessels endothelial cells were immunoreactive for Factor VIII. Immunohistochemical analysis of hormone receptors completed on formaldehyde-fixed paraffin-embedded specimens, showed estrogen and progesterone receptors positivity in 9 cases and estrogen positive progesterone negative receptors in one case. In all cases the receptorial positivity was limited to the epithelial elements. These data revealed that 1) breast hamartoma is a benign, tumor-like lesion, histologically dissimilar from other lesions such as fibroadenoma and pseudoangiomatous hyperplasia; and 2) hamartoma tissue is influenced by hormones like the surrounding normal breast parenchyma.

Pseudoangiomatous hyperplasia of mammary stroma associated with gynaecomastia.

AIMS: To evaluate the prevalence of pseudoangiomatous hyperplasia of mammary stroma in gynaecomastia and its immunohistochemical profile in this setting. METHODS: Eighty eight cases of gynaecomastia recovered from the files...

Additional mimics of mucinous mammary carcinoma: fibroepithelial lesions.

To determine the origin and nature of mucinlike material in fine-needle aspiration (FNA) smears of the breast in noncancerous breast lesions, we studied breast FNA smears from four patients. All smears contained epithelial cells floating in a mucinlike background, which raised suspicion for mucinous (colloid) carcinoma. Mucicarmine stain was performed on one smear from each case. Subsequent tissue biopsy specimens were studied using mucicarmine, periodic acid-Schiff with and without diastase, and alcian blue stains at pH 2.7 and 0.9 on selected tissue sections. Correlation of the cytologic and histologic findings of each lesion was performed. The mucinlike background in all four FNA smears stained strongly with mucicarmine. Corresponding biopsy specimens revealed pseudoangiomatous hyperplasia in the first case, fibroadenoma and atypical ductal hyperplasia in the second, benign phyllodes tumor in the third, and fibroadenoma in the fourth. Each lesion in cases 1 to 3 was associated closely with fibrocystic changes. In case 4, cystic changes were located within the fibroadenoma. On tissue sections of all four cases, the cyst contents and 10% to 50% of normal lobule and duct contents stained with mucicarmine, indicating that the cyst contents were the most probable source of mucin in the FNA smears. The presence of pools of mucicarmine-positive material in FNA smears of the breast is not an exclusive feature of mucinous carcinoma; mucicarmine-positive mucin can arise from benign cystic changes as well as from normal lobules and ducts.

Pseudoangiomatous Hyperplasia of the Mammary Stroma: True Entity or Phenotype?

We report the clinical and pathologic features seen in 14 cases of pseudoangiomatous hyperplasia of the mammary stroma. The lesion manifested as a discrete palpable lump in twelve women and two men. The excisional biopsy specimens showed firm, circumscribed grey-tan lesions measuring from 3 to 5.5 cm in largest dimension. Histologic study revealed diffuse, anastomosing, enlongated slits with open lumina, covered by spindle cells with bland nuclei and no mitotic activity. Strong reactivity for vimentin and CD34 was noted. Smooth muscle actin was focally noted in most cases. Immunostains for other markers, including CD31 and factor VIII related antigen was negative. Spindle cells from two cases expressed both estrogen and progesterone receptors. In all the cases, there was some degree of glandular hyperplasia accompanying the stromal changes. Fibroadenoma or fibrocystic disease were also common features. Our study confirms that pseudoangiomatous hyperplasia of mammary stroma represents a proliferation of local myofibroblasts, likely related to a hormonal stimulus. The wide range of associated changes of breast parenchyma further indicates that this lesion may represent a local, non specific change rather than a true clinico-pathologic entity.

Pseudoangiomatous hyperplasia of male breast

Ninety-three male breast specimens have been examined for the presence of pseudoangiomatous hyperplasia of the mammary stroma which has hitherto been described almost exclusively in females. Forty-four cases (47.4%) showed some degree of hyperplasia, varying from small microscopic foci to extensive change involving 90% of the mammary tissue. All but one were found in association with gynaecomastia, early and intermediate stage. The association between pseudoangiomatous hyperplasia and benign proliferative lesions mirrors that reported in the female breast, and our findings suggest that the change may represent a stage in the maturation of newly formed mammary stroma.

Mammary hamartoma--a review of 35 cases.

Mammary hamartomas are macroscopically well-delineated tumours composed of a variable mixture of epithelial elements, fat and fibrous tissue. Such lesions are an under-recognized entity and, as they can be visualized by mammography, may be seen more frequently with the advent of the UK National Breast Screening Programme. The clinical and pathological features of 35 cases of mammary hamartoma seen at the Imperial Cancer Research Fund Clinical Oncology Unit at Guy's Hospital between 1979 and 1990 have been reviewed. Hormone receptor analysis on nine cases gave high progesterone with low oestrogen levels, probably reflecting their premenopausal status. Immunohistochemistry showed that the positive receptor staining was confined to the epithelial elements. In 25 cases pseudo-angiomatous hyperplasia was evident in the stroma of the lesion. The importance of distinguishing the inter-anastomosing stromal spaces seen in the latter condition from low-grade angiosarcoma is emphasized; the relationship between pseudo-angiomatous hyperplasia and mammary hamartoma is discussed; and the possibility that the former represents a permanently dilated form of the lymphatic labyrinth suggested.

Immunocytochemical Analysis of Estrogen and Progesterone Receptors in Benign Stromal Lesions of the Breast

Five cases of pseudoangiomatous hyperplasia of mammary stroma, together with seven examples of mammary hamartoma, were probed with monoclonal antibodies H222 and KD68 to investigate the possible role of estrogen and progesterone receptor expression in the pathogenesis of these benign stromal proliferations. All five cases of pseudoangiomatous hyperplasia showed patchy, intense labelling of the stromal cells with progesterone receptor antibodies, a pattern contrasting markedly with the absence of immunoreactivity in normal (nongestational) mammary stroma or the stromal component of common juvenile mammary hyperplasia. The stroma of the hamartoma group labeled inconsistently, with the notable exception of three myoid hamartomas. Stromal immunoreactivity was diffuse and intense in two of these, and patchy and distinct in the remaining case. These findings (a) support the contention that pseudoangiomatous hyperplasia represents a localized form of stromal overgrowth with a hormonal (primarily progestagenic) etiology and (b) further highlight the heterogeneity of so-called mammary hamartomas by demonstrating dramatically different progesterone receptor immunoreactivity patterns in myoid lesions as compared with other hamartoma variants.

Pseudoangiomatous hyperplasia of mammary stroma

Grossly circumscribed, nonhemorrhagic breast masses consisting of mammary stromal proliferations that simulated vascular lesions were studied in nine women. Histologically, a striking pattern, which appeared to consist of complex inter-anastomosing channels lined by slender spindle cells, was present in the mammary parenchyma. The importance of this benign lesion, referred to as pseudoangiomatous hyperplasia of mammary stroma, is its distinction from angiosarcoma. The patients ranged in age from 22 to 52 years; all were premenopausal. Each presented with a palpable unilateral mass, measuring up to 7 cm in diameter. The patients were treated by excisional biopsy and remained well for up to 2.5 years after excision. One patient had two local recurrences within one year of the original excision, and a second patient had a local recurrence at 14 months. No patient had another concurrent or metachronous malignant tumor of the breast or other organ, and no abnormal hormonal status was found. Complete local excision appears to be adequate treatment. It remains to be determined whether this is a neoplastic process. However, there is no evidence that it is a precursor of angiosarcoma, and ultrastructural observations demonstrate that the spaces found in the lesion are not true vascular channels. Rather, they appear to arise by a process that involves disruption and separation of stromal collagen fibers. Since small foci of this change are common in hyperplastic breast tissue from premenopausal women, it is likely that the development of a discrete tumor with this pattern represents an exaggerated form of stromal hyperplasia.