PSEUDOANGIOMATOUS STROMAL HYPERPLASIA (PASH) OF THE BREAST IN AN ADOLESCENT: A CASE REPORT AND LITERATURE REVIEW/ADOLESAN KIZ COCUGUNDA PSODOANJIYOMATOZ STROMAL HIPERPLAZI: OLGU SUNUMU VE LITERATUR DERLEMESI.
Pseudoangiomatous stromal hyperplasia (PASH) is a benign proliferative lesion of the breast stroma characterized by slit-like pseudovascular spaces lined by endothelial-like spindle cells. It is frequently found as incidental microscopic foci in the female breast and in gynecomastia of the male breast, but it rarely presents as a clinical problem. We report a case of bilateral extreme breast enlargement caused by PASH in an adolescent girl who was treated with subcutaneous mastectomy and reconstruction with breast implants following a recurrence after treatment with free nipple graft breast reduction.
Keywords: Pseudoangiomatous stromal hyperplasia; mammaplasty; breast implants.
Psodoanjiyomatoz stromal hiperplazi (PASH) meme bag dokusunun benign proliferatif lezyonlarindan birisidir. Endotel benzeri igsi hucrelerle doseli yarik benzeri psodovaskuler alanlarla karakterizedir. Meme eksizyonu ve jinekomasti piyeslerinde siklikla insidental olarak gorulse de nadiren klinik bir sorun olarak karsimiza cikmaktadir. Bu olgu sunumunda PASH nedeniyle asiri derecede bilateral meme buyumesi ile klinigimize basvuran adolesan bir kiz cocugunun klinik seyri ve tedavisi bildirilmektedir. Bu hastaya, serbest meme grefti kullanilarak meme kucultme ameliyati yapildiktan sonra gelisen nuks nedeniyle subkutan mastektomi yapilmis ve meme implanti yerlestirilmistir.
Anahtar Kelimeler: Psodoanjiyomatoz stromal hiperplazi; mamoplasti; meme Implantlari.
Pseudoangiomatous stromal hyperplasia (PASH) is a benign proliferative lesion of the breast stroma which was first described by Vuitch et al in 1986 (1). It is characterized by slit-like pseudovascular spaces lined by endothelial-like spindle cells. The term "pseudoangiomatous" describes the histological pattern which resembles an angiomatous proliferation. It is believed to originate from mammary myofibroblasts and, for that reason, Leon et al. proposed the term nodular myofibroblastic hyperplasia of the mammary stroma to denote its true histogenesis (2).
It is frequently found as incidental microscopic foci in the female breast and in gynecomastia of the male breast (3-4) , but it rarely presents as a clinical problem Although some case series regarding PASH are published previously, literature on PASH consists primarily of case reports (5-7). PASH has two primary clinical presentations- either distinct nodular growth or diffuse enlargement of the breast.
We report a case of bilateral extreme breast enlargement caused by PASH in an adolescent girl who was treated with subcutaneous mastectomy and reconstruction with breast implants following a recurrence after treatment with free nipple graft breast reduction.
A 13 years old girl presented with a 12 month history of extreme bilateral breast enlargement which started with menarche (Fig. 1).
She was referred to our clinic from the department of pediatrics with a preliminary diagnosis of virginal breast hypertrophy. At the time of presentation, her nipple to midclavicle distance was measured as 43 cm on the right side, and 50 cm on the left side. Breast enlargement was diffuse and homogenous with an absence of any solitary tumours. Multiple cutaneous ulcerations were present on both of her breast. Both breasts were extremely edematous and many dilated venous vessels were visible. There were no ly mphadenopathies present.
Hormone levels were found to be normal. Her past medical history was unremarkable. She did not have a family history of breast disease. She was not on any medication and she recently started menarche. Her breast ultrasonography revealed ectatic retroareolar ductal structures and an expanded fibroglandular breast paranchime.
The patient underwent bilateral breast amputation with a free nipple areolar graft using a Wise pattern resection. Breast projection was preserved using a bipedicled dermoglandular flaps (8). Total weight of the resected tissue was approximately 9000 g (Fig. 2). Histologic examination of the resected breast tissue revealed that breast enlargement was caused by the diffuse form of PASH. Immunohistochemical study of the specimen revealed positive immunoreactivity for CD34 and negative immunoreactivity for CD31.
There was a 4 x 5 cm large skin flap necrosis at the midline on the patient's right breast during the early postoperative course, which was treated by debridement and primary repair. No other problems were reported until the end of first year, when the parents noticed a homogenous growth of both breasts. The available treatment options were discussed with the patient and her parents and a subcutaneous mastectomy and immediate reconstruction with anatomic implants was performed. The 600 ml implants were placed under the pectoral muscle on both sides. The patient was followed-up for 30 months after the second operation. There were no problems related to skin flaps, and the nipple areolar complex which was previously transferred as a graft was viable, although both breasts have markedly lost their projections (Fig. 3). Due to changes in patients stature in the years following reconstruction with implants, the patient is scheduled for a replacement of implants with larger implants.
Rapid breast enlargement shortly after the beginning of puberty is termed as juvenile gigantomastia (9). Extreme cases requiring as far as 20 kg of breast tissue resection has been reported in the literature (10). Differential diagnosis of juvenile gigantomastia includes fibroadenomas, cystosarcoma phylloides, gravid hypertrophy of the breast, breast hypertrophy secondary to endocrine disorders, pseudogigantomastia associated with obesity, and malignant tumors such as lymphomas and sarcomas (11). To our knowledge, PASH of the breast was not seen associated with juvenile gigantomastia previously.
Pseudoangiomatous stromal hyperplasia was first reported by Vuitch et al. in 1986 as a benign breast disease characterized by a dense, collagenous proliferation of mammary stroma, forming inter-anastomosing capillary-like spaces lined by slender spindle cells (1). Empty spaces bordered with myofibroblasts were observed within the lesions, instead of true vessels covered with endothelial cells. In a study of 200 consecutive breast specimens evaluated for the presence of PASH, Ibrahim et al. reported that these changes could be detected microscopically in 23% of all routine breast biopsy specimens (12).
One of the largest series from a single institution was reported by Bowman et al. in 2012, consisting of 24 cases with PASH lesions (13). The reported size of tumors in this series ranged from 0.3 cm to 7.0 cm. All women in the series were premenopausal or perimenopausal at diagnosis except two cases. A hormonal basis for the development of PASH was suggested by the authors due to the positive staining of the specimes for hormonal receptors. Our case, however, is presented with a substantially different profile from the cases presented in this series, both in terms of age and size of the lesions. Due to extreme enlargement of the breasts and young age of the patient, we preferred to perform a breast reduction first, considering that it might be a safer and predictable alternative to bilateral subcutaneous mastectomy and reconstruction, which can be done at a later stage.
The exact etiology of PASH is debated but a hormonal component is suggested, based on observations that it appears most commonly in young premenopausal women or in elderly women receiving hormone-replacement therapy (12). Although this hypothesis is supported by the fact that similar histologic findings are seen in normal mammary stroma during the luteal phase of the menstrual cycle, the lesion is also seen in men and in women not taking hormone therapy.(14) Interestingly, some studies reported that only a small percentage of PASH cases are found to be positive for estrogen receptors or for progesterone receptors (15-16) Preoperative diagnosis with fine needle aspiration or core needle biopsies may be misleading due to the low cellularity of lesions. PASH has to be differenciated from a low-grade angiosarcoma, benign myofibroblastoma and mammary hamartoma. In the case of PASH, there are no blood cells in the slit-like spaces, and the nuclei of the spindle cells in the lesions are attenuated, lack atypia and do not exhibit mitotic activity. Immunohistochemical studies of the specimens may show positive immunoreactivity for CD34 and negative immunoreactivity for CD31 and desmin. Microscopically, PASH is composed of an almost normal duct and lobular structure, and an increased amount of fibrous stroma. A complex pattern of anastomosing empty spaces in the dense collagenous stroma is also seen.
Medical and surgical treatment options have been proposed with varying success. Some authors have recommended local excision.(17) Nevertheless, PASH may recur after an excision. Powel et al. reported a case in which a bilateral mastectomy was performed for recurrences. A case report by Pruthi et al. describes relief from symptomatic, biopsy-proven PASH with the administration of tamoxifen, with persistence of the lesion and subsequent recurrence of breast pain and engorgement upon discontinuation of the drug caused by adverse effects.(16) Resuming administration of the antagonist was, however, followed again by improvement of symptoms. This may be an indirect additional support of the hypothesis regarding the hormonal etiology of PASH.
There have been no cases in which PASH undergoes malignant transformation. The prognosis for patients with PASH is good, and there have been no reports of distant metastasis or death related to PASH. In a study of 9065 biopsies by Degnim et al., PASH was identified in 579 (6.4%) cases (18). Breast cancers occurred in 34 women with PASH (5.9%) and 789 without (8.8%). The study concluded that women with PASH do not have an increased future risk of breast cancer. Ferreira et al. suggested that PASH tumors diagnosed by biopsy but not subsequently excised remained stable and therefore, offering the option of close clinical surveillance instead of surgery may be a viable strategy.(19)
As our case has demonstrated, an extreme bilateral breast enlargement should raise clinical suspicion of PASH along with other more common conditions. The risk of recurrence should be noted. In cases where a diffuse enlargement of the breast is present, a two stage approach with subcutaneous mastectomy following a free nipple graft breast reduction can be safely performed.
(1.) Vuitch MF, Rosen PP, Erlandson RA. Pseudoangiomatous hyperplasia of mammary stroma. Hum Pathol 1986;17(2):185-91.
(2.) Leon ME, Leon MA, Ahuja J, Garcia FU. Nodular myofibroblastic stromal hyperplasia of the mammary gland as an accurate name for pseudoangiomatous stromal hyperplasia of the mammary gland. Breast J 2002;8(5):290-3.
(3.) Badve S, Sloane JP. Pseudoangiomatous hyperplasia of male breast. Histopathology 1995;26(5):463-6
(4.) Haj M, Loberant N, Cohen I. Pseudoangiomatous hyperplasia of mammary stroma in a male breast presenting as a discrete nodule. Breast J 2005;11(6):472-3.
(5.) Wieman SM, Landercasper J, Johnson JM, Ellis RL, Wester SM, Lambert PJ, Ross LA. Tumoral pseudoangiomatous stromal hyperplasia of the breast. Am Surg 2008;74(12): 1211-4.
(6.) Drinka EK, Bargaje A, Ersahin CH, Patel P, Salhadar A, Sinacore J, Rajan P. Pseudoangiomatous Stromal Hyperplasia (PASH) of the Breast: A Clinicopathological Study of 79 Cases. Int J Surg Pathol 2011;20(1):54-8.
(7.) Ozturk S, Yavuz E, Dursun M, Asoglu O, Ilhan R, Tuzlali S, Iplikci A. Pseudoangiomatous stromal hyperplasia of the breast (morphological and immunohistochemical analysis of 14 cases). Turk J Pathol 2005;21(3-4): 54-7.
(8.) Guven E, Aydin H, Basaran K, Aydin U, Kuvat SV. Reduction mammaplasty using bipedicled dermoglandular flaps and free-nipple transplantation. Aesthetic Plast Surg 2010;34(6):738-44.
(9.) Dancey A, Khan M, Dawson J, Peart F. Gigantomastia--a classification and review of the literature. J Plast Reconstr Aesthet Surg 2008;61(5):493-502.
(10.) Schumacher O, Ashkar W, Dabernig J, Nenadic I, Ingianni G. Juvenile gigantomastia of extreme magnitude: a case report. Ann Plast Surg 2009;63(4):369-72.
(11.) Baker SB, Burkey BA, Thornton P, LaRossa D. Juvenile gigantomastia: presentation of four cases and review of the literature. Ann Plast Surg 2001;46(5):517-25.
(12.) Ibrahim RE, Sciotto CG, Weidner N. Pseudoangiomatous hyperplasia of mammary stroma. Some observations regarding its clinicopathologic spectrum. Cancer 1989;63(6):1154-60.
(13.) Bowman E, Oprea G, Okoli J, Gundry K, Rizzo M, Gabram-Mendola S, Manne U, Smith G, Pambuccian S, Bumpers HL. Pseudoangiomatous stromal hyperplasia (PASH) of the breast: a series of 24 patients. Breast J 2012;18(3):242-7.
(14.) Guray M, Sahin AA. Benign breast diseases: classification, diagnosis, and management. Oncologist 2006;11(5):435-49.
(15.) Castro CY, Whitman GJ, Sahin AA. Pseudoangiomatous stromal hyperplasia of the breast. Am J Clin Oncol 2002;25(2):213-6
(16.) Pruthi S, Reynolds C, Johnson RE, Gisvold JJ. Tamoxifen in the management of pseudoangiomatous stromal hyperplasia. Breast J 2001;7(6):434-9.
(17.) Powell CM, Cranor ML, Rosen PP. Pseudoangiomatous stromal hyperplasia (PASH). A mammary stromal tumor with myofibroblastic differentiation. Am J Surg Pathol 1995; 19(3) :270-7
(18.) Degnim AC, Frost MH, Radisky DC, Anderson SS, Vierkant RA, Boughey JC, Pankratz VS, Ghosh K, Hartmann LC, Visscher DW. Pseudoangiomatous stromal hyperplasia and breast cancer risk. Ann Surg Oncol 2010;17(12):3269-77.
(19.) Ferreira M, Albarracin CT, Resetkova E. Pseudoangiomatous stromal hyperplasia tumor: a clinical, radiologic and pathologic study of 26 cases. Mod Pathol 2008;21(2):201-7.
Hasan Utkan AYDIN (*), Salih Onur BASAT (*), Burcu CELET OZDEN (*), Ufuk EMEKLI (*), Sitki TUZLALI (**)
Date received/Dergiye geldigi tarih: 13.04.2017. -- Date accepted/Dergiye kabul edildigi tarih: 29.05.2017
Istanbul Universitesi, Istanbul Tip Fakultesi, (*) Plastik, Rekonstruktif ve Estetik Cerrahi Anabilim Dali, (**) Patoloji Anabilim Dali, Istanbul, Turkiye
(Corresponding author/Iletisim kurulacak yazar: email@example.com)
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|Title Annotation:||CASE REPORT/ OLGU SUNUMU|
|Author:||Aydin, Hasan Utkan; Basat, Salih Onur; Ozden, Burcu Celet; Emekli, Ufuk; Tuzlali, Sitki|
|Publication:||Journal of Istanbul Faculty of Medicine|
|Date:||Jun 1, 2017|
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