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Table of Contents
CASE REPORT
Year : 2022  |  Volume : 19  |  Issue : 1  |  Page : 51-53

Giant pseudoangiomatous stromal hyperplasia of breast: Management challenge of gigantomastia with ptosis


Department of General Surgery, Apollo (Main) Hospital, Chennai, Tamil Nadu, India

Date of Submission23-Aug-2021
Date of Decision01-Oct-2021
Date of Acceptance11-Oct-2021
Date of Web Publication06-Dec-2021

Correspondence Address:
Uma Krishnaswamy
Department of General Surgery, Apollo (Main) Hospital, Chennai - 600 006, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/am.am_100_21

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  Abstract 


Despite increasing information on Pseudoangiomatous Stromal Hyperplasia of Breast (PASH), the challenges of management of giant PASH remain for the surgeon. A pragmatic and individualized approach is the solution in the absence of reliable algorithms for management. This instance of a giant PASH tumor producing gigantomastia with severe ptosis is a case to point. When PASH presents as a giant mass, leading to gigantomastia with inevitable severe ptosis, the surgical options range from the excision of mass with reduction mammoplasty or a simple mastectomy with or without reconstruction. The key to surgical decision-making is individualization of surgery.

Keywords: Giant pseudoangiomatous stromal hyperplasia of breast, gigantomastia, ptosis


How to cite this article:
Krishnaswamy U, Balachandar K, Kathirazhagan T. Giant pseudoangiomatous stromal hyperplasia of breast: Management challenge of gigantomastia with ptosis. Apollo Med 2022;19:51-3

How to cite this URL:
Krishnaswamy U, Balachandar K, Kathirazhagan T. Giant pseudoangiomatous stromal hyperplasia of breast: Management challenge of gigantomastia with ptosis. Apollo Med [serial online] 2022 [cited 2022 May 22];19:51-3. Available from: https://www.apollomedicine.org/text.asp?2022/19/1/51/331847




  Introduction Top


Since the description of Pseudoangiomatous Stromal Hyperplasia of Breast (PASH) in 1986 by Vuitch,[1] clinical, radiological, and pathological information has been accruing steadily. There are approximately 1500 cases documented.[2] The clinical presentation varies from microscopic PASH found incidentally to that of clinically palpable breast masses. Gigantomastia due to tumorous PASH is a relatively uncommon presentation with less than ten instances reported in the literature.[3] Guidelines on the surgical technique to be adopted do not exist and decision-making falls on the individual surgeon factoring in the patient's preference.


  Case Report Top


A 47-year-old obese, premenopausal lady presented with increasing size of the left breast of 1 year duration. There was the heaviness of the breast and mid back pain interfering with daily activities as well as social embarrassment. The delay in the presentation was ascribed to the COVID pandemic. Her other concern was long-standing menorrhagia with secondary anemia for which the option of an intrauterine device, to gradually release a progestin (levonorgestrel) was being considered.

On examination, there was gigantomastia of the left breast with severe ptosis, with the nipple at the level of the umbilicus. An 18 cm × 15 cm, firm, mobile mass was presently occupying all four quadrants of the breast. The contralateral breast and both axillae were normal.

The mammogram revealed a radiodense mass occupying all quadrants of the breast [Figure 1]. The contralateral breast and both axillae were normal. An ultrasound correlation suggested minimal internal vascularity and led to an impression of a benign giant phyllodes tumor.
Figure 1: Left mammograms

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A core needle biopsy was next performed [Figure 2]. This revealed distorted lobules and tubular ducts with expanded interlobular stroma. The stroma in turn showed areas of hyalinized collagenous tissue with slit-like spaces lined by spindle cells. These cells were uniform with hyperchromatic nucleus with no evidence of mitosis, atypia, or malignancy. The pathological impression was that of a mildly cellular stromal spindle cell proliferation suggestive of a diffuse PASH tumor.
Figure 2: Core biopsy Pseudoangiomatous Stromal Hyperplasia of Breast

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An immunohistochemistry panel followed. CD-34 was positive in the myofibroblasts lining the slit-like spaces [Figure 3]. CD-31 was negative in the slit-like spaces. Estrogen receptor, progesterone receptor, and Desmin were negative and SMA (Smooth Muscle Actin) was positive, confirming PASH.
Figure 3: CD34 stain Pseudoangiomatous Stromal Hyperplasia of Breast

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After extensive counseling on the surgical options available, the patient chose and underwent a simple mastectomy without immediate reconstruction. The patient was fitted with an external prosthesis and was satisfied with the outcome. The option of delayed reconstruction remains open. Follow-up is by periodic clinical surveillance once in 6 months and imaging annually.

Histopathology of the operated specimen confirmed that the tumor was 20 cm × 16 cm × 10 cm and weighed 1.5 kg. Microscopically, the lesion was composed of multiple inter anastomosing spaces with the interlobular stroma expanded by dense collagenous tissue. [Figure 4]. The spaces were lined by spindle-shaped myofibroblast cells. There was no evidence of atypia, increased mitosis, or malignancy. Adjacent breast tissue was normal, and all margins were free of the lesion.
Figure 4: Diffuse Pseudoangiomatous Stromal Hyperplasia of Breast operative specimen

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  Discussion Top


Consensus guidelines,[4] on the management of PASH confine themselves to small masses with concordant triple assessment, wherein surgical excision is not necessary and follow-up alone suffices. With the proviso, that if there is increase in size, imaging-pathology discordance, associated suspicious imaging, or a strong family history of breast cancer, excision is undertaken with long-term surveillance. In what is already a giant tumor, conservative management is a moot point as surgery is mandatory.

There is no mention of giant PASH in the guidelines. Here, the surgeon must arrive at the optimal surgical technique by analyzing the risk–benefit ratio for a given patient. The factors for input are:

In the assessment of gigantomastia caused by a huge PASH tumor ruling out associated malignancy or other high-risk lesions is a challenge, with the large mass potentially masking such lesions. Hence reliance is placed on core needle biopsies harvesting multiple cores from all quadrants, rather than on fine needle aspiration cytology which is diagnostically unreliable.[5] The sensitivity of core needle biopsies has been reported to be 83%,[6] this does not preclude the possibility of missing a malignant lesion within the bulk of the tumor during preoperative assessment and having to make this discovery in the histopathology of the resected specimen. In such an eventuality, a second surgery may be needed to address malignancy.

While PASH is not considered premalignant, there have been instances of associated malignancy diagnosed in the same core needle biopsy, though separate from PASH. It has been reported that 25% of the patients with PASH were identified as having ductal carcinoma in situ, lobular carcinoma in situ, or infiltrating carcinoma.[7] In such instances, the surgical technique must primarily address the synchronous malignancy along with excision of the associated PASH.

PASH is not considered a high-risk lesion. It has been noted that only 5.9% of the patients develop future breast cancer, mostly in the ipsilateral breast (85%), more than 5 years later.[8] On the one hand, PASH has a negative correlation with breast cancer, but on the other hand, the ipsilaterality of future breast cancer suggests that PASH may also produce a permissive stromal environment for breast cancer.[9],[10] This is a paradox. Hence, though the incidence of future malignancy is relatively low, long-term surveillance of the patients becomes mandatory. Such surveillance can be arduous for younger patients with attendant worry and cost implications.

There are reports of recurrence after simple excision. These range from 15%–22%.[1],[11] The causes of recurrence are incomplete excision with residual PASH or de novo growth. In diffuse PASH where there is no well-defined capsule, a breast-conserving excision may not secure clear margins.[12]

The higher incidence of PASH in premenopausal women, particularly those who were taking oral contraceptive medication,[13] and the common occurrence of progesterone receptors in PASH, has given rise to a hypothesis of a hormonal basis for PASH, with specific reference to progesterone-related proliferation of stromal cells.[14],[15] With the possibility of this patient opting for an intrauterine device with levonorgestrel in the near future for her menorrhagia, recurrence was a possibility with excision and a reduction mammoplasty.

All of the above factors make it imperative to individualize surgical treatment, particularly in those presenting with gigantomastia giving due weightage to the patient's individual needs and concerns.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgment

The author would like to thank Department of Pathology, Apollo (Main) Hospital, Chennai.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Vuitch MF, Rosen PP, Erlandson RA. Pseudo angiomatous hyperplasia of mammary stroma. Hum Pathol 1986;17:185-91.  Back to cited text no. 1
    
2.
Estes A, Cao L, Miller ME. Pseudo angiomatous stromal hyperplasia: Overview and clinical management. Ann Breast Surg 2020;4:22-36.  Back to cited text no. 2
    
3.
Kim JH, Baek SE, Yoo TK, Lee A, Oh DY. Relapsed bilateral gigantomastia caused by pseudo angiomatous stromal hyperplasia after reduction mammoplasty. Arch Aesthetic Plast Surg 2018;24:78-82.  Back to cited text no. 3
    
4.
The American Society of Breast Surgeons Consensus Guideline on Concordance Assessment of Image-Guided Breast Biopsies and Management of Borderline or High-Risk Lesions; November 02, 2016. Available from: https://www.breastsurgeons.org/docs/statements/Consensus-Guideline-on-Concordance-Assessment-of-Image-Guided- Breast-Biopsies.pdf. [Last accessed on 2021 Aug 08].  Back to cited text no. 4
    
5.
Levine PH, Nimeh D, Guth AA, Cangiarella JF. Aspiration biopsy of nodular pseudo angiomatous stromal hyperplasia of the breast: Clinicopathologic correlates in 10 cases. Diagn Cytopathol 2005;32:345-50.  Back to cited text no. 5
    
6.
Wieman SM, Landercasper J, Johnson JM, Ellis RL, Wester SM, Lambert PJ, et al. Tumoral pseudo angiomatous stromal hyperplasia of the breast. Am Surg 2008;74:1211-4.  Back to cited text no. 6
    
7.
Gresik CM, Godellas C, Aranha GV, Rajan P, Shoup M. Pseudo angiomatous stromal hyperplasia of the breast: A contemporary approach to its clinical and radiologic features and ideal management. Surgery 2010;148:752-7.  Back to cited text no. 7
    
8.
Degnim AC, Frost MH, Radisky DC, Anderson SS, Vierkant RA, Boughey JC, et al. Pseudo angiomatous stromal hyperplasia and breast cancer risk. Ann Surg Oncol 2010;17:3269-77.  Back to cited text no. 8
    
9.
Radisky ES, Radisky DC. Stromal induction of breast cancer: Inflammation and invasion. Rev Endocr Metab Disord 2007;8:279-87.  Back to cited text no. 9
    
10.
Rozenchan PB, Carraro DM, Brentani H, de Carvalho Mota LD, Bastos EP, E Ferreira EN, et al. Reciprocal changes in gene expression profiles of cocultured breast epithelial cells and primary fibroblasts. Int J Cancer 2009;125:2767-77.  Back to cited text no. 10
    
11.
Powell CM, Cranor ML, Rosen PP. Pseudo angiomatous stromal hyperplasia (PASH). A mammary stromal tumor with myofibroblastic differentiation. Am J Surg Pathol 1995;19:270-7.  Back to cited text no. 11
    
12.
Sng KK, Tan SM, Mancer JF, Tay KH. The contrasting presentation and management of pseudo angiomatous stromal hyperplasia of the breast. Singapore Med J 2008;49:e82-5.  Back to cited text no. 12
    
13.
Surace A, Liberale V, D'Alonzo M, Pecchio S, Baù MG, Biglia N. Pseudo angiomatous stromal hyperplasia (PASH) of the breast: An uncommon finding in an uncommon patient. Am J Case Rep 2020;21:e919856.  Back to cited text no. 13
    
14.
Ibrahim RE, Sciotto CG, Weidner N. Pseudo angiomatous hyperplasia of mammary stroma. Cancer 1989;63:1154-60.  Back to cited text no. 14
    
15.
Rosen PP. Pseudo angiomatous hyperplasia of mammary stroma. In: Rosen PP, editor. Rosen's Breast Pathology. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2001. p. 757-66.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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