Worried BRCA+ no ovaries dx DE, SA & Inflammatory Mastitis

YellowGirl

Hello Everyone,

I am desperate for some advice on advocacy and Surgical Oncology preparation. About a month ago I looked in the mirror and noticed that my left breast had a nice sized red spot a few cm away from the nipple. It did not hurt to press on the red liquidy area but my breast felt engorged and was very swollen. I have never run a fever and my swollen breast hurts deeper but not to the touch. My PCP kinda freaked out on me, called two additional practitioners in to evaluate me with her, for advice, placed me on antibiotics, immediately ordered a diagnostic mamm, ultrasound and MRI. The antibiotics didn't do much but helped the red spot "somewhat" ... but my breast continued to swell. I also began having stinging like pain in my nipple and a dull pain into my armpit. The mamm & ultrasound came out fine only noted "very dense" breast tissue ... as it always says. I kinda figured the density might be better since I had recently gained 40lbs!

But the MRI was not good. It showed areas of high suspicion with "type III washout kinetics" and "interval development of non mass-like enhancement extending from the nipple" BIRADS 4. The MRI biopsy was immediately scheduled as they were very worried about IBC with my BRCA status. The first biopsy attempt failed when they blew a vein in my hand pushing through the contrast but the Radiologist was in a panic because she now wanted to biopsy two areas. I was quickly rescheduled and on the second attempt only one biopsy was performed yet the area was unchanged on imaging. The biopsy area took some time to heal as the skin on my breast is very sensitive due to the swelling. But my doctor put a STAT on the results and they came back: Scelrosis Adenosis, Stromal Necrosis, Chronic inflammation and Periductal Ectasia. My PCP said she was relieved there was "no obvious cancer" but that I still needed a Surgical Oncology consult.

In the meantime the breast stays swollen and painful. Sometimes it aches, sometimes the nipple itches and burns, sometimes it feels like a needle and I have had a small amount of clear discharge that began prior to biopsy and increased after. My PCP wanted to make sure she had eyes on me so she called me in and didn't really like what she saw. She called the oncologist and immediately did a skin punch biopsy to rule out IBC, that thing leaked a lot of clearish fluid, I assume to be lymph fluid for days. The skin punch just came back "inflammation throughout with lymphocytes" but no cancer cells present (they read the report to me over the phone). My surgical oncology appointment is this Friday. My question is: has anyone been diagnosed with BC, not IBC, after a mastitis dx? They have absolutely ruled out infectious mastitis and are now thinking maybe inflammatory mastitis like a plasma cell mastitis or GM. I know something is VERY wrong in this left breast. BTW I am 44 years old, hysterectomy in 2006, have been on low dose HRT since. BRCA + with father, grandmother, her sister and my sister dx with BC. I also inherited my Mama's nice perky but fibro dense breasts, they classify mine as a 3 or 4, but always VERY dense.

Since my hysterectomy in 2006 I have had no breast pain or changes. Now I feel like I have a foreign object for a left breast. I do not know how to explain it but it is just not right. My right breast feels normal "dense" but no pain, while the left feels very heavy and swollen. What should I ask at surgical? ANY advice would be appreciated!!!

YellowGirl

Hello anyone with advice? I'm not sure what to ask at my surgical appt? I have a feeling that they will want to go in and look at a bigger chunk of tissue and I'm actually hoping for that. But if they don't ... what do I ask to make sure there isn't more lurking down there causing this? I am very worried!

Michelle_in_cornland

I would ask for a consult with a dermatologist, as well as onc surgeon. The fact that your breast leaked clear fluid from your punch biopsy, is not remarkable. But, if you feel like your breast is engorged or swollen and leaking clear fluid, could be something else entirely. Do you have any autoimmune disorders that you know of?

So you had:

1. mammogram

2. mri

3. biopsy of tissue

4. biopsy of skin

which all came back negative for cancer cells??

You are having fat (stromal) necrosis, which is basically globs of fat melting into liquid and have gained 40 pounds. The weight gain may have something to do with your breast inflammation. I would also ask for a consult of a registered dietition, as your diet could be a contributing factor. After I delivered my children, I ended up with bad mastitis which I needed an antibiotic to clear up. I used disposable diapers soaked in warm water and microwaved for a few seconds, to apply to the affected breast. Ask if you can use a heating pad // alternating with ice therapy. You may also ask for a referral to a physical therapist who can help you drain the affected area through exercise and other therapy.

YellowGirl

Thank you Michelle for your help! I guess the Radiologist and my PCP are not in accordance with the biopsy results this I just found this out.

Yes, I have had a mammogram and ultrasound, my breasts are very dense and mamms always come up clear but they always do ultrasounds after and they come back always come back fibroglandular tissue, this time it came back some sort of hyperechoic mass? Then MRI showed two poorly differentiated lesions and interval enhancement with type III washout kinetics. There is a lot of stuff going on I guess making it appear as if I have mastitis in there but three rounds of strong antibiotics have not changed things. Honestly my biopsy was botched. It was a circus from the get go. My PCP is still concerned but thankful that they have ruled out IBC. She's not happy this is happening in just one breast. My skin punch was more precautionary but the breast swelling actually buried the stitch. Just had that out today and it opened up again as they tried to remove it. So now have steri-strips and a leak again. They now think that the breast swelling is causing irritation henceforth the redness and irritated areas.

Yes, I have really thought about the weight gain but it has been slow and I have pretty much stayed this weight for over a year ... but you never know. My left breast is three sizes larger as of this morning. I agree, I feel like my diet might be contributing so I am eating very clean right now!

I am on HRT from my 2006 hysterectomy so my hormones are very stable. But this is really making me rethink staying on them. Since my hysterectomy my breasts have been very happy no cyclical soreness etc. This just kinda came out of the blue. I do have some sort of autoimmune thing that they can't seem to figure out. At one point they thought SLE but my numbers dropped after a partial colectomy due to a kinked redundant colon. My markers barely registered after that. It was all inflamed and infected so they took that option out and my body was so happy. It's not happy anymore. My body definitely has a very over active inflammatory response to any issue.

I love the recommendation about the diaper! My last child is 16 but I do have a grandson. The ice really does help a lot so I bet I can use one for ice and one for heat.

I have a surgical oncology appointment this Friday. I think the precancerous cells they found around my cervix in 2006 and my BRCA and family history has them moving fairly quickly to figure this out. It feels like forever. I want to be as prepared as possible when I get to Friday. Thank you so much for your input I REALLY appreciate it!

Michelle_in_cornland

FROM THE NATIONAL INSTITUTE OF HEALTH - NIH

Idiopathic granulomatous mastitis: a diagnostic dilemma for the breast radiologist

Smiti Sripathi,Anurag Ayachit, Archana Bala, Rajagopal Kadavigere, and Sandeep KumarAuthor information ► Article notes ► Copyright and License information ►Go to:

Abstract

Background

Idiopathic granulomatous mastitis is a chronic inflammatory disease of the breast, which is often difficult to differentiate both clinically and radiologically from infectious aetiologies such as tuberculosis, fungal infections, and also from malignancy, thus posing a diagnostic dilemma. We present a pictorial review of the commonly encountered imaging findings in idiopathic granulomatous mastitis on mammography and ultrasound.

Materials and methods

Mammographic and ultrasound findings of histopathologically proven cases of granulomatous mastitis are discussed.

Conclusion

Idiopathic granulomatous mastitis has varied and non-specific appearances on ultrasound and mammography. Histopathology is essential to establish diagnosis.

Teaching Points

• Idiopathic granulomatous mastitis often poses a diagnostic dilemma for the radiologist by mimicking malignancy.

• It has varied and non-specific appearances on mammography and ultrasound.

• Histopathology is mandatory to establish the diagnosis and decide management.

Keywords: Granulomatous, Mastitis, Ultrasound, Mammography, Histopathology

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Introduction

Idiopathic granulomatous mastitis is an uncommon chronic inflammatory condition of the breast of unknown aetiology, seen commonly in women of childbearing age, although peri-menopausal women may also be affected. The clinical and imaging diagnosis of this benign condition is often difficult as it can simulate many conditions including malignancy. Histopathology is essential to solve the dilemma and make a definitive diagnosis, thus avoiding unnecessary mastectomies. Therefore, adequate recognition of its radiological patterns is vital to differentiate it from malignancy.

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Epidemiology

Idiopathic granulomatous mastitis was first described by Kessler and Wolloch in 1972 [1]. Its true prevalence is unknown since it is often a diagnosis of exclusion. In a study by Baslaim et al., histopathologically proven cases of idiopathic granulomatous mastitis were found in 1.8 % of 1,106 women with benign breast disease. Although it is seen globally, a higher racial predilection in Latin and Asian women is known [2]. The diagnostic dilemma is because of its clinical and radiological picture, which is often non-specific and may mimic a malignant mass. The final diagnosis is confirmed by histopathology where there is non-necrotizing granulomatous inflammation of lobules [3]. As a breast radiologist, it is essential to be aware of the imaging features of this rare entity to prevent unnecessary mastectomies.

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Clinical presentation

The most common clinical presentation of this entity is a breast lump that may be of firm to hard consistency. Bilateral involvement is rare. Although the lump may be present in any quadrant, there is a tendency to involve the subareolar region, or there may be diffuse involvement of entire breast. The patient may also present with pain, erythema, swelling, or axillary lymphadenopathy [4] although inflammation may not always be present clinically, thus leading to misdiagnosis as a malignant lesion. Other chronic inflammatory conditions that should be considered in the differential diagnosis include plasma cell mastitis, tuberculosis, histoplasmosis, Wegener's granulomatosis. [5].

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Etiopathogenesis

The exact aetiology is unknown and is controversial; however, response to steroids points towards an autoimmune origin and is the most widely accepted theory [6]. The association with lactation (up to 9 months after delivery) is explained by the extravasated lactational secretions (due to local trauma or infection) damaging the ductal epithelium and leading to a granulomatous inflammatory response [4]. Oral contraceptives can cause a chemically induced granulomatous reaction [7].

Duct ectasia, periductal mastitis complex: Non-puerperal mastitis may be seen in patients with underlying duct ectasia or cysts where chemical inflammation is produced because of cyst or duct rupture [8]. Later bacterial infection may also occur. In duct ectasia there is weakening of duct wall due to stasis of fatty inflammatory secretions, ductal dilatation, and duct wall rupture, leading to periductal chemical mastitis. Further necrosis and infection may lead to abscess formation, especially in peri-areolar region.

Inflammation and rupture of cysts can also cause focal chemical mastitis and abscess formation.

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Radiological features

Mammography

Routine cranio-caudal (CC) and mediolateral oblique (MLO) views are obtained. Additional views such as spot compression and magnification views are also carried out as and when required.

Focal asymmetric density (Figs. 1, ,2,2, and and3)3) is the most common mammographic pattern seen in idiopathic granulomatous mastitis, according to Yilmaz et al. [9] and Memis et al. [10]. Diffuse unilateral increase in breast density, more often seen in malignancies, may also be encountered. Mammograms of dense breasts may be reported negative since the findings cannot be appreciated well.

Fig. 1

A 38-year-old woman presented with right breast lump of 3-week duration. Mammogram (cranio-caudal view) of the right breast shows an asymmetric opacity (arrow). Histopathology was s/o idiopathic granulomatous mastitis

Fig. 2

Mammogram (MLO view) of left breast of a 35-year-old woman presenting with a tender lump in left breast of 1-week duration revealed retraction of the left nipple (small arrow) with increased density in the retroareolar region (large arrow). A few benign ...

Fig. 3

Mammogram (CC view) of a 30-year-old woman who presented with painless lump in the right breast of 20-day duration showed an ill-defined dense irregular opacity (arrow) with architectural distortion involving outer quadrant of right breast. A possibility ...

Han et al. [11] reported no visible changes in the skin or nipple areolar region in granulomatous mastitis; however, in a study by Lee et al. [12] overlying inflammatory skin thickening (Fig. 4) was demonstrated in 63.6 % of mammograms.

Fig. 4

Mammogram (MLO view) of right breast in a 42-year-old woman presenting with progressively increasing lump in the right breast showed an ill-defined asymmetric opacity in the retroareolar region of the right breast (arrow) and right areolar skin thickening, ...

Lee et al. [12] also described other associated features, including parenchymal distortion (Fig. 5), skin thickening, and benign appearing axillary lymph nodes with maintained fatty hila in 63.7, 63.7, and 54.5 % of subjects respectively.

Fig. 5

A 48-year-old woman presented with a tender lump of 2-week duration. There was no history of fever. Mammogram (cranio-caudal view) of right breast revealed increased density in retroareolar region (arrow) with overlying skin thickening (arrowhead). Histopathology...

Bilateral involvement is occasionally seen (Fig. 6a, b, c, d).

Fig. 6

A middle-aged woman presented with swelling in the left breast associated with nipple discharge. (a, b) Mammogram (CC view) shows asymmetric breast density with skin thickening in nipple areolar region of the left breast. Histopathology was s/o. Granulomatous ...

Ultrasound imaging

Ultrasound is done using a high frequency (7-10 MHz) linear probe and appearances of this entity are varied. Generally, a hypoechoic or heterogeneous mass(es) is noted, with characteristic tubular hypoechoic extensions connecting the dominant mass to smaller nearby masses (Fig. 7) [13]. Larsen et al., in their study of 54 cases, found an irregular hypoechoic lesion with tubular extension as the most frequent finding [14] and an isolated ill-defined hypoechoic or heterogeneous lesion (Figs. 8 and and9)9) as the second most common finding. When a mass is detected, colour Doppler examination may be done to assess vascularity.

Fig. 7

Ultrasound of the left breast in a 28-year-old woman who presented with breast lump and pain showed a few ill-defined hypoechoic lesions containing internal echoes (arrows) communicating with each other by tubular hypoechoic extensions (arrowhead). Biopsy ...

Fig. 8

Ultrasound of right breast in a 33-year-old woman presenting with hard painless lump of 3 months duration shows a well-defined hypoechoic lesion with irregular margins. Ultrasound guided biopsy was done and histopathology showed granulomatous ...

Fig. 9

Ultrasound of left breast in a 35-year-old woman presenting with a left breast lump of 1-month duration shows a small ill-defined heterogeneously hypoechoic lesion with central echogenic areas, which was confirmed to be granulomatous mastitis on histopathology...

Parenchymal heterogeneity and distortion with or without acoustic shadowing may also be seen with absence of a definite mass.

Sometimes a well-defined collection with low level mobile internal echoes may be present with tubular hypoechoic extension to the subcutaneous tissues and skin (Fig. 10). Associated changes such as overlying skin thickening and nipple retraction can also be seen in a few cases (Fig. 11).

Fig. 10

Ultrasound of the right breast from the same patient mentioned in Fig. 1 shows an ill-defined heterogenous lesion with increased vascularity (arrow) and tubular extension (arrowhead). Biopsy was done and histopathology was suggestive of granulomatous ...

Fig. 11

A 32-year-old woman presented with a lump in the left breast of 6-week duration with no history of associated fever. Ultrasound of the left breast showed an ill-defined heterogeneously hypoechoic collection/lesion (arrow) with overlying skin thickening ...

In a study by Kiyak et al. [15], parenchymal heterogeneity, irregular hypoechoic mass, and abscess formation were the most common findings. They opined that parenchymal heterogeneity with abscess formation and axillary lymphadenopathy favor an inflammatory process though histopathology is still essential to establish the diagnosis.

Lee et al. [12] found other synchronous findings such as subcutaneous fat obliteration in almost all cases and skin thickening in 91.7 % cases. Increased surrounding parenchymal vascularity on Doppler ultrasound has also been reported [16].

The real dilemma in diagnosis

Since the mammographic and ultrasound findings of idiopathic granulomatous mastitis are nonspecific and may often mimic carcinoma, a definitive histopathological diagnosis is essential before contemplating any surgical procedure.

When there is diffuse involvement of the breast, it is imperative to distinguish idiopathic granulomatous mastitis from inflammatory carcinoma. The most common mammographic finding in the latter is irregular densities with architectural distortion, skin thickening, trabecular prominence due to oedema, and nipple retraction (Fig. 12). Similar mammographic findings may be seen with idiopathic inflammatory mastitis (Fig. 13a and b), which can be confirmed on ultrasound (Fig. 13c, d).

Fig. 12

Mammogram (MLO view) of the right breast of a 48-year-old woman presenting with a lump in right breast of 1-month duration shows diffusely increased density in central glandular component (thick arrow) extending to the retromammary space with trabecular ...

Fig. 13

A 42-year-old female presented with swelling and lump in the left breast of 1-month duration. Mammogram (a, b) showed focal asymmetric density in the lower inner quadrant of the left breast. Ultrasound (c) showed a well-defined heterogeneously hypoechoic ...

However, the ultrasound feature in favour of benignity is the presence of an ill-defined mass with long axis of lesion parallel to the chest wall. In addition, multifocal abscess cavities, as seen in 28 % cases in a study by Seo et al. [17], may favour a diagnosis of mastitis over malignancy.

Tubercular mastitis, like idiopathic granulomatous mastitis, is also an inflammatory pathology occurring in young parous women and presents with increased breast density with diffuse skin thickening; however, their management differs completely. Seo et al. [17] found that patients with idiopathic granulomatous mastitis were younger, with a mean age at presentation of 33.5 years, as opposed to 40 years in the case of tuberculous mastitis. Also, a higher proportion of patients with idiopathic granulomatous mastitis presented with acute onset mastalgia. The occurrence of axillary lymphadenopathy was more common in tubercular mastitis than idiopathic granulomatous mastitis with an incidence of 50 and 20.6 %, respectively.

On ultrasound, tubercular mastitis has similar imaging features as idiopathic granulomatous mastitis and causes a diagnostic dilemma. It may be seen as a well-defined hypoechoic collection/abscess formation with low level internal echoes or an ill-defined hypoechoic mass with surrounding inflammation. Histopathogical and microbiological confirmation is required to make a definitive diagnosis of tubercular mastitis.

Duct ectasia, periductal mastitis complex, as described earlier, is a result of duct rupture with inflammation in periductal tissues, which may later become infected and cause an abscess formation.

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Role of MRI

Rieber et al. [18] found that MRI did not provide any additional information that was critical in differentiating idiopathic granulomatous mastitis from inflammatory carcinoma, since both exhibit signs of inflammation. In a study by de Bazelaire et al. [19], dynamic contrast MR imaging did not prove to be really discriminatory either, as intense early enhancement (>100 % before 90 s) was found in the majority of cases of inflammatory breast carcinoma and almost half of the patients with mastitis, the enhancement kinetics of both conditions being analogous produced by vascular endothelial growth factor (VEGF). At best, MRI may play a complementary role to increase conspicuity of lesions that are not visualised by mammograms and ultrasound adequately.

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Histopathological evaluation

Fine needle aspiration cytology (FNAC) may show polymorphonuclear leucocytes, giant cells, and epitheloid cells (Fig. 14). Histopathology shows chronic lobulitis with non-caseating granulomatous inflammation. In a study of 19 cases by Gangopadhyay et al. [20], caseous necrosis was absent and giant cells were morphologically of foreign body type and Langerhans's type.

Fig. 14

Granulomatous mastitis: 4x magnification showing a forming granuloma (thin arrow) in the background of diffuse lympho-plasmacytic infiltration (thick arrow) of breast parenchyma with scattered giant cells (inset, 40x)

The single most important differential diagnosis of granulomatous mastitis in the Asian sub-continent is tuberculosis. Predominance of neutrophils in the background and relative absence of caseous necrosis favour a diagnosis of granulomatous mastitis. Sometimes these granulomas become confluent and lead to suppuration and liquefactive necrosis. Biopsy should be performed to establish diagnosis before deciding further treatment options.

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Management

The treatment of idiopathic granulomatous mastitis remains controversial, with options ranging from conservative management with antibiotics to wide local excision (WLE) and corticosteroid therapy. Treatment must be tailored to the patient's clinical presentation. Lai et al. found that spontaneous resolution occurs in 50 % cases in a mean time interval of 14.5 months without any treatment [21]. Kiyak et al. reported that WLE was not the ideal treatment in complicated granulomatous mastitis with abscess formation, fistulas, and diffuse involvement of breast. Most of these patients recovered spontaneously over a mean interval of 5.1 months. They suggested that a short interval follow-up should be done before deciding on steroid treatment.

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Conclusion

1. Idiopathic granulomatous mastitis is a benign entity which has varied and nonspecific appearances on ultrasound and mammography and often mimics malignancy.
2. Mammogram commonly shows focal asymmetric density and skin thickening while parenchymal heterogeneity, irregular mass, and hypoechoic mass with tubular extension are seen on ultrasound.
3. It often mimics breast carcinoma clinically and radiologically, hence histopathological evaluation is necessary to establish diagnosis before deciding upon treatment options.

sbelizabeth

Yellowgirl, just my $0.02 worth...being BRCA + with a long family history of breast cancer--

Consider (strongly consider) getting off the HRT. Low dose or not, exposing your cells to artificial hormones raises your BC risk.

Have you considered prophylactic mastectomies?

YellowGirl

Thank you Michelle that is very helpful and seems like what they are thinking I've got here! Thank you so much for doing that research!!! Huge help!

sbelizabeth I am hearing you. Since I was so young we started a low dose after hysterectomy but it was not to be long term. I've been a bit scared to go off the hormones but I am now not feeling so scared, it's going to happen. My PCP has been kinda nudging me towards the prophylactic mastectomy with all that you mention and add it the super dense breasts. I didn't want to lose my nipples but I'm also losing that fear now. I think I might end up having to go that route. I've been kinda waiting for technology to get better. My screenings have always been very easy and MRI's uneventful ... until now. I'd like the DIEP flap if I go that route. Do you favor that? I definitely have a lower pooch to help reconstruct!

sbelizabeth

I had a DIEP in 2013 and it was a good decision for me. With my diagnosis I wasn't a candidate for nipple-sparing, immediate reconstruction, which can be done with either DIEP or implants, but you probably would be. You need to speak with a very experienced plastic surgeon.

Again my $0.02 worth...don't wait. My Stage III BC sneaked up on me fast and hard, and I partly blame the HRT I was on. I wish I'd had the choices you have right now. Deciding against prophylactic mastectomies would be a fine decision, but it should be a well-considered, informed decision.