Article

Idiopathic granulomatous mastitis masquerading as carcinoma of the breast: A case report and review of the literature

Dept. of Radiation Oncology, Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA.
International Seminars in Surgical Oncology 02/2007; 4(4):21. DOI: 10.1186/1477-7800-4-21
Source: PubMed

ABSTRACT

Idiopathic granulomatous mastitis is an uncommon, benign entity with a diagnosis of exclusion. The typical clinical presentation of idiopathic granulomatous mastitis often mimics infection or malignancy. As a result, histopathological confirmation of idiopathic granulomatous mastitis combined with exclusion of infection, malignancy and other causes of granulomatous disease is absolutely necessary.
We present a case of a young woman with idiopathic granulomatous mastitis, initially mistaken for mastitis as well as breast carcinoma, and successfully treated with a course of corticosteroids.
There is no clear clinical consensus regarding the ideal therapeutic management of idiopathic granulomatous mastitis. Treatment options include expectant management with spontaneous remission, corticosteroid therapy, immunosuppressive agents and extensive surgery for refractory cases.

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International Seminars in Surgical
Oncology
Open Access
Case report
Idiopathic granulomatous mastitis masquerading as carcinoma of
the breast: a case report and review of the literature
Richard Tuli
1
, Brian J O'Hara
2
, Janet Hines
3
and Anne L Rosenberg*
2
Address:
1
Dept. of Radiation Oncology, Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA,
2
Dept. of Surgery, Thomas
Jefferson University Hospital, Philadelphia, PA 19107, USA and
3
Infectious Diseases Division, Dept. of Medicine, Hospital of the University of
Pennsylvania, Philadelphia, PA 19104, USA
Email: Richard Tuli - rtuli1@jhmi.edu; Brian J O'Hara - brian.o'hara@jefferson.edu; Janet Hines - jhines@mail.med.upenn.edu;
Anne L Rosenberg* - raf0608@hotmail.com
* Corresponding author
Abstract
Background: Idiopathic granulomatous mastitis is an uncommon, benign entity with a diagnosis of
exclusion. The typical clinical presentation of idiopathic granulomatous mastitis often mimics
infection or malignancy. As a result, histopathological confirmation of idiopathic granulomatous
mastitis combined with exclusion of infection, malignancy and other causes of granulomatous
disease is absolutely necessary.
Case Presentation: We present a case of a young woman with idiopathic granulomatous
mastitis, initially mistaken for mastitis as well as breast carcinoma, and successfully treated with a
course of corticosteroids.
Conclusion: There is no clear clinical consensus regarding the ideal therapeutic management of
idiopathic granulomatous mastitis. Treatment options include expectant management with
spontaneous remission, corticosteroid therapy, immunosuppressive agents and extensive surgery
for refractory cases.
Background
Originally described by Kessler and Wolloch in 1972 [1],
idiopathic granulomatous mastitis (IGM) is a rare disease
of unknown etiology, which often mimics infection or
malignancy and remains a diagnosis of exclusion. As a
granulomatous form of lobular mastitis, IGM may be dif-
ferentiated from granulomatous forms of periductal mas-
titis, as well as from granulomatous mastitis caused by
sarcoidosis, Wegener's granulomatosis, giant cell arteritis,
polyarteritis nodosum, foreign body reaction, and tuber-
culous, syphilitic, parasitic and mycotic infections [2,3].
Herein, we present a case of a young woman with IGM,
initially mistaken for mastitis as well as breast carcinoma,
and successfully treated with a course of corticosteroids.
Case presentation
A 36 year-old pre-menopausal Asian woman previously in
excellent health, presented with a three-month history of
a progressively worsening tender right breast lump, with
associated ulceration, induration, and erythema, draining
serosanguinous fluid. She denied systemic symptoms, as
well as any known recent fungal or tuberculosis exposure.
The patient is a G2 P2002 who nursed her children with-
out difficulty. She has had no history of breast disease,
and her past medical and surgical histories are otherwise
Published: 27 July 2007
International Seminars in Surgical Oncology 2007, 4:21 doi:10.1186/1477-7800-4-21
Received: 23 June 2007
Accepted: 27 July 2007
This article is available from: http://www.issoonline.com/content/4/1/21
© 2007 Tuli et al; licensee BioMed Central Ltd.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0
),
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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unremarkable. She denied tobacco use, any family history
of breast diseases, use of any medications including oral
contraceptives, or any known drug allergies. Physical
examination was otherwise unremarkable with no palpa-
ble lymphadenopathy.
During initial presentation to an outside hospital, mam-
mography and breast ultrasound revealed an increased
density and ductal dilatation of the right breast with asso-
ciated edematous changes. All laboratory and other radio-
logical studies were otherwise normal. Although
malignancy had not been excluded, the patient received a
presumptive diagnosis of mastitis and was treated with a
7-day course of amoxicillin and cefadroxil without
improvement. This was followed by a course of cefdinir
and gatifloxacin, which was complicated by an antibiotic-
induced erythema nodosum. As a result, antibiotics were
discontinued and the patient was scheduled for an open
breast biopsy. Interestingly, the patient's symptoms spon-
taneously resolved before tissue sampling was performed.
Two months after her initial presentation, a second simi-
lar lump appeared in the right breast (Figure 1). A repeat
ultrasound of this area again demonstrated dilated ducts
and debris. Bilateral breast magnetic resonance imaging
(MRI) revealed regionally dilated ducts with prominent
regional ductal and parenchymal plateau enhancement.
Given these findings were not typical for mastitis, and
clinical and radiological data could not exclude breast car-
cinoma, tissue sampling was performed. Microscopic
examination revealed chronic inflammation and macro-
phage, giant histiocyte and epithelioid-like cellular infil-
tration, with cytologic features suggestive of a
granulomatous process (Figure 2). Further histopatholog-
ical analysis showed no evidence of carcinoma or abscess
formation, and all cultures and stains for infectious organ-
isms remained negative. Secondary to exclusion of malig-
nancy, infection, and other causes, the patient was given
the presumptive diagnosis of idiopathic granulomatous
mastitis (IGM). She was treated with a 6-month tapered
course of prednisone with an excellent response, and has
since remained free from recurrence.
Discussion
Although the exact etiology of IGM remains unclear, asso-
ciations with autoimmune disorders, oral contraceptive
use, pregnancy, hyperprolactinemia and alpha-1-antit-
rypsin deficiency have been suggested [2,4-6]. Most stud-
ies report an average age of presentation in the third
decade of life (range 11 to 83 years) with symptoms often
developing within a few years of pregnancy [3]. Moreover,
conflicting data exists regarding the significance of oral
contraceptive use in patients diagnosed with IGM, with
percentages of patients using contraception ranging from
0% to 33% [2,7,8]. However, no true associations with
pregnancy, breast-feeding, prolactin levels, or oral contra-
ceptive use have been established to date.
Interestingly, the majority of published reports of IGM
have come from outside the U.S [9-13]. Whether this dis-
proportional lack of published reports from the U.S. rep-
resents a lower prevalence of IGM, under diagnosis in the
U.S., over diagnosis in developing countries, or a combi-
Photomicrograph of hematoxylin and eosin specimen from (1)Figure 2
Photomicrograph of hematoxylin and eosin specimen from
(1). Arrows indicate granulomatous inflammation centered
on breast lobules, while arrowhead indicates the presence of
multinucleated giant cells within non-caseating granuloma-
tous inflammation (magnification 100×; bar = 150 µm).
A patient with IGM of the right breast status-post incisional breast biopsyFigure 1
A patient with IGM of the right breast status-post incisional
breast biopsy. Clinical presentation of IGM may mimic com-
mon entities such as breast mastitis, as well as more involved
diagnoses such as malignancy.
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nation of the above remains to be addressed, yet under-
scores the necessity of appropriate diagnosis and
treatment of IGM. Most frequently, the primary clinical
finding is a unilateral firm breast mass affecting any quad-
rant of the breast, which may be tender in 25% of cases
and present bilaterally in 25% of cases [2,3,14]. Patients
with a more chronic presentation may go on to develop
fistulae, abscesses, nipple inversion, and skin inflamma-
tion and ulceration over the course of several years [15].
In a recent study, Lai et al. reported that 100% of women
with a histopathologically confirmed diagnosis of IGM
initially presented with palpable breast masses [12].
Moreover, approximately 56% of women within this
study were initially suspected to have breast carcinoma
[12]. Given this initial presentation, combined with mam-
mography, ultrasound and fine needle aspiration biopsy
results that often mimic malignancy, several studies have
documented the use of unnecessary mastectomies in
patients with IGM [2,4,5]. Clearly, this underscores the
importance of thorough histopathological analyses in
patients with suspected breast carcinoma. A recent study
assessed the potential utility of dynamic contrast-
enhanced MRI in diagnosing IGM. Using patients with a
histopathologically confirmed diagnosis of IGM,
Kocaoglu et al. found varied appearances on MR and were
unable to identify any imaging features characteristic of
the disease, thereby suggesting its limited diagnostic util-
ity [16].
The predominant and characteristic histopathological fea-
ture of IGM, granuloma formation, is also commonly
found in other entities, thereby rendering it a diagnosis of
exclusion. The lobular distribution of IGM represents a
mixed chronic inflammatory process composed of lym-
phocytes, plasma cells, epithelioid histiocytes, multinu-
cleated giant cells and less frequently, neutrophils [13,17].
However, confirmatory diagnosis is obtained only
through identification of granulomatous inflammation
centered on lobules (granulomatous lobulitis) with an
absence of caseating necrosis. In more severe cases, con-
fluency of the granulomatous inflammation may obliter-
ate its typical lobulocentric distribution, thereby further
complicating the diagnosis. Additionally, microabscess
formation may occasionally involve the entire lobule, and
squamous metaplasia of lobular and ductal epithelium
may occur [3,18,19]. Although IGM patients rarely
present with systemic signs of infection, culture speci-
mens must nevertheless be analyzed for subtle microor-
ganisms.
Likely secondary to the lack of published reports, particu-
larly from the U.S., there is no clear clinical consensus
regarding the ideal therapeutic management of IGM.
Although several studies have reported varying
approaches to the treatment of IGM, many of these treat-
ment algorithms were formulated without a definitive ini-
tial diagnosis. Histopathological confirmation of IGM
combined with exclusion of malignancy and other causes
of granulomatous disease is of utmost importance in
guiding clinical decision making and preventing inappro-
priate and unnecessary treatments. Therefore, following
careful confirmation of diagnosis, the initial treatment for
IGM clearly should be non-operative. Indeed, in a recent
study, 50% of patients receiving expectant management
had spontaneous and complete resolution of disease fol-
lowing a mean of 14.5 months [12]. An initial treatment
option for patients with new-onset IGM with mild to
moderate symptoms may be expectant management with
close regular surveillance. For patients with clinically
advanced disease or more severe symptoms, in whom
infectious etiologies have been excluded, oral daily pred-
nisone, starting with 0.8 mg/kg/day and tapering with
clinical improvement, is a common regimen. Unfortu-
nately, aside from the well-established side-effects of cor-
ticosteroid therapy, patients often relapse with cessation
of therapy with one study reporting recurrence rates as
high as 50% [11]. In cases of recurrent disease or those
refractory to the above therapies, immunosuppressive
agents, like methotrexate or azothioprine, have been uti-
lized with variable responses [9,20], but minimal clinical
evidence exists. Finally, surgical options should be
explored in refractory cases or those with persistent collec-
tions. A retrospective study by Erhan et al. reviewed 18
women with clinicopathologically confirmed IGM treated
with excisional biopsy [14]. According to the authors,
recurrence was seen in only 3 women, 2 of whom were
found to have hyperprolactinemia that was successfully
treated with repeat excision and anti-prolactinemic ther-
apy without subsequent recurrence. In cases with persist-
ent abscess and fistula, wide surgical excision and even
mastectomy may be indicated [2,10].
Conclusion
IGM is a rare and benign inflammatory process com-
monly mistaken for malignancy and other disease enti-
ties. As a result, it is often incorrectly treated. It typically
occurs in women during the third decade of life, present-
ing as an inflammatory palpable mass without systemic
symptoms. Correct diagnosis requires the exclusion of
infectious etiologies, other causes of granulomatous mas-
titis, and malignancy, combined with definitive his-
topathological confirmation. Treatment should initially
be non-operative and depending upon the severity of ini-
tial presentation, may range from expectant management
to corticosteroid therapy. In refractory cases, immunosup-
pressive agents and surgical excision may be indicated.
Competing interests
The author(s) declare that they have no competing inter-
ests.
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Authors' contributions
RT performed chart review, literature search, and wrote
and prepared the manuscript. BJO carried out histopatho-
logical analyses and review of the manuscript. JH treated
the patient and reviewed the manuscript. ALR was the
patient's surgeon and helped write and edit the manu-
script. All authors have read and approved the final man-
uscript.
Acknowledgements
AR has personally spoken to the patient and obtained consent to present
the case and included photographs.
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  • Source
    • "In our series, the disease was commonly unilateral (87 patients unilateral, 6 patients bilateral), and the right and left breasts were affected equally (40 cases in the right breast and 47 cases in the left breast). Idiopathic GM is more frequent in premenopausal women, but can be seen in different ages, such as an 11-year-old girl [5, 25] as well as the cases in the 6 th , 7 th and 8 th decades [25, 26]. The majority of our patients were of childbearing age, with a mean age of 34.4 years, and most of them were multiparous and had breastfed their children. "
    [Show abstract] [Hide abstract] ABSTRACT: Objective: In this study we aimed to define clinical, radiologic and pathological specialties of patients who applied to General Surgery Department of Atatürk University Medical Faculty with granulomatous mastitis and show medical and surgical treatment results. With the help of this study we will be able to make our own clinical algorithm for diagnosis and treatment. Materials and methods: We searched retrospectively addresses, phone numbers and clinical files of 93 patients whom diagnosed granulomatous mastitis between a decade of January 2001 - December 2010. We noted demographic specialties, ages, gender, medical family history, main complaints, physical findings, radiological and laboratory findings, medical treatments, postoperative complications and surgical procedures if they were operated; morbidity, recurrence and success ratios, complications after treatment for patients discussed above. Results: In this study we evaluated 93 patients, 91 females and 2 males, with granulomatous mastitis retrospectively who applied to General Surgery Department of Atatürk University Medical Faculty between January 2001 and December 2010. Mean age was 34.4 years. The diagnosis was confirmed by histopathologic examination of the lesions. Seventy three patients had idiopathic granulomatous lobular mastitis and 20 patients had specific granulomatous mastitis IGM (18 tuberculosis mastitis, 1 alveolar echinococcosis and 1 silk reaction). All the patients had surgical debridement or antibiotic, and anti-inflammatory treatment with results bad clinical response before applied our clinic. Conclusion: Empiric antibiotic therapy and drainage of the breast lesions are not enough for complete remission of idiopathic granulomatous mastitis. The lesion must be excised completely. In selected patients, corticosteroid therapy can be useful. In the patients with tuberculous mastitis, abscess drainage and antituberculous therapy can be useful, but wide excision must be chosen for the patients with recurrent disease.
    Full-text · Article · Nov 2015 · Eurasian Journal of Medicine
  • Source
    • "Known etiologies of CM are diverse and include diabetes, lupus erythematosus, Mycobacterium tuberculosis, Staphylococcus aureus, and Candida albicans, as well as several species of Corynebacterium and other rare syndromes and infections [3] [4] [5]. Several other suspected predisposing or related diseases have been mentioned in the literature, including Weber- Christian disease, Sjogren's syndrome, hyperprolactinemia, IgG4 sclerosing disease, immune response to local trauma, and even cat scratch disease [6] [7] [8] [9] [10]. "
    [Show abstract] [Hide abstract] ABSTRACT: Chronic mastitis is a prolonged inflammatory breast disease, and little is known about its etiology. We identified 85 cases and 112 controls from 5 hospitals in Morocco and Egypt. Cases were women with chronic mastitis (including periductal, lobular, granulomatous, lymphocytic, and duct ectasia with mastitis). Controls had benign breast disease, including fibroadenoma, benign phyllodes, and adenosis. Both groups were identified from histopathologically diagnosed patients from 2008 to 2011, frequency-matched on age. Patient interviews elicited demographic, reproductive, breastfeeding, and clinical histories. Cases had higher parity than controls (OR = 1.75, 1.62-1.90) and more reported history of contraception use (OR = 2.73, 2.07-3.61). Cases were less likely to report wearing a bra (OR = 0.56, 0.47-0.67) and less often used both breasts for breastfeeding (OR = 4.40, 3.39-5.72). Chronic mastitis cases were significantly less likely to be employed outside home (OR = 0.71, 0.60-0.84) and more likely to report mice in their households (OR = 1.63, 1.36-1.97). This is the largest case-control study reported to date on risk factors for chronic mastitis. Our study highlights distinct reproductive risk factors for the disease. Future studies should further explore these factors and the possible immunological and susceptibility predisposing conditions.
    Full-text · Article · Nov 2013
  • Source
    • "The primary presentation is a firm breast mass, frequently associated with local pain and eventually skin ulcerations, abscesses and fistulae develop [3,5]. The clinical presentation frequently mimics breast abscess, infective mastitis and breast cancer [6]. Histopathological study is required to make the diagnosis, and findings include noncaseating granulomatous lobulitis making it imperative to rule out other granulomatous diseases. "
    [Show abstract] [Hide abstract] ABSTRACT: Background Idiopathic granulomatous mastitis is a rare, benign, inflammatory chronic condition of unclear etiology. This case is reported because it illustrates how idiopathic granulomatous mastitis can mimic other diseases, making it difficult to associate the presenting symptoms and the correct diagnosis; This disease is a challenge for clinicians to diagnose, manage and avoid iatrogenic complications, and requires consultation with experts in several specialties. Case presentation The patient was 30 years old, South-American, eleven weeks pregnant, and with an apparent infectious mastitis. She presented with progressive worsening of her breast symptoms and multiple negative laboratory tests. She suffered different side effects from several prescribed treatments and endured a prolonged recovery. The article emphasizes the need for ruling out common pathologies to arrive at the correct diagnosis such as bacterial and fungal infections; granulomatous conditions like tuberculosis and sarcoidosis; and inflammatory breast carcinoma. It also describes frequently used pharmacological and supplementary forms of treatment for patients with this condition. Conclusion Idiopathic granulomatous mastitis is a rare unusual condition of unknown etiology. Pathological confirmation is required for its diagnosis and optimal management is still unclear. The presentation and management of this case is intended to advance its awareness to physicians from different specialties.
    Full-text · Article · Mar 2013 · BMC Research Notes
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