Clinical and imaging features of idiopathic granulomatous mastitis - The diagnostic challenges and a brief review

doi:10.1016/j.clinimag.2020.06.022

Clinical Imaging

Volume 69, January 2021, Pages 126-132

https://doi.org/10.1016/j.clinimag.2020.06.022 Get rights and content

Highlights

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We present a series of three cases of a rare entity: Idiopathic Granulomatous Mastitis, one of the subgroups of granulomatous mastitis.
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Importance of awareness of IGM: characteristic and more frequent occurrence in younger women, especially pregnant or lactating women.
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The clinical and radiological presentations often mimic inflammatory carcinoma, further highlighting the need to increase awareness of IGM.
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Moreover, since imaging evaluation is the primary investigative approach, the importance of our report is self-explanatory.
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The imaging literature on IGM is thus far, not so vast. We have presented and discussed the role of multimodality imaging of this rare entity.

Abstract

Idiopathic granulomatous mastitis (IGM), or granulomatous lobular mastitis is a rare, benign, inflammatory condition of the breast, without an identifiable underlying etiology. The clinical and imaging diagnosis of this entity is challenging with the presentation frequently mimicking inflammatory breast carcinoma (IBC). Mammography and breast ultrasound (US) have an important role in its detection, however, biopsy is imperative for histopathological confirmation. We present three cases of biopsy proven IGM, highlighting the variability of the clinical and imaging features,brieflyreview the relevant literature and discuss the challenges associated with its diagnosis and management.

Introduction

Granulomatous mastitis (GM), or granulomatous lobular mastitis (GLM) is a rare, benign, inflammatory condition of the breast characterized by non-necrotizing granulomatous inflammation in lobules of breast tissue and breast parenchyma. The granulomas may be associated with neutrophilic micro-abscesses on histopathology [[1], [2], [3]]. GM has two distinct forms: specific GM (SGM) and Idiopathic GM (IGM) [[4], [5]]. SGM, is a rare presentation of an underlying systemic inflammatory disease like Wegener's granulomatosis, sarcoidosis or due to chronic granulomatous infections like tuberculosis (TB), brucellosis, histoplasmosis, syphilis and fungal infections, cat scratch disease, foreign body reaction from a leaking breast implant, or due to Corynebacterial infections. However, when no underlying etiology or association is identified, it is referred to as Idiopathic Granulomatous mastitis (IGM). The exclusion of infections and other etiology like Wegener's and sarcoidosis necessitates meticulous microbiological and histopathology analysis for a conclusive diagnosis [[4], [5], [6], [7]]. IGM, which was first described by Kessler and Wolloch in 1972, usually occurs in women of child bearing age and more frequently in post-partum and breast-feeding mothers. [[1], [2], [3], [4], [5], [6], [7]]. The disease has a characteristic clinical course, with frequent recurrences and sometimes an eventual burn out in a few cases [3]. The condition most often clinically and radiologically masquerades as an inflammatory breast carcinoma (IBC), rendering it a diagnostic challenge [[2], [3], [4], [5], [6], [7], [8]]. We report a series of three cases of biopsy proven IGM, highlighting the variability of the clinical and imaging features, briefly review the relevant literature and discuss the challenges associated with its diagnosis and management.

Section snippets

Patient no 1

A 28-year-old female presented to the hospital after an accidental intravenous heroin overdose. The drug overdose was non-fatal and during her hospital course, she complained of a painful right breast lump. On physical examination, there was erythema and thickening of the peri-areolar skin of the right breast, without any sinus or fistula tracts. There was no reported history of injectable drug abuse at the site of the right breast abnormality. On palpation, there was an ill-defined, but firm

Discussion

SGM and IGM are both rare, with few case series and case reports in the literature [,[1], [3], [5],]. The exact incidence of the disease remains unknown [1,5]. The usual manifestation of disease is unilateral with bilateral involvement being unusual [[4], [5], [6], [7]]. The entity often proves to be a diagnostic challenge to clinicians, radiologists and cytologists alike, due to its close simulation of IBC; thereby necessitating a histopathological examination for definitive diagnosis [[4], [5]

Conclusion

In conclusion, IGM, although rare, deserves increased awareness amongst both clinicians and breast Radiologists, as it closely mimics inflammatory carcinoma. The entity should be considered in the differential diagnosis when a breast mass with typical clinical and imaging findings is observed in young pregnant or post-partum women. The characteristic features are hypoechoic mass with tubular ramifications on ultrasound and an asymmetrical increased density on mammography. MRI is helpful in

Grant support

None.

Declaration of competing interest

Authors declare no conflict of Interest.

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Idiopathic granulomatous mastitis: manifestations at multimodality imaging and pitfalls
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Granulomatous mastitis: a ten-year experience at a University Hospital
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Imaging findings in patients with granulomatous mastitis
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Idiopathic granulomatous mastitis associated with risperidone-induced hyperprolactinemia
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Idiopathic granulomatous mastitis: imaging update and review
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Idiopathic granulomatous mastitis: dilemmas in diagnosis and treatment
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Cited by (13)

Multidisciplinary consensus on granulomatous mastitis: Diagnostic and treatment algorithm
2024, Clinica e Investigacion en Ginecologia y Obstetricia
La mastitis granulomatosa idiopática (MGI) es una enfermedad inflamatoria crónica benigna que afecta al estroma mamario. El diagnóstico es por exclusión y requiere confirmación histopatológica, ya que su presentación clínica y radiológica puede simular un carcinoma de mama. El tratamiento es principalmente médico, complementado con drenaje percutáneo y en última instancia quirúrgico.
Aunque se trata de una enfermedad benigna, puede provocar un importante deterioro de la calidad de vida. Debido a su naturaleza, requiere un enfoque multidisciplinar. Es necesario protocolizar el manejo de esta entidad tanto para establecer el diagnóstico como para iniciar el tratamiento adecuado de forma precoz y evitar su evolución a fases avanzadas.
El objetivo de este trabajo es estandarizar el diagnóstico y tratamiento de la MGI desde un abordaje multidisciplinar tras consenso médico. Se describe el algoritmo diagnóstico teniendo en cuenta factores de riesgo de MGI, signos radiológicos típicos para poder realizar una detección precoz ante casos de sospecha y asegurar posteriormente un tratamiento precoz que se describe en el siguiente algoritmo referente al tratamiento. También se hace referencia a un circuito de seguimiento concreto.
Se proponen estos algoritmos diagnóstico-terapéuticos multidisciplinares para dar a conocer y estandarizar el abordaje de esta enfermedad poco frecuente, acelerando su diagnóstico y optimizando su tratamiento.
Idiopathic granulomatous mastitis (IGM) is a benign chronic inflammatory disease affecting the breast stroma. Diagnosis is by exclusion and requires histopathological confirmation, as its clinical and radiological presentation may be similar to breast carcinoma. Treatment is mainly medical, complemented by percutaneous drainage and ultimately surgical.
Although it is a benign pathology, it can cause significant impairment of quality of life. Due to its nature, it requires a multidisciplinary approach. It is necessary to protocolise the management of this entity both to establish the diagnosis and to initiate appropriate treatment early and prevent progression to advanced stages.
The aim of this work is to standardise the diagnosis and treatment of IGM from a multidisciplinary approach following medical consensus. The diagnostic algorithm is described, considering risk factors for IGM, typical radiological signs in order to be able to detect suspected cases early and subsequently ensure early treatment, which is described in the following algorithm for treatment. We also describe a specific follow-up circuit.
These multidisciplinary diagnostic-therapeutic algorithms are proposed to raise awareness and standardise the approach to this rare disease, accelerating its diagnosis and optimising its treatment.
Granulomatous mastitis with erythema nodosum: A breast cancer mimicking entity
2023, Radiology Case Reports
We present the case of a 42-year-old Hispanic patient who consulted with a left breast mass that showed clinical and imaging signs of breast cancer. During preprocedural examination before needle biopsy, the patient was found to have bilateral, purplish-brown skin lesions on her lower legs, suggestive of erythema nodosum. This clinical finding raised the diagnostic suspicion of granulomatous mastitis, which was later confirmed by histopathology. Granulomatous mastitis is a rare, nonmalignant entity that should be considered in patients of childbearing age who present with a breast mass. The coexistence with erythema nodosum contributes to the clinical suspicion of granulomatous mastitis; the mechanism of this association and the optimal treatment approach remain unknown.
Idiopathic granulomatous mastitis in seventy seven-female patients: Management, follow up of an overlooked immune-mediated disease, and review of literature
2023, Egyptian Rheumatologist
Idiopathic granulomatous mastitis (IGM) cases have been increasingly recognized in the context of autoimmune diseases. Hence rheumatologists need to be acquainted with IGM detection and management. Although studies evaluating the response to the emerging immunosuppressive drugs were promising, prospective studies still remain scarce.
This prospective study aimed to evaluate previous immunosuppressive regimens in the management of IGM cases.
Seventy seven female IGM patients were followed up, and stratified into 2 groups based on the treatment modalities received: double therapy (Group D, n = 17) who received steroids and methotrexate (MTX), and triple therapy (Group T, n = 60) who received steroids, MTX and mycophenolate mofetil (MMF). Each group was followed up monthly till the end of treatment then every 3 months after stopping treatment for at least 2 years, and the patients' data were recorded on 3 visits (V1, V2, and V3).
Female patients mean age was 33.9±6.2 years. IGM lesions improved in the form of pain relief, resolution of lumps; replacement of inflammatory lesions by granulation tissue, with superiority of triple therapy group T over double therapy in group D regarding significantly shorter duration of treatment (26.5±3.1 vs 18.1±4.4 months)(p < 0.001), and decreased time needed to steroid tapering (14.1±3.6 vs 5.5±5.2 months)(p < 0.001). No recurrence was noticed.
Treatment of IGM has been successful using MTX, or MTX and MMF besides to steroid therapy. Triple therapy was significantly superior with shorter duration of treatment and faster feasibility of steroid tapering.
Imaging manifestations of idiopathic granulomatous lobular mastitis on cone-beam breast computed tomography
2022, European Journal of Radiology
Citation Excerpt :
The differential diagnoses of IGM include infectious mastitis and breast malignancy. However, distinguishing among these is very difficult, as the imaging findings are heterogeneous and non-specific [6–9]. Cone-beam breast computed tomography (CBBCT) is a novel three-dimensional imaging modality that is based on cone-beam X-ray and flat-panel X-ray detectors [10–13].
To evaluate the imaging manifestations of idiopathic granulomatous mastitis (IGM) on cone-beam breast computed tomography (CBBCT).
We retrospectively evaluated the clinical data of 22 female patients (mean age, 34.73 ± 10.41 years; range 20–58 years) with IGM pathologically confirmed by biopsy or resection. The non-contrast-enhanced CBBCT features, contrast-enhanced CBBCT features, contrast enhancement rate, time-density curve (TDC) and Breast Imaging-Reporting and Data System category of IGM were assessed. The contrast enhancement rates of IGM lesions at 60 s, 120 s and 180 s after injection of contrast agent were compared using ANOVA.
All 22 patients with IGM showed non-mass enhancement on CBBCT. Approximately 40.9% (9/22) of IGM lesions displayed diffuse patchy or focal nodular enhancement on CBBCT, 31.8% (7/22) showed mammary duct dilation, 13.6% (3/22) showed pseudocystic appearance, and 13.6% (3/22) manifested as honeycomb cysts. Among the 22 patients, 72.7% (16/22) displayed type I TDC (persistently enhancing pattern) and 27.3% showed type II TDC (plateau pattern) on contrast-enhanced CBBCT.
IGM mainly manifests as non-mass enhancement on CBBCT, with persistently enhancing or plateau TDC. CBBCT efficiently displays detailed features of IGM with high-density resolution and hemodynamic characteristics.
Inflammatory diseases of the breast
2022, Best Practice and Research: Clinical Obstetrics and Gynaecology
Citation Excerpt :
Ultrasound is the most common and best initial imaging tool for the diagnosis of IGM. IGM often appears as a hypoechoic, irregular mass with tubular projections [22,24,27,30,33–36]. Multiple masses, parenchymal edema, sinus tracts to the skin, dilated ducts, and axillary adenopathy can be seen (Fig. 4) [22,24,30,33,35–37].
Inflammatory disorders of the breast are common benign breast conditions. Lactational mastitis occurs in breastfeeding women and may be associated with breast abscess in severe cases. Non-lactational inflammatory disorders are less common and include idiopathic granulomatous mastitis, periductal mastitis, and tuberculous mastitis. While these disorders have some similarities in their presentation, each disorder requires a specific treatment regimen for resolution, and correct diagnosis is crucial for appropriate treatment. In this chapter, we will review the presentation, diagnosis, and management of each of these distinct clinical entities.
A retrospective study of idiopathic granulomatous mastitis diagnosis and clinical presentation
2022, Journal of the American Academy of Dermatology

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Breast ImagingClinical and imaging features of idiopathic granulomatous mastitis - The diagnostic challenges and a brief review

Highlights

Abstract

Introduction

Section snippets

Patient no 1

Discussion

Conclusion

Grant support

Declaration of competing interest

Pathology

Chronic Dis Transl Med

Diagn Interv Imaging

Idiopathic granulomatous mastitis: manifestations at multimodality imaging and pitfalls

Radiographics

Granulomatous mastitis: a ten-year experience at a University Hospital

Eurasian J Med

Imaging findings in patients with granulomatous mastitis

Iran J Radiol

Idiopathic granulomatous mastitis associated with risperidone-induced hyperprolactinemia

Diagn Pathol

Idiopathic granulomatous mastitis: imaging update and review

Insights Imaging

Idiopathic granulomatous mastitis: dilemmas in diagnosis and treatment

Electron Physician

Granulomatous mastitis: a spectrum of disease

Breast Care

Breast Imaging
Clinical and imaging features of idiopathic granulomatous mastitis - The diagnostic challenges and a brief review