Idiopathic granulomatous lobular mastitis: A rare entity with diagnostic  dilemma on fine needle aspiration

Shirish S Chandanwale; Charusheela Gore; Madhuri Singh; Akshi Raj; Rakesh Kumar Rashmi

doi:10.4103/jmedsci.jmedsci_26_20

CASE REPORT

Year : 2021 | Volume : 41 | Issue : 5 | Page : 240-242

Idiopathic granulomatous lobular mastitis: A rare entity with diagnostic dilemma on fine needle aspiration

Shirish S Chandanwale, Charusheela Gore, Madhuri Singh, Akshi Raj, Rakesh Kumar Rashmi
Department of Pathology, Dr. D Y Patil Medical College, Pune, Maharashtra, India

Date of Submission	11-Feb-2020
Date of Decision	23-Jul-2020
Date of Acceptance	25-Feb-2021
Date of Web Publication	04-May-2021

Correspondence Address:
Dr. Shirish S Chandanwale
75/1+2/1, Krishna Appt, New Sangvi, Pune - 411 027, Maharashtra
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/jmedsci.jmedsci_26_20

Abstract

Idiopathic granulomatous lobular mastitis (IGLM) is an uncommon and rare chronic inflammatory breast disease. The exact etiology is not known. Although various etiologies have been implicated, autoimmunity is favored and widely accepted. In countries with a high prevalence of tuberculosis, the diagnosis of IGLM is often challenging. Clinical presentation is quite variable. Ultrasonography and mammography findings lack specificity. Fine-needle aspiration (FNA) cytology features of idiopathic granulomatous mastitis (IGM) have been described in few reports, but they are still debatable. We report the case of a 36-year-old female with a hard lump in the left breast. Aspiration cytology smears showed many well-formed epithelioid granulomas and few scattered epithelioid cells. The background showed numerous polymorphonuclear leukocytes with few reactive lymphoid cells and foreign-body giant cells. There was no necrosis. Acid-fast bacilli (AFB) and fungal stains did not show AFB and fungal hyphae. A diagnosis of IGM was suspected on aspiration cytology. Histopathology examination confirmed the diagnosis. To conclude, though tuberculosis is highly prevalent in India, if breast FNA smears show epithelioid granulomas with numerous polymorphonuclear leukocytes with no necrosis or AFB, IGLM must be suspected.

Keywords: Aspiration, cytology, mastitis, granulomatous, idiopathic

How to cite this article:
Chandanwale SS, Gore C, Singh M, Raj A, Rashmi RK. Idiopathic granulomatous lobular mastitis: A rare entity with diagnostic dilemma on fine needle aspiration. J Med Sci 2021;41:240-2

How to cite this URL:
Chandanwale SS, Gore C, Singh M, Raj A, Rashmi RK. Idiopathic granulomatous lobular mastitis: A rare entity with diagnostic dilemma on fine needle aspiration. J Med Sci [serial online] 2021 [cited 2023 Mar 29];41:240-2. Available from: https://www.jmedscindmc.com/text.asp?2021/41/5/240/315477

Introduction

Idiopathic granulomatous lobular mastitis (IGLM) is an uncommon and rare chronic inflammatory breast disease. Clinically and radiologically, it can mimic breast carcinoma.^[1] Although the exact etiology is still unclear, autoimmunity is a favored cause.^[2] In developing countries, fine-needle aspiration (FNA) is widely accepted as a reliable technique for initial preoperative evaluation of palpable breast lumps.^[3] In countries with a high prevalence of tuberculosis, the diagnosis of IGLM is often challenging. With the increased use of FNA as an initial investigation for breast lesions, more cases will be encountered by cytopathologists. There is a need to increase awareness of this disease entity. Although various treatment modalities such as surgical excision, steroids, methotrexate, and close follow-up are used currently, there is no consensus regarding the ideal method of treatment.^[4]

Case Report

A 36-year-old married female came to the cytology clinic with complaints of discharge of pus from the wound in the left breast with swelling for 15 days. She gave a history of minor operative procedure at the same site 1 month back and the details of which patients did not have. On physical examination, there was a firm lump measuring 2.5 cm × 1.5 cm in the outer quadrant of the left breast with a small ulcer on it. Axillary lymph nodes were not palpable. She had no other past significant history of illness. Her elder child was 7 years old, while younger was 1-year-old. She stopped breastfeeding of younger child 6 months back.

Her routine hematological parameters were within the normal limits. She was human immunodeficiency virus seronegative. Ultrasonography of the left breast showed an irregular hypoechoic mass. Mammography showed a mass with heterogeneous density. Axillary lymph nodes were not enlarged. Clinically, neoplastic lesion or chronic inflammatory lesion was suspected.

After prior written consent of the patient, FNA was performed. Smears were cellular and showed many well-formed compact epithelioid granulomas mixed with predominant polymorph nuclear leukocytes and giant cells. Few scattered epithelioid and reactive lymphoid cells were seen in the background. There was no necrosis. Granulomas were compact and mixed with polymorph nuclear leukocytes and lymphocytes [Figure 1]a and [Figure 1]b. Smears were negative for acid-fast bacilli (AFB) and fungi on Ziehl–Neelsen (Z. N) and periodic acid–Schiff (PAS) stains. A differential diagnosis of tuberculosis, sarcoidosis, and idiopathic granulomatous mastitis (IGM) was suspected. Tuberculin test was negative. Repeat aspiration was done and aspirate was sent for AFB culture which revealed no growth. Subsequent chest X-ray, computed tomography (CT) scan, and magnetic resonance imaging did not reveal any findings which were suggestive of sarcoidosis or tuberculosis. A diagnosis of IGM was suspected on FNA.

Figure 1: Smears show (a) Well-formed epithelioid granulomas with few giant cells (Leishman's stain, ×100). (b) Compact granuloma with infiltration by polymorphonuclear leukocytes. (Leishman's stain, ×400)

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Biopsy of the lesion was received for histopathological examination. Sections showed breast lobules and fibromyxoid stroma. Breast lobules were infiltrated by inflammatory infiltrate comprised lymphocytes, plasma cells, and polymorph nuclear leukocytes, with occasional ill-formed epithelioid granuloma and giant cell [Figure 2]a and [Figure 2]b. Stroma was free inflammatory infiltrate. There was no necrosis. Z. N and PAS stains did not reveal AFB or fungi. A final histopathological diagnosis of IGLM was made.

Figure 2: Section shows breast lobules infiltrated by inflammatory cells (H and E, ×100). (b) Section show lobules infiltrated by epithelioid cells, lymphocytes, and neutrophils (H and E, ×400)

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Discussion

Granulomatous mastitis was first described by Kessler and Wallooch in 1972 in the literature.^[5] Two defined forms of genetically modified (GM) have been identified: (1) IGLM and (2) GM with specific etiologies such as tuberculosis, foreign-body reactions, sarcoidosis, and mycotic and parasitic infection. Some of the forms clinically simulate carcinoma.^[1],[2] Although the exact etiology of IGLM is not known, various etiologies such as hypersensitivity to extravasated lactation products, local breast trauma, association with mammary duct ectasia, Corynebacteria infection, ethnicity, and autoimmunity have been implicated.^[6] Autoimmune process is the favored cause. IGLM and autoimmune mastitis are synonyms of IGLM. Our patient did not have history or significant findings suggestive of other autoimmune diseases such as erythema nodosum, Wegener's granulomatosis, polyarteritis nodosa, or systemic lupus erythematosus.

The disease usually occurs in women of reproductive age and may be associated with lactation or may occur in the postpartum period. Clinically, the patient may present as a chronic draining sinus tract or abscess cavity, or solitary mass in the breast.^[2],[3],[5] Ultrasonography and mammography findings are considered nonspecific.^[2]

Histological features of IGLM have been well described in many reports, but cytomorphological features have not been widely discussed. Few large series reports describing IGLM have been described in the literature. Kumarasinahe and Yip et al., confirmed the useful role of FNA in the diagnosis.^[7],[8] In contrast, some authors concluded that the diagnosis of IGM requires histopathological examination, negative microbiological investigations for AFB and fungi, and clinical correlation.^[5],[9],[10]

Tse et al.^[10] observed a high number of scattered epithelioid histiocytes in the absence of epithelioid granulomas and predominant neutrophilic inflammatory infiltrate and absence of necrosis on the background in FNA smears. Similar observations were made in FNA smears by M Gangopadhyay.^[5] They even found that the number of scattered epithelioid histiocytes was directly proportional to the number of granulomas in the smears. In our case, epithelioid granulomas were compact and mixed with predominantly polymorph nuclear leukocytes. We did not find many single epithelioid cells in the background. A pattern of granuloma, absence of necrosis and AFB or fungi in FNA smears, and other clinical findings made us alert to suspect IGM on FNA. Larger series reports are required to document these findings.

Biopsy tissue from the site showed well-described histological features of IGLM such as noncaseating epithelioid granulomas, lymphocytes plasma cells, and polymorph nuclear leukocytes confined to lobules. The breast stroma was free from inflammatory infiltrate.

Although there is no consensus regarding the ideal method of treatment because of the rarity of the disease, treatment with corticosteroids is the first line of treatment.^[2] The patient was treated with antibiotics, surgical excision, and oral prednisolone 30 mg/day for 4 weeks. The patient is on follow-up for 1 year and there is no history of recurrence.

Conclusion

ILGM is uncommon and a very rare disease. Clinical presentation is quite variable. In developing countries like India, tuberculosis is the most common cause of granulomatous inflammation. However, epithelioid granuloma mixed with polymorphonuclear leukocytes with the absence of caseous necrosis and AFB in FNA of breast smears should alert us to suspect IGLM. Definitive diagnosis requires histopathological examination.

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 initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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