Case Report: Sclerosing lymphocytic lobulitis of the breast: case report and literature review

Introduction

It has long been believed that the mammary gland is not affected by degenerative complications of diabetes. This was thought to be true until 1984, when Soler and Khardori first described a case of mammary lesions associated with type I diabetes.¹ Sclerosing lymphocytic lobulitis (SCLL) is a rare and benign tumor that accounts for less than 1% of all benign breast lesions.² SCLL mostly occurs in women with type I diabetes at the stage of degenerative complications. However, cases of SCLL have also been described in patients with type II diabetes, autoimmune diseases such as Hashimoto’s thyroiditis, as well as in healthy individuals and including men.³ It sets a diagnostic trap since it mimics clinically a breast carcinoma.⁴ We present a case of SCLL occurring in a patient with type I diabetes for 22 years, detailing the associated clinical, radiological, and evolutionary aspects, along with the particularities of its management.

Case report

Our patient was a 33-year-old nulliparous woman without a family history of breast cancer, who presented with swelling in her right breast. She was diagnosed with type I diabetes 22 years ago and was administered a high insulin dose. The patient had poor diabetic control because she was not disciplined and did not adhere to the diet. Her diabetes was at the stage of degenerative complications, with hypertension for three years, managed with an angiotensin-converting enzyme inhibitor, and stage III retinopathy. The glycosylated hemoglobin level was 8.2%.

The patient was referred to us for consultation with a right-sided breast tumor. On inspection, asymmetry of the two breasts was noted, with an increased right breast size. On palpation, a 5 cm retroareolar tumor on the right side was identified, characterized by stony consistency and slight mobility with respect to the deeper layers, without signs of inflammation or palpable lymph nodes. The left breast was normal.

Breast ultrasound revealed a 4.62 × 2.27 cm hypoechoic oval mass with circumscribed margins, parallel orientation to the chest wall, and posterior acoustic shadowing ( Figure 1).

Figure 1. Breast ultrasound findings.

It revealed a 4.62 × 2.27 cm hypoechoic oval mass with circumscribed margins, parallel orientation to the chest wall, and posterior acoustic shadowing.

A microbiopsy was performed, concluding that SCLL is a pathological finding with intralobular, periductal, and perivascular nodular lymphocyte infiltrations associated with marked lobular atrophy and extensive interlobular hyaline fibrosis ( Figure 2).

Figure 2. Histological findings.

(HE X400): High-power photomicrograph of a hematoxylin and eosin-stained section of a breast biopsy specimen showingintralobular lymphocytic infiltration.

Given the poorly controlled diabetes, the risk of mutilating surgery (small overall breast volume) and high risk of recurrence, annual clinic, and radiological monitoring werechosen. The patient has been monitored regularly since 2019, and there has been no increase in tumor size or changes in the breast ultrasound findings.

Discussion

SCLL or diabetic mastopathy are rare and benign pathological entity.¹ The prevalence of this disease is unknown because of poor knowledge of the condition. The incidence of SCLL ranges from 0.6% to 13%.^1,5 According to Hunfeld and Bassler,⁶ SCLL accounts for less than 1% of all benign breast lesions. This percentage increased to 13% when considering only patients with type I diabetes.⁶

This condition mainly affects young women with type I diabetes, frequently at the stage of degenerative complications.^7,8 Similar histological lesions have been less frequently described in other autoimmune pathologies (such as thyroiditis and lupus) and in patients with type II diabetes requiring insulin.³ The occurrence of the disease is very rare in men with only five cases reported in literature to date.^9,10

Several physiopathological factors have been identified; notably, hyperglycemiaisbelieved to be responsible for abnormal accumulation and decreased connective tissue degradation in the extracellular matrix.¹¹

Clinically, SCLL typically presents as one or multiple unilateral or bilateral breast lumps with recent onset. These lumps had irregular contours, were hard or even stony in consistency, and were painless. They often grow rapidly and are mobile with respect to both superficial and deeper layers.³ It is frequently retroareolar and measures from 0.5 to 6cm in size.³ It was not associated with nipple discharge or inflammatory skin phenomena. Our patient presented with a stony retroareolar nodule that was poorly circumscribed, measured 5 cm, and suspected malignant.

These lesions clinically mimic those of breast cancer. Therefore, the use of complementary examinations or surgery is essential.

Mammography frequently shows overdensities, more or less regular opacities, and even dense breasts, but without architectural distortion or calcific foci.^12–15 However, mammography remains difficult to interpret because of the young age of patients and the density of breast tissue.

Ultrasound, on the other hand, is more concerning, as the lesion often appears as a hypoechoic formation with irregular contours and strong ultrasound attenuation, which are characteristics frequently observed in polymitotic processes.¹⁵ Among the signs suggestive of malignancy, posterior acoustic shadowing and indistinct margins.¹⁶ Ultrasonographic abnormalities were observed in our patient.

Contrast-enhanced MRI may be indicative of doubts regarding the diagnosis of malignant lesion.³ SCLL lesions present a homogeneously low enhancement with a gradual and progressive course or no definite increase in enhancement at all.¹⁷

Fine-needle aspiration is difficult to achieve because of the sclerotic nature of the lesion. Williams et al⁸ and Logan⁵ report an uninterpretable aspiration rate of 50% due to the poverty of cellular elements.

Biopsy is therefore the examination of choice, which establishes the diagnosis.¹⁶Fine-needle biopsy is not recommended due to increased fibrosis. Coreneedle biopsy is the gold standard.¹⁴ Histological study revealed three types of elementary, non-specific associated lesions¹⁶: lymphocytic lobulitis: mononuclear inflammatory infiltration, more or less dense, associated with small B lymphocytes, plasma cells, and histiocytes. It is found mainly in the mammary lobules and less markedly in the perivascular spaces and sometimes in the periductal spaces.

There are two types of lymphocytic lobulitis (LL): SCLL and non-sclerosing LL (NSCLL).¹⁸ The two types of LL are distinguished by stromal features and a predominant population of B-cells in the former and T-cells in the latter.¹⁸

Immunohistochemistry is only of interest to exclude the differential diagnosis of infiltrating lobular carcinoma (anti-keratin antibody) in cases where epithelioid cells are particularly numerous^4,11

If the diagnostic of SCLL is confirmed by a breast biopsy, simple monitoring is recommended, with clinical and radiological monitoring.² It is possible for diabetic mastopathy to resolve spontaneously.¹¹

In cases in which a conservative approach is chosen, it is crucial to educate the patient on proper breast self-examination techniques. A schedule for regular follow-up visits should be established, and an annual mammography is recommended.¹⁹

For cases involving a single lesion, surgical excision with an adequate margin of normal breast tissue remains the primary treatment option, particularly when no definitive histological diagnosis has been made, if the core biopsy findings are inconclusive, or if the patient has significant concerns regarding the breast lump.²⁰

A literature review indicated that 90% of the patients underwent excisional biopsy.² The reasons cited included patient requests, anxiety, insufficient findings on fine-needle aspiration, the necessity to definitively rule out breast carcinoma, and clinician unfamiliarity with this condition.²

Wider margins during excision may help decrease the recurrence rate.²¹

Despite treatment, a high recurrence rate (30–60% within 5 years) has been reported, with recurrences often affecting a wider region than the initial lesion.²² Bilateral and contralateral recurrences have also been documented.²² Most patients with recurrence undergo re-excision; however, this should be avoided in patients with a history of SCLL.² In cases of multiple or recurrent lesions, once the diagnosis is established, subcutaneous mastectomy can be considered, especially for patients with multiple symptomatic lesions and small breasts, to avoid significant distortions from lumpectomy.²³

Each diagnosed case of SCLL should be individually managed. The type of surgery (mastectomy with immediate reconstruction or lumpectomy) should be carefully planned to ensure the best quality of life and optimal aesthetic outcomes.³ The surgical treatment plan should also consider the tumor and breast size, number of lesions, patient expectations, family history, and experience of the surgical team.³

Conclusion

SCLL is a rare and benign disease associated with degenerative complications in women with type I diabetes. It is a diagnostic trap because it can clinically imitate breast carcinoma. Histological examination of the microbiopsies is sufficient to establish the diagnosis, thus avoiding mutilating surgery in these young diabetics.

Consent

Written informed consent for publication of their clinical details and images was obtained from the patient on January 2024.

Author’s contribution

Khaoula Magdoud: Conception, manuscript drafting, final approval. Sirine Bayar: Conception, manuscript drafting, and final approval. Nejlaarifi: manuscript drafting and final approval. Sana Menjli: manuscript drafting and final approval. Rim Ben Hmid: conception, manuscript drafting, and final approval.