Abstract
Background/Aim: Kimura’s disease is a rare chronic inflammatory disorder that commonly affects the head and neck regions, occurring predominantly in Asian men. Elevated eosinophil count and IgE levels in the peripheral blood examination are suggestive of this disease. In this study we report two cases of Kimura’s disease, treated with wide excision. Case Report: The first case was a 58-year-old man presented with asymptomatic left neck mass. The second case was a 69-year-old man with swelling of the right upper arm, which was suggestive of soft tissue mass. Needle biopsy results were suggestive of Kimura’s disease in both cases. Elevated WBCs at 8,380/μl (neutrophils: 45%, eosinophils: 33%) for the first case and 5,370/μl (neutrophils: 61.8%, eosinophils: 3.5%) for the second one, and serum IgE at 14.988 IU/ml for the first case and 1,315 IU/ml for the second one were observ. For definitive treatment and diagnosis, wide excisions were performed. Final histopathological results revealed Kimura’s disease. Surgical margins were negative even though an ill-demarcated lesion for the first case and high infiltration to the muscle for second case were confirmed. Conclusion: Wide excision was performed in both cases of Kimura’s disease and no recurrence was observed until the final follow-up. Wide excision with negative surgical margin should be recommended for the treatment of Kimura’s disease.
Kimura’s disease was first described in 1948 by Kimura et al. (1). The disease is a rare chronic inflammatory disorder that commonly affects the head and neck regions presenting as a painless soft tissue mass (2-4). Elevated eosinophil count and IgE levels in the peripheral blood examination are suggestive of this disease (5, 6). Definitive diagnosis requires histological examination. There is no established treatment for the disease, while high recurrence rates have been reported (7-9). Surgery allows the greatest diagnostic accuracy and has been the mainstay of treatment (10). However, there is little literature on whether wide or marginal excision is better for Kimura’s disease. Herein, we report two cases of Kimura’s disease that were diagnosed in our institution and treated by wide excision.
Case Report
Case 1. The patient was a 58-year-old Japanese man, who initially presented with painless swelling of the left neck. His past medical history revealed appendicitis and atopic dermatitis. A magnetic resonance imaging (MRI) was requested where malignant soft tissue tumour was suspected, hence, the referral to our department. An elastic and soft palpable mass over the left neck was observ (Figure 1). On MRI, T1-weighted images of the tumour showed intermediate signal intensity and high-signal intensity on T2-weighted images (Figure 2). A needle biopsy was suggestive of Kimura’s disease (Figure 3). Pertinent laboratory results revealed elevated white blood cells (WBCs) at 8,380/μl (neutrophils: 45%, eosinophils: 33%); creatinine, 0.92 mg/dl; estimated glomerular filtration rate (eGFR), 66.3 ml/min; and serum IgE, 14,988 IU/ml. A wide excision was performed for definitive diagnosis. Final histopathological results revealed Kimura’s disease. Surgical margin was negative for residual disease, although an ill-demarcated lesion was seen (Figure 4). No recurrence was observ 5 years after wide resection.
Patient presented with painless mass (arrows) in the left neck region (Case 1).
Preoperative axial magnetic resonance imaging (MRI) revealed a soft tissue tumour at left neck (arrow). MRI with the T1-weighted image showed iso-signal (a). MRI with the T2-weighted image showed high signal (b).
Histological result of the needle biopsy for case 1. Lymphoid tissue with eosinophilic infiltration.
Histological result of the surgical specimen for case 1. a: Ill-demarcated lesion measuring 3.5×2.5×1.5 cm was observed. The lesion was an elastic, soft, gray-white mass (a). Histologically, the lesion was composed of lymphoid tissue with follicle and eosinophil infiltration (b).
Case 2. A 69-year-old Japanese man presented with asymptomatic swelling over his right upper arm. An MRI revealed soft tissue tumour over the right upper arm prompting referral to our department. The patient’s past medical history included surgical procedure for kidney tumour, of which details were unknown. His physical examination showed a palpable elastic soft tissue mass over the right upper arm (Figure 5). On MRI, T1-weighted images showed intermediate- and high- signal intensity on T2-weighted images (Figure 6). Core needle biopsy result was suggestive of Kimura’s disease (Figure 7). Laboratory data revealed elevated WBCs 5,370/μl (neutrophils: 61.8%, eosinophils: 3.5%); creatinine, 1.0 mg/dl; eGFR 55.1, ml/min; and serum IgE, 1,315 IU/ml. Wide excision was performed for definitive diagnosis. Final pathological results confirmed Kimura’s disease. Surgical margin was negative even though high infiltration to the muscle was confirmed (Figure 8). No recurrence was reported 1 year after surgery.
Patient presented with painless mass (arrows) in the right upper arm region (Case 2).
Preoperative axial magnetic resonance imaging (MRI) revealed a soft tissue tumour in the right upper arm (arrow). MRI with the T1-weighted image showed iso-signal (a). MRI with the T2-weighted image showed high signal (b).
Histological result of the needle biopsy for case 2. Lymphoid tissue with eosinophilic infiltration.
Histological result of the surgical specimen for case 2. Lesion with well-demarcated subcutaneous area and infiltrative to the muscle measuring 3.5×3.3×2.7 cm was observed. The lesion was an elastic, soft, gray-white mass (a). Histologically, the lesion was composed of lymphoid tissue with follicle and eosinophil infiltration (b).
Discussion
Kimura’s disease is a rare disease often seen in middle-aged Asian men (10-16). The disease is considered as type I allergy based on the increased eosinophil count and IgE levels in peripheral blood examination results, although the pathogenesis of the disease is unclear (17-22). Frequently, renal impairment is concurrent with Kimura’s disease (18, 23-25). Other complications brought about by the disease, such as asthma, allergic rhinitis, atopic dermatitis, and other allergic symptoms have been reported (20). In this case series, the first patient exhibited increased eosinophil count, IgE levels, and atopic dermatitis. The second patient had elevated IgE levels and renal impairment.
Radiographically, T1-weighted MRI images of Kimura’s disease show intermediate-to-high- and high-signal intensity on T2 (26, 27). Histological features of Kimura’s disease include lymphoid follicle, eosinophil infiltration, and capillary proliferation (12, 18, 19, 28, 29). The disease is commonly misdiagnosed as angiolymphoid hyperplasia with eosinophilia, where elevated IgE levels and renal impairment are observ (1, 16, 19, 30). Some inflammatory diseases, such as lymphoma, Langerhans cell histiocytosis, epithelioid haemangioma, and soft tissue sarcoma, are also common differential diagnoses (5, 26, 27, 31-34).
Currently, no standard treatment for Kimura’s disease exists due to its rarity. Other treatment options, such as surgical excision, steroid therapy, and radiotherapy, have been reported (25, 35, 36). In a systematic review by Lee et al. (10), surgical excision showed a recurrence rate of 30.5%, and steroid therapy and radiotherapy alone were 45% and 60%, respectively. Lesions were noted to initially shrink in steroid therapy; however, after steroid discontinuation, recurrence was likely to occur (19, 27, 37). The duration of treatment is also difficult to assess. In addition, long-term steroid treatment can cause numerous adverse effects, such as adrenal insufficiency, increased risk of wound infections, arteriosclerosis, and diabetes mellitus (38-42). Kim et al. (43) described that it was reasonable to use a steroid as a second-line treatment in KD while considering the risk of recurrence and side effects. Radiation therapy is also a complex treatment strategy to utilize because Kimura’s disease is a benign lesion, which does not show significant symptoms. Moreover, radiation can induce secondary malignancy (44). Radiotherapy is considered an appropriate option for recurrent cases or poor surgical candidates (43). Li et al. (31) described that surgery was the most widely used treatment method and is also the treatment option that can help reach a definitive diagnosis. Kimura’s disease, however, commonly presents with ill-defined margin and infiltrative characteristic. A positive surgical margin has been reported as one risk factor for recurrence (4, 10, 36). Some recent reports have shown that surgery was the most common treatment method; however, the surgical margin was not discussed (45, 46). In both patients presented here, wide excisions with negative margins were performed, and no recurrence was observ at final follow-up. Surgical treatment with wide surgical margins was an effective treatment method in these two cases.
Conclusion
The two cases of Kimura’s disease presented here were highly infiltrative with ill-defined borders as other reports described. Our treatment method was wide excision with negative margin and no recurrence was observed until the final follow-up. Hence, when surgery is performed, wide excision with negative surgical margin for the treatment of Kimura’s disease should be recommended.
Acknowledgements
The Authors thank Editage (www.editage.com) for English language editing and publication support.
Footnotes
Authors’ Contributions
SM, NY, KH, AT, SM, KI, YT, YA, TN, and HT determined the treatment plan. SM and NY conducted the follow-up. SM was a major contributor in writing the manuscript. HT oversaw the study. All Authors read and approved the final manuscript.
Conflicts of Interest
The Authors have declared that there are no competing interests.
- Received January 7, 2023.
- Revision received February 6, 2023.
- Accepted February 22, 2023.
- Copyright © 2023 The Author(s). Published by the International Institute of Anticancer Research.
This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY-NC-ND) 4.0 international license (https://creativecommons.org/licenses/by-nc-nd/4.0).
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