Abstract
Introduction
Kimura disease is a rare, benign, chronic, lymphoproliferative disorder of unknown etiology that affects the skin and lymph nodes and mostly observed in males of Asia.
Case Report
We report a 55-year-old man who presented neck and intraparotid lymphadenopathy. Surgical removal of the growth confirmed the histological diagnosis of Kimura disease.
Discussion
Kimura disease is endemic in the Far East (China and Japan) but exceptional in Europe; < 200 cases have been reported in the literature, with less than 40 involving a parotid region. The histological characteristics of Kimura disease are lymphocyte follicular hyperplasia, eosinophilic tissue infiltration, fibrocollagenous deposition and vascular proliferation. Main treatment is surgical, but recurrence is frequent.
Conclusion
Kimura disease is a rare condition, but should be considered in the case of cervical mass associated with hypereosinophilia and/or strongly elevated IgE levels, especially in Far Eastern patients.
Introduction
Kimura disease (KD) is a rare, benign, chronic immune-mediated inflammatory disorder, which was first described in 1938 by Kimura and Szeto [1]. The specific mechanism is unknown. However, deregulation of eosinophil dynamics and IgE synthesis and altered systemic immune-mediated reaction have all been postulated as causative [2]. This inflammatory disorder affects predominantly Asian men and is characterized by recurrent subcutaneous masses in the head and neck region with eosinophilia and elevated IgE levels [3]. There is still no definitive treatment for this disease; surgery, thalidomide, cyclosporine, interferon-α, omalizumab (an anti-IgE antibody), corticosteroids and radiotherapy have been recommended [4], but none are clearly standard of care. Parotid locations are exceptional, with less than 40 cases described in the literature; cases reported in Europe are even rarer. We present a case of Kimura intraparotid disease treated with surgery in China 30 years ago with a neck recurrence.
Case Report
A 55-year-old man was referred to Oral and Maxillofacial Surgery Department. He exhibited a cervical right mass. He had a surgery in the parotid region (partial parotidectomy); 30 years previously, no records of this surgery were available for us; however, we can assume that a partial parotidectomy was performed by the scanner images. The patient reported itching in hands, feet and neck, and he had no allergies. He was treated with oral corticosteroid after the first resection in his country of origin.
Laboratory examination showed WBC of 13.50 × 103/L; eosinophil count was 6.2 × 103/L (normal is 0–0.7 × 103/L). The percentage of eosinophils was 46%. Serum total IgE was elevated. Urine was sampled for the measurement of urinary albumin in the morning after an overnight fast. Urine samples were collected for the measurement of albumin concentrations, which were expressed as the ratio of urinary albumin to creatinine concentrations (UACR [mg/g Cr]). The patient showed 201.25 mg/g Cr (normal is 0,00-30.00). Extramembranous glomerulonephritis is frequently associated with Kimura disease but it was not present in this patient. The erythrocyte sedimentation rate, liver and thyroid function tests and lactic dehydrogenase were normal. Treponema pallidum hemagglutination assay, venereal disease research laboratory test, human immunodeficiency virus serology, hepatitis A, B, C tests, antinuclear antibodies, rheumatoid factor, complement serum C3 and C4 levels, toxoplasmosis titer were all normal or negative. Chest X rays were normal.
Computed tomography (CT) and magnetic resonance imaging (MRI) examination showed several enlarged cervical lymph nodes of different morphologies and sizes up to 2,5 cm in diameter (greatest dimension) (Figs. 1 and 2).
Fig. 1.
MRI coronal section of cervical lymphadenopathy
Fig. 2.
MRI axial section of neck lymphadenopathy
Several fine needle aspiration cytology of a level II lymph node was performed. But any of them give us a definitive diagnosis. The first needle aspiration reported lymphoid cellularity with polymorphic aspect, the second aspiration reported nondiagnostic cytology and the last one showed isolated fusiform cells of fibroblastic appearance without atypia. The Pathology Department recommended to make an open biopsy of level II lymph node for definitive typing.
Surgical treatment involving biopsy of the cervical mass was proposed. Informed consent was obtained.
The surgery consisted of cervical lymph nodes biopsy by cervical incision under general anesthesia (Fig. 3). An incisional biopsy was performed, due to the tumor size and the benignity signs with a 15 blade scalpel and chasing a blunt dissection. The macroscopic mass tissue was elastic and nodular. Due to the characteristics of the mass, we did not perform margins as in oncological surgery. Electrocoagulation was used in order to achieve hemostasis involving the surgical field, and absorbable 3/0 Vicryl was used to suture the subcutaneous cell tissue and monofilament suture was used for the skin incision.
Fig. 3.
Cervical lymph nodes biopsy
Intraoperative biopsies identified a tissue with heterogeneous lymphoid background and abundant histiocytes with a tendency to aggregation, and the presence of numerous tingible bodies, compatible with sinus histiocytosis. No signs of malignancy were found.
Postoperative course was free of facial palsy or other complications.
Histopathological examination demonstrated eosinophil tissue infiltration (Fig. 4) and lymphocyte follicular hyperplasia (Fig. 4). The infiltrate consisted of plasma cells, lymphocytes and numerous eosinophils, which formed occasional eosinophilic abscesses. On immunohistochemistry, the lymphoid follicle phenotype was CD20+ CD5− CD10+ BCL2− BCL6+. Based on the clinical and histological findings, the diagnosis of Kimura disease was made (Fig. 5).
Fig. 4.
Eosinophilic predominance of the interfollicular area
Fig. 5.
Lymphocyte follicular hyperplasia
Case Discussion
Kimura disease is a rare condition, endemic in the Far East (China and Japan) but exceptional in Europe. Less than 200 cases have been reported in the literature, with less than 40 involving a parotid region. Patients are typically males in their 30s. The histological characteristics of Kimura disease are lymphocyte follicular hyperplasia, eosinophilic tissue infiltration, fibrocollagenous deposition and vascular proliferation. Blood eosinophilia is an important marker in Kimura disease [5]. This rare condition may be easily mistaken for a malignant disorder, including T cell lymphoma, Kaposi sarcoma, Hodgkin disease or parotid tumor [6].
In reactive lymph node hyperplasia and pseudolymphomas, WFLC can be observed either inside or both inside and outside germinal centres but rarely exclusively located in interfollicular zones. These findings are at variance with those of Kjeldsberg and Kim [7] who identified their more frequent presence in interfollicular areas. WFLC appear mostly inside germinal centers which may contain several cells of this type; when lymphoma develops in these lymph nodes, germinal centers are destroyed and WFLC are incorporated into the lymphomatous proliferation. This also could explain the identification by Kjeldsberg and Kim [7] of WFLC in lymphomas of T cell origin as such mycosis fungoides, or in malignant lymphoma with a high content of epithelioid cells.
Clinically, the disease presents as painless pruriginous cutaneous head and neck nodules, progressively increasing in volume. Extramembranous glomerulonephritis is frequently associated with Kimura disease but it was not present in this patient. Blood analysis found hypereosinophilia and total IgE elevation. Salivary glands involvement is rare; most cases are isolated [8].
Surgical removal of lesions (when feasible) appears to be the best option, but rates of recurrence following treatment may be up to 62% [9]. Corticosteroids have been recommended, especially in the case of renal involvement, but raise the issue of long-term adverse events. In some cases, postoperative radiation therapy has been applied, but failed to prevent recurrence [10].
Conclusion
Kimura disease is a rare condition, but should be considered in the case of a cervical mass associated with hypereosinophilia and/or strongly elevated IgE levels, especially in Far Eastern patients. Surgical treatment is essential; however, surgical resection is not always feasible and other treatment options may need to be considered. We report a case of KD revealed by routine CT scan and confirmed by histological examination. This case raises the question of differential diagnosis in patients with neck lymphadenopathy.
Acknowledgements
The authors acknowledge the Miguel Servet University Hospital, the Oral and Maxillofacial Department and all the patients who were treated.
Authors' Contributions
Esparza Lasaga contributed to conceptualization, methodology, investigation and writing—reviewing and editing. Bermúdez Cameo was involved in data curation and writing—original draft preparation. Valero Torres was involved in visualization and investigation. Delso Gil contributed to supervision, software and validation.
Compliance with Ethical Standards
Competing interest
The authors have no conflict of interest to disclose.
Footnotes
Publisher's Note
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