Indian Journal of Cancer
Home  ICS  Feedback Subscribe Top cited articles Login 
Users Online :6426
Small font sizeDefault font sizeIncrease font size
Navigate here
  Ahead of print
Resource links
    Search Pubmed for
    -  Muangwong P
    -  Chitapanarux I
    -  Ya-in C
    -  Klibngern H
    -  Kongmebhol P

  In this article
   Materials and Me...
   Article Figures
   Article Tables

 Article Access Statistics
    PDF Downloaded42    

Recommend this journal


Previous Article  Table of Contents  Next Article
Ahead of print publication

Natural history and treatment outcome of radiotherapy for Kimura's disease

1 Divisions of Radiation Oncology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
2 Department of Pathology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
3 Department of Otolaryngology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
4 Diagnostic Radiology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand

Date of Submission29-Jan-2020
Date of Decision04-Feb-2020
Date of Acceptance03-Jul-2020
Date of Web Publication07-Aug-2021

Correspondence Address:
Imjai Chitapanarux,
Divisions of Radiation Oncology, Faculty of Medicine, Chiang Mai University, Chiang Mai
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijc.IJC_69_20

PMID: 34380861


Background: Kimura's disease is a rare inflammatory disease that usually appears in head and neck region. We reported natural history of the disease and treatment outcome of radiotherapy (RT) in Chiang Mai University Hospital.
Methods: A retrospective review was performed for all Kimura's disease patients treated with radiotherapy at our center between 2002 and 2017.
Results: A total of 20 patients with Kimura's disease were reviewed. There were 14 men and 6 women. All patients presented with the mass in head and neck region. Eleven patients were treated with a definitive intent with RT, 9 patients were treated with RT after recurrence from other modalities. All patients were treated with local external beam RT with 2 Gy per fraction to a median total dose of 30 Gy (range 30–40). The median follow-up time was 4 years (range 1–16.5 years). One patient died from HIV opportunistic infection after 1.5 year of radiation with complete response of Kimura's disease. Most of the patients responded to radiotherapy and controlled the disease at the time of analysis. Two patients had multiple recurrences at new areas outside radiotherapy field. Skin toxicity grade 1 was the most common late side effect which was found in 8 (44%) patients. We did not find severe late toxicity or second malignancy in this patient cohort.
Conclusion: Good local control of Kimura's disease can be achieved, with a radiation dose of 30–40 Gy, with insignificant late toxicities. We suggest that radiotherapy should be one of the treatments of choice for Kimura's disease both in primary or recurrence setting.

Keywords: Kimura's disease, long-term outcome, radiotherapy
Key Message: Radiotherapy shows a good control of Kimura's disease with no in-field recurrence in all 20 patients with minimal side effect. It should be considered as a treatment option for the disease.

How to cite this URL:
Muangwong P, Chitapanarux I, Ya-in C, Klibngern H, Kongmebhol P. Natural history and treatment outcome of radiotherapy for Kimura's disease. Indian J Cancer [Epub ahead of print] [cited 2022 Nov 29]. Available from:

  Introduction Top

Kimura's disease is a rare chronic inflammatory disease with unclear etiology. This disease usually affects young Asian men, and its clinical manifestation is an asymptomatic slow-growing subcutaneous mass in head and neck region or major salivary glands with frequently regional lymphadenopathy with elevated serum immunoglobulin E (IgE) and peripheral eosinophil[1],[2],[3] that is often confused with angiolymphoid hyperplasia with eosinophilia (ALHE). However, Kimura's disease represents an eosinophilic inflammatory process with secondary vascular proliferation, while ALHE usually represents an arteriovenous malformation with secondary inflammation.[2],[3],[4],[5] The clinical course is indolent, and no malignant transformation is reported. Treatment options for the mass are observation, surgery, radiotherapy,[6] or medication such as steroid or cyclosporin[7],[8] but no standard treatment has been established. Hereby, we reported the long term follow-up result of our previous report of 8 cases,[9] with additional 12 patients treated with radiotherapy.

  Materials and Methods Top

After the approval from the institutional ethics committee, all patients with Kimura's disease in head and neck region who received radiotherapy in Chiang Mai University Hospital, Chiang Mai, Thailand between 2002 and 2017 were retrospectively evaluated using the medical records. Patient's data including demographic characteristics, the locations of disease, previous treatment(s) prior to radiotherapy, image studies, pathological characteristics, radiotherapy doses and techniques, and treatment outcomes were reviewed. Descriptive statistics were performed to summarize the patient, tumor, and treatment characteristics.

  Results Top


A total of 20 patients with Kimura's disease were treated with radiotherapy during this period, of which 14 (70%) were men, and 6 (30%) were women, with a median age of 41 (range 20–58) years at the time of radiotherapy. There were pre-existing diseases in two patients; one with HIV-positive receiving antiretroviral therapy, and the other one with end-stage renal disease (ESRD) receiving hemodialysis. Patient and treatment characteristics are shown in [Table 1]. Details of the treatment of each patient in this study are demonstrated in [Table 2].
Table 1: Summary of clinical profile of the patients

Click here to view
Table 2: Clinical profile of the patients

Click here to view

Clinical presentation

All patients presented with slow-growing painless neck mass. The median onset of the symptom before receiving the initial treatment was 3 years (range 1–20). Seventeen (85%) patients presented with unilateral neck mass, while the other 3 (15%) patients had bilateral neck mass. The locations of the tumor were in the preauricular region in 17 (85%) patients followed by submandibular area, and temporal region. The largest Kimura's disease mass was 10 x 6 x 13 centimeters which infiltrated right parotid gland and extended to preauricular, post-auricular, and right temporal region.

Radiologic findings

Ten patients had computed tomographic (CT) scan of the head and neck available in the hospital picture archiving and communication system (PACS) for evaluation. All 10 patients had disease in the preauricular areas. The mass also extended into ipsilateral parotid gland in 8 (80%) patients. All lesions had ill-defined margin with heterogeneous enhancement [Figure 1]. Covering skin thickening and subcutaneous fat stranding around the lesion were found in all patients. Five (50%) patients had cervical lymphadenopathy (short axis at least 10 mm). All lymphadenopathies were well circumscribed and showed no necrotic appearance [Figure 2]. Three in 5 lymphadenopathies had heterogeneous enhancement, while the other two had homogeneous enhancement. The mean lymph node size was 13 millimeters (range 10–17).
Figure 1: Contrast enhanced axial computed tomography (CT) scan of a 45-year-old woman with bilateral parotid gland involvement. There is heterogeneous enhancement within bilateral parotid glands which is typical imaging finding. The posterior margins are ill-defined and showed the infiltration into subcutaneous fat and skin

Click here to view
Figure 2: Contrast enhanced axial computed tomography (CT) scan of a 36-year-old man with Kimura disease of right parotid gland and peri-auricular region. There is right level 2 lymphadenopathy with well circumscribed margin and no central necrosis (arrow)

Click here to view

Histopathological features

Histopathological section of all cases showed several well-formed lymphoid follicles, many with hyperplastic germinal centers. These follicles were surrounded by thin and thick fibrous bands and vascular channels that possess medium to high endothelial cells [Figure 3]. The paracortical hyperplasia away from the reactive lymphoid follicles were infiltrated by small lymphocytes, plasma cells and occasional mast cells. Numerous eosinophils were noted predominantly around lymphoid follicles and generally in the fibrous tissue. Eosinophilic amorphous hyaline bodies deposition on follicular dendritic cells were also noted. Many lesions showed eosinophilic abscesses [Figure 4].
Figure 3: (x 100) Show several lymphoid follicles with well form germinal centers with thin and thick fibrous bands that around the follicles

Click here to view
Figure 4: (x 400) Show numerous eosinophils around the lymphoid follicle with eosinophilic abscess (Arrow)

Click here to view

Treatments prior to radiotherapy

Eight patients were treated with surgery and one with medical treatment before radiotherapy. For the surgical series, four patients received superficial parotidectomy and the other four patients received wide local excision as an initial treatment. All of them had recurrence of neck mass at the same site that was previously treated with surgery with the median time of 3 years (range 1–15) after operation. Cervical lymph node excision for preauricular and neck area lesions as a second operation was performed in one patient for who the disease resurfaced at the surgical site that received parotidectomy with facial nerve preservation 10 years ago. The mass recurred at the same site 5 years after second surgery. There was a complication (facial nerve palsy) in another patient from surgery that was performed outside our center. Oral prednisolone was prescribed as an initial treatment in one patient. He had progression of the neck mass after 6 months of the medical treatment.


Eleven (55%) patients received radiotherapy as the initial treatment. The other 9 patients were treated with radiotherapy after failing from the other treatment modalities. All patients received radiotherapy with 2 Gy per fraction, 5 fractions per week to a total median dose of 30 Gy (range 30–40). The treatment techniques were mixed beam between photon and 10–16 MeV electron in 5 patients, conventional photon beam in 2 patients, and three-dimensional conformal radiotherapy (3D-CRT) in the remaining 13 patients. The radiation field covered only neck mass and involved lymph nodes which were seen from the clinical examination and imaging. Prophylactic lymph node irradiation was not performed in this study.

Outcome of radiotherapy

There were 20 patients treated with radiotherapy with a median follow-up of 4 years (range 1–16.5 years). One patient died from the opportunistic infection after 1.5 years of radiotherapy without recurrence of Kimura's disease. No in-field recurrence was found in the remaining 19 patients. There was out-field recurrence in two (10%) patients. One patient (patient No. 6), who was previously irradiated 40 Gy at submandibular region with right and left opposing radiotherapy using conventional technique, developed the new lesion of Kimura's disease at the right supraclavicular and infraclavicular area which was outside the radiotherapy field after 10.8 years of treatment. She decided not to receive any further treatments. Another patient (patient No. 4), who had the first lesion at right preauricular region and received 30 Gy irradiation with 3D-CRT technique, also developed the new lesion (contralateral side; left preauricular area) after 2 years of treatment. We irradiated her left preauricular mass with a total dose of 30 Gy. Four years later she had out-field recurrence at both sides of lower neck regions. The third radiotherapy with total dose 30 Gy was performed. Six years later, she had the new large mass at left side of forehead which was also out of previous radiation field. Surgical excision of this mass was performed, and the pathology revealed Kimura's disease. Three years after surgery, she had the recurrent mass at the forehead; the CT scan showed the ill-defined mass at her left temporal and frontal area. She received the fourth radiotherapy of 30 Gy. All the irradiated lesions remained subsided from this disease. Apart from these two patients, the tumor has been controlled in remaining 17 patients (85%).

Late radiotherapy toxicities were assessed according to the radiation therapy oncology group (RTOG)/European organisation for research and treatment of cancer (EORTC) late morbidity scoring[10] during the follow-up visits after radiotherapy and the data was available in 18 patients. The maximal grade of late toxicities was reported and found the number of skin toxicity grade 1, subcutaneous grade 1, and salivary grade 1 were 8 (44%), 5 (28%), and 4 (22%), respectively. No severe late toxicity or second malignancy was found in this cohort.

  Discussion Top

Kimura's disease is a rare disease that usually occurs in the head and neck region. The clinical presentation is a painless, slow-growing mass. The median onset of symptoms in our study before getting treatment was three years. CT imaging findings of Kimura's disease are non-specific, and definite diagnosis usually needs pathologic confirmation. However, some CT imaging characteristics can help suggest diagnosis of Kimura disease. Ill-defined enhancing masses in the parotid glands and nearby tissue with lymphadenopathy are suggested as typical characteristic imaging findings of Kimura disease.[11] In our study, all 10 patients who had the CT images in PACS had typical characteristics of ill-defined masses that commonly involved parotid gland. Lymphadenopathy was also frequently seen (50%). We also noticed the appearance of well-circumscribed margin and no central necrosis in our patients; this may help to differentiate from some malignancies that may show extra capsular spreading (ill-defined margin) or central necrosis. Histopathology findings of Kimura's disease are well-formed lymphoid follicles with eosinophilic infiltration, and hyperplastic germinal centers.

The treatment options of Kimura's disease include observation, surgery, radiotherapy, steroid, and cytotoxic therapy. Observation is an acceptable treatment option due to benign nature of the lesions which is slow-growing and asymptomatic. Complete excision of the mass is a preferred treatment of choice as it provides the definite pathologic diagnosis and immediate therapeutic outcome with reasonable disease control.

Complete excision of the mass can provide histopathology data and offers good local control. However, recurrence after surgery is usually observed. All the eight patients who underwent surgery had recurrence of disease. Most of the lesions in this study involved the parotid gland and were located around preauricular region. Therefore, removal of the mass would involve parotid gland resection, with added risk of facial nerve injury. Complete surgical resection is difficult to achieve in Kimura's disease due to its infiltrative nature. Second, multiplicity of the lesions at parotid and neck area which are widely separated usually result in long cervical incision or even several separate incisions. Third, subcutaneous involvement may need wide excision of the involved skin, requiring complex reconstruction. The recent meta-analysis by Ye et al. compared the local control of Kimura's disease for each treatment modality.[12] They found similar local control for surgery and radiotherapy. Also, a combination of surgery and postoperative radiotherapy was superior to radiotherapy or surgery alone in local disease control. However, no mention was made on the toxicity or morbidity of the combined modality of treatment versus single modality, or a comparison of surgery versus radiotherapy.

Steroid was not effective in this patient cohort. One patient in our study had progression of disease after receiving 6 months of steroid. Sato et al.[8] also reported the unsatisfactory outcome of steroid. They found that the tumor relapsed within a few months after tapering dose of steroids.

Radiotherapy is a good option for treating Kimura's disease. Patients in our series had high disease control rate after radiotherapy similar to other published literatures[2],[3] with minimal toxicity. There was no recurrence in radiotherapy field, and only 2 (10%) patients had the recurrent masses outside of radiotherapy fields, but in the head and neck region. One of them received repeat RT and still had a good response and controlled disease until the date of analysis. The radiation fields covered only the masses and involved lymph nodes only, no elective lymph node area was irradiated. Hareyama et al.[6] reported the optimal radiotherapy dose of 26–30 Gy for Kimura disease. Chang et al.[13] also report a 63% local control of Kimura disease treated with radiotherapy dose range 20–45 Gy with a median follow-up of 65 months. We gave a median radiation dose of 30 Gy (range 30–40 Gy), with good tumor control. Late toxicity from radiotherapy was only skin grade 1, subcutaneous grade 1, and saliva grade 1.

Radiotherapy offers good local control, in the primary and recurrent settings as shown in our study. Combining radiotherapy and surgery may be the best treatment option for Kimura's disease.[12] If surgical procedure is very morbid and deforming, radical radiotherapy alone should be considered as it offers reasonable disease control, with improved quality of life. However, because of the rarity of the disease, it is difficult to generate strong evidence on the standard of care for this disease. Toxicity and patient's quality of life for each treatment modality and their combinations need to be studied. Larger multicentric studies need to be conducted prospectively to establish the best treatment option for Kimura's disease.

  Conclusion Top

Good local control of Kimura's disease can be achieved, with a radiation dose of 30–40 Gy, with insignificant late toxicities. We suggest that radiotherapy should be one of the treatments of choice for Kimura's disease both in primary or recurrence setting.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Nyrop M. Kimura's disease: Case report and brief review of the literature. J Laryngol Otol 1994;108:1005–7.  Back to cited text no. 1
Meningaud JP, Pitak-Arnnop P, Fouret P, Bertrand JC. Kimura's disease of the parotid region: Report of 2 cases and review of the literature. J Oral Maxillofac Surg 2007;65:134–40.  Back to cited text no. 2
Chen H, Thompson LDR, Aguilera NSI, Abbondanzo SL. Kimura disease: A clinicopathologic study of 21 cases. Am J Surg Pathol 2004;28:505–13.  Back to cited text no. 3
Ramchandani PL, Sabesan T, Hussein K. Angiolymphoid hyperplasia with eosinophilia masquerading as Kimura disease. Br J Oral Maxillofac Surg 2005;43:249–52.  Back to cited text no. 4
Chong WS, Thomas A, Goh CL. Kimura's disease and angiolymphoid hyperplasia with eosinophilia: Two disease entities in the same patient: Case report and review of the literature. Int J Dermatol 2006;45:139–45.  Back to cited text no. 5
Hareyama M, Oouchi A, Nagakura H, Asakura K, Saito A, Satoh M, et al. Radiotherapy for Kimura's disease: The optimum dosage. Int J Radiat Oncol Biol Phys 1998;40:647–51.  Back to cited text no. 6
Miki H, Tsuboi H, Kaneko S, Takahashi H, Yokosawa M, Asashima H, et al. A case of refractory Kimura disease with a buccal bulky mass successfully treated with low-dose cyclosporine A: Report and review of the literature. Allergol Int 2016;65:212–4.  Back to cited text no. 7
Sato S, Kawashima H, Kuboshima S, Watanabe K, Kashiwagi Y, Takekuma K, et al. Combined treatment of steroids and cyclosporine in Kimura disease. Pediatrics 2006;118:e921–3.  Back to cited text no. 8
Chitapanarux I, Ya-In C, Kittichest R, Kamnerdsupaphon P, Lorvidhaya V, Sukthomya V, et al. Radiotherapy in Kimura's disease: A report of eight cases. J Med Assoc Thail 2007;90:1001–5.  Back to cited text no. 9
Cox JD, Stetz JA, Pajak TF. Toxicity criteria of the Radiation Therapy Oncology Group (RTOG) and the European organization for research and treatment of cancer (EORTC). Int J Radiat Oncol Biol Phys 1995;31:1341–6.  Back to cited text no. 10
Park S-W, Kim H-J, Sung KJ, Lee JH, Park IS. Kimura Disease: CT and MR imaging findings. Am J Neuroradiol 2012;33:784–8.  Back to cited text no. 11
Ye P, Wei T, Yu GY, Wu LL, Peng X. Comparison of local recurrence rate of three treatment modalities for Kimura disease. J Craniofac Surg 2016;27:170–4.  Back to cited text no. 12
Chang AR, Kim K, Kim HJ, Kim IH, Park C Il, Jun YK. Outcomes of Kimura's disease after radiotherapy or nonradiotherapeutic treatment modalities. Int J Radiat Oncol 2006;65:1233–9.  Back to cited text no. 13


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1], [Table 2]


Previous Article  Next Article


  Site Map | What's new | Copyright and Disclaimer | Privacy Notice
  Online since 1st April '07
  2007 - Indian Journal of Cancer | Published by Wolters Kluwer - Medknow