• Users Online: 68
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 

 Table of Contents  
Year : 2015  |  Volume : 2  |  Issue : 3  |  Page : 62-65

Kimura's Disease: A cytodiagnostic dilemma with brief review of literature

1 Department of Pathology, Employees State Insurance Corporation Medical College and Post Graduate Institute of Medical Science and Research, Bangalore, Karnataka, India
2 Department of Pathology, Kamineni Institute of Medical Sciences, Narketpally, Nalgonda, Telangana, India

Date of Web Publication8-Oct-2015

Correspondence Address:
Dr. Srinivasa V Murthy
Department of Pathology, Employees State Insurance Corporation Medical College and Post Graduate Institute of Medical Science and Research, Rajajinagar, Bangalore - 560 010, Karnataka
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2148-7731.166863

Rights and Permissions

Kimura's disease (KD) is a nonneoplastic, chronic, inflammatory disorder of unknown etiology. The present study was done to know the varied spectrum of cytohistologic features in KD. The predominant cytological features of KD are significant number of eosinophils in a background of lymphoid population of cells. Though FNAC can just be a pointer to the diagnosis, it still plays a very pivotal role. Constellation of blood eosinophilia, raised serum immunoglobulin E (IgE), and cytological features help in confident diagnosis of KD.

Keywords: Cytology, histopathology, Kimura′s disease

How to cite this article:
Murthy SV, Geethamala K, Rao SM. Kimura's Disease: A cytodiagnostic dilemma with brief review of literature. Sifa Med J 2015;2:62-5

How to cite this URL:
Murthy SV, Geethamala K, Rao SM. Kimura's Disease: A cytodiagnostic dilemma with brief review of literature. Sifa Med J [serial online] 2015 [cited 2022 May 16];2:62-5. Available from: https://www.imjsu.org/text.asp?2015/2/3/62/166863

  Introduction Top

Kimura's disease (KD) synonymously called eosinophilic lymphogranuloma is a nonneoplastic, chronic, inflammatory disorder of unknown etiology. [1] Clinically telltale signs are a triad of tumor-like swelling in the head and neck region with or without painless cervical adenopathy, blood eosinophilia, and elevated serum immunoglobulin E (IgE) levels. [2] The predominant cytological findings of KD are significant numbers of eosinophils in a background of lymphoid cells. Other features include polykaryocytes, vascular proliferation, and fibrous fragments. [1],[3],[4],[5],[6] This continuum of cytologic features with appropriate clinical setting helps in confident diagnosis of KD, although histopathology still remains necessary for a definitive diagnosis. [1],[2] Herein we present our experiences of cytodiagnostic difficulties with a report of four cases.

  Case Reports Top

Case 1

A 28-year-old South Indian man presented with right cervical lymphadenopathy since 2 months. There was no history of sore throat, fever, or loss of weight. On examination; swelling was nontender, mobile, and measured 3 × 2 cm. Fine-needle aspiration cytology (FNAC) of the lesion showed epithelioid cell granulomas in a lymphoid background and fibrosis [Figure 1]. Ziehl-Neelsenstaining for acid-fast bacilli (AFB) was negative. Hence, diagnosis of granulomatous lesion was considered and biopsy suggested. Pre-excision workup showed blood eosinophilia of >52% with total leukocyte count (TLC) being increased (14 × 10 9 /L). Excision biopsy and histopathology showed structure of lymphnode with preserved architecture, follicular hyperplasia with reactive germinal centers, eosinophilic infiltrates, and eosinophilic microabscesses. Diagnosis of KD was confirmed. Serum IgE levels were increased, >1,000 IU/ml. Patient remained well on follow-up.
Figure 1: Hematoxylin and eosin (H and E) stained cytology smear showing epithelioid cell granuloma in a background of lymphocytes (×100)

Click here to view

Case 2

A 30-year-old man presented with painless right upper cervical swelling since 6 months which was slow growing. The patient's medical history was otherwise unremarkable. On examination; swelling was firm, nontender, and measured 2.5 × 3 cm in right upper cervical region. Complete blood investigation were within normal except for increased TLC > 15 × 10 9 /l and differential count of eosinophils of 42%. FNAC of lesion showed numerous eosinophils, lymphocytes, and occasional presence of atypical mononuclear Reed-Sternberg like cell. This led to a diagnosis of Hodgkin's disease [Figure 2]. Excision biopsy and histopathological examination; however, showed features of KD. The patient was lost for follow-up.
Figure 2: H and E stained cytology smear showing atypical mononuclear Reed– Sternberg like cell in a background of lymphocytes (× 100)

Click here to view

Case 3

A 37-year-old male presented to medical outpatient block with left-sided neck swelling since nearly 6 months, which progressively increased in size for 1 month. On examination, patient had left cervical lymphnode enlargement swelling measuring 4 × 3 cm, firm, immobile, and nontender. Routine blood investigations were unremarkable except for increased eosinophils of 54%. Although white cell count were normal, IgE concentration was markedly increased to over 1,670 IU/ml. FNAC of lymph node revealed numerous eosinophils, lymphocytes, endothelial cells, in a hemorrhagic background. A diagnosis of KD was suggested on the constellation of blood eosinophilia, increased IgE levels, and cytology findings; but biopsy confirmation was asked for. Excision of the swelling and histopathology confirmed the diagnosis of KD. Patient remained well on follow-up.

Case 4

A 30-year-old male presented with neck swelling since1 year, which progressively increased since 1 month. On examination, the left cervical lymphnode swelling measured 3 × 3 cm, firm, mobile, and nontender. FNAC of the lesion on repeated aspiration yielded only fibrous fragments [Figure 3]. Excision was suggested. Histopathology of the lesion confirmed the diagnosis of KD. On retrospective analysis, the patient was found to have increased blood eosinophils of 44%, but total white cell count was normal. IgE concentration was markedly increased (1,340IU/ml). Though cytology smears merely showed only fibrous fragments, entangled amidst them were eosinophils which were missed initially. Patient remained well on follow-up.
Figure 3: H and E stained smear showing fibrous fragments (×100)

Click here to view

  Discussion Top

KD was first described in 1937 by Kimm and Szeto in China, who reported seven cases of benign lymphnode enlargement with eosinophilic infiltrate which they termed as eosinophilic hyperplastic lymphogranuloma. However, the entity became widely known as KD after Kimura et al., in 1948 from Japan, reported similar findings and titled his manuscript as: "On the unusual granulation combined with hyperplastic changes of lymphatic tissue". [7],[8]

This disease is endemic in Asian middle-aged males of Chinese and Japanese origin and rarely seen sporadically in non-Asian population. [9] However Chen et al., concluded in his study that though rare, if clinical suspicion arises, KD should be included in the differential diagnosis for people of any race. [10] The disease can become apparent at any age, the 2 nd and 3 rd decades of life being usual. [7],[8],[9] Men are more commonly affected than women, with a ratio of 3:1. [7],[8],[9],[11] In the present study, all four reported cases were males in their 2 nd or 3 rd decade of life.

KD typically presents with an insidious onset of painless subcutaneous soft tissue masses and adenopathy in head and neck region. [7] The disease usually involves lymphnodes of preauricular, axillary, cervical, and inguinal group and also, salivary glands like parotid, submandibular, and rarely the oral mucosa as well. Other less common sites are scalp, orbit, auricle, limbs, spermatic cord, skeletal muscle, and prostate. [2],[7] In the present study, all four men presented with cervically mphadenopathy. Accessory signs of KD include juvenile temporal arteritis, coronary artery disease, and sleep apnea. Many patients also develop asthma, chronic eczema, pruritus, rhinitis, cutaneous eosinophilic vasculitis, alopecia, and coexisting renal involvement usually presenting as nephrotic syndrome. [2],[7],[9],[12] In the present study, all four individuals presented with cervical lymphadenopathy and had no other complaints.

Over the years, etiology and pathogenesis of KD has been the subject of controversy. Among them, current theories on causation are trauma, autoimmune reactions, and allergic reaction to parasites, virus (Epstein-Barr virus and human herpes virus 8), fungus (Candida albicans), or toxin. Eosinophilia, increased mast cells, elevated levels of interleukin (IL)-5, and IgE suggest an abnormal T-cell stimulation and a hypersensitivity type IV reaction. Specifically CD4 T-helper cells would produce IL-4, -5, and -13, which in turn would stimulate B-cellsfavoring the production of antigen specific IgE. [2],[7],[9],[12],[13]

The varied spectrum of cytologic aspects characterized in KD range from nonspecific lymphadenitis to features simulating many other lesions. Characteristic FNAC findings in KD are polymorphous population of lymphocytes and significant number of eosinophils. Warthin-Finkeldey type of giant cells, epithelioid granulomas, and necrosis may also be seen. [1],[3],[4],[5],[6],[14],[15],[16]

Deshpande et al., who studied eight cases of KD concluded by stating that various conditions both benign and malignant may mimic KD clinically and on smears. Hence, these have to be ruled out before making a diagnosis of KD. [3] In another study by Jayaram and Peh, two out of three cases, suggested the KD diagnosis on FNA smears; whereas in the third, a suspicion of Hodgkin's disease and biopsy established the diagnosis of KD. [4] Chow et al., studied eight cases of KD on FNA and quoted cytology as helpful and valuable in the diagnosis of recurrent lesions of KD and spare the patient from repeated biopsies. [1] Yet another study by Kini and Shariff stated that KD diagnosis can be prompted on cytology itself and later confirmed by histology. [5] Viswanath are porteda rare bilateral presentation of KD in post-auricular and submandibular region, wherein cytology was inconclusive and biopsy confirmed the diagnosis. [17] Kim et al., highlighted the fact that, a good number of eosinophils on cytology smears is an important feature which can be missed due to thick fibrosis or any technical error during aspiration and suggested that Giemsa stain would be helpful for detection of eosinophils. [6]

In all the above mentioned studies, the commonest attribute which points towards the diagnosis of KD on cytology smears are a polymorphous lymphoid population with a substantial number of interspersed eosinophils, fragments of collagenous tissue, endothelial cells, and occasional Warthin-Finkeldey polykaryocytes. Most of these characteristics were noted in our cases also. [1],[3],[4],[5],[6],[17]

The commonest histopathological changes in KD include preserved nodal architecture, follicular hyperplasia with reactive germinal centers. Eosinophilic infiltrates involving interfollicular areas, sinusoidal areas, perinodal soft tissue, and subcutaneous tissue. Eosinophilic microabscesses, smalleosinophilic granulomas, rarely Warthin-Finkeldey type of giant cells and variable degrees of sclerosis [Figure 4]a and b. [2],[7],[8],[12],[13]
Figure 4: (a) H and E stained histology picture showing diffuse infiltration by eosinophils (×400). (b) H and E stained histology picture showing eosinophilic microabscesses (×100)

Click here to view

The differential diagnosis for KD are angiolymphoid hyperplasia with eosinophilia (ALHE), Hodgkin's disease, eosinophilic granuloma, epithelioid hemangioma, Mikulicz disease, tuberculosis, Kaposi's sarcoma, hamartoma, drug reactions, parasitic lymphadenitis, Castleman's disease, allergic granulomatosis, and many more. [5],[7],[13] The cytological features of ALHE are the same, except for spindle-shaped and polygonal cells with vesicular nuclei and deeply eosinophilic cytoplasm containing well-defined vacuoles. [18] Chong et al., in his study said that KD and ALHE form a spectrum of the same disease. [19] The huge list of differentials can be distinguished from KD by clinical, cytological, and histological features [Table 1]. [5],[7],[13],[18],[19],[20] Recently immunocytochemistry and immunohistochemistry on the aspirate smears and tissue sections with markers like IgE and various CD markers help in further ruling out the differentials. [5],[7],[13],[18],[19],[20]
Table 1: Kimura's disease: Cytologic and histopathological mimickers

Click here to view

Imaging is often not diagnostic as they give nonspecific variable appearance except for CT and MRI which delineates the extent of disease process. [7],[9],[12] Management strategies range from conservative 'wait and watch' policy for asymptomatic individuals to surgical excision, steroid therapy, or radiotherapy. KD is benign, but a disfiguring disease and needs complete surgical excision whenever feasible. [5],[7],[9],[12],[13] In all four of our cases, surgical excision was the preferred modality of treatment.

  Conclusion Top

Though FNAC can just be a pointer to the diagnosis, it still plays a very pivotal role. FNA can be a useful diagnostic tool in KD as it is a readily available, rapid procedure; hence, a high index of suspicion coupled with diligent search for the characteristic features correlated with clinical findings help in arriving at an accurate diagnosis of KD.

  References Top

Chow LT, Yuen RW, Tsui WM, Ma TK, Chow WH, Chan SK. Cytologic features of Kimura′s disease in fine needle aspirates. A study of eight cases. Am J Clin Pathol 1994;102:316-21.  Back to cited text no. 1
Shetty AK, Beaty MW, McGuirt WF Jr, Woods CR, Givner LB. Kimura′s Disease: A Diagnostic challenge. Pediatrics 2002;110:e39.  Back to cited text no. 2
Deshpande AH, Nayak S, Munshi MM, Bobhate SK. Kimura′s disease. Diagnosis by aspiration cytology. Acta Cytol 2002;46:357-63.  Back to cited text no. 3
Jayaram G, Peh KB. Fine-needle aspiration cytology in Kimura′s disease. Diagn Cytolpathol 1995;13:295-9.  Back to cited text no. 4
Kini U, Shariff S. Cytodiagnosis of Kimura′s disease. Indian J Pathol Microbiol 1998;41:473-7.  Back to cited text no. 5
[PUBMED]  Medknow Journal  
Kim SH, Kim HY, Kim SF, Yang WI, Hong SW, Le KG. Fine needle aspiration cytology of kimura′s disease of parotid gland - Report of a case cytologically failed to diagnose as kimura′s disease. Korean J Cytopathol 2003;9:86-90.  Back to cited text no. 6
Rahman RA, Ismail F, Ramli R, Nor GM, Putra SP. Kimura′s disease in malaysian patients: Three case reports. J Sains Kesihatan Malaysia 2005;3:1-8.  Back to cited text no. 7
Leonardo BP. Kimura disease is not angiolymphoid hyperplasia with eosinophilia: Clinical and pathological correlation with literature review and definition of diagnostic criteria. An Bras Dermatol 2006;81:167-73.  Back to cited text no. 8
Fouda MA, Gheith O, Refaie A, El-Saeed M, Bakr A, Wafa E, et al. Kimura disease: A case report and review of the literature with a new management protocol. Int J Nephrol 2011;2010:673908.  Back to cited text no. 9
Chen H, Thompson LD, Aguilera NS, Abbondanzo SL. Kimura disease: A clinicopathologic study of 21 cases. Am J Surg Pathol 2004;28:505-13.  Back to cited text no. 10
Tseng CF, Lin HC, Huang SC, Su CY. Kimura′s disease presenting as bilateral parotid masses. Eur Arch Otorhinolaryngol 2005;262:8-10.  Back to cited text no. 11
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. 12
Abuel-Haija M, Hurford MT. Kimura disease. Arch Pathol Lab Med 2007;131:650-1.  Back to cited text no. 13
Kocjan G. Fine needle aspiration cytology. Cytopathology 2003; 14:307-8.  Back to cited text no. 14
Caraway NP, Katz RL. Diagnostic cytology and its histopathologic bases. Koss LG, Melamed MR. Editors. 5 th ed. Lippincott Williams and Wilkins; 2006;1:1192-93.  Back to cited text no. 15
Hosaka N, Minato T, Yoshida S, Toki J, Yang G, Hisha H, et al. Kimuras disease with unusual eosinophilic epithelioid granulomatous reaction: A finding possibly related to eosinophil apoptosis. Hum Pathol 2002;33:561-4.  Back to cited text no. 16
Viswanatha B. Kimura disease: An unusual cause of head and neck masses. Report of 2 cases. Ear Nose Throat J 2010;89:87-9.  Back to cited text no. 17
Pettinato G, Insabato L, De Chiara A, Ruocco V, Bucci L. Fine-needle aspiration cytology of angiolymphoid hyperplasia with eosinophilia: A case report with electron microscopy and immunohistochemistry. Diagn Cytopathol 1989;5:88-94.  Back to cited text no. 18
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. 19
Ioachim HL, Medeiros LJ, eds. Kimura lymphadenopathy inioachim′s lymph node pathology. 4 th ed. Lipincott Williams and Wilkins; 2008. p. 191-3.  Back to cited text no. 20


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

  [Table 1]

This article has been cited by
1 Kimura disease A case report and review of the literature
Hasnur Zaman Hashim,Fan Kee Hoo,Sazlyna Mohd Sazlly Lim,Mohd Hazmi Mohamed,Vasudevan Ramachandran,Siew Mooi Ching,Wan Aliaa Wan Sulaiman
Polish Annals of Medicine. 2017;
[Pubmed] | [DOI]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Case Reports
Article Figures
Article Tables

 Article Access Statistics
    PDF Downloaded300    
    Comments [Add]    
    Cited by others 1    

Recommend this journal