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Kimura disease in larynx: big lesion with soft symptoms
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  1. Edgardo Abelardo1,2,
  2. Kannan Ramachandran1,
  3. Daniel Housa3 and
  4. Vinod Prabhu1
  1. 1ENT-HNS, Hywel Dda University Health Board, Carmarthen, UK
  2. 2Institute of Life Sciences 2, Swansea University Medical School, Swansea, UK
  3. 3Pathology, Hywel Dda University Health Board, Carmarthen, Carmarthenshire, UK
  1. Correspondence to Edgardo Abelardo; e.abelardo{at}swansea.ac.uk

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Description

A 75-year-old man was initially admitted for elective inguinal hernia repair but was noted by the anaesthetist to have a ‘uvula-like’ mass in the oropharynx arising inferiorly, either from the base of the tongue or supraglottis, as seen in figure 1. He denied difficulties in swallowing, throat pain or voice changes. The anaesthetist cancelled the routine procedure and referred the patient to Ear, Nose, and Throat (ENT) department. His medical history was unremarkable. His full blood count was essentially normal apart from slight eosinophilia.

Figure 1

Incidental finding of a ‘uvula-like’ mass in the oropharynx but arising inferiorly, either from base of the tongue or supraglottis.

On fibreoptic examination, the mass was noted to be smooth surfaced lying on the anterior surface of the epiglottis and extending to the left supraglottis. Neck palpation was normal. Neck CT scan showed 3 cm×1.5 cm lobulated soft tissue mass over the left side of the epiglottis extending to the pharyngoepiglottic fold, as seen in figure 2. Intraoperatively, the 3-cm pedunculated mass was noted arising from the left arytenoid and was completely excised. The surrounding structures of the larynx and the hypopharynx were all normal. His postoperative events were unremarkable and he was discharged the following day. The initial histopathology showed features compatible with either Kimura disease (KD) or angiolymphoid hyperplasia with eosinophilia (ALHE), as seen in figure 3. Review of the specimen in a specialist pathology centre confirmed the diagnosis of KD. The patient had been followed up yearly for the past 8 years. Recurrence was noted after 6 years from initial presentation and once again, he denied any symptoms from his throat. The lesion which arose from the previous surgical site was successfully re-excised.

Figure 2

CT scan of the neck showing 3 cm×1.5 cm size lobulated soft tissue mass on the left side of the epiglottis with posterior extension through the left pharyngoepiglottic fold.

Figure 3

Histology of specimen showing dense organised lymphoid tissue with a polymorphic infiltrate rich in eosinophils and plasma cells. (H&E, original magnification ×100).

KD is seen in young male adults (M:F=5:1; mean age 32.8 years old), endemic in Oriental Asia, but has been described in Caucasians, Blacks, Hispanics and Arabs.1 2 Hashim et al noted that KD, also known as eosinophilic lymphogranuloma, is a clinical dilemma as specific diagnostic guidelines do not exist.3 It is a chronic inflammatory disorder with unknown cause and presents as large painless solitary mass in the head and neck region, noted in deep-seated tissues or subcutaneous area with intact overlying skin and commonly with regional lymph node and salivary gland involvement. It is also found in the limbs, groin and trunk and intriguingly associated with nephropathy in 12%–16% of cases. Serum eosinophilia and raised IgE, mast cells, interleukin (IL)-4, IL-5 and IL-13 are common suggestive of underlying autoimmune pathology or potential parasitic infestation. Differential diagnoses would include necrotising lymphadenitis such as Kikuchi disease, Mikulicz’s disease or lymphoma. Histologically, it could be confused with ALHE.4 KD has prominent lymphoid follicles, eosinophilic infiltrates and folliculolysis in germinal centres with infiltrating hyalinised blood vessels and interfollicular/interstitial fibrosis, which are not features of ALHE. Imaging shows characteristics of a benign lesion. Management is primarily surgical excision although systemic corticosteroid treatment, cytotoxic therapy and radiotherapy have been used.5 6 Yamamoto et al reported performing prophylactic tracheostomy in a patient with KD in the larynx even the main symptom of the patient was slight dysphonia and dysphagia.2 No malignant transformation has been reported but recurrence is common, as high as 40%.3 7

Patient’s perspective

To anybody who got this lesion, even if it is big, I say do not worry. You will not feel anything at all after the operation. I heard many things need to be learnt from it but what I know is that it is not sinister.

Learning points

  • Kimura disease (KD) in the larynx is a benign indolent lesion which potentially can cause airway emergency.

  • KD can present as a large supraglottic lesion but the patient is surprisingly asymptomatic.

  • Management of KD is primarily surgical excision although steroids, chemotherapy and radiotherapy have been used; no malignant transformation has been reported but recurrence is possible.

Ethics statements

Patient consent for publication

Acknowledgments

Ms Abi Thomas of Glangwili General Hospital Library provided all the full manuscripts in this work.

References

Footnotes

  • Contributors All authors contributed equally and wholly to the planning, contributed to conducting and reporting of the work described in this article, gave substantial contributions to the conception of the work and the interpretation of data, gave final approval of the version published and agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. EA drafted the work and KR, DH and VP revised it critically for important intellectual content.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.