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Kimura Disease of the Parotid Gland Masquerading

as a Venolymphatic Malformation Daniel Faden, MD1; Christine Glastonbury, MD2; Annemieke van Zante, MD, Ph.D3, Jolie Chang, MD1

1Department of Otolaryngology, UCSF; 2Department of Radiology, UCSF; 3Department of Pathology, UCSF

Daniel Faden Email: Dfaden@ohns.ucsf.edu

Contact 1. Birol A, Bozdogan O, Keles H, et al. Kimura's disease in a Caucasian male treated with cyclosporine. Int J Dermatol. Dec 2005;44(12):1059-60 2. Hareyama M, Oouchi A, Nagakura H, et al. Radiotherapy for Kimura's disease: the optimum dosage. Int J Radiat Oncol Biol Phys. Feb 1

1998;40(3):647-51 3. Iwai, H., et al. (2007). "Kimura disease: diagnosis and prognostic factors." Otolaryngol Head Neck Surg 137(2): 306-311. 4. akeishi, M., et al. (2007). "Kimura disease: diagnostic imaging findings and surgical treatment." J Craniofac Surg 18(5): 1062-1067.

References

Educational Objective: At the conclusion of this presentation, the participants should be able to discuss the presentation, imaging characteristics, differential diagnosis, workup and treatment options for Kimura disease.

Objectives: Kimura disease is an extremely rare, chronic inflammatory disorder that most commonly presents as painless lymphadenopathy or masses in the head or neck. The disease occurs most frequently in young Asian males. The etiology is unknown and up to 20 percent of patients have associated kidney disease. The rarity of the disease often leads to misdiagnosis. Imaging characteristics can be easily confused with more common lesions such as venolymphatic malformations. We describe the presentation and diagnosis of a case of Kimura disease in a patient with nephrotic syndrome, previously misdiagnosed as a venolymphatic malformation. Current literature supports treatment of the primary lesion as a means to achieve remission of the kidney disease.

Study Design: Case report.

Methods: Physical exam findings, radiographic imaging features, pathologic findings and management of this unique entity are described. Additionally, a review of the existing literature is provided.

Results: A 32 year old man was referred for management of a longstanding venolymphatic malformation. Due to imaging characteristics inconsistent with venolymphatic malformation, a biopsy of the lesion was recommended. Fine needle aspiration biopsy revealed findings suggestive of Kimura Disease. Serum IgE testing confirmed the diagnosis.

Conclusions: Kimura disease is an extremely rare chronic inflammatory disorder which occurs most commonly in the head and neck. Because of its rarity, the diagnosis is often missed; however, treatment may offer significant benefit to those who suffer from nephrotic syndrome. An understanding of the common presentation, pathology and imaging characteristics is necessary to make a timely

and accurate diagnosis.

Abstract

In contrast to this patient’s MRI, venolymphatic malformations are relatively well-defined and have less surrounding inflammation. FNA was recommended to work up the lesion. As lymphoma was also a concern, flow-cytometry was performed which was negative for a lymphoproliferative disorder. Cytologic features, including tingible body macrophages, dense, sclerotic stroma, scattered eosinophils and Warthin-Finkeldey type multinucleated cells (Image 2), along with the patient’s history of nephrotic syndrome, raised Kimura disease as a possible diagnosis. Serum IgE testing was recommend, which showed significantly elevated IgE, supporting the diagnosis.

A 32 year old man presented to the UCSF Otolaryngology clinic with a 16 year history of fluctuating right cheek swelling. One year prior to presentation he had undergone an MRI at an outside institution which was read a venolymphatic malformation. Of note, the patient had a history of chronic nephrotic syndrome, being diagnosed with minimal change disease at 8 years old. He had been treated with oral steroids for the last twenty years, in addition to various immunosuppressants. Interestingly, the patient described that fluctuations in his facial swelling corresponded to worsening of his kidney disease and that higher doses of prednisone decreased the size of his facial swelling. Physical exam revealed fullness of the right lower cheek anterior to the parotid, in the region of the masseter muscle, without overlying skin changes or oral cavity mucosal changes. The patient was referred to the UCSF Vascular Anomalies Clinic where a panel of experts, including Otolaryngologists, reviewed the case. Imaging was inconsistent with a venolymphatic malformation (Images 1a-e).

Presentation

Kimura disease is a chronic inflammatory disorder of unknown etiology. Kimora disease is primarily an inflammatory process with secondary vascular proliferation unlike vascular malformations which are primarily a vascular aberration. Kimura disease is extremely rare, with an unknown prevalence. It occurs most commonly in Asian males with an average onset of 30 years old. Typically Kimura Disease presents as painless masses in the head and neck, especially in the parotid region. Renal disease, nephrotic syndrome in particular, is present in up to 20% of patient. Work up should include serum IgE levels and peripheral eosinophil counts as

Discussion

Kimura disease is a rare condition of unknown etiology which presents most commonly with painless masses in the region of the parotid. Up to 20% of patients have concurrent nephrotic syndrome. Diagnosis is made though a combination of imaging and pathologic findings and is supported by an elevated serum IgE. Currently there is no standard of care for treatment, although some success has been obtained with corticosteroids, immunosuppressants, radiation or surgical resection.

Work UP

Image 1: MRIs Axial T1 (1a),T2 (1b), T1 w/ gad (1c) and coronal T1 w/ gad (1d,e) showing a T1 hypointense, T2 hyperintense, intensely enhancing ill-defined soft-tissue lesion involving the anterior aspect of the right superficial lobe of the parotid. The lesion extends into the buccal fat and overlying cheek fat, but does not infiltrate the underlying masseter muscle. The parotid gland is otherwise normal.

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e d

b c

Image 2: FNA showing mixed lymphoid elements with germinal center elements. Tingible body macrophages can be seen (2a, arrow) which are the result of brisk cell turnover. The H&E stained cell block shows dense, eosinophilic stroma suggestive of a long-standing, partially sclerotic process (2b, arrow), scattered eosinophils (2c, arrow head) and Warthin-Finkeldey type multinucleated cells (2c, arrow).

nearly all patients have both elevated IgE and peripheral eosinophilia. Current thinking is that the renal dysfunction is due to accumulation of IgE in the glomeruli. BUN, CR and urinary protein should be obtained to exclude renal dysfunction Biopsy is necessary in order to establish the diagnosis. Histologic sections typically show discrete germinal centers, an eosinophilic infiltrate, tingible body macrophages and Warthin-Finkeldey type multinucleated cells. Flow cytometric evaluation of the lymphoid component demonstrates a polyclonal population of cells, arguing against a diagnosis of lymphoma. Classic imaging characteristics include multiple ill-defined, enhancing lesions around the parotid gland with associated lymphadenopathy. Observation is appropriate if the patient is asymptomatic. Oral corticosteroids are effective, although kidney disease recurs as soon as the steroids are stopped. Various immunosuppressants have been used with variable success.1 Radiation is effective, although concern exists regarding radiation induced malignancy considering that patients are generally young2. Surgery has been used successfully with a 20-50% recurrence rate,3,4

In the case presented here, due to the diffuse nature of the lesion, and concern for damage to the facial nerve with surgical intervention,, a trial of cyclosporine for better control of the patients renal disease is being performed. Radiation therapy will be considered as an alternative therapy if medical management of his kidney disease is unsuccessful.

Conclusions

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a c b

Conclusion