Ann Thorac Surg 2009;87:314-316. doi:10.1016/j.athoracsur.2008.06.053
© 2009 The Society of Thoracic Surgeons
Case Reports
Kimura's Disease Presenting as the Middle Mediastinal Mass
Chong Zhang, MDa,*,
Jian Hu, MDa,
Zhiying Feng, MDb,
Tao Jin, MDa
a Department of Thoracic and Cardiovascular Surgery, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, China
b Department of Anaesthesiology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, China
Accepted for publication June 17, 2008.
* Address correspondence to Dr Zhang, Department of Thoracic and Cardiovascular Surgery, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, China (Email: haiyanzhangchong{at}yahoo.com).
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Abstract
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Kimura's disease usually located in the periauricular area most frequently; however, we believe that there have been no reports about kimura's disease presenting as middle mediastinal mass. we describe the diagnosis and treatment of kimura's disease in the middle mediastinum.
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Introduction
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Kimura's disease is an uncommon idiopathic, chronic inflammatory disease that usually affects young and middle-aged men of Asian background, especially in Japan and China [1]. It presents mainly as single or multiple subcutaneous mass most frequently in the periauricular area [2]. However, we believe that so far there have been no reports about Kimura's disease presenting as a middle mediastinal mass, which might cause some difficulties in diagnosis and effective treatment for clinicians.
A 32-year-old Chinese man was admitted to our hospital with complaints of low-grade fever and cough for approximately 20 days. He had no history of allergic diseases, such as asthma or dermatitis. The elevated eosinophils count was 540/uL with differential of 6.5% and the erythrocyte sedimentation rate C-reactive protein was normal. On physical examination some small lymph nodes were palpable in the neck and axillary region. The chest roentgenogram revealed right pleural effusion. Electrocardiogram showed sinus arrhythmia with grade one atrioventricular block. Echocardiography showed diffused thick ventricular and aortic wall. The computed tomographic scan confirmed a mediastinal mass with right pleural effusion (Fig 1A). All these radiological findings suggested a possible diagnosis of lymphoma, tuberculosis, or other malignant mass. With purpose for diagnosis we advised biopsy; however, the patient rejected and requested discharge to the local hospital where clinicians gave him diagnostic anti-tubercular therapy. The anti-tubercular drugs included isoniazid, rifampicin, and ethambutol. One month later, computed tomographic scan revealed that the mass size was unchanged, but the pleural effusion disappeared. Three months later after anti-tubercular therapy, he was admitted to our hospital again presenting with severe swelling of the neck. Enlarged lymph nodes were found in the neck and axillary area. Varicosity was also seen on the chest. The eosinophils count was 963/uL with differential of 9.7%. The tests for parasitic infection, cytomegalovirus, Epstein-Barr virus, and toxoplasma serologies were all negative. Echocardiography showed that the mediastinal mass invaded cardiac base, especially the right and left atrium (Fig 1B). Computed tomographic scan confirmed pleural effusion, which almost filled the whole right thoracic cavity and the giant mediastinal mass involved cardiac structure, such as the right atrium, left atrium, superior vena cava, aorta, coronary artery, pulmonary artery, and veins (Fig 1C). The positron emission tomography revealed high metabolic mass. The right axillary and neck lymph nodes were biopsied (Fig 2A). With computed tomographic-guided tissue biopsy, multiple tissues of the mass were also taken. All pathologic reports showed eosinophilic hyperplastic lyphmogranuloma (Kimura's disease). The microscopic manifestation of mediastinal mass was massive infiltration by eosinophils with predominantly eosinophilic mass in some areas (Fig 2B). After confirmatory diagnosis, the patient received oral prednisolone therapy. The usage was 10 mg once and regularly 3 times a day. Ten days after steroid treatment, the eosinophils count was down to only 223/uL with differential of 2.6%.
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Fig 1. (A) The mediastinal mass with right pleural effusion. (B) Echocardiographic scan shows mediastinal mass invaded cardiac base, especially the right and left atrium. (C) Pleural effusion filled almost all of the right thoracic cavity and the giant mediastinal mass involving cardiac structure. (D) Pleural effusion disappeared and mediastinal mass did not decrease to some extent. (LA = left atrium; M = mediastinal Kimura's disease; PE = pleural effusion; RA = right atrium.)
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Fig 2. (A) Photomicrograph of Kimura's disease in the right axillary and neck lymph nodes. Microscopically, the involved nodes showed marked hyperplasia of germinal centers and extensive infiltration by mature eosinophils with formation of eosinophilic abscesses. (Hematoxylin and eosin; x100.) (B) Photomicrograph of Kimura's disease in the middle mediastinum. The manifestation was massive infiltration by eosinophils, with predominantly eosinophilic aggregation in some areas. (Hematoxylin and eosin; x400.)
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The patient continued the same therapy after discharge. In another hospital, the elevated serum immunoglobulin E was measured at 427 IU/mL. The pleural effusion was drained and the characteristics were exudates with light yellow in color, but without any malignant evidences. On follow-up, neck swelling and chest varicosity gradually disappeared. Ninety days later after steroid therapy, lymph nodes were not palpable in the neck and axillary regions; however, the computed tomographic scan showed mediastinal mass did not decrease to some extent (Fig 1D). Because there had been no reports about treatment experiences, we continued follow-up and searched for other possible treatments. Although radiotherapy, cyclosporine, or other treatments for common Kimura's disease were under our consideration, the patient requested to continue the same steroid therapy. Three months later, the patient felt well and worked every day. However computed tomography demonstrated the mass still remained with similar size. The serum concentration of immunoglobulin E tested in another hospital was 375 IU/mL, which was still higher than normal. A needle re-biopsy for the mass was performed. The pathology of multiple tissues still demonstrated Kimura's disease (Fig 3). The patient continued the steroid and decided to receive radiotherapy treatment. The prognosis was unknown, and now we made advance follow-up.
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Fig 3. Microscopical pathology of re-biopsied mediastinal mass demonstrated Kimura's disease. (Hematoxylin and eosin; x400.)
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Comment
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Kimura's disease presents mainly as single or multiple subcutaneous mass most frequently in the periauricular area, and other sites, such as the oral cavity, orbital, axilla, groin, limbs, and trunk also may be involved. Cases showing Kimura's disease in skeletal muscles, prostate, and kidney had also been reported [2]. Kimura's disease is usually devoid of systemic symptoms; however, complications with immune disorders, such as nephrotic syndrome and bronchial asthma had been reported [3]. However, Kimura's disease might cause some diagnostic difficulties and need strict criteria for confirmatory diagnosis. Due to the unusual location in our case, the clinical presentation is confused with inflammatory or neoplastic conditions, such as malignant lymphoma, tuberculosis, or other diseases. Prior to biopsy, it is initially mistaken for other diseases, despite elevated peripheral eosinophilia and immunoglobulin E level. Even after biopsy it may be difficult to make a correct diagnosis if pathologists are not familiar with the histology of Kimura's disease. The main differential diagnosis for Kimura's disease is angiolymphoid hyperplasia with eosinophilia and Castleman's disease, which are mainly according to the confirmatory pathohistologic findings. Histopathologic findings in Kimura's disease are characterized by lymphoid follicle formation with prominent germinal centers, infiltration of eosinophils, and fibrosis. In contrast, vascular proliferation is the most significant finding in angiolymphoid hyperplasia with eosinophilia, forming aggregates or lobules comprised of plump endothelial cells frequently demonstrating cytological atypia and vacuolization. The microscopical finding in Castleman's disease is divided into two types. The hyaline-vascular type shows marked vascular proliferation and hyalinization of abnormal germinal centers of large follicles. Follicular dendritic cells present in the hyaline centers and a tight concentric layering of lymphocytes around follicles with onion-skin appearance. The plasma cell type shows a diffuse plasma cell proliferation in the interfollicular tissue and a deposition of an amorphous acidophilic material in the center of follicles.
The diagnosis often requires clinical and histologic data, but the real challenge is the treatment. The treatment includes surgical excision, steroid, radiation, and others, but the choices are still controversial. The first preferred treatment is surgical removal. However, the procedure is plagued by following problems: Kimura's disease frequently recurs at the excision sites, complete excision is frequently difficult because it is infiltrative, and multiple sites are involved [4]. Therefore, repeated recurrences after surgery are characteristic, especially for lesions, which are incompletely excised initially due to infiltration, because the heart was severely infiltrated in our case; so after confirmatory diagnosis the patient was initially treated with systemic steroid. Moreover systemic steroid had been effective in some patients. However, steroid withdrawal could often result in tumor relapse, which becomes refractory to the continued steroid therapy. Radiotherapy had been occasionally used in recurrent or refractory cases. From Chang's report [5], the radiation field should be limited to the lesion and its adjacent swelling lymph nodes with a radiation dose from 26 to 30 Gy, which was sufficient for local control, regardless of tumor size. However, the concerns of secondary malignancy for the use of radiotherapy had dampened enthusiasm. Immunosuppressive drugs such as cyclosporine or chemotherapy drugs such as vincristine had been successful in a few cases [6, 7].
We believe that there have been no reports about Kimura's disease presenting as middle mediastinal mass and treatment experiences. Although the prognosis of our patient was unknown, we still continued follow-up and explored some effective treatment strategies. We presented our case and hoped to render the value for attention.
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References
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- Gumbs MA, Pai NB, Saraiya RJ, et al. Kimura's disease: a case report and literature review J Surg Oncol 1999;70:190-193.[Medline]
- Hui PK, Chan JKC, Ng CS, et al. Lymphadenopathy of Kimura's disease Am J Surg Pathol 1989;13:177-186.[Medline]
- Qunibi WY, A1-Sibai MB, Akhtar M. Mesangio-proliferative glomerulonephritis associated with Kimura's disease Clinical Nephrology 1988;30:111-114.[Medline]
- Urabe A, Tsuneyoshi M, Enjoji M. Epitheloid hemangioma versus Kimura's disease, a comparative clinicopathologic study Am J Surg Pathol 1987;11:758-766.[Medline]
- Chang AR, Kim K, Kim HJ, et al. Outcomes of Kimura's disease after radiotherapy or nonradiotherapeutic treatment modalities Int J Radiation Oncology Biol Phys 2006;65:1233-1239.[Medline]
- Kaneko K, Aoki M, Hattori S, et al. Successful treatment of Kimura's disease with cyclosporine J Am Acad Dermatol 1999;41:893-894.[Medline]
- Connelly A, Powell HR, Chan YF, et al. Vincristine treatment of nephrotic syndrome complicated by Kimura disease Pediatr Nephrol 2005;20:516-518.[Medline]
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Abstract
Introduction
