Case report 63
Kimura’s disease involving the peripheral nerve of the arm in a child: a case report Kwang Soon Songa and Da Eun Woob A 7-year-old Korean boy was referred for a nontender mass in the left arm. He had a 5-year history of a poorly defined painless mass on the medial side of the left elbow. The ultrasonogram of the left arm indicated an ill-defined, lobulating solid mass involving the deep soft tissue layer. The fat-suppressed T2-weighted magnetic resonance image showed a poorly marginated, homogeneous highsignal intensity mass. On further follow-up of 1 year and 6 months, the mass showed no change in size and nature. At surgery, a poorly demarcated inflammatory mass was dissected from a branch of the superficial radial nerve and excised completely. There was no recurrence and any dysfunction, except temporary localized hypoesthesia of
Introduction Kimura’s disease is a rare inflammatory disorder of unknown cause, primarily seen in young Asian males. The disease is characterized by a triad of painless subcutaneous masses in the head or neck region, blood and tissue eosinophilia, and elevated serum immunoglobulin E (IgE) levels [1]. We could not find any report of Kimura’s disease involving the nerve of extremity in children, except for two reports in adults [2,3]. The characteristics of the Kimura disease in childhood have not been well described. We report a case of Kimura’s disease involving the medial brachial cutaneous nerve of the arm in a child.
Case report A 7-year-old Korean boy was referred for a nontender mass in the left arm. He had a 5-year history of a poorly defined painless mass. There were no overlying skin changes. On physical examination, the mass was located at the medial side of the left elbow. It was movable and nontender. The overlying skin was unremarkable (Fig. 1). He experienced no alteration in the sensory and motor function of the left arm. Laboratory tests indicated peripheral eosinophilia of 29.8% (normal range: 0–7%). The serum IgE level was not determined. The ultrasonogram of the left arm indicated an ill-defined, lobulating solid mass involving the deep soft tissue layer. The fat-suppressed T2-weighted magnetic resonance image showed a poorly marginated, homogeneous high-signal intensity mass located in the subcutaneous fat layer proximal of the medial epicondyle (Fig. 2). With intravenous gadolinium enhancement, the mass was enhanced strongly and homogeneously. Perilesional edema and inflammatory change were 1060-152X © 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins
the medial aspect of the left arm at the 3.6-year followup. J Pediatr Orthop B 24:63–66 © 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins. Journal of Pediatric Orthopaedics B 2015, 24:63–66 Keywords: child, Kimura’s disease, nerve a Department of Orthopedic Surgery, Dongsan Medical Center, School of Medicine, Keimyung University and bDepartment of Pediatrics, School of Medicine, Yeungnam University, Korea
Correspondence to Kwang Soon Song, MD, PhD, Department of Orthopedic Surgery, School of Medicine, Dongsan Medical Center, Keimyung University, 56 Dalsungro, Jung-gu, Daegu 700-712, Korea Tel: + 82 53 250 7250; fax: + 82 53 250 7205; e-mails: [email protected] and [email protected]
observed. Needle biopsy for the mass was performed and the diagnosis of Kimura’s disease was established. On further follow-up of 1 year and 6 months, the mass showed no change in size and nature. At surgery, a poorly demarcated inflammatory mass was dissected from a branch of the superficial radial nerve and excised completely. On gross examination, a poorly demarcated mass was found that measured 7.5 × 3.0 × 2.7 cm in size (Fig. 3). The cutting surface of the mass was solid, slightly nodular, and pale pink to tan in color. No necrosis or hemorrhage was observed in the removed mass (Fig. 4). On microscopic examination, the mass was composed of numerous lymphoid follicles of variable sizes with germinal centers and infiltration of intervening inflammatory cells and associated with proliferation of blood vessels. Focal fibrosis was also observed. The intervening inflammatory cells were composed of mostly eosinophils and occasional lymphocytes and plasma cells. Fascicles of nerve bundles with perineurium were scattered within the mass (Figs 5 and 6). At the 3.5-year follow-up, there was no recurrence and any dysfunction, except temporary localized hypoesthesia of the medial aspect of the left arm.
Discussion Kimura disease is a chronic inflammatory disease, which frequently affects middle-aged Asian men, although children are seldom affected by it. Therefore, the characteristics of the Kimura disease of childhood have not been well described [3,4]. The cause of Kimura’s disease is unknown and many theories have been proposed [4–7]. The disease is characterized by a triad of painless subcutaneous masses in the head or neck region, blood and tissue eosinophilia, and markedly elevated serum DOI: 10.1097/BPB.0000000000000114
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
64 Journal of Pediatric Orthopaedics B 2015, Vol 24 No 1
IgE levels [1]. Kimura’s disease typically occurs in the head and neck region, but cases involving the limbs, trunk, scalp, and groin have been reported [2,8]. Two cases of Kimura’s disease involving the median nerve have been reported [2,3], but to our knowledge, there has been no similar case of Kimura’s disease involving a purely sensory nerve in a child. Kimura’s disease should be considered as a possible diagnosis when a partially or a poorly defined subcutaneous mass of high-signal intensity on T1-weighted and T2-weighted images with homogeneous enhancement, surrounding subcutaneous edema, and internal flow voids is seen in the medial epitrochlear region in an Asian individual, especially if accompanied by peripheral
eosinophilia [9]. Ultrasound scanning and MRI, used in combination, are the most effective imaging techniques, but may not differentiate Kimura’s disease from malignant tumors [3]. The diagnosis was made on the basis of the characteristic histopathologic findings after surgical excision in conjunction with peripheral eosinophilia [1]. The differential diagnosis is broad [3,6,7,10]. The main condition with which Kimura’s disease can and has been confused histologically is angiolymphoid hyperplasia with
Fig. 3
Fig. 1
The mass was located at the medial side of right distal humerus above the elbow. It was movable and nontender. The overlying skin was unremarkable.
Poorly demarcated mass was found that measured 7.5 × 3.0 × 2.7 cm in size.
Fig. 2
The fat-suppressed T2-weighted magnetic resonance image shows a poorly marginated, homogeneous high-signal intensity mass located in the subcutaneous fat layer proximal of the medial epicondyle.
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Kimura’s disease involving nerve in children Song and Woo 65
eosinophilia (ALHE), which itself most often involves the head and neck region and which has now been determined to be a separate condition [3].
Fig. 4
No cases of ALHE affecting a nerve have been described, but a case of Kimura’s disease, the lesions of which have repeatedly been confused with ALHE, has been reported involving the median nerve in an adult [10]. Several reports have described the modalities of treatment [2,4,5,11–19]. Complete surgical resection is the main form of treatment and the best modality of treatment [3,4,8]. Although Kimura’s disease is a rare chronic inflammatory disorder with a high rate of recurrence [4,8,11,19,20], Fig. 6
The cutting surface of the mass showed solid and nodular shape. No necrosis or hemorrhage was observed in the removed mass.
The mass consists of inflammatory infiltrates and hyperplastic lymphoid follicles (black solid arrow) with entrapped nerve fascicles (empty arrow) (H&E, × 20).
Fig. 5
A nerve fascicle (solid black arrow) is surrounded by Kimura’s disease (H&E, × 100) (a). The mass shows proliferation of blood vessels (solid black arrow) and dense infiltration of inflammatory cells, especially eosinophils (arrow head) (H&E, × 400) (b).
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
66 Journal of Pediatric Orthopaedics B 2015, Vol 24 No 1
fortunately, there was no recurrence or dysfunction on our case at the 3.5-year follow-up.
10
11
Acknowledgements Conflicts of interest
12
There are no conflicts of interest. 13
References 1 2 3 4
5
6 7 8 9
Shetty AK, Beaty MW, McGuirt WF Jr, Woods CR, Givner LB. Kimura’s disease: a diagnostic challenge. Pediatrics 2002; 110:e39. Lee YS, Ang HK, Ooi LL, Wong CY. Kimura’s disease involving the median nerve: a case report. Ann Acad Med Singapore 1995; 24:462–464. Vermaak PV, Lyons CB, Harley OJ. Kimura’s disease affecting the superficial branch of the radial nerve. J Hand Surg (Eur Vol) 2014; 39:208–209. Xu X, Fu J, Fang Y, Liang L. Kimura disease in children: a case report and a summary of the literature in Chinese. J Pediatr Hematol Oncol 2011; 33:306–311. Hernandez-Bautista V, Yamazaki-Nakashimada MA, Vazquez-García R, Stamatelos-Albarrán D, Carrasco-Daza D, Rodríguez-Lozano AL. Treatment of Kimura disease with intravenous immunoglobulin. Pediatrics 2011; 128: e1633–e1635. Chen H, Thompson LD, Aguilera NS, Abbondanzo SL. Kimura disease: a clinicopathologic study of 21 cases. Am J Surg Pathol 2004; 28:505–513. Punia RP, Aulakh R, Garg S, Chopra R, Mohan H, Dalal A. Kimura’s disease: clinicopathological study of eight cases. J Laryngol Otol 2013; 1–5. Viswanatha B. Kimura’s disease in children: a 9 years prospective study. Int J Pediatr Otorhinolaryngol 2007; 71:1521–1525. Park SW, Kim HJ, Sung KJ, Lee JH, Park IS. Kimura disease: CT and MR imaging findings. Am J Neuroradiol 2012; 33:784–788.
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15
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20
Martorell M, Pérez-Vallés A, García-García JA, Calabuig C, Aguilella L. Angiolymphoid hyperplasia with eosinophilia involving the cubital nerve. Acta Neuropathol 2004; 107:372–376. Abbas S, Jerjes W, Upile T, Vincent A, Hopper C. Treatment of Kimura disease with photodynamic therapy: a case study. Photodiagnosis Photodyn Ther 2012; 9:83–86. Choi JA, Lee GK, Kong KY, Hong SH, Suh JS, Ahn JM, et al. Imaging findings of Kimura’s disease in the soft tissue of the upper extremity. Am J Roentgenol 2005; 184:193–199. Baidya DK, Chhabra A, Raj R. Anesthetic management of a patient with Kimura’s disease for superficial parotidectomy. J Anaesthesiol Clin Pharmacol 2011; 27:383–385. Sato S, Kawashima H, Kuboshima S, Watanabe K, Kashiwagi Y, Takekuma K, Hoshika A. Combined treatment of steroids and cyclosporine in Kimura disease. Pediatrics 2006; 118:e921–e923. Liu S, Huang L, Zhang Z, Ke C, Qi J. A clinical analysis of 33 cases of Kimura’s disease. Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2011; 25:297–300. Chang AR, Kim K, Kim HJ, Kim IH, Park CI, Jun YK. Outcomes of Kimura’s disease after radiotherapy or nonradiotherapeutic treatment modalities. Int J Radiat Oncol Biol Phys 2006; 65:1233–1239. Soeria-Atmadja S, Oskarsson T, Celci G, Sander B, Berg U, Gustafsson B. Maintenance of remission with cyclosporine in paediatric patients with Kimura’s disease – two case reports. Acta Paediatr 2011; 100:e186–e189. Nakahara C, Wada T, Kusakari J, Kanemoto K, Kinugasa H, Sibasaki M, et al. Steroid-sensitive nephrotic syndrome associated with Kimura disease. Pediatr Nephrol 2000; 14:482–485. Kim GE, Kim WC, Yang WI, Kim SK, Oh WY, Suh HS, et al. Radiation treatment in patients with recurrent Kimura’s disease. Int J Radiat Oncol Biol Phys 1997; 38:607–612. Lin YY, Jung SM, Ko SF, Toh CH, Wong AM, Chen YR, et al. Kimura’s disease: clinical and imaging parameters for the prediction of disease recurrence. Clin Imaging 2012; 36:272–278.
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Kimura’s disease involving the peripheral nerve of the arm in a child: a case report Kwang Soon Songa and Da Eun Woob A 7-year-old Korean boy was referred for a nontender mass in the left arm. He had a 5-year history of a poorly defined painless mass on the medial side of the left elbow. The ultrasonogram of the left arm indicated an ill-defined, lobulating solid mass involving the deep soft tissue layer. The fat-suppressed T2-weighted magnetic resonance image showed a poorly marginated, homogeneous highsignal intensity mass. On further follow-up of 1 year and 6 months, the mass showed no change in size and nature. At surgery, a poorly demarcated inflammatory mass was dissected from a branch of the superficial radial nerve and excised completely. There was no recurrence and any dysfunction, except temporary localized hypoesthesia of
Introduction Kimura’s disease is a rare inflammatory disorder of unknown cause, primarily seen in young Asian males. The disease is characterized by a triad of painless subcutaneous masses in the head or neck region, blood and tissue eosinophilia, and elevated serum immunoglobulin E (IgE) levels [1]. We could not find any report of Kimura’s disease involving the nerve of extremity in children, except for two reports in adults [2,3]. The characteristics of the Kimura disease in childhood have not been well described. We report a case of Kimura’s disease involving the medial brachial cutaneous nerve of the arm in a child.
Case report A 7-year-old Korean boy was referred for a nontender mass in the left arm. He had a 5-year history of a poorly defined painless mass. There were no overlying skin changes. On physical examination, the mass was located at the medial side of the left elbow. It was movable and nontender. The overlying skin was unremarkable (Fig. 1). He experienced no alteration in the sensory and motor function of the left arm. Laboratory tests indicated peripheral eosinophilia of 29.8% (normal range: 0–7%). The serum IgE level was not determined. The ultrasonogram of the left arm indicated an ill-defined, lobulating solid mass involving the deep soft tissue layer. The fat-suppressed T2-weighted magnetic resonance image showed a poorly marginated, homogeneous high-signal intensity mass located in the subcutaneous fat layer proximal of the medial epicondyle (Fig. 2). With intravenous gadolinium enhancement, the mass was enhanced strongly and homogeneously. Perilesional edema and inflammatory change were 1060-152X © 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins
the medial aspect of the left arm at the 3.6-year followup. J Pediatr Orthop B 24:63–66 © 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins. Journal of Pediatric Orthopaedics B 2015, 24:63–66 Keywords: child, Kimura’s disease, nerve a Department of Orthopedic Surgery, Dongsan Medical Center, School of Medicine, Keimyung University and bDepartment of Pediatrics, School of Medicine, Yeungnam University, Korea
Correspondence to Kwang Soon Song, MD, PhD, Department of Orthopedic Surgery, School of Medicine, Dongsan Medical Center, Keimyung University, 56 Dalsungro, Jung-gu, Daegu 700-712, Korea Tel: + 82 53 250 7250; fax: + 82 53 250 7205; e-mails: [email protected] and [email protected]
observed. Needle biopsy for the mass was performed and the diagnosis of Kimura’s disease was established. On further follow-up of 1 year and 6 months, the mass showed no change in size and nature. At surgery, a poorly demarcated inflammatory mass was dissected from a branch of the superficial radial nerve and excised completely. On gross examination, a poorly demarcated mass was found that measured 7.5 × 3.0 × 2.7 cm in size (Fig. 3). The cutting surface of the mass was solid, slightly nodular, and pale pink to tan in color. No necrosis or hemorrhage was observed in the removed mass (Fig. 4). On microscopic examination, the mass was composed of numerous lymphoid follicles of variable sizes with germinal centers and infiltration of intervening inflammatory cells and associated with proliferation of blood vessels. Focal fibrosis was also observed. The intervening inflammatory cells were composed of mostly eosinophils and occasional lymphocytes and plasma cells. Fascicles of nerve bundles with perineurium were scattered within the mass (Figs 5 and 6). At the 3.5-year follow-up, there was no recurrence and any dysfunction, except temporary localized hypoesthesia of the medial aspect of the left arm.
Discussion Kimura disease is a chronic inflammatory disease, which frequently affects middle-aged Asian men, although children are seldom affected by it. Therefore, the characteristics of the Kimura disease of childhood have not been well described [3,4]. The cause of Kimura’s disease is unknown and many theories have been proposed [4–7]. The disease is characterized by a triad of painless subcutaneous masses in the head or neck region, blood and tissue eosinophilia, and markedly elevated serum DOI: 10.1097/BPB.0000000000000114
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
64 Journal of Pediatric Orthopaedics B 2015, Vol 24 No 1
IgE levels [1]. Kimura’s disease typically occurs in the head and neck region, but cases involving the limbs, trunk, scalp, and groin have been reported [2,8]. Two cases of Kimura’s disease involving the median nerve have been reported [2,3], but to our knowledge, there has been no similar case of Kimura’s disease involving a purely sensory nerve in a child. Kimura’s disease should be considered as a possible diagnosis when a partially or a poorly defined subcutaneous mass of high-signal intensity on T1-weighted and T2-weighted images with homogeneous enhancement, surrounding subcutaneous edema, and internal flow voids is seen in the medial epitrochlear region in an Asian individual, especially if accompanied by peripheral
eosinophilia [9]. Ultrasound scanning and MRI, used in combination, are the most effective imaging techniques, but may not differentiate Kimura’s disease from malignant tumors [3]. The diagnosis was made on the basis of the characteristic histopathologic findings after surgical excision in conjunction with peripheral eosinophilia [1]. The differential diagnosis is broad [3,6,7,10]. The main condition with which Kimura’s disease can and has been confused histologically is angiolymphoid hyperplasia with
Fig. 3
Fig. 1
The mass was located at the medial side of right distal humerus above the elbow. It was movable and nontender. The overlying skin was unremarkable.
Poorly demarcated mass was found that measured 7.5 × 3.0 × 2.7 cm in size.
Fig. 2
The fat-suppressed T2-weighted magnetic resonance image shows a poorly marginated, homogeneous high-signal intensity mass located in the subcutaneous fat layer proximal of the medial epicondyle.
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Kimura’s disease involving nerve in children Song and Woo 65
eosinophilia (ALHE), which itself most often involves the head and neck region and which has now been determined to be a separate condition [3].
Fig. 4
No cases of ALHE affecting a nerve have been described, but a case of Kimura’s disease, the lesions of which have repeatedly been confused with ALHE, has been reported involving the median nerve in an adult [10]. Several reports have described the modalities of treatment [2,4,5,11–19]. Complete surgical resection is the main form of treatment and the best modality of treatment [3,4,8]. Although Kimura’s disease is a rare chronic inflammatory disorder with a high rate of recurrence [4,8,11,19,20], Fig. 6
The cutting surface of the mass showed solid and nodular shape. No necrosis or hemorrhage was observed in the removed mass.
The mass consists of inflammatory infiltrates and hyperplastic lymphoid follicles (black solid arrow) with entrapped nerve fascicles (empty arrow) (H&E, × 20).
Fig. 5
A nerve fascicle (solid black arrow) is surrounded by Kimura’s disease (H&E, × 100) (a). The mass shows proliferation of blood vessels (solid black arrow) and dense infiltration of inflammatory cells, especially eosinophils (arrow head) (H&E, × 400) (b).
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
66 Journal of Pediatric Orthopaedics B 2015, Vol 24 No 1
fortunately, there was no recurrence or dysfunction on our case at the 3.5-year follow-up.
10
11
Acknowledgements Conflicts of interest
12
There are no conflicts of interest. 13
References 1 2 3 4
5
6 7 8 9
Shetty AK, Beaty MW, McGuirt WF Jr, Woods CR, Givner LB. Kimura’s disease: a diagnostic challenge. Pediatrics 2002; 110:e39. Lee YS, Ang HK, Ooi LL, Wong CY. Kimura’s disease involving the median nerve: a case report. Ann Acad Med Singapore 1995; 24:462–464. Vermaak PV, Lyons CB, Harley OJ. Kimura’s disease affecting the superficial branch of the radial nerve. J Hand Surg (Eur Vol) 2014; 39:208–209. Xu X, Fu J, Fang Y, Liang L. Kimura disease in children: a case report and a summary of the literature in Chinese. J Pediatr Hematol Oncol 2011; 33:306–311. Hernandez-Bautista V, Yamazaki-Nakashimada MA, Vazquez-García R, Stamatelos-Albarrán D, Carrasco-Daza D, Rodríguez-Lozano AL. Treatment of Kimura disease with intravenous immunoglobulin. Pediatrics 2011; 128: e1633–e1635. Chen H, Thompson LD, Aguilera NS, Abbondanzo SL. Kimura disease: a clinicopathologic study of 21 cases. Am J Surg Pathol 2004; 28:505–513. Punia RP, Aulakh R, Garg S, Chopra R, Mohan H, Dalal A. Kimura’s disease: clinicopathological study of eight cases. J Laryngol Otol 2013; 1–5. Viswanatha B. Kimura’s disease in children: a 9 years prospective study. Int J Pediatr Otorhinolaryngol 2007; 71:1521–1525. Park SW, Kim HJ, Sung KJ, Lee JH, Park IS. Kimura disease: CT and MR imaging findings. Am J Neuroradiol 2012; 33:784–788.
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Martorell M, Pérez-Vallés A, García-García JA, Calabuig C, Aguilella L. Angiolymphoid hyperplasia with eosinophilia involving the cubital nerve. Acta Neuropathol 2004; 107:372–376. Abbas S, Jerjes W, Upile T, Vincent A, Hopper C. Treatment of Kimura disease with photodynamic therapy: a case study. Photodiagnosis Photodyn Ther 2012; 9:83–86. Choi JA, Lee GK, Kong KY, Hong SH, Suh JS, Ahn JM, et al. Imaging findings of Kimura’s disease in the soft tissue of the upper extremity. Am J Roentgenol 2005; 184:193–199. Baidya DK, Chhabra A, Raj R. Anesthetic management of a patient with Kimura’s disease for superficial parotidectomy. J Anaesthesiol Clin Pharmacol 2011; 27:383–385. Sato S, Kawashima H, Kuboshima S, Watanabe K, Kashiwagi Y, Takekuma K, Hoshika A. Combined treatment of steroids and cyclosporine in Kimura disease. Pediatrics 2006; 118:e921–e923. Liu S, Huang L, Zhang Z, Ke C, Qi J. A clinical analysis of 33 cases of Kimura’s disease. Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2011; 25:297–300. Chang AR, Kim K, Kim HJ, Kim IH, Park CI, Jun YK. Outcomes of Kimura’s disease after radiotherapy or nonradiotherapeutic treatment modalities. Int J Radiat Oncol Biol Phys 2006; 65:1233–1239. Soeria-Atmadja S, Oskarsson T, Celci G, Sander B, Berg U, Gustafsson B. Maintenance of remission with cyclosporine in paediatric patients with Kimura’s disease – two case reports. Acta Paediatr 2011; 100:e186–e189. Nakahara C, Wada T, Kusakari J, Kanemoto K, Kinugasa H, Sibasaki M, et al. Steroid-sensitive nephrotic syndrome associated with Kimura disease. Pediatr Nephrol 2000; 14:482–485. Kim GE, Kim WC, Yang WI, Kim SK, Oh WY, Suh HS, et al. Radiation treatment in patients with recurrent Kimura’s disease. Int J Radiat Oncol Biol Phys 1997; 38:607–612. Lin YY, Jung SM, Ko SF, Toh CH, Wong AM, Chen YR, et al. Kimura’s disease: clinical and imaging parameters for the prediction of disease recurrence. Clin Imaging 2012; 36:272–278.
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
