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Multiple Sites of Kimura’s Disease Occuring Along
Multiple Peripheral Nerves in the Same Patient:
A Case Report
Kumaran Rasappan, Vaikunthan Rajaratnam,
Chin Teck Yew, Lang Tee Ung
Introduction
 There can be multiple differential diagnosis for asymptomatic
swellings in the limbs
 Radiological imaging guide identification of the lump
 However, radiological diagnosis may not always be accurate
 We report a case of an asymptomatic swelling in a patient
who’s histological diagnosis differed from radiological
diagnosis
History
 25 year old Chinese male
 Noticed swelling over
medial aspect of left elbow
incidentally
 4 months duration
 Noted it getting slightly
bigger over the last month
 Asymptomatic
Examination
 2 x lumps over anterior medial
aspect of elbow and distal arm
 Non tender, firm, deep
 Slightly mobile transversely but
not mobile longitudinally
 Tinnel’s negative
 Completely asympthomatic
Source:
http://img05.deviantart.net/3a9f/i/2
011/148/e/8/hanging_right_arm_by
_treeclimber_stock-d3hfvaj.png
• (1.5 x 1.0 x 2 cm) Lump
• Close relation to the
medial cutaneous nerve
of forearm
Axial Saggital
Coronal
Source:
http://img05.deviantart.net/3a9f/i/2
011/148/e/8/hanging_right_arm_by
_treeclimber_stock-d3hfvaj.png
• (3.5 x 0.7 x 1.5 cm) Lump
• Following the course of
the median nerve
Axial Saggital
Coronal
Source:
http://img05.deviantart.net/3a9f/i/2
011/148/e/8/hanging_right_arm_by
_treeclimber_stock-d3hfvaj.png
• (1.3 x 1.3 x 2.6cm) Lump
• Close relation to the median nerve and/or
anterior interosseous nerve
Axial Coronal
Source:
http://img05.deviantart.net/3a9f/i/2
011/148/e/8/hanging_right_arm_by
_treeclimber_stock-d3hfvaj.png
Compatible with multiple peripheral nerve sheath tumours
Source:
http://img05.deviantart.net/3a9f/i/2
011/148/e/8/hanging_right_arm_by
_treeclimber_stock-d3hfvaj.png
Source:
http://img05.deviantart.net/3a9f/i/2
011/148/e/8/hanging_right_arm_by
_treeclimber_stock-d3hfvaj.png
Source:
http://img05.deviantart.net/3a9f/i/2
011/148/e/8/hanging_right_arm_by
_treeclimber_stock-d3hfvaj.png
Histological Images
Preserved nodal
architecture with
reactive follicular
hyperplasia
Intermediate power
Histological Images
Germinal centre
hyperplasia
Intermediate power
Histological Images
Interfollicular
eosinophilia
Intermediate power
Histological Images
Eosinophilic
mirco abscess
with folliculolysis
and necrosis
Intermediate power
Histological Images
Intermediate power
The findings are representative of Kimura's Disease
Kimura’s disease / Eosinophilic
Lymphogranuloma
 First described in 1937 in the Chinese literature by HT Kimm and C Szeto
 Definitive histological description published in 1948 by Kimura et al
 Endemic in Asia (China and Japan) but rare
 Only about 200 cases reported since 1948
 M/F sex ratio 7 : 3.5
 Peak age incidence is during 3rd
decade
 DDx: Angiolymphoid hyperplasia
with eosinophilia
Clinical presentation of Kimura’s Disease
 Usual location: Head and Neck
 Accompanied by satellite adenopathies with hypertrophy of salivary glands
 Nodules can be pruritic or painful but the overlying skin is normal
 Extra membranous glomerulonephritis is found in half of pts
 Cutaneous eosinophilic vasculitis has been described
 Epitrochlear, axillary, inguinal and popliteal lesion have been described
 Eyes, ears, sperm ducts and nerves
very rarely affected
Our Case
 EMBASE, PubMed, CINAHL and Google Scholar were used
 Only 4 cases involving the peripheral nerves were found
 2 case reports describe involvement of the superficial radial nerve
 1 case report describes involvement of the median nerve
 1 case report describes involvement of the ulnar nerve
 No case reports of Anterior Interosseous Nerve or medial cutaneous
nerve of forearm
 No case reports of multiple peripheral nerve involvement in the same
patient
 First such finding of 3 locations of
Kimura’s Disease in 3 different peripheral
nerves in the same patient.
Take Home Message
 MRI for multiple unknown lumps in limb
 Take MRI report only as a guide
 Benign Peripheral Nerve Sheath Tumour
(BPNST) vs Kimura’s Disease
BPNST vs Kimura’s Lymph Node
Source: http://www.columbianeurosurgery.org/conditions/neurofibroma/
References
 Song KS, Woo DE. Kimura’s disease involving the peripheral nerve of the arm in a child:
a case report. Journal of Pediatric Orthopaedics B. 2015 Jan 1;24(1):63-6.
 Vermaak PV, Lyons CB, Harley OJ. Kimura’s disease affecting the superficial branch of
the radial nerve. Journal of Hand Surgery (European Volume). 2014 Feb 1;39(2):208-9.
 Lee YS, Ang HK, Ooi LL, Wong CY. Kimura's disease involving the median nerve: a case
report. Annals of the Academy of Medicine, Singapore. 1995 May;24(3):462-4.
 Martorell M, Pérez-Vallés A, García-García JA, Calabuig C, Aguilella L. Angiolymphoid
hyperplasia with eosinophilia involving the cubital nerve. Acta neuropathologica. 2004
Apr 1;107(4):372-6.
 Larroche C, Bletry O. Kimura's disease. Orphanet encyclopedia, February. 2005 Feb.
THANK YOU

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Multiple Sites of Kimura’s Disease Along Peripheral Nerves

Editor's Notes

  1. 3 distinct lumps within left arm 1st Lesion (1.5 x 1 x 2 cm ): Lying over the lower medial aspect of arm in close relation to the medial cutaneous nerve of the forearm 2nd Lesion (3.5 x 0.7 x 1.5 cm ): Adjacent to 1st lump following the course of the median nerve 3rd Lesion (1.3 x 1.3 x 2.6cm): Within flexor compartment of forearm in close relation to the median nerve and/or anterior interosseous nerve All  the  lesions  have  identical  signal  characteristics  with  high  T2  and  intermediate  T1  signal.    They  enhance  homogeneously  post  contrast. Compatible with multiple peripheral nerve sheath tumours
  2. 3 distinct lumps within left arm 1st Lesion (1.5 x 1 x 2 cm ): Lying over the lower medial aspect of arm in close relation to the medial cutaneous nerve of the forearm 2nd Lesion (3.5 x 0.7 x 1.5 cm ): Adjacent to 1st lump following the course of the median nerve 3rd Lesion (1.3 x 1.3 x 2.6cm): Within flexor compartment of forearm in close relation to the median nerve and/or anterior interosseous nerve All  the  lesions  have  identical  signal  characteristics  with  high  T2  and  intermediate  T1  signal.    They  enhance  homogeneously  post  contrast. Compatible with multiple peripheral nerve sheath tumours
  3. 3 distinct lumps within left arm 1st Lesion (1.5 x 1 x 2 cm ): Lying over the lower medial aspect of arm in close relation to the medial cutaneous nerve of the forearm 2nd Lesion (3.5 x 0.7 x 1.5 cm ): Adjacent to 1st lump following the course of the median nerve 3rd Lesion (1.3 x 1.3 x 2.6cm): Within flexor compartment of forearm in close relation to the median nerve and/or anterior interosseous nerve All  the  lesions  have  identical  signal  characteristics  with  high  T2  and  intermediate  T1  signal.    They  enhance  homogeneously  post  contrast. Compatible with multiple peripheral nerve sheath tumours
  4. 3 distinct lumps within left arm 1st Lesion (1.5 x 1 x 2 cm ): Lying over the lower medial aspect of arm in close relation to the medial cutaneous nerve of the forearm 2nd Lesion (3.5 x 0.7 x 1.5 cm ): Adjacent to 1st lump following the course of the median nerve 3rd Lesion (1.3 x 1.3 x 2.6cm): Within flexor compartment of forearm in close relation to the median nerve and/or anterior interosseous nerve All  the  lesions  have  identical  signal  characteristics  with  high  T2  and  intermediate  T1  signal.    They  enhance  homogeneously  post  contrast. Compatible with multiple peripheral nerve sheath tumours
  5. Intra op findings: 2 x 2cm nerve sheath tumour adjacent to median nerve just proximal to cubital fossa 1 x 2cm tumour arising from lateral cutaneous nerve of arm just proximal to cubital fossa all tumours arising from nerve sheaths. 1 x 1cm tumour arsing just posterior to Anterior Interosseous Nerve (AIN) at AIN branching junction from median nerve in flexor compartment of forearm All tumours surrounding but not involving main nerves
  6. Intra op findings: 2 x 2cm nerve sheath tumour adjacent to median nerve just proximal to cubital fossa 1 x 2cm tumour arising from lateral cutaneous nerve of arm just proximal to cubital fossa all tumours arising from nerve sheaths. 1 x 1cm tumour arsing just posterior to Anterior Interosseous Nerve (AIN) at AIN branching junction from median nerve in flexor compartment of forearm All tumours surrounding but not involving main nerves
  7. Intra op findings: 2 x 2cm nerve sheath tumour adjacent to median nerve just proximal to cubital fossa 1 x 2cm tumour arising from lateral cutaneous nerve of arm just proximal to cubital fossa all tumours arising from nerve sheaths. 1 x 1cm tumour arsing just posterior to Anterior Interosseous Nerve (AIN) at AIN branching junction from median nerve in flexor compartment of forearm All tumours surrounding but not involving main nerves
  8. All the three lumps are lymph nodes Preserved nodal architecture with reactive follicular hyperplasia Prominent eosinophilic infiltrate in the interfollicular and sinusoidal areas, with infiltration into perinodal tissue Eosinophilic infiltration of germinal centres resulting in folliculolysis and necrosis and Eosinophilic microabscesses present No evidence of malignancy.
  9. All the three lumps are lymph nodes Preserved nodal architecture with reactive follicular hyperplasia Prominent eosinophilic infiltrate in the interfollicular and sinusoidal areas, with infiltration into perinodal tissue Eosinophilic infiltration of germinal centres resulting in folliculolysis and necrosis and Eosinophilic microabscesses present No evidence of malignancy.
  10. All the three lumps are lymph nodes Preserved nodal architecture with reactive follicular hyperplasia Prominent eosinophilic infiltrate in the interfollicular and sinusoidal areas, with infiltration into perinodal tissue Eosinophilic infiltration of germinal centres resulting in folliculolysis and necrosis and Eosinophilic microabscesses present No evidence of malignancy.
  11. All the three lumps are lymph nodes Preserved nodal architecture with reactive follicular hyperplasia Prominent eosinophilic infiltrate in the interfollicular and sinusoidal areas, with infiltration into perinodal tissue Eosinophilic infiltration of germinal centres resulting in folliculolysis and necrosis and Eosinophilic microabscesses present No evidence of malignancy.
  12. All the three lumps are lymph nodes Preserved nodal architecture with reactive follicular hyperplasia Prominent eosinophilic infiltrate in the interfollicular and sinusoidal areas, with infiltration into perinodal tissue Eosinophilic infiltration of germinal centres resulting in folliculolysis and necrosis and Eosinophilic microabscesses present No evidence of malignancy.
  13. Unknown etiology Chronic inflammatory process ? Aberrant immune reaction to an unknown antigenic stimulus ? Genetic Mast cells play important role by regulating IgE synthesis and orchestrating eosinophilic infiltration