February 2014

Chronic Inguinal Soft Tissue Masses Presenting in an Otherwise Asymptomatic Vietnamese Male

Tiffany Minehart MS4;  Brian Jones MS4; James Carrier MD; Jeffrey Stead, MD

Overview

 A 47 year old Vietnamese male presented with bilateral inguinal soft tissue masses which had been present for many years.  He denied fever and night sweats, and had not experienced any weight loss.  Physical examination was significant for a large apparent lipomatous mass of the right groin and suspected lymphadenopathy in the left groin. Laboratory studies included a white blood cell count of 15.7 x 109/L with 68% eosinophils, total protein 8.8gm/dL (6.0 – 8.0) , albumin 3.9g/dL (3.2 – 5.0), alkaline phosphatase 139 IU/L  (35 -120) , AST 21U/L (0 – 45), ALT 20 IU/L (0 – 63).  Computerized topography showed extensive lymphadenopathy in the right inguinal region and pelvis which involved both external and internal iliac nodes.  Excisional biopsy of the right sided mass yielded a 12 x 5 x 8 cm portion of firm, rubbery tan yellow to tan brown soft tissue. A 3.5 x 3 x 2.8 cm similar soft tissue mass was excised from the left groin.  Histologically, these were lymph nodes and surrounding tissues.

Gross Description

Histopathological features are illustrated below.

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Figure 1: Hyperplastic lymphoid follicles, one with progressive transformation.
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Figure 2: Follicular hyperplasia with numerous eosinophils.
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Figure 3: Sheets of eosinophils.
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Figure 4: Eosinophilic microabscess.
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Figure 5: Charcot-Leyden crystals in macrophages and stroma.
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Figure 6: Warthin-Finkeldy type giant cells in hyperplastic follicle.

Diagnosis

Based upon the illustrated findings, the most appropriate pathologic diagnosis is :

Answer

Please select an answer above.

Discussion

Kimura Disease is a benign inflammatory disease of lymph nodes and subcutaneous soft tissues that is seldom reported in the United States. Etiology is unknown.  Prevalence is highest in middle aged men of Asian descent [2, 5].  Clinically, the most common presentation is a soft tissue mass with regional lymph node involvement and no constitutional symptoms.  Because this is a chronic disease, many patients will describe an extended disease course or a mass that has been present for many years. The most common site is the head and neck; however, the inguinal region and lymph nodes in other areas may also be involved [1].

Eosinophilia and elevated serum IgE are distinctive features, and may assist in diagnosis if Kimura Disease is suspected [4].  Surgical excision or biopsy is necessary for definitive diagnosis and to rule out a malignancy.  Although this disease is benign, there is a high rate of local recurrence after surgical excision [3].   The tissue excised from this patient demonstrated lymph node hyperplasia with eosinophilia, sheets of eosinophils , eosinophilic microabscesses, Charcot-Leyden Crystals in fibrotic stroma and Warthin-Finkeldy type giant cells in hyperplastic follicles [1]. These histologic features, in addition to the clinical presentation of this patient, are characteristic of Kimura Disease.  

Angiolymphoid hyperplasia with eosinophilia, perhaps more than any other lesion, is apt to be confused with Kimura disease; indeed, the two were formerly grouped together.  Both contain eosinophils and tend to involve the head and neck.  The former is a vascular tumor, a variant of epithelioid hemangioma, which involves the skin and subcutis and may be accompanied by lymphoid aggregates.  The latter is a reactive condition involving subcutaneous tissues and lymph nodes.  Peripheral blood eosinophilia and increased serum IgE are common in Kimura disease, but not angiolymphoid hyperplasia with eosinophilia.  While Kimura Disease has a distinctive predilection for middle aged oriental males, angiolymphoid hyperplasia with eosinophilia does not. 

In Kimura Disease, histopathological examination of surgically excised specimens commonly reveals most or all of the following :

  1. Preserved lymph nodal architecture
  2. Lymph node follicular hyperplasia with reactive germinal centers
  3. Eosinophilic infiltrates in lymph node sinuses, internodal spaces, perinodal and subcutaneous soft tissue     
  4. Germinal centers with eosinophils, proteinaceous deposits, vascularization, and/or necrosis
  5. Warthin-Finkeldy type giant cells, mostly in follicles
  6. Eosinophilic microabscesses
  7. Postcapillary venule proliferation
  8. Stromal fibrosis
  9. Perivenular sclerosis
Differential diagnosis for Kimura Disease includes:
  1. Hodgkin Lymphoma
  2. Angioimmunoblastic T-cell lymphoma
  3. Castleman disease
  4. Parasitic infection
  5. Florid follicular hyperplasia
  6. Angiolymphoid hyperplasia with eosinophilia (smaller more superficial lesions, no predominance for Asian males)
  7. Dermatopathic lymphadenitis

Conclusion

The differential diagnosis for a given clinical presentation can vary in different parts of the world.  For example, Kimura Disease is more likely to be considered in the differential diagnosis of lymphadenopathy in Asian countries, where it is endemic, than in the United States.  However, as more people around the world work or travel outside of their own country, the likelihood that diseases may be detected in unusual geographical locations increases.  Regardless of geographic location, it is beneficial to be aware of the distinctive characteristics of Kimura Disease in order to differentiate the presenting features from malignant, hypersensitivity, or infectious causes.  This case is a classical example of Kimura Disease, typically seen in the Asian countries, diagnosed in a community hospital setting in West Virginia.

References

  1. Abdul-Haiji M, Hurford MT. Kimura Disease. Arch Pathol Lab Med. 2007, 131: 650-651.
  2. Chen H, Thompson LDR, et al. Kimura Disease: A Clinicopathologic Study of 21 Cases. Am J Surg Pathol. 2004, 28 (4): 505-513.
  3. Chim CS, Fung A et al. Analysis of Clonality in Kimura Disease. Am J Surg Pathol.  2002, 26(8): 1083-1086.
  4. Hsi ED. Hematopathology: Foundations in Diagnostic Pathology2007, Ch. 4.
  5. Wanat KA. Kimura Disease. Medscape Reference.2010