March 2021 — Hematopathology Case 1
A 74-year-old man is being evaluated for a 2.5 cm well-circumscribed right parotid mass with accompanying regional lymphadenopathy. He has a history of an unspecified immune deficiency disorder, chronic lymphocytic leukemia (diagnosed five years prior), and recurrent MSSA abscesses and cellulitis in the chin, scalp, and chest regions.
Based on morphology from an excisional biopsy of the right parotid gland, what is the most likely diagnosis?
- Castleman disease
- Kimura disease
- Angiolymphoid hyperplasia with eosinophilia (ALHE)
- Hodgkin disease, mixed cellularity type
The correct answer is ...
The diagnosis in this case is: Kimura disease.
Correct answer: Kimura disease
Kimura disease is a chronic inflammatory disorder that involves subcutaneous tissues and lymph nodes predominantly in the head and neck region and is characterized by angiolymphoid proliferation and eosinophilia. Diffuse eosinophilia, eosinophilic microabscesses, and infiltration of germinal centers, sometimes resulting in folliculolysis, are part of the process. Vascular hyperplasia may be pronounced. Described in 1948, the disease is endemic in Asia an sporadic in the West. Kimura disease is suggestive of an infectious origin, but no pathogen has been demonstrated.
Incorrect: Hodgkin disease, mixed cellularity type
Has in common with Kimura disease eosinophils, plasma cells, and sclerosis, but lacks the hyperplastic germinal centers and deposits of IgE. The presence of Reed-Sternberg cells determines a positive diagnosis.
Incorrect: Angiolymphoid hyperplasia with eosinophilia (ALHE)
Owing to histologic similarities, ALHE has been confused with or mistaken for an early stage of Kimura disease. In contrast with Kimura disease, which is common in Asians, predominantly males, and seated deep in subcutaneous tissues, ALHE is seen in Caucasians, more often in females, involves the superficial skin forming clusters of papules, and is characterized by hypertrophic endothelial cells protruding into or occluding the vascular lumina. More important, the lymphadenopathy is an essential part of Kimura disease and is not present in ALHE.
Incorrect: Castleman disease
This entity includes vascular hyperplasia but lacks eosinophilia and has involuted hyalinized rather than hyperplastic germinal centers.
- Chen, Hong, et al. "Kimura disease: a clinicopathologic study of 21 cases." The American journal of surgical pathology 28.4 (2004): 505-513.
- Hui PK, Chan JK, Ng CS, Kung IT, Gwi E. Lymphadenopathy of Kimura's disease. The American journal of surgical pathology. 1989 Mar 1;13(3):177-86.
- Googe, P. B., N. L. Harris, and M. C. Mihm Jr. "Kimura's disease and angiolymphoid hyperplasia with eosinophilia: two distinct histopathological entities." Journal of cutaneous pathology 14.5 (1987): 263-271.
Mazen Atiq, M.B.B.S.
Resident, Anatomic and Clinical Pathology
Ellen McPhail, M.D.
Associate Professor of Laboratory Medicine and Pathology
Mayo Clinic College of Medicine and Science