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             Malakoplakia of Ear


 

                
Rare cases of Malakoplakia have been reported in the middle ear.

Malakoplakia (from the Greek malacos, soft, and placos, plaques) is a rare granulomatous disease that occurs commonly in the urinary tract. It was first described by Michaelis and Gutmann in 1902.

Other organs like the genito-urinary tract, testis, epididymis, lymph nodes, nasopharynx , tonsil and retroperitoneal tissue have been also involved though less frequently.

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Pathogenesis:  1) Microorganisms might play a role in the pathogenesis. The organisms include Escherichia coli (found in more than two thirds of cases), Mycobacterium tuberculosis , Proteus ,and Staphylococcus aureus. Specific bacteria are found in certain categories of patients, for example, coliform bacteria in patients with chemotherapy and Rhodococcus equi in patients with acquired immunodeficiency syndrome. 2) An abnormal or altered immune response has been also implicated in the pathogenesis. 3) The third hypothesis is an abnormal macrophage response because of defective lysosomal function. It is suggested that macrophages in malakoplakia are capable of phagocytosis but unable to digest the bacteria. Partially digested bacteria accumulate in monocytes or macrophages and lead to the deposition of calcium and iron on residual bacterial glycolipid.

Gross features: Grossly, malakoplakia can present as soft tan yellow plaques and nodules or even extensive bands. The lesion is usually solitary but can be multiple

Microscopic features: Histologically, it is defined by sheets of histiocytes (Hansemann cells) with accumulation of granular basophilic periodic acid-Schiff - positive, diastase-resistant inclusions and calcified Michaelis-Gutmann bodies, which are pathognomonic but not necessary for diagnosis.  The composition of Michaelis-Gutmann bodies has been shown to be 94.6% organic and 5.4% inorganic, with the inorganic components being calcium, phosphorus, and iron.

                  

Malakoplakia outside the urinary tract.:  Arch Pathol Lab Med.2007 Feb;131(2):297-300.

CONTEXT: Malakoplakia is a rare granulomatous disease that occurs commonly in the urinary tract. Histologically, it is defined by sheets of histiocytes (Hansemann cells) with accumulation of granular basophilic periodic acid-Schiff-positive, diastase-resistant inclusions and calcified Michaelis-Gutmann bodies, which are pathognomonic but not necessary for diagnosis. In addition to the urinary tract, malakoplakia has been reported in other organs, including the gastrointestinal tract, central nervous system, female genital tract, and the tongue. OBJECTIVES: To review the literature of reported sites of malakoplakia outside the urinary tract and their variable clinical presentations, and to discuss the main diagnostic features and differential diagnoses of malakoplakia. The pathogenesis and possible etiologic factors are also presented. DATA SOURCES: Data for this work were collected from the published literature, textbooks, and the Internet. CONCLUSIONS: It is important to be aware of the existence of this entity in abnormal locations. In many situations, malignancy can be mimicked, especially when the lesion is ulcerated and is accompanied by lymph node involvement. Misinterpreting large, rapidly growing nodules of malakoplakia as tumor might lead to overstaging. Pathologists should be also aware of the possibility of malakoplakia coexisting with other lesions, such as tuberculosis and carcinoma, in the same specimen.

Malakoplakia of the middle ear. Histopathology.1991 Sep;19(3):276-8.

Malakoplakia of middle ear: a case report. Histopathology.1983 Jan;7(1):129-34

Case presentation of malacoplakia of the middle ear. Acta Otorrinolaringol Esp.1995 Jul-Aug;46(4):315-6.

Malakoplakia is a chronic inflammatory process usually located in the urinary bladder but it may occasionally affect other organs. It is a rarely found in ENT cases. Such a case was diagnosed and treated in our department. Possible pathogenesis is also discussed.

Malakoplakia of the temporal bone in a nine-month-old infant. J Laryngol Otol.1991 Jul;105(7):568-70.

A case of malakoplakia, of the temporal bone in a nine-month-old male child is reported. The lesion presented as an aural polyp, associated with a lower motor neuron facial palsy. On exploration, the granuloma was noted to involve the temporal bone, eroding the bony labyrinth. It was successfully treated with surgical debridement, and antibiotics. A review of the relevant literature is presented.


November 2007

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