Genito urinary Malakoplakia : a 14 case retrospective study.

Malacoplakia is a rare disease with pseudo tumoral lesions of nonphagocyted coliform germs due to microtubules macrophagic alterations.The disease is characterized by specific Von Hansemanncells granuloma with eosinophilic cytoplasm within whichthere are P.A.S. positive Michaelis-Gutmann calcifications.Urological localizations are the most frequent, mainly in the bladderand genitals. Colon and rectal localizations are possible.We did a multicentric and retrospective study of fourteen patients (9men, 5 women), mean age was 62 year old (37 to 85). The localizationwas unique on prostate, epididymis and bladder or extensive onureter and bladder with five cases with dilatation of upper urinarytract and impairment of renal function.Results of treatments were evaluated with a 4 to 120 month followup.Medical treatment : antibiotics were used in 14 cases. Bethanecholchloride (UrecholineR or MyocholineR) was used in 5 cases on 14.In two extensive cases the patients were cured.Surgical treatment : localized disease on prostate and epididymiswere cured with endoscopic treatment, excision and drainage in associationwith medical treatment.In cases with upper urinary tract dilatation, endo urological treat -ment with per cutaneous nephrostomy tube and uni or bilateral double« J » stents and medical treatment obtained normalization andimprovement of renal function.On 14 cases, one was lost of follow-up and 13 cases were curedwithout non evolutive disease. Two patients died from their evolut -ive cancer without malacoplakia. Two female patients are still undertreatment with antibiotic and bethanechol chloride; bladder biopsieswere negative.Frequency of malacoplakia is underestimated. Factors as intercurrentimmuno suppressive disease or corticoids therapy have beenreported. The most frequent germ is Escherichia Coli. On pathophysiology,the respective role of cholinergics on cyclic-GMP andadrenergic drugs increasing intracellular cyclic-AMP are discussed.The decrease of intra-cellular c-GMP / c-AMP ratio has been observedin malacoplakia and in Chediak-Higashi disease. Cholinergicagonists revamp the intra cellular c- GMP / c-AMP ratio, increasingtreatment efficacy. Isolated testis and prostatic localizations are rare with 50 reportedcases : a 4 week antibiotic therapy usually cured the patients.Isolated and extensive bladder localization with upper urinary tractdilatation impaired renal function. The prognosis of lower urinarytract lesions is favourable on long-term antibiotic therapy. In severecases, large excisions have been described. In one case with multipleureteral stenoses, percutaneous nephrostomy tube, double « J »stent with long term antibiotic therapy and bethanechol chloride,one female patient was cured without any toxicity. It is difficult tomake dose and length treatment recommendations. Long term follow-up is mandatory because of the risk of recurrence.Medical treatment of malacoplakia is based on cotrimoxazoletrimethoprime or fluoroquinolones therapy; bethanechol chlorideeffect has to be precised. Three patients groups can be distinguished:- in chronic infected patients, the disease can be cured with antibiotictherapy in association with surgical excision or endourologicalprocedures, depending on the localization of the disease.- in immunodepressive patients, corticoid therapy is a factor of impairmentof the disease and must be stopped. In association with antibiotics,surgery is efficient.- in patients with cancer, the prognosis is under control of the neoplasmeven if malakoplakia has been controlled with antibiotic therapy.