Extensive inflammatory eosinophilic bladder tumors in children: experience with three cases.
Eosinopbilic cystitis, an uncommon lesion, is rare in children; <25 cases have been reported. The intense inflammatory changes in the bladder wall associated with this lesion may produce heaped-up excrescences, which resemble vesical vesical /ves·i·cal/ (ves´i-k'l) pertaining to the urinary bladder. Cf. cystic.
pertaining to or emanating from the urinary bladder. rhabdomyosarcoma rhabdomyosarcoma /rhab·do·myo·sar·co·ma/ (mi?o-sahr-ko´mah) a highly malignant tumor of striated muscle derived from primitive mesenchymal cells. . Our experience with 3 patients shows that the initial diagnosis of eosinophilic eosinophilic /eo·sin·o·phil·ic/ (-fil´ik)
1. readily stainable with eosin.
2. pertaining to eosinophils.
3. pertaining to or characterized by eosinophilia. cystitis may not be easily made, and that the lesions produced may mask other disease processes. We also report the fifth case of eosinophilic infiltration of the bladder occurring in association with chronic granulomatous disease Chronic Granulomatous Disease Definition
Chronic granulomatous disease (CGD) is an inherited disorder in which white blood cells lose their ability to destroy certain bacteria and fungi. .
EOSINOPHILIC CYSTITIS, an uncommon lesion, is rare in children; <25 cases have been reported. (1-5) The intense inflammatory changes in the bladder wall associated with this lesion may produce extensive, heaped-up excrescences, which resemble vesical rhabdomyosarcoma and for which the term inflammatory eosinophilic tumor has been recommended. (6) Three boys <5 years of age presented to our hospital over an 18-month period and were subsequently found to have vesical excrescences, histologic examination of which was consistent with a diagnosis of eosinophilic cystitis. One patient ultimately was proven to have chrornic granulomatous disease, which is of note, since the association of chronic granulomatous disease and eosinophilic infiltralion of the bladder has only been reported 4 previous times. (7-9) This report shows that the initial diagnosis of eosinophilic cystitis may not be easily made and that the lesions produced may mask other disease process.
Case 1. A 4-year-old boy presented with an episode of gross hematuria hematuria
Blood in the urine. It usually indicates injury or disease of the kidney or another structure of the urinary system or possibly, in males, the reproductive system. It may result from infection, inflammation, tumours, kidney stones, or other disorders. and dysuria dysuria /dys·uria/ (dis-u´re-ah) painful or difficult urination.dysu´ric
Difficult or painful urination. , preceded by a 1-month history of frequency and enuresis enuresis
Repeated urination into bedding or clothing, usually at night, in a normal child old enough to have completed toilet training. Enuresis may be voluntary or involuntary. It may run in families. . Initial laboratory tests showed no bacteria on urinalysis and a serum creatinine level of 0.4 mg/dL. An excretory ex·cre·to·ry
Of, relating to, or used in excretion.
pertaining to excretion.
see elimination behavior. urogram urogram /uro·gram/ (u´ro-gram) a film obtained by urography.
A radiograph of the urinary tract.
a radiograph obtained by urography. demonstrated bilateral ureteropyelocaliectasis to the level of the bladder, the most pronounced obstruction being noted on the left side, and an irregularity of the bladder wall. Two days later, his serum creatinine level had increased to 1.8 mg/dL (normal, 0.6-1.4 mg/dL), prompting the placement of a left-sided percutaneous nephrostomy. Magnetic resonance imaging magnetic resonance imaging (MRI), noninvasive diagnostic technique that uses nuclear magnetic resonance to produce cross-sectional images of organs and other internal body structures. of the bladder delineated a mass lesion on the posterior wall and floor of the bladder.
At cystoscopy Cystoscopy Definition
Cystoscopy (cystourethroscopy) is a diagnostic procedure that is used to look at the bladder (lower urinary tract), collect urine samples, and examine the prostate gland. , the trigone trigone /tri·gone/ (tri´gon)
2. the first three cusps of an upper molar tooth.
trigone of bladder vesical t. and posterior wall of the bladder were obscured by firm, pink, irregular excrescences that resembled rhabdomyosarcoma. Examination of frozen sections of the transurethral biopsies of the lesion did not yield a conclusive diagnosis; therefore, an implanted infusion port was placed. Permanent section evaluation showed a heavy infiltration of eosinophils Eosinophils
A leukocyte with coarse, round granules present.
Mentioned in: Histiocytosis X
eosinophils , confirming a diagnosis of eosinophilic cystitis. It was elected not to initiate any other treatment, but to perform serial nephrostograms at follow-up visits. Eight days following the bladder biopsy, a nephrostograrn demonstrated prompt drainage, and ultrasonography ultrasonography /ul·tra·so·nog·ra·phy/ (-so-nog´rah-fe) the imaging of deep structures of the body by recording the echoes of pulses of ultrasonic waves directed into the tissues and reflected by tissue planes where there is a change in showed greatly decreased ectasia of the collecting structures on the right side. One month following biopsy, ultrasound examination of the kidneys and bladder was normal, and the implanted infusion port was removed. The child has been asymptomatic for 3 years, and results of ultrasonography have been normal.
Case 2. A 7-month-old boy was admitted to our hospital with an 8-day history of increasing irritability and diarrhea. Serum creatinine level on admission was 2.7 mg/dL. Ultrasonography of the kidneys and bladder demonstrated a large bladder mass with marked bilateral ureteropyelocaliectasis and thin renal parenchyma Parenchyma
A ground tissue of plants chiefly concerned with the manufacture and storage of food. The primary functions of plants, such as photosynthesis, assimilation, respiration, storage, secretion, and excretion—those associated with living on the left side. With catheter drainage of the bladder, the serum creatinine level decreased to 1 mg/dL in 48 hours. Large, heaped-up excrescences of the posterior wall of the bladder and trigone were noted on cystoscopy. The ureteral ureteral
pertaining to or emanating from the ureter.
ureterectasis. orifices could not be identified. Malignancy could not be conclusively ruled out on examination of the frozen sections of transurethral biopsy specimens of the bladder lesions, so an implanted infusion port was placed. On examination of permanent sections, however, marked infiltration of the submucosa submucosa /sub·mu·co·sa/ (sub?mu-ko´sah) areolar tissue situated beneath a mucous membrane.
A layer of loose connective tissue beneath a mucous membrane. and muscularis by eosinophils was seen, establishing a diagnosis of eosinophilic cystitis. To allow for upper tract drainage and to permit removal of the catheter from the bladder, a right percutaneous nephrostomy was placed. No other treatment was instituted, and within 1 month, the vesical lesions were no longer identifiable on ultrasonography. The upper tracts, however, were still greatly dilated, and it seemed apparent that the massive dilatation was not a result of the transient lesions of eosinophilic cystitis. A voiding cystourethrogram showed bilateral grade V vesicoureteral reflux and posterior urethral valves Posterior urethral valves are an obstructive congenital defect of the male urethra. Classification
Posterior urethral obstruction was first classified by Hugh Hampton Young, urology pioneer, in 1919. . Three years later, following fulguration ful·gu·ra·tion
The destruction of tissue, usually malignant tumors, by means of a high-frequency electric current applied with a needlelike electrode. of the posterior ureteral valves and bilateral ureteral reimplantation, he continues to do well.
Case 3. A 4 Y2-year-old boy was referred for evaluation of dysuria that had occurred every 2 to 3 months during the previous 2 years. There had been no episodes of urinary tract infection urinary tract infection (UTI),
n infection in one or more of the structures that make up the urinary system. Occurs more often in women and is most commonly caused by bacteria. or gross hematuria, but he did have marked urgency and frequency. He occasionally had also been noted to have microscopic hematuria. He had been evaluated 1 week previously with voiding cystourethrography and renal and bladder ultrasonography. The bladder had been poorly filled on cystography, but the study had still suggested an irregularity of the posterior bladder wall, which was also noted on ultrasonography. For this reason, endoscopy of the bladder was performed. Surprisingly, the trigone was spared, but the posterior wall of the bladder was interspersed with blebs and excrescences suggestive of botyroid rhabdomyosarcoma. Examination of frozen sections of transurethral biopsies showed no evidence of malignancy, but there was evidence of cystitis. Permanent sections were examined and confirmed acute and chronic inflamma tion, and eosinophils were identified with Giemsa staining, resulting in a diagnosis of eosinophilic cystitis. No treatment was given. Bladder ultrasonography 3 weeks after the biopsy no longer showed any evidence of the irregularity of the bladder wall, and there was only minimal thickening. The child's irritative ir·ri·ta·tive
Adj. 1. irritative - (used of physical stimuli) serving to stimulate or excite; "an irritative agent"
irritating symptoms persisted, however, and were intermittently relieved with oxybutynin chloride treatment.
Fourteen months later, the patient presented with dysphagia. At that time, a partial obstruction of the esophagus was diagnosed as a manifestation of chronic granulomatous disease, confirmed by flow cytometry. Although ultrasound examinations of the bladder remain normal, he has intermittently developed pronounced episodes of dysuria and frequency that are quickly relieved with the administration of steroids.
The presentation and early clinical findings in children with eosinophilic cystitis have been well documented. (1-5) The most common initial complaints are hematuria, dysuria, and frequency. The etiology is unknown. (2) Imaging of the urinary tract with ultrasonography may demonstrate upper tract obstruction and extensive lesions of the bladder that mimic a tumor. The patients in cases 1 and 2 were exceptional, in that they were both noted to have rapidly rising serum creatinine levels and pronounced bilateral upper tract obstruction that necessitated a proximal diversion. Although the diagnosis is more straightforward if eosinophilic infiltration is noted in the vesical wall, it has been suggested that Qiemsa staining for eosinophils, or electron microscopy, if the eosinophils are deteriorating, be used to confirm the diagnosis. (2) In addition, inflammatory myofibroblastic tumors in children may, on occasion, contain extensive infiltration of eosinophils. (10) The initial pathologic diagnosis may be consist ent with malignancy, as noted by others, which will drive the early steps of management (as in our patients, who received implanted infusion ports) (6) As has been previously emphasized, eosinophilic cyscitis is self-limiting, and no specific therapy is needed. (1) Barring other contraindications, the use of steroid therapy has been recommended, due to the apparently more rapid resolution of symptoms and obstruction with this treatment. (2,11)
In retrospect, aspects of the management of the patients in cases 1 and 2 proved to be unnecessary. Eosinophilic cystitis does not lead the list of differential diagnoses, however, and our treatment course was primarily directed toward providing proximal drainage and preparing for management of a vesical malignancy. Because of the presence of brightly eosinophilic cells, as seen in rhabdomyosarcoma, the frozen section examinations in cases 1 and 2 did not rule out that a malignancy was not present. Biopsy of the bladder can rule out rhabdomyosarcoma and the clinician should observe that an apparent eosinophilic cystitis with or without tumefaction tumefaction /tu·me·fac·tion/ (-fak´shun) swelling.
1. The act or process of puffing or swelling.
2. A swollen condition.
3. may be a manifestation of chronic granulomatous disease. Our experience also shows that the upper tract dilatation seen in conjunction with eosinophilic cystitis may have more than one cause.
(1.) Sutphin M, Middleton AW Jr: Eoisnophilic cystitis in children: a self-limited process. J Urol 1984; 132:11-119
(2.) Axelrod SL, Ring KS, Collins MH, et al: Eosinophilic cystitis in children. Urology 1991; 37:549-552
(3.) Sujka SK, Fisher JE, Greenfield SP: Eosinophilifc cystitis in children. Urology 1992; 40:262-264
(4.) Gerharz EW, Grueber M, Melekos MD, et al: Tumor-forming eosinophilic cystitis in children, case report and review of literature. Eur Urol 1994; 25:138-141
(5.) van den Ouden D: Diagnosis and management of eosinophilic cystitis. a pooled analysis of 135 cases. Eur Urol 2000; 37:386-394
(6.) Netto JMB, Perez LM, Kelly DR, et al: Pediatric pediatric /pe·di·at·ric/ (pe?de-at´rik) pertaining to the health of children.
Of or relating to pediatrics. inflammatory bladder tumors: myofibroblastic and eosinophilic subtypes. J Urol 1999; 162:1424-1429
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Resembling a tumor made of granular material. cystitis as a manifestation of chronic granulomatous disease of childhood. J Urol 1973; 110:357-359
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(10.) Albores-Saavedra J, Manivel JC, Essenfeld H, et al: Pseudo-sarcomatous myofibroblastic proliferations in the urinary bladder of children. Cancer 1990; 66:1234-1241
(11.) LaDocsi LT, Sullivan B, Hanna MK: Eosinophilic granulomatous cystitis in children. Urology 1995; 46:732-735
RELATED ARTICLE: KEY POINTS
* Eosinophilic cystitis is a rare lesion in children.
* The resultant inflammatory change produces heaped up excrescences, which resemble rhabdomyosarcoma.
* Chronic granulomatous disease may be associated with eosinophilic infiltration of the bladder.
* Eosinophilic cystitis is self-limiting and no specific therapy is needed.
* Steroid therapy may give a more rapid resolution of symptoms and obstruction.
From the Departments of Urology and Pathology, University of Arkansas The University of Arkansas strives to be known as a "nationally competitive, student-centered research university serving Arkansas and the world." The school recently completed its "Campaign for the 21st Century," in which the university raised more than $1 billion for the school, used College of Medicine and Arkansas Children's Hospital Arkansas Children's Hospital, an affiliate of the University of Arkansas for Medical Sciences, is the only pediatric medical center in Arkansas and one of the largest in the United States, serving children from birth to age 21. , Little Rock.
Reprint requests to John F. Redman, MD, Department of Urology, University of Arkansas College of Medicine, 4301 W Markham, Slot 540, Little Rock, AR 72205-7199.