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| CASE REPORT |
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| Year : 1991 | Volume
: 37
| Issue : 2 | Page : 115-6 |
Neurogenic tumor of the urinary bladder (a case report).
Sane SY, Garpure SS, Borwankar SS
Department of Pathology, K.E.M. Hospital, Parel, Bombay, Maharashtra.
Correspondence Address:
Department of Pathology, K.E.M. Hospital, Parel, Bombay, Maharashtra.
Neurogenic tumors of urinary bladder are rare. A ganglionure fibroma arising from the trigons of urinary bladder in two year old boy presenting with hematuria, is described here.
How to cite this article:
Sane S Y, Garpure S S, Borwankar S S. Neurogenic tumor of the urinary bladder (a case report). J Postgrad Med 1991;37:115 |
Neurogenic tumours are very rare in the urinary bladder. We had under our care a two-year-old boy with neurogenic tumour of bladder. The same case is presented here with review of relevant literature.
A 2-year-old boy presented with hematuria for 6 days. There was no history of pyuria/dysuria/or trauma. On general examination, he was an afebrile thin child with frontal bossing and dental caries. Six Cafe-au-Lait spots were observed on the skin. Family history revealed, father having neurofibromatosis. His blood pressure was 100/60 mm of Hg. Abdominal examination, revealed a lump in the hypogastrium and hepatomegaly of 2 cm. Investigations revealed haemoglobin of 11.5 gm% with a white blood count of 8,300/cm the differential count showed polymorphs 55% and lymphocytes 45%. Bone marrow was normal. Biochemical investigations were found to be normal. Urine routine showed RI3Cs, and in urine culture Klebsiella were grown (as contaminants). Urinary V. M. A. levels were found to be marginally elevated 20.90 mg/g (normal 1-10 mg/g).
X-ray chest showed a left paravertebral soft tissue mass showing indentation laterally. Ultrasonography showed a mass in the urinary bladder arising from posterior wall. Intravenous pyclography showed both kindneys, pelvicalyceal system and ureters to be normal. The fundus of the bladder showed a indentation.
Cystoscopy showed a polypoidal fleshy, globular mass under the mucosa of the posterior bladder wall. A wedge biopsy of the mass was done after exploration. The mass was adherent to the posterior wall of the bladder extending upto trigone. Mucosa was normal. The child was discharged against medical advice without further treatment.
Pathology:
A firm wedge 1 x 0.5 cm in size was received. Sections from the biopsy showed part of thinned out mucosa on one surface with underlying tumor. The tumor was composed of thick and thin nerve bundles separated by fibrous tissue. There were large bundles of spindle cells, showing palisaded nuclei representing schwann cells. Some areas showed ganglion cell differentiation (See [Figure - 1]) and formation of verrucae bodies like nerve endings. Small clusters of neuroblast like cells were present. There were no pigment-laden cells. The diagnosis of neurogenic tumor urinary bladder or differentiated ganglioneuro-fibroma was given.
Neurogenic tumors are very rare in the urinary bladder and only a few have been reported. Presentation of this type of tumor may be of two types[5] (a) due to hormone disturbances like elevation of blood pressure during micturation and (b) vesicle complaints like hematuria, urgency etc. In our case the child presented with bladder complaints viz. hematuria, There were no hormonal disturbances though on investigation the child was found to have elevated VMA levels.
The patient may have other signs of the neurognic tumors like manifestation of Ven Reckling Hausens disease. In our case apart from the tumor in the bladder the only external manifestation was, Cafe-Au-Lait spots and also the father was a known case of neurofibromatosis. Para-vertebral soft tissue mass seen on X-ray chest could be another nurogenic tumor, but it was not biopsied. It has been seen that pubertyl may be a precipitating factor for skin involvement. However, cutaneous manifestation are present only in 40%[1],[2] of patients with bladder involvement. The males are more commonly affected with ratio of 3:1[3]. The tumors may be solitary or multiple [7],[2]. The mucosa overlying the tumor is generally normal indicating that the tumor arises in the submucosa. Presence of tumor in the muscle coat, vascular spaces or islands of neuroblast like cells are not valid of the biological behaviour of the tumor. The presence of tumor in the muscle coat[4] is suggestive of origin of the tumor rather than infiltration.
Trigone of urinary bladder derives cells from neural crest origin and neoplasms of such remnant may differentiate as paraganghoma and/or neurofibromatous tissues[6]. Our case showed various stage of maturation from neuroblast to ganglion cells, nerve-fibres nerve endings. So it was labelled as ganglionuro-fibroma.
We thank the Dean, Seth GS Medical College, Parel, Mumbai, for allowing this material to be published.
| 1. |
Clark S, Marlett M, Prudencio RF, Dasgupta TK. Neurofibrornatosis of bladder in children, case report and literature review. J Urol 1977; 118:654-656.  |
| 2. | Gonzalez AA, Reyes HA. Neurofibromatosis involving the lower urinary tract. J Urol 1963; 89:804-811. |
| 3. | Kramer SA, Burett DM, Utz DC. Neurofibromatosis of bladder in children. J Urol 1981; 126:693-694. |
| 4. | Mittal BV, Sane SY. Pathological diagnosis, J Applied Med 1988; 14:65-66. |
| 5. | Pugh RCB, Gresham, GA, Mullancy J. Pheochromocytoma of the urinary bladder, J Pathol & Bacteriol 1960; 79:89-107. |
| 6. | Tsokos M, Scrupa S, Ross R, Triche T. Differentiation of neuroblastoma recapitulates neural crest development. Amer J Pathol 1987; 128:484-496. |
| 7. | Winfield HN, Catalona WJ. An isolated plexiform neurofibroma of the bladder. J Urol 1985; 126:693-694.
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