Ali Akbar Zehri ( Department of Surgery, Urology Section, Aga Khan Hospital, Dar es Salaam, Tanzania. )
Athar Ali ( Department of General and Laparoscopic Surgery, Aga Khan Hospital, Dar es Salaam, Tanzania. )
Farah Iqbal ( Department of Radiology, Aga Khan Hospital, Dar es Salaam, Tanzania. )
Muthoni Jessca ( Aga Khan Health Services, Aga Khan Hospital, Dar es Salaam, Tanzania. )
A case of huge primary leiomyoma of the ureter in which nephroureterectomy was performed is presented. To the best of our knowledge, this case is a unique form of leiomyoma of the ureter due to its large size. There have been only twelve cases of primary leiomyoma of the ureter reported since 1955 and eleven of them were very small and one was big in size but smaller than the present case. Our case is considered to be the thirteenth.
Keywords: Leiomyoma, Ureteral neoplasm, Hydronephrosis.
Leiomyomas of the genitourinary tract are rare, and the ureter is an uncommon site for them.1 Renal leiomyoma, a slow growing benign neoplasm, can arise from the capsule or peripelvic tissues and less often from the renal vein. Since 1955 Leighton et al.2 reported leiomyoma of the ureter, and now 12 cases have been reported to date. This case will be the 13th. This is a unique form of leiomyoma of the ureter due to its large size. Ureteral leiomyoma often causes hydronephrosis, making it difficult to diagnose. Therefore, total nephroureterectomy is performed because of the possible diagnosis of malignancy. As the technology of ureteroscopy has advanced in recent years, the possibility of a malignant tumour can be excluded by preoperative examination, increasing the number of reports of cases in which the kidney was preserved.3 We experienced a huge primary leiomyoma of the ureter in which nephroureterectomy was performed. The present report describes this case together with a review of published literature.
Ureteral leiomyoma as Non-epithelial benign ureteral tumour of mesodermal origin is a rare disease.1 According to our research, only three cases in Japan and nine cases in other countries have been reported since the case report by Leighton et al. in 1955,2,4-10 making the present case the 13th (Table 1). Most of the cases were in patients aged 24-60 years except for one case, in an infant, including the present case 32 years. Location of the lesion in six cases was left side of ureter and six on the right side. Eight cases were in men and five in women. The sites of the lesion were in the upper, middle and lower parts in six, three and four cases, respectively. There were no significant differences in the location of the lesion, sex and site of development.
Exact mechanism of development of ureteric leiomyoma is not clear, however inflammation, chronic stimulation, occlusion and trauma are suspected reasons. In a couple of cases, there was history of ureterolithiasis, but not in the present case. Ikota et al. reported a diffuse leiomyoma of the ureter as a complication of multiple endocrinoma (MEN) type 1.10 Shailesh et al, reported that if preoperative studies and intraoperative findings strongly suggest a benign lesion, some urologists recommend a ureterotomy and biopsy of the lesion for frozen section with either excision of the tumour or segmental ureteral resection.11 However, the decision to violate strict principles of cancer operation in an effort to save the involved kidney is a difficult one. Such conservative approach is probably justified in a child if the ipsilateral kidney function is normal. In adults with a poorly functioning kidney, as was the present case who also had non functioning kidney on nuclear scan, nephroureterectomy was the logical option Nouralizadeh et al.reported the second largest leiomyoma of ureter which was diagnosed preoperatively and partial ureterectomy was done.12
In MEN type 1, the complication of multiple leiomyoma is recognized in a variety of organs including the oesophagus, stomach, lungs, uterus and skin, but this case was the only one to develop in the ureter. It has been suggested that the MEN type 1-associated gene may have a causal relationship to multiple leiomyoma. In most of the cases diagnosis was made by diagnostic imagings such as excretory urography, retrograde urography or computed tomography (CT) and urinary cytology, as for other ureteral tumours, but no characteristic findings were encountered.3 Surgical treatment was performed in twelve out of 13 cases. Seven cases were treated by Nephroureterectomy even without defferential diagnosis of benign or malignant tumour before surgery including the present case. Partial ureterectomy preserving the kidney was performed in only four cases and ureterectomy was done in one case. It has become possible to diagnose benign tumours by preoperative examination as a result of advancements in ureteroscopic technology. It is important to pay careful consideration to the preservation of renal function but in the present case, nuclear scan showed non functioning left kidney.
Leiomyomas of the genitourinary tract are rare and the ureter is an uncommon site for them. Ureteral leiomyoma often causes hydronephrosis, making diagnosis difficult. As the technology of ureteroscopy has advanced in recent years, the benign nature of the mass can be excluded
If the preoperative suspicion of ureteric tumour is made otherwise, the nephroureterectomy is the logical option.
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