Long-term survival after radical cystectomy and mesenteric lymph node dissection for squamous cell carcinoma arising from augmented bladder with lymph node metastasis: a case report

Abstract

Surgical resection is commonly performed for augmented bladder cancer, yet an optimal treatment strategy for augmented bladder cancer with lymph node metastasis has not been established. Here, we report a case that achieved 7 years of survival after radical cystectomy and mesenteric lymph node dissection for squamous cell carcinoma arising from augmented bladder with lymph node metastasis. Extended surgery could be a useful treatment option for locally advanced augmented bladder cancer including mesenteric lymph node metastasis.

Introduction

It is known that cancer often develops long after augmentation cystoplasty [1, 2]. While the dominant histological types are adenocarcinoma and urothelial carcinoma, squamous cell carcinoma (SCC) is also seen in some cases [3]. For augmented bladder cancer, surgical resection is commonly performed, yet an optimal treatment strategy for augmented bladder cancer with lymph node metastasis has not yet been established. For augmented bladder cancer arising from the ileal segment, treatment strategies for small intestine cancer can be applied. According to the current guidelines on small intestine cancer [4, 5], a recommended treatment for resectable disease is up-front radical surgical resection including the mesenteric lymph nodes and adjacent organs if needed. The need for adjuvant chemotherapy is still controversial.

Here, we report a case of SCC arising from the ileal segment of an augmented bladder with mesenteric lymph node metastasis, diagnosed 47 years after augmentation cystoplasty.

Case report

A 73-year-old man who had undergone left nephrectomy and augmentation cystoplasty for left renal and bladder tuberculosis at the ages of 27 and 29, respectively, presented with gross hematuria in 2008. Urine cytology, cystoscopy, computed tomography, and magnetic resonance imaging revealed no malignant findings at that time. For the next 4 years, he received regular follow-up examinations. In May 2012, urine cytology turned positive, suggesting SCC. Although the native bladder tissue that could be observed by cystoscopy was intact, an augmented bladder tumor with a mesenteric lymph node swelling of 1.5 cm was identified by computed tomography (Fig. 1 a, b). In magnetic resonance images, the tumor demonstrated low signal intensity in T2-weighted images, high signal intensity in diffusion-weighted images, and low signal intensity in apparent diffusion coefficient map images (Fig. 1 c–e), which indicated malignant findings. Therefore, the patient was diagnosed with augmented bladder cancer with mesenteric lymph node metastasis. Several serum tumor markers were negative, though the SCC level was slightly elevated at 1.8 ng/mL.

Fig. 1.

Radiological findings. Contrast enhanced computed tomography of a augmented bladder tumor (white arrowhead), and b a swelling mesenteric lymph node (yellow arrowhead), and magnetic resonance images of augmented bladder tumor (white arrowhead); c T2-weighted image, d diffusion-weighted image, and e apparent diffusion coefficient map

In September 2012, radical cystectomy with mesenteric lymph node dissection and right ureterocutaneostomy was performed. The ileal segment of augmented bladder firmly adhered to the adjacent small intestine, where direct invasion was suspected. Therefore, en bloc resection of the augmented bladder, mesenterium of the ileal segment including the mesenteric lymph nodes, and the adherent intestine was carried out.

Macroscopically, a sessile tumor of 2.8 cm was located on the ileal segment, while the native bladder including the anastomotic site was intact (Fig. 2a, b). A lymph node of 1.5 cm in the mesenterium was swelling.

Fig. 2.

Macroscopic and microscopic findings. a The resected specimen; the native bladder (lower part), the ileal segment of the augmented bladder (central part), and the adherent intestine (right upper part). b The tumor (arrowheads) of 2.8 cm located on the ileal segment. c Well- to poorly-differentiated SCC proliferating with keratinization. d Squamous metaplasia in the ileal segment apart from the tumor

Histologically, a well-to poorly-differentiated SCC infiltrated into the subserous layer of the ileal segment (Fig. 2c) and involved the swelling mesenteric lymph node. The other resected nodes were negative for cancer. Apart from the cancer lesion, squamous metaplasia was observed in the ileal segment of the augmented bladder (Fig. 2d), and the neck of the native bladder. Mild mucosal atrophy and lymphocyte infiltration were observed in the native bladder, which suggested chronic inflammation. The resected adjacent intestine was free from cancer invasion, while dense fibrous tissue was observed between the intestine and the augmented bladder.

After surgical site infection had been resolved with conservative treatments including hyperbaric oxygen therapy, antibiotic therapy, debridement, and wound cleansing, the patient was discharged on hospital day 57 with the ureteral catheter removed. No adjuvant treatments were given thereafter.

To date, the patient has received careful follow-up and has survived for 7 years without any evidence of recurrence including elevation of serum tumor markers and with good renal function and quality of life.

Discussion

We experienced a case of SCC arising from the ileal segment of an augmented bladder with mesenteric lymph node metastasis. SCC arising from augmented bladder is rare and no treatment strategy has been established for cases with lymph node metastasis. In the present case, 7 years of recurrence-free survival have been achieved after extended surgery alone.

When intestinal bacteria are exposed to urine through augmentation cystoplasty, they generate inflammatory substances such as N-nitrosamine, which can cause chronic inflammation [2]. This chronic inflammation can induce squamous metaplasia [6] and facilitate carcinogenesis [7] as observed in the present case. Cell-to-cell interaction is also considered to be a mechanism of the development of augmented bladder cancer [1, 8]. While some investigators have reported that cancer can develop long after augmentation cystoplasty at a constant rate, Higuchi et al. have reported that augmentation cystoplasty is not associated with cancer development [9]. Therefore, further studies are needed to clarify the etiology and epidemiology of augmented bladder cancer.

Given the low annual incidence of augmented bladder cancer and the high cost of cystoscopy, annual screening cystoscopy is not recommended for patients receiving augmentation cystoplasty [10, 11], although the procedure does confer a long-term risk of cancer development. In the present case, it took 4 years to obtain the diagnosis of augmented bladder cancer, since the patient had marked hematuria despite regular follow-up examinations including cystoscopy, urine cytology, and imaging studies. Some surgical techniques used in augmentation cystoplasty might complicate the anatomy of the augmented bladder and obscure any cancer, which would result in delayed diagnosis and advanced stage at the time of diagnosis. When patients receiving augmentation cystoplasty complain of hematuria or present positive urine cytology, repeated careful examinations should be carried out with the possibility of augmented bladder cancer in mind. Functional imaging studies such as diffusion-weighted magnetic resonance imaging and positron emission tomography would be useful to diagnose the augmented bladder cancer that was difficult to be diagnosed by conventional examinations including cystoscopy and computed tomography.

In general, augmented bladder cancer, of which the dominant histological types are adenocarcinoma and urothelial carcinoma, has poor prognosis because of the high incidence of advanced stage at the time of diagnosis [12], although the prognosis of SCC arising after augmentation cystoplasty has not been well studied due to its rarity [1]. Surgery plays a central role in treatments for augmented bladder cancer. Systemic chemotherapy is also used in advanced cases [1], although, to our knowledge, no regimens including adjuvant and neoadjuvant therapy have been established. In the present case, we performed extended surgery, including resection of the augmented bladder and the adherent intestine and dissection of the mesenteric lymph nodes that were regional nodes of the small intestine according to the small intestine cancer guidelines, because the tumor was located on the ileal segment of the augmented bladder. Partial resection of the augmented bladder for non-urothelial carcinoma might be oncologically safe. However, careful consideration must be required for the application of bladder preservation, because it would raise concerns about reduced bladder capacity. Adjuvant chemotherapy was not given, since the efficacy of adjuvant chemotherapy has not been established to date. Although the latest version of the National Comprehensive Cancer Network guidelines recommend adjuvant chemotherapy for node-positive disease [4], the evidence supporting this is scant and the National Cancer Institute guidelines do not mention adjuvant chemotherapy [5]. An ongoing clinical trial evaluating the potential benefits of adjuvant chemotherapy for small intestine cancer will answer this clinical question [13]. However, close follow-up should be planned irrespective of adjuvant chemotherapy.

Conclusion

We reported a case of SCC arising from the ileal segment of an augmented bladder with mesenteric lymph node metastasis, which we treated with radical cystectomy and mesenteric lymph node dissection. The patient has survived for 7 years.

Extended surgery could be a treatment option for locally advanced augmented bladder cancers including mesenteric lymph node metastasis.