Squamous cell carcinoma in a long-standing suprapubic cystostomy: a urological oddball

Abstract

Squamous cell carcinoma (SCC) rarely accounts for 2–5% of urinary bladder cancer. Chronic irritation of the bladder from infection or indwelling catheter is a risk factor for SCC. Only a handful of cases of suprapubic cystostomy (SPC) site SCC have been reported and have been always seen in paraplegics or urethral stricture requiring long-standing catheterization. We report a case of a 69-year-old male with an indwelling (SPC) of 25 years duration for a urethral stricture who presented with a fungating growth around the SPC site enveloping the catheter confirmed to be squamous cell carcinoma. Imaging revealed involvement of abdominal wall and urinary bladder with no metastatic spread. He underwent wide local excision (WLE) of the lesion with radical cystectomy and ileal conduit reconstruction with bilateral ilio-inguinal lymphadenectomy and pedicled anterolateral thigh flap for abdominal wall reconstruction. He received adjuvant radiotherapy and was found to be recurrence free at 3 months. Among the cases previously reported, 9 of the 12 cases were treated surgically with 4 of those combined with partial cystectomy and 4 with radical cystectomy. SPC site SCC tends to be localized around the catheter and into the abdominal tract and wide surgical excision offers the best hope for cure. Regular cystoscopy and biopsy from bladder around the catheter site can help early detection and the treatment should be personalized to each patient.

Introduction

Urinary bladder (UB) is the most common site of lower urinary tract cancers. Majority of UB carcinoma arises from the transitional cell epithelium and is called as urothelial carcinoma (UC) which accounts for 90–95% of the cases [1]. Squamous cell carcinoma (SCC) of the urinary bladder is rare and hardly amounts to 2–5% of all cases [2]. Chronic irritation of the bladder from infection or indwelling catheter is a risk factor for SCC. Long standing catheter (> 10 years) has been shown to cause squamous metaplasia in up to 80% patients [3]. Invasive SCC can arise in this metaplastic tissue or arise de novo. We report here a case of an elderly gentleman with a history of indwelling suprapubic cystostomy of 25 years which developed into a squamous cell carcinoma.

Case report

A 69-year-old male presented with an indwelling long-standing suprapubic cystostomy (SPC). He was treated 25 years earlier for a urethral stricture and underwent SPC with stricture dilatation. The records were not available, but the surgery failed, leaving the patient with a permanent SPC in situ. Over the past 3 months, he noticed a lesion growing around the stoma site which was gradually progressive and associated with localized pain and hematuria. There were no associated systemic complaints. Clinical examination demonstrated a 7 × 5 cm fungating growth around the SPC site enveloping the catheter (Fig. 1). Biopsy from the lesion revealed a squamous cell carcinoma. Computerized tomography (CT) showed an ill-defined enhancing infiltrative mass of 48 × 50 × 60 mm involving the soft tissues of the abdominal wall circumferentially around the SPC site infiltrating skin, subcutaneous tissue, muscles with extension in pelvic cavity into anterior wall of urinary bladder (Fig. 2). Positron emission tomographic (PET) scan revealed no regional lymphadenopathy or distant metastasis. Cystoscopic evaluation was omitted due to imaging confirmation of bladder involvement and inability to pass the scope due to urethral stricture. Urine cytology was attempted but due to persistent hematuria, the results were inconclusive.

Fig. 1.

Fungating growth around the SPC site enveloping the catheter

Fig. 2.

Contrast-enhanced CT showing the ill-defined enhancing mass involving the soft tissues of the abdominal wall circumferentially around the SPC site infiltrating skin, subcutaneous tissue, muscles with extension into anterior wall of urinary bladder

He underwent wide local excision (WLE) of the lesion with radical cystectomy (Fig. 3) and ileal conduit reconstruction with bilateral ilio-inguinal lymphadenectomy. The anterior abdominal wall defect was large and was closed with a pedicled anterolateral thigh (ALT) flap (Fig. 4). The duration of surgery was 280 min and the approximate blood loss was 300 ml. There was no clinical evidence of lymphadenopathy or other intra-abdominal metastasis. The tumor was found to be involving the suprapubic skin and urinary bladder with adequate negative margins (Fig. 5). Surgical histopathology revealed malignant epithelial tumor showing poorly differentiated squamous cells in sheets and islands with high mitotic activity and minimal keratin pearl formation (Fig. 6). Bilateral inguinal and iliac lymph nodes were found to be free of tumor. As per the decision of the multidisciplinary tumor board meeting, in view of poorly differentiated and squamous cell histology, the patient was referred for adjuvant radiotherapy (50.4 Gy in 25 fractions though EBRT) which was tolerated well, and he was found to be recurrence free at 3 months post-treatment.

Fig. 3.

En bloc wide local excision of the SPC tract with radical cystectomy. Bilateral ureters have been catheterized for ileal implantation later

Fig. 4.

Post-operative picture with ileal conduit and anterolateral thigh flap cover for abdominal wall

Fig. 5.

Surgical specimen showing tumor involving the SPC site and tract with extension into urinary bladder

Fig. 6.

Histopathology showing malignant epithelial tumor showing poorly differentiated squamous cells in sheets and islands with high mitotic activity and minimal keratin pearl formation

Discussion

Urinary bladder cancer is the most common urothelial malignancy and UC accounts for 90–95% of the bladder carcinoma cases. SCC of the urinary bladder (UB-SCC) is typically known to arise due to chronic inflammation of the bladder and was the most frequent variety (60% of cases) in the developing and underdeveloped nations due to bilharzial infections. However, it forms a small section of bladder cancer cases in recent times where the etiology can be attributed to chronic irritation and inflammation due to urinary retention in paraplegics, BOO, bladder diverticula, chronic indwelling catheterization and neurogenic bladder, bladder calculi and recurrent cystitis [1, 2].

UB-SCC is potentially more aggressive than UC with a dismal 5-year survival of 35–48%. It is mostly high grade with muscle invasion [1]. The most frequent cause of mortality is locoregional recurrence especially in the pelvis. Radical cystectomy (RC) is the treatment of choice as it provides good locoregional control when combined with pelvic lymphadenectomy. Adjuvant radiation therapy (RT) is indicated in high-risk cases such as T3, T4 tumors, positive lymph node metastasis and lymphovascular invasion.

UC classically responds to chemotherapy and the M-VAC (methotrexate, vinblastine, adriamycin and cisplatin) regimen is standard of care, in contrast to SCC which is chemoresistant. Combination therapy with cisplatin and gemcitabine has been tried and found to have some effect in these cases. Cisplatin and doxorubicin have been used as first-line therapy in metastatic SCC with irinotecan as second line, but none has been found to be effective. Among EGFR-positive cases, cetuximab can be used in combination with platinum agents with modest benefit [4].

A suprapubic cystostomy is a safe and effective technique of urinary diversion which provides a non-continent, direct bladder drainage in cases of an inaccessible urethra. It may be performed safely via an open approach using a trocar or a percutaneous technique with a Seldinger wire [5].

The first case of a SPC site SCC was reported in an 80-year-old male with a 5-year duration of SPC for urethral stricture in 1993 [6]. The second reported case was seen in a 50-year-old paraplegic with 25 years SPC [7]. The former patient had the lesion confined to the tract without bladder involvement, whereas the latter lesion extended into the bladder. The longest reported duration by Metke et al. was 50 years where the patient was treated surgically with excision and partial cystectomy [8]. Surgical excision is the preferred treatment option, with wide excision of the tract and abdominal wall in cases where the bladder is spared, partial excision of the bladder dome when the lesion is abutting the bladder, and a radical cystectomy (RC) when the bladder mucosa is involved [9].

Shreyas N et al. reported a case where a SPC of 1 year duration for urethral stricture developed SCC after being SPC free for 2 years. He was also treated similarly with RC and ALT flap cover. The patient, however, developed local recurrence after 2 months and expired after 3 months [10]. Vertical rectus abdominis muscle (VRAM) flap is another reconstructive option for abdominal wall reconstruction [11]. In patients where a flap reconstruction is not feasible, an abdominoplasty can be tried to close the defect primarily [12]. Ito et al. reported a case where the tumor around SPC site had metastasized to the inguinal and para-aortic lymph nodes and the patient was treated with palliative RT [13].

Only a handful of cases of SPC site SCC have been reported in the literature over the past 3 decades and have had various extent of bladder involvement and spread but have been almost always seen in paraplegics or urethral stricture requiring long-standing catheterization. Among the cases previously reported, 9 of the 12 cases were treated surgically with 4 of those combined with partial cystectomy and 4 with radical cystectomy (Table 1). 3 patients required a flap cover for abdominal wall reconstruction, ALT in 2 and VRAM in one case. One patient received neoadjuvant RT and 3 were treated with palliative RT.

Table 1.

Published cases of squamous cell carcinoma of the suprapubic cystostomy tract

Year	Author	Age	Duration of SPC (years)	Indication for SPC	Bladder involvement	T stage	Treatment	Survival
1993	Stroumbakis et al. [6]	80	5	Urethral stricture	−	T3	Radiation followed by tract WLE and partial cystectomy	N/A
1995	Stokes et al. [7]	50	25	Paraplegia	+	T4	WLE with radical cystectomy	Death at 8 months
1999	Schaafsma et al. [9]	63	37	Urethral stricture	−	T3	WLE	Death at 5 months of pneumonia
2011	Ito et al. [13]	58	35	Paraplegia	+	T4	Palliative radiation	Alive at 6 months
2012	Min Chung et. al [18]	56	9	Urethral stricture	+	T4	Radiation	Death at 6 months
2015	Boaz et al. [14]	65	3 months	Urethral stricture	Concurrently with urethral carcinoma	T3	WLE with radical cystoprostatourethrectomy and chemotherapy	Alive at 6 months
2017	Subramaniam et al. [11]	88	25	Urethral stricture	+	T4	WLE + VRAM	Alive at 6 months
2018	Khadhouri et al. [12]	53	20	paraplegia	+	T4	WLE with partial cystectomy	Alive at 8 months
2022	Metke et al. [8]	71	50	Paraplegia	−	T3	WLE with partial cystectomy	N/A
2022	Metke et al. [8]	81	20	BOO	−	T3	Palliative EBRT	N/A
2023	Sawazaki et al. [4]	61	34	Paraplegia	+	T4	WLE with partial cystectomy	Death at 14 months
2023	Shreyas N et al. [10]	63	1 year SPC. 2 year SPC free	Urethral stricture	+	T4	WLE with radical cystectomy + ALT flap	Recurrence at 2 months
2024	Present study	69	25	Urethral stricture	+	T4	WLE with RC + ALT flap	Recurrence free at 3 months

WLE wide local excision, RC radical cystectomy, BOO bladder outlet obstruction, VRAM vertical rectus abdominis myocutaneous flap, ALT anterolateral thigh flap, EBRT external beam radiotherapy

Although most cases of SPC site malignancy have been seen to arise in the setting of a long-term in situ catheter, Boaz et al. reported a case where a SPC site tumor was seen with concurrent urethral carcinoma with a short duration of 3 months [14]. They hypothesized this was likely due to transdermal lymphatic or retrograde urinary spread. SCC has been reported in various parts of the urinary tract and is commonly seen in the urethra. Rare instance of SCC presenting in a long-standing perineal urethrostomy has been reported which was managed with radical resection with en block cystoprostatectomy [15]. Another case of SCC presenting in a patient who previously underwent bladder exstrophy repair was also treated with radical cystectomy and ileal conduit with pedicled ALT cover [16].

MRI is a preferred option to assess the tumor depth and bladder invasion. However, in our case, the tumor was not localized to the bladder but involved the abdominal wall and the tract. In addition, gross extensive involvement of the UB was evident on the CT scan. Hence, MRI was not deemed necessary for further staging or management. Biopsy from the tract growth was done and revealed SCC. As there was gross invasion of the bladder, cystoscopy was not deemed necessary as it would not change the management plan. Due to involvement of lower abdominal wall, inguinal dissection and for bladder involvement, pelvic node dissection was done. Total 25 nodes were harvested, all of which were negative for metastatic disease. As the disease invaded the SPC tract with no perineal urethral involvement, complete urethrectomy was not necessary. The case was not a straightforward bladder cancer but rather a squamous cell cancer of a chronic tract. Hence, the TNM staging is not appropriately applicable in this case. However, if it is considered along lines of urinary bladder staging, it would be pT4bN0M0. Advanced urothelial cancer staged T3 or higher should be subjected to neoadjuvant chemotherapy. But in our case, the histology was a squamous cell carcinoma where there is no proven role of chemotherapy and RT is indicated in high-risk cases [17].

SPC site SCC tends to be localized around the catheter and into the abdominal tract at the time of diagnosis and wide surgical excision offers the best hope for cure. Regular cystoscopy and biopsy from bladder around the catheter site may be followed in such patients for early detection. The treatment is mostly anecdotal and comes from various reports and experiences and should be personalized to each patient.

Conclusion

Although infrequent, this rare complication of SPC site developing into squamous cell carcinoma has been reported in the literature and is a known entity. Surgeons and physicians dealing with such patients with a long-term indwelling SPC should be wary of the chronic irritation and possibility of malignant transformation. A thorough examination and regular follow-up of the SPC site will help in early detection and treatment in these patients.

Author contributions

Rajat Choudhari: conceptualization, writing—original draft, writing review and editing, visualization. Kamal Kishor Lakhera: writing review and editing. Suresh Singh: writing review and editing. Pinakin Patel: writing review and editing. Naina Kumar: writing review and editing. Yashasvi Patel: writing review and editing.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Data availability

The data that support the findings of this study are available from the corresponding author upon reasonable request.

Declarations

Conflict of interest

The authors declare no conflict of interest.

Ethical approval

The Sawai Man Singh medical college, Jaipur Research and Ethical committee have confirmed that no ethical approval is required for case reports.

Consent to participate

Well-informed consent was taken from the participant. No identifiable information was included in the manuscript.