Surgical Oncology
Volume 21, Issue 1 , Pages 1-5, March 2012

Peritoneal pseudomyxoma arising from the urachus

  • Alejandra Martínez

      Affiliations

    • Institut Claudius Regaud (ICR), Surgery, 20-24, Rue du Pont Saint Pierre, 31052 Toulouse, France
    • Corresponding Author InformationCorresponding author. Tel.: +33 650493489; fax: +33 561424117.
    • ,
  • Gwénael Ferron

      Affiliations

    • Institut Claudius Regaud (ICR), Surgery, 20-24, Rue du Pont Saint Pierre, 31052 Toulouse, France
    • ,
  • Eliane Mery

      Affiliations

    • Institut Claudius Regaud (ICR), Pathology, 20-24, Rue du Pont Saint Pierre, 31052 Toulouse, France
    • ,
  • Laurance Gladieff

      Affiliations

    • Institut Claudius Regaud (ICR), Medical Oncology, 20-24, Rue du Pont Saint Pierre, 31052 Toulouse, France
    • ,
  • Jean Pierre Delord

      Affiliations

    • Institut Claudius Regaud (ICR), Medical Oncology, 20-24, Rue du Pont Saint Pierre, 31052 Toulouse, France
    • ,
  • Denis Querleu

      Affiliations

    • Institut Claudius Regaud (ICR), Surgery, 20-24, Rue du Pont Saint Pierre, 31052 Toulouse, France

Accepted 29 December 2009. published online 17 October 2011.

Article Outline

Abstract 

Pseudomyxoma peritonei (PMP) arising from urachal tumors is extremely rare. To our knowledge, natural history, tumor biological behaviour, morbidity, treatment, and prognosis of PMP arising from the urachus are determined by the associated PMP. Management of urachal tumors with associated PMP should be based on aggressive locorregional therapy with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy, similar to PMP arising from other origins.

Keywords: Urachus, Pseudomyxoma, HIPEC, Peritonectomy, Cytoreductive surgery

 

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Introduction 

Pseudomyxoma peritonei (PMP) arising from urachal tumors is extremely rare. Despite the appendix is the alleged dominant origin for PMP, other primary tumors can associate PMP [1], [2], [3], [4], [5]. Pseudomyxoma peritonei (PMP) is a rare disease with an estimated incidence of 2 per 10,000 laparotomies [3]. It is characterized by mucinous ascites produced by adenomucinous tumor cells implanted on the peritoneal surface.

The objective of our study was to report on a case of PMP arising from the urachus treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC), and to review medical literature concerning the management and outcome of this entity.

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Methods 

A 32-year-old man was admitted to our hospital for evaluation and further treatment of an urachal mucinous cystoadenocarcinoma associated with pseudomyxoma peritonei. The patient presented complained of mucosuria, with no other associated symptoms. Physical examination was normal. Ultrasonography and computed tomography (CT) revealed a thick-wall cystic mass located adjacent to the bladder dome in contact with the midline abdominal wall (Figure 1, Figure 2). After preoperative diagnose of bladder cancer, the patient underwent exploratory laparotomy. A large cystic perforated mass superior to the bladder dome, suggestive of an urachal tumor, as well as large amount of gelatinous ascites throughout the peritoneal cavity were found. Radical excision of the cystic mass including partial resection of the bladder, the superior urachus and the umbilicus was performed in mono-bloc. At the end of the procedure, the patient received abdominal lavages to remove the mucinous ascites but small gelatinous implants remained at the pouch of Douglas, considering the procedure as an incomplete surgery type R1. Histological examination revealed an urachal mucinous adenocarcinoma with positive margins. The patient received 6 cycles of postoperative chemotherapy with a triple combination of 5FU, irinotecan and oxaliplatin before referral to our center.

After chemotherapy, magnetic resonance imaging (MRI) of the abdomen and positron emission tomography (PET) did not show any evidence of disease progression except for small residual nodules at the right hepatocolic ligament and on the fatty tissue at the right side of the mesentery. Decision to reoperate the patient and to perform hyperthermic intraoperative intraperitoneal chemotherapy (HIPEC) was based on the consensus of a multidisciplinary team. At laparotomy, millimetric mucinous implants were seen through the abdomen, mainly located beneath the right hemidiaphragm, in the sub-hepatic region and Glisson's capsule, Douglas pouch, bladder and mesenteric surface. The peritoneal cancer index (PCI) was 7 and there were 5 anatomic regions involved. Partial bladder resection, appendectomy and four peritonectomy procedures [6] with resection of greater omentum and right colic gutter, right upper quadrant (peritoneal surface of right hemidiaphragm, right sub-hepatic space and surface of the liver), pelvic peritoneum and electroevaporation of mesenteric implants were done. Abdominal scar of previous surgery was also resected. In addition, HIPEC with oxaliplatin 360 mg/M2 at 42 °C for 30 min [7] was performed after complete cytoreductive surgery (CC0). Histopathology exam disclosed a peritoneal adenomucinosis. Postoperative course was uneventful except for a transitory retarded gastric empting. Two years later the patient remains free of clinical and radiological disease.

We searched the MEDLINE database in order to identify articles on PMP and on urachal tumors. Literature review was carried out using the following terms: “pseudomyxoma peritonei”, “urachal adenocarcinoma”, “cytoreductive surgery” and “HIPEC”.

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Discussion 

Urachal remnants persist because of a failure or a delay in the obliteration of the allantois [8] and are identified in 30–70% of all autopsy series [9]. However, the range of urachal remnants reported in the literature is 31–43% for urachal cysts [8]. Urachal carcinoma is rare, and accounts for less than 1% of all bladder cancers [10], [11], [12], it consists predominantly of adenocarcinoma arising from the epithelial mucosa of the urachal remnant and typically displays an enteric type histology believed to originate from enteric rests during embryological development [13]. Most urachal tumors associate poor prognosis due their anatomic position, which minimizes early symptoms and precludes a timely diagnosis [13], [14]. Advance disease is encountered at clinical presentation, with bladder invasion and extravesical tumor growth, favoring local recurrency and distant metastasis [12]. Surgical resection with a partial cystectomy and en bloc resection of the urachal ligament with the umbilicus is the treatment of choice in the setting of this disease, when localised [15], [16]. The role of adjuvant radiotherapy and chemotherapy is not clear [14], and there is no established chemotherapy regimen [12]. A systematic review of the literature by Tsiouris et al. reveals a 5-year survival rate of less than 20% [17]. Henly et al. reported a 43% 5-year survival rate in 34 patients. The authors found no difference between surgical approach with segmental cystectomy and umbilectomy versus en bloc cystoprostatectomy and umbilectomy as initial treatment [13]. M.D. Anderson experience on a cohort of 42 patients found a 5-year survival rate of 44% [15]. Of the 50 patients reported by Herr et al., 93% with tumor confined to the urachus and bladder survived compared to 69% with extravesical or peri-urachal tumor invasion. All patients with tumor invasion into the peritoneal cavity deceased [14]. During the follow-up, the rate of distant metastasis in this series was 32% [14]. Mayo large case series of 66 patients reported a 59% rate of systemic metastasis at some point of the evolution, and a 5-year cancer-specific survival rate of 49% [17]. Negative surgical margins and stage are the most important reported prognostic factors [14], [15], [17]. Omission of umbilectomy and tumor grade are also indicators of poor prognosis [17].

In exceptional cases urachal tumors can associate with PMP. PMP is an untrue tumor of the peritoneum as tumor cells do not originate from the peritoneal surface, but from a perforated tumor of the appendix or less frequently of the ovary, pancreas, colon, or urachus [1], [2], [3], [4], [5]. In most cases, PMP arises from an appendiceal adenonomucinous neoplasm that enlarges and obstructs the lumen of the appendix leading to appendiceal perforation [1], [2]. Mucinous tumor cells spread and implant throughout the peritoneal cavity producing a progressive amount of mucinous ascites. Urachal tumors with associated PMP probably share the same pathophysiology as PMP arising from the appendix. Urachal cysts can contain mucinous adenomas or well-differentiated adenocarcinomas that produce large amount of intraluminal mucus. Consequently, the rupture of the urachal wall by the intraluminal pressure results in the spread of mucinous epithelial cells throughout the peritoneal surface. PMP classification proposed by Ronnett categorizes the disease process into disseminated peritoneal adenomucinosis, which is a minimally invasive disease, peritoneal mucinous carcinoma, were mucinous cancer cells invade surrounding tissues, or a hybrid type [18]. A broad range of histhologic variations have been described for PMP arising from the urachus in previous reports [5], [10], [19], [20], [21], [22], [23], [24], [25], [26], [27], [28]. We support the term “mucinous urachal neoplasms” defined by Sugarbaker, to describe this entity [51]. PMP arising from the urachus has been reported fourteen times in the international literature according to the MEDLINE database [5], [9], [19], [23], [24], [25], [26], [27], [28], [29], [45], [46], [47] (Table 1).

Table 1. Literature review of urachus carcinoma and PMP.
AuthorCase descriptionTreatmentFollow-up
Faulkner JW 1954 [47]Rupture of a urachal mucinous cystic tumorSurgery (just tumor removal) and roentgen therapyNS
Mendeloff J 1971 [45]Peritoneal carcinomatosis from invasive urachal adenocarcinomaDebulking surgeryDOD 28 months
Sasano H 1997 [47]Rupture of urachal mucinous cystic tumorSurgeryaNS
Loggie 1997 [44]Urachal signet ring-cell adenocarcinomaCRS and HIPEC-MTCDOD 31 months
De Bree E 2000 [5]Local recidive well-differentiated UMACRS and HIPEC-MTCNS
Yanagisawa 2003 [27]Rupture of urachal mucinous cystoadenocarcinomaSurgeryaNS
Takeuchi 2004 [52]UMASurgeryaNS
Soto Delgado 2006 [10]UMASurgeryaNED 1 year
Stenhouse 2003 [50]Rupture of non-invasive urachal mucinous cystic tumorSurgeryaNED 6 months
Sinohara 2006 [48]Rupture of urachal cyst consisting of non-invasive UMASurgerya and intraperitoneal lavageNED 7 years
Sugarbaker 2008 [51]UMACRS and HIPEC-MTCDOD 11 years
Sugarbaker 2008 [51]UMACRS and EPICNED 18 years
Khalid 2008 [28]UMASurgeryaNED 6 months
Sugiyama 2009 [29]UMASurgeryaNS
Present caseUMACRS and HIPEC-OxaliplatinNED 2 years

NS, not stated; MTC, mitomicin; CRS, cytoreductive surgery; DOD, died of disease; NED, no evidence of disease; EPIC, early postoperative intraperitoneal chemotherapy; UMA, Urachal mucinous adenocarcinoma.

aPartial resection of the bladder, urachal tumor ± umbilicus ± appendicectomy (no CRS).

Diagnosis delay over a period of years is constant due to the lack of symptoms in early stages of the disease. During this time, tumoral cells spread widely throughout the peritoneal cavity producing large amounts of mucus. Various symptoms have been reported for for urachal mucinous tumors such as hematuria [5], [10], mucosuria [51], abdominal fullness [26], [27], and abdominal pain [50]. On CT or MRI the most characteristic image is a mid-situated cystic mass attached to the abdominal wall accompanied by various amounts of fluid collected along the tumor [29]. However, final diagnosis is usually confirmed at surgery.

Management of reported cases, except four [5], [44], [51], is based on the surgical approach recommended for urachal carcinoma without PMP (Table 1). Urachal adenocarcinoma has an aggressive behaviour characterized by local invasion and metastatic spread. Initial treatment is en bloc surgical resection encompassing the umbilicus, tumor and urachus with negative margins. In mucinous urachal neoplasms there is usually no invasion of the bladder. Disease progresses by migration of tumoral cells throughout the peritoneal cavity, where they follow physiological peritoneal fluid flow. Tumoral cells implant on the peritoneal surface producing mucin deposits as PMP arising from other origins. Contrary to the natural history of urachal adenocarcinoma, PMP rarely causes lymphatic or distant metastases as it does not invade and tends to remain confined within the peritoneal cavity [30]. Therefore, peritoneal spread remains the major problem in the management of PMP [51].

The historical standard treatment of PMP has been surgery with repeated debulking to remove tumor and mucinous ascites. However, disease was always left behind and there was limited expectation of long-term survival. Patients died from intestinal obstruction, terminal starvation or treatment complications [2]. M.D Anderson series of 38 patients managed by tumor reduction provided a 54% survival rate at 5 years and 18% at ten years [31]. Mayo Clinic series of 56 patients treated by repetitive surgical debulking resulted in 5 and 10-year survival rate of 53% and 32%, respectively [32]. 76% of the patients presented disease recurrence after a median follow-up of 12 years, and 50% of the recurrences were detected within 2.5 years of initial surgery [32].

Currently, a more aggressive approach through complete cytoreductive surgery (CRS) by peritonectomy procedures and resection of involved viscera is warranted in patients with PMP [33]. There is a survival advantage of complete vs incomplete cytoreduction of 80 vs 20% [34]. Miner et al. attempted complete cytoreduction in 97 patients with PMP. Although maximal debulking was achieved in 55%, disease recurred in 91% of patients at a median disease-free interval of 24 months [35]. Ten year survival was attained in 21% of the patients, and was most likely associated to low-grade pathologic features and complete cytoreduction at some time in their clinical course [32].

Although complete cytoreduction is associated with prolonged overall survival, recurrence is common and multiple operations are frequently required. The combination of surgery and perioperative intraperitoneal chemotherapy (EPIC), introduced by Sugarbaker, permits to eradicate any microscopic tumor residue to prevent recurrence. The aim of surgery is to remove all visible tumor by cytoreductive surgery with peritonectomy procedures, or if it is not possible, to leave tumor deposits less than 2.5 mm (2.5 being the maximum direct penetration of locally applied chemotherapy) [33]. CRS and EPIC are especially favourable in PMP because of the dissemination pattern and the non-invasive character of the disease. However, there are no randomized trials or comparative studies regarding PMP treated by CRS combined with locoregional chemotherapy vs CRS alone. Hyperthermic intraperitoneal chemotherapy (HIPEC) is increasingly employed in PMP [36], [37], [38], [39], [41], [42], [43], and there are actually multiple prospective trials that support a benefit of the procedure in terms of improved survival, as compared with historical controls [44]. This combination results in 5-year survival rate from 50% to 96% [40], [43], and associates with 27–54% morbidity, and 2% mortality [45]. Table 2. The French experience on PMP reveals an overall survival of 73% and 55% at 5 and 10 years, respectively [46].

Table 2. Cytorreductive surgery combined with hyperthermic intraperitoneal chemotherapy for PMP.
InvestigatorNMedian Follo-upChemotherapy modalityMorbidity (%)Mortality (%)5-year survival (%)
Gonzalez Moreno S 04 [48]50148MMC + 5-FU*N.SNS72
Deraco 04 [43]3329MMC + CIS33396
Glehen 05 [49]2723MMC + CIS44052
Guner 05 [42]28N.SMMC or CIS367NS
Smeenk RM 06 [50]10352MMC54360
Stewart JH 06 [51]11035MMC38N.S53
Sugarbaker 06 [52]35635MMC + 5-FU*402N.S
Yan TD 06 [52]5022MMC + 5-FU*48469
Smeenk 06 [41]10351MMC543>80
Elias 08 [38]10548Oxaliplatin67.67.680

5-FU*early postoperative intraperitoneal chemotherapy within 7 days of surgery; MMC, mitomycin; CIS, cisplatin.

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Conclusions 

To our knowledge, natural history, tumor biological behaviour, morbidity, treatment, and prognosis of PMP arising from the urachus are determined by the associated PMP. However, the reduced number of reported cases of PMP arising form the urachus, and the lack of available follow-up in most patients, do not permit to asses the impact of extent of primary surgical therapy on local control of the disease.

Although management of urachal carcinoma consists on en bloc resection of the urachal tumor and urachus coupled with partial cystectomy, we believe that treatment of urachal mucinous tumors should be based on the same approach as PMP arising from the appendix.

As this disease is very rare, randomised studies will never be possible. Then, we strongly believe that complete cytoreductive surgery, and HIPEC should be now considered the standard therapy in the management of these patients.

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Conflict of interest statement 

The authors have no conflicts of interest to declare.

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Acknowledgments 

Alejandra Martínez work at ICR is supported by grant from the Fundación Alfonso Martín Escudero, Madrid, Spain.

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PII: S0960-7404(10)00002-2

doi:10.1016/j.suronc.2009.12.004

Surgical Oncology
Volume 21, Issue 1 , Pages 1-5, March 2012

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