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SM Journal of Gastroenterology & Hepatology

Pseudomyxoma Peritonei, an Uncommon Tumor with Ongoing Debatable Nomenclature and Classification. Report of a Case and Review of the Literature

[ ISSN : 3067-977X ]

Abstract Citation ABBREVIATION INTRODUCTION CASE PRESENTATION DISCUSSION ACKNOWLEDGMENT REFERENCES
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Received: 23-Mar-2021

Accepted: 23-Apr-2021

Published: 26-Apr-2021

Case Report | Volume 5 - Issue 1 | Article DOI :

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Allison Parrill*, Samaan Mahmoudzadeh, Kimberly Eaton, Tiffany Tsao, Jessica Eisold, Nicole Forte, Amber Boudreaux and Mohamed Aziz

Department of Pathology, American University of the Caribbean, School of Medicine, USA

Corresponding Author:

Allison Parrill, Department of Pathology, American University of the Caribbean School of Medicine, 1 University Drive at Jordan Road, Cupecoy, St. Maarten, Tel:1-516-946-5164; Email: allisonparrill@students.aucmed.edu

Keywords

Pseudomyxoma, Peritonei, Mucin, Appendix, Ovary, Peritoneum

Abstract

Pseudomyxoma peritonei is a rare condition, characterized by gelatinous ascites of the abdominal and pelvic cavities accompanied by mucinous implantations of the peritoneum and omentum. It is usually presented as a disseminated mucinous tumor with dissection of the abdominal and pelvic organs producing pseudomyxoma peritonei. Mucinous implants usually involve the serosal surface of multiple pelvic and abdominal organs. Although it is accepted that most investigators believe that the ovarian tumors are secondary in almost all cases to a primary appendiceal tumor, a synchronous origin in both organs has also been proposed. The agreement on the association of certain tumor morphologies with certain behaviors is persistently plagued by disagreement regarding tumor nomenclature and classification. We present a case of recurrent pseudomyxoma peritonei and review the literature of nomenclature, classification, diagnosis, and management of these tumors.

Citation

Parrill A, Mahmoudzadeh S, Eaton K, Tsao T, Eisold J, et al. (2021) Pseudomyxoma Peritonei, an Uncommon Tumor with Ongoing Debatable Nomenclature and Classification. Report of a Case and Review of the Literature. SM J Gastroenterol Hepatol 5: 5.

ABBREVIATION

Pseudomyxoma peritonei (PP), disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA), hyperthermic intraperitoneal chemotherapy (HIPEC), cytoreductive surgery (CRS)

INTRODUCTION

Pseudomyxoma peritonei (PP) is a rare condition, with an incidence of 1 to 2 in 1 million per year [1]. PP represents a broad spectrum of neoplastic disorders ranging from benign to borderline and malignant lesions. It is originally reported as disseminated peritoneal adenomucinosis (DPAM), which describes the benign variant whereas peritoneal mucinous carcinomatosis (PMCA) describes the malignant variant [2,3]. Gelatinous ascites of the abdominal and pelvic cavities accompanied by mucinous implantations of the peritoneum and omentum characterize findings associated with PP [4].

Histopathologically, the primary tumor predominately resembles a mucinous epithelial neoplasm of the appendix [1,5 7]. PP displays a wide range in the mucus/cell ratio in addition to the level of differentiation and grade of atypia of epithelial cells. However, proper histopathological characterization of PP remains difficult due to the heterogeneous appearance [1,2].

PP more commonly presents in women (male:female ratio = 9:11), with an average age of 53 years. In women, usual presentation is increasing abdominal girth and a primary ovarian lesion [8,9]. Most cases of ovarian involvement likely represent metastasis from an appendiceal or other gastrointestinal source [10]. Immunohistochemistry studies are useful in confirmation of appendiceal origin as they usually stained negative for CK-7 and positive for CK-20 and CDX2 in support of appendiceal origin. This pattern of immuno profile is reversed if it was of ovarian origin.

Diagnosis of PP requires the presence of mucinous neoplastic epithelial cells and diffuse intra-abdominal mucinous ascites. Some clinicians require the presence of diffuse mucinous peritoneal implants for definitive diagnosis [3].

PP warrants early intervention due to its high mortality rate when not properly managed [11]. Without treatment, mucin accumulation and large volume ascites compress vital internal organs such as the colon, liver, kidneys, stomach, spleen, and pancreas resulting in bowel obstruction and eventual death [1,5,9]. Proper diagnostic investigations include an ultrasonographic examination of the abdomen followed by computed tomography scans, showing the extent of the disease [12]. Additionally, the evaluation of tumor markers in serum, such as CA 19-9 and carcinoembryonic antigen (CEA), help determine prognosis [13]. The current optimal treatment involves macroscopic tumor excision cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) [14].

CASE PRESENTATION

A 39-year-old woman presented to her surgeon with complaint suggestive of umbilical hernia. The patient has history of appendectomy with mucinous cystadenoma and pseudomyxoma peritonei (PP) two years prior to current presentation. Prior tumor was treated with repeated paracentesis of the mucinous ascites and surgical debulking of the primary tumor with appendectomy and removal of mucinous material. Thoracoabdominal contrast enhanced MRI and CT scan showed no ovarian masses or other solid tumor. However, multiple peritoneal implants were noted with extension to the surface of the ovaries. Serum tumour markers showed elevated levels of CEA, CA-125, and CA19-9. The clinical presentation was consistent with recurrent disseminated PP with aggressive mucinous dissecting features.

The tumor was treated with surgical debulking with macroscopic tumor excision cytoreductive surgery (CRS) in addition to removal of both ovaries, spleen, portions of diaphragm, colon and small bowel as well as all peritoneal implants. Pathologic microscopic examination showed disseminated mucinous tumor with dissection of the ovarian and peritoneal stroma producing pseudomyxoma ovarii/pseudomyxoma peritonei (Figure 1A). Mucinous implants involved the serosal surface of multiple pelvic and abdominal organs including spleen, diaphragm, small intestine, colon and omentum (Figure 1B). No evidence of infiltration into skeletal muscle was noted and two removed lymph nodes were negative for malignant epithelium. Epithelial glandular structures were seen within the dissecting mucous. Some nuclear atypia was noted (Figure 1C). Although there was no definitive evidence of invasion, occasional epithelium within stromal fragments suggestive of possible invasion was noted. For this reason, the tumor was classified as “with intermediate features’ (between low grade adeno mucinosis and well differentiated mucinous carcinomatosis). The epithelial atypical cells stained negative for CK-7 and positive for CDX2 (Figure 1D) and CK-20 (Figure 1E) in support of appendiceal origin. Surgery was combined with hyperthermic intraperitoneal chemotherapy (HIPEC) followed by postoperative chemotherapy utilizing cisplatin, and oxaliplatin.

Figure 1: Pathological examination of the excised tumor
1A: disseminated mucinous tumor with dissection of the ovarian and peritoneal stroma producing pseudomyxoma ovarii/pseudomyxoma peritonei (H&E stain X20)
1B: Mucinous implants involved the serosal surface of multiple pelvic and abdominal organs (H&E stain X40)
1C: Tumor cells showing focal nuclear atypia (H&E stain X60)
1D: Tumor cells positive for CDX2 with nuclear staining
1E: Tumor cells positive for CK-20 with cytoplasmic staining

The patient was followed up for five years with no evidence of recurrence or metastasis after which she was lost to follow up.

DISCUSSION

Pseudomyxoma peritonei (PP) is a rare disease characterized by disseminated mucinous ascites with peritoneal, serosal, and omental mucinous implants [3]. Typically, the pathologic process initiates with neoplastic proliferation of appendiceal goblet cells into the peritoneal cavity following perforation of the appendix [15]. Mucin overexpression results in intraperitoneal voluminous PP [16]. Gravity and physical factors such as movement of peritoneal fluid in the abdomen lead to accumulation of mucinous deposits at the omentum, retrohepatic region, and rectovesical pouch [17]. Visceral “scalloping” arises from mucinous compression and fibrosis of organs and is pathognomonic of PP and can be visualized on CT [18]. While generally regarded as benign, PP frequently demonstrates a borderline-malignant presentation with unfavorable progression of the disease and advanced-stage at diagnosis [19,20].

Although it is accepted that most investigators believe that the ovarian tumors are secondary in almost all cases to a primary appendiceal tumor, a synchronous origin in both organs has also been proposed [21]. The agreement on the association of certain tumor morphologies with certain behaviors is persistently plagued by disagreement regarding tumor nomenclature and classification. Some investigators may consider the current tumor as a tumor with distinctive features of intermediate/ hybrid tumors and even grouped it with the carcinoma. Other investigators may classify the current tumor with the concept of a clinically malignant, but pathologically benign, and in such case, they would classify it as well differentiated mucinous adenocarcinoma [22].

The clinical presentation of PP encompasses a broad spectrum with many non-specific findings resulting in up to 20% of cases diagnosed incidentally at laparoscopy or laparotomy [11,23]. In patients with an appendiceal mucocele, seeding of a mucinous epithelial neoplasm secondary to perforation, may not always appear macroscopically. Although small deposits tumor cells may appear on the surface of the appendix, frequently no sign of intraperitoneal tumor or mucus presents itself [1]. The lack of pathognomonic symptoms combined with the insidious clinical course results in local invasion of nearby structures, such as a reported case of urinary bladder involvement at the time of diagnosis [24]. Some common presenting features include increased abdominal girth (40%), bilateral or unilateral ovarian tumors (20%), hernia sac tumors (20%), appendicitis-like syndrome (10%) and infertility [8,9,26]. A retrospective review of 410 patients revealed acute appendicitis as the most common clinical presentation, occurring in 27% of male and female patients. However, ovarian mass presented most commonly in female patients (34%) [8].

Additionally, new-onset hernia caused by increased abdominal pressure often presents as a patient’s chief complaint [20]. Abdominal exam may reveal a palpable omental cake or ovarian mass indicating peritoneal extension. Mucinous deposits in the pouch of Douglas or rectovesical pouch may be present upon rectal exam. In patients with vague abdominal complaints and distention CT served as the most common imaging technique used to diagnose PP. Abdominal and pelvic magnetic resonance imaging (MRI) may help determine any small bowel or hepatoduodenal ligament involvement [14].

Serum tumour markers may predict the aggressiveness and prognosis of PP [20]. A study of 519 PP patients revealed that elevation levels of CEA, CA-125, and CA19-9 prior to CRS and HIPEC correlated with decreased overall survival and disease free survival [27].

PP rarely results in blood-borne or lymphatic metastasis but instead demonstrates a tendency for local recurrence [25,28,29]. Studies suggest that ovarian disease results from local invasion due to appendiceal perforation rather than as the site of origin [10]. In fact, most cases represent primary appendiceal mucinous neoplasms [2,30,31], while other reports indicate other cases with ovarian origin [32]. One literature review presented eighteen cases of the urachus as the tissue of origin [33]. Ultimately, the degree of local invasion throughout the peritoneal cavity represents a major factor in disease prognosis [11]. In our presented case, two lymph nodes were removed and showed no evidence of metastasis.

An early study conducted an aggressive treatment approach consisting of cytoreductive surgery with multiple operations. The center reported an average of 2.2 debulking operations required to reach complete cytoreduction in 55% of patients. Their treatment strategy resulted in a 10-years survival rate of 21%, with a 12% disease free rate at conclusion of follow-up. However, repetitive surgical debulking may result in imminent recurrence or disease progression due to intraperitoneal seeding of microscopic tumor residue (34; 1). HIPEC showed reduced recurrence or progression of disease in patients with microscopic to minimal macroscopic tumor residue with some studies reporting a potential cytotoxic effect up to a tumor depth of 2.5 mm (1, 35).

Intraperitoneal chemotherapy provides the benefit of decreased systemic effects and therapeutic concentrations reached at lower doses [36]. Mitomycin C, 5-fluorouracil, cisplatin, and oxaliplatin represent the most commonly used chemotherapeutic agents [37]. Following CRS + HIPEC, a CT scan alongside physical examination and serum tumor markers represent invaluable tools for detecting disease progression. CEA, CA19-9 and CA12.5 tumor markers are measured every 3 months while a CT scan is every 6 months for 5 years status post treatment. Following, a CT scan is performed every 2 years for up to 10 years [38-40].

Previously, management of PP involved repeated paracentesis of ascites or surgical debulking of the primary tumor and mucinous material [41]. Repeated debulking surgery and intraperitoneal chemotherapy limited conventional treatment. The current optimal treatment involves macroscopic tumor excision, known as cytoreductive CRS, combined with HIPEC [14]. Further studies demonstrated the efficacy of this combined approach, with a 10-year morbidity and mortality in a specialized unit setting reaching 63% [41]. Another study observed that following CRS and HIPEC combined treatment, low-grade mucinous adenocarcinomas demonstrated a 5-year survival range from 62.5 to 100%, while high-grade mucinous adenocarcinomas demonstrated 0–65% [19]. Chua et al. (2012) demonstrated that HIPEC alone improved the rate of progression-free survival, but not overall survival. Therefore, while HIPEC improves disease control, long-term survival may require complete cytoreduction. Our patient was treated with debulking surgery combined with hyperthermic intraperitoneal chemotherapy (HIPEC) and followed by postoperative chemotherapy utilizing cisplatin, and oxaliplatin.

Pseudomyxoma peritonei secondary to appendicular mucinous tumors is an exceedingly uncommon tumor with a varied presentation and outcomes. It is our hope that this report raises awareness of clinicians and pathologists to these type of tumors with definitive diagnosis and management of various ovarian and peritoneal malignancies. Furthermore, that the continued investigation drives further development of efficacious diagnosis and safe treatments for improving patient outcomes.

ACKNOWLEDGMENT

Special thanks to Adam El-Newihi, Elizabeth O’Grady, and Kirk Sheplay, MD candidates, American University of the Caribbean for their assistance in reviewing the final manuscript.

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Prediction and Diagnosis of Fatty Liver in Dairy Cows

Fatty liver is defined as an accumulation of fat, mainly Triacylglycerol (TAG) in liver. Fatty liver in dairy cows is categorized into mild, moderate and severe fatty liver. Obesity in pregnant cow is crucial etiological factor for postpartal development of fatty liver. Namely, obese cows have a greater decrease in feed intake during the period around parturition and, therefore, have a more severe negative energy balance during early lactation which leads to increased lipolysis of adipose tissue. Non Esterified Fatty Acids (NEFA), released from the stored TAG in the adipose tissue, are readily taken up by liver. The excess of NEFA may be converted to TAG and deposit in liver. Liver biopsy is the only reliable method for the detection of fatty liver, especially for the determination of its severity through the estimation of total lipids and TAG. Ecography may be considered as noninvasive, on-farm method for diagnosis of fatty liver which is not commonly used do to the not widely achievable equipment. Since some metabolic and endocrine parameters are significantly correlated with fatty liver degree, they may be used as diagnostic indicators of fatty liver. The significance of those indicators for diagnosis of fatty liver is present in this review. Additionally, indicators that may be used for prediction of fatty liver are explained. Specially attention was given to glucose, NEFA, BHBA, liver enzymes, total bilirubin, total protein, albumin and urea as well as some hormones involved in lipid metabolism. Achievements of novel methods, like genomic and proteomic profiling of biological fluids of diseased cows, in providing tools for diagnosis and prognosis of this metabolic disease are presented.

Danijela Kirovski¹* and Zeljko Sladojevic²

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Primary Gastric Lymphoma

The treatment of primary gastric lymphoma is evolving in post-rituximab era. The role of Helicobacter pyroli eradication alone can cure not only Mucosa-Associated Lymphoepithelial Tumor (MALT) but also some of diffuse large B cell lymphoma with or without MALT component. The efficacy of rituximab containing chemotherapy is so effective that the role of surgery is overshadowed. There are many studies, although most of them were retrospective trials, however it highlights the current mainstay of immune-chemotherapy provided an outstanding long term survival more than 80-90%.H pylori In real world there are substantial patients may receive surgery first, yet still needs post-operative adjuvant chemotherapy for some of them has a risk of relapse of lymphoma. And recent studies showed there’s no statistical difference between the two modalities. The main reason for patients proceeded to surgery as primary treatment is the gastroenterologist preference and showed no difference in terms of progression free survival and overall survival. The rituximab was introduced to lymphoma treatment since 1999, and demonstrated a superior long term survival in diffuse large B cell lymphoma for R-CHOP relatively to CHOP regimen. The highly effective treatment made PGL being easily curable disease; furthermore there are new insights of why and how the antibiotic therapy as exclusive treatment for limited disease will be a mainstay in treating this malignancy. We make a proposal how to treat the primary gastric lymphoma and MALT, and highlight the changing treatment modalities with regards to the integration of Helicobacter pyroli eradication to conventional chemotherapy as well as the complimentary role of surgery and radiotherapy.

Ming-Chih Chang¹* and Sung-Hsin Kuo²*

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Bleeding Peptic Ulcer: Epidemiology, Treatment and Prognosis

Aims: The aim of this study was to demonstrate epidemiological, clinical and endoscopic characteristics of acute Upper Gi Bleeding (UGIB) with a focus on Peptic Ulcer Bleeding (PUB).

Methods: This study included 2198 consecutive patients that were referred to our emergency department due to acute Upper Gi Bleeding (UGIB) from January 2008 to December 2012. All patients under went urgent upper GI endoscopies within 24 hours of admission, and 842 patients diagnosed with PUB were enrolled and prospectively followed.

Results: The cumulative incidence of UIGB was 126/100000 for a 5-year period. Two out of five patients had a bleeding peptic ulcer, of which 440 (52.3%) patients had a bleeding gastric ulcer, 356 (42.3%) had a bleeding duodenal ulcer, 17 (2%) had both bleeding gastric and duodenal ulcers and 29 (3.5%) patients had bleeding ulcers on gastroenteric anastomosis. PUB was more common in men. Average patient age was 65.9 years. The majority of patients with PUB were taking agents that attenuate the cytoprotective function of the gastric and duodenal mucosa (57%).Half of the patients received a red blood cell transfusion, with a median of 2.2 units. Re-bleeding occurred in 77(9.7%) patients and 47 (5.9%) required surgical intervention. The thirty-day morality was 5.2%, and 10% of patients died from uncontrolled bleeding and concomitant diseases.

Conclusion: PUB is the main cause of UGIB, characterized by a significant re-bleeding rate and mortality.

Budimir I¹, Stojsavljevic S¹*, Nikolic M¹, Kralj D¹, Biscanin A¹, Kirigin LS², Zovak M³, Babic Z⁴, Bohnec S⁵ and Budimir I⁶

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An Update on Hepatorenal Syndrome

The Hepatorenal Syndrome (HRS) is one of many potential causes of Acute Kidney Injury (AKI) in patients with decompensated liver disease.

HRS is associated with poor prognosis and represents the end-stage of a sequence of reductions in renal perfusion induced by progressively severe hepatic injury.

The pathophysiology of HRS is complex with multiple mechanisms interacting simultaneously, although HRS is primarily characterised by renal vasoconstriction.

A recently revised diagnostic criteria and management algorithm for AKI has been developed for patients with cirrhosis, allowing physicians to commence treatment promptly.

Vasopressor therapy and other general management, such as antibiotic prophylaxis, need to be initiated while patients are assessed for eligibility for transplantation. Liver transplantation remains the treatment of choice for HRS but is limited by organ shortage.

Other management options, such as transjugular intrahepatic portosystemic shunt, renal replacement therapy and molecular absorbent recirculating system, may provide short term benefit for patients not responding medical therapy while awaiting transplantation.

Clinicians need to be aware of the pathophysiology and management principles of HRS to provide quality care for patients with multi-organ failure.

Samuel Chan*

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Abdominal CT Findings of Cholecystogastric Fistula

Enteric fistulas are abnormal connections between the gastrointestinal tract and other organs, chest or skin. Fistulas between the gall bladder and the gastrointestinal system are common [1]. Cholecystoduodenal and cholecystocolic fistulas are seen frequently while cholecystogstric ones are rare [2]. In our case, we accidentally discovered a cholecystogastric fistula in a 71 years old male patient with gastric adenocarcinoma in orally and intravenous contrast enhanced abdomen CT.

Husam Vehbi¹* and Cagri Agirgun²

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Post-Colonoscopy Colorectal Carcinomas (PostCRCs): Have we Improved Over Time?

Background and Aim: There is an increasing concern on Post-Colonoscopy Colorectal Carcinomas (PostCRCs). Little is known about how these figures have evolved over time. We aim to compare the rates of PostCRCs in two periods of time and identify the risk factors.

Methods: Retrospective control-case study in our Endoscopic Unit. We studied two separate intervals (March 2004-September 2011 and October 2011 - December 2016). In both periods of time all patients diagnosed with CRC were identified. Patients with a previous colonoscopy performed 12 to 60 months before were retrieved (cases) and compared with those who did not have a previous procedure (controls).

Results: 712 and 743 patients diagnosed with CRC in both periods of time. 24 patients in the first period (3.6%) and 28 patients in the second one (3.8%) had a previous colonoscopy performed. PostCRCs were mainly located on the right side of the colon (63% vs. 35% p=0.006 and 68% vs. 33% p< 0.001), were smaller in size (3.17 vs.4.46 p< 0.001 and 3.61 vs. 4.44 p=0.086), with a tendency to host a better TNM stage. No differences in sex and age were found.

More than half of PostCRCs (58.3% and 60.7%) were attributed to procedure causes, meanwhile10 PostCRCs in both periods were considered new developed CRCs.

Conclusions: Despite what could be expected, we did not find a decrease in the rate of PostCRCs over time. A combination of preventable as much as biological factors would account for their etiology.

María Lourdes Ruiz Rebollo¹* and María Fe Muñoz Moreno²

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