Pseudomixoma Peritonei as an Unusual Tumor in a Kidney Donor

Alejandro Rossano*; Bernardo Bravo; J. Manuel Portela; Eduardo Montalvo; Layla L. Monroy; Victor Ortiz; Manuel Lavariega; Cecilia Gallegos; Armando López (MEXICO)

INTERNATIONAL JOURNAL OF CLINICAL INVESTIGATION AND CASE REPORTS - Volume 1, Issue 3, Nov-Dec

Pages: 79-84

Date of Publication: 30-Dec-2022

Pseudomixoma Peritonei as an Unusual Tumor in a Kidney Donor

Author: Alejandro Rossano*, Bernardo Bravo, J. Manuel Portela, Eduardo Montalvo, Layla L. Monroy, Victor Ortiz, Manuel Lavariega, Cecilia Gallegos, Armando López (MEXICO)

Category: Surgery

Abstract:

Introduction: Pseudomixoma peritonei is a neoplasia originating from the appendix, with an incidence of 2-4 per million habitants. Clinical presentation is variable, from asymptomatic to appendicitis, intestinal obstruction, or perforation, and is usually diagnosed incidentally at image studies or by laparoscopy when performing a conventional appendectomy.

Case presentation: 40-year-old male with history of successful left kidney donation developed a pseudomixoma peritonei. At image examination, a CT revealed hypermetabolic activity consistent with peritoneal carcinomatosis, treatment with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy was established with good results and has been followed up for 4 years with no evidence of recurrent disease and adequate kidney function.

Conclusion: Association of appendiceal neoplasms in organ donors has been unusual when developed most common present as carcinomatosis, combined treatment with cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy has shown the best long-term survival for appendiceal neoplasms.

Keywords: Pseudomixoma Peritonei, Appendiceal Neoplasm, Carcinomatosis, Kidney Donation, Cytoreductive Surgery, Hyperthermic Intraperitoneal Chemotherapy, Neoplasm in Organ Donor

DOI: 10.55828/ijcicr-13-15

DOI URL: http://dx.doi.org/10.55828/ijcicr-13-15

Full Text:

INTRODUCTION

Appendiceal mucinous neoplasms are exceptional gastrointestinal malignancies, reported in less than 1% of appendectomies. The majority of mucinous appendiceal neoplasms develop at middle-aged and are discovered incidentally, with a low incidence of 2-4 per million habitants [1,2].

The term pseudomyxoma comes from pseudomucin, the prefix "myxo" latin form for muxa from the Greek, and meaning mucus, used to be employed when mucus was present in the nosological condition. The term “Pseudomyxoma Peritonei'' was introduced by Werth [2] in 1884, when he described the case of a woman with gelatinous masses in the peritoneal cavity from alleged ruptured pseudomucinous cystadenoma of the ovary, in which he found to be pseudo mucin.

The peritoneum is one of the main sites of metastases from abdominal organs, Pseudomyxoma Peritonei (PMP) results from excessive production of mucin from the primary appendiceal tumor, which is released and spreads to the peritoneal cavity however, PMP differs from other peritoneal malignancies due to its high and characteristically mucin component [3].

Clinical presentation is variable, ranging from asymptomatic to appendicitis, intestinal obstruction, progressive abdominal distention, ascites, or perforation. It is usually diagnosed incidentally in imaging studies or by laparoscopy when performing a conventional appendectomy. Cytoreductive Surgery-Hyperthermic Intraperitoneal Chemoperfusion (CRS-HIPEC) is considered standard therapy for PMP.

CLINICAL PRESENTATION

A 40-year-old male with a history of left kidney donation at age 21 to a brother who was in chronic kidney failure and underwent a successful kidney transplant with a current functional graft, further developed dyslipidemia and systemic arterial hypertension at the follow-up and with close medical surveillance. Patient complained of left iliac fossa pain, nausea, and sporadic vomiting for one month of evolution. Physical examination showed any sign of palpable masses, organomegaly, and no acute abdomen.

IMAGING EXAMINATION

Abdominal ultrasound reported abundant free fluid and a PET-CT (Figure 1) showed positive focal hypermetabolic activity at the right colon suggestive of a primary tumor and with diffuse hypermetabolism at the mesentery as a secondary deposit consistent with peritoneal carcinomatosis. As part of the study protocol, upper and lower gastrointestinal endoscopies showed any primary suspected lesion.

Figure 1: A: PET CT with evidence of hypermetabolic activity at right colon and peritoneum; B: CT coronal view with the presence of mucin at the pelvis, right paracolic flank, and subdiaphragmatic area.

Preoperative and postop laboratories include liver function test (Table 1), tumor markers (Table 2), and blood chemistry panel to evaluate kidney function, with a basal cystatin C of 0.8 mg/dL, glomerular filtration rate of 117 ml/min/1.73m² and MDRD of 72.7 ml/min.

Table 1: Biochemical evolution.

Day	Parameter
Day	TB	DB	HB	AP	GGT	AST	ALT	Amylase	Lipase	Cr
Preop Day 1	0.7	0.2	14.9	183	32	18	12	95	84	0.7
Postop Day 1	0.6	0.3	10.9	40	28	133	107	83	118	1
Postop Day 5	0.6	0.3	9.5	45	55	69	55	89	87	0.8
Postop Day 10	0.5	0.2	10.7	119	100	37	31	80	77	0.7
Postop Month 1	0.5	0.3	16.7	113	181	34	36	195	212	0.7
Postop Month 8	0.8	0.1	17	98	45	31	30	85	94	0.9
Postop Year 1	0.4	0.1	16	107	34	45	34	98	88	0.9
Postop Year 4	1	0.1	16.4	74	48	59	56	125	221	1.1
TB: Total Bilirubin; DB: Direct Bilirubin; IB: Indirect Bilirubin; AP: Alkaline Phosphatase; GGT: Gamma-Glutamyl Transferase; AST: Aspartate Aminotransferase; ALT: Alanine Transaminase.

Table 2: Follow-up tumor markers.

Time	AFP	CA 19-9	CEA
Day -1	2.3	<2	20.91
Year 1	2.5	<2	1.74
Year 2	2.7	<2	1.86
Year 4	2.3	<1	1.5
AFP: alpha-fetoprotein, CA 19-9: carbohydrate antigen 19-9, CEA: Carcinoembryonic Antigen

TREATMENT

Multiple peritoneal biopsies of implants and appendectomy were realized at diagnostic laparoscopy in December 2017 (Figure 2). The final pathology report confirmed a low-grade and well-differentiated appendiceal mucinous neoplasm.

Figure 2: Diagnostic laparoscopy showed multiple implants in the parietal, visceral omentum, liver, and diaphragm.

According to the intraoperative findings and the histological result, a second surgical event was planned. In January 2018, an arterial line and a right central venous catheter were placed previously at CRS-HIPEC. CRS included cholecystectomy, splenectomy, cytoreduction of lesions at ileum, right colon, left lateral segment hepatectomy, and dissection of the right subdiaphragmatic area which present the greatest number of lesions, with these findings peritoneal carcinomatosis Index (PCI) was established of 21 points. During dissection, a pleural incidental lesion occurred with secondary transitory hemodynamic decompensation that resolved with a pleural seal while surgery was temporally stopped.

HIPEC started with two Y-connected catheters and chemotherapy infusion with Mitomycin C 20 mg+ Oxyplatin 400 mg at 41? for 30 min, later Mitomycin C 20 mg at 41.5? for 60 min; suction was performed and 4 closed drains were placed, in two toward each subphrenic space and two regarding pelvic cavity. Additional laboratory tests were realized at the surgery with initial hemoglobin of 13.5 g/dL and final of 10.9 g/dL, platelet 234 × 109/L, and final of 117 × 109/L. Other surgery parameters are shown in Table 3.

Table 3: Surgical Parameters.

Parameter	Result
PCI (Points)	21
Operation time (minutes)	876
Blood loss (L)	0.75
Transfusions (GP)	3
HIPEC time (minutes)	90
Anesthesia time (minutes)	900
ICU stay (days)	4
Total hospital stay (days)	26
L: Liter; GP: Globular packages

PATHOLOGY AND IMMUNOHISTOCHEMISTRY

Immunohistochemistry exhibited neoplastic glands and expression of cytokeratin 20, CDX 2, and villin, compatible with well-differentiated mucinous adenocarcinoma of the appendix (Figures 3 and 4). According to the 7^th World Health Organization (WHO) classification of Tumors of the Digestive System of the American Joint Committee on Cancer (AJCC), PP is divided into high and low grade, this one with predominate of small and regular nuclei and little cellular atypia; while high-grade predominate atypia, mitosis and signet ring cells [4-6].

Figure 3: HE 4x panoramic view of splenic parenchyma, the capsule (red dotted line) of the thickened spleen, adhesions of loose fibrous tissue with mild chronic inflammation (blue arrow), and implantation of predominantly acellular mucin (yellow).

Figure 4: HE 4x panoramic view slice of the omentum infiltrated by mucin lakes, reactive mesothelial hyperplasia is observed (yellow arrow), and congestive vessels.

FOLLOW UP

In the immediate postoperative period, the patient went to ICU for surveillance, supported with mechanical ventilation, oxygen saturation of 100%, with FiO₂ of 40%, and RF 18, hemodynamically stable, without vasopressors, and a Foley catheter with normal urine output.

Four days later was discharged to surgery floor where he remained for 22 days without wound complications, maintaining adequate renal function and following a rehabilitation program that included an incentive spirometer, early ambulation, and enteral feeding.
After 26 days of total hospital stay, he was discharged home, free of complications and with a personalized diet. Monthly follow-up was carried out with clinical, biochemical, and ultrasonographic assessment during the first year. Actually, CT and tumor markers (Table 1) are annually requested, patient is currently clinically free of disease, with no tomographic evidence of recurrence (Figure 5) and adequate kidney function.

Figure 5: A(Sagittal View) and B(Coronal View) showing splenectomy, cholecystectomy, and nephrectomy due to surgical history, with no evidence of abdominal tumor lesions.

DISCUSSION

There is currently little statistical information on the development of neoplasms in organ donors. At present database taken from the National Cancer Institute Surveillance, Epidemiology, and End Results Program (SEER) includes donors who developed primary tumors. After follow-up for 7 years, an increase in the incidence of renal and colorectal cancer was observed; risk of renal cancer was associated with hyperfiltration, proteinuria, and tendency to hypertension of the remaining kidney [7].

For the rest of associated neoplasms in kidney donors, a higher incidence of prostatic adenocarcinoma, breast cancer, and lung cancer has been observed. There are no reports of appendiceal neoplasms in living kidney donors. Within appendiceal neoplasms, pseudomyxoma peritonei represents 8% according to the AJCC they are classified according to their histological grade (high or low) [8].

In PMP both CEA and CA 19-9 are related to tumor load, however, normal pre-operative tumor markers correlated with significantly improved survival [9-11]. Furthermore, an elevated CEA tumor marker seemed to be associated with a significantly reduced prognosis. At Computed Tomography (CT) there are no characteristic features that distinguish PMP deposits from other mucinous causes; the accumulation pattern only suggests the diagnosis [12-15].

Complete surgical cytoreduction is defined as the removal of all disease or reduction of tumor deposits to 2.5 mm in thickness in combination HIPEC, which is delivered using a closed technique, time varies between 30-90 minutes depending on the pharmacokinetic chemotherapy drug used and its total dose, most recommended agent is Mitomycin C [10]. Chemotherapy agents used are hydrophilic, with a high molecular weight that allows intra-abdominal concentration, other commonly used drugs include cisplatin, doxorubicin, oxaliplatin, and irinotecan.

CRS- HIPEC presents systemic effects secondary to extensive resection and great exposure surface as dilutional hyponatremia, and hyperdynamic state coagulopathy. Strictly perioperative management includes normothermia control by physical means or hot infusions since hypothermia favors coagulopathy, alters platelet function and increases wound infection incidence, while HIPEC increases the temperature up to values of 41? enhancing the metabolic demand [16-19].

Postoperative follow-up is recommended according to colorectal cancer guidelines as recurrence and distant metastases are frequently observed, comparable with the same gastrointestinal mucinous adenocarcinoma [12]. Complete CRS-HIPEC has demonstrated maximal efficacy, without complete cytoreduction the median survival average of 6 months is similar to outcomes observed without surgical intervention [20-24].

CONCLUSION

PP is a rare asymptomatic entity with vague abdominal symptoms and usually detected at advanced stages. Surveillance of organ donors with neoplasms must be whole life, and protocol is carried out of clinical examination, and laboratory tests including tumor markers and imaging, usually CT.

Additional statistical information is required to define surveillance in patients with PMP treated with CRS-HIPEC, as a complex procedure requires experienced surgeons, a multidisciplinary team, and an individualized approach to each patient. Four years after surgery, our patient has an adequate life quality, without recurrence of the PMP, and with good renal function.

RECOMMENDATIONS

Organ donors are so special people because of their genuine wish to help close family, and friends, or as a good samaritans to patients in need of an organ transplant. They need close medical follow-up, health surveillance, and physical, psychological, and emotional support throughout their entire life. Those who developed a disease or in this case a neoplasm, they need a fast and effective medical approach, in order to preserve their life, health, integrity, and the function of the organ that support the missing one or part of the organ that was donated. Of special interest for the medical community and organ donor society is to report the incidence and presentation of malignancies that allows approaching organ donors in a similar and effective way to obtain a curative treatment and to establish a close follow-up and effectiveness of the treatment.

AUTHORS CONTRIBUTIONS

Alejandro Rossano is the first author. AR and LM wrote the manuscript. The study is designed by Alejandro Rossano. All authors read and approved the final manuscript.

CONFLICT OF INTEREST

The authors declare no conflicts of interest.

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