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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 4  |  Issue : 3  |  Page : 191-195

Malignant peritoneal mesothelioma in a young patient, diagnostic challenge


1 College of Medicine, Taif University; KAASH, Taif, KSA
2 KAASH, Taif, KSA; College of Medicine, Beni.Suef University, Beni Suef, Egypt
3 KAASH, Taif, KSA

Date of Web Publication9-Dec-2015

Correspondence Address:
Dalal M Nemenqani
Consultant and Associate Professor of Pathology, College of Medicine, Taif University and King AbdulAziz Specialist Hospital, Taif, Kingdom of Saudi Arabia

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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-0521.171438

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  Abstract 

Malignant peritoneal mesothelioma (MPM) is a very rare diagnosis with an incidence of 1 case per 4–5 million of the population. It accounts for 20–30% of all mesothelioma type cancers. Its diagnosis is challenging for both surgeon and pathologist. The clinical and radiological features of the disease are usually nonspecific, most of cases diagnosed after laparotomy; even the gross appearance during laparotomy is confusing. In this article, we report a case of a young patient who never been exposed to asbestos, presented with clinical picture of bowel obstruction, computed tomography scan with contrast was done for abdomen, showed rim of free fluid in Morrison pouch with localized collection in right lower abdomen and fat stranding. Laparotomized twice for acute abdomen, ended with right hemicolectomy and we could confirm the diagnosis of MPM after completion of immunohistochemical staining of specimens. Patients with peritoneal mesothelioma often present late, and as a result treatment is often palliative. Interventions include cytoreductive (debulking) surgery and intraperitoneal chemotherapy or radiotherapy. There are a number of factors that have been consistently recognized as important in predicting better outcome in patients undergoing cytoreduction and hyperthermic intraoperative intraperitoneal perfusion with chemotherapy, such as age, histology, and the ability to achieve a complete gross cytoreduction. We present this case to draw attention of both surgeons and pathologists to this rare type of malignancy, especially when patient comes in vague abdominal symptoms and signs.

Keywords: Asbestos, mesothelioma, peritoneal


How to cite this article:
Nemenqani DM, Abdulaziz AM, Hamdounah MH. Malignant peritoneal mesothelioma in a young patient, diagnostic challenge. Saudi J Health Sci 2015;4:191-5

How to cite this URL:
Nemenqani DM, Abdulaziz AM, Hamdounah MH. Malignant peritoneal mesothelioma in a young patient, diagnostic challenge. Saudi J Health Sci [serial online] 2015 [cited 2019 May 19];4:191-5. Available from: http://www.saudijhealthsci.org/text.asp?2015/4/3/191/171438


  Introduction Top


Pleural mesothelioma is the most common type of malignant mesotheliomas. Second is peritoneal type (10–20%). Other types include pericardial and tunica vaginalis of the testis.[1],[2] More than 50% of malignant mesothelioma cases are associated with asbestos exposure.[3],[4] This tumor can occur up to 20 years after exposure.[4] Malignant peritoneal mesothelioma (MPM) affects men in the fifth to sixth decade of life.[4] Life expectancy does not exceed 1 year after diagnosis.[5] Types of MPM are wet, dry and mixed form. All share the common gross feature of nodular masses in affected part of peritoneum.[6] It is difficult to diagnose the case of MPM depending on clinical or radiological findings. Diagnosis is usually done after laporotomy.[7]


  Case Report Top


A young man 31 years presented to emergency room with 2 weeks history of central abdominal pain, distention, constipation, and frequent vomiting. Patient had no previous attacks and insignificant past medical or surgical diseases. On examination, he was in stable condition, afebrile and mildly dehydrated. Abdomen was distended with tenderness over right iliac fossa, bowel sounds were sluggish, hernia orifices were closed and rectum was empty. Patient had leukocytosis (15,000), chemistry was normal. Plain abdominal X-ray showed dilated both small and large bowel with air-fluid levels [Figure 1] and [Figure 2]. The clinical picture goes with ileus secondary to abdominal sepsis. Computed tomography (CT) scan with contrast was done for abdomen, showed rim of free fluid in Morrison pouch with localized collection in right lower abdomen and fat stranding [Figure 3] and [Figure 4].
Figure 1: Plain abdominal X-ray (erect position) showing dilated small bowel loops with multiple air-fluid levels

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Figure 2: Plain abdominal X-ray (supine) showing dilated central small bowel loops

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Figure 3: Computed tomography scan abdomen with intravenous and oral contrast showing fat stranding in the right iliac fossa

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Figure 4: Computed tomography scan abdomen with intravenous and oral contrast showing rim of free fluid in Morrison pouch with localized collection

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Diagnosis was made as complicated appendicitis, midline laparotomy done and the findings were localized pus collection around the cecum, diffuse edema and erythema of the terminal ileum with thickened mesentery and enlarged lymph nodes. The appendix looked grossly normal [Figure 5],[Figure 6],[Figure 7]. Appendix removed, for biopsy, swab taken for culture and wash of peritoneal cavity was done.
Figure 5: Operative view showing dilated edematous small bowel loops with enlarged mesenteric lymph nodes

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Figure 6: Operative view showing thick inflamed mesentery at the right iliac fossa

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Figure 7: Operative view showing dilated inflamed bowel with suppurative surface of right iliac fossa collection

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No relevant result was reached through bacteriologic examination.

Three specimens were sent for pathologic examination. First, an appendectomy specimen, the outer surface showed irregular friable tissue. Second, are soft tissue pieces, from mesentery. Last specimen was tissue over the cecum.

Microscopic examination from all revealed solid nests and papillary structures in complex pattern of mesothelial-like cells. Focal cytoplasmic vacuolations were seen. Focal areas of necrosis were seen. With no definite invasion [Figure 8],[Figure 9][Figure 10].
Figure 8: H and E, ×200 section showing solid nests and papillary structures in complex pattern of mesothelial cells (red arrow)

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Figure 9: H and E, ×400 section red arrow marking the well-formed fibrovascular core and the blue arrow pointing to cytoplasmic vacuoles

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Figure 10: H and E, ×200 section black arrow pointing to necrotic area surrounded by malignant mesothelial cells

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Immunohistochemistry proved that these cells forming the papillary structures express calretinin and epithelial membrane antigen (EMA) [Figure 11] and [Figure 12]. P53 and Ki-67 were positive in 30–40% of lesion cells. While, carcinoembryonic antigen (CEA) and cytokeratin (CK)-5/6 were negative. Hence, the final diagnosis reached was atypical mesothelial proliferation, most likely malignant mesothelioma, epithelioid type (low grade malignancy).
Figure 11: IHC, ×400 calretinin cytoplasmic positivity (arrow)

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Figure 12: IHC, ×400 showing cytoplasmic epithelial membrane antigen positivity (arrow)

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Following surgery, patient condition deteriorated in the form of high fever, more abdominal distention, increase leukocytosis (34,000), and coagulopathy (international normalized ratio 2.5).

Ultrasound abdomen showed free fluid in the abdomen, tumor markers were elevated (CEA 258 IU – normal up to 4) case discussed with the pathologist who assured that there were no criteria of inflammatory bowel disease in the specimen the appendix was normal and the lymph nodes were subjected to immunohistochemistry study to rule out malignancy.

Patient condition was worsening, so re-exploration done on the 3rd postoperative day, findings were dirty peritoneal fluid and indurated mass in the ascending colon with the same edematous terminal ileum. Decision taken to do right hemicolectomy (including the diseased terminal ileum) with primary anastomosis and drain.

Postoperatively patient condition improved clinically and discharged home on the 9th postoperative day to be followed in the outpatient clinic.

The specimen sent for histopathology consisted of part of colon, on the outer surface of colon there are multiple, small nodules, and plaques with diameter of 1–3 cm.

Microscopic examination revealed solid areas of atypical mesothelial cells and papillae with vascularized fibrous core lined by atypical mesothelial cells, some cells with vacuolated cytoplasm. Areas of necrosis and lymphocytic infiltration are also seen. These cells invade the serosa of the colon and the surrounding fatty tissue [Figure 13]. The results of immunohistochemical studies were same as the first specimens.
Figure 13: H and E, ×400 section malignant cells invade the serosa of the colon and the surrounding fatty tissue (arrow)

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The final pathology report was well-differentiated MPM. Patient referred to oncology center for cytoreduction and hyperthermic intraoperative intraperitoneal perfusion with chemotherapy (HIPEC).


  Discussion Top


Malignant mesotheliomas are rare fatal tumors arising from various serous surface such as the pleura in 65–70%, peritoneum in 30%, tunica vaginalis testis, or pericardium in 1–2%.[1]

One of the main causative agents of malignant mesothelioma is known to be asbestos fibers.[4] However, malignant mesothelioma also occurs in 20% of patients without previous exposure to asbestos and in these patients, MPM seems to be more common. Other agents are suggested to be involved in pathogenesis, such as mineral fibers, chronic peritonitis, remote abdominal radiation, and simian virus 40.[4]

Many nonspecific clinical symptoms associated with MPM include abdominal distention, abdominal pain, abdominal mass, loss of appetite, weight loss, fever, and diarrhea, but there are no disease specific symptoms.[5] Abdominal hernia was reported to occur in 6–12% of patients with MPM,[6],[7] majority of these hernias were in the inguinal region.[3]

MPMs tend to spread in sheets of tissue over the parietal and visceral peritoneal surfaces and to become confluent, and encasing the abdominal organs. Such extensive lesions may be accompanied by development of ascites.[5] However, several investigators have reported that the amount of ascites is disproportionately small in relation to the degree of tumor extension.[7]

The CT scan features of MPMs range from a "dry" picture, consisting of peritoneum-based masses, to a "wet" appearance, consisting of ascites, associated with irregular or nodular peritoneal thickening.[8] Direct invasion of adjacent abdominal organs may also be seen. Calcified plaques are uncommon in MPMs in contrast to the pleural counterpart.[9]

Ascetic fluid cytology is rarely helpful for diagnosis because the sensitivity of fluid cytology is low (32–76%), and the distinction between benign and malignant mesothelial tumors often depends on the degree of invasion, which cannot be ascertained by cytology.[10]

A final accurate diagnosis can be established only by laparoscopy or open surgery with biopsy, followed by immunohistochemical studies. Histological examination may reveal an epithelial, sarcomatoid, or biphasic pattern.[2]

Epithelial-type neoplasms account for 75% of cases and vary from well-differentiated tumors with tubulo-papillary pattern to solid sheets formed of rounded or polygonal cells.[11] This is important diagnostic dilemma to distinct between epithelioid mesothelioma and metastatic carcinoma, particularly adenocarcinoma.[12] The sarcomatoid-type neoplasms also may be indistinguishable from fibrosarcoma upon histology alone, immunohistochemical studies help a lot to solve these issues.[2]

Positive immunoreactivity for calretinin markedly increases the accuracy of diagnosis.[13] Mesothelioma cells are diffusely positive for calretinin, CK and EMA, and negative for S-100 protein, Leu-M1, CEA, thrombomodulin and placental alkaline phosphatase.[14] The tumor cells in our case also showed positive immunoreactivity for calretinin, CK-5/6 and EMA were positive while CEA was negative.

Surgical treatment, although is recommended for the MPM, complete resection is often difficult.[15]

Because MPM progresses almost exclusively in the abdominal cavity, loco regional therapies designed to control disease progression in the abdomen appear justified. Cytoreduction and HIPEC take advantage of two complementary treatments. Cytoreduction has the goal of achieving a complete resection of all grossly seen tumor, and high-dose chemotherapy is intended to treat the micrometastatic residual sites of the neoplasm.[16]

The use of HIPEC has the advantage of being the most effective way of uniformly distributing high-dose intraperitoneal chemotherapy to all of the peritoneal surfaces at risk of harboring disease and allows one to apply chemotherapy with clinically relevant amounts of hyperthermia, which is known to enhance the cytotoxic effects of multiple different types of chemotherapeutic agents.[17]

Patient outcome after this therapy is variable and that treatment is associated with some morbidity and even occasional mortality. It is applicable in selected cases with a disease burden amenable to resection and with a good performance status have been the topic of numerous reports.[18],[19]

Many independent factors associated with increased survival are suggested as epithelioid histology, low mitotic count, complete gross cytoreduction, and pathologically negative nodes as.[16],[17]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Falkenstern-Ge RF, Kimmich M, Bode-Erdmann S, Friedel G, Ott G, Kohlhäufl M. Pleural mesothelioma presenting as periumbilical metastasis: The first clinical documentation. Case Rep Oncol Med 2013;2013:198729.  Back to cited text no. 1
    
2.
Pillai K, Pourgholami MH, Chua TC, Morris DL. Ki67-BCL2 index in prognosis of malignant peritoneal mesothelioma. Am J Cancer Res 2013;3:411-23.  Back to cited text no. 2
    
3.
Tsuruya K, Matsushima M, Nakajima T, Fujisawa M, Shirakura K, Igarashi M, et al. Malignant peritoneal mesothelioma presenting umbilical hernia and Sister Mary Joseph's nodule. World J Gastrointest Endosc 2013;5:407-11.  Back to cited text no. 3
    
4.
Gemba K, Fujimoto N, Kato K, Aoe K, Takeshima Y, Inai K, et al. National survey of malignant mesothelioma and asbestos exposure in Japan. Cancer Sci 2012;103:483-90.  Back to cited text no. 4
    
5.
Yan TD, Popa E, Brun EA, Cerruto CA, Sugarbaker PH. Sex difference in diffuse malignant peritoneal mesothelioma. Br J Surg 2006;93:1536-42.  Back to cited text no. 5
    
6.
Shih CA, Ho SP, Tsay FW, Lai KH, Hsu PI. Diffuse malignant peritoneal mesothelioma. Kaohsiung J Med Sci 2013;29:642-5.  Back to cited text no. 6
    
7.
Shin MK, Lee OJ, Ha CY, Min HJ, Kim TH. Malignant mesothelioma of the greater omentum mimicking omental infarction: A case report. World J Gastroenterol 2009;15:4856-9.  Back to cited text no. 7
    
8.
Turner K, Varghese S, Alexander HR. Current concepts in the evaluation and treatment of patients with diffuse malignant peritoneal mesothelioma. J Natl Compr Cancer Netw 2012;10:49-57.  Back to cited text no. 8
    
9.
Park JY, Kim KW, Kwon HJ, Park MS, Kwon GY, Jun SY, et al. Peritoneal mesotheliomas: Clinicopathologic features, CT findings, and differential diagnosis. AJR Am J Roentgenol 2008;191:814-25.  Back to cited text no. 9
    
10.
Kindler HL. Peritoneal mesothelioma: The site of origin matters. Am Soc Clin Oncol Educ Book 2013:182-8.  Back to cited text no. 10
    
11.
Ahmed I, Ahmed Tipu S, Ishtiaq S. Malignant mesothelioma. Pak J Med Sci 2013;29:1433-8.  Back to cited text no. 11
    
12.
Munkholm-Larsen S, Cao CQ, Yan TD. Malignant peritoneal mesothelioma. World J Gastrointest Surg 2009;1:38-48.  Back to cited text no. 12
    
13.
Sandeck HP, Røe OD, Kjærheim K, Willén H, Larsson E. Re-evaluation of histological diagnoses of malignant mesothelioma by immunohistochemistry. Diagn Pathol 2010;5:47.  Back to cited text no. 13
    
14.
Panjkovic M, Lovrenski A, Eri Z, Usaj SK, Tegeltija D, Krcedinac J. The role of immunohistochemical evaluation in the diagnosis of malignant mesothelioma of the pleura. Vojnosanit Pregl 2013;70:1010-4.  Back to cited text no. 14
    
15.
Mitsuka Y, Yamazaki S, Miyakuni T, Iwama A, Funada T, Watanabe Y, et al. Malignant peritoneal mesothelioma mimicking ischemic colitis. Case Rep Gastroenterol 2010;4:238-242.  Back to cited text no. 15
    
16.
Alexander HR Jr. Surgical treatment of malignant peritoneal mesothelioma: Past, present, and future. Ann Surg Oncol 2010;17:21-2.  Back to cited text no. 16
[PUBMED]    
17.
Baratti D, Kusamura S, Cabras AD, Bertulli R, Hutanu I, Deraco M. Diffuse malignant peritoneal mesothelioma: Long-term survival with complete cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy (HIPEC). Eur J Cancer 2013;49:3140-8.  Back to cited text no. 17
    
18.
Alexander HR, Hanna N, Pingpank JF. Clinical results of cytoreduction and HIPEC for malignant peritoneal mesothelioma. Cancer Treat Res 2007;134:343-55.  Back to cited text no. 18
    
19.
Moore AJ, Parker RJ, Wiggins J. Malignant mesothelioma. Orphanet J Rare Dis 2008;3:34.  Back to cited text no. 19
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13]



 

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