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Mesothelioma of the peritoneum represents a malignant tumour of the mesotheliomatous cells of the peritoneum, and is similar to the mesothelioma of the pleura. The causative relation with chronic exposure to asbestos which has been proven for mesothelioma of the pleura has been confirmed also for abdominal mesothelioma[1,2]
The annual incidence of the tumour in the general population is 1-2 cases per million,[3] and it usually affects older males, over 50 years of age. The symptomatology of the tumour is usually not specific, leading to a delay in diagnosis.[4] Often, the patient presents with gradually developing ascites and weight loss, which in most cases indicates an intraperitoneal spread of the tumour. We here present the case of a 31-year-old woman with postmortem diagnosis of peritoneal mesothelioma to sensitise physicians to the possibility of mesothelioma as an aetiological consideration in cases of recalcitrant ascites and upper gastrointestinal haemorrhage.
Case Report
A 31-year-old female postgraduate student who was on therapy for dysfunctional uterine bleeding was referred to the Gynaecology department on account of progressive abdominal swelling of eight months duration with associated vomiting, weight loss and constipation. There was no history of fever, fleeting arthralgia, cough or ingestion of unpasteurised milk.
Physical examination revealed gross ascites, umbilical nodule and an irregular mass in the posterior fornix and anterior rectal wall. This mass was later confirmed as a cervical polyp. Diagnostic possibilities considered were abdominal tuberculosis to exclude an intra-abdominal malignancy.
Investigations carried out included liver function tests, electrolytes and urea, creatinine, carcinoembryonic antigen, alpha-fetoprotein, hepatitis B surface antigen, hepatitis C virus antibody and retroviral screening, results of which were all normal. Gastroduodenoscopy and colonoscopy did not reveal any intraluminal or mucosal lesion. Ascitic fluid cytology and cytology of the umbilical nodule both showed inflammatory cells. A peritoneal biopsy was not done due to lack of an appropriate needle.
Computerised tomography of the abdomen showed the presence of ascitic fluid and right suprarenal mass . She was placed on oral empirical antituberculous drugs, which were poorly tolerated because of the profound nausea and vomiting she suffered.
She subsequently developed a massive upper gastrointestinal bleed and petechial rash and died in a few days. Post-mortem examination revealed an extensive tumour of the peritoneum, infiltrating the small bowel loops as shown in Figure 1.
Discussion
Mesothelioma of the peritoneum presents as an extremely rare malignancy of the abdominal cavity. Most cases occur in patients over 40 years of age, but it has also been described in young adults, children and neonates.[5] A significant male predisposition towards development of this tumour has been noted.[6,7] This case involved a 31-year-old female patient whose diagnosis was only made at autopsy.
About half of the cases seem to be associated with chronic heavy exposure to asbestos, with a latency period of approximately 15 years. Exposure to thorotrast, which has also been implicated as a possible causative factor[8,9] was not seen in our patient. Malignant mesothelioma has been known to arise from any serous surface of the human body, and the coexistence of malignant peritoneal and pleural mesotheliomas has also been described, usually associated with heavy chronic asbestos exposure.[10,11] The pleurae were normal in this index case. Ascoli et al[12] have also described a case of concomitant malignant mesothelioma of the pleura, peritoneum and tunica vaginalis testis and extension to the retroperitoneal space is said to be extremely rare but possible.[13]
The symptomatology of the tumour is usually not specific, leading to a delay in diagnosis and possible effective treatment of the tumour.[3,6,10,14,15] In some instances, the tumour may manifest in the umbilicus, and rarely, metastatic inguinal or cervical lymph nodes may be the first signs of the tumour.[16] This interesting observation is true for our patient who presented with recurrent ascites, weight loss and an umbilical nodule. The cytology of the umbilical nodule (Sister Joseph’s) was not helpful in guiding us towards a definitive diagnosis. Tumour markers (alpha-feto protein and carcinoembryonic antigen), assayed in our patient were both within the normal reference range. Her case was rather unusual in that none of the investigations performed assisted in arriving at a possible diagnosis.
Cytology of aspirated ascitic fluid may lead to a diagnosis, although the method cannot always distinguish malignant mesothelioma from metastatic carcinoma.17 However, diagnostic laparoscopy, which permits direct biopsy of the tumour3 may increase the diagnostic yield of laboratory tests. The main differential diagnoses of malignant mesothelioma include reactive mesothelial hyperplasia and metastatic carcinoma, particularly of the serous papillary type.[17] Abdominal tuberculosis is also a close differential and has to be excluded in an endemic region for tuberculosis as Nigeria.
The prognosis of malignant peritoneal mesothelioma is extremely poor. Most patients die of the disease within 2 years of diagnosis. Some encouraging results were however reported with a combination of surgical debulking and intraperitoneal or systemic chemotherapy.[18,19] The use of cisplatin and mitomycin or anthracycline has been proposed by some authors.[20,21]
Though primary mesothelioma is an extremely rare malignancy, early diagnosis and appropriate treatment has been shown to improve the patient’s quality of life, and we suggest that this rare condition be considered when dealing with a case of unexplained ascites in the absence of other common conditions.
References
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