Tuesday 3 November 2020

Motic Path Case - Granulomatous disease of peritoneum

GRANULOMATOUS DISEASE OF PERITONEUM MIMICKING CLINICALLY A PERITONEAL CARCINOMATOSIS.

A 52 year-old woman, with a background of surgery by pelvic endometriosis two years before, was submitted for showing symptoms of abdominal distension with pseudo obstructive episodes and malaise. Apart from endometriosis, there was no a history of chronic inflammatory diseases or other interesting pathological events. At the physical exam, ascites is discovered coinciding with radiological study by abdominal scan (SC), which reveals multiple and heterogeneous nodules at the peritoneal surface. The clinical diagnosis was of peritoneal carcinomatosis. A laparoscopic study was realized in which no other abdominal abnormalities were perceived apart from the irregular peritoneal thickening, suggesting tumour process. Two biopsies of different nodules were made.  During histological exam, no neoformative tissue was found. On the contrary, the global alteration consisted of multiple isolated or confluent epithelioid granulomas in a fibrotic background. Basically they were made of histiocyte aggregates and less often multinucleated giant cells. Neither central necrosis nor characteristically morphological signs concerning to specific inflammatory disorders were seen. Specific stains such as Ziehl-Neelsen, Periodic Acid Schiff, Giemsa or Methenamine Silver did no visualized pathogen germs. In addition, in order to rule out tuberculosis, a PCR for BK bacilli was also negative. After the fact that specific aetiologies were discarded, the patient is being treated by anti-inflammatories and she is having a slow but good response.


Delimited by a narrow layer of fibrous tissue with mature lymphocytes.

Neither central caseous necrosis nor other specific histological characteristics are seen. 

DISCUSSION

Peritoneal granulomatosis use to be a common non-specific histological response in front of multiple aetiologies. In this sense, some infections, specific chronic inflammatory processes, neoplasias, and many others can have similar microscopic responses, so that an accurate differential diagnosis must take into account. Clinically, peritoneal granulomatosis is unspecific in which patients can have abdominal distention, obstructive intestinal episodes or malaise, simulating even sometimes malignant tumours. There also exist asymptomatic cases where this entity may be discovered by radiological images or by observing directly abdominal cavity in surgeries realized by other causes. 
 
As far as infections are concerned, the tuberculosis seems to be the most frequent cause. Despite not having central caseous necrosis in the granulomas, tuberculostatic aetiology should be considered in our medium. At this respect, peritoneum use to be the most common location of the tuberculosis outside of lungs. The immigration phenomenon, immunosuppressive therapies or immunodeficiency logically have contributed to the increase of its frequency [1]. The diagnosis is difficult because not always caseous necrosis is present at histology, typical bacilli cannot be demonstrated by routine stains, or microbiological cultures can even be negatives. The diagnosis of tuberculosis continues being a challenging and new method of diagnosis, such as PCR of affected tissues improve the results [2].

More rarely other infection agents have also been incriminated in the development of peritoneal granulomatosis. Infections by Actinomyces Israelii of female genital tract exceptionally can extend to peritoneum occasioning a granulomatous response [3].

Uncommonly in children, some parasites as Ascaris lumbricoides have been related to peritoneal granulomatosis [4], making similar symptoms of intestinal or peritoneal malignant tumours [5].

Chronic inflammatory diseases have also been related with peritoneal granulomatosis and are capable of mimicking an omental-cake [6]. The sarcoidosis can appear as ascites or peritoneal granulomatosis, predominantly in females [7]. Peritoneal sarcoidosis must be considered as diagnostic possibility, and then of the tuberculosis should be ruled it out [8]. In this sense, elevated levels of Angiotensin Converting Enzyme (ACE) can contribute to differential diagnosis with other chronic inflammatory processes [9]. Many vasculitis diseases can occasionally provoke peritoneal granulomatosis, such as Wegener granulomatosis, Churg-Strauss, syndrome or rheumatoid arthritis-associated vasculitis [10], [11]. Special caution must be taken in consideration with eosinophilic granulomatosis because could be mistaken with a peripheral T-Cell lymphoma or vice versa, given to both entities could show similar histological characteristics [12], [13].

Peritoneal keratin granulomatosis is a rare event that can be secondary to neoplasias of female genital tract and mimic carcinomatosis intraoperatively. This condition can appear associated to uterine or ovarian endometrioid carcinoma with associated squamous differentiation. Histologically these granulomas contain keratin and the absence of malignant epithelium at them improves the prognosis [14], [15]. The manoeuvres of gallbladder stones during surgery could trigger the development of peritoneal granulomatosis [16]. Some foreign bodies can also produce peritoneal granulomatosis one of them is the starch. Since some decades, it is known that starch can be involved with this sort of peritoneal reaction. Starch glove powder is used by surgeons and although generally is considered as innocuous material, sometimes could develop a peritoneal reaction simulating a tuberculosis. The diagnosis is the histologic exam in which sometimes different ancillary techniques are required [17]. Similar situation can also occur with talcum powder [18].

Despite having multiple causes of peritoneal granulomatosis, there still are cases in which no aetiological agent is discovered and they are considered as idiopathic aetiology.

In summary, peritoneal granulomatosis is a chronic entity that can have multiple aetiologies. An accurate differential diagnosis must be realized to proceed the better suitable treatment in every case.


REFERENCES

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