second opinion - Peritoneal Carcinosis of Undefined Nature

This is a summary of 57 years old patient that was interested with receiving an expert second opinion. When the patient was 2 years old he had appendectomy, at 9 years old - intestinal invagination operation affecting the right side and iliac fossa, with subsequent hardening of the scar and the appearance of a sub-scar asymptomatic mass, interpreted as a cicatricial reaction. When the patient was 38 Years old - Dupuytren and at 50 years old - Laparoscopic Cholecystectomy.

On December 2004 and several months later the patient suffered from intestinal sub-occlusion ileus. A colonoscopy was performed which was negative. On November 2005, a surgical intervention took place with the finding of an adhesion mass in mid right abdomen. 700 cc of brown exudates was drained. Right Hemi-Colectomy was preformed.

The sections that were macroscopically tested was an adhesive mass in the size of 8*10*6 cm found that consisted of the terminal iliem and the cecum at a length of 18 cm.

Microscopically the sections of the intestines were diagnosed (by the Histopathological and Cytodiagnostic laboratory at the Riunit hospital of Trieste) as Carcinoma of low grade differentiation. Same findings were found in adipose tissue with pseudo glandular aspects. Other parts of intestine showed the same microscpical appearance also with papillar aspects. Markers - negative (CEA-2.10, Ca19.-2.5, Ca125-5.4).

On CT: small amount of fluid. Modest evidence of peritoneal inflammation and some adhesions on abdominal wall.

Re-examination of the surgical material on Januarys 5th by the National Tumor Institute suggested the diagnosis of malignant Mesothelioma monophasic of epithelial type.

Conclusion: Patient with Epithelia Peritoneal Mesothelioma that experienced his first episode of intestinal sub-occlusion on 2004.

On the 01.10.06, the patient has undergone a new examination at the Clinical Pharmacology and New Pharmaceuticals Division of the European Institute of Oncology, whose anamnesis reports an. In December 2004 a sub-occlusive episode is reported, affecting the small intestine, which spontaneously healed. A CAT scan is performed, with irrelevant results. During summer 2005, the abovementioned episodes occur again and the patient undergoes a colonoscopy with irrelevant results.

In November 2005 he undergoes the examinations and operation we have mentioned in the previous report.

In light of the information above, the specialist suggests to await the results from new histology analyses and to repeat a thorax, abdomen and pelvis CAT scan.

Should the hypothesis of a mesothelioma be confirmed, it is suggested to consult the opinion of a colleague surgeon who is expert in peritonectomy and intraperitoneal hyperthermic treatments, as this is considered the most efficient approach.

In the alternative, it is suggested to monitor the clinical trend throughout time (CAT and PET scans after 3 months); however, only when presenting an evolving situation or if a clear pathology is denounced via the CAT scan, the specialist would suggest a systemic chemotherapy treatment.

On the other hand, should the histology be different, it is suggested to nonetheless repeat a CAT and a PET scan in a month, and, in absence of a clear primitiveness, it is advised to still consult the colleague surgeons for a peritonectomy.

The new histopathology examination performed at the European Institute of Oncology on the 01.11.2006 reports: "Evidence compatible with a malignant epithelial mesothelioma infiltrating the small intestine's wall. Immunophenotype of the neoplastic population: positive as per calretinin, cytokeratin 5/6 and WT1; negative as per CDX-2, CEA 5 and desmin."

Another histology examination performed at the Milan Cancer Institute on the 01.13.2006 reports: "Morphological and immunophenotypic pictures coherent with an epithelial type of malignant mesothelioma. Immunoreactivity: Calretinin +, CK 5/6 +, WT 180 +, CD31 -."

The thoracic-abdominal CAT scan with contrast performed on the 01.16.2006 reports: "In the thorax area neither parenchymal nor pleural alterations are reported, nor mediastinal lymphadenopathies. In the abdominal region no focal hepatic lesions are appreciated, nor signs of dilation of the bile-duct subsequently to a cholecystectomy. A minimal perihepatic and perisplenic liquid layer is at all times appreciable, with a modest and homogeneous peritoneal inspissation of the suprahepatic and suprasplenic zones; pancreas, adrenal glands and kidneys in normal conditions (30mm cortical cyst with greater diameter between the middle third and the lower third of the right kidney); lymph nodal granules (with dimensions not exceeding one centimeter) in periaortocaval area and along the iliac femoral axis. Diffused and modest inspissation of the months, with ansae that appear slightly conglutinated and adhering to the abdominal wall and with a minor reduction in the transparency of the mesenterial adipose tissue, in a situation that could also be compatible with the sequence of repeated sub-occlusive episodes and the consequent surgical actions. In the pelvic hole, normally extended bladder, with regular walls; no abnormal tumefaction is evident."

On the 01.20.2006, the patient finally visited the surgeon he had addressed to by the medical doctor who had examined him on the 01.10.2006, and the former procured the following conclusion:

"Patient with peritoneal epithelial mesothelioma that, by interpreting the first sub-occlusive episode in 2004 as secondary to such pathology, seems to date back to some time ago and appears with a low degree of biological malignity. The CAT scan seems to show diaphragmatic involvement and a significant adhesion syndrome between ansae and abdominal wall. In order to apply a precise surgical indication, an interview with the surgeon who operated the patient in November 2005 seems indispensable, so as to evaluate the involvement of the visceral peritoneum and above all of the small intestine, the latter being a true contraindication to a surgical approach.

The cytoreduction via chemo-hyperthermia, followed by systemic chemotherapy seems to be the best option (even though experimental). Should there be, on the other hand, doubts about the surgical indication, one would opt for systemic chemotherapy, eventually with neoadjuvant intention.

It is very important for the patient to know if there are other diagnostic procedures. Assuming the histological diagnosis is Peritoneal Mesothelioma, what is the recommended therapy and if there are experimental protocols, including immunotherapy.

The case was sent to Medical Opinion ( ) for second opinion evaluation. The case was sent to senior professor from Tel Aviv University to review the case.

The professor assumed that the diagnosis was mesothelioma according to the various pathological reports. It is important to have immunohistochemical staining for c-kit, EGFR, VEGFR, PDGFR-alpha for possible targeted therapies.

The best treatment option for mesothelioma is radical surgery: peritonectomy + hyperthermic intra-operative administration of chemotherapy. However, it is hard to imagine the real intra-abdominal involvement by the tumor according to the descriptions given by the radiologists. It is recommended to review the CT scans and perform a PET -CT with FOG to locate all tumor sites.

If the tumor is inoperable, it is better to go for chemotherapy: cisplatin + pemetrexed (Alimta), or cisplatin + gemcitabine, as a palliative treatment or as a neo-adjuvant therapy.