Case Report
Open Access
Peritoneal Mesothelioma – A Rare Varient
M Tarakeshwari1, P Ganesh2 and AK Koushik3*
1Associate consultant Medical Gastroenterology, Sri Ramachandra Medical College, India
2Senior consultant and HOCS Medical Gastroenterology, Sri Ramachandra Medical College, India
3DM PG, Medical Gastroenterology, Sri Ramachandra Medical College, India
2Senior consultant and HOCS Medical Gastroenterology, Sri Ramachandra Medical College, India
3DM PG, Medical Gastroenterology, Sri Ramachandra Medical College, India
*Corresponding author: AK Koushik, DM PG, Medical Gastroenterology, Sri Ramachandra Medical College, Chennai, Tamil Nadu 600116, India, Tel:
9551556666; E-mail:
@
Received: September 29, 2017; Accepted: October 19, 2017; Published: October 30, 2017
Citation: Koushik AK, Tarakeshwari M, Ganesh P (2017) Peritoneal Mesothelioma – A Rare Varient. Gastroenterol Pancreatol Liver Disord 5(2): 1-3. DOI: http://dx.doi.org/10.15226/2374-815X/5/2/001112
Abstract
Primary peritoneal mesothelioma is a rare entity with an incidence
of 1-2 cases per million. It can occur in any age group, although the
50-60 years of age group is the most affected. Mesotheliomas are
common in pleura, only 20 percent arise from peritoneum. Peritoneal
mesothelioma histologically occurs as two variants, solid and cystic.
Solid variant is common and cystic being rare. Multicystic presentation
is rarer comparatively. High index of suspicion is essential as
histopathology alone helps in the diagnosis and early initiation of
treatment is crucial for survival. We present this case for its rarity
since very few cases of it are reported in the literature.
Keywords: Peritoneal Mesothelioma; Peritoneal Cyst
Keywords: Peritoneal Mesothelioma; Peritoneal Cyst
Case report
36 year old gentleman presented with abdominal distension
and mild diffuse pain for 3 months duration. His past medical
history was unremarkable and he had been evaluated in
various hospitals for the same complaints without a final
diagnosis (Figure 1). On physical examination he was found to
be moderately built had mild pallor and bilateral pitting pedal
edema. Abdomen examination revealed three hard nodular non
tender masses in the right hypochondrium and left iliac fossa, the
largest measuring 10 x 8 cm in the right hypochondrium. He had
graded 2 ascites.
Figure 1: He was a theatre artist by occupation and had history of swallowing
earthworms and snakes as a stunt for which he even held a Guinness
record
Liver function test was normal. Latex agglutination test
performed for hydatid disease was negative. USG abdomen
showed multiple echogenic masses in the peritoneum with
moderate ascites. Ascitic fluid analyses showed exudative ascites
with a negative cytology screen for malignancy.
CECT abdomen showed multiple ill defined enhancing non calcified peritoneal masses in entire abdomen and pelvis (Figure 2), diffuse omental fat stranding, moderate free fluid in abdomen and pelvis, with multiple para-aortic, per-iportal, peri-pancreatic lymphadenopathy (Figure 3).
CECT abdomen showed multiple ill defined enhancing non calcified peritoneal masses in entire abdomen and pelvis (Figure 2), diffuse omental fat stranding, moderate free fluid in abdomen and pelvis, with multiple para-aortic, per-iportal, peri-pancreatic lymphadenopathy (Figure 3).
Figure 2: Multiple ill-defined enhancing non calcified peritoneal masses,
Omental fat strandling
CT guided biopsy of mass was done and histopathological
examination showed features consistent with malignant cystic
lesion probably mesothelioma –multicystic variant.
Immuno-histochemistry was diagnostic of malignant mesothelioma.
Immuno-histochemistry was diagnostic of malignant mesothelioma.
NSE |
Negative |
PCK |
Positive+++ |
S 100 |
Positive++ |
Ki |
67.5% of epithelial cells |
Patient is on chemotherapy- paclitaxel based regimen and has
shown response on follow up
Figure 3: Omental fat strandling
Figure 4: Free fluid in abdomen. Ill-defined peritoneal masses
Discussion
Malignant Peritoneal Mesothelioma (MPM) was first
described in 1908 by Miller and Wynn. It is a rare neoplasm
with a rapid fatal course and a median survival of 6-12 months
The mean time from symptoms to death was 345 days [1]. MPM
accounts for 0.2% of all malignant disease and only 12.5-25% of
malignant mesotheliomas [2, 3]. It usually occurs in middle-aged
men who commonly complain of abdominal pain or a feeling of
fullness, abdominal distention or increasing abdominal girth,
nausea, anorexia, and weight loss, as in the present case. Only
50% of patients with a peritoneal origin of MPM have a history
of asbestos exposure. This association increases to 80% in
mesotheliomas with the more common pleural origin (Figure 5)
[4].
Figure 5: Gross left pleural effusion causing partial collapse of adjacent
lung
Three radiological types are described in MPM. ‘Dry-painful’
type is the most common, in which CT shows 1 large mass
or multiple small peritoneal masses, with no signs of ascites.
The ‘wet’ type is associated with intestinal distension and
ascites, widespread small nodules and plaques, and no solid
Figure 6: Histopathological examination - Numerous cystic spaces lined
by bland 1-2 cell layered cuboidal cells with minimal cytoplasm. Cysts
are separated by loosely arranged fibroblast bundles. Frequent mitoses
are seen. HPE was suggestive of malignant mesothelioma
masses. Finally, there is the ‘mixed’ type [5]. A precise diagnosis
based on imaging findings alone is not possible. Furthermore,
distinguishing a benign from a malignant process as well as a
primary from a metastatic process is also challenging. Therefore,
the definitive diagnosis of peritoneal mesothelioma depends
on histologic and immunohistochemical examination. The
three basic histological types of MPM are epithelioid (the most
frequent), sarcomatoid and mixed (biphasic). Apart from these
two variants, multicystic and well differentiated papillary have
also been reported (Figure 6).
A large number of immunohistochemical markers have been suggested for diagnostic aid, but none of the markers alone is diagnostic. However, they become very useful when used as a panel. Malignant MPM is characterized by positive staining for EMA, calretinin, WT1, cytokeratin 5/6, antimesothelial cell antibody-1, and mesothelin. Cytokeratins help to confirm invasion and to distinguish mesothelioma from sarcoma and melanoma. Immunohistochemistry is also useful to distinguish peritoneal mesotheliomas from primary papillary serous carcinoma of peritoneum, serous ovarian carcinomas, colorectal adenocarcinoma diffusely involving the peritoneum, and borderline serous tumors. In particular, calretinin, cytokeratin, and thrombomodulin are typically positive in patients with mesotheliomas and negative in those with serous carcinomas. In our case cytokeratin was positive and Ki was positive confirming the diagnosis of mesothelioma [6-8].
Peritoneal mesothelioma usually remains confined to the peritoneal cavity for most of its natural history. However, parasternal, retroperitoneal, mediastinal, axillary, supraclavicular, and cervical lymph nodes, lung, bone, liver, and umbilical (‘Sister Mary Joseph’s nodule’) metastases have all been reported as was the scenario in the present case
Mesotheliomas are almost universally considered a fatal neoplasm, and until recently the treatment options were very limited and ineffective. Tumor histopathology, previous surgical score, lesion size, gender, distribution (assessed with Gilly classification and peritoneal cancer index), and completeness of cytoreduction score represent the main prognostic index [9].
Park et al reported in his study on 18 patients undergoing Cytoreductive surgery (CRS) +hyperthermic intraperitoneal chemotherapy (HIPEC) had a two year survival rates of 80%. In studies reporting at least 2 year outcome data, median survival has varied greatly ranging between 29.5 and 100 months and five year overall survival between 30%-90% [10].
Yan et al published the largest longitudinal series with 405 patients in a multi-institutional review between 1989 and 2009. Here, 92% of patients received HIPEC most commonly with cisplatin and doxorubicin and an additional 23% subsequently received early post-operative chemotherapy between postoperative day 1 and 5, most commonly with paclitaxel. Overall median survival was 53 months and five year survival was 47% [11].
Nodal statuses, histological subtype, mitotic count, completeness of cytoreductive surgery are all prognostic factors associated with improved survival.
Molecular targeted therapy is the latest therapeutic advance that improves survival. Mesothelin, epidermal growth factor receptor, MUC 1, sphingosine kinase 1 are the various molecules targeted and of this sphingosine kinase holds promising results [12].
FTY720 (Fingolimod; trade name Gilenya, Novartis) is a FDAapproved drug for treating relapsing forms of multiple sclerosis. In addition to the immunosuppressant property, several reports about FTY720 as an anti-cancer drug in various malignancies have rapidly accumulated [12, 13].
A large number of immunohistochemical markers have been suggested for diagnostic aid, but none of the markers alone is diagnostic. However, they become very useful when used as a panel. Malignant MPM is characterized by positive staining for EMA, calretinin, WT1, cytokeratin 5/6, antimesothelial cell antibody-1, and mesothelin. Cytokeratins help to confirm invasion and to distinguish mesothelioma from sarcoma and melanoma. Immunohistochemistry is also useful to distinguish peritoneal mesotheliomas from primary papillary serous carcinoma of peritoneum, serous ovarian carcinomas, colorectal adenocarcinoma diffusely involving the peritoneum, and borderline serous tumors. In particular, calretinin, cytokeratin, and thrombomodulin are typically positive in patients with mesotheliomas and negative in those with serous carcinomas. In our case cytokeratin was positive and Ki was positive confirming the diagnosis of mesothelioma [6-8].
Peritoneal mesothelioma usually remains confined to the peritoneal cavity for most of its natural history. However, parasternal, retroperitoneal, mediastinal, axillary, supraclavicular, and cervical lymph nodes, lung, bone, liver, and umbilical (‘Sister Mary Joseph’s nodule’) metastases have all been reported as was the scenario in the present case
Mesotheliomas are almost universally considered a fatal neoplasm, and until recently the treatment options were very limited and ineffective. Tumor histopathology, previous surgical score, lesion size, gender, distribution (assessed with Gilly classification and peritoneal cancer index), and completeness of cytoreduction score represent the main prognostic index [9].
Park et al reported in his study on 18 patients undergoing Cytoreductive surgery (CRS) +hyperthermic intraperitoneal chemotherapy (HIPEC) had a two year survival rates of 80%. In studies reporting at least 2 year outcome data, median survival has varied greatly ranging between 29.5 and 100 months and five year overall survival between 30%-90% [10].
Yan et al published the largest longitudinal series with 405 patients in a multi-institutional review between 1989 and 2009. Here, 92% of patients received HIPEC most commonly with cisplatin and doxorubicin and an additional 23% subsequently received early post-operative chemotherapy between postoperative day 1 and 5, most commonly with paclitaxel. Overall median survival was 53 months and five year survival was 47% [11].
Nodal statuses, histological subtype, mitotic count, completeness of cytoreductive surgery are all prognostic factors associated with improved survival.
Molecular targeted therapy is the latest therapeutic advance that improves survival. Mesothelin, epidermal growth factor receptor, MUC 1, sphingosine kinase 1 are the various molecules targeted and of this sphingosine kinase holds promising results [12].
FTY720 (Fingolimod; trade name Gilenya, Novartis) is a FDAapproved drug for treating relapsing forms of multiple sclerosis. In addition to the immunosuppressant property, several reports about FTY720 as an anti-cancer drug in various malignancies have rapidly accumulated [12, 13].
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- Lugaresi ML, Mattioli S, Pilotti V, Guernelli N. Pulmonary metastasis of malignant peritoneal mesothelioma. Review of the literature and a case report. Minerva Chir. 2000;55(5):357–361.
- Park BJ, Alexander HR, Libutti SK, Wu P, Royalty D, Kranda KC, et al. Treatment of primary peritoneal mesothelioma by continuous hyperthermic peritoneal perfusion (CHPP).Ann Surg Oncol.1999;6(6):582–590.
- Yan TD, Deraco M, Baratti D, Kusamura S, Elias D, Glehen O, et al. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for malignant peritoneal mesothelioma: multi-institutional experience. J Clin Oncol. 2009;27(36):6237–6242. Doi: 10.1200/JCO.2009.23.9640
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