What is Pseudomyxoma Peritonei (“PMP”)?
The term Pseudomyxoma Peritonei has been and still is used to mean a
number of different things. This has created much confusion for patients
and physicians. Efforts are underway to better define PMP in the
medical literature so that there is a better understanding of the term
and communication between physicians and patients. Generally speaking,
PMP is a rare condition characterized by the presence of mucin in the
abdominal cavity. A number of types of tumors can cause PMP, but the
most common cause is appendix cancer – including low grade mucinous
neoplasms of the appendix.
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abstract
BACKGROUND:
Synchronous
(existing or occurring at the same time) ovarian/appendiceal mucinous neoplasms sometimes occur in the absence
of clinical
pseudomyxoma peritonei (PMP), which raises a question about
whether the 2 tumors could be independent.
METHODS:
We
identified 11 cases of synchronous ovarian/appendiceal mucinous
neoplasms without PMP and subclassified them into groups 1 and 2 based
on the presence or absence of microscopic peritoneal/ovarian surface
mucin deposits. A 7-marker panel (CK7, CK20, CDX2, PAX8, MUC1, MUC2, and
MUC5AC) immunohistochemistry was performed on both tumors.
RESULTS:
Between
the 2 groups, there were no significant differences in age, laterality,
size, and histology of ovarian/appendiceal tumors. In group 1, 2 of 4
cases developed PMP later, and both had ovarian surface and
contralateral ovarian involvement and appendiceal perforation with
microscopic mucin deposits on the peritoneum. No patients in group 2
developed PMP. All group 1 cases showed a high degree of concordance of
immunoprofile between the synchronous tumors, with an identical
expression of appendiceal pattern in greater than 90% of the markers. In
group 2, only 1 of 7 cases showed concordance in all markers.
CONCLUSIONS:
If
peritoneal mucin deposits present, even microscopic and acellular, the
synchronous tumors are most likely of a single appendiceal origin.
Otherwise, they are more heterogeneous, and some may be truly dual
primaries.
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