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RA, reactive aspecic; WDPM, well-differentiated papillary mesothelioma; AT, adenomatoid tumor; NHMH, nodular histiocytic/mesothelial hyperplasia; PMM, peritoneal
multicystic mesothelioma.
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Sometimes AT has to be histologically differentiated from adenocarcinomas, vascular tumors, and on rare occasions, from malignant
epithelial mesotheliomas with a dominant tubular/glandular pattern.
Appropriate immunohistochemical reactions, such as CEA and other
possible markers for specic adenocarcinomas as well as endothelial
markers, usually help to clarify the diagnosis in selected cases.
Rarely, cases of AT having an inltrating local pattern have been
reported, but the behavior of AT is usually indolent and benign.
Mesothelioma of the Atrioventricular Node
The so-called mesothelioma of the atrioventricular node is not a true
mesothelioma. This denition is a misnomer based on historical observations regarding the similarity of the proliferative cells with mesothelial cells and the lesions pattern with that of an adenomatoid tumor.
Today, it has been accepted that it arises from a congenital heterotopia
of endodermal tissue (2224). The large majority of these tumors has
been detected during autopsy (some sporadic cases have been reported
in transplanted hearts) and most of them, although inconspicuous,
range in size from a few millimeters to 1 to 2 cm and have been considered the cause of death in cardiac arrest or ventricular brillation (23).
Macroscopically, this tumor often exhibits micropolycystic features
in the area of the atrioventricular node. Microscopically, microcystic
spaces are lined by bland, at, mesothelioid cells that are immunoreactive for CK; positivity for CEA also has been reported.
Cystic/Microcystic Pattern
Although cases of AT with a dominant microcystic pattern have been
reported, the best example of a lesion characterized by this pattern is
the peritoneal multicystic mesothelioma.
Peritoneal Multicystic Mesothelioma
Peritoneal multicystic mesothelioma (PMM) arises almost exclusively
from the peritoneum (2,25); exceptional cases have been described in
the testis (26) and pleura (27). Like adenomatoid tumor, the histogenesis of PMM has also been controversial, the true mesothelial origin
having been conrmed only recently by ultrastructural and immunohistochemical studies. Cystic mesotheliomas, arising from serosal peritoneal membranes, can apparently involve the parenchyma of single
peritoneal and pelvic organs. The common clinical setting is the pelvic
peritoneum of young female patients; on the basis of the size of the
proliferation, it can be accidentally detected, present vague symptoms,
or show a palpable abdominal mass and pain; ascitis is rarely present.
It can be also multifocal with synchronous or metachronous proliferating lesions in several parts of the abdomen and pelvis. Macroscopically, one cyst (in this case, terms such as cystic mesothelioma and
mesothelial cyst seem more appropriate) or several cysts with thin
walls and variable size are present; cysts usually have a uid content
(2,25).
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Reliable criteria of malignancy, in absence of frank sarcomatous overgrowths, are currently being considered (6): absence of a zonal effect
(consisting of a supercial high cellularity and deep paucicellularity,
usually present in chronic brotic reactions); invasion of surrounding
tissues (adipose tissue, skeletal muscle, lung parenchyma); the socalled bland necrosis, typical of DMM and consisting of circumscribed
areas in which necrosis is demonstrated by a poorly stained eosin; and
absence of an elongated capillary vessel perpendicular to the serosal
surface as an expression of the reactive granulation tissue usually
present in CFP.
Immunohistochemistry is usually of little help; it is remarkable that,
after an injury causing denudation of mesothelial layers, submesothelial
broblasts that normally expressed vimentin only acquire immunoreactivity for low molecular weight cytokeratin. For this reason, in the presence of mesenchymal mesothelial proliferations, the positivity for
cytokeratin should not be considered as diagnostic of desmoplastic
mesothelioma (2,6). Nevertheless, a clear immunonegativity, or a
weakly focal positivity for cytokeratin, favors the diagnosis of brosing
pleurisy, and the immunopositivity of brosclerosing proliferation inltrating lung parenchyma or striated muscle favors that of DMM.
Localized Fibrous Tumor of the Pleura
Localized brous tumor (LFT) of the pleura, although variously
named, has been thought for many decades to arise from surface
mesothelial cells and, therefore, to be a benign mesothelioma. Today, it
is considered a pleural localization of a potentially ubiquitous lesion of
mesenchymal origin (34). It can arise in the pleura of patients of both
sexes and of any age. In about 50% of cases, the tumor is asymptomatic
and incidentally found; otherwise, cough, pain, and dyspnea are
common symptoms. Typically, LFT is separated from the (generally
visceral) pleura by a peduncle, resulting in a polypoid mass, which
can also reach great size (up to 40 cm) and consistent weight. The cut
surface is rmly brous.
Microscopically, several features have been described: sclerosing,
myxoid, and hemangiopericytomatous. Typically LFT is immunoreactive for CD 34 (35); the positivity for this marker is needed to conrm the diagnosis (Fig. 30.6). Bcl-2 (36) and CD 99 (37), both positive
in the majority of cases, are considered useful to distinguish LFT from
sarcomatoid malignant mesothelioma, which is only sporadically
immunoreactive for them (37,38).
Some cases of LFT have a malignant behavior; histological criteria
for selecting them are similar to those of other mesenchymal neoplasias: an increase in cellularity, nuclear atypias, an inltrative pattern,
and a greater mitotic index (more than 4 high-power elds). Solitary
brous tumors have been described everywhere, including the pericardium (39), vaginalis testis (16) and the peritoneum (40).
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Figure 30.6. Solitary brous tumor of the pleura. The diagnostic positivity for
CD 34 is shown.
References
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2. Battifora H, McCaughey WTE. Tumors of the Serosal Membranes. Atlas of
Tumors Pathology, 3rd series, fascicle 15. Washington, DC: Armed Forces
Institute of Pathology, 1995.
3. Bolen JW, Hammar SP, McNutt MA. Reactive and neoplastic serosal tissue.
A light microscopic, ultrastructural and immunohistochemical study. Am
J Surg Pathol 1986;10:3437.
4. Bolen JW, Hammar SP, McNutt MA. Serosal tissue: reactive tissue as a
model for understanding mesotheliomas. Ultrastruct Pathol 1987;11:251
262.
5. Henderson DW, Shilkin KB, Whitaker D. Reactive mesothelial hyperplasia
vs mesothelioma, including mesothelioma in situ. Am J Clin Pathol 1998;
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