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Desmoplastic malignant mesothelioma of the pericardium: Description of a case and review of the literature

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Antonello Nicolini at Don Gnocchi Foundation milan
Antonello Nicolini
  • Don Gnocchi Foundation milan
Alessandro Perazzo
Alessandro Perazzo
Sergio Lanata
Sergio Lanata
Sergio Lanata
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Abstract

Desmoplastic mesothelioma (DMM) is a rare and highly lethal subtype of diffuse malignant mesothelioma and is often difficult to distinguish from reactive pleural fibrosis. The term "desmoplastic" refers to the growth of fibrous or connective tissue. We report the clinical, radiological, and pathological features of a primary DMM of the pericardium and a short review of the literature. A 72-year-old man was admitted presenting shortness of breath, cough, and asthenia. Computed tomography scan showed thickenings and effusions both in the pleura and in the pericardium. Histopathological diagnosis was performed by surgical pericardial biopsy and confirmed by autopsy. The patient had a history of asbestos exposure. Primary mesothelioma of the pericardium is a rare tumor occurring in the fourth to seventh decades with nonspecific symptoms and a rapid clinical course. The diagnosis is difficult and often needing a surgical pericardial biopsy. The prognosis is poor although newer antiblastic drugs seem to prolong survival times.
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Lung India • Vol 28 • Issue 3 • Jul - Sep 2011 219
epithelioid foci can occasionally be seen. The presence
of frankly sarcomatoid areas, in conjunction with one
or more of the following features, is considered highly
specific for DMM: Bland infarct-like necrosis and invasion
of the wall adipose tissue or the muscle or the lung. These
allow us to distinguish DMM from reactive serositis.[1,6]
We report a case and describe the histopathological and
immunohistochemical findings of pericardial DMM and
a short review of the literature on the subject.
CASE REPORT
Clinical picture
A 72-year-old man, previously healthy, was admitted to
the hospital with a 3-week history of shortness of breath,
cough, and fatigue. He was a lifelong nonsmoker but
with a known asbestos exposure. Physical examination
revealed diminished breath sounds and tachycardia (heart
rate 112 beats per minute); chest radiography showed
an enlarged cardiac silhouette with bilateral pleural
effusion and a cardiothoracic ratio of 53%. Transthoracic
echocardiography demonstrated a large circumferential
pericardial effusion; computed tomography scan of the
thorax showed bilateral thickening and effusion of the
parietal and mediastinal pleura associated with pericardial
thickening and circumferential effusion. Thoracentesis
INTRODUCTION
Desmoplastic mesothelioma (DMM) is a rare and highly
lethal variant of malignant pleural mesothelioma:
This subtype, which accounts for 5–10% of malignant
mesotheliomas, most commonly affects the pleura[1] and
less commonly the peritoneum and the pericardium.[2-4]
DMM was first described by Kannerstein and Churg in
1980[5] and since then the number of reports, although
sporadic, has been constantly increasing.[2] Findings of the
disease include a male-to-female ratio 2:1, a wide range of
ages (12–77 years), and a documented asbestos exposure in
14% of cases.[3] DMM is histopathologically characterized
by dense paucicellular hyalinized collagen among which
spindle or stellate tumor cells, often associated with
slit-like spaces, are arranged in a storiform patternless
arrangement. Sarcomatoid foci are usually present and
Address for correspondence: Dr. Antonello Nicolini, Division of Respiratory Diseases, Via Terzi - 43 16039, Sestri Levante, Genoa, Italy.
E-mail: antonello.nicolini@fastwebnet.it
Case Report
Desmoplastic mesothelioma (DMM) is a rare and highly lethal subtype of diffuse malignant mesothelioma and is often
difcult to distinguish from reactive pleural brosis. The term “desmoplastic” refers to the growth of brous or connective
tissue. We report the clinical, radiological, and pathological features of a primary DMM of the pericardium and a short
review of the literature. A 72-year-old man was admitted presenting shortness of breath, cough, and asthenia. Computed
tomography scan showed thickenings and effusions both in the pleura and in the pericardium. Histopathological diagnosis
was performed by surgical pericardial biopsy and conrmed by autopsy. The patient had a history of asbestos exposure.
Primary mesothelioma of the pericardium is a rare tumor occurring in the fourth to seventh decades with nonspecic
symptoms and a rapid clinical course. The diagnosis is difcult and often needing a surgical pericardial biopsy. The
prognosis is poor although newer antiblastic drugs seem to prolong survival times.
KEY WORDS: Asbestos exposure, desmoplastic malignant mesothelioma, involvement, pleura-pericardial
Desmoplastic malignant mesothelioma of the pericardium:
Description of a case and review of the literature
Antonello Nicolini, Alessandro Perazzo, Sergio Lanata1
Division of Respiratory Diseases, 1Histopathology Unit, Hospital of Sestri Levante, Genoa, Italy
ABSTRACT
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DOI:
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220 Lung India • Vol 28 • Issue 3 • Jul - Sep 2011
Nicolini, et al.: DMM of the pericardium
was initially performed: Cytological evaluation of the
fluid was negative for neoplastic cells and hyaluronic
acid level was 0.8 mg/ml. Follow-up echocardiography
revealed an augmenting of pericardial fluid and presence of
effusive constrictive pericarditis. The patient underwent a
pericardial window that allowed us to treat the pericardial
effusion. In the following days the patient’s clinical
status deteriorated and his condition prevented us from
beginning antineoplastic chemotherapy. The patient died
20 days after the cardiac operation.
Histopathological ndings (Pericardial biopsy obtained
during surgery)
The pericardial sample was very thickened homogeneously
due to inflammatory infiltrates and fibrosis; at one side
there is mesothelium without alterations (pleura); at
the other side the mesothelial and submesothelial cells
are very atypical and have an anomalous phenotype
(they stain with cytokeratin pool and vimentin, but not
with calretinin, HBME-1, cytokeratin 5/6 desmin D 33,
epithelial membrane antigen (EMA) and carcinoembryonic
antigen CEA). The case is of difficult interpretation and
controversial: The atypical cells are suggestive of malignant
mesothelioma (pleomorphic or lymphohistiocytoid), but
the inflammatory fibrosis is suggestive of a pericarditis
with reactive mesothelial atypias. The final diagnosis was
concordant with desmoplastic malignant mesothelioma.
Postmortem examination
The autopsy revealed a huge white tumor mass that
surrounded and encased the heart and the large vessels
(aortic arch, pulmonary artery, and veins and venae cavae)
with strong pleuropulmonary adherence; macroscopically
this infiltrates myocardial tissue. Microscopically it is a
paucicellular tumor consisting of dense collagenized tissue
in which there are spindle or stellate malignant mesothelial
cells arranged in a storiform pattern. The cells have
eosinophilic cytoplasm, indistinct cytoplasmic border, and
central atypical pleomorphic nucleus with hyperchromasia
and central nucleolus and variable numbers of mitoses.
Inflammatory infiltrate is present and this consists of
lymphocytes and histiocytes.
Immunohistochemistry
Neoplastic elements stain with calretinin, cytokeratin 5/6,
WT1, D2-40, and cytokeratin 7; no staining with MOC31.
There is infiltration at one side of the myocardium and
adipose tissue and at the other side of the chest wall, with
foci of necrosis. The lungs have no neoplastic localization.
DISCUSSION
Primary mesotheliomas of the pericardium are exceedingly
rare tumors, but paradoxically they are the most common
tumors of the pericardium(they may occur in diffuse,
multiple, or localized form). In one of the largest necropsy
series in a Canadian epidemiology survey, the incidence
of the disease was reported to be 1 in 40 million with
an incidence of 0.0022%. Most of the pericardial
mesotheliomas are multiple or diffuse growths encasing the
heart.[7] The disease occurs in over half of the cases in the
fourth to seventh decades[3,6-11] with a male-to-female ratio
2:1,[3,6] although it is lower than the ratio for mesothelioma
of the pleura (approximately 3.5:1).[8-11] Presenting signs
and symptoms are nonspecific and are related mostly
to the compromise of the cardiac function caused by
tumor mass, cardiac, or pleural and pericardial effusion
or both.[3,6-10] The role of asbestos exposure is not clear,
although it has been documented in a few patients.
[3,9,10]
The clinical course of the disease is often rapid and the
mean survival time from onset of symptoms to death is
5–8 months for the sarcomatous variant and 6–8 months
for the biphasic variant.[4,6,10] Cardiac tamponade is a well-
known complication of the malignancy and often responds
initially to pericardiocentesis or to pericardial window,[10]
but some fatal cases have been reported.[11] Besides,
commonly used imaging studies (echocardiography
and computed tomography) do not appear to offer great
sensitivity to the presence of a pericardial mass,[3] and
effusion cytology reveals malignant cells in only 20% of
cases.[3] Magnetic resonance imaging and positron emission
tomography-computed tomography (PETTC) have in some
cases successfully identified the presence of a pericardial
mass.[11] The treatment of the disease tends to be mainly
palliative rather than radical and based on surgery,
chemotherapy, and radiotherapy. Radiotherapy has not
proved beneficial.[12] The use of new drugs offers further
therapy options: The therapeutic schemes generally used
are mainly a combination of platin with gemcitabine or
paclitaxel. In recent years, pemetrexed, a new antifolate
drug, in combination with cisplatin has achieved a
significantly increased patient survival compared with
the other antiblastic drugs.[13-15] In conclusion, the
diagnosis of the malignancy is very difficult and often
incidental and prognosis extremely poor, although newer
chemotherapeutic regimes seem to prolong survival times.
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How to cite this article: Nicolini A, Perazzo A, Lanata S. Desmoplastic
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... Similarly, core biopsy samples may not definitively establish the diagnosis and an open pericardial biopsy may be required. 6 Desmoplastic malignant mesothelioma is a variant of the sarcomatoid subtype, characterized by paucicellular hyalinized collagen among which spindle or stellate tumour cells occur in a storiform patternless arrangement. Sarcomatoid foci are usually present 6 and areas of necrosis and distinct tissue infiltration are required for definitive diagnosis. ...
... 6 Desmoplastic malignant mesothelioma is a variant of the sarcomatoid subtype, characterized by paucicellular hyalinized collagen among which spindle or stellate tumour cells occur in a storiform patternless arrangement. Sarcomatoid foci are usually present 6 and areas of necrosis and distinct tissue infiltration are required for definitive diagnosis. Only a few cases have been reported in the literature. ...
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Article
  • Mar 2011
Primary malignant pericardial mesothelioma is an extremely rare tumor. One of the largest autopsy series gave an incidence of primary pericardial tumors of 0.0022%, of which mesothelioma is the most common type. A male predominance of the disease has been described, and the majority of cases occur in the fourth to seventh decades of life. Unlike peritoneal and pleural mesothelioma, there has been no definite correlation between asbestos exposure and pericardial disease. Malignant pericardial mesothelioma carries a poor prognosis with few successful treatment strategies and little benefit from radiation and chemotherapy. We report a case of a 66-year-old man who presented with shortness of breath, right shoulder pain, and peripheral edema of the lower limbs. A large pericardial effusion was seen on echocardiography, which was drained but the patient died the following day. A malignant tumor was found on autopsy and a final diagnosis of primary malignant pericardial mesothelioma was made following histopathological examination.
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Desmoplasic diffuse mesothelioma
Article
  • May 1982
  • AM J SURG PATHOL
Twenty-seven cases of desmoplastic diffuse malignant mesothelioma (26 pleural, one peritoneal) are described. In 19 cases the tumor cell type was sarcomatous and in six others it was biphasic (malignant elements of both epithelial and mesenchymal aspect). There were only two cases where the tumor cell type was purely epithelial. The clinical course was often rapid; the mean survival period in 11 cases of purely sarcomatous type was 6.18 months. Only one case of purely sarcomatous type lived for more than 1 year as opposed to four of eight cases with an epithelial component. Metastases occurred more frequently in desmoplastic (60.1%) than in nondesmoplastic diffuse mesothelioma (42.5%). The tumor cell type (epithelial, mesenchymal) accompanying desmoplastic mesotheliomas and not the extent of desmoplasia determines their behavior. Desmoplasia in diffuse mesotheliomas is often the result of tumor cells assuming the functional capacity of fibroblasts and has frequently been confused with reactive fibrosis. Cytologic abnormalities, tissue infiltration, and foci of necrosis indicate the neoplastic nature of the process in most instances.
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Malignant pericardial mesotheliomas and asbestos exposure: A case report
Article
  • Feb 1982
Three cases of malignant pericardial mesotheliomas are presented with evidence of occupational asbestos exposure. Examination results are compared with findings from experimental and epidemiological research on biological effects of asbestos dust. There are sufficient indications that time-limited effects of asbestos dust established either by measurements or assessment of the amount of concentration after a latency of more than 20 years are apt to result in the development of mesotheliomas of the pleura and peritoneum and, moreover, the pericardium. It is suggested that malignant pericardial mesothelioma also be recognized as another form of occupational disease caused by asbestos dust.
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Primary malignant mesothelioma of the pericardium: Case report and literature review
Article
  • Feb 1994
Pericardial mesothelioma is a highly lethal and fortunately rare cardiac neoplasm. We present the clinical and pathologic features of a primary sarcomatoid mesothelioma. To better understand the clinical, radiographic, and pathologic features of this entity, we reviewed 27 cases described in the English literature from 1972 through 1992, which, together with our case, provided a total of 28 cases. Findings of the review include a male-female ratio of 2:1, a wide age range (12 to 77 years; mean, 47 years), and documented asbestos exposure in 4 of 28 (14%) patients. Commonly used imaging studies do not appear to offer great sensitivity, for a mass was detected by echocardiography in only 2 of 16 (12%) patients and by computed tomography in 4 of 9 (44%). Pathologic findings revealed a diffuse growth pattern in most cases (18 of 25, or 72%), together with an equal distribution between the biphasic, epithelioid, and sarcomatoid variants. Effusion cytology revealed malignant cells in only 2 of 10 (20%) cases. With or without therapy, prognosis was uniformly poor, since 24 of 27 patients were dead of the disease at the time the reports were published.
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