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Prognostic factors and surgical indications of pulmonary epithelioid
hemangioendothelioma: a review of the literature.
Ann Thorac Surg. 2006 Dec;82(6):2010-3
BACKGROUND:
Pulmonary epithelioid hemangioendothelioma is a rare vascular tumor of
low-grade malignancy, the prognosis of which remains unpredictable.
The purpose of this analysis based on 80 patients was to determine
prognostic factors and to evaluate results of surgery. METHODS: We
recorded data of 75 patients from reports published in the English and
French literature using the terms "intravascular bronchoalveolar
tumor" or "pulmonary epithelioid hemangioendothelioma" or a
combination of both. We added to this database 5 more cases of
pulmonary epithelioid hemangioendothelioma operated on in our thoracic
surgery department from 1989 to 2005. Univariate and multivariate
analyses of prognostic factors were performed using the log rank test
and the Cox model. The factors we tested were age, sex, clinical
symptoms, biologic and radiologic findings, and surgical treatment.
RESULTS: There were 49 women and 31 men with a mean age of 39.7 years
(range, 7 to 72 years). The 5-year survival probability was 60%
(range, 47% to 71%). Univariate analysis showed that loss of weight,
anemia, pulmonary symptoms, and more particularly pleural hemorrhagic
effusions were significant factors of poor prognosis, with a median
survival of less than 1 year. Multivariate analysis showed a
statistically worse survival in patients with hemorrhagic symptoms (hemoptysis,
p < 0.0001; pleural effusion, p < 0.0001). CONCLUSIONS: Pulmonary
epithelioid hemangioendothelioma typically occurs among young
patients. Surgery can be proposed in cases of unilateral single or
multiple nodules. There is no single effective treatment in cases of
bilateral multiple nodules. Lung transplantation should be evaluated
in patients with vascular aggressivity with pleural hemorrhagic
effusion and anemia.
Risk factors and independent predictors of survival
in patients with pulmonary epithelioid haemangioendothelioma. Review
of the literature and a case report.Respirology.
2006 Nov;11(6):818-25
Pulmonary
epithelioid haemangioendothelioma (PEH) is a rare pulmonary neoplasm.
A patient with PEH with lymph node and pleural metastases that were
discovered incidentally is described. An abnormal left upper lobe
shadow was noticed on CXR in a 70-year-old woman during an assessment
for the sudden onset of nausea and vomiting. Transbronchial lung
biopsy did not provide a diagnosis. Lobectomy and lymph node resection
were performed. The histological diagnosis of PEH was confirmed
immunohistochemically by positive reactions to factor VIII-related
antigen and CD34. Data on 93 patients with PEH including the present
case report were analysed by Cox regression analysis using forward
stepwise method to identify the risk factors, and the independent
predictors of survival in patients with PEH. It revealed that male,
symptomatic patients, presence of cough, haemoptysis, chest pain,
multiple unilateral nodules, pleural effusion, metastases to more than
one site and lymph node metastases were all significant risk factors
for PEH (P<0.05). Symptomatic patients and presence of pleural
effusion were the independent predictors of survival in patients with
PEH.
Pulmonary
epithelioid hemangioendothelioma: report of a case with fine needle
aspiration biopsy.Acta
Cytol. 2006 Jul-Aug;50(4):455-9
BACKGROUND:
Pulmonary epithelioid hemangioendothelioma (PEH) is a rare, low
grade, malignant vascular tumor that typically presents with
multiple pulmonary nodules in young women. This report details the
cytopathologic and pathologic findings in an unusual case presenting
in an older male with a pleural effusion, dominant nodule and
multiple bilateral infiltrates. CASE: A 62-year-old, male nonsmoker
was referred due to increasing dyspnea. Chest radiography revealed a
pleural effusion and nodular infiltrate in the right upper lobe of
the bronchus. Thoracocentesis and thoracoscopy were performed, with
a pleural drain inserted. Bronchoscopy revealed a right upper lobe
bronchus occluded by a greyish, necrotic mass. Various
cytopathologic sampling techniques, including fine needle aspiration
biopsy, as well as traditional histopathologic biopsies were
performed. Cytologic specimens showed loosely cohesive, epithelioid
cells that were binucleated and multinucleated. Chromatin was
granular, with scattered, small, multiple nucleoli with occasional,
variably sized cytoplasmic vacuoles. The patient's condition
deteriorated, and he died 3 weeks after admission. CONCLUSION:
Pulmonary epithelioid hemangioendotheliomas are unusual neoplasms
with a epithelioid, discohesive cellular appearance. It can mimic
other, more commonly seen pulmonary neoplasms. Careful attention to
cytomorphologic features and application of ancillary studies assist
in making the diagnosis.
Epithelioid
hemangioendothelioma of the lung (IVBAT)--clinicopathological and
immunohistochemical analysis of 11 cases.
Pathologe. 2006 Mar;27(2):106-15.
Epithelioid
hemangioendothelioma (EHE) of the lungs (syn.: intravascular
bronchioloalveolar tumor/IVBAT) is a neoplasm with a vascular line of
differentiation. This study provides a clinicopathological analysis of
11 cases of this tumor. There were 7 female and 4 male patients.
Patients' age ranged from 33 to 83 years (mean: 49.8, median: 48
years) showing 2 peaks (third/fourth and sixth/eighth decades). The
typical radiological appearance revealed multiple bilateral lung
nodules from 0.5 to 2 cm. In 2 cases, a striking perivascular and
intravascular growth pattern associated to pulmonary veins was
evident. All tumors were characterized by epithelioid tumor cells with
nests, cords and strands embedded in a sclerosing, hyaline matrix with
alveolar, bronchiolar and vascular extension. Necrosis was identified
in 8 tumors and ossification in 2 tumors. The mitotic index was below
one mitosis in 10 HPF. By immunohistochemistry, all tumors were
positive for CD 31, CD 34, and vimentin, 60% positive for factor VIII,
and negative for pancytokeratin and calretinin. Follow-up was
available for 8 patients with a range of 6 to 105 months (mean: 51.5
months, median: 52 months): 3 patients died (2 certainly from their
tumors), 4 are alive disease-free, and one is alive with metastases.
EHE of the lungs (IVBAT) represents an intermediate-grade, malignant,
mesenchymal neoplasm with a characteristic histological appearance and
immunohistochemical profile.
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