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Clinicopathological characteristics of pulmonary epithelioid hemangioendothelioma: A report of four cases and review of the literature.

Shao J, Zhang J - Oncol Lett (2014)

Bottom Line: Patient prognosis is unpredictable, with life expectancy ranging from 1 to 15 years due to the fact that estrogenic receptors behave inconsistently within the tumor and the occurence of the disease in male patients does not support the usual hormonal hypothesis.In the present study, the clinicopathological features of four cases of PEH were investigated and the associated literature was reviewed.The results of this study may improve understanding with regards to the diagnosis and therapeutic options for patients with PEH.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Shanghai Chest Hospital, Shanghai Jiao Tong University, Shanghai 200030, P.R. China.

ABSTRACT
This study aimed to investigate the clinicopathological characteristics, differential diagnosis and prognosis of pulmonary epithelioid hemangioendothelioma (PEH). PEH is a rare low-grade malignant vascular tumor. The cause of PEH remains unclear. Patient prognosis is unpredictable, with life expectancy ranging from 1 to 15 years due to the fact that estrogenic receptors behave inconsistently within the tumor and the occurence of the disease in male patients does not support the usual hormonal hypothesis. The clinical manifestations, imaging findings, histopathological characteristics and immunohistochemical phenotypes of four cases of epithelioid hemangioendothelioma occurring in the lung were retrospectively analyzed, and a review of the associated literature was conducted. The age of onset for the four PEH cases was 25-54 years, and the disease manifested as multiple nodules in the lungs or pleura. All of the patients underwent lobectomy or pulmonary wedge resection. The morphology of the tumor cells was epithelioid or spindle-shaped with abundant eosinophilic cytoplasm in which lumina or vacuoles containing erythrocytes were observed. The cells were arranged in nests and cords with degenerated interstitial mucoid. The morphology of the majority of the tumor cells was moderate, including mild atypia and little mitosis or necrosis. Immunohistochemical staining showed positive results for CD31, CD34 and F8. PEH is a rare low- to moderate-level tumor occurring in the lungs with differentiation toward vascular endothelial cells. Clinically, it is difficult to distinguish from a variety of other benign and malignant lung diseases. For diagnosis, a distinction must be made from other diseases such as chronic granulomatous disease, amyloid nodules, hamartoma, primary and metastatic lung cancers, malignant mesothelioma and vascular sarcoma. In the present study, the clinicopathological features of four cases of PEH were investigated and the associated literature was reviewed. The results of this study may improve understanding with regards to the diagnosis and therapeutic options for patients with PEH.

No MeSH data available.


Related in: MedlinePlus

Histological examination characteristics of pulmonary epithelioid hemangioendothelioma (hematoxylin and eosin staining) (A, case 1; B, case 2; C, case 3; D, case 4). (A) Neoplasms are composed of short cords and nested tumor cells, and interstitial mucus degeneration (magnification, ×200). (B) Typically, the lumen or cavity in the tumor cytoplasm contain single or multiple erythrocytes (magnification, ×200). (C) Tumor cells are rich in certain areas, with marked atypia. The tumor cells are arranged in solid nests and duct-like structures, and form papillary structures in the blood vessels (magnification, ×200). (D) Tumor cells show multiple small nodules and local cerebral calcification at the necrotic center (magnification, ×100).
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f2-ol-08-06-2517: Histological examination characteristics of pulmonary epithelioid hemangioendothelioma (hematoxylin and eosin staining) (A, case 1; B, case 2; C, case 3; D, case 4). (A) Neoplasms are composed of short cords and nested tumor cells, and interstitial mucus degeneration (magnification, ×200). (B) Typically, the lumen or cavity in the tumor cytoplasm contain single or multiple erythrocytes (magnification, ×200). (C) Tumor cells are rich in certain areas, with marked atypia. The tumor cells are arranged in solid nests and duct-like structures, and form papillary structures in the blood vessels (magnification, ×200). (D) Tumor cells show multiple small nodules and local cerebral calcification at the necrotic center (magnification, ×100).

Mentions: The tumors were composed of the tumor cells arranged in short cords and nests with degenerated stromal mucoid. The tumor cells were medium in size, polygonal or spindle shaped, with unclear cell boundaries. The cytoplasm was abundant and eosinophilic, and the nuclei were round, with small nucleoli showing mild or moderate atypia. The lumen or vacuolization containing one or more erythrocytes was commonly observed in the cytoplasm of the tumor cells (Fig. 2A and B). In case 4, the tumor cells were abundant in some areas, with obvious atypia; the tumor cells were arranged in solid nests and pseudoglandular structures, growing toward the surrounding alveoli and filling the alveolar space. The tumor cells in other areas formed papillary structures in the blood vessels (Fig. 2C). In case 3, the tumor cells were growing in multiple small nodules, with abundant cells surrounding the nodules and sclerosis at the nodular centers that was similar to hyaline degeneration or necrosis, and calcifications were observed in the necrotic center (Fig. 2D).


Clinicopathological characteristics of pulmonary epithelioid hemangioendothelioma: A report of four cases and review of the literature.

Shao J, Zhang J - Oncol Lett (2014)

Histological examination characteristics of pulmonary epithelioid hemangioendothelioma (hematoxylin and eosin staining) (A, case 1; B, case 2; C, case 3; D, case 4). (A) Neoplasms are composed of short cords and nested tumor cells, and interstitial mucus degeneration (magnification, ×200). (B) Typically, the lumen or cavity in the tumor cytoplasm contain single or multiple erythrocytes (magnification, ×200). (C) Tumor cells are rich in certain areas, with marked atypia. The tumor cells are arranged in solid nests and duct-like structures, and form papillary structures in the blood vessels (magnification, ×200). (D) Tumor cells show multiple small nodules and local cerebral calcification at the necrotic center (magnification, ×100).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4214485&req=5

f2-ol-08-06-2517: Histological examination characteristics of pulmonary epithelioid hemangioendothelioma (hematoxylin and eosin staining) (A, case 1; B, case 2; C, case 3; D, case 4). (A) Neoplasms are composed of short cords and nested tumor cells, and interstitial mucus degeneration (magnification, ×200). (B) Typically, the lumen or cavity in the tumor cytoplasm contain single or multiple erythrocytes (magnification, ×200). (C) Tumor cells are rich in certain areas, with marked atypia. The tumor cells are arranged in solid nests and duct-like structures, and form papillary structures in the blood vessels (magnification, ×200). (D) Tumor cells show multiple small nodules and local cerebral calcification at the necrotic center (magnification, ×100).
Mentions: The tumors were composed of the tumor cells arranged in short cords and nests with degenerated stromal mucoid. The tumor cells were medium in size, polygonal or spindle shaped, with unclear cell boundaries. The cytoplasm was abundant and eosinophilic, and the nuclei were round, with small nucleoli showing mild or moderate atypia. The lumen or vacuolization containing one or more erythrocytes was commonly observed in the cytoplasm of the tumor cells (Fig. 2A and B). In case 4, the tumor cells were abundant in some areas, with obvious atypia; the tumor cells were arranged in solid nests and pseudoglandular structures, growing toward the surrounding alveoli and filling the alveolar space. The tumor cells in other areas formed papillary structures in the blood vessels (Fig. 2C). In case 3, the tumor cells were growing in multiple small nodules, with abundant cells surrounding the nodules and sclerosis at the nodular centers that was similar to hyaline degeneration or necrosis, and calcifications were observed in the necrotic center (Fig. 2D).

Bottom Line: Patient prognosis is unpredictable, with life expectancy ranging from 1 to 15 years due to the fact that estrogenic receptors behave inconsistently within the tumor and the occurence of the disease in male patients does not support the usual hormonal hypothesis.In the present study, the clinicopathological features of four cases of PEH were investigated and the associated literature was reviewed.The results of this study may improve understanding with regards to the diagnosis and therapeutic options for patients with PEH.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Shanghai Chest Hospital, Shanghai Jiao Tong University, Shanghai 200030, P.R. China.

ABSTRACT
This study aimed to investigate the clinicopathological characteristics, differential diagnosis and prognosis of pulmonary epithelioid hemangioendothelioma (PEH). PEH is a rare low-grade malignant vascular tumor. The cause of PEH remains unclear. Patient prognosis is unpredictable, with life expectancy ranging from 1 to 15 years due to the fact that estrogenic receptors behave inconsistently within the tumor and the occurence of the disease in male patients does not support the usual hormonal hypothesis. The clinical manifestations, imaging findings, histopathological characteristics and immunohistochemical phenotypes of four cases of epithelioid hemangioendothelioma occurring in the lung were retrospectively analyzed, and a review of the associated literature was conducted. The age of onset for the four PEH cases was 25-54 years, and the disease manifested as multiple nodules in the lungs or pleura. All of the patients underwent lobectomy or pulmonary wedge resection. The morphology of the tumor cells was epithelioid or spindle-shaped with abundant eosinophilic cytoplasm in which lumina or vacuoles containing erythrocytes were observed. The cells were arranged in nests and cords with degenerated interstitial mucoid. The morphology of the majority of the tumor cells was moderate, including mild atypia and little mitosis or necrosis. Immunohistochemical staining showed positive results for CD31, CD34 and F8. PEH is a rare low- to moderate-level tumor occurring in the lungs with differentiation toward vascular endothelial cells. Clinically, it is difficult to distinguish from a variety of other benign and malignant lung diseases. For diagnosis, a distinction must be made from other diseases such as chronic granulomatous disease, amyloid nodules, hamartoma, primary and metastatic lung cancers, malignant mesothelioma and vascular sarcoma. In the present study, the clinicopathological features of four cases of PEH were investigated and the associated literature was reviewed. The results of this study may improve understanding with regards to the diagnosis and therapeutic options for patients with PEH.

No MeSH data available.


Related in: MedlinePlus