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Imaging diagnosis of epithelioid hemangioendothelioma in thoracic vertebrae and liver
Journal Pre-proof Imaging diagnosis of epithelioid hemangioendothelioma in thoracic vertebrae and liver Yuping Zeng, MM, Xiaoming Leng, MD, Ping Chen, MB, Jiandong Luo, MD, Zhiyang Zhou, MD PhD PII:
S0003-4975(19)31822-3
DOI:
https://doi.org/10.1016/j.athoracsur.2019.10.034
Reference:
ATS 33274
To appear in:
The Annals of Thoracic Surgery
Received Date: 27 August 2019 Revised Date:
9 October 2019
Accepted Date: 11 October 2019
Please cite this article as: Zeng Y, Leng X, Chen P, Luo J, Zhou Z, Imaging diagnosis of epithelioid hemangioendothelioma in thoracic vertebrae and liver, The Annals of Thoracic Surgery (2019), doi: https://doi.org/10.1016/j.athoracsur.2019.10.034. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 by The Society of Thoracic Surgeons
Imaging diagnosis of epithelioid hemangioendothelioma in thoracic vertebrae and liver Running Head: Thoracic EHE diagnosed by PET/MRI
Yuping Zeng MM, Xiaoming Leng MD, Ping Chen MB, Jiandong Luo MD, Zhiyang Zhou MD PhD*
Department of Radiology, Guangzhou Universal Medical Imaging Diagnostic Center, Guangzhou, 510080, China
*Address for correspondence: Zhiyang Zhou (Email: [email protected]) Department of Radiology, Guangzhou Universal Medical Imaging Diagnostic Center, 80 Zhongshan 2nd Road, Guangzhou, China, 510080.
Abstract
Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor of uncertain biologic behavior. Most cases come out as a single lesion of the soft tissue but also may appear in the lung, liver, and other locations. EHE in bone, especially in thoracic vertebrae, is a extremely rare occurrence and signifies a challenge for the imaging diagnosis. This article presents a rare case of EHE occurring in thoracic vertebrae and liver revealed by fluoride-18-fluorodeoxyglucose-positron emission tomography and magnetic resonance imaging (18F-FDG-PET/MRI) to provide a better understanding of its clinical application and further insight into diagnosing a rare thoracic tumor.
Epithelioid hemangioendothelioma (EHE) is a rare vascular malignancy with intermediate behavior between that of benign hemangioma and malignant angiosarcoma[1]. Most cases develop as lung and liver nodules[2]. EHE of bone is an extremely rare tumor , it represents only 0.5% to 1.0% of bony malignant tumors[3], with a thoracic vertebrae location occurring in even much lower percentage of these. Because of its low prevalence, variable presentation, and unknown latency period, it can be easily missed at the imaging diagnosis. Recently, 18
F-FDG -PET/MRI technology has emerged as a promising tool for the diagnosis and
evaluation of primary tumor. However, the imaging diagnosis of thoracic EHE using 18F-FDG PET/MRI has been hardly reported. In this article, we present the 18F-FDG PET/MRI findings of a rare case of EHE occurring in thoracic vertebrae and liver at diagnosis.
A 42 year old man was admitted to the emergency department with a complaint of severe thorax, abdomen and low back pain for 3 months. He reported no other symptoms, including jaundice, abdominal distention or fatigue, and has no past medical history of cirrhosis, hepatitis B and diabetes. Abdominal computed tomography (CT) revealed findings suspicious hypodense lesion in the right lobe of the liver. Subsequently, he was referred to 18
F-FDG-PET/MRI examination to well depicte the internal architecture of the hypodense
lesion and detect other suspicious lesions. 18F-FDG-PET/MRI showed a hypermetabolic lytic lesion with a maximal-standardized uptake value of 7.9 in the T5 vertebra (Fig 1A, Fig 1B, Fig 1D). The lesion showed an intermediate T1-weighted imaging (Fig 1F), a high T2-weighted imaging (Fig 1E) and a high diffusion weighted imaging (Fig 1C) signal intensity. The surrounding soft tissue mass protruding into the spinal canal and compressing
the corresponding spinal cord. A mild to moderate hypermetabolic nodule with a maximal-standardized uptake value of 3.35 was found in S8 liver segment (Fig 2A, Fig 2B, Fig 2D), it showed an intermediate T1-weighted imaging (Fig 2F), a high-hybrid T2-weighted imaging (Fig 2E) and a high diffusion weighted imaging (Fig 2C) signal intensity, a halo sign around the lesion was presented after enhancement, with visible enhancement at the inner edge, and gradually toward the center (Fig 2G). Taken together, these results suggested that a low grade malignancy was considered, the likelihood of EHE was the highest. For further pathology diagnosis, the biopsy from paravertebral tissue and hepatic nodule were obtained, and it showed that both of they were formed by neoplastic tissue displaying similar histologic characteristics (Fig 3A, Fig 3C). The neoplastic tissue was formed by epithelioid cells arranged in glands, cords and nests surrounded by abundant myxoid stroma with hyaline areas (Fig 3B). The tumor cells were large, polygonal or round, with well-defined borders and ample eosinophylic cytoplasm, and with intracellular lumina containing erythrocytes in some cells. The nuclei were central with partly offset, ovoid, and with little changes in size and shape (Fig 3D). Immunohistochemistry showed that the neoplastic cells of paravertebral tissue and hepatic nodule were positive for CD34, CD31, and cytokeratin (CK), and negative for epithelial membrane antigen (EMA). The pathologic diagnosis was “EHE occurring in thoracic vertebrae and liver ”.
Comment EHE, was described in 1982 by Weiss and Enzinger[4], is a rare tumor of vascular origin and uncertain malignant potential with an incidence less than 1 in 1000000[5]. Most cases
reported occurs in soft tissues, lungs and liver, and can be also transferred to other organs. EHE of bone is rare, it represents 0.5% to 1.0% of the bony malignant tumors[3], with a thoracic vertebrae location occurring in even much lower percentage of these. Our present case of EHE with occurrences in thoracic vertebrae and liver is hardly reported.
The pathological characteristics described in EHE, such as polygonal cell, arrangment in trabeculae and cords surrounded by myxoid stroma, and ample eosinophylic cytoplasm with intracellular lumina containing erythrocytes[6], could be seen typically in our case, and the results of immunohistochemistry that the neoplastic cells of paravertebral tissue and hepatic nodule were positive for CD34, CD31[7] further supported the diagnosis of EHE.
Since thoracic EHE lacks characteristic clinical symptoms, imaging diagnosis is an important method for the diagnosis. Because of its low prevalence, variable presentation, and unknown latency period, it can be easily missed at the imaging diagnosis. With the development of imaging diagnostic technology, 18F-FDG-PET has been widely developed, and due to the fact that 18F-FDG-PET scans the whole body with high sensitivity, it is useful for the searching of the tumors. Recently, 18F-FDG-PET combined with MRI has emerged as a cutting-edge and promising tool for the imaging diagnosis of tumor, thanks to its high resolution of soft tissue, multi-parameter, multi-sequence, and whole body scanning. In our case, during the 18F-FDG-PET/MRI scanning, a hypermetabolic lesion in T5 vertebrae and a mild to moderate hypermetabolic nodule in liver could be clearly seen with their increasing FDG uptake. The internal architecture of hepatic nodule is well depicted on MRI, and the mild peripheral rim enhancement, which could be one of the characteristic signs
of hepatic EHE[8], were clearly presented. On MRI, the osseous lesions of EHE usually demonstrate nonspecific low to intermediate T1 and high T2 signal intensity with homogenous enhancement, and they are commonly lytic and often restricted to the axial skeleton with the bony cortex destruction surrounding the soft tissue mass [8]. These signs were evident in our case. Previously, there are only few case reports showing the usefulness of 18F-FDG-PET/CT in EHE diagnosis. This is the first case that 18F-FDG-PET/MRI depicted the EHE occurring in thoracic vertebrae and liver at diagnosis in the literature. It demonstrated that 18
F-FDG-PET/MRI can be very useful in searching and depicting tumor lesions especially in
the EHE with soft tissue involved and multiple lesions as in our case.
References [1] A. Sardaro, L. Bardoscia, M. F. Petruzzelli, M. Portaluri. Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor, Oncol Rev, 8 (2014), pp. 259. [2] L. Yu, T. Gu, Z. Xiu, E. Shi, X. Zhao. Primary pleural epithelioid hemangioendothelioma compressing the myocardium, J Card Surg, 28 (2013), pp. 266-268. [3] C. Fletcher, K. Unni, F. Mertens. World Health Organization classification of tumours. Pathology & Genetics. Tumours of Soft Tissue and Bone, IARC Press, Lyon (2002). [4] S.W. Weiss, F. M. Enzinger. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer, 50 (1982), pp. 970-981. [5] B. E. Balansay, X. Zhang, P. D. Loftus, et al. Diagnosing Epithelioid Hemangioendothelioma With Pericardial Involvement, Ann Thorac Surg, 106 (2018), pp.173-175. [6] L. I. Gómez-Arellano, T. Ferrari-Carballo, H. R. Domínguez-Malagón. Multicentric epithelioid hemangioendothelioma of bone. Report of a case with imaging-pathologic correlation. Annals of Diagnostic Pathology, 16 (2012), pp. 43-47. [7] U. Flucke, R. J. C. Vogels, N. de Saint Aubain Somerhausen, et al. Epithelioid hemangioendothelioma: clinicopathologic, immunohistochemical, and molecular genetic analysis of 39 cases. Diagn Pathol, 9 (2014), pp. 131. [8] Y. Epelboym, D. R. Engelkemier, F. Thomas-Chausse, et al. Imaging findings in epithelioid hemangioendothelioma. Clinical Imaging, 58 (2019), pp. 59-65.
Figure Legends Figure. 1 18F-FDG PET/MRI showed a hypermetabolic lytic lesion with a maximal-standardized uptake value of 7.9 in the T5 vertebra (arrow in A maximum intensity projection image, in B fusion axial PET/MRI images and in D metabolism images).The lesion showed an intermediate T1-weighted imaging (F), a high T2-weighted imaging (E) and a high diffusion weighted imaging (C) signal intensity. The surrounding soft tissue mass protruding into the spinal canal and compressing the corresponding spinal cord.
Figure. 2 18F-FDG PET/MRI showed a mild to moderate hypermetabolic nodule with a maximal-standardized uptake value of 3.35 in S8 liver segment (arrow in A maximum intensity projection image, in B fusion axial PET/MRI images and in D metabolism images). The nodule showed an intermediate T1-weighted imaging (F), a high-hybrid T2-weighted imaging (E) and a high diffusion weighted imaging (C) signal intensity. Dynamic contrast-enhanced MRI (G1~G4) scan showed a halo sign around the lesion with visible enhancement at the inner edge, and gradually toward the center.
Figure. 3 Vertebral biopsy showed neoplastic tissue was formed by epithelioid cells arranged in glands, cords and nests surrounded by abundant myxoid stroma with hyaline areas (A, B). Hepatic nodule biopsy showed similar histologic characteristics (C). The tumor cells were large, polygonal or round, with well-defined borders and ample eosinophylic cytoplasm, and with intracellular lumina containing erythrocytes in some cells. The nuclei were central with partly offset, ovoid, and with little changes in size and shape (D).
S0003-4975(19)31822-3
DOI:
https://doi.org/10.1016/j.athoracsur.2019.10.034
Reference:
ATS 33274
To appear in:
The Annals of Thoracic Surgery
Received Date: 27 August 2019 Revised Date:
9 October 2019
Accepted Date: 11 October 2019
Please cite this article as: Zeng Y, Leng X, Chen P, Luo J, Zhou Z, Imaging diagnosis of epithelioid hemangioendothelioma in thoracic vertebrae and liver, The Annals of Thoracic Surgery (2019), doi: https://doi.org/10.1016/j.athoracsur.2019.10.034. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 by The Society of Thoracic Surgeons
Imaging diagnosis of epithelioid hemangioendothelioma in thoracic vertebrae and liver Running Head: Thoracic EHE diagnosed by PET/MRI
Yuping Zeng MM, Xiaoming Leng MD, Ping Chen MB, Jiandong Luo MD, Zhiyang Zhou MD PhD*
Department of Radiology, Guangzhou Universal Medical Imaging Diagnostic Center, Guangzhou, 510080, China
*Address for correspondence: Zhiyang Zhou (Email: [email protected]) Department of Radiology, Guangzhou Universal Medical Imaging Diagnostic Center, 80 Zhongshan 2nd Road, Guangzhou, China, 510080.
Abstract
Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor of uncertain biologic behavior. Most cases come out as a single lesion of the soft tissue but also may appear in the lung, liver, and other locations. EHE in bone, especially in thoracic vertebrae, is a extremely rare occurrence and signifies a challenge for the imaging diagnosis. This article presents a rare case of EHE occurring in thoracic vertebrae and liver revealed by fluoride-18-fluorodeoxyglucose-positron emission tomography and magnetic resonance imaging (18F-FDG-PET/MRI) to provide a better understanding of its clinical application and further insight into diagnosing a rare thoracic tumor.
Epithelioid hemangioendothelioma (EHE) is a rare vascular malignancy with intermediate behavior between that of benign hemangioma and malignant angiosarcoma[1]. Most cases develop as lung and liver nodules[2]. EHE of bone is an extremely rare tumor , it represents only 0.5% to 1.0% of bony malignant tumors[3], with a thoracic vertebrae location occurring in even much lower percentage of these. Because of its low prevalence, variable presentation, and unknown latency period, it can be easily missed at the imaging diagnosis. Recently, 18
F-FDG -PET/MRI technology has emerged as a promising tool for the diagnosis and
evaluation of primary tumor. However, the imaging diagnosis of thoracic EHE using 18F-FDG PET/MRI has been hardly reported. In this article, we present the 18F-FDG PET/MRI findings of a rare case of EHE occurring in thoracic vertebrae and liver at diagnosis.
A 42 year old man was admitted to the emergency department with a complaint of severe thorax, abdomen and low back pain for 3 months. He reported no other symptoms, including jaundice, abdominal distention or fatigue, and has no past medical history of cirrhosis, hepatitis B and diabetes. Abdominal computed tomography (CT) revealed findings suspicious hypodense lesion in the right lobe of the liver. Subsequently, he was referred to 18
F-FDG-PET/MRI examination to well depicte the internal architecture of the hypodense
lesion and detect other suspicious lesions. 18F-FDG-PET/MRI showed a hypermetabolic lytic lesion with a maximal-standardized uptake value of 7.9 in the T5 vertebra (Fig 1A, Fig 1B, Fig 1D). The lesion showed an intermediate T1-weighted imaging (Fig 1F), a high T2-weighted imaging (Fig 1E) and a high diffusion weighted imaging (Fig 1C) signal intensity. The surrounding soft tissue mass protruding into the spinal canal and compressing
the corresponding spinal cord. A mild to moderate hypermetabolic nodule with a maximal-standardized uptake value of 3.35 was found in S8 liver segment (Fig 2A, Fig 2B, Fig 2D), it showed an intermediate T1-weighted imaging (Fig 2F), a high-hybrid T2-weighted imaging (Fig 2E) and a high diffusion weighted imaging (Fig 2C) signal intensity, a halo sign around the lesion was presented after enhancement, with visible enhancement at the inner edge, and gradually toward the center (Fig 2G). Taken together, these results suggested that a low grade malignancy was considered, the likelihood of EHE was the highest. For further pathology diagnosis, the biopsy from paravertebral tissue and hepatic nodule were obtained, and it showed that both of they were formed by neoplastic tissue displaying similar histologic characteristics (Fig 3A, Fig 3C). The neoplastic tissue was formed by epithelioid cells arranged in glands, cords and nests surrounded by abundant myxoid stroma with hyaline areas (Fig 3B). The tumor cells were large, polygonal or round, with well-defined borders and ample eosinophylic cytoplasm, and with intracellular lumina containing erythrocytes in some cells. The nuclei were central with partly offset, ovoid, and with little changes in size and shape (Fig 3D). Immunohistochemistry showed that the neoplastic cells of paravertebral tissue and hepatic nodule were positive for CD34, CD31, and cytokeratin (CK), and negative for epithelial membrane antigen (EMA). The pathologic diagnosis was “EHE occurring in thoracic vertebrae and liver ”.
Comment EHE, was described in 1982 by Weiss and Enzinger[4], is a rare tumor of vascular origin and uncertain malignant potential with an incidence less than 1 in 1000000[5]. Most cases
reported occurs in soft tissues, lungs and liver, and can be also transferred to other organs. EHE of bone is rare, it represents 0.5% to 1.0% of the bony malignant tumors[3], with a thoracic vertebrae location occurring in even much lower percentage of these. Our present case of EHE with occurrences in thoracic vertebrae and liver is hardly reported.
The pathological characteristics described in EHE, such as polygonal cell, arrangment in trabeculae and cords surrounded by myxoid stroma, and ample eosinophylic cytoplasm with intracellular lumina containing erythrocytes[6], could be seen typically in our case, and the results of immunohistochemistry that the neoplastic cells of paravertebral tissue and hepatic nodule were positive for CD34, CD31[7] further supported the diagnosis of EHE.
Since thoracic EHE lacks characteristic clinical symptoms, imaging diagnosis is an important method for the diagnosis. Because of its low prevalence, variable presentation, and unknown latency period, it can be easily missed at the imaging diagnosis. With the development of imaging diagnostic technology, 18F-FDG-PET has been widely developed, and due to the fact that 18F-FDG-PET scans the whole body with high sensitivity, it is useful for the searching of the tumors. Recently, 18F-FDG-PET combined with MRI has emerged as a cutting-edge and promising tool for the imaging diagnosis of tumor, thanks to its high resolution of soft tissue, multi-parameter, multi-sequence, and whole body scanning. In our case, during the 18F-FDG-PET/MRI scanning, a hypermetabolic lesion in T5 vertebrae and a mild to moderate hypermetabolic nodule in liver could be clearly seen with their increasing FDG uptake. The internal architecture of hepatic nodule is well depicted on MRI, and the mild peripheral rim enhancement, which could be one of the characteristic signs
of hepatic EHE[8], were clearly presented. On MRI, the osseous lesions of EHE usually demonstrate nonspecific low to intermediate T1 and high T2 signal intensity with homogenous enhancement, and they are commonly lytic and often restricted to the axial skeleton with the bony cortex destruction surrounding the soft tissue mass [8]. These signs were evident in our case. Previously, there are only few case reports showing the usefulness of 18F-FDG-PET/CT in EHE diagnosis. This is the first case that 18F-FDG-PET/MRI depicted the EHE occurring in thoracic vertebrae and liver at diagnosis in the literature. It demonstrated that 18
F-FDG-PET/MRI can be very useful in searching and depicting tumor lesions especially in
the EHE with soft tissue involved and multiple lesions as in our case.
References [1] A. Sardaro, L. Bardoscia, M. F. Petruzzelli, M. Portaluri. Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor, Oncol Rev, 8 (2014), pp. 259. [2] L. Yu, T. Gu, Z. Xiu, E. Shi, X. Zhao. Primary pleural epithelioid hemangioendothelioma compressing the myocardium, J Card Surg, 28 (2013), pp. 266-268. [3] C. Fletcher, K. Unni, F. Mertens. World Health Organization classification of tumours. Pathology & Genetics. Tumours of Soft Tissue and Bone, IARC Press, Lyon (2002). [4] S.W. Weiss, F. M. Enzinger. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer, 50 (1982), pp. 970-981. [5] B. E. Balansay, X. Zhang, P. D. Loftus, et al. Diagnosing Epithelioid Hemangioendothelioma With Pericardial Involvement, Ann Thorac Surg, 106 (2018), pp.173-175. [6] L. I. Gómez-Arellano, T. Ferrari-Carballo, H. R. Domínguez-Malagón. Multicentric epithelioid hemangioendothelioma of bone. Report of a case with imaging-pathologic correlation. Annals of Diagnostic Pathology, 16 (2012), pp. 43-47. [7] U. Flucke, R. J. C. Vogels, N. de Saint Aubain Somerhausen, et al. Epithelioid hemangioendothelioma: clinicopathologic, immunohistochemical, and molecular genetic analysis of 39 cases. Diagn Pathol, 9 (2014), pp. 131. [8] Y. Epelboym, D. R. Engelkemier, F. Thomas-Chausse, et al. Imaging findings in epithelioid hemangioendothelioma. Clinical Imaging, 58 (2019), pp. 59-65.
Figure Legends Figure. 1 18F-FDG PET/MRI showed a hypermetabolic lytic lesion with a maximal-standardized uptake value of 7.9 in the T5 vertebra (arrow in A maximum intensity projection image, in B fusion axial PET/MRI images and in D metabolism images).The lesion showed an intermediate T1-weighted imaging (F), a high T2-weighted imaging (E) and a high diffusion weighted imaging (C) signal intensity. The surrounding soft tissue mass protruding into the spinal canal and compressing the corresponding spinal cord.
Figure. 2 18F-FDG PET/MRI showed a mild to moderate hypermetabolic nodule with a maximal-standardized uptake value of 3.35 in S8 liver segment (arrow in A maximum intensity projection image, in B fusion axial PET/MRI images and in D metabolism images). The nodule showed an intermediate T1-weighted imaging (F), a high-hybrid T2-weighted imaging (E) and a high diffusion weighted imaging (C) signal intensity. Dynamic contrast-enhanced MRI (G1~G4) scan showed a halo sign around the lesion with visible enhancement at the inner edge, and gradually toward the center.
Figure. 3 Vertebral biopsy showed neoplastic tissue was formed by epithelioid cells arranged in glands, cords and nests surrounded by abundant myxoid stroma with hyaline areas (A, B). Hepatic nodule biopsy showed similar histologic characteristics (C). The tumor cells were large, polygonal or round, with well-defined borders and ample eosinophylic cytoplasm, and with intracellular lumina containing erythrocytes in some cells. The nuclei were central with partly offset, ovoid, and with little changes in size and shape (D).