Primary malignant vascular tumors of the liver in children: Angiosarcoma and epithelioid hemangioendothelioma

AbstractHepatic angiosarcoma and epithelioid hemangioendothelioma (EHE) might be clinically considered a spectrum since, although more frequently presenting indolent behavior, EHE occasionally evolves to high-grade neoplasms. However, in most circumstances, pathological and immunohistochemical patterns define this differential diagnosis. More recently, molecular pathways for angiosarcoma and for EHE from other organs and from soft tissue have been proved different, paving the way for future morpho-molecular assessment of their hepatic counterpart. The frequency of liver involvement by Kaposi sarcoma in HIV-infected patients is lower nowadays. Histological findings and immunostaining for HHV-8 Ag are characteristic. Hepatic small vessel neoplasms have been recently recognized as important mimickers of angiosarcoma. The criteria for this differential diagnosis and the clinical behavior, up to now considered favorable, must be further studied.

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Comparison of the clinical and MRI features of patients with hepatic hemangioma, epithelioid hemangioendothelioma, or angiosarcoma

10.21203/rs.2.16278/v1 ◽

2019 ◽

Author(s):

Zhuangsheng Liu ◽

Lilei Yi ◽

Junhao Chen ◽

Ruqiong Li ◽

Keming Liang ◽

...

Keyword(s):

Vascular Endothelial Cell ◽

Measurement Data ◽

Epithelioid Hemangioendothelioma ◽

Vascular Tumors ◽

Hepatic Hemangioma ◽

Mri Findings ◽

Mri Features ◽

Rim Enhancement ◽

The Difference ◽

Magnetic Resonance Imaging Mri

Abstract Background Comparisons of hepatic epithelioid hemangioendothelioma (HEHE), hepatic hemangioma, and hepatic angiosarcoma (HAS) have rarely been reported. The purpose of our study was to analyze the clinical and magnetic resonance imaging (MRI) findings of these conditions.Methods A total of 57 patients (25 with hemangioma, 13 with HEHE, and 19 with HAS) provided hepatic vascular endothelial cell data between June 2006 and May 2017. The measurement data were represented as means±SDs. Between-group comparisons were performed using independent-samples t-tests. Count data were represented as frequencies or rates, and χ2- or Fisher’s exact tests were performed.Results The proportions of cases with circumscribed margins were 88% (22/25), 84.6% (11/3), and 31.6% (6/19) for hemangioma, HEHE, and HAS, respectively (P<0.001). HAS lesions were less likely to have circumscribed margins. The proportions of lesions with hemorrhaging were 4% (1/25), 44.4% (4/13), and 36.8% (7/13) for hemangioma, HEHE, and HAS, respectively (P=0.014). HEHE and HAS cases were more likely to show heterogeneous signals on T1-weighted (T1WI) MRI. HEHE and HAS cases were more likely to show heterogeneous signals on T2-weighted (T2WI) MRI. Centripetal enhancement was the most common pattern in vascular tumors, with proportions of 100%, 46.2% (6/13), and 68.4% (13/19) for hemangioma, HEHE, and HAS, respectively. The difference in enhancement pattern between HEHE and HAS was not significant, but rim enhancement was more common for HEHE (46.2%, 6/13).Conclusions Our study revealed clinical and imaging differences between HEHE and HAS. The platelet count (PLT) and coagulation function of the HAS group decreased, whereas the alpha-fetoprotein (AFP) level increased. The 5-year survival rate for HAS was significantly lower than that of HEHE. A higher malignancy degree indicated a more blurred lesion margin, easier occurrence of hemorrhaging, and more heterogeneous plain T1WI and T2WI signals.

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Pediatric Angiosarcoma of Soft Tissue: A Rare Clinicopathologic Entity

Archives of Pathology & Laboratory Medicine ◽

10.5858/134.3.481 ◽

2010 ◽

Vol 134 (3) ◽

pp. 481-485 ◽

Cited By ~ 5

Author(s):

Lobna Ayadi ◽

Abdelmajid Khabir

Keyword(s):

Spindle Cell ◽

Immunohistochemical Study ◽

Kaposi Sarcoma ◽

Neck Region ◽

Epithelioid Hemangioendothelioma ◽

Vascular Tumors ◽

Clinical Settings ◽

Vascular Differentiation ◽

Head And Neck Region ◽

Poorly Differentiated

Abstract Angiosarcomas are rare tumors that predominantly affect adults and elderly patients. Although angiosarcomas are well described in a variety of clinical settings, they have been poorly studied in children and little is known about their biology, natural history, or optimal treatment. Childhood angiosarcomas are exceedingly rare. The head and neck region and mediastinum seem to be the preferred locations. Most tumors are high-grade tumors. Vasoformative architecture is not always obvious on light microscopy requiring the benefit of immunohistochemical study. The differential diagnosis includes Kaposi sarcoma, epithelioid hemangioendothelioma, hemangiopericytoma, and spindle cell hemangioendothelioma whose prognosis is different. Complete resection is required for patients with localized tumors. Malignant vascular tumors are rare in children in the first 2 decades of life and when they do occur they seem to be more aggressive than in adults. Pathologic diagnosis is difficult particularly in poorly differentiated angiosarcomas requiring immunohistochemical study to confirm vascular differentiation.

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Pathologic Angiogenesis of Malignant Vascular Sarcomas: Implications for Treatment

Journal of Clinical Oncology ◽

10.1200/jco.2017.74.9812 ◽

2018 ◽

Vol 36 (2) ◽

pp. 194-201 ◽

Cited By ~ 8

Author(s):

Jalal A. Khan ◽

Robert G. Maki ◽

Vinod Ravi

Keyword(s):

Treatment Options ◽

Growth Factor Receptor ◽

Kaposi Sarcoma ◽

Clinical Trial Data ◽

Epithelioid Hemangioendothelioma ◽

Vascular Tumors ◽

Angiogenic Growth Factors ◽

Notch Pathways ◽

And Behavior ◽

Endothelial Growth Factor

Angiosarcoma, epithelioid hemangioendothelioma, and Kaposi sarcoma are classified according to the line of differentiation that these neoplastic cells most closely resemble: the endothelial cell. Although these malignant vascular sarcomas demonstrate immunohistochemical and ultrastructural features typical of this lineage, they vary dramatically in presentation and behavior, reflecting oncologic mechanisms unique to each. Antineoplastic therapies offer significant benefit, but because of the rarity of these cancers, novel therapies are slow to develop, and treatment options for these cancers remain limited. Antiangiogenic approaches that have shown benefit in other malignancies have not fully realized their promise in vascular tumors, suggesting that these tumors do not depend entirely on either angiogenic growth factors or on neighboring endothelia that are affected by these agents. Nonetheless, translational studies have begun to unravel these distinct pathologies, identifying novel translocation products, targets of oncogenic virulence factors, and genomic mutations that hijack angiogenic signaling and drive malignant growth. Concurrently, an elaborate and highly regulated model of angiogenesis and lymphangiogenesis involving vascular endothelial growth factor–receptor tyrosine kinase and TGF-β and Notch pathways has emerged that informs treatment of these tumors as well as cancer in general. This review summarizes the literature on malignant vascular sarcomas in the context of current models of angiogenesis and, in light of recent clinical trial data, could help clinician-scientists generate novel therapeutic approaches.

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Malignant Vascular Tumors of the Liver Presenting as Liver Failure and Portal Hypertension

Liver Transplantation and Surgery ◽

10.1002/lt.500010304 ◽

1995 ◽

Vol 1 (3) ◽

pp. 156-161 ◽

Cited By ~ 11

Author(s):

Sergio Rojter ◽

Federico G. Villamil ◽

Lidija M. Petrovic ◽

Jeffrey M. Silverman ◽

Graham M. Woolf ◽

...

Keyword(s):

Portal Hypertension ◽

Liver Failure ◽

Vascular Tumors ◽

Tumors Of The Liver

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Malignant Vascular Tumors of the Head and Neck—Which Type of Therapy Works Best?

Cancers ◽

10.3390/cancers13246201 ◽

2021 ◽

Vol 13 (24) ◽

pp. 6201

Author(s):

Susanne Wiegand ◽

Andreas Dietz ◽

Gunnar Wichmann

Keyword(s):

Head And Neck ◽

Treatment Options ◽

Age Distribution ◽

Case Series ◽

Epithelioid Hemangioendothelioma ◽

Current Data ◽

Vascular Tumors ◽

Retrospective Case ◽

Standard Medical Therapy ◽

New Treatment

Malignant vascular tumors of the head and neck are rare neoplasms with variable clinical presentation, wide age distribution, and variable clinical courses. The heterogeneous presentation of angiosarcomas and epithelioid hemangioendothelioma often leads to misdiagnosis and unsuitable treatment. While risk factors for angiosarcomas are previous radiation, chronic lymphedema, and exposure to arsenic, thorium oxide, or vinyl chloride, there are only limited and retrospective data available on prognostic factors in EHE. In both angiosarcomas and EHE, surgery is the mainstay of treatment. There is limited evidence regarding the role of radiotherapy in EHE, although EHE is considered relatively radiosensitive. In angiosarcomas, adjuvant radiotherapy is recommended according to retrospective case series. A standard medical therapy for metastasized malignant vascular tumors is lacking. Chemotherapy, which is effective in angiosarcoma, is mostly ineffective in EHE. Targeted therapy, antiangiogenetic drugs and immunotherapy have been studied as new treatment options. The goal of this review is to summarize the current data regarding malignant vascular tumors along with their diagnosis and management.

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Hepatic Epithelioid Hemangioendothelioma

Journal of radiology and nuclear medicine ◽

10.20862/0042-4676-2019-100-6-372-378 ◽

2020 ◽

Vol 100 (6) ◽

pp. 372-378

Author(s):

I. Z. Korobkova ◽

D. A. Dremin ◽

S. M. Kakalov ◽

I. V. Kirsan' ◽

I. S. Skrynnikov ◽

...

Keyword(s):

Imaging Techniques ◽

Integrated Approach ◽

Epithelioid Hemangioendothelioma ◽

Vascular Tumors

The paper gives a clinical example of diagnosing hepatic epithelioid hemangioendothelioma. With allowance made for that the tumor occurs rarely (less than 1% of cases of all vascular tumors), the authors provide the clinical example to demonstrate the capabilities of imaging techniques, by applying an integrated approach.

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Maxillary epithelioid hemangioendothelioma: an especially rare malignant tumor mimicking periodontal disease

BMC Oral Health ◽

10.1186/s12903-020-01291-4 ◽

2020 ◽

Vol 20 (1) ◽

Author(s):

Gintaras Januzis ◽

Dovydas Sakalys ◽

Martynas Mantas Krukis ◽

Dmitrij Seinin

Keyword(s):

Correct Diagnosis ◽

Vascular Tumor ◽

Epithelioid Hemangioendothelioma ◽

Vascular Tumors ◽

Low Grade ◽

X Rays ◽

Adequate Treatment ◽

Clinical Appearance ◽

Allogenic Bone ◽

Bony Destruction

Abstract Background Epithelioid hemangioendothelioma (EHE) is an especially rare, low-grade malignant vascular tumor that, according to WHO classification, is described as locally aggressive tumor with possible metastasis and makes up 1% of all vascular tumors. EHE is characterized by the accumulation of round, eosinophil-infiltrated endothelium cells; with vacuolation of their cytoplasm; frequent angiocentric inflammation; and myxohyaline stroma. This tumor is usually found in the liver, lungs, and bones and is especially rare in the mouth. Case presentation We present an 18-year-old Caucasian female whose oral cavity lesion had been misdiagnosed as marginal periodontitis. The patient was treated improperly for 2 years until she was referred to a maxillofacial surgeon. The patient complained only about gingival recession in the palatal area of her upper-right-side 13th, 14th, and 15th teeth. The lesion’s clinical appearance was of locally ulcerated painless lesion that affect the underlying bone as seen in X-rays in the palatal side of the right canine and the first and second premolars. Patient underwent surgery for her present defect and reconstruction using allogenic bone transplant. The diagnosis of EHE was based on the bony destruction as seen in x-rays and in the accumulation of tumor cells that were 100% positive to CD31; CD34 and ERG to endothelial markers. During the 31-month follow-up period, the patient exhibited no clinical and radiographic complications. Conclusions With this clinical case, we demonstrate that this rare tumor must be included in differential diagnoses of periodontal pathologies to perform histomorphological examination in a timely manner, which could lead to correct diagnosis and adequate treatment.

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Sarcoma: Vascular Tumors (EHE and Angiosarcoma)

10.2310/7800.16115 ◽

2017 ◽

Author(s):

Anne Grand'Maison ◽

Kilian Salerno ◽

John M Kane III

Keyword(s):

Radiation Therapy ◽

Adjuvant Chemotherapy ◽

Metastatic Disease ◽

Systemic Therapy ◽

Primary Tumor ◽

Epithelioid Hemangioendothelioma ◽

Wide Resection ◽

Vascular Tumors ◽

Adjuvant Radiation ◽

Chronic Lymphedema

Angiosarcoma (AS) and epithelioid hemangioendothelioma (EHE) are rare but clinically disparate vascular sarcomas. Common AS scenarios include the head and neck/scalp region in older patients or in association with chronic lymphedema or radiation therapy (often in the setting of breast cancer treatment). AS behaves like a high-grade tumor with a propensity for multifocality and hematogenous metastases. The potentially curative treatment is negative margin wide resection. Adjuvant radiation may be considered to does not reduce the risk of local recurrence. Unresectable localized tumor can be treated with definitive radiation therapy. Although data are limited, neoadjuvant and adjuvant chemotherapy are occasionally used. Extensive primary tumor or metastatic disease is treated with systemic therapy. EHE can arise in the liver, bone, lung, pleura, or skin/soft tissue and can also metastasize hematogenously. The tumor is frequently asymptomatic and can be clinically indolent. Therefore, specific therapy is reserved for documented progression. Limited primary tumor is treated with surgical resection and occasionally adjuvant radiation. There is no proven benefit to adjuvant chemotherapy. Liver-directed therapy and occasionally transplantation are used for unresectable hepatic disease. Metastatic disease is treated with systemic therapy, but only in the setting of clinical progression. Key words: angiosarcoma, breast, cutaneous, epithelioid hemangioendothelioma, lymphedema, neoadjuvant chemotherapy, radiation associated, visceral

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Benign Vascular Bone Tumors

Musculoskeletal Imaging Volume 1 ◽

10.1093/med/9780190938161.003.0060 ◽

2019 ◽

pp. 303-307

Author(s):

G. Scott Stacy

Keyword(s):

Vascular Malformation ◽

Bone Tumors ◽

Treatment Strategies ◽

Epithelioid Hemangioendothelioma ◽

Vascular Tumors ◽

Slow Flow ◽

Imaging Features ◽

Epithelioid Hemangioma ◽

Vascular Bone Tumors

The classification of vascular tumors of the skeleton can be thought of as a spectrum of disease including both benign and malignant entities. Chapter 60 focuses on the benign entities, hemangioma and epithelioid hemangioma. The term hemangioma refers to a collection of blood vessels and vascular spaces, perhaps better classified as a slow-flow vascular malformation. Epithelioid hemangioma is a rare and controversial entity that may be confused with its malignant counterpart, the epithelioid hemangioendothelioma. This chapter will describe the clinical, pathophysiological, and imaging features of these benign vascular bone tumors, with a brief mention of treatment strategies. Familiarity with the imaging appearances and characteristics of these entities is essential in order to guide clinical management and to avoid unnecessary investigation into clearly benign lesions.

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