[MRI manifestations of 40 cases with the hepatic epithelioid hemangioendothelioma classification based on the morphology and size].

Objective: To explore the MRI characteristics of the hepatic epithelioid hemangioendothelioma (HEHE) classification according to morphology and size. Methods: The clinical, pathological, and MRI imaging data of 40 cases with HEHE confirmed pathologically from December 2009 to September 2021 were retrospectively analyzed. A paired sample t-test was used for comparison between the two groups. Results: There were 40 cases (5 solitary, 24 multifocal, 9 local fusion, and 2 diffuse fusion) and 214 lesions (163 nodules, 31 masses, and 20 fusion foci). The most common features of lesions were subcapsular growth and capsular depression. The signal intensity of lesions ≤1cm was usually uniform with whole or ring enhancement. Nodules and mass-like lesions ≥1cm on a T1-weighted image had slightly reduced signal intensity or manifested as a halo sign. Target signs on a T2-weighted image were characterized by: target or centripetal enhancement; fusion-type lesions; irregular growth and hepatic capsular retraction, with ring or target-like enhancement in the early stage of fusion and patchy irregular enhancement in the late stage; blood vessels traversing or accompanied by malformed blood vessels; focal bleeding; an increasing proportion of extrahepatic metastases and abnormal liver function with the type of classified manifestation; primarily portal vein branches traversing; and reduced overall intralesional bleeding rate (17%). Lollipop signs were presented in 19 cases, with a high expression rate in mass-type lesions (42%). The fusion lesions were expressed, but the morphological manifestation was atypical. The diffusion-weighted imaging mostly showed high signal or target-like high signal. An average apparent diffusion coefficient of lesions was (1.56±0.36) ×10(-3)mm(2)/s, which was statistically significantly different compared with that of adjacent normal liver parenchyma (t=8.28, P<0.001). Conclusion: The MRI manifestations for the HEHE classification are closely related to the morphology and size of the lesions and have certain differences and characteristics that are helpful for the diagnosis of the disease when combined with clinical and laboratory examinations.

Vascular Tumors of Bone: The Evolvement of a Classification Based on Molecular Developments.

The classification of vascular tumors of bone has been under debate over time. Vascular tumors in bone are rare, display highly overlapping morphology, and, therefore, are considered difficult by pathologists. Compared with their soft tissue counterparts, they are more often multifocal and sometimes behave more aggressively. Over the past decade, with the advent of next-generation sequencing, recurrent molecular alterations have been found in some of the entities. The integration of morphology and molecular changes has led to a better characterization of these separate entities.

Cytopathologic characteristics of epithelioid vascular malignancies.

OBJECTIVES: Epithelioid hemangioendothelioma (EHE) and epithelioid angiosarcoma (EAS) are rare vascular neoplasms that share many morphological characteristics on histology but demonstrate different clinical behavior. Given the many reported clinical and morphological features shared between EAS and EHE, we examined all cases of EAS and EHE diagnosed primarily on fine needle aspiration (FNA) at our three institutions that were confirmed by a tissue diagnosis. STUDY DESIGN: A total of 29 cases from 25 patients were identified: 15 EHE from 11 patients and 14 EAS from 14 patients. RESULTS: Many cytomorphological features existed on a spectrum that overlapped considerably between EAS and EHE cases. Common features between the two entities include epithelioid morphology and eccentrically placed nucleus. Intracytoplasmic lumens (ICL), a morphological feature that may suggest vascular origin, can be found in both entities but are not always present. CONCLUSIONS: Given the general absence of vascular cytomorphological features, such as ICL, the proper classification of these tumors depends on the successful use of immunoperoxidase markers such as factor VIII-related antigen, ERG, or CD31 as well as a high index of suspicion. The distinction between EAS and EHE on FNA alone is treacherous at best.

Primary vascular tumors of bone: a spectrum of entities?

Vascular tumors of bone are a heterogeneous group. Numerous terms have been introduced as well as different classification systems. None of the classification schemes have been accepted due to lack of consistent terminology, accepted histologic criteria, and limited correlation with clinical outcome. It is acknowledged that vascular tumors of bone originate from endothelial cells, resulting in variable expression of endothelial markers. None of these markers are useful to discriminate between benign and malignant lesions. Although radiologic appearance is not specific, radiologic multifocality should trigger to include a vascular neoplasm in the differential diagnosis. This review gives an overview of current literature by describing all different histologic subtypes in correspondence with clinical, radiologic and genetic data. We propose the classification of vascular tumors of bone according to the three-tiered World Health Organization classification scheme for soft tissue tumors dividing entities into a benign, intermediate and malignant category. Hemangioma is the most often and commonly recognized benign lesion. Epithelioid hemangioma has been better defined over the past few years. Based on its locally aggressive behavior and occurrence of lymph node metastases, classification within the intermediate category could be considered. Angiosarcoma is the only accepted term for high-grade malignant vascular tumor of bone and so far, epithelioid hemangioendothelioma is the only accepted low-grade malignant vascular tumor of bone. It is still unclear whether other low-grade malignant vascular tumors of bone (e.g. hemangioendothelioma) truly exist. Unfortunately, molecular / genetic studies of vascular tumors of bone which might support the proposed classification are very sparse.

Vascular tumors of bone: A study of 17 cases other than ordinary hemangioma, with an evaluation of the relationship of hemangioendothelioma of bone to epithelioid hemangioma, epithelioid hemangioendothelioma, and high-grade angiosarcoma.

Cases filed as vascular tumor of bone other than ordinary hemangioma were reviewed. They were included in the study if there was adequate histologic material and clinical information, clear evidence of bone origin, and at least 5 years follow-up. The study group comprised 17 cases, of which 13 were categorized as hemangioendothelioma of bone, 1 as epithelioid hemangioendothelioma, and 3 as high-grade angiosarcoma. Hemangioendothelioma of bone had growth patterns varying from vasoformative to solid, but well-formed vessels were present in at least some area in all cases. The cells generally had a rounded, epithelioid character, regular nuclei, and relatively few mitotic figures; occasional features included spindle cells and scattered enlarged, hyperchromatic or pleomorphic nuclei. Lymphoplasmacytic and eosinophilic inflammatory infiltrate ranged from prominent to slight or absent, and myxoid or hyaline stroma was never more than focal. Epithelioid hemangioma could not be separated from hemangioendothelioma of bone. The single epithelioid hemangioendothelioma for the most part had cords of relatively uniform epithelioid cells in a prominent myxoid stroma but focally demonstrated an angiosarcoma-like appearance, with irregular vascular spaces and marked nuclear pleomorphism. The high-grade angiosarcomas exhibited predominantly irregular vasoformation combined with solid areas, diffuse nuclear hyperchromatism and pleomorphism, and, in 2 cases, numerous mitotic figures (the third case had only a small biopsy and a postradiation amputation specimen). Of the hemangioendotheliomas of bone, 7 were unicentric and 6 were regionally multicentric either concurrently or sequentially. Three patients had intraosseous local recurrence, 2 had discontinuous regional skin or soft tissue involvement (including the popliteal artery in 1), and 1 had a solitary lung metastasis, but none died of tumor. The patient with epithelioid hemangioendothelioma had multicentric tumors in widely separated bones and died with liver and lung metastases. Two of the high-grade angiosarcomas were unicentric, and the third was regionally multicentric, with a popliteal artery-soft tissue component as well. All 3 of these patients died with metastases in various sites.

Epithelioid hemangioendothelioma of the stomach: clinical-pathological features, nosologic setting, and surgical therapy. Report of a case.

Vascular tumors of the stomach represent 0.9%-3.3% of all gastric neoplasms. A 41-year-old man was admitted to our department with a 3-month history of early postprandial epigastric pain, sluggish digestion, nausea, asthenia, and occasional alimentary emesis. Preoperative staging detected a submucosal neoformation in the prepyloric zone, which narrowed the lumen, without any infiltrative features; a wedge gastric resection was performed and the definitive diagnosis was an epithelioid hemangioendothelioma of stomach. An 8-month follow up did not show any relapse of the disease. The term hemangioendothelioma is controversial because of disagreements regarding the nosologic setting and treatment. As a result, the latest WHO classification calls such neoplasms "borderline." The correct diagnosis depends on the histological findings supported by immunohistochemistry. Surgery represents the treatment of choice; however, a conservative approach is preferred whenever possible. However, due to the borderline biological behavior of this neoplasm, it is important that detailed clinical evaluations be carried out for such patients along with a thorough follow-up.

Epithelioid vascular tumors of bone: a review and proposal of a classification scheme.

Skeletal vascular tumors composed of epithelioid endothelial cells commonly result in diagnostic difficulty. Although tumors with this morphology have been recognized for many years, there is a considerable degree of confusion regarding their nomenclature and classification. In this article what is believed to represent the morphologic features of this family of tumors is outlined, the historical context of epithelioid endothelial tumors is briefly discussed, and pertinent literature and texts pertaining to the subject is reviewed. It is proposed that the osseous epithelioid endothelial tumors should be classified in a similar manner to their soft tissue counterparts and it is suggested that this approach should help to clarify the confusion surrounding this subject of surgical pathology.

[Eruptive epithelioid hemangioendothelioma with spindle cells. Nosological place in the spectrum of epithelioid vascular tumors]. / L'hémangioendothéliome épithélioïde éruptif à cellules fusiformes. Place nosologique dans le spectre des tumeurs vasculaires épithélioïdes.

The epithelioid vascular tumors include epithelioid hemangioma, epithelioid hemangioendothelioma and epithelioid angiosarcoma. We report the case of a difficult to define vascular epithelioid tumor. The tumor localized in the head developed in an eruptive way in multiple bone and skin locations in a 22-year-old man. The lesions had the same histological features as those of epithelioid hemangioma and epithelioid hemangioendothelioma with a spindle-cell component. This tumor was called eruptive epithelioid hemangioendothelioma with spindle cells. In our case, its development with recurrences and destructive features has some similarities to tumors of intermediate malignancy.

[Hemangioendotheliomas--evolution of a concept of a heterogeneous group of vascular neoplasms]. / Hämangioendotheliome--Entwicklung eines Konzeptes einer heterogenen Gruppe vaskulärer Tumoren.

The term haemangioendothelioma has been used in the past for a number of vascular lesions, which vary not only by their morphological features, but more importantly, also by their biological behavior. In the recent WHO-classification of mesenchymal tumours haemangioendotheliomas have been defined as vascular tumours of "intermediate" or "borderline" malignancy, and spindle cell haemangioendothelioma (SHE), epithelioid haemangioendothelioma (EHE), and rare malignant endovascular papillary angioendothelioma (Dabska's tumour) were included in this category. To this list might be added the more recently delineated kaposiform (KHE), retiform (RHE), polymorphous (PHE), and composite haemangioendothelioma (CHE). Although very popular, the concept of "borderline" or "intermediate" malignancy encompasses a wide variety of clinical situations, prognosis, and biological behavior. Therefore uncritical use of the term haemangioendothelioma represents a potential source of confusion to patients and oncologists, and it should not be used without further clarification. SHE was originally described as low-grade angiosarcoma, however, the study of large series with expanded follow-up information clarified that these lesions are often multicentric in one anatomic region, whereas true recurrences are rather rare, and systemic metastases and tumour progression do not occur. Therefore redesignation of these lesions as spindle cell haemangioma has been proposed. EHE of skin and soft tissues represents a distinctive vascular neoplasm characterized by nests and cords of epithelioid endothelial tumour cells with characteristic cytoplasmic vacuoles, which are set in a myxohyaline matrix. The reported rates of systemic metastases (20-30%), and tumour related death of patients (13-17%) in EHE, and the occurrence of multicentric EHE argue against the classification of EHE as a low-grade or "borderline" malignant neoplasm; EHE should be better regarded as a clearly malignant vascular tumour (G2). Although it seems that KHE is associated with a high mortality rate, the deaths are almost always related to locally invasive effects or as result of bleeding and consumption coagulopathy. So far no metastasizing case of KHE has been reported, and it seems that the prognosis in KHE is mainly related to size, anatomical site and depth of the lesion. KHE should be classified as a locally aggressive, non-metastasizing vascular tumour. The remaining entities (RHE, Dabska's tumour, PHE, and CHE) are characterized by an infiltrative growth, a high rate of (often repeated) local recurrences, and a definitive risk of metastases. Therefore these lesions fulfil criteria for low-grade malignant vascular neoplasms.