Epithelioid Hemangioendothelioma. Ibtehal Al-Harbi Medical Intern

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1 Epithelioid Hemangioendothelioma Ibtehal Al-Harbi Medical Intern

2 Hemangioendothelioma Hemangioendothelioma (HE) is the term used to describe a diverse group of vascular cancers. Very rare, accounting for only a fraction of 1% of all cancers. Involve soft tissue, bone, skin, liver, lymph node and/or lung, Generally slow and indolent. However, in some cases it can progress to devastating disease. The tumors are often locally invasive and destroy surrounding tissue. the predominant proliferating cell is related to an endothelial cell.

3 CLASSIFICATIONS OF HEMANGIOENDOTHELIOMA 1. Epithelioid hemangioendothelioma (EHE), having epithelial-like cells lining the vascular channels and can occur simultaneously at multiple sites 2. Malignant endovascular papillary angioendothelioma, also known as a Dabska tumor, a. low-grade angiosarcoma that can affect the skin of children 3. Retiform (RKE) - retiform pattern of proliferating vessels with intraluminal papillae and hyaline cores. Histologically similar to those seen in Dabska tumor. 4. Spindle cell hemangioendothlioma (SCHE), has spindle cells,smooth muscle cells normally supporting the vessels. 5. Kaposiform hemangioendothelioma (KHE) which is a locally invasive tumor, occuring in young children and having no capacity to metastasize. 6. Angiolymphoid *Recenly? Composite and Polymorphous

4 Epithelioid hemangioendothelioma Described for the first time in 1975 by Dail and Liebow as aggressive form of bronchoalveolar cell carcinoma (P-EHE). Rare vascular tumor with an epithelioid and histiocytoid appearance, originating from vascular endothelial or pre-endothelial cells. Features between hemangioma and angiosarcoma (ergo, low grade malignancy) Locally aggressive with metastatic potential

5 What causes Epithelioid Hemangioendothelioma? 1. WWTR/CAMTA translocation Ch. (3:1) 2. Association with the infection of the Bartonella bacterium (unproven) 3. Complex unbalanced translocation between chromosomes 7 and 22 with multiple breakpoints, 4. Robertsonian translocation of chromosome 14, and the loss of chromosome Y. 5. YAP1-TFE3 fusion

6 Sites:

7 Sites:

8 Clinical Features Asymptomatic! History of intermittent epistaxis slowly growing firm swelling Involving the sinuses? Visual disturbances

9 Diagnostic Tools LABROTORY: NORMAL

10 Imaging CT MRI PET BONE SCINTIGRAPHY

11 CT Increased soft-tissue density anterior to the right middle turbinate (arrow).

12 Histopathology MICROSCOPY IMMUNOHISTOCHEMISTRY

13 Microscopy

14 "blister cells" with intracytoplasmic vacuoles.

15 Epithelioid tumor cells are arranged in a nest-like pattern (H&E).

16 Tumor cells are vacuolated, with intracellular lumen formation (arrows); erythrocytes are also present in intracytoplasmic vacuoles (arrowhead) (H&E).

17 Immunohistochemistry Fli-1 protein CD31 CD34 (sensitive) Ulex europaeus agglutinin type 1 lectin (UEA-1) Factor VIII-related antigen Cytokeratin & EMA negetive! Podoplanin (liver) CD68 (bone)

18 Tumor cells are immunoreactive to Ulex europaeus agglutinin type 1 lectin (UEA-1).

19 Differential Diagnosis A definitive diagnosis depends on histopathologic and immunochemical features! Clinically, it is hard to differentiate from benign lesions, including pyogenic granuloma, hemangioma, angiomyoma, arteriovenous malformation, and hamartoma. The microscopic differential diagnosis includes Metastatic Carcinomas: greater degrees of nuclear atypia, more mitotic figures, and are positive for cytokeratins, Epithelioid Angiosarcoma: shows vascular differentiation with irregular anastomotic vascular channels. Epithelioid Sarcoma: composed of rounded eosinophilic cells surrounding cores of necrotic debris and collagen, and develops in the distal extremities of younger people.

20 Treatments For Hemangioendot helioma LOCATION SPEED GRADE STAGE CONDITION OF THE PT.

21 Treatment Options SURGICAL RADIOTHERAPY CHEMOTHERAPY BACTERIAL ERADICATION?

22 The Role of Radiotherapy

23 Refrences Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor Angela Sardaro,1 Lilia Bardoscia,1 Maria Fonte Petruzzelli,1 Maurizio Portaluri2,3 1Department of Interdisciplinary Medicine, Section of Diagnostic Imaging and Radiotherapy, University Aldo Moro, Bari; 2Department of Radiotherapy, A. Perrino Hospital, Brindisi; 3Clinical Physiology Institute, National Research Council (IFC-CNR), Pisa-Lecce, Italy Epithelioid Hemangioendothelioma of the Nasal Cavity Chih-Chieh Tseng1, Shyh-Haw Tsay2,3, Tung-Lung Tsai1*, Chih-Hung Shu1,3 Departments of 1Otolaryngology and 2Pathology, Taipei Veterans General Hospital, and 3National Yang-Ming University School of Medicine, Taipei, Taiwan, R.O.C.

24 Thank You

25 Epithelioid hemangioendothelioma Rare vascular tumor originating from vascular endothelial or pre-endothelial cells.(less than 1%) The term EHE is to describe vascular tumor of bone and soft tissue showing features between hemangioma and angiosarcoma. Etiology and hypothesis are still unkown. Often misdiagnosed which leads to poor prognosis. WHO classification 2002: locally aggressive with metastatic potential. Indolent course, with the potential for recurrence, but rarely metastasizes. M:F 1:4, middle age

26 PROGRESSION OF HEMANGIOENDOTHELIOMA The clinical course is unpredictable and different treatment modalities are offered depending on the patient s condition. The prognosis is also unpredictable, in some cases the lesion disappear and re-occur later. Others may grow and later spontaneously regress. Recurrances are more common when the tumor hasn t been completely resected. 1/3 has multiple affected sites in lymph nodes, lungs, liver or bones.

27 Differential Diagnosis adenocarcinoma (whose cytoplasmic vacuoles do not contain erythrocytes - positive for mucin stains, instead - as well as diffuse positivity for cytokeratin and epithelial membrane antigen); Malignant mesothelioma (showing little cytologic atypia, two-toned cytoplasm known as fuzzy cytoplasmic borders, lack of cytoplasmic vacuoles, positivity for mesothelial markers such as calretinin, cytokeratin 5/6 or WT-1); melanoma (with greater amounts of cytologic atypia and more frequent mitoses, necrotic debris and melanin pigment, and expression of S100 protein, MART-1, and other melanocytic markers); Epithelioid angiosarcoma (presenting greater cellularity, larger cells, more prominent mitotic activity, greater nuclear and nucleolar pleomorphism, and more frequent tightly, cohesive cell clusters without myxohyaline matrix, immunoreactivity for vimentin and cytokeratin); Epithelioid sarcoma (negative for vascular markers) meningioma (in case of intracranial localization, negative for vascular markers and positive for cytokeratin and S-100 protein)

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