Micrograph of a kaposiform hemangioendothelioma with "glomeruloid" nodules of endothelial cells.
Specialty
Oncology
Hemangioendotheliomas are a family of vascular neoplasms of intermediate malignancy.
Contents
1Signs and symptoms
2Cause
3Diagnosis
3.1Classification
4Treatment
5References
6External links
Signs and symptoms
They have been described as masses that fall between a hemangioma and angiosarcoma. They are vascular tumors that commonly present with an enlarging mass and most commonly involve the lungs, liver, and musculoskeletal system, although many other body sites have been reported, including the head and neck, intestines, lymph nodes, pleura, retroperitoneum, heel, stomach.
Cause
Possibly Bartonella spp bactaraemia
Diagnosis
Classification
Hemangioendotheliomas may be classified as:
Epithelioid hemangioendothelioma is an uncommon vascular tumor of intermediate malignancy that was first described by Steven Billings, Andrew Folpe, and Sharon Weiss in 2003.[1] These tumors are so named because their histologic appearance resembles a proliferation of epithelioid cells, with polygonal shape and eosinophilic cytoplasm.
Composite hemangioendothelioma is a low-grade angiosarcoma typically occurring in adults, although it has been described in infancy.[2]:601
Spindle-cell hemangioendothelioma[3]) is a vascular tumor that was first described in 1986 by Sharon Weiss, M.D.,[4] and commonly presents in a child or young adult who develops blue nodules of firm consistency on a distal extremity.[2]:599 These tumors were reclassified by Dr. Weiss in 1996 as "spindle cell hemangioma", rather than hemangioendothelioma, due to the excellent prognosis observed in a group of 78 patients.[5]
Retiform hemangioendothelioma (also known as a "Hobnail hemangioendothelioma"[3]) is a low-grade angiosarcoma, first described in 1994, presenting as a slow-growing exophytic mass, dermal plaque, or subcutaneous nodule.[2]:601
Kaposiform hemangioendothelioma (also known as "Infantile kaposiform hemangioendothelioma"[3]) is an uncommon vascular tumor, first described by Niedt, Greco, et al. (Hemangioma with Kaposi's sarcoma-like features: report of two cases.(Niedt GW, Greco MA, Wieczorek R, Blanc WA, Knowles DM 2nd. that affects infants and young children, with rare cases having also been reported in adults.Pediatr Pathol. 1989;9(5):567-75.)[2]:596[3]:1782
Endovascular papillary angioendothelioma, also known as "Dabska tumor",[2] "papillary intralymphatic angioendothelioma"[3] (PILA),[6] "Dabska-type hemangioendothelioma", "hobnail hemangioendothelioma", and "malignant endovascular papillary angioendothelioma",[3] is a rare low-grade angiosarcoma [2]:601 of lymphatic channels.[6] Approximately 30 such tumors have been described in the medical literature.[7] Although included in the World Health Organization tumor classification, there is uncertainty as to whether EPA is a distinct entity or a heterogenous group of tumours.[7][8] The lesion usually presents as a slow-growing tumor of the skin and subcutaneous tissues[9] of the head, neck, or extremity, of infants or young children.[2]:601 However, EPA has involved the testicle,[6][9] deep muscle tissue as a neoplastic transformation of a larger existing benign cavernous hemangioma,[10] bone[11] and spleen, and has been found in adults.[7][11][12] Some reports indicate a good prognosis[13] but metastasis is occasionally seen.
Infantile hemangioendothelioma is a rare benign vascular tumour arising from mesenchymal tissue and is usually located in the liver. It often presents in infancy with cardiac failure because of extensive arteriovenous shunting within the lesion. It is the third most common liver tumor in children, the most common benign vascular tumor of the liver in infancy, and the most common symptomatic liver tumor during the first 6 months of life.[14] These hemangioendotheliomas have 2 growth phases: an initial rapid growth phase, which is followed by a period of spontaneous involution (usually within the first 12 to 18 months of life). Detection of the hemangioendothelioma within the first 6 months of life is attributed to the initial rapid growth during this time; however, the tumor has been detected with fetal ultrasonography.[15] Histopathologically, there are 2 types of hepatic hemangioendotheliomas:
Type I: Hemagioendotheliomas of this type have multiple vascular channels that are formed by an immature endothelial lining with stromal separation from bile ductules.
Type II: These hemangioendotheliomas have an appearance that is more disorganized and hypercellular, and there are no bile ductules.
In children, distinguishing between a primary malignant liver tumor (hepatoblastoma) and a benign primary hepatic lesion (hemangioendothelioma) is crucial. The absence of urinary catecholamines supports the diagnosis of hemangioendothelioma. In patients with hemangioendotheliomas, elevations in α1-fetoprotein levels are milder than those found in patients with hepatoblastomas. Infantile hepatic hemangioendothelioma is strongly suggested by the presence of a vascular lesion on imaging studies. A complex, heterogeneous mass is often seen on ultrasonograms; a complex tumor that lacks central enhancement can be see on CT scans; and the vascular nature of the lesion along with dilation of the aorta proximal to the origin of the celiac artery and a decrease in the diameter distally, indicating significant shunting, is seen on angiograms.[16] Because most hemangioendotheliomas in infants sponanteously involute and regress within the first 12 to 18 months of life, asymptomatic lesions are generally managed conservatively. Infants who have severe anemia and/or thrombocytopenia can be given blood products; for those who have cardiac failure, diuretics and digoxin are often given. To stop further growth and to speed regression of lesions in infants with more significant clinical sequelae, treatment with corticosteroids or interferon-α-2a is administered. To slow the growth of tumors that are rapidly enlarging, chemotherapy and radiation therapy have been used. Surgical resection, partial hepatectomy, and embolization of afferent vessels should be considered for severe cases.[15]
Low power photomicrograph of an endovascular papillary angioendothelioma showing papillae with hyalinized cores
High power view showing a vascular tumor with cuboidal endothelium lining the vessels. Few entrapped seminiferous tubules are also noted (arrow).
Treatment is varied and depends on the site and extent of tumor involvement, site(s) of metastasis, and specific individual factors. Surgical resection, radiotherapy, and chemotherapy have all been used to treat these masses, although studies on survival have yet to be conducted to delineate various treatment regimens.
^ abcdefgJames, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
^ abcdefRapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
^Weiss SW, Enzinger FM (1986). "Spindle cell hemangioendothelioma. A low-grade angiosarcoma resembling a cavernous hemangioma and Kaposi's sarcoma". Am J Surg Pathol. 10 (8): 521–30. doi:10.1097/00000478-198608000-00001. PMID 3740350.
^Perkins P, Weiss SW (October 1996). "Spindle cell hemangioendothelioma. An analysis of 78 cases with reassessment of its pathogenesis and biologic behavior". Am. J. Surg. Pathol. 20 (10): 1196–204. doi:10.1097/00000478-199610000-00004. PMID 8827025.
^ abcSchultheis AM, Sandmann M, Steurer S (2013). "Strong ERG Positivity in Papillary Intralymphatic Angioendothelioma of the Testis of a 24-Year-Old Male: A Case Report". Case Rep Pathol. 2013: 531479. doi:10.1155/2013/531479. PMC 3626176. PMID 23607024.
^ abBhatia A, Nada R, Kumar Y, Menon P (2006). "Dabska tumor (endovascular papillary angioendothelioma) of testis: a case report with brief review of literature". Diagn Pathol. 1: 12. doi:10.1186/1746-1596-1-12. PMC 1555613. PMID 16859564.
^Argani P, Athanasian E (Sep 1997). "Malignant endovascular papillary angioendothelioma (Dabska tumor) arising within a deep intramuscular hemangioma". Arch Pathol Lab Med. 121 (9): 992–5. PMID 9302935.
^Yamada A, Uematsu K, Yasoshima H, Sakurai K, Hori K, Ohya M, Ohkubo E, Ogasawara H (Feb 1998). "Endovascular papillary angioendothelioma (Dabska tumor) in an elderly woman". Pathol. Int. 48 (2): 164–7. doi:10.1111/j.1440-1827.1998.tb03887.x. PMID 9589482.
… consensus classification of hepatoblastoma has been proposed . Epithelioid hemangioendothelioma (EHE) is a low-grade malignant vascular neoplasm that affects middle-aged patients. Women are affected more …
… 100 percent of cases, that has not been detected in other tumors. Epithelioid hemangioendothelioma (EHE) is a malignant vascular neoplasm that is characterized by recurrent translocations involving chromosome …
… example, malignant fibrous histiocytoma , angiosarcoma , leiomyosarcoma , epithelioid sarcoma , hemangioendothelioma , and osteosarcoma . Melanomas are the most aggressive form of skin cancer and can occur …
…sinusoids continuous with normal vascular channels . Epithelioid hemangioendothelioma – Epithelioid hemangioendothelioma (EHE) is a rare, malignant soft tissue tumor of endothelial origin characterized by indolent …
English Journal
Variant WWTR1 gene fusions in epithelioid hemangioendothelioma-A genetic subset associated with cardiac involvement.
The genetic hallmark of epithelioid hemangioendothelioma (EHE) is a recurrent WWTR1-CAMTA1 fusion, which is present in most cases bearing a conventional histology. A subset of cases is characterized by a distinct morphology and harbors instead of YAP1-TFE3 fusion. Nevertheless, isolated cases lack t
Neutering is associated with developing hemangiosarcoma in dogs in the Veterinary Medical Database: An age and time-period matched case-control study (1964-2003).
The Canadian veterinary journal = La revue veterinaire canadienne. 2020 May;61(5)499-504.
The hypothesis that neutered dogs in the Veterinary Medical Database (VMDB) are at increased risk for developing hemangiosarcoma (HSA) was tested. Dogs ( = 5736) were diagnosed with HSA from a population of 2 106 324 dogs in the VMDB from 1964 to 2003. A case-control design matched on age and time p
Both EWSR1 and TFE3 are well-known oncogenes. EWSR1 encodes an RNA-binding protein involved in multiple soft tissue tumors, including Ewing's sarcoma/peripheral neuroectodermal tumor, desmoplastic small round cell tumor, soft tissue clear cell sarcoma (malignant melanoma of soft parts), extraskeleta
… <p>Pseudomyogenic hemangioendothelioma (PMHE) is a new entity. … It is an intermediate soft tissue tumor clinically and/or histopathologically mimicking some other high-grade malignant tumors and some inflammatory diseases. …
Introduction Epithelioid hemangioendothelioma (EH) is an uncommon low-grade malignant tumor of vascular origin that may develop in the soft tissue, lung, bone, brain, liver, and small intestine.Primary hepatic EH is rare.1 In the literature, approximately 200 cases have been reported. ...
malignant hemangioendothelioma: An uncommon, aggressive mesenchymal malignancy, arising in the skin, liver, breast, soft tissues and viscera, primarily affecting the elderly; male:female, 2:1.Aetiology Most are idiopathic; some are linked to chronic lymphoedema (as occurs following axillary dissection for breast CA), others to radiation ...
Iain D et al: Hepatic Epithelioid Hemangioendothelioma: Sonographic, CT and MR imaging Appearances: American Journal of Roentgenology 180: 1359-1364, 2003 Soo-heui Baek et al: Computed Tomography and Magnetic Resonance Imaging Findings of a Malignant Hepatic Epithelioid Hemangioendothelioma: A Rare Case of Solitary Small Nodular Form: Journal of Investigative Medicine High Impact Case Reports ...