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Hemangioendothelioma

January 01, 1970

Hemangioendotheliomas are a family of vascular neoplasms of intermediate malignancy.

Hemangioendothelioma
Micrograph of a kaposiform hemangioendothelioma with "glomeruloid" nodules of endothelial cells.
SpecialtyOncology 

Signs and symptoms edit

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.[citation needed]

Cause edit

Possibly Bartonella spp bacteraemia[citation needed]

Diagnosis edit

Classification edit

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 seen 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).
  •  
    Characteristic budding, hobnail-like endothelial cells visible.

Treatment edit

Treatment is varied and depends on the site and extent of tumor involvement, sites 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. Kaposiform hemangioendothelioma might respond to chemotherapy or antiangiogenic therapies. Recently propanolol and steroids have been shown to be very effective in kaposiform hemangioendothelioma.[17]

References edit

  1. ^ Billings SD, Folpe AL, Weiss SW (January 2003). "Epithelioid sarcoma-like hemangioendothelioma". Am. J. Surg. Pathol. 27 (1): 48–57. doi:10.1097/00000478-200301000-00006. PMID 12502927. S2CID 28749740.
  2. ^ a b c d e f g James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
  3. ^ a b c d e f Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
  4. ^ 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. S2CID 31168575.
  5. ^ 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.
  6. ^ a b c Schultheis 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.
  7. ^ a b c Schwartz, RA. Dabska Tumor. Medscape, updated: Apr 5, 2013
  8. ^ Fukunaga M (Oct 1998). "Endovascular papillary angioendothelioma (Dabska tumor)". Pathol. Int. 48 (10): 840–1. doi:10.1111/j.1440-1827.1998.tb03847.x. PMID 9788271. S2CID 38650082.
  9. ^ a b Bhatia 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.
  10. ^ 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.
  11. ^ a b McCarthy EF, Lietman S, Argani P, Frassica FJ (Feb 1999). "Endovascular papillary angioendothelioma (Dabska tumor) of bone". Skeletal Radiol. 28 (2): 100–3. doi:10.1007/s002560050482. PMID 10197456. S2CID 23809159.
  12. ^ 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. S2CID 24778725.
  13. ^ Patterson K, Chandra RS (Jul 1985). "Malignant endovascular papillary angioendothelioma. Cutaneous borderline tumor". Arch Pathol Lab Med. 109 (7): 671–3. PMID 3839367.
  14. ^ Roos J, Pfiffner R, Stallmach T, Stuckmann G, Marincek B, Willi U (2003). "Infantile hemangioendothelioma". Radiographics. 23 (6): 1649–55. doi:10.1148/rg.236035024. PMID 14615570.Full text
  15. ^ a b Banks T, Podraza J. What's Your Diagnosis? Newborn with abdominal mass and distention 2010-07-02 at the Wayback Machine. Consultant for Pediatricians. 2010;9:168-173.
  16. ^ Banks T, Podraza J. What's Your Diagnosis? Newborn with abdominal mass and distention 2010-07-02 at the Wayback Machine. Condultant for Pediatricians. 2010;9:168-173.
  17. ^ Verma, Saurav; Dhamija, Ekta; Barwad, Adarsh; Kumar, Venkatesan S.; Rastogi, Sameer (30 July 2020). "A case report of Kaposiform haemangioendothelioma; response with propranolol and steroids". Clinical Sarcoma Research. 10 (1): 12. doi:10.1186/s13569-020-00134-8. PMC 7394668. PMID 32765826.

External links edit

January 01, 1970
hemangioendothelioma, family, vascular, neoplasms, intermediate, malignancy, micrograph, kaposiform, hemangioendothelioma, with, glomeruloid, nodules, endothelial, cells, specialtyoncology, contents, signs, symptoms, cause, diagnosis, classification, treatment. Hemangioendotheliomas are a family of vascular neoplasms of intermediate malignancy HemangioendotheliomaMicrograph of a kaposiform hemangioendothelioma with glomeruloid nodules of endothelial cells SpecialtyOncology Contents 1 Signs and symptoms 2 Cause 3 Diagnosis 3 1 Classification 4 Treatment 5 References 6 External linksSigns and symptoms editThey 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 citation needed Cause editPossibly Bartonella spp bacteraemia citation needed Diagnosis editClassification edit 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 a1 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 seen 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 a 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 nbsp Low power photomicrograph of an endovascular papillary angioendothelioma showing papillae with hyalinized cores nbsp High power view showing a vascular tumor with cuboidal endothelium lining the vessels Few entrapped seminiferous tubules are also noted arrow nbsp Characteristic budding hobnail like endothelial cells visible Treatment editTreatment is varied and depends on the site and extent of tumor involvement sites 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 Kaposiform hemangioendothelioma might respond to chemotherapy or antiangiogenic therapies Recently propanolol and steroids have been shown to be very effective in kaposiform hemangioendothelioma 17 References edit Billings SD Folpe AL Weiss SW January 2003 Epithelioid sarcoma like hemangioendothelioma Am J Surg Pathol 27 1 48 57 doi 10 1097 00000478 200301000 00006 PMID 12502927 S2CID 28749740 a b c d e f g James William Berger Timothy Elston Dirk 2005 Andrews Diseases of the Skin Clinical Dermatology 10th ed Saunders ISBN 0 7216 2921 0 a b c d e f Rapini 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 S2CID 31168575 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 a b c Schultheis 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 a b c Schwartz RA Dabska Tumor Medscape updated Apr 5 2013 Fukunaga M Oct 1998 Endovascular papillary angioendothelioma Dabska tumor Pathol Int 48 10 840 1 doi 10 1111 j 1440 1827 1998 tb03847 x PMID 9788271 S2CID 38650082 a b Bhatia 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 a b McCarthy EF Lietman S Argani P Frassica FJ Feb 1999 Endovascular papillary angioendothelioma Dabska tumor of bone Skeletal Radiol 28 2 100 3 doi 10 1007 s002560050482 PMID 10197456 S2CID 23809159 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 S2CID 24778725 Patterson K Chandra RS Jul 1985 Malignant endovascular papillary angioendothelioma Cutaneous borderline tumor Arch Pathol Lab Med 109 7 671 3 PMID 3839367 Roos J Pfiffner R Stallmach T Stuckmann G Marincek B Willi U 2003 Infantile hemangioendothelioma Radiographics 23 6 1649 55 doi 10 1148 rg 236035024 PMID 14615570 Full text a b Banks T Podraza J What s Your Diagnosis Newborn with abdominal mass and distention Archived 2010 07 02 at the Wayback Machine Consultant for Pediatricians 2010 9 168 173 Banks T Podraza J What s Your Diagnosis Newborn with abdominal mass and distention Archived 2010 07 02 at the Wayback Machine Condultant for Pediatricians 2010 9 168 173 Verma Saurav Dhamija Ekta Barwad Adarsh Kumar Venkatesan S Rastogi Sameer 30 July 2020 A case report of Kaposiform haemangioendothelioma response with propranolol and steroids Clinical Sarcoma Research 10 1 12 doi 10 1186 s13569 020 00134 8 PMC 7394668 PMID 32765826 External links edit Retrieved from https en wikipedia org w index php title Hemangioendothelioma amp oldid 1188186542, wikipedia, wiki, book, books, library,

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