{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,18]],"date-time":"2026-05-18T13:27:27Z","timestamp":1779110847855,"version":"3.51.4"},"reference-count":29,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,3,15]],"date-time":"2021-03-15T00:00:00Z","timestamp":1615766400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2021,3,15]],"date-time":"2021-03-15T00:00:00Z","timestamp":1615766400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Diagn Pathol"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignant potential which shows locally aggressive growth but only rarely metastasizes. It is mostly considered to be a tumor of pediatric population but its occurrence in the adults is not uncommon as once considered. Histologically, KHE can mimic other soft tissue neoplasms of different behaviors (e.g. Kaposi Sarcoma, hemangioma) and establishing the correct diagnosis is important for appropriate treatment. Herein, we describe the clinicopathological features of 8 cases of KHE which will be helpful in making their diagnosis.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>We reviewed pathology reports, microscopy glass slides and obtained follow up information about 8 cases of KHE which were diagnosed at our institution from January 2008 till June 2020. Immunohistochemical stain for HHV8 was also performed.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>Age ranged from 7 months to 25 years. Seven patients were less than 20 years of age and one patient was 25 years old. Equal gender distribution was observed. Extremities were the most common sites of involvement, followed by head and neck, pancreas and ischiorectal region. 2 cases were resection specimen and all others were incisional biopsies. The largest tumor size was 5.5\u00a0cm in one of the resections. The incisional\/fragmented tissues were all less than 5\u00a0cm in aggregate. Most cases showed predominance of nodular growth and a minor component of spindle cell population along with lymphangiomatosis like vascular channels, with evidence of microthrombi in 2 cases. Few multinucleated giant cells were observed in 2 cases. None of the cases exhibited significant nuclear atypia or mitotic activity. One of the cases arising in dermis showed underlying bone involvement. HHV8 was negative in 7\/7 cases.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>KHE can also involve adult population and it should always be considered in the differential diagnoses of a vascular lesion. Presence of multinucleated giant cells is a rare finding. Knowledge about histological features and potential mimics is helpful in avoiding misdiagnosis.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13000-021-01080-9","type":"journal-article","created":{"date-parts":[[2021,3,15]],"date-time":"2021-03-15T13:02:51Z","timestamp":1615813371000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Kaposiform Hemangioendothelioma: clinicopathological characteristics of 8 cases of a rare vascular tumor and review of literature"],"prefix":"10.1186","volume":"16","author":[{"given":"Qurratulain","family":"Chundriger","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Muhammad Usman","family":"Tariq","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jamshid","family":"Abdul-Ghafar","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arsalan","family":"Ahmed","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nasir Ud","family":"Din","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,3,15]]},"reference":[{"key":"1080_CR1","volume-title":"WHO Classification of Tumors of Soft Tissue and Bone","year":"2020","unstructured":"Christopher DM, Fletcher JAB, Pancras CW, Hogendoorn F, Mertens, editors. WHO Classification of Tumors of Soft Tissue and Bone. 5th ed. Lyon: IARC; 2020.","edition":"5"},{"issue":"4","key":"1080_CR2","doi-asserted-by":"publisher","first-page":"321","DOI":"10.1097\/00000478-199304000-00001","volume":"17","author":"LR Zukerberg","year":"1993","unstructured":"Zukerberg LR, Nickoloff BJ, Weiss SW. Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis. Am J Surg Pathol. 1993;17(4):321\u20138.","journal-title":"Am J Surg Pathol"},{"issue":"2","key":"1080_CR3","doi-asserted-by":"publisher","first-page":"106","DOI":"10.1016\/j.ejim.2008.06.008","volume":"20","author":"Y Fern\u00e1ndez","year":"2009","unstructured":"Fern\u00e1ndez Y, Bernabeu-Wittel M, Garc\u00eda-Morillo JS. Kaposiform hemangioendothelioma. Eur J Intern Med. 2009;20(2):106\u201313.","journal-title":"Eur J Intern Med"},{"issue":"1","key":"1080_CR4","doi-asserted-by":"publisher","first-page":"142","DOI":"10.1016\/j.jpeds.2012.06.044","volume":"162","author":"SE Croteau","year":"2013","unstructured":"Croteau SE, Liang MG, Kozakewich HP, Alomari AI, Fishman SJ, Mulliken JB, et al. Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr. 2013;162(1):142\u20137.","journal-title":"J Pediatr"},{"key":"1080_CR5","unstructured":"Weiss SW, Goldblum JR, Folpe AL. Enzinger and Weiss\u2019s soft tissue tumors: Elsevier Health Sciences; 2007."},{"issue":"5","key":"1080_CR6","doi-asserted-by":"publisher","first-page":"448","DOI":"10.1080\/00313020310001602657","volume":"35","author":"A Hong","year":"2003","unstructured":"Hong A, Davies S, Lee CS. Immunohistochemical detection of the human herpes virus 8 (HHV8) latent nuclear antigen-1 in Kaposi\u2019s sarcoma. 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Since this was a retrospective observational study and did not involve actual patients or patient\u2019s images, it was exempted from by the Aga Khan University Ethical Review Committee. Informed consent was obtained from parents and\/or legal guardians for participants who are under age 18.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from patients or parents and\/or legal guardians for participants who are under age 18.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"It is declared that all authors have no conflict of interest.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"23"}}