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It is very rarely described in the head and neck region, and is exceptionally uncommon within the submandibular region. We report a very rare case of EHE originating in a lymph node within the submandibular salivary gland of a 54-year-old female patient. The tumour was resected and the patient was regularly followed up, with no recurrence of disease at 24 months postoperatively. A review of existing literature, clinical and immunohistopathological features are discussed, which highlight the diagnostic dilemma, absence of consensus for management and appropriate surveillance method associated with EHE.<\/jats:p>","DOI":"10.1007\/s12070-024-04752-2","type":"journal-article","created":{"date-parts":[[2024,6,6]],"date-time":"2024-06-06T14:02:11Z","timestamp":1717682531000},"page":"3956-3961","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Primary Intranodal Epithelioid Haemangioendothelioma in the Submandibular Region: A Case Report"],"prefix":"10.1007","volume":"76","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9816-0712","authenticated-orcid":false,"given":"Khilan","family":"Shukla","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Touraj","family":"Taheri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hooman","family":"Baghaie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Omar","family":"Breik","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,6,6]]},"reference":[{"issue":"9","key":"4752_CR1","doi-asserted-by":"publisher","first-page":"2108","DOI":"10.1002\/cncr.22225","volume":"107","author":"A Mehrabi","year":"2006","unstructured":"Mehrabi A, Kashfi A, Fonouni H et al (2006) Primary malignant hepatic epithelioid hemangioendothelioma. 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