Pulmonary Epithelioid Hemangioendothelioma Diagnosed With Endobronchial Biopsies: A Case Report and Literature Review
We report the case of a 46-year-old man with a diagnosis of PEHE from endobronchial biopsies of an intraluminal nodule, a rare presentation of this disease. We summarize a review of the literature and the bronchoscopic findings of PEHE.
Modern Pathology, Published online: 19 September 2019; doi:10.1038/s41379-019-0368-8Prognostic stratification of clinical and molecular epithelioid hemangioendothelioma subsets
Conditions: Hemangioma; Kaposiform Hemangioendothelioma Interventions: Drug: Sirolimus(0.8mg/m2); Drug: Sirolimus(0.7mg/m2) Sponsor: Children's Hospital of Fudan University Recruiting
Pseudomyogenic hemangioendothelioma (PMHE) is a rare endothelial neoplasm that involves the bones in only 14% of all cases. The optimal treatment strategy has not been established. We herein report a case of p...
We present a non-healing lesion on the gingivae attached to the mandible that mimicked periodontal disease, and was refractory to local debridement and oral hygiene measures. Biopsy examination showed a metastatic epithelioid haemangioendothelioma. This rare vascular tumour is considered to be malignant and management is controversial, owing to the paucity of published data. Treatment was provided in a multi-disciplinary setting as a result of established pulmonary and musculoskeletal metastases. The patient opted for conservative management and close surveillance of the lesion, which continues to remain stable at the time...
Conclusion: Malignant chondroid syringoma is genetically related to tumors with PHF1 rearrangements such as low-grade endometrial sarcoma and ossifying fibromyxoid tumor, but also with tumors having TFE3 rearrangements such as renal cell carcinoma, alveolar soft part sarcoma, PEComa, and epithelioid hemangioendothelioma. Further investigations on malignant chondroid syringomas are needed in order to determine whether genetic heterogeneity exists among them and the clinical impact of the PHF1-TFE3 fusion.
We report a case of a radically resected mediastinal EHE originating from the superior vena cava. A 79-year-old woman was referred to our hospital for colon cancer surgery. Computed tomography showed a 50-mm middle mediastinal tumor in direct contact with the left and right brachiocephalic veins, aortic arch, and right brachiocephalic artery. Right lateral thoracotomy was performed, and the tumor was resected en bloc with the right brachiocephalic vein and vagal nerve. Recurrence was not observed at 7 years after surgery, even without adjuvant therapy.
We present a non-healing lesion on the gingivae attached to the mandible that mimicked periodontal disease, and was refractory to local debridement and oral hygiene measures. Biopsy examination showed a metastatic epithelioid haemangioendothelioma. This rare vascular tumour is considered to be malignant and management is controversial, owing to the paucity of published data. Treatment was provided in a multi-disciplinary setting as a result of established pulmonary and musculoskeletal metastases.
AbstractThe classification of vascular neoplasms continues to evolve as we accumulate more genetic and clinical data, particularly for rare tumor types. Because of tumor rarity, changes to classification schema, overlapping histologic features, and in some cases, lack of morphologic evidence of vasoformation, vascular neoplasms present a diagnostic challenge. Here, we discuss recent developments in our understanding of vascular tumors, with a detailed discussion of epithelioid hemangioma, tufted angioma, kaposiform hemangioendothelioma, composite hemangioendothelioma, pseudomyogenic hemangioendothelioma, epithelioid hemang...
Condition: Tacrolimus, Kaposiform Hemangioendothelioma, Tufted Angioma Intervention: Drug: Tacrolimus ointment Sponsor: West China Hospital Not yet recruiting
This report describes the diagnostic problems and therapeutic challenges in the management of this rare tumor over a four-year follow-up period. The clinical course emphasizes the highly unpredictable nature of this tumor. PMID: 26674363 [PubMed]