Study Design: The authors report two cases of vascular tumors of the spine, classified originally as benign and malignant hemangioendothelioma, and after revision, as cellular hemangioma and angioblastomatosis, respectively.
Objectives: Problems in interpretation of the confusing term hemangioendothelioma and treatment modalities for vascular tumors of the spine are discussed.
Summary Of Background Data: Hemangioendothelioma is a confusing term and is often used to cover bewilderment at the biological behavior of a vascular tumor. Its spectrum ranges, depending the references used, from benign to malignant and can mistakenly include benign lesions like cellular hemangioma and angioblastoma (solitary and multicentric).
Methods: Of two patients with a cellular tumor of the spine, the clinicopathologic data and modes of treatment are reviewed. The relevant literature is discussed.
Results: In the first case, the diagnosis of benign cellular hemangioendothelioma was changed to cellular hemangioma. In the second case, the original diagnosis of malignant hemangioendothelioma with metastasis to liver and lungs was changed to angioblastomatosis, most probably benign. In both cases, a correct interpretation of the initial diagnosis or proper diagnosis would have influenced the mode of treatment.
Conclusion: Avoid the confusing term hemangioendothelioma. If a vascular lesion is benign, it should be classified as a variant of hemangioma. If malignant as angiosarcoma, use a separate category, in which lesions like angioblastoma and angioblastomatosis can be put until their nature has been clarified.
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http://dx.doi.org/10.1097/00007632-199404150-00021 | DOI Listing |
Cell Mol Life Sci
January 2025
Department of Anesthesiology, Shenzhen Children's Hospital, Yitian Road 7019, Shenzhen, 518000, China.
Hair follicle (HF) development and pigmentation are complex processes governed by various signaling pathways, such as TGF-β and FGF signaling pathways. Nestin + (neural crest like) stem cells are also expressed in HF stem cells, particularly in the bulge and dermal papilla region. However, the specific role and differentiation potential of these Nestin-positive cells within the HF remain unclear, especially regarding their contribution to melanocyte formation and hair pigmentation.
View Article and Find Full Text PDFCureus
January 2025
Oral and Maxillofacial Surgery, Queen Elizabeth Hospital Birmingham, Birmingham, GBR.
Epithelioid haemangioendothelioma (EHE) is a rare vascular neoplasm characterised by proliferation of vascular endothelial and pre-endothelial cells. The prevalence is less than one in a million people. It is principally observed in the soft tissues of the extremities but can also occur in the bone, brain, liver, lung and lymph nodes.
View Article and Find Full Text PDFJ Int Med Res
December 2024
Department of Hepatobiliary Surgery, Affiliated Hangzhou First People's Hospital, Westlake University, School of Medicine, Hangzhou, China.
Epithelioid hemangioendothelioma is a low-grade malignant tumor of vascular origin. The rarity of hepatic epithelioid hemangioendothelioma (HEHE) makes the diagnosis and treatment of this entity challenging. We report a case of a 69-year-old female patient who suffered from HEHE and complained of abdominal distension pain with dizziness and appetite loss for more than half a month.
View Article and Find Full Text PDFGen Thorac Cardiovasc Surg Cases
December 2024
Department of Thoracic Surgery, Kyoto University Graduate School of Medicine, Kyoto, 606-8507, Japan.
Background: Lung transplantation is a viable lifesaving option for patients with diffuse pulmonary arteriovenous malformations (AVMs). We present a case of diffuse pulmonary AVMs associated with juvenile polyposis and hereditary hemorrhagic telangiectasia (JP-HHT) that was successfully managed by lung transplantation.
Case Presentation: A 19-year-old woman developed severe hypoxemia due to pulmonary AVMs diagnosed at 4 years of age.
Pediatr Rep
December 2024
Pediatric and Rare Diseases Clinic, Microcitemico Hospital "A. Cao", Department of Medical Sciences and Public Health, University of Cagliari, 09124 Cagliari, Italy.
Background: Houge-Janssens syndrome 1 is a condition with onset in early childhood caused by heterozygous pathogenic variants in the gene, which encodes a B56 regulatory subunit of the serine/threonine protein phosphatase 2A (PP2A). There is evidence that the PP2A-PPP2R5D complex is involved in regulating the phosphatidylinositol 3-kinase (PI3K)/AKT signalling pathway, which is crucial for several cellular processes, including the pathogenesis and progression of haemangiomas.
Case Presentation: We report the first -related neurodevelopmental disorder case from Sardinia, a child with transient hypoglycaemia, facial dysmorphisms, and multiple haemangiomas.
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