Presentation
Presentation in the oral cavity is rare with only two previously reported cases. We report a case of spindle cell hemangioma that presented as an asymptomatic mass of the buccal mucosa in a 44-year-old man. [ncbi.nlm.nih.gov]
Clinical presentation: These lesions usually present as a single or multiple, often painful, small circumscribed reddish blue nodules. [histopathology-india.net]
Here, we present a young adult who presented to us with multiple spindle cell hemangioendotheliomas. The case is reported due to its rarity. [e-ijd.org]
Entire Body System
- Lymphedema
The lesions developed in an area of lymphedema. CHEs are rare vascular tumors in which various types of hemangioendothelioma are combined in 1 lesion. [ncbi.nlm.nih.gov]
[…] hemangioendothelioma Definitions Benign vascular tumor characterized by cavernous vascular spaces and spindled cell areas ETIOLOGY/PATHOGENESIS Unknown Some cases associated with Mafucci syndrome, Klippel-Trenaunay syndrome, varicosities, and congenital lymphedema [basicmedicalkey.com]
Associations Maffucci syndrome varicose veins congenital lymphedema Differential diagnosis Kaposi sarcoma angiosarcomas Molecular Pathology IDHs mutations : IDH1 and IDH2 somatic mutations in : glial tumors spindle cell hemangioma See also vascular tumors [humpath.com]
Spindle cell hemangioma may be associated with anomalies such as Mafucci's syndrome, Klippel-Trenaunay syndrome, lymphedema and early onset varicose vein. Treatment consists of wide local excision without adjuvant chemotherapy or radiotherapy. [histopathology-india.net]
(Case of an SCH that presents in association with lymphedema) Yáñez, S, Val-Bernal, JF, Mira, C, Echevarría, MA, González-Vela, MC, Arce, F. [clinicaladvisor.com]
Skin
- Xanthoma
Contains granules that stain with azocarmine dye. foam cell a cell with a vacuolated appearance due to the presence of complex lipoids; seen in xanthoma. ganglion cell a large nerve cell, especially one of those of the spinal ganglia. giant cell a very [medical-dictionary.thefreedictionary.com]
• Stroma may show myxoid change or hyalinization and some xanthoma like cells. • Deep fibrous histiocytoma usually is similar to cutaneous form, but show more prominent storiform pattern and fewer secondary elements like xanthoma cells. [pedorthpath.com]
Neurologic
- Ataxia
Thieme, Stuttgart Google Scholar Smith LL, Conerly SL (1985) Ataxia-teleangiectasia or Louis-Bar syndrome. [link.springer.com]
Workup
Colonoscopy
- Polyps
NIH: National Cancer Institute Biopsy - polyps (Medical Encyclopedia) Cherry angioma (Medical Encyclopedia) [ Read More ] Birthmarks Also called: Cafe au lait spot, Hemangioma, Mongolian spot, Nevus, Strawberry mark Birthmarks are abnormalities of the [icdlist.com]
FORME CLINIQUE : LE POLYPE SAIGNANT DE LA CLOISON NASALE: UNE PLACE PARTICULIÈRE DOIT ÊTRE FAITE AU POLYPE SAIGNANT DU SEPTUM APPARAISSANT COMME UNE MASSE ANGIOMATEUSE SIÉGEANT AU NIVEAU DE LA TACHE VASCULAIRE, PARFOIS APPELÉ POLYPE ANGIOMATEUX. [fr.slideshare.net]
Requena et al (1997) were of the opinion that pyogenic granuloma presents typically as a papule or polyp with a glistening surface, which bleeds easily. [ispub.com]
Treatment
Spindle Cell Hemangiomas do not cause any symptoms in most cases, and therefore may not require any treatment. [dovemed.com]
Treatment Options Treatment options are summarized in Table I. Table I. [clinicaladvisor.com]
Reports of additional cases will be required to determine the most appropriate treatment for this tumor. [ncbi.nlm.nih.gov]
Treatment Several treatments are used, depending on the severity of the tumor. For many benign tumors, a 'wait and see' stance will be taken or steroids will be administered. [rare-cancer.org]
Sicht wurde mir zu einer operativen Entfernung der Lipome geraten.What Are my Lipoma Treatment Options? I might have a lipoma and I am just curious about what kind of lipoma treatments exist. [oxidepuzupolyyoceti.xpg.uol.com.br]
Prognosis
Prognosis: • Benign with occasional recurrence. [pedorthpath.com]
The authors describe in-depth the clinical and morphologic aspects of each entity and detail their clinicopathologic correlation, differential diagnosis, prognosis, and therapy. [books.google.com]
The 5-year overall survival rate was 69.3%, and the histological assessment according to the tumor necrotic rate mainly corresponded to the prognosis of these patients in comparison with the graphical assessment. [ci.nii.ac.jp]
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 [en.wikipedia.org]
Etiology
TERMINOLOGY Synonyms Formerly known as spindle cell hemangioendothelioma Definitions Benign vascular tumor characterized by cavernous vascular spaces and spindled cell areas ETIOLOGY/PATHOGENESIS Unknown Some cases associated with Mafucci syndrome, Klippel-Trenaunay [basicmedicalkey.com]
Etiology The etiology of spindle cell hemangioma is not clearly known at this time. The literature contains a great deal of discussion regarding the fundamental nature of these lesions. [clinicaladvisor.com]
The ultrasonography (USG) report was suggestive of a right intraparotid mass of uncertain etiology; whereas magnetic resonance imaging (MRI) report inclined toward a mass associated with chronic inflammatory pathology. [jcytol.org]
(Etiology) The exact cause for development of Spindle Cell Hemangiomas is unknown Since family history is an important risk factor, it may be caused by genetic mutations that are yet to be well-characterized Spindle Cell Hemangiomas have been known to [dovemed.com]
The etiology of kaposiform hemangioendothelioma remains unknown. It is not associated with HHV8 infection or Kaposi sarcoma. Tumors often involve soft tissue of extremities as ill-defined, violaceous lesions and may encase normal structures. [path.upmc.edu]
Epidemiology
Epidemiology: • Rare lesion on the digits of young children. [pedorthpath.com]
Other malignancies associated with Maffucci syndrome include pancreatic and hepatic adenocarcinoma, ovarian tumors, brain gliomas, astrocytomas, and other types of sarcomas. [5] Epidemiology Frequency United States Maffucci syndrome is rare. [emedicine.medscape.com]
Epidemiology, pathogenesis, histology, clinical spectrum, staging criteria and therapy. [link.springer.com]
Pathophysiology
Pathophysiology Maffucci syndrome affects the skin and the skeletal systems. Superficial and deep vascular lesions (venous malformations) often protrude as soft nodules or tumors, usually on the distal extremities, but they can appear anywhere. [emedicine.medscape.com]
Pathophysiology This entity was originally described in 1986 by Weiss and Enzinger as a low-grade form of angiosarcoma. It seems that this classification was errant. [clinicaladvisor.com]
Am J Surg Pathol 21:1083–1089 PubMed CrossRef Google Scholar Navarro-Nunez L, Langan SA, Nash GB et al (2013) The physiological and pathophysiological roles of platelet CLEC-2. [link.springer.com]
Prevention
Currently there are no specific methods or preventative techniques for the formation of Spindle Cell Hemangiomas. [dovemed.com]
Is chronic lung disease in low birth weight infants preventable? A survey of eight centers. Página 84 - Tanner JM, Whitehouse RH, Marubini E, Resele LF. The adolescent growth spurt of boys and girls of the Harpenden growth study. [books.google.es]
Low-dose interferon alpha 2b and intralesional and intra-arterial administration of recombinant interleukin 2 have been successful in treating and preventing recurrence of inaccessible or multiple SCH. [5], [6] Radiotherapy should be considered cautiously [ccij-online.org]
Although there may be multiple agents that can be used in conjunction with surgery to prevent recurrence, it is not clear, based on the current evidence, which agents will be best and how they should be utilized. [clinicaladvisor.com]