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Spindle Cell Hemangioma

Spindle Cell Hemangioendothelioma


Presentation

  • A 27-year-old woman presented with multiple nodules closely grouped on her right upper distal extremity. The lesions, dating from childhood, increased slowly in time.[ncbi.nlm.nih.gov]
  • In the present case, gross-total resection of the tumor was possible with no loss of function from baseline.[einstein.pure.elsevier.com]
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]
Collapse
  • Most interestingly, histopathology of the patient revealed multiple vascular spaces; some were dilated and other were collapsed with slit-like openings. Some of the intravascular areas were showing intramural thrombi.[e-ijd.org]
  • The thin walled cavernous spaces are either dilated, partially collapsed or filled with blood, organizing thrombi or phleboliths. [3] SCH generally lacks significant nuclear atypia and show no or low level of mitotic activity and the distribution and[ccij-online.org]
  • Later, lesions show collapsed vascular lumina, fibrosis and presence of abundant hemosiderin.[ispub.com]
Soft Tissue Swelling
  • X-ray of the hand and feet showed soft tissue swelling with calcification without any bony abnormality. We carried out color Doppler study of the patient which revealed dilated veins with phleboleiths.[e-ijd.org]
  • Radiographs showed an ill-defined lytic area in the distal metadiaphysis of right femur without obvious soft tissue swelling or intralesional calcification. The knee joint was normal [Figure 1].[ccij-online.org]
  • Poorly circumscribed diffuse network of vascular structures within a soft tissue Seen in childhood or adolescence presents as deep soft tissue swelling Two patterns : Mixed vessel type lesion resembling intramuscular angioma Capillary predominant type[slideshare.net]
Constitutional Symptom
  • There was no preceding trauma or constitutional symptoms. General and systemic examinations were normal.[ccij-online.org]
Aspiration
  • Fine needle aspiration showed few benign thyroid follicular cells against extensive hemorrhagic background. Right hemithyroidectomy was performed.[indianjcancer.com]
  • Fine Needle Aspiration Cytology. 5 th ed. New Delhi: Elsevier; 2012. p. 15-6. 7. MacLoed CB, Frable WJ. Fine-needle aspiration biopsy of the salivary gland: Problem cases. Diagn Cytopathol 1993;9:216-25. Correspondence Address : Dr.[jcytol.org]
Pharyngitis
  • Rare locations for SCH have included pharyngeal, genital, and even (in one report) within the frontal bone. Intramuscular lesions have been reported as well.[clinicaladvisor.com]
Pelvic Mass
  • Examination of a 39-year-old patient presenting with pelvic pain revealed a remarkable pelvic mass consisting of a 10-cm tumor extending from the cervix to the retroperitoneum. The pathology report demonstrated spindle cell hemangioma.[ncbi.nlm.nih.gov]
Suggestibility
  • Because this is the second case reported from the same institution, it is suggested that SCH is an underdiagnosed lesion.[ncbi.nlm.nih.gov]
  • SCH is usually recognized as a disease originating from blood vessels, but some reports suggest a lymphatic origin.[jstage.jst.go.jp]
  • In recent years it has been suggested that spindle cell hemangioma is probably a vascular malformation or the lesion results from abnormalities of local blood flow.[histopathology-india.net]
Pelvic Pain
  • Examination of a 39-year-old patient presenting with pelvic pain revealed a remarkable pelvic mass consisting of a 10-cm tumor extending from the cervix to the retroperitoneum. The pathology report demonstrated spindle cell hemangioma.[ncbi.nlm.nih.gov]
Recurrent Headache
  • A 41-year-old male was admitted to our hospital with recurrent headaches, nausea, and vomiting persisting for 5 days.[ncbi.nlm.nih.gov]

Treatment

  • 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]
  • Spindle Cell Hemangiomas do not cause any symptoms in most cases, and therefore may not require any treatment.[dovemed.com]
  • Stay at the forefront of your field with updated treatment methods throughout, as well as an increased focus on patients with skin of color.[books.google.de]
  • Treatment consists of wide local excision without adjuvant chemotherapy or radiotherapy. Local recurrence of the lesion is common after incomplete excisions. Age: Spindle cell hemangiomas may occur at any age.[histopathology-india.net]

Prognosis

  • (A large case series that provides support for counseling patients that the overall prognosis for SCH is excellent, while reinforcing its high potential for recurrence) Hisaoka, M, Kouho, H, Aoki, T, Hashimoto, H.[clinicaladvisor.com]
  • Prognosis: • Benign with occasional recurrence.[pedorthpath.com]
  • Though spontaneous remission of SCH is rarely reported, wide excision of the lesion showed excellent prognosis for single or small multiple lesions. [5], [6], [10] However, recurrence may develop even many years after the initial excision. [1], [3], [[ccij-online.org]
  • 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]
  • (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 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]
  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • 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]
  • 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]

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