Benign vascular tumor: Difference between revisions

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| colspan="2" style="background:#7d7d7d; color: #FFFFFF;" + |<small>'''Adapted from International Society for the Study of Vascular Anomalies'''<ref name="urlClassification | International Society for the Study of Vascular Anomalies">{{cite web |url=http://www.issva.org/classification |title=Classification &#124; International Society for the Study of Vascular Anomalies |format= |work= |accessdate=}}</ref></small>  
| colspan="2" style="background:#7d7d7d; color: #FFFFFF;" + |<small>'''Adapted from International Society for the Study of Vascular Anomalies'''<ref name="urlClassification | International Society for the Study of Vascular Anomalies">{{cite web |url=http://www.issva.org/classification |title=Classification &#124; International Society for the Study of Vascular Anomalies |format= |work= |accessdate=}}</ref></small>  
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* Most common [[tumor]] of [[infancy]]. Usually appearing after [[birth]], [[infantile hemangiomas]] undergo a period of proliferation in few weeks after [[birth]] followed by regression and involution in first year of life. Superficial [[lesions]] appear as red or “strawberry” colored nodules, papules, or plaques while deeper [[hemangiomas]] are typically bluish or skin colored. Mixed [[tumors]], involving both [[epidermis]] and deeper structures, may display characteristics of both. They may also be classified as focal, that appear in a specific [[anatomical]] area, and segmental that shows varied pattern of growth following developmental growth regions. Segmental type is often associated with other developmental abnormalities.<ref name="pmid28402073">{{cite journal |vauthors=Smith CJF, Friedlander SF, Guma M, Kavanaugh A, Chambers CD |title=Infantile Hemangiomas: An Updated Review on Risk Factors, Pathogenesis, and Treatment |journal=Birth Defects Res |volume=109 |issue=11 |pages=809–815 |date=July 2017 |pmid=28402073 |pmc=5839165 |doi=10.1002/bdr2.1023 |url=}}</ref><ref name="pmid12472344">{{cite journal |vauthors=Chiller KG, Passaro D, Frieden IJ |title=Hemangiomas of infancy: clinical characteristics, morphologic subtypes, and their relationship to race, ethnicity, and sex |journal=Arch Dermatol |volume=138 |issue=12 |pages=1567–76 |date=December 2002 |pmid=12472344 |doi= |url=}}</ref><ref name="pmid22229118">{{cite journal |vauthors=Greenberger S, Bischoff J |title=Infantile hemangioma-mechanism(s) of drug action on a vascular tumor |journal=Cold Spring Harb Perspect Med |volume=1 |issue=1 |pages=a006460 |date=September 2011 |pmid=22229118 |pmc=3234458 |doi=10.1101/cshperspect.a006460 |url=}}</ref>
* Most common [[tumor]] of [[infancy]]. Usually appearing after [[birth]], infantile hemangiomas undergo a period of proliferation in few weeks after [[birth]] followed by regression and involution in first year of life. Superficial [[lesions]] appear as red or “strawberry” colored nodules, papules, or plaques while deeper [[hemangiomas]] are typically bluish or skin colored. Mixed [[tumors]], involving both [[epidermis]] and deeper structures, may display characteristics of both. They may also be classified as focal, that appear in a specific [[anatomical]] area, and segmental that shows varied pattern of growth following developmental growth regions. Segmental type is often associated with other developmental abnormalities.<ref name="pmid28402073">{{cite journal |vauthors=Smith CJF, Friedlander SF, Guma M, Kavanaugh A, Chambers CD |title=Infantile Hemangiomas: An Updated Review on Risk Factors, Pathogenesis, and Treatment |journal=Birth Defects Res |volume=109 |issue=11 |pages=809–815 |date=July 2017 |pmid=28402073 |pmc=5839165 |doi=10.1002/bdr2.1023 |url=}}</ref><ref name="pmid12472344">{{cite journal |vauthors=Chiller KG, Passaro D, Frieden IJ |title=Hemangiomas of infancy: clinical characteristics, morphologic subtypes, and their relationship to race, ethnicity, and sex |journal=Arch Dermatol |volume=138 |issue=12 |pages=1567–76 |date=December 2002 |pmid=12472344 |doi= |url=}}</ref><ref name="pmid22229118">{{cite journal |vauthors=Greenberger S, Bischoff J |title=Infantile hemangioma-mechanism(s) of drug action on a vascular tumor |journal=Cold Spring Harb Perspect Med |volume=1 |issue=1 |pages=a006460 |date=September 2011 |pmid=22229118 |pmc=3234458 |doi=10.1101/cshperspect.a006460 |url=}}</ref>
* Rarely, [[infantile hemangioms]] can cause life-threatening [[complications]] such as [[congestive cardiac failure]], respiratory difficulty and [[respiratory]] compromise, and loss of [[vision]]. There may also be long-term sequela including permanent disfigurement and [[scarring]]. If [[lesions]] are multiple, there is an increased risk of visceral involvement. There may be an association with certain syndromes such as [[PHACE syndrome]].<ref name="pmid28402073">{{cite journal |vauthors=Smith CJF, Friedlander SF, Guma M, Kavanaugh A, Chambers CD |title=Infantile Hemangiomas: An Updated Review on Risk Factors, Pathogenesis, and Treatment |journal=Birth Defects Res |volume=109 |issue=11 |pages=809–815 |date=July 2017 |pmid=28402073 |pmc=5839165 |doi=10.1002/bdr2.1023 |url=}}</ref><ref name="pmid29924216">{{cite journal |vauthors=Rotter A, Samorano LP, Rivitti-Machado MC, Oliveira ZNP, Gontijo B |title=PHACE syndrome: clinical manifestations, diagnostic criteria, and management |journal=An Bras Dermatol |volume=93 |issue=3 |pages=405–411 |date=June 2018 |pmid=29924216 |pmc=6001075 |doi=10.1590/abd1806-4841.20187693 |url=}}</ref>
* Rarely, [[infantile hemangioms]] can cause life-threatening [[complications]] such as [[congestive cardiac failure]], respiratory difficulty and [[respiratory]] compromise, and loss of [[vision]]. There may also be long-term sequela including permanent disfigurement and [[scarring]]. If [[lesions]] are multiple, there is an increased risk of visceral involvement. There may be an association with certain syndromes such as PHACE syndrome.<ref name="pmid28402073">{{cite journal |vauthors=Smith CJF, Friedlander SF, Guma M, Kavanaugh A, Chambers CD |title=Infantile Hemangiomas: An Updated Review on Risk Factors, Pathogenesis, and Treatment |journal=Birth Defects Res |volume=109 |issue=11 |pages=809–815 |date=July 2017 |pmid=28402073 |pmc=5839165 |doi=10.1002/bdr2.1023 |url=}}</ref><ref name="pmid29924216">{{cite journal |vauthors=Rotter A, Samorano LP, Rivitti-Machado MC, Oliveira ZNP, Gontijo B |title=PHACE syndrome: clinical manifestations, diagnostic criteria, and management |journal=An Bras Dermatol |volume=93 |issue=3 |pages=405–411 |date=June 2018 |pmid=29924216 |pmc=6001075 |doi=10.1590/abd1806-4841.20187693 |url=}}</ref>
* Some studies have indicated autosomal-dominant and maternal patterns of [[inheritance]]. Some studies suggest that environmental factors play the key role. Some [[risk factors]] that have been identified in association with [[infantile hemangioma]] include [[female]] [[gender]], [[preterm birth]], low weight at [[birth]], increasing maternal age at [[birth]], [[placenta previa]], [[pre-eclampsia]], [[progesterone]] use by mother, and Caucasian race.<ref name="pmid27940781">{{cite journal |vauthors=Castrén E, Salminen P, Vikkula M, Pitkäranta A, Klockars T |title=Inheritance Patterns of Infantile Hemangioma |journal=Pediatrics |volume=138 |issue=5 |pages= |date=November 2016 |pmid=27940781 |doi=10.1542/peds.2016-1623 |url=}}</ref><ref name="pmid28402073">{{cite journal |vauthors=Smith CJF, Friedlander SF, Guma M, Kavanaugh A, Chambers CD |title=Infantile Hemangiomas: An Updated Review on Risk Factors, Pathogenesis, and Treatment |journal=Birth Defects Res |volume=109 |issue=11 |pages=809–815 |date=July 2017 |pmid=28402073 |pmc=5839165 |doi=10.1002/bdr2.1023 |url=}}</ref><ref name="pmid17307549">{{cite journal |vauthors=Haggstrom AN, Drolet BA, Baselga E, Chamlin SL, Garzon MC, Horii KA, Lucky AW, Mancini AJ, Metry DW, Newell B, Nopper AJ, Frieden IJ |title=Prospective study of infantile hemangiomas: demographic, prenatal, and perinatal characteristics |journal=J. Pediatr. |volume=150 |issue=3 |pages=291–4 |date=March 2007 |pmid=17307549 |doi=10.1016/j.jpeds.2006.12.003 |url=}}</ref><ref name="pmid18940356">{{cite journal |vauthors=Drolet BA, Swanson EA, Frieden IJ |title=Infantile hemangiomas: an emerging health issue linked to an increased rate of low birth weight infants |journal=J. Pediatr. |volume=153 |issue=5 |pages=712–5, 715.e1 |date=November 2008 |pmid=18940356 |doi=10.1016/j.jpeds.2008.05.043 |url=}}</ref>
* Some studies have indicated [[Autosomal dominant|autosomal-dominant]] and maternal patterns of [[inheritance]]. Some studies suggest that environmental factors play the key role. Some [[risk factors]] that have been identified in association with [[infantile hemangioma]] include [[female]] [[gender]], [[preterm birth]], low weight at [[birth]], increasing maternal age at [[birth]], [[placenta previa]], [[pre-eclampsia]], [[progesterone]] use by mother, and Caucasian race.<ref name="pmid27940781">{{cite journal |vauthors=Castrén E, Salminen P, Vikkula M, Pitkäranta A, Klockars T |title=Inheritance Patterns of Infantile Hemangioma |journal=Pediatrics |volume=138 |issue=5 |pages= |date=November 2016 |pmid=27940781 |doi=10.1542/peds.2016-1623 |url=}}</ref><ref name="pmid28402073">{{cite journal |vauthors=Smith CJF, Friedlander SF, Guma M, Kavanaugh A, Chambers CD |title=Infantile Hemangiomas: An Updated Review on Risk Factors, Pathogenesis, and Treatment |journal=Birth Defects Res |volume=109 |issue=11 |pages=809–815 |date=July 2017 |pmid=28402073 |pmc=5839165 |doi=10.1002/bdr2.1023 |url=}}</ref><ref name="pmid17307549">{{cite journal |vauthors=Haggstrom AN, Drolet BA, Baselga E, Chamlin SL, Garzon MC, Horii KA, Lucky AW, Mancini AJ, Metry DW, Newell B, Nopper AJ, Frieden IJ |title=Prospective study of infantile hemangiomas: demographic, prenatal, and perinatal characteristics |journal=J. Pediatr. |volume=150 |issue=3 |pages=291–4 |date=March 2007 |pmid=17307549 |doi=10.1016/j.jpeds.2006.12.003 |url=}}</ref><ref name="pmid18940356">{{cite journal |vauthors=Drolet BA, Swanson EA, Frieden IJ |title=Infantile hemangiomas: an emerging health issue linked to an increased rate of low birth weight infants |journal=J. Pediatr. |volume=153 |issue=5 |pages=712–5, 715.e1 |date=November 2008 |pmid=18940356 |doi=10.1016/j.jpeds.2008.05.043 |url=}}</ref>
* The [[diagnosis]] is made clinically and a thorough investigation should be carried out for visceral [[hemangiomas]] and other associative abnormalities if suspicion arises. Majority of these [[lesions]] do not require any treatment but treatment is indicated if there is risk for [[complications]] such as visual or respiratory involvement. Elective treatment is also offered to prevent disfigurement or [[scarring]]. Recently there have been an increased usage of oral [[beta-blockers]] such as [[timolol]] over systemic [[glucocorticoids]] because of higher efficacy.  [[Vincristine]] and [[interferon alpha]] have been used in some high risk [[hemangiomas]] but carry the risk of severe [[complications]]. Visceral hemangioms may require [[embolization]] or [[surgery]] if they do not respond to systemic therapy. [[Laser]] therapy especially PDL is another modality used in cases of [[hemangiomas]] unresponsive to [[medication]].<ref name="pmid28402073">{{cite journal |vauthors=Smith CJF, Friedlander SF, Guma M, Kavanaugh A, Chambers CD |title=Infantile Hemangiomas: An Updated Review on Risk Factors, Pathogenesis, and Treatment |journal=Birth Defects Res |volume=109 |issue=11 |pages=809–815 |date=July 2017 |pmid=28402073 |pmc=5839165 |doi=10.1002/bdr2.1023 |url=}}</ref><ref name="pmid26416928">{{cite journal |vauthors=Darrow DH, Greene AK, Mancini AJ, Nopper AJ |title=Diagnosis and Management of Infantile Hemangioma: Executive Summary |journal=Pediatrics |volume=136 |issue=4 |pages=786–91 |date=October 2015 |pmid=26416928 |doi=10.1542/peds.2015-2482 |url=}}</ref><ref name="pmid14871317">{{cite journal |vauthors=Ceisler EJ, Santos L, Blei F |title=Periocular hemangiomas: what every physician should know |journal=Pediatr Dermatol |volume=21 |issue=1 |pages=1–9 |date=2004 |pmid=14871317 |doi= |url=}}</ref><ref name="pmid26859502">{{cite journal |vauthors=Tal R, Dotan M, Lorber A |title=Approach to haemangiomatosis causing congestive heart failure |journal=Acta Paediatr. |volume=105 |issue=6 |pages=600–4 |date=June 2016 |pmid=26859502 |doi=10.1111/apa.13359 |url=}}</ref><ref name="pmid21788220">{{cite journal |vauthors=Hogeling M, Adams S, Wargon O |title=A randomized controlled trial of propranolol for infantile hemangiomas |journal=Pediatrics |volume=128 |issue=2 |pages=e259–66 |date=August 2011 |pmid=21788220 |doi=10.1542/peds.2010-0029 |url=}}</ref><ref name="pmid11559219">{{cite journal |vauthors=Bennett ML, Fleischer AB, Chamlin SL, Frieden IJ |title=Oral corticosteroid use is effective for cutaneous hemangiomas: an evidence-based evaluation |journal=Arch Dermatol |volume=137 |issue=9 |pages=1208–13 |date=September 2001 |pmid=11559219 |doi= |url=}}</ref><ref name="pmid12102167">{{cite journal |vauthors=Perez J, Pardo J, Gomez C |title=Vincristine--an effective treatment of corticoid-resistant life-threatening infantile hemangiomas |journal=Acta Oncol |volume=41 |issue=2 |pages=197–9 |date=2002 |pmid=12102167 |doi= |url=}}</ref><ref name="pmid20936416">{{cite journal |vauthors=Schiestl C, Neuhaus K, Zoller S, Subotic U, Forster-Kuebler I, Michels R, Balmer C, Weibel L |title=Efficacy and safety of propranolol as first-line treatment for infantile hemangiomas |journal=Eur. J. Pediatr. |volume=170 |issue=4 |pages=493–501 |date=April 2011 |pmid=20936416 |doi=10.1007/s00431-010-1324-2 |url=}}</ref><ref name="pmid26711436">{{cite journal |vauthors=Chinnadurai S, Sathe NA, Surawicz T |title=Laser treatment of infantile hemangioma: A systematic review |journal=Lasers Surg Med |volume=48 |issue=3 |pages=221–33 |date=March 2016 |pmid=26711436 |doi=10.1002/lsm.22455 |url=}}</ref>
* The [[diagnosis]] is made clinically and a thorough investigation should be carried out for visceral [[hemangiomas]] and other associative abnormalities if suspicion arises. Majority of these [[lesions]] do not require any treatment but treatment is indicated if there is risk for [[complications]] such as visual or respiratory involvement. Elective treatment is also offered to prevent disfigurement or [[scarring]]. Recently there have been an increased usage of oral [[beta-blockers]] such as [[timolol]] over systemic [[glucocorticoids]] because of higher efficacy.  [[Vincristine]] and [[interferon alpha]] have been used in some high risk [[hemangiomas]] but carry the risk of severe [[complications]]. Visceral hemangioms may require [[embolization]] or [[surgery]] if they do not respond to systemic therapy. [[Laser]] therapy especially PDL is another modality used in cases of [[hemangiomas]] unresponsive to [[medication]].<ref name="pmid28402073">{{cite journal |vauthors=Smith CJF, Friedlander SF, Guma M, Kavanaugh A, Chambers CD |title=Infantile Hemangiomas: An Updated Review on Risk Factors, Pathogenesis, and Treatment |journal=Birth Defects Res |volume=109 |issue=11 |pages=809–815 |date=July 2017 |pmid=28402073 |pmc=5839165 |doi=10.1002/bdr2.1023 |url=}}</ref><ref name="pmid26416928">{{cite journal |vauthors=Darrow DH, Greene AK, Mancini AJ, Nopper AJ |title=Diagnosis and Management of Infantile Hemangioma: Executive Summary |journal=Pediatrics |volume=136 |issue=4 |pages=786–91 |date=October 2015 |pmid=26416928 |doi=10.1542/peds.2015-2482 |url=}}</ref><ref name="pmid14871317">{{cite journal |vauthors=Ceisler EJ, Santos L, Blei F |title=Periocular hemangiomas: what every physician should know |journal=Pediatr Dermatol |volume=21 |issue=1 |pages=1–9 |date=2004 |pmid=14871317 |doi= |url=}}</ref><ref name="pmid26859502">{{cite journal |vauthors=Tal R, Dotan M, Lorber A |title=Approach to haemangiomatosis causing congestive heart failure |journal=Acta Paediatr. |volume=105 |issue=6 |pages=600–4 |date=June 2016 |pmid=26859502 |doi=10.1111/apa.13359 |url=}}</ref><ref name="pmid21788220">{{cite journal |vauthors=Hogeling M, Adams S, Wargon O |title=A randomized controlled trial of propranolol for infantile hemangiomas |journal=Pediatrics |volume=128 |issue=2 |pages=e259–66 |date=August 2011 |pmid=21788220 |doi=10.1542/peds.2010-0029 |url=}}</ref><ref name="pmid11559219">{{cite journal |vauthors=Bennett ML, Fleischer AB, Chamlin SL, Frieden IJ |title=Oral corticosteroid use is effective for cutaneous hemangiomas: an evidence-based evaluation |journal=Arch Dermatol |volume=137 |issue=9 |pages=1208–13 |date=September 2001 |pmid=11559219 |doi= |url=}}</ref><ref name="pmid12102167">{{cite journal |vauthors=Perez J, Pardo J, Gomez C |title=Vincristine--an effective treatment of corticoid-resistant life-threatening infantile hemangiomas |journal=Acta Oncol |volume=41 |issue=2 |pages=197–9 |date=2002 |pmid=12102167 |doi= |url=}}</ref><ref name="pmid20936416">{{cite journal |vauthors=Schiestl C, Neuhaus K, Zoller S, Subotic U, Forster-Kuebler I, Michels R, Balmer C, Weibel L |title=Efficacy and safety of propranolol as first-line treatment for infantile hemangiomas |journal=Eur. J. Pediatr. |volume=170 |issue=4 |pages=493–501 |date=April 2011 |pmid=20936416 |doi=10.1007/s00431-010-1324-2 |url=}}</ref><ref name="pmid26711436">{{cite journal |vauthors=Chinnadurai S, Sathe NA, Surawicz T |title=Laser treatment of infantile hemangioma: A systematic review |journal=Lasers Surg Med |volume=48 |issue=3 |pages=221–33 |date=March 2016 |pmid=26711436 |doi=10.1002/lsm.22455 |url=}}</ref>


===Congenital hemangioma===
===Congenital hemangioma===
* Rare [[tumor]] that arises in utero and presents as fully developed [[lesion]] at [[birth]]. Following [[birth]] they can regress completely, partially or not at all. So they can be classified as Rapidly involuting (RICH), Non-involuting (NICH), Partially involuting (PICH).<ref name="urlwww.issva.org">{{cite web |url=http://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf |title=www.issva.org |format= |work= |accessdate=}}</ref><ref name="pmid27058448">{{cite journal |vauthors=Ayturk UM, Couto JA, Hann S, Mulliken JB, Williams KL, Huang AY, Fishman SJ, Boyd TK, Kozakewich HP, Bischoff J, Greene AK, Warman ML |title=Somatic Activating Mutations in GNAQ and GNA11 Are Associated with Congenital Hemangioma |journal=Am. J. Hum. Genet. |volume=98 |issue=4 |pages=789–95 |date=April 2016 |pmid=27058448 |pmc=4833432 |doi=10.1016/j.ajhg.2016.03.009 |url=}}</ref>
* Rare [[tumor]] that arises in utero and presents as fully developed [[lesion]] at [[birth]]. Following [[birth]] they can regress completely, partially or not at all. So they can be classified as rapidly involuting (RICH), non-involuting (NICH), partially involuting (PICH).<ref name="urlwww.issva.org">{{cite web |url=http://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf |title=www.issva.org |format= |work= |accessdate=}}</ref><ref name="pmid27058448">{{cite journal |vauthors=Ayturk UM, Couto JA, Hann S, Mulliken JB, Williams KL, Huang AY, Fishman SJ, Boyd TK, Kozakewich HP, Bischoff J, Greene AK, Warman ML |title=Somatic Activating Mutations in GNAQ and GNA11 Are Associated with Congenital Hemangioma |journal=Am. J. Hum. Genet. |volume=98 |issue=4 |pages=789–95 |date=April 2016 |pmid=27058448 |pmc=4833432 |doi=10.1016/j.ajhg.2016.03.009 |url=}}</ref>
*# '''Rapidly involuting (RICH)'''
*# '''Rapidly involuting (RICH)'''
*#* This fast flow [[tumor]] can be detected in utero and appears as raised gray single [[lesions]] with dilated [[veins]], [[telangiectasias]] and a halo at birth. This tumor may be complicated by [[thrombocytopenia]] and [[congestive cardiac failure]] due to its high-flow nature. [[Tumor]] typically regresses spontaneously in 1 to 2 years of life. Sometimes it can occur in [liver]] where it follows the same pattern of involution as that of skin.<ref name="pmid27058448">{{cite journal |vauthors=Ayturk UM, Couto JA, Hann S, Mulliken JB, Williams KL, Huang AY, Fishman SJ, Boyd TK, Kozakewich HP, Bischoff J, Greene AK, Warman ML |title=Somatic Activating Mutations in GNAQ and GNA11 Are Associated with Congenital Hemangioma |journal=Am. J. Hum. Genet. |volume=98 |issue=4 |pages=789–95 |date=April 2016 |pmid=27058448 |pmc=4833432 |doi=10.1016/j.ajhg.2016.03.009 |url=}}</ref><ref name="pmid15018449">{{cite journal |vauthors=Berenguer B, Mulliken JB, Enjolras O, Boon LM, Wassef M, Josset P, Burrows PE, Perez-Atayde AR, Kozakewich HP |title=Rapidly involuting congenital hemangioma: clinical and histopathologic features |journal=Pediatr. Dev. Pathol. |volume=6 |issue=6 |pages=495–510 |date=2003 |pmid=15018449 |doi= |url=}}</ref>
*#* This fast flow [[tumor]] can be detected in utero and appears as raised gray single [[lesions]] with dilated [[veins]], [[telangiectasias]] and a halo at birth.
*#* This tumor may be complicated by [[thrombocytopenia]] and [[congestive cardiac failure]] due to its high-flow nature.
*#* [[Tumor]] typically regresses spontaneously in 1 to 2 years of life. Sometimes it can occur in [liver]] where it follows the same pattern of involution as that of skin.<ref name="pmid27058448">{{cite journal |vauthors=Ayturk UM, Couto JA, Hann S, Mulliken JB, Williams KL, Huang AY, Fishman SJ, Boyd TK, Kozakewich HP, Bischoff J, Greene AK, Warman ML |title=Somatic Activating Mutations in GNAQ and GNA11 Are Associated with Congenital Hemangioma |journal=Am. J. Hum. Genet. |volume=98 |issue=4 |pages=789–95 |date=April 2016 |pmid=27058448 |pmc=4833432 |doi=10.1016/j.ajhg.2016.03.009 |url=}}</ref><ref name="pmid15018449">{{cite journal |vauthors=Berenguer B, Mulliken JB, Enjolras O, Boon LM, Wassef M, Josset P, Burrows PE, Perez-Atayde AR, Kozakewich HP |title=Rapidly involuting congenital hemangioma: clinical and histopathologic features |journal=Pediatr. Dev. Pathol. |volume=6 |issue=6 |pages=495–510 |date=2003 |pmid=15018449 |doi= |url=}}</ref>
*# '''Non-involuting (NICH)'''
*# '''Non-involuting (NICH)'''
*#* Fast flow [[tumor]] that presents as well defined, [[plaque]] like [[lesion]] with pink to purple color, [[telangiectasias]] and pale borders. Typically remains stable but there have been some reports of growth and expansion.<ref name="pmid27058448">{{cite journal |vauthors=Ayturk UM, Couto JA, Hann S, Mulliken JB, Williams KL, Huang AY, Fishman SJ, Boyd TK, Kozakewich HP, Bischoff J, Greene AK, Warman ML |title=Somatic Activating Mutations in GNAQ and GNA11 Are Associated with Congenital Hemangioma |journal=Am. J. Hum. Genet. |volume=98 |issue=4 |pages=789–95 |date=April 2016 |pmid=27058448 |pmc=4833432 |doi=10.1016/j.ajhg.2016.03.009 |url=}}</ref>
*#* Fast flow [[tumor]] that presents as well defined, [[plaque]] like [[lesion]] with pink to purple color, [[telangiectasias]] and pale borders.
*#* Typically remains stable but there have been some reports of growth and expansion.<ref name="pmid27058448">{{cite journal |vauthors=Ayturk UM, Couto JA, Hann S, Mulliken JB, Williams KL, Huang AY, Fishman SJ, Boyd TK, Kozakewich HP, Bischoff J, Greene AK, Warman ML |title=Somatic Activating Mutations in GNAQ and GNA11 Are Associated with Congenital Hemangioma |journal=Am. J. Hum. Genet. |volume=98 |issue=4 |pages=789–95 |date=April 2016 |pmid=27058448 |pmc=4833432 |doi=10.1016/j.ajhg.2016.03.009 |url=}}</ref>
*# '''Partially involuting (PICH)'''
*# '''Partially involuting (PICH)'''
*#* These lesions start involution as RICH but become stable over time and persist as NICH.<ref name="pmid24176519">{{cite journal |vauthors=Nasseri E, Piram M, McCuaig CC, Kokta V, Dubois J, Powell J |title=Partially involuting congenital hemangiomas: a report of 8 cases and review of the literature |journal=J. Am. Acad. Dermatol. |volume=70 |issue=1 |pages=75–9 |date=January 2014 |pmid=24176519 |doi=10.1016/j.jaad.2013.09.018 |url=}}</ref>
*#* These lesions start involution as RICH but become stable over time and persist as NICH.<ref name="pmid24176519">{{cite journal |vauthors=Nasseri E, Piram M, McCuaig CC, Kokta V, Dubois J, Powell J |title=Partially involuting congenital hemangiomas: a report of 8 cases and review of the literature |journal=J. Am. Acad. Dermatol. |volume=70 |issue=1 |pages=75–9 |date=January 2014 |pmid=24176519 |doi=10.1016/j.jaad.2013.09.018 |url=}}</ref>

Revision as of 19:00, 22 October 2018


For information about classification of vascular tumors, click here.

Vascular Tumor

Home

Overview

Classification

Benign Vascular tumor
Locally aggressive or borderline vascular tumors
Malignant vascular tumors

Benign Vascular Tumor Home Page

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hannan Javed, M.D.[2]


Overview

Benign vascular tumors, are benign growths formed from blood vessels; such as, hemangioma, hemangioendothelioma, Kaposi sarcoma. They exhibit a wide range of clinical manifestations, and may occur as isolated lesions or may occur as manifestation of multi-system syndromes and diseases. Their diagnosis and management depends on their clinical manifestations and coexistent anomalies. International Society for the Study of Vascular Anomalies (ISSVA) has classified these lesions into benign vascular tumors and related lesions.

Benign Vascular Tumors

Infantile hemangioma / Hemangioma of infancy

Pattern Different types
  • Focal
  • Multifocal
  • Segmental
  • Indeterminate
  • Superficial
  • Deep
  • Mixed (superficial + deep)
  • Reticular / abortive / minimal growth
  • Others
Adapted from International Society for the Study of Vascular Anomalies[1]
Association with other lesions
PHACE association /

syndrome

Posterior fossa malformations, Hemangioma, Arterial

anomalies, Cardiovascular anomalies, Eye anomalies ,

sternal clefting and ⁄ or supraumbilical raphe

LUMBAR (SACRAL,

PELVIS) association /

syndrome

Lower body hemangioma, Urogenital anomalies,

Ulceration, Myelopathy, Bony deformities, Anorectal

malformations, Arterial anomalies, and Renal anomalies

Adapted from International Society for the Study of Vascular Anomalies[1]

Congenital hemangioma

  • Rare tumor that arises in utero and presents as fully developed lesion at birth. Following birth they can regress completely, partially or not at all. So they can be classified as rapidly involuting (RICH), non-involuting (NICH), partially involuting (PICH).[17][18]
    1. Rapidly involuting (RICH)
    2. Non-involuting (NICH)
      • Fast flow tumor that presents as well defined, plaque like lesion with pink to purple color, telangiectasias and pale borders.
      • Typically remains stable but there have been some reports of growth and expansion.[18]
    3. Partially involuting (PICH)
      • These lesions start involution as RICH but become stable over time and persist as NICH.[20]
  • Somatic mutations in GNAQ/GNA11 are thought to cause the congenital hemangioma. GNAQ and its paralogue GNA11 function in intracellular signaling pathways as Gq alpha subunit.[18][21]
  • Diagnosis is usually clinical but imaging techniques such as MRI, CT scan, contrast-enhanced ultrasound and later biopsy can be considered if required. Surgical excision should be considered in case of complications, NICH and PICH.[22][23]

Tufted angioma

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Spindle-cell hemangioma

  • Rare benign tumor that manifests as solitary or multiple nodules confined to dermis and subcutaneous tissues in almost all of the cases. Histopathologically it appears as solid areas that are cellular and consist of spindle cells seen attached to vessel walls, and cavernous spaces that can be thrombosed. Size may increase over time and patient usually complains of swelling and pain. The nodules or masses can be mobile and elastic or can be firm and immobile.[36][37][38]
  • Somatic mutations in IDH1 and IDH2 have been found to be present in 70% of spindle-cell hemangiomas. IDH1 and IDH2 are important enzymes in cell energy cycles (α-ketoglutarate and NADPH generation).[39][40]
  • Diagnosis often requires biopsy and imaging studies such as MRI to ascertain the extent of the tumor. Local excision is the treatment modality of choice with excellent prognosis in majority of the cases although recurrence is very common.[36][37][38][41][42]

Epithelioid hemangioma

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Pyogenic granuloma

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Hobnail hemangioma

  • Benign tumor that typically presents as solitary growth with often, but not always, tagetoid appearance of a central papule and peripheral brown ring that may or may not disappear over time. Characteristic histopathological feature include plump endothelial cells in superficial dermis that line ectatic and irregular vessels, and project into lumina like hobnails. Deeper dermis shows vessels dissecting collagen fibers. Majority of the lesions are fund on trunk and extremities with head and neck, and oral cavity as uncommon locations. Patient may present with pain, or an asymptomatic growing lesion.[63][64][65]
  • Etiology is not well understood but trauma may play a key role in pathogenesis. Some studies have found congenital etiology in some lesions.[63][64][66][67]
  • Diagnosis is based on clinical features and histopathological studies. Treatment is usually by excision. Other modalities of treatment include intermittent triamcinolone intralesional injections and pulsed dye laser treatment.[63][64]

Microvenular hemangioma

  • Rare lesion that most often manifests as single asymptomatic nodule, plaque or papule with color varying from red to bluish-red. Majority of the lesions are located on trunk and limbs. Histologically, the tumor consists of irregular and branching venous structures with inconspicuous lumina. Endothelial cells display absence of atypia and mitotic figures. Some lesions may be painful and/or tender.[68][69][70][71]
  • Etiology and pathogenesis have not been well-understood but a recent study may associate progesterone with microvenular hemangioma.[72]
  • Diagnosis requires biopsy because of rarity of this tumor. Treatment is through surgical excision.[68][69][70][71]

Anastomosing hemangioma

Glomeruloid hemangioma

  • Characterized by red blood cells filled clumps of capillaries inside dilated vascular spaces. These collections of capillaries, lined by swollen endothelial cells, resemble renal glomeruli and stain positive for periodic acid-Schiff (PAS)-positive, diastase-resistant eosinophilic globules. Clinical presentation varies and are not discernible from other cutaneous lesions. Majority of the lesions manifest as multiple, asymptomatic pauples or nodules..[81][82]
  • Glomeruloid hemangioma is associated with POEMS syndrome in majority of the cases and rarely with Castleman's disease. Very few isolated cases of glomeruloid hemangioma have been reported.[81][82]
    • POEMS stands for peripheral neuropathy (P), organomegaly (O), endocrinopathy (E) monoclonal plasma-cells proliferative disorder (M) and skin changes (S) although diagnosis does not require presence of all of these symptoms. Other manifestations of POEMS syndrome may include sclerotic bone lesions, papilledema, edema, ascites, effusions, pulmonary hypertension, Castleman’ disease (CD), thrombocytosis and erythrocytosis, and increased serum VEGF.[83]
    • Castleman's disease is characterized as lymphoproliferative disorder with inflammatory response involving multiple systems. Clinical presentation ranges from asymptomatic lymphadenopathy to severe systemic manifestations such as weight loss, fever and organomegaly.[84]
  • Etiology is not well-understood but some theories suggest role of vascular endothelial growth factor (VEGF), increased estrogen levels, human herpesvirus-8 and increased cytokines in its pathogenesis.[81][82]
  • Diagnosis relies on characteristic histology. Patients who present with glomeruloid hemangioma should undergo evaluation for POEMS syndrome and should be kept under follow-up because these lesions can precede full-blown POEMS syndrome in some cases.[81][82]

Papillary hemangioma

Intravascular papillary endothelial hyperplasia

  • Also called as Masson's tumor, this benign lesions is characterized by presence of intravascular papillary structures that are enveloped by proliferating endothelial cells. It is considered to be a reactive lesion associated with an organizing thrombus. Clinically it manifests as solitary painless mass in head-neck and the extremities especially the hand, that may grow rapidly in size and become painful and/or tender. Some lesions have been found intra-abdominallly such as in the liver that can bleed and present with anemia.[88][89][90][91]
  • This lesions appears to be associated with vascular trauma, and thrombus that may lead to chronic irritation and increased levels of fibroblast growth factor (FGF), hypoxia-inducible factor-1 (HIF-1α), and vascular endothelial growth factor (VEGF) stimulating endothelial cells proliferation.[89][90][92]
  • Histopathological studies are generally required for diagnosis and may also require immunohistochemical confirmation. Treatment is surgery with uncommon recurrence.[92][93]

Cutaneous epithelioid angiomatous nodule

Acquired elastotic hemangioma

Littoral cell hemangioma of the spleen

Related lesions

Eccrine angiomatous hamartoma

Reactive angioendotheliomatosis

Bacillary angiomatosis

  • Bacillary angiomatosis is characterized by the proliferation of blood vessels, resulting in them forming tumor-like masses in the skin and other organs. Symptoms vary depending on which parts of the body are affected; for example, those whose livers are affected may have an enlarged liver and fever, while those with osseous BA will experience intense pain in the affected area. These lesions may take several forms such as papules or nodules and plaque.
  • Bacillary angiomatosis (BA) is a bacterial infection caused by either Bartonella henselae or Bartonella quintana.
  • BA responds dramatically to several antibiotics. Usually, erythromycin will cause the skin lesions to gradually fade away in the next four weeks, resulting in complete recovery. Doxycycline may also be used. However, if the infection does not respond to either of these, the medication is usually changed to tetracycline.

For more information on bacillary angiomatosis, click here.

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