[考生加油]Dermatological Recall: Chapter 126. Vascular Tumors 血管腫瘤

Fitzpatrick's Dermatology in General MedicineChapter 126. Vascular Tumors

鄭煜彬

如果要儘快掌握本章重點，不妨看一下筆者仿照Surgical Recall寫的Dermatological Recall。

• 建議可以先遮住右邊，想一下答案大概是什麼。
• (5)表示答案有五項。(訣)表示有筆者自編的口訣。

Vascular Tumors: Introduction
1.      Vascular malformations與vascular tumors的差異為何？	Vascular malformations: errors of morphogenesis. Vascular tumors: cellular proliferation.
2.      Hemangioma有哪些特徵時要特別注意、處理、介入? (4，訣)	(Mn)55CMs >5公分(facial hemangioma>5公分為PHASE syndrome) >5個(容易有extracutaneous hemangiomas) Congenital hemangioma(不是IH) Midline/ Segmental (central face, periocular, beard, lumbosacral, perineal)
Infantile Hemangiomas(IH)
1.      Infants最常見的腫瘤為何？	Infantile hemangiomas(4% infants)
2.      IH好發族群特徵為？(5)	Females, 早產(< 2,500 g), Caucasian, 多胎產婦, 高齡產婦(>30y/o)
3.      那些hemangioma會引起Kasabach-Merritt phenomanon	kaposiform hemangioendothelioma  & tufted angioma，IH不會
4.      哪種分子和IH的形成最有關？ 哪種人類組織也有這種分子？	GLUT1 (a glucose transporter protein) Human placenta
5.      IH在剛出生時的premonitory mark為?	An area of pallor, telangiectasias, or duskiness
6.      Superficial/deep IH的病程分別為何？	Onset Proliferative phase (快) Involution phase(慢) Superficial 1st month 1st-5th month months to years Deep, large, segmental 2nd month(晚superficial一個月) months to years months to years
7.      IH的歷程大約為其幾年?	7–10 years
8.      IH消退後的皮膚變化為?	Normal skin or telangiectasias, atrophy, fibrofatty residuum, or scarring(可能拿來考實物題)
9.      IH有哪兩種分類方式?	1.      Superficial(strawberry red) , deep(blue/skin-color), mixed 2.      Localized, segmental, or indeterminate.
10.  IH的atypical presentations有哪2種型式?分別有何特徵?	Deep IH(blue/skin-color, flat) Persistent premonitory IH: little proliferation, lower body, fine telangiectasia
Medical and Extracutaneous Risks
1.      何謂PHACE(face)?	(Mn) PHACEs A neurocutaneous syndrome: Posterior fossa brain malformations(Dandy–Walker→Moyamoya, ischemia, & stroke) Segmental cervicofacial Hemangioma Arterial anomalies(head & neck) Cardiac defects & Coarctation of the aorta Eye anomalies Sternal defects(sternal clefting/supraumbilical raphe)
2.      PHACE應做什麼檢查?	MRI, MRA, ophthalmologic examination, ECG, NE
3.      Periocular hemangioma有何risk?	Amblyopia(弱視), visual loss…各式眼睛問題。
4.      Beard Area Hemangiomas有何risk?( preauricular, mandibular, chin, and neck skin)	Symptommatic airway disease(stridor, URI, RDS …) Parotid gland involvement
5.      Lumbosacral Hemangiomas有何risk?	Spinal, bony, and genitourinary anomalie (Mn)SACRAL syn.: Spinal dysraphism, Anogenital anomalies, Cutaneous anomales, Renal and urologic anomalies, Angioma of Lumbosacral localization
6.      Perineal Hemangiomas有何risk?	Spinal, bony, and genitourinary anomalie (Mn)PELVIS syndrome: Perineal hemangioma, External genitalia malformations, Lipomyelomeningocele, Vesicorenal abnormalities,Imperforate anus, & Skin tag
7.      Multifocal hemangiomas有何risk?	Extracutaneous hemangiomas (Hepatic最常見)
8.      Hepatic Hemangioma有哪三種分類?	Christison-Lagay 分類(多、廣、大) Multifocal, diffuse, large solitary
9.      三種Hepatic Hemangioma有何risk?請簡述如何治療	大多無症狀 Multifocal: high-output CHF (Embolization) Diffuse: hypothyroidism(TH replacement), abdominal compartment syndrome(移植) Large solitary: arteriovenous shunting
10.  Hepatic hemangioma較像哪種hemangioma? (true IH, RICH, or NICH?)	Rapidly involuting congenital hemangioma(RICH)
Laboratory Tests
1.      Hemangioma出現何種狀況時，分別需做哪些lab檢查?	Massive hepatic hemangiomas, PHACE: T3, TSH, T4(Hemangioma會消耗T3/T4) Kasabach-Merritt phenomenon: platelet
Complications: ulceration, secondary infection, hypothyroidism, internal organ involvement
1.      最常見的IH併發症為何?如何治療? 一般多久會好?	1.      Ulceration 2.      Wound care, barrier protection, pain control(acetaminophen, codeine, lidocaine) Pulsed dye laser, Becaplermin 0.01% gel,  IL & systemic steroids, or excision 3.      3 weeks
2.      Secondary infection出現如何治療?	Mupirocin or metronidazole 必要時用systemic antibiotics
3.      其他嚴重併發症為何。	Hypothyroidism, internal organ involvement,
Treatments:
1.      治療時機為何?	Growth(proliferative) phase 美容手術要在入學前進行。
2.      治療的適應症為?	Deforming, endangering, life-threatening
3.      IH的一線藥物治療有哪些?(2)	Propranolol (stabilize+regression), steroids (just stabilize)
4.      Systemic, intralisional, topical steroids分別用於何種IH?	Systemic: deforming, endangering, life-threatening Intralesional: Small localized hemangiomas at high-risk sites(lip, nasal tip, cheek, and ear) (periocular小心失明) Topical: small, superficial hemangiomas
5.      Systemic steroid如何給藥?	Prednisone/prednisolone 2–3 mg/kg/day(4–8 weeks)，之後taper。
6.      Propranolol如何給藥	Propranolol: 1.5-3 mg/kg/day given(2X-3X) per day(at least 6 months)
7.      Propranolol如停藥後有rebound growth怎麼辦?	Retreatment，一樣有效
8.      Propranolol的side effect為何?	hypotension, agitation, sleep alteration, sweating, wheezing, cold hand, & hypoglycemia(fatal)
9.      如何避免hypoglycemia?	frequent feeding and stopping the medication if oral intake is poor
10.  那些病人使用propranolol要小心低血壓副作用?	PHACE syndrome: ↓arterial blood flow (因為血壓/心跳下降)
11.  何時使用Topical βblocker?如何使用?	Superficial hemangiomas, timolol 0.5% bid
12.  IH的二線藥物治療有哪些?(2)	Interferon-α, vincristine
13.  何時使用Interferon-α	第一線用藥無效或有禁忌症時
14.  Interferon-α最著名的副作用為何?如何避免?	Neurotoxicity(spastic diplegia) 一歲後再用。
15.  何時使用vincristine?	KMP & KHE/TA。 第一線用藥無效或有禁忌症時
16.  Pulsed dye laser(585/595)治療的時機?	1.      Ulceration 2.      Superficial IH 3.      Erythema after proliferative phase
17.  Surgery治療的時機?	1.      完全Involution後 2.      3–5 y/o(學齡前)，即使involution未完全
18.  Surgery的適應症	1.      Nasal tip and lip(美容考量) 2.      Pedunculated(一刀完治，可提早) 3.      Very ulcerated(會留疤，可提早) 4.      Extremely thick dermal involvement(會留疤，可提早)
Other Vascular Tumors: Congenital hemangiomas(CH)
1.      CH的定義為何?分為哪兩型?	Fully formed at birth, not proliferate after birth Rapidly involuting CH(RICH) & noninvoluting CH(NICH)
2.      CH的病理與表現分子分別有什麼和IH不同處?	Densely fibrotic stroma and sclerosis GLUT-1 negative
3.      CH好發位置為?	Extremities or postauricular skin
4.      RICH和NICH外觀/病程的差異為?	RICH NICH A raised, violaceous tumor + radiating vein/telangiectasia+ a pale halo (high-flow) 外觀 Raised Flatter 病程 14 months前(部分)消失，留atrophic scar 不消失
5.      CH治療的適應症為?	同IH , Deforming(scaring), endangering(visual loss), life-threatening(CHF)
6.      CH的治療方法為?	同IH
Tufted angiomas(TA)
1.      TA一般的外觀為?	Subtle firm, pink or dusky-red patches/plaques/nodules (limited KHE)
2.      TA的病程為?	Early childhood出現, 病程長，不會消。
3.      TA的病理特徵為?	Vascular tufts of tightly packed capillaries(cannonball)+ 外圍crescentic spaces
4.      那些血管瘤也會有一坨坨血管聚集?(3)	Reactive angioendotheliomasis(每團很近), TA(間距大，細胞大), glomeruloid hemangioma(間距大，有hyaline顆粒，有crescent)
Kaposiform hemangioendothelioma(KHE)
1.      KHE一般的外觀為?	A brown-red stain or purpuric plaque/nodule(as TA)
2.      KHE的病程為?	Present at birth or develop in early childhood，病程長，不會消，會淋巴轉移，不遠端轉移。
3.      KHE的病理特徵為?	Spindled cells lining slit-like or crescentic vessels Minimal atypia and infrequent mitoses
4.      TA/KHE的免疫染色有何特色與差異?(3)	TA KHE Glut-1(IH) Negative D2–40(lymphatic) + part +all VEGFR-3(lymphatic) +
Kasabach-Merritt phenomenon(KMP)
1.      有KMP的血管瘤為？(2)	KHE>TA
2.      KMP血管瘤的臨床特徵為?	A tender, expanding vascular tumor + thrombocytopenia(Plt <5,000/mm3)
3.       KMP和venous / venous-lymphatic malformations出血原因差在?	KMP是platelet困在hemangioma中。 Malformation是clotting factors消耗過度。
4.      KMP的出血位於?	skin & musculature, 偶在deeper viscera
5.      KMP大多何時消退?	by 1 y/o, or after treatment(此時hemangioma仍在)
6.      KMP的治療為何?	類似Kaposi’s的部分: Vincristine, actinomycin, interferon-α, excision, radiotherapy. 特有的治療: corticosteroids, ticlopidine+aspirin, embolization
7.      KMP能否輸platelet?	不能！除非大出血或要開刀。(常考)
Multifocal lymphangiomatosis with thrombocytopenia((MLT)= cutaneovisceral angiomatosis with thrombocytopenia
1.      MLT的臨床特徵為?	Blueberry muffin-like papules/plaques/prominent exophytic tumor + intermittent thrombocytopenia + GI bleeding.
2.      MLT的病程為?	Present at birth, 之後漸長大
3.      MLT的病理特徵為?	Thin-walled vessels with hobnailed endothelial cells+ intraluminal papillary projections(as Dabska tumor)
4.      MLT免疫染色的特徵為?	IH: GLUT1(-) Lymphatic(+): LYVE-1(+), D2–40(+)
5.      MLT的治療用藥為?	Corticosteroids, interferon α, vincristine (類似KHE) bevacizumab & thalidomide (總之，效果都很有限)
Spindle cell hemangioendothelioma
1.      Spindle cell HE的臨床特徵為?	Multiple red-blue nodules at extremities
2.      Spindle cell HE的病程為	Present at any age & site(多半在10~30 y/o), locally aggressive，病程長，不會消。
3.      Spindle cell HE常跟哪種syndrome有關?	Maffucci syndrome
4.      Spindle cell HE的病理特徵為?	A nodular, dense, spindle cell proliferation with dilated dysplastic veins/ thin-walled cavernous spaces (類似肺泡的外觀)
5.      Spindle cell HE的治療為?	Excision, 但會recur
Congenital eccrine angiomatous hamartoma(CEAH)
1.      CEAH的臨床特徵為?	Ill-defined plaques on the extremities/abdomen + ↑lanugo hair /↑sweating + tenderness
2.      CEAH的病理特徵為?	eccrine glands+ dilated capillaries + a dense collagenous matrix(因為是harmatoma)
Pyogenic granuloma(PG)= lobular capillary hemangioma
1.      PG的臨床特徵為?	Rapidly growing papules/nodules with a collarette of scale or eroded surface
2.      PG的病理特徵為?	Masses or lobules of capillary vessels
3.      PG的病程為?	不自消，治療後易recur/satellite lesions(常考)
4.      PG好發在那些人?那些位置?	Adults(esp. pregnant women), cheek/forehead
5.      PG的治療為?	Excision or electrocautery, CO2/PDL, cryotherapy, imiquimod
6.      Targetoid hemosiderotic hemangioma=hobnail hemangioma
1.      Hobnail hemangioma的臨床特徵為?	A violaceous papul+ a pale rim/peripheral ecchymotic halo
2.      Hobnail hemangioma的病理特徵為?	Dilated vascular channels within intraluminal papillary projections dissecting into collagen bundles+ extravasation/hemosiderin
Endovascular papillary angioendothelioma (Dabska tumor)
1.      Dabska tumor和angiosarcoma的關係為?	A low-grade angiosarcoma
2.      Dabska tumor的臨床特徵為?	A dermal nodule or a diffuse swelling on the head, neck, or extremities(都是末端)
3.      低中度惡性的血管瘤主要有哪些?(5 )	Retiform HE, KHE, Composite HE, Dabska, Kaposi’s sarcoma(只有spindle HE良性)
4.      高度惡性的血管瘤有哪些?( 2)	Angiosarcoma, Epithelioid HE