Saturday, April 15, 2017

[考生加油]Dermatological Recall: Chapter 129. Neoplasms of Subcutaneous Fat 皮下脂肪腫瘤

Fitzpatrick's Dermatology in General Medicine

Chapter 129. Neoplasms of Subcutaneous Fat

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。
  1. Mesenchymal neoplasms中最常見的是?
Adipocyte tumors(lipoma & angiolipoma占了大半)
  1. Mesenchymal malignant neoplasms中最常見是?
  1. Adipocyte tumors從良性到惡性的spectrum為?
Lipomaatypical lipomatous tumorpleomorphic liposarcoma
  1. 主要治療方式為?
  1. Lipoma的臨床型態為?
A small circumscribed mass
  1. Lipoma的分布與好發年齡層?
全身皆可,除了 head, hands, and feet較少見。
  1. Intramuscular lipoma與intermuscular lipoma分別好發在何處?
  1. Trunk
  2. Anterior abdominal wall
  1. 哪些類型的lipoma好發女性?
Adiposis dolorosa, Chondroid lipoma, Myolipoma of soft tissue
  1. 哪些類型的lipoma好發男性?
Madelung’s disease, spindle cell/pleomorphic lipoma
  1. 前額的lipoma好發在那些族群,那些位置?
Male adult, submuscular or subgaleal site(deep-sited)
  1. Familial multiple lipomatosis(AD)好發位置在?
Arms and thighs (proximal limbs對稱分布)
  1. Lipoma相關的syndrome有哪些? 有何共同特徵?
Bannayan(搬那羊) syndrome, Gardner's syndrome
都有polyposis(多發性腸息肉)/multiple lipoma
  1. Bannayan–Riley–Ruvalcaba  syndrome(Bannayan syn.)有何特徵?
AD, (Mn) 血脂腸大頭 (鞋子裡藏了大頭)
Intestinal polyposis(腸), multiple lipomas(脂), macrocephaly(頭大), lymphangiomas, and hemangiomas(血) , genital pigmented macules, & other hamartomas
  1. Gardner's syndrome
AD, (Mn) 腸脂硬骨囊
Familial adenomatous polyposis(腸), lipomas(脂), desmoid fibromatosis(desmoid tumor硬纖維瘤), osteomas(骨), & cysts(囊)
  1. PTEN hamartoma-tumor syndromes有哪幾種?(4)
Bannayan syndrome, Cowden syndrome, Proteus syndrome, Lhermite–Duclos disease
  1. (補充)PTCH mutation有哪幾種? (4)
1. BCC 2. TE 3. SCAP 4.BFH
  1. Lipoma大多encapsulated,但哪兩種subtype除外?
Dermal lipoma , intramuscular lipomas
  1. 哪種lipoma subtype的細胞密度較低?
  1. Adenolipoma中的gland是?
Cysticlly dilated sweat ducts and sweat glands
  1. 哪種subtype最會recur?
Infiltrative variant of intramuscular lipoma
  1. Lipomatosis的定義為?
Diffuse overgrowth of unencapsulated adipose tissue infiltrating through preexisting structures
  1. Lipomatosis好發於?
Young children
  1. Lipomatosis會造成什麼問題?
長太大導致肢體disfigurement /loss of function。
  1. Benign symmetric lipomatosis(Madelung's disease作肺)有何特徵?
Multiple symmetric nonencapsulated adipose tissue in subcutaneous tissue/skeletal muscle of the neck, shoulder, and proximal upper limbs(upper limb girdle) (考過)
  1. Madelung’s disease好發於哪些族群?
Male adult with↑ alcohol intake(考過)
  1. Type /1 & Type 2 Madelung’s disease的臨床分別是如何?
Type 1: pseudoathletic
Type 2: generalized obesity-like
  1. Madelung’s disease會有什麼神經問題?
Polyneuropathy(sensory, motor, & autonomic)
  1. Madelung’s disease的原因是?
A defect in adrenergic lipolysishypertrophy of brown fat
  1. Pelvic lipomatosis的特徵為何?(無皮膚表現,不考)
Lipomatosis in pelvis and retroperitoneal space
Male adult, blacks好發
Obstructive uropathy and renal failure
  1. Adiposis dolorosa (Dercum's disease)的臨床特徵為何?
Multiple painful subcutaneous lipomatous lesions at arms and trunk (±weakness, depression, confusion, lethargy, or dementia)
  1. Adiposis dolorosa好發於哪些族群?
Middle-aged obese women
  1. Adiposis dolorosa如何治療?
Pain relief(一般止痛藥), excision or liposuction, weight reduction
  1. Lipomatosis of Nerve (Fibrolipomatous Hamartoma of Nerve) 的臨床特徵為?
A growing mass on hands with pain and paresthesia median n.> ulnar n.
(adipose and fibrous tissue/ concentric fibrosis in the epineurium & perineurium.)
  1. Lipomatosis of Nerve好發於哪些族群?
Infants and children
  1. Nevus Lipomatosus Superficialis(NLS)的臨床特徵為?
Multiple papules and nodules at buttocks, lumbar back, and posterior thighs
  1. NLS好發於哪些族群?
Multiple: children and young adults.
Solitary: adult
  1. NLS形成confluent linear plaques along the skinfolds 時的好笑暱稱為?
Michelin tire man
  1. NLS的病理特徵為?
Bands of mature adipose tissue infiltrate dermal collagen
  1. 單一NLS在成人常常要和那些entities作D/D?(2)
fibroepithelial polyp containing adipose tissue or an intradermal lipoma.
  1. Intradermal lipoma和NLS的差異為?
前者spare the papillary dermis,後者會長到superficial dermis
  1. Piezogenic Pedal Papules(PPP)的臨床特徵為?
Painful, papules/ nodules at heel,受壓才出現
Ehlers–Danlos syndrome可以長在wrist
  1. PPP的原因為?
Herniation of subcutaneous fat into dermis (connective tissue defects)
  1. PPP的病理特徵為?
Loss of compartmentalization/ fibrous septa of fat lobules in deep dermis/superficial subcutis.
  1. Precalcaneal congenital fibrolipomatous hamartoma(PCFH) 的臨床特徵為?
Bilateral, symmetrical, 1 cm, painless,skin-colored papules on the medial plantar aspect of the foot( precalcaneal location) at birth
  1. PCFH的病理特徵為?
Mature fat surrounded/separated by hypocellular fibrous sheaths
  1. Angiolipoma和其他lipoma的成因有何不同?
Angiolipoma: reactive or hamartomatous(會痛)
Lipoma: neoplastic
  1. Angiolipoma好發於何處?何人?
Forearm, young adults
  1. 幾乎都是血管的angiolipoma稱為?
Cellular angiolipoma
  1. Angiolipoma的病理特徵為?
Mature adipose tissue + capillary-sized vessels
  1. Spindle Cell and Pleomorphic Lipoma好發於何處?何人?
posterior neck, upper back, and shoulder(同Madelung), 中年男性
  1. Spindle Cell Lipoma的病理特徵為?
Mature fat + bland and uniform spindle cells+ a myxoid stroma+ mast cells
  1. 那些Soft tissue tumor有鬆散stroma+mast cells (2)
Spindle Cell and Pleomorphic Lipoma, Neurofibroma
  1. Pleomorphic lipoma的病理特徵為?
Biarre multinucleate giant cells(floret cells), stroma類似spinmdle cell lipoma,兩者是spectrum
  1. Spindle cell/Pleomorphic lipoma免疫染色可染何物?
  1. Chondroid lipoma好發於何處?何人?
proximal extremities and limb girdle, female adult
  1. Chondroid lipoma的病理特徵為?
Mature adipose tissue+ lipoblasts + chondroid matrix
Involve deep subcutis, fascia, and skeletal muscle
  1. Chondroid lipoma免疫染色可染何物?
  1. Myolipoma of soft tissue好發於何處?何人?
Retroperitoneum, abdominal cavity, and inguinal area, trunk and extremities. Female adult.
  1. Myolipoma of soft tissue的別名為?(提示:和女性有關)
extrauterine lipoleiomyoma(所以成分是平滑肌)
  1. Myolipoma of soft tissue的病理特徵為?
Mature adipose tissue + evenly distributed traversing fascicles of smooth muscle, NO thick-walled vessels(不同於angiiomyolipoma)
  1. Myolipoma of soft tissue免疫染色可染何物?
Smooth muscle component: SMA & desmin
  1. Hibernoma的字源為何?
Hibernate: 冬眠。Hibernoma長得很像嬰幼兒用來產熱的brown fat。這種脂肪在準備過冬的動物(棕熊)會特別多。
  1. Hibernoma好發於何種年齡?
Young adults
  1. Hibernoma好發於那些位置?
Thigh(最多)和其他brown fat分布區( scapular area, shoulder, head, and neck)
  1. Hibernoma的臨床特徵為?
  1. Hibernoma的病理特徵為?
Multivacuolated adipocytes with small, centrally placed nuclei and pale to eosinophilic and granular cytoplasm(不能只認顏色)
  1. Hibernoma有哪六種subtypes.
Pale cell subtype: pale cells>50%
Eosinophilic subtype: eosinophilic cells>50%
Mixed subtype: pale=eosinophilic
Myxoid, Lipoma-like, and spindle cell variants
  1. Hibernoma的免疫染色為?
S100(+). CD34(+) in spindle cell variant
  1. Lipoblastoma和Lipoblastomatosis的差異為何?
Lipoblastoma: multilobular/circumscribed/single
Lipoblastomatosis: infiltrative/multiple
  1. Lipoblastoma像何種組織?
Fetal adipose tissue
  1. Lipoblastoma好發於何處?何人?
Male infants and young children.< 5y/o
  1. Lipoblastoma是哪個基因變異造成?
Plag1 (pleomorphic adenoma gene)
  1. Lipoblastoma的病理特徵為?
Mature +immature adipocytes +Lipoblasts(indented and scalloped nuclei)+ myxoid stroma + delicate branching capillaries + primitive stellate cells (類似Myxoid liposarcoma,但無cellular atypia)
  1. Lipoblastoma免疫染色為?
S100(+) and CD34(+)
Hemosiderotic fibrohistiocytic lipomatous tumor(HFLT)
  1. HFLT好發於何處?何人?
Distal extremities( dorsal ankle/foot), female adult
  1. HFLT的原因可能是?
A reactive condition after trauma
  1. HFLT的病理特徵為?
An adipocyte/ a spindle cell component(honeycomb) +hemosiderin deposition
  1. HFLT的免疫染色為?
CD34(+) in spindle cells.
  1. Lipofibromatosis好發於何處? 何人?
Hads and feet, male infants
  1. Lipofibromatosis的病理特徵為?
Mature adipose tissue+ uniform spindle cells + fibrous septa
  1. 最常見的liposarcoma為?第二常見為?
Atypical Lipomatous Tumor(ALT)/Well-Differentiated Liposarcoma(WD)> Myxoid liposarcomas
  1. ALT/WD好發於何處?何人?
Extremities, retroperitoneum, and paratesticular tissue, elder adult.
  1. 決定ALT/WD的prognostic predictor為?
Anatomic location:
Retroperitoneum/paratesticular region: 不好切,poor
Extremities: 好切,well
  1. ALT和WD的差異為何?
ALT: 切得乾淨   WD: 切不乾淨
  1. ALT/WD主要位於?
Deep soft tissue(muscle), sometimes subcutis of thigh (一般不會在皮膚切到)
  1. ALT的病理特徵為?
Lipoma-like, focal adipocyte atypia + lipoblast + hyperchromatic/ multinucleate bizarre stromal cells + Floret-type giant cells
  1. ALT/WD的免疫染色特徵為?
MDM2-CDK4 amplification
Dedifferentiated Liposarcoma(Dd)90%來自WD,10%de novo,易轉移復發,預後差
  1. Dd好發於何處?何人?
Larg masses in retroperitoneum of adults.(非皮膚病,不考)
  1. Dd的病理特徵為?
Solid, nonlipogenic areas(pleomorphic and storiform malignant fibrous histiocytoma-like, myxofibrosarcoma-like) adjacent to WD +a very sharp demarcation
  1. 決定Dd的prognostic predictor為?
Anatomic location:
Retroperitoneum/paratesticular region: 不好切,poor
Extremities: 好切,well
Myxoid/Round Cell Liposarcoma(ML/RL)
  1. ML/RL好發於何處?何人?
Extremities( thigh) of middle-aged adults
  1. ML/RL的臨床特徵為?
Large, well circumscribed, multinodular mass,常轉移lung、bone
  1. ML/RL的病理特徵為?
WD area + myxoid matrix + primitive mesenchymal cells and lipoblasts+ chicken wire or crow's feet血管
  1. RL和ML的關係?
High-grade end of spectrum of myxoid liposarcoma.(round cell>5%是不好的指標)
Pleomorphic liposarcoma(PL)
  1. PL好發於何處?何人?
Extremities(lower limbs) of middle-aged adults
  1. PL的臨床特徵為?
High rate of metastasis and mortality
  1. PL的病理特徵為?
a pleomorphic high-grade sarcoma containing variable numbers of lipoblasts.

No comments:

Post a Comment