Fitzpatrick's Dermatology in General Medicine
Chapter 129. Neoplasms of Subcutaneous Fat
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。
Introduction
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Adipocyte tumors(lipoma & angiolipoma占了大半)
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Liposarcoma
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Lipoma→atypical lipomatous tumor→pleomorphic liposarcoma
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手術切除(惡性切不乾淨可考慮化放療)
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Lipoma
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A small circumscribed mass
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全身皆可，除了 head, hands, and feet較少見。
Adulthood
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Adiposis dolorosa, Chondroid lipoma, Myolipoma of soft tissue
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Madelung’s disease, spindle cell/pleomorphic lipoma
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Male adult, submuscular or subgaleal site(deep-sited)
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Arms and thighs (proximal limbs對稱分布)
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Bannayan(搬那羊) syndrome, Gardner's syndrome
都有polyposis(多發性腸息肉)/multiple lipoma
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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
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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)
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Bannayan syndrome, Cowden syndrome, Proteus syndrome, Lhermite–Duclos disease
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1. BCC 2. TE 3. SCAP 4.BFH
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Dermal lipoma , intramuscular lipomas
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Fibrolipomas
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Cysticlly dilated sweat ducts and sweat glands
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Infiltrative variant of intramuscular lipoma
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Lipomatosis
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Diffuse overgrowth of unencapsulated adipose tissue infiltrating through preexisting structures
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Young children
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長太大導致肢體disfigurement /loss of function。
切不乾淨且容易復發。.
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Multiple symmetric nonencapsulated adipose tissue in subcutaneous tissue/skeletal muscle of the neck, shoulder, and proximal upper limbs(upper limb girdle) (考過)
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Male adult with↑ alcohol intake(考過)
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Type 1: pseudoathletic
Type 2: generalized obesity-like
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Polyneuropathy(sensory, motor, & autonomic)
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A defect in adrenergic lipolysis→hypertrophy of brown fat
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Lipomatosis in pelvis and retroperitoneal space
Male adult, blacks好發
Obstructive uropathy and renal failure
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皮誌報過
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Multiple painful subcutaneous lipomatous lesions at arms and trunk (±weakness, depression, confusion, lethargy, or dementia)
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Middle-aged obese women
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Pain relief(一般止痛藥), excision or liposuction, weight reduction
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皮誌報過
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A growing mass on hands with pain and paresthesia median n.> ulnar n.
(adipose and fibrous tissue/ concentric fibrosis in the epineurium & perineurium.)
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Infants and children
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Multiple papules and nodules at buttocks, lumbar back, and posterior thighs
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Multiple: children and young adults.
Solitary: adult
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Michelin tire man
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Bands of mature adipose tissue infiltrate dermal collagen
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fibroepithelial polyp containing adipose tissue or an intradermal lipoma.
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前者spare the papillary dermis，後者會長到superficial dermis
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Painful, papules/ nodules at heel，受壓才出現
Ehlers–Danlos syndrome可以長在wrist
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Herniation of subcutaneous fat into dermis (connective tissue defects)
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Loss of compartmentalization/ fibrous septa of fat lobules in deep dermis/superficial subcutis.
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Bilateral, symmetrical, 1 cm, painless,skin-colored papules on the medial plantar aspect of the foot( precalcaneal location) at birth
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Mature fat surrounded/separated by hypocellular fibrous sheaths
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Angiolipoma: reactive or hamartomatous(會痛)
Lipoma: neoplastic
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Forearm, young adults
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Cellular angiolipoma
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Mature adipose tissue + capillary-sized vessels
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posterior neck, upper back, and shoulder(同Madelung), 中年男性
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Mature fat + bland and uniform spindle cells+ a myxoid stroma+ mast cells
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Spindle Cell and Pleomorphic Lipoma, Neurofibroma
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Biarre multinucleate giant cells(floret cells), stroma類似spinmdle cell lipoma，兩者是spectrum
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CD34
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proximal extremities and limb girdle, female adult
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Mature adipose tissue+ lipoblasts + chondroid matrix
Involve deep subcutis, fascia, and skeletal muscle
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S100(lipoblasts)
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Retroperitoneum, abdominal cavity, and inguinal area, trunk and extremities. Female adult.
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extrauterine lipoleiomyoma(所以成分是平滑肌)
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Mature adipose tissue + evenly distributed traversing fascicles of smooth muscle, NO thick-walled vessels(不同於angiiomyolipoma)
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Smooth muscle component: SMA & desmin
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Hibernoma
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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。
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Young adults
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Thigh(最多)和其他brown fat分布區( scapular area, shoulder, head, and neck)
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一顆10cm左右，無症狀的腫瘤。
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Multivacuolated adipocytes with small, centrally placed nuclei and pale to eosinophilic and granular cytoplasm(不能只認顏色)
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Pale cell subtype: pale cells>50%
Eosinophilic subtype: eosinophilic cells>50%
Mixed subtype: pale=eosinophilic
Myxoid, Lipoma-like, and spindle cell variants
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S100(+). CD34(+) in spindle cell variant
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Lipoblastoma/Lipoblastomatosis
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Lipoblastoma: multilobular/circumscribed/single
Lipoblastomatosis: infiltrative/multiple
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Fetal adipose tissue
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Male infants and young children.< 5y/o
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Plag1 (pleomorphic adenoma gene)
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Mature +immature adipocytes +Lipoblasts(indented and scalloped nuclei)+ myxoid stroma + delicate branching capillaries + primitive stellate cells (類似Myxoid liposarcoma，但無cellular atypia)
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S100(+) and CD34(+)
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Hemosiderotic fibrohistiocytic lipomatous tumor(HFLT)
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Distal extremities( dorsal ankle/foot), female adult
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A reactive condition after trauma
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An adipocyte/ a spindle cell component(honeycomb) +hemosiderin deposition
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CD34(+) in spindle cells.
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Lipofibromatosis
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Hads and feet, male infants
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Mature adipose tissue+ uniform spindle cells + fibrous septa
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Liposarcomas
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Atypical Lipomatous Tumor(ALT)/Well-Differentiated Liposarcoma(WD)> Myxoid liposarcomas
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Extremities, retroperitoneum, and paratesticular tissue, elder adult.
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Anatomic location:
Retroperitoneum/paratesticular region: 不好切，poor
Extremities: 好切，well
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ALT: 切得乾淨 WD: 切不乾淨
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Deep soft tissue(muscle), sometimes subcutis of thigh (一般不會在皮膚切到)
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Lipoma-like, focal adipocyte atypia + lipoblast + hyperchromatic/ multinucleate bizarre stromal cells + Floret-type giant cells
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MDM2-CDK4 amplification
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Dedifferentiated Liposarcoma(Dd)90%來自WD，10%de novo，易轉移復發，預後差
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Larg masses in retroperitoneum of adults.(非皮膚病，不考)
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Solid, nonlipogenic areas(pleomorphic and storiform malignant fibrous histiocytoma-like, myxofibrosarcoma-like) adjacent to WD +a very sharp demarcation
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Anatomic location:
Retroperitoneum/paratesticular region: 不好切，poor
Extremities: 好切，well
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Myxoid/Round Cell Liposarcoma(ML/RL)
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Extremities( thigh) of middle-aged adults
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Large, well circumscribed, multinodular mass，常轉移lung、bone
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WD area + myxoid matrix + primitive mesenchymal cells and lipoblasts+ chicken wire or crow's feet血管
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High-grade end of spectrum of myxoid liposarcoma.(round cell>5%是不好的指標)
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Pleomorphic liposarcoma(PL)
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Extremities(lower limbs) of middle-aged adults
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High rate of metastasis and mortality
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a pleomorphic high-grade sarcoma containing variable numbers of lipoblasts.
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