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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5192_Библиотеки_им_академика_М_И_Перельмана.pdf
Fig. 6.36 Congenital dermatobrosarcoma protuberans of the back at presentation in a 16-year-old boy, featuring various morphologic clinical features, including plaques, nodules, atrophy, telangiectasia, and scar-like changes. (From
Johnson-Jahangir H, Ratner D. Advances in management of dermatobrosarcoma protuberans. Dermatol Clin. 2011;29[2]:191–201.)
• Histology: monotonous spindle cells (cells are more bland
and uniform than DF!) with a storiform architecture in
dermis and throughout SQ fat; characteristic “honeycomb”
inltration of fat; 5% with melanin (Bednar tumor)
■
CD34 strongly positive
■
Negative for Factor XIIIa and stromelysin-3
• Fibrosarcomatous degeneration:
■
Occurs in 9%–20% (more common in recurrent DFSP)
■
Histology: ↑ cellularity, ↑ mitoses, ↑ atypia, “herringbone
pattern,” and ↓ CD34 staining (weak or lost)
■
Recent study (Hoesly et al., J Am Acad Dermatol, 2015)
showed ↑ recurrence rate and ↑ metastasis (18% vs.
0% for conventional DFSP) for lesions with
brosarcomatous change
• Treatment: Mohs (ToC) . WLE w/ 3 cm margins down to
deep fascia
■
Imatinib is approved for unresectable or metastatic
disease (46% partial response rate) → blocks activity of
COL1A1-PDGFB fusion protein
• Prognosis: propensity for local recurrence (1% with
Mohs; average of 15% w/ WLE; up to 50% recurrence
with WLE for head/neck lesions); very low risk of
metastatic disease (,1%)
■
Multiply recurrent lesions have ↑ risk of brosarcomatous
transformation → ↑↑ metastatic potential
• Giant cell broblastoma (pediatric variant): occurs in
early childhood; affects boys » girls; possesses same
COL1A1-PDGFB translocation as DFSP; favors head/
neck, trunk, and groin; histologically resembles DFSP, but
has distinctive pseudovascular spaces surrounded by
giant cells
Atypical broxanthoma (AFX)
• Relatively common low-grade, supercial (dermally-
based) sarcoma that arises on CSD skin (head and neck
#1 . upper trunk and extremities) of elderly (70–80 years
old); p/w rapidly-growing, ulcerated red nodule
• Recurs in up to 5% of cases, but almost never metastasizes
• Treatment: Mohs . WLE
6.10 Fibrohistiocytic Neoplasms
Fig. 6.37 Atypical broxanthoma. Low-power view of a pleomorphic, ulcerated
tumor. Note the lateral collarette. (From Brinster NK, Liu V, Diwan H, McKee PH.
Atypical broxanthoma. In: Dermatopathology: A Volume in the High Yield Pa-
thology Series. Philadelphia: Elsevier; 2011:474.)
• Histology: fairly well-circumscribed, overtly malignant
dermal proliferation slammed up against an atrophic/
ulcerated epidermis; tumor extends down to deep dermis in
a “pushing” fashion; tumor is composed of variable mixture
of four main cell types: (1) spindle cells, (2) histiocyte-
like cells, (3) xanthomatous cells, and (4) bizarre,
multinucleated giant cells; all cell types are notable for
hyperchromatic nuclei, pleomorphism, and a high mitotic
rate w/ numerous wildly atypical mitoses (Fig. 6.37)
■
AFX never extensively inltrates SQ fat → if present,
should consider “supercial undifferentiated
pleomorphic sarcoma” (UPS), or “pleomorphic dermal
sarcoma” (PDS; discussed below)
■
Immunostains: no specic immunostain to conrm
Dx of AFX! Stains positively with CD10 (nonspecic),
procollagen I, CD99, CD68 (histiocyte-like cells), and
SMA in a “tram-track” pattern (pattern consistent with
myobroblastic differentiation, rather than true
smooth muscle)
• AFX is a diagnosis of exclusion → must rst rule out other
entities in the “SLAM” DDx (malignant dermal spindle cell
neoplasm SLAMmed up against the epidermis):
■
SCC (sarcomatoid/spindle cell variant): stains
positively with high-molecular-weight keratin (CK903
and CK5/6) or broad-spectrum keratin (MNF-116),
p63 and p40 (newest and most specic marker)
■
LMS: desmin1 and SMA1 (diffuse cytoplasmic
staining vs. tram-track in AFX)
■
AFX: negative for high-molecular-weight keratin, p63,
p40, S100, SOX10, and desmin
■
Melanoma (spindle cell or desmoplastic variants):
S1001 and SOX-101
• Lesions related to AFX:
■
Pleomorphic dermal sarcoma (PDS): recently-described
entity; arises on same sites as AFX, but has deeper
subcutaneous invasion, necrosis, lymphovascular
invasion or PNI; a/w ↑ recurrence (28%) and ↑↑
metastases (10%)
Clinical relevance: if an AFX-like lesion has
signicant involvement of fat → would be wise to
call it a PDS instead
375
CHAPTER 6 • Neoplastic Dermatology
■
Undifferentiated pleomorphic sarcoma (UPS): “UPS” has
replaced the old term “malignant brous histiocytoma”
(MFH); UPS has similar ugly cell types as AFX, but is a
deep sarcoma that arises in deep soft tissues (thigh #1) of
middle-aged adults; 5-year mortality 5 50%
Other broblastic proliferations
Angiobroma
• Clinical features
■
Fibrous papule: solitary domed papule; nose/face of
adults; mimics BCC
■
Pearly penile papules: aggregated pearly papules;
corona/sulcus of glans penis
■
Facial/periungual angiobromas: a/w various syndromes
• Histopathologic features
■
Dermal proliferation of stellate (triangular) or
multinucleated broblasts with brotic stroma and
ectatic thin-walled vessels
• Other high-yield facts/associations
■
Multiple facial angiobromas: seen in TS, MEN-1, and
Birt-Hogg-Dubé
■
Periungual broma (Koenen tumor) is also
characteristic of TS
Sclerotic broma
• Clinical features
■
Firm/pearly papule or nodule; any site; can be solitary
or multiple
• Histopathologic features
■
Sclerotic collagen bundles arranged as intersecting
stacks (“plywood” or “starry night” pattern);
inconspicuous spindle cells between collagen bers
• Other high-yield facts/associations
■
Boards tip: the characteristic histology and association
with Cowden syndrome are the only two commonly
tested points
Pleomorphic broma
• Clinical features
■
Domed or pedunculated papules on extremities of
adults; F . M; resemble skin tags clinically; benign
• Histopathologic features
■
Looks similar to acrochordon, but has scattered
hyperchromatic, bizarre, multinucleated, or stellate
cells; lacks mitoses
■
CD341, loss of RB1
• Other high-yield facts/associations
■
May simply represent a skin tag with “ancient change”
■
Part of the newly-described “RB1-deleted soft tissue
tumor family” (spindle cell lipoma, pleomorphic lipoma,
pleomorphic broma, and other less common entities)
Multinucleate cell angiohistiocytoma
• Clinical features
■
Multiple, grouped red papules on dorsal hands or legs
of women in their 40s; benign
• Histopathologic features
■
Looks like a “cell-poor DF” w/ characteristic
multinucleated giant cells and prominent
proliferation of dilated vessels in dermis; stains like
DF (Factor XIIIa1 and S100 negative)
Epithelioid cell histiocytoma (epithelioid
brous histiocytoma)
• Clinical features
■
Solitary, pyogenic granuloma-appearing papules; most
common on thighs of 50-year-old women
• Histopathologic features
■
Well-circumscribed dermal proliferation of epithelioid
cells (resembles Spitz nevus cells) w/ epidermal
collarette and dermal sclerosis; stains like a DF
• Other high-yield facts/associations
■
Know the characteristic histology!
■
ALK gene rearrangement, ALK1 on IHC
Acral brokeratoma
• Clinical features
■
Middle-aged adults; nger; exophytic keratotic
papule w/ surrounding collarette
• Histopathologic features
■
Hyperkeratosis/epidermal acanthosis w/ dermal collagen
bers oriented perpendicular to skin surface; lacks nerves
• Other high-yield facts/associations
■
DDx: supernumerary digit (has abundant nerve
fascicles) and periungual broma (more vascular)
Dermatomyobroma
• Clinical features
■
Young adults; F . M; solitary, well-circumscribed 1 to
2 cm oval plaque resembling plaque type DFSP or DF;
most commonly on upper trunk/neck; benign
• Histopathologic features
■
Reticular dermis has long fascicles of spindled
myobroblasts arrayed parallel to the skin surface;
respects adnexal structures (vs. ablated in DF)
• Other high-yield facts/associations
■
Derived from myobroblasts → SMA1 (tram-track
staining pattern); CD34 negative (distinguishes from
plaque-type DFSP), S100 negative (distinguishes from
NF), Factor XIIIa negative (vs. DF), and desmin
negative (vs. pilar leiomyoma)
Inclusion body bromatosis (infantile
digital broma)
• Clinical features
■
Infants; multiple rm papules on dorsolateral ngers
and toes (spares thumb/rst toe); benign; often
spontaneously regress, but can recur after excision
(50% recurrence rate)
• Histopathologic features
■
Entire dermis lled w/ intersecting fascicles of
plump spindle cells; on high power can see the
pathognomonic pink-red inclusion bodies (same size
as an RBC) (Fig. 6.38)
376
Fig. 6.38 Infantile digital bromatosis. Infantile digital bromatosis is composed
of fascicles of bland spindle cells that have perinuclear intracytoplasmic eosinophilic inclusions. (Courtesy of Dr. KJ Busam. From Busam KJ, ed. Dermatopa-
thology: A Volume in the Series: Foundations in Diagnostic Pathology . 2nd ed.
Philadelphia: Elsevier; 2016.)
• Other high-yield facts/associations
■
Pink inclusions are composed of actin laments →
SMA1, calponin1, desmin1, and stain red w/ trichrome
Fibromatosis
• Clinical features
■
Supercial variants:
Palmar (Dupuytren’s)
Plantar (Ledderhose)
Penile (Peyronie’s)
Knuckle pads
■
Deep variants: seen in abdominal wall, intra- and extraabdominal → all a/w ↑ morbidity and mortality
• Histopathologic features
■
Dermal/subcutaneous; extremely long fascicles of
bland spindled, wavy broblasts and myobroblasts;
often inltrates fascia and skeletal muscle
6.10 Fibrohistiocytic Neoplasms
• Other high-yield facts/associations
■
Deep desmoid tumors may be a/w Gardner syndrome,
have b-catenin mutations, and stain b-catenin1
■
Supercial bromatoses: benign but locally destructive
→ excision 1 fasciotomy is ToC
Nodular fasciitis
• Clinical features
■
Young to middle-aged adults; rapidly growing
1–5 cm subcutaneous nodule; classically on upper
extremities (#1 site overall) and head/neck (#1 site
in children); may have history of trauma; benign self-
limited lesions
• Histopathologic features
■
Well-circumscribed, deep subcutaneous nodule often
a/w fascia; plump spindle cells with “tissue culture”
appearance and frequent mitoses (but none atypical);
admixed lymphocytic inammation; characteristic
myxoid stroma (early), collagenous stroma (later);
numerous small blood vessels with extravasated RBCs
(Fig. 6.39)
■
Prototypical “pseudosarcoma” because of its
↑ cellularity (composed of spindle cells) and frequent
mitoses
• Other high-yield facts/associations
■
Boards tip: histology is frequently tested b/c it is a classic
“pseudosarcoma” → potential medico-legal pitfall
■
Major clues: myxoid stroma, sharp circumscription,
lack of atypical mitoses, and RBC extravasation
■
MYH9-USP6 translocation (present in .75%; other
USP6 gene fusions present in remainder of cases)
Fibrous hamartoma of infancy
• Clinical features
■
Infants; M . F (3:1); skin-colored subcutaneous nodule;
shoulder/arm/axilla; recurrence uncommon after excision
• Histopathologic features
■
Poorly circumscribed hamartoma; benign
Pale stroma
Spindle cells
Hemorrhage
Fig. 6.39 Nodular fasciitis histology. Note the myobroblasts in the mucinous loose stroma with a “tissue culture” appearance. (From Rapini RP. Fibrohistiocytic proliferations and neoplasms. In: Rapini RP, ed. Practical Dermatopathology. 3rd ed. Philadelphia: Elsevier; 2021:393–411.)
377
CHAPTER 6 • Neoplastic Dermatology
■
Triphasic proliferation:
Plump spindle cells in fascicles a/w collagenous
stroma
Small aggregates of immature mesenchymal cells
Mature fat
• Other high-yield facts/associations
■
Frequently tested on dermpath specialty boards
Myobroma (infantile myobromatosis)
• Clinical features
■
Infantile form: 50% at birth; p/w multiple pinkviolaceous dermal/SQ nodules on head (.trunk); F .
M; can involve bones and internal organs → ↑ morbidity
and mortality
■
Adult form: solitary 1–3 cm nodules; most commonly
on head/neck; benign
• Histopathologic features
■
Biphasic proliferation:
Hypocellular areas of blue-pink nodules (may
appear cartilaginous, “myoid,” or hyalinized) w/
fascicles of bland myobroblasts
Hypercellular areas w/ primitive-appearing round,
blue cells with ↑ N:C ratio, and ectatic staghorn
(“hemangiopericytoma-like”) vessels
■
Stains conrm myobroblastic derivation (SMA1
tram-track and desmin negative)
• Other high-yield facts/associations
■
Most common form of bromatosis in children
■
If limited to soft tissue and bone involvement → selfresolves; good prognosis
■
Visceral involvement is a/w high mortality
■
Variant: when hypercellular areas predominate →
termed infantile hemangiopericytoma
Giant cell tumor of tendon sheath (tenosynovial giant cell tumor)
• Clinical features
■
Adults; F . M; rm subcutaneous nodule; most
common on ngers; benign, but 30% recur
• Histopathologic features
■
Nodular proliferation of round polygonal cells and
osteoclastic giant cells; variable collagen,
inammation and hemosiderin in the background
• Other high-yield facts/associations
■
Boards tip: this is the only testable neoplasm w/
numerous osteoclastic giant cells
Connective tissue nevus (collagenoma and elastoma)
• Clinical features
■
Firm papulonodules or plaque; any site
• Histopathologic features
■
Collagenoma: haphazard, thickened collagen bundles
■
Elastoma: ↑ elastic bers; histologic changes may be
very subtle → need VVG stain
• Other high-yield facts/associations
■
Syndromes a/w connective tissue nevi:
TS: Shagreen patch (pebbly plaque on the lower
back)
Fig. 6.40 Collagenomas. Periumbilical skin-colored papulonodules. (From Zeller
S, Marx SJ, Lungu AO, Cowen EW, Turner ML. Multiple angiobromas and collagenomas in a 45-year-old man with recurrent nephrolithiasis, fatigue, and vision
loss. J Am Acad Dermatol. 2009;61[2]:319–322.)
MEN-1: pedunculated collagenomas (Fig. 6.40) 1
facial angiobromas 1 endocrine neoplasia
Buschke-Ollendorf (aka dermatobrosis lenticularis
disseminata): either collagenomas or elastomas 1
osteopoikilosis
Proteus syndrome: cerebriform plantar connective
tissue nevi
6.11 VASCULAR PROLIFERATIONS
• As a general rule, benign vascular lesions:
■
Have: lobular growth patterns (multiple lobules/
nodules of vessels) and well-circumscribed borders
■
May have: mitoses (but lack atypical mitoses) and
reactive atypia (enlarged/“revved-up” endothelial cells)
■
Do not have: inltrative architecture, hyperchromatic nuclei,
markedly pleomorphic cells, atypical mitoses, or necrosis
Benign vascular lesions
Vascular malformation (includes “port wine stain,” “cavernous hemangioma” old terminology)
• Present at birth; may be capillary, venous, lymphatic, or
arteriovenous malformation → does not rapidly enlarge
(because it is a malformation rather than a true
neoplasm), unlike infantile hemangiomas
• Variable clinical appearances; tend to persist and become
more verrucous over time
• GLUT-1 negative
• Associations: Maffucci syndrome, Klippel-Trenaunay,
Sturge-Weber, Blue Rubber Bleb (cavernous hemangiomas),
Kasabach-Merritt syndrome, and Proteus syndrome
Intravascular papillary endothelial hyperplasia (Masson tumor, pseudoangiosarcoma)
• Reactive phenomenon (organization of a thrombus)
most commonly seen within a vein (.in a vascular
malformation/neoplasm . extravascular hematoma)
378
6.11 Vascular Proliferations
• Slow-growing dusky nodules; most commonly in veins
of head/neck or ngers
• Histology: thrombosed vessel with intraluminal
proliferation of endothelial cells → forms papillary
structures mimicking angiosarcoma
■
Major clues 5 sharply circumscribed (entirely
contained within the thrombosed vessel), lacks
multilayering of endothelial cells, and cells are not
hyperchromatic
Angiokeratoma
• Supercial keratotic/verrucous vascular lesions
• Five types:
■
Angiokeratoma of Mibelli: 10 to 15 years old; ngers
and toes
■
Angiokeratoma of Fordyce: older men (scrotum) or
females (vulva)
■
Angiokeratoma corporis diffusum: multiple lesions
in childhood/adolescence; bathing suit distribution;
seen in Fabry disease and other enzyme
deficiencies
■
Angiokeratoma circumscriptum: children; F . M;
aggregate of lesions forming a plaque
■
Solitary and multiple angiokeratomas: children
and adults; arise on any site; may be related to
chronic irritation/trauma of supercial dermal
vessel
• Histology: dilated supercial dermal vessels with
epidermal hyperplasia; rete hug vessels
Pyogenic granuloma (lobular capillary hemangioma)
• Benign capillary proliferation; a/w trauma, pregnancy,
and medications (oral contraceptive pills, oral retinoids,
indinavir, BRAF and EGFR inhibitors)
• Most common in children and young adults; rapidly
growing, exophytic, and hemorrhagic papule w/
epidermal collarette
• Common sites: gingiva (pregnancy)/oral cavity, lips, and
digits
• Histology: well-circumscribed, lobular proliferation of
small capillaries w/ RBC extravasation; ↑ mitotic activity
(no atypical mitoses) and reactive atypia of endothelial
cells
Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophils, ALHE)
• Grouped nodules or plaques on head/neck (most
commonly around ear) of young to middle-aged adults
• Histology: lobular dermal proliferation of capillaries and
larger, thicker vessels w/ large epithelioid endothelial
cells lining the vessel lumen; intracytoplasmic vacuoles
within endothelial cells (represents primitive vascular
lumens); background of lymphocytic and eosinophilic
inammation (often intense, with occasional lymphoid
follicles); brotic stroma (Fig. 6.41)
• FOS rearrangements by FISH
Infantile hemangioma
• Onset in rst couple months of life (usually not obvious
at birth) → rapid growth for 4 to 6 months → slow
involution over years
■
Standard teaching—50% involute by 5 years and 90%
by 9 years
• ↑ Incidence in: premature infants, females and placental
abnormalities
• Most are supercial and therefore clinically bright red;
arise at any site
■
Deeper lesions are purple-blue
• Histology: dense dermal 1/– subcutaneous capillary
proliferation that is GLUT-1 positive
• Treatment for problematic lesions (ulceration, sensitive
location): b-blockers (rst line), corticosteroids, and
surgical or laser therapies
■
Airway hemangioma: concern for involvement if a
plaque-type hemangioma is present from the
preauricular cheek along the mandible, lower lip, chin,
or anterior neck (beard distribution)
■
Periorbital hemangioma: concern for development
of astigmatism (from direct pressure on the globe)
and amblyopia (caused by obstruction of the visual
axis)
• Rapidly involuting infantile hemangioma (RICH): fully
developed at birth, no postnatal proliferation, and
involutes over 1 year; GLUT-1 negative
• Noninvoluting infantile hemangioma (NICH): fully
developed at birth, grows proportionally with patient;
does not involute; GLUT-1 negative
Targetoid hemosiderotic lymphatic malformation (hobnail hemangioma, targetoid hemosiderotic hemangioma)
• Clinically distinctive, acquired lesion; affects children and
young adults; legs (. arms . trunk); red-brown papules
with “target-like” appearance (dark centrally → pale area
is rst ring → bruise-like patch in outer ring); often a/w
trauma
Fig. 6.41 Angiolymphoid hyperplasia with eosinophils (a.k.a., epithelioid hemangioma, ALHE). The vascular channels are lined by plump, partly vacuolated endothelial cells. There are scattered eosinophils in the stroma (H&E). (From Patterson JW. Vascular tumors. In: Weedon’s Skin Pathology. 4th ed. Philadelphia:
Elsevier, 2016:1069–1115.)
379
CHAPTER 6 • Neoplastic Dermatology
Fig. 6.42 Hobnail hemangioma: the endothelial cells are prominent and protrude
into the lumen. Note the papillary processes. (From Calonje E, Damaskou V, Lazar
AJ. Connective tissue tumors. In: Calonje E, Brenn T, Lazar AJ, Billings SD, eds.
McKee’s Pathology of the Skin. 5th ed. Philadelphia: Elsevier; 2020:1698–1894.)
• New terminology reects expression of lymphatic markers
by lesional cells
• Histology: biphasic lesion
■
Upper dermis (Boards favorite): markedly dilated thinwalled vessels lined by thin, elongated endothelial
cells that protrude (“hobnail”) into lumen (Fig. 6.42)
■
Lower dermis: vessels become more slit-like; RBC
extravasation and hemosiderin deposition within dermis
Tufted angioma
• Pink-red macules, plaques located on neck or trunk →
spreads to involve large areas; may represent a supercial
form of Kaposiform hemangioendothelioma (KHE)
• Most commonly appears in rst year of life (25%
congenital); congenital form can be a/w Kasabach-Merritt
phenomenon (occurs less frequently than with KHE)
• Histology: dermal/subcutaneous tightly packed lobules of
capillaries in a “cannonball” pattern; a characteristic
empty-appearing “crescent” surrounds the periphery of
each lobule (represents dilated lymphatic channels)
Glomeruloid hemangioma
• Rare vascular proliferation seen in POEMS syndrome
(.multicentric Castleman disease); most common on
trunk/proximal extremities; does not clinically appear
different than common cherry angiomas
• a/w ↑ VEGF levels → vascular proliferation
• Histology (Boards favorite): well-circumscribed dermal
proliferation comprised of dilated vessels that are lled
centrally with a small ball of well-formed capillary
loops → resultant architecture resembles renal glomeruli
Glomus tumor/glomangioma
• Benign proliferations of perivascular epithelioid cells
derived from Suquet-Hoyer canal
• Glomus tumor (more common):
■
Solitary; painful; favors young adults; subungual
(most common site)
Fig. 6.43 Glomus tumor. Cytologically bland round tumor cells with uniform nuclei and sharp cellular membranes surround blood vessels. (From Buehler D,
Billings SD. Soft tissue tumors and tumor-like reactions. In: Busam KJ, ed. Der-
matopathology: A Volume in the Series: Foundations in Diagnostic Pathology
2nd ed. Philadelphia: Elsevier; 2016:513–594.)
■
Histology: dense proliferation of glomus cells
surrounding small vascular spaces (Fig. 6.43)
• Glomangioma/glomulovenous malformation (less
common):
■
Arises in infancy or childhood; frequently multiple
lesions, less painful than glomus tumors (may be
tender on palpation, pain may increase with
menstruation/pregnancy)
■
Histology: main feature is large, dilated vessels
surrounded by a smaller number of glomus cells
• Treatment: surgery is curative
Borderline vascular neoplasms
Kaposiform hemangioendothelioma
• Rare vascular tumor of childhood; a/w Kasabach-Merritt
phenomenon; GLUT-1 negative
• Violaceous plaque; becomes massively engorged when
Kasabach-Merritt occurs
■
Usually involves an extremity; can involve subcutis and
deeper tissues
■
Can be present in retroperitoneum and present as
ecchymoses
• Histology: nodules of densely packed spindle cells with
slit-like lumens (resembles nodular Kaposi sarcoma); may
resemble tufted hemangioma but has deeper involvement
■
Positive for CD34 and CD31; negative for Factor VIII
Kaposi sarcoma (KS)
• HHV-8 (present in 100%) induced vascular proliferation
w/ variable clinical behavior
• Clinical variants:
■
Classic KS: Mediterranean, Ashkenazi Jewish descent;
elderly males; initial lesions on distal extremities →
some progress to disseminated involvement
380
6.11 Vascular Proliferations
Dilated vessel
Promontory
Slit-like vascular space
Fig. 6.44 Kaposi sarcoma (medium mag). (From Rapini RP. Vascular proliferations and neoplasms. In: Practical Dermatopathology. 2nd ed. Elsevier; 2012:353–365.)
■
African endemic: young African males in endemic
regions; LN involvement and fulminant/fatal course
■
Iatrogenically immunocompromised: seen in patients
with organ transplants, cancer, and autoimmune diseases
■
AIDS-associated: most common in homosexual males;
solitary (trunk and midface common) or multiple
lesions; may disseminate
• Slowly growing violaceous patches, plaques, or nodules
• Histology:
■
Patch stage: subtle inltrative small vessels with bland
endothelium and associated plasma cells; RBC
extravasation 1 hemosiderin 1/– promontory sign
(Fig. 6.44)
■
Plaque stage: proliferation more pronounced and
extends deeper into dermis/subcutis with plasma cells
■
Nodular stage: cellular nodules of plump spindle cells
with slit-like lumina containing red blood cells (sieve-
like appearance); cells are never as atypical as those
seen in angiosarcoma; may have more ectatic vessels
in the periphery; plasma cells
• IHC: nuclear positivity for latency-associated nuclear
antigen (LANA-1) of HHV-8 is very helpful diagnostically
(100% sensitive and specic)
• Treatment: cryotherapy, laser surgery, PDT, topical
alitretinoin gel, and RT
■
Rapidly progressive KS w/ visceral involvement is
treated w/ systemic chemotherapy
Other borderline vascular neoplasms (rare; not commonly tested)
■
Dabska-type hemangioendothelioma, retiform
hemangioendothelioma (architecture resembles rete
testes), and epithelioid hemangioendothelioma
(1
vacuoles; t(1;3)(p36;q25) CAMTA1-WWTR1
rearrangement in 90%)
High-grade malignant vascular neoplasms
Angiosarcoma
• Cutaneous angiosarcoma seen in a variety of clinical
settings:
■
Elderly, sun-damaged sites (head/neck #1) (Fig. 6.45)
Fig. 6.45 Dark blue-purple plaques and nodules of angiosarcoma on the forehead and scalp of a 70-year-old man. The circular area is the biopsy site. (From
Schaffer JV, Bolognia JL. Vascular neoplasms. In: Callen JP, Jorizzo JL, Zone JJ,
Piette WW, Rosenbach MA, Vleugels RA, eds. Dermatological Signs of Systemic
Disease. 5th ed. Philadelphia: Elsevier, 2017:192–204.)
■
Stewart-Treves syndrome: chronic lymphedema
associated (mostly following breast cancer treatment
with axillary LN dissection)
■
Postradiation: most commonly on breast; arises after
RT for breast cancer
• Histology: large, hyperchromatic, pleomorphic tumor
cells dissecting between collagen bundles → forms
anastomosing vascular networks; endothelial cells lining
the vessels have “multilayered” or “piled-on” architecture
(many malignant endothelial cells crowded on top of
each other with some tumor cells oating freely inside the
lumen → this is never seen in benign vascular
neoplasms!); prominent hemorrhage (Fig. 6.46)
■
Poorly differentiated areas may have large epithelioid
cells which resemble carcinoma and lack clear vascular
differentiation
■
Immunostains: CD311, CD341, ERG1 (most
sensitive and specic), and FLI-11
381
CHAPTER 6 • Neoplastic Dermatology
Fig. 6.46 Angiosarcoma histopathology. Anastomosing dilated vessels, lined by
crowded endothelial cells, extend between preexisting collagen bundles. (H&Esaffron stain; original magnication: 40x) (From Karkouche R, Kerob D, Battistella M,
et al. Angiosarcoma in patients with xeroderma pigmentosum: less aggressive and
not so rare? J Am Acad Dermatol. 2013;69[3]:e142–e143.)
• Poor prognosis
• Treatment: surgical excision with wide margins, RT
• Boards fodder: c-MYC amplications (detected by
immunostaining or FISH) reliably distinguishes between
atypical vascular lesions (“AVLs,” which are negative) and
radiation-induced angiosarcoma (positive)
Vascular neoplasm associations
See Fig. 6.47
Angiolipoma
• Young adults; forearms (#1); often multiple lesions; painful
• Small subcutaneous nodules
• Histology: mature fat with areas containing proliferative
capillary lobules that are characteristically thrombosed
(→ hence painful) (Fig. 6.48)
• Benign; excision is curative
Spindle cell/pleomorphic lipoma
• Firm, subcutaneous nodule on posterior neck/shoulder
of adult males
• Histology: mature fat, areas of bland spindle cells with a
myxoid background and characteristic thick “ropey”
collagen bers (Fig. 6.49)
■
Pleomorphic lipoma: identical, except it additionally
has large “oret-like” cells (Fig. 6.50)
• Spindle cells are CD341, w/ RB1 loss (differentiates from
liposarcoma)
■
Part of the newly-described “RB1-deleted soft tissue
tumor family” (spindle cell lipoma, pleomorphic lipoma,
pleomorphic broma, and other less common entities)
• Benign; excision is curative
Hibernoma
• Benign tumors of brown fat
• Young adults; trunk and neck; slowly growing
subcutaneous nodules
• Histology: hibernoma cells (polygonal cells with
eosinophilic, multivacuolated cytoplasm) admixed with
normal appearing adipocytes
• Benign; excision is curative
• Boards tip: only the histology is likely to be tested
6.12 NEOPLASMS OF ADIPOCYTIC LINEAGE
Lipoma
• Benign neoplasm composed of mature adipose tissue;
subset with heterogeneous clonal aberrations involving
chromosome 12q13-15 region (→ ↑ HMGA2 gene) as
well as 6p21 and 13q
• Soft subcutaneous nodule; any anatomic site; typically
middle-aged adults
• Histology: well-circumscribed sheets of mature adipocytes
w/ minimal to no increase in brous tissue
• Conditions a/w multiple lipomas:
■
Familial multiple lipomatosis: AD; multiple painless
lipomas on trunk/extremities
■
Madelung disease: multiple large lipomas around neck/
shoulders; generally in middle-aged alcoholic males
■
Gardner syndrome
■
Bannayan-Riley-Ruvalcaba syndrome
■
Proteus syndrome
■
CLOVES syndrome (Congenital Lipomatous
asymmetric Overgrowth, Vascular malformations,
Epidermal nevi, Skeletal and spinal anomalies)
■
PTEN hamartoma tumor syndrome
Well-differentiated liposarcoma (atypical
lipomatous tumor)
• Uncommon in the skin; typically involves deep soft tissue
or retroperitoneum; large lesions that slowly enlarge
• MDM2 amplication (12q13-15 ring/giant chr) detected
in .99% → extremely sensitive and specic tool
• Histology: mixture of mature fat and brous bands
containing hyperchromatic atypical stromal cells (most
important nding); lipoblasts may be seen but are not
essential for diagnosis
• Prone to local recurrence; dedifferentiation to a high-
grade sarcoma can also occur
Myxoid/round cell liposarcoma
• Rare type of liposarcoma; most testable point is its
characteristic histology (buzzword: “chicken-wire” vessels)
6.13 DERMOSCOPY
• Synonyms: dermatoscopy, epiluminescence microscopy,
skin surface microscopy, and magnied oil immersion
diascopy
382
SELECTED VASCULAR NEOPLASM ASSOCIATIONS
383
Glomeruloid
hemangioma
Angiolymphoid
hyperplasia
Tufted
hemangioma
Kaposiform
hemangioendothelioma
Kaposi sarcoma
Fig. 6.47 Selected vascular neoplasm associations. HHV8, Human herpesvirus 8; HIV, human immunodeciency virus; IFE, immunoxation electrophoresis; PAS,
periodic acid–Schiff stain; SPEP, serum protein electrophoresis. (From North PE. Vascular neoplasms and neoplastic-like proliferations. In: Bolognia JL, Schaffer JV,
Cerroni L, eds. Dermatology. 4th ed. Philadelphia: Elsevier; 2018:2020–2049.)
Adults, trunk
and extremities
Adults, scalp region
Children and young
adults, trunk and neck
Less than 2 years old,
any location
Adults, face and trunk
if HIV-associated;
legs if non-HIV-associated
Glomeruloid-like
structures,
PAS-positive globules
Cobblestone endothelial
cells, eosinophils,
lymphocytes
Cannonball pattern
Slit-like lumina
Slit-like lumina HHV8, HIV
POEMS syndrome,
Castleman disease
Kasabach-Merritt
syndrome
SPEP, IFE of serum and
urine, endocrine tests
Eosinophilia
Thrombocytopenia
6.13 Dermoscopy
CHAPTER 6 • Neoplastic Dermatology
Vascular proliferation
Adipocyte
Thrombi
Fig. 6.48 Angiolipoma. (From Rapini RP. Miscellaneous remnants and neoplasms. In: Practical Dermatopathology. 2nd ed. Philadelphia: Elsevier; 2012:405–415.)
• An in vivo, noninvasive technique to enhance the color
and structure of the epidermis, DEJ and supercial dermis
→ reveals features that cannot be seen with the naked eye
• May enhance diagnostic accuracy (↑ sensitivity skin cancer
detection; ↓ benign malignant biopsy ratio)
• Helps distinguish melanocytic from nonmelanocytic lesions
• Dermoscopy colors of keratinizing, melanocytic, and
vascular tumors (see Fig. 6.51)
Fig. 6.49 Spindle cell lipoma. Low-power view of an encapsulated tumor composed of spindled cells with admixed foci of adipocytes. (From Brinster NK, Liu
V, Diwan H, McKee PH. Spindle cell lipoma. In: Dermatopathology: A Volume in
the High Yield Pathology Series. Philadelphia: Elsevier; 2011:530–531.)
Dermoscopic features of
nonmelanocytic lesions (see Table 6.8
and Figs. 6.52–6.59)
Seborrheic keratosis
• Milia-like cysts
• Comedo-like openings
• Fissures and ridges
• Moth-eaten borders
• Sharp demarcation
• Fingerprint-like pattern
Actinic keratosis
• Strawberry pattern
Basal cell carcinoma
• Leaf-like structures at periphery
• Blue-gray ovoid nests and globules
• Pigmented specks
• Spoke-wheel structures/concentric structures
• Arborizing (branch-like) telangiectasias (non-supercial BCCs)
• Serpentine vessels (supercial BCCs)
• Ulceration/erosion
Fig. 6.50 Pleomorphic lipoma. High-power view of oret giant cells. (From Brinster
NK, Liu V, Diwan H, McKee PH. Pleomorphic lipoma. In: Dermatopathology: A
Volume in the High Yield Pathology Series. Philadelphia: Elsevier; 2011:532.)
384
Squamous cell carcinoma in situ
• Atypical clusters of glomerulus-like (coiled) vessels
• Dark globules/globules in lines