6. Fully formed at birth, non progressive
Types:
Rapidly Involuting Congenital Hemangioma (RICH)
Non-Involuting Congenital Hemangioma (NICH)
GLUT-1 negative
Sites - extremities or post auricular skin, can occur elsewhere
7.
8.
9. TYPES
Juvenile capillary hemangioma
Most common type
Complete involution by age 6-7 yrs
C/F : purple to reddish macule or nodule centered in skin or
subcutaneous tissue
M/E : Lobules of capillary sized vessels supplied by feeder
vessel
Vascular space formations varies
10. Glucose transporter-1 (GLUT-1) - immunohistochemical marker
CD31 & CD34- endothelial markers & CD 133, expressed in
primitive cell - endothelial progenitor cells are involved in
pathogenesis.
Myeloid markers- CD14, CD15, CD32 and CD83.
14. Variants :
a) Lobular capillary hemangioma (Pyogenic granuloma)
Common proliferative lesion, related to trauma
Site- superficial dermis like lips, gums, hands, fingers, face of
pregnant women (epulis)
M/ELow power - organised pattern seen. lateral edges show lobular
arrangement – group of capillaries proliferate
High power –Small capillary epithelium lined spaces seen, also
surrounded by perithelial or pericytic layer of cells
15.
16. b) Tufted angioma:
Acquired in childhood (1-5 yrs), congenital forms exist
May persist unchanged or regress completely in few years
resembles cellular JCH but discontinuous capillary lobules.
17.
18. C) Cherry angioma :
suspected involuted LCH
Bright red, soft, raised, dome-shaped lesion of varying in size
often present in large numbers.
M/ENumerous capillaries with narrow lumina
Prominent endothelial cells, arranged in lobular fashion in
subpapillary region
Intercapillary stroma edema and collagen
19. Age and site same as JH but less frequent
No tendency to regress
Locally destructive due to pressure symptoms
M/E: Pattern of grouped dilated thin walled blood vessels
with inconspicuous endothelial lining
Associated Syndromes :
Blue-rubber-bleb nevus syndrome – cavernous hemangioma
of skin and GIT
Maffuci syndrome – Cavernous hamangiomas and
enchondromas
23. Wart like, dark blue papules or nodules
Predilection for distal lower limbs
Histology - mixture of cavernous and capillary vessel
immediately below hyperkeratotic and acantholytic
epidermis
24.
25. Small, reddish lesion in young to middle-aged
individuals
Favored sites- arms, trunk, legs
Thin, branching capillaries and small venules
widely throughout the dermis
Infiltration of arrector pili muscles by
proliferating vascular channels
26. Usually over trunk or extremities of young or middle-aged adults
Male predominance
Small solitary brown to violaceous papule surrounded by a thin,
pale area and a peripheral ecchymotic ring
27. Superficial reticular dermis- thin
walled, dilated, and irregular vascular
spaces
Lined by endothelial cells with scanty
cytoplasm and rounded nuclei that
protrude into the lumina, resembling
hobnails
Extensive red blood cell
extravasation, inflammatory
aggregates, extensive stromal
hemosiderin deposition seen
28. Develops in sun-exposed skin of forearms &
neck
Middle-aged to elderly patients
Small, red or blue, circumscribed &
asymptomatic plaque
Superficial, proliferation of capillaries in
background of solar elastosis
Capillary surrounded by a layer of pericytes
29. Solitary dark red papule or nodule on
face, lips, extremities of adults
Densely aggregated, thick & thin-walled
vessels lined by a single layer of
endothelial cells in dermis
Walls of vessels consist of fibrous tissue
Most of the blood vessels are veins
30. Young adults
Skin and subcutis
Distal extremities – hand
M/E : thin walled cavernous vessels lined by bland
flattened endothelium admixed with solid areas
composed of plump endothelial cells
Recurrence is common (> 50%) with discontinuous
growth pattern
34. Lower extremity – thigh
M/E : Poorly circumscribed, diffusely infiltrating mass in the
muscle composed of thick walled veins to cavernous
vascular spaces
Mitotic activity and intraluminal capillary tufting is seen
Freely anastomosing vascular channels are absent
36. Seen in women, 20-40 years
Small dull erythematous plaque in head and neck
M/E : -Well circumscribed nodule in the dermis or subcutis
less in deep tissue
- Vague lobular pattern of clustered small capillary sized
vessels around a feeder vessel
- Endothelial cells are plump with abundant cytoplasm
with impingement on lumen of vascular channel
“tomb stone appearance”
39. INTRAVASCULAR PAPILLARY ENDOTHELIAL HYPERPLASIA
o It represents an exuberant organisation and recanalisation of
thrombus
o Can occur in de novo (pure) form in extremities and head and
neck region or on a pre-existing vascular disorder (mixed) form
in the trunk region
o M/E- Exclusive intravascular nature with characteristic fibrinous
or hyaline appearance of the papillary stalks
Residual organising thrombi are often seen.
41. Poorly circumscribed diffuse network of vascular structures
within a soft tissue
Seen in childhood or adolescence
presents as deep soft tissue swelling
Two patterns :
Mixed vessel type lesion resembling intramuscular angioma
Capillary predominant type lesion with lobular pattern
42. An uncommon deep soft tissue tumor
Seen in children and teenagers
Associated with Kasabach Meritt syndrome especially if
retroperitoneal mass > 20 cm
M/E : - Lobular architecture, Highly cellular
In Individual lobules there is capillary sized vessels with
absence of mitosis or cytologic atypia
- Microscopic features overlap with hemangioma
43. Early lesions – ecchymotic macules or patches
Later lesions – bluish purple papules,nodules, plaques or tumors
Regardless of type, it is Borderline malignancy with slowly
progressive but may involve internal organs
M/E : Early lesions – show dermal proliferation of irregular slit
like vascular channels with extravasated erythrocytes,
hemosiderin and plasma cells.
44. The vascular channels infiltrate between collagen bundles
and surround existing blood vessels (promontary sign) and
appendages
Endothelial cells are plump or inconspicuous with no
significant cytologic atypia
Later stage – Increased spindle cells between poorly
defined slit like vessels
Tumor cells – Intracytoplasmic PAS positive hyaline globules
45.
46.
47. Skin of distal extremities
Young adults
Gross : Reddish purple, slowly growing plaque centered in
reticular dermis, < 2-3 cm
M/ E : Elongated, arborizing narrow vessels that resemble rete
testis .
Endothelial cells are monomorphic with low mitotic rate,
hyperchromatic nuclei and hobnail morphology
Sarcoma between vascular channels is prominent aand shows
abundant lymphocytic infiltrate
48.
49. seen in infants and children in skin
Slow growing nodule or plaque
M/E : Thin walled vascular spaces that contain intraluminal
papillary tufts of endothelial cells within hobnail morphology
are present
50.
51. Mixture of benign, intermediate or
malignant histologic patterns
Long standing red-blue nodules or plaques
on hands & feet in adults
Poorly circumscribed, with infiltrative
growth pattern
Different componentsRetiform, Epithelioid, Spindle cell,
Angiosarcoma, Lymphangioma,
Arteriovenous malformation
52. Primary sites: Superficial and deep soft tissue, viscera (liver),
bone
Gross : poorly circumscribed, multilobular infiltrative mass
upto 10 cm or as multiple masses.
M/E : seen as Cords, short strands, solid nests or individual
cells that have rounded to slightly spindled features
The cells are low grade with a low mitotic rate
Endothelial differentiation is evident by formation of
intracytoplasmic lumina(signet ring like features)
53. Distinct vascular channels are not prominent
Neoplastic cells are embedded into chondroid like hyalinized
stroma
Atypical morphological features :
Increased mitotic rate
increased nuclear pleomorphism
more spindled cytology, necrosis
IHC : tumor cells express – CD13, CD34, Ulex Europaeus
antigen, variably express Factor XIII related antigen
54.
55. It is divided into several groups:
Cutaneous angiosarcoma
Angiosarcoma of breast
Radiation induced angiosarcoma
Angiosarcoma of deep soft tissue
Gross : Poorly circumscribed hemorrhagic mass from 1-2 cm to
> 10 cm
M/E : Varies from hemangioma like features but with
scattered, enlarged, atypical endothelial cells with occasional
mitotic figures and infiltrating growth pattern to that of high
grade spindle cell sarcoma
59. Variant
Epithelioid Angiosarcoma
seen in deep soft tissue
Malignant epithelioid cells with abundant eosinophilic or
amhophilic cytoplasm, large vesicular nuclei and prominent
nucleoli
62. LYMPHANGIOMA
Cavernous or cystic vascular lesions composed of dilated
lymphatic channels
Seen in Head and neck region in young children
M/E : Thin walled dilated lymphatic vessels of varying sizes
lined by flattened endothelium, beneath which are lymphoid
aggregates
Can be Mediastinal or retroperitoneal
Lung tumor – multiple, stellate lesions with slips of smooth
muscle around spaces filled with proteinaceous lymph fluid
67. Lymphangiosarcoma is a rare malignant tumor which occurs in
long-standing cases of Primary or Secondary Lymphedema
SITE- upper extremities
Presents as a bluish or purplish skin discoloration or tender skin
nodule. Often multiple.
It progresses to an ulcer with crusting, and finally extensive
necrosis.
Postmastectomy Lymphangiosarcoma (Stewart-Treves
Syndrome)
68. HistologyThere is an admixture of spindle cells and vascular spaces
deformed by pleiomorphic endothelial cells.
Lymphangiosarcoma cells express positive endothelial markers CD34, vimentin, keratine, VIII factor antigen
It metastasises rapidly to the lungs, chest walls, liver and or bone
and the reoccurrence rate is high
69.
70.
71. GLOMUS TUMOR
Originates in the neuromyoarterial glomus
Classically located in subungual region but may also be found in
skin, soft tissue, nerves, stomach, nasal cavity and trachea
Tend to be multiple and of infiltrative nature in children
Presents as varicosities of lower extremities
Superficial lesions are well circumscribed
72. M/E- consist of blood vessels lined by normal endothelial cells
and surrounded by a solid proliferation of round or cuboidal
“epithelioid” cells with round nuclei and acidophilic cytoplasm
IHC- positive for myosin, vimentin, actin, and basal lamina
components but not for desmin
Microscopic types of glomus tumor:
solid
Angiomatous
Myxoid
75. Sternberg’s Diagnostic Surgical Pathology – 5th edition
Rosai and Ackerman’s Surgical Pathology – 10th edition
Robbins and Cotron Pathologic Basis of Disease – 8th edition
internet
Editor's Notes
Most accepted Classification of vascular tumors is the WHO classification which divides vascular tumors into benign , intermediate and malignant tumors.Rapidly Involuting Congenital Hemangioma (RICH) Non-Involuting Congenital Hemangioma (NICH)
Hemangiomas are Most common cutaneous and soft tissue tumor of infancy and childhood(7% of benign soft tissue tumor)
Gross= Raised, violaceous tumor with large, radiating veins or with overlying telangiectasia and a halo of pallor M/E=Architecture is lobularBands of fibrosis, focal inflammation, dystrophic calcification, hemosiderin depositionLobules - congested capillaries, surrounded by a layer of pericytes
Gross= Flatter than RICH, well-circumscribed round to ovalSlightly indurated or raised soft-tissueMass with overlying telangiectasias and a rim of pallor.M/E=Vascular lobules are large Composed of larger, thicker blood vessels
M/E= Minimal with cellular lobules and scattered mitotic figures to obvious luminal formation
immunohistochemical marker is Glucose transporter-1 (GLUT-1) ..
Low power= A thin collagen layer surrounds each lobule Arrangement is disrupted at base focal anastomosing channelsHigh power= Small frequent mitosis and enlarged endothelial nuclei. Superficial region of ulceration and acute inflammation – neutrophils abound superficial lobulesLacks steroid hormone receptors
Gross= Solitary, red, rapidly growing papule or nodule, with a subtle collarette of scaleM/E=Polypoid mass of angiomatous tissue protruding of skin. Superficial inflammatory cell reaction - appearance of granulation tissueSurrounded by myxoidstroma, scattered spindle & stellate shaped connective tissue cells Dilated network of blood-filled capillary vessels
JCH- juvenile capillary hemangioma
Gross= Large, plaque-like, infiltrated, red or purple lesionM/E= Cannonball appearance- vascular tufts of tightly packed capillaries, randomly dispersed in the dermis
LCH- lobular capillary hemangioma
JH-juvenile hemangioma…………Those occuring in the skin are traditionaly known as port wine nevus or nevus flammeus.
shows Large, irregular spaces containing red blood cells and fibrinous material lined by a single layer of thin endothelial cells
It is a cavernous hemangioma containing dilated inter connecting thin walled channels with occasional pseudopapillary projections.
VerrucuoushemangiomaGross-dark blue papules or nodules seen on legm/e= Congested capillaries and cavernous vascular spaces extend into the deep dermis and subcutaneous tissue Lined by flattened endothelial cells Lobular growth pattern in deeper component
Common in young adults,,,sites are--
Sites---
There isAngiomatoid hyperplasia with eosinophilia
Also called as Massons hemangioma or massons lesion. not a true neoplasm but has capacity to simulate microscopically vascular tumor
Mature adipose tissue may be intermixed
It is Multinodular, poorly circumscribed, infiltrating tumorLethal in 20 % due to assoiatedcoagulopathy
1st slide shows AIDS associated kaposi sarcoma2nd slide shows Kaposi sarcoma in Immunocompromised patient
It is Uncommon neoplasm..sites is skin of distal extremities…seen in young adults.
Picture showing elongated, arborizing blood vessels lined by monomorphic endothelial cells with prominent nuclei and scanty cytoplasm
Picture showing Markedly dilated, thin-walled vascular channels Lined by endothelial cells with protruding nuclei & very scanty cytoplasmProminent intra- and extravascular lymphocytic cell infiltrate Intravascular papillae with collagenous cores
Intravascular bronchioloalveolar tumor seen in All ages, rare in early childhoodLow grade malignant endothelial neoplasm. 10 % multiorgan disease at presentation
In 25-30% cases - focal cytokeratin expression – lead to misdiagnosis
Tumor cells are arranged in short fascicles, small nests, “indian file” pattern Hyalinized or mucoidstroma can be seen. No obvious vascular channels Intracytoplasmic vacuoles containing erythrocytes can be seen
*Rare malignancy of endothelial cellsIntraluminal papillae lined by hyperchromatic cellsVascular channels with a dissecting pattern of infiltration through dermis or surrounding tissue – is of concern
Picture shows
Results from failure of the lymphatic system to communicate with the venous systemAsso with tuberous sclerosis - TSC2 mutationsChylous pleural effusion and ascites – exclusively seen in females.They can Replace local lymph nodes. show Pericytomatous histology
Present in deep soft tissue
Cystic lymphangioma has been traditionally called hygroma.
Picture showing
Amputated upper extrimity in postmastectomylymphangiosarcoma
Post-mastectomy lymphangiosarcoma showing an intricate network of neoplastic vessels.
Also called glomangioma
The distribution of round glomus cells around the open vascular lumen is a key to the diagnosis.
