1. DR. SAURAV SINGH

2. Congenital hemangiomas
RICH
NICH
Capillary hemangioma
Variants:
Juvenile hemangioma
Cherry angioma
Tufted angioma
Lobular capillary
hemangioma(pyogenic
granuloma)
Cavernous hemangioma
Verrucous hemangioma
Microvenular
hemangioma
Hobnail hemangioma
Epithelioid hemangioma
Acquired elastotic
hemangioma
Arteriovenous hemangioma
Angiomatosis
Spindle cell hemangioma

3. Tumor-like Conditions
Papillary endothelial hyperplasia (Masson's tumor)
Reactive angioendotheliomatosis
Glomeruloid hemangioma
Bacillary angiomatosis

4. Locally Aggressive
Kaposi-like hemangioendothelioma
Giant cell angioblastoma
Rarely Metastasizing
Kaposi's sarcoma
Retiform hemangioendothelioma
Papillary intravascular angioendothelioma
Composite hemangioendothelioma

5. Epithelioid hemangioendothelioma
Angiosarcoma
Idiopathic (head and neck)
Associated with chronic lymphedema (Stewart-Treves)
Postirradiation
Epithelioid

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

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.

11. Infantile hemangioendothelioma showing vaguely lobulated architecture

12. High cellularity and high mitotic activity of juvenile hemangioendothelioma should not
lead to overdiagnosis of malignancy

13. Perineural spread of juvenile hemangioendothelioma

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

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.

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

20. Cavernous hemangioma of soft tissue of orbit

22. Sinusoidal hemangioma : the vascular spaces are widely dilated

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

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

31. Spindle cell hemangioma : Cavernous hemangioma like area

32. Kaposi sarcoma like area in the same

33. Chracteristic spongy low power appearance

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

35. Skeletal hemangioma

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”

37. Epithelioid hemangioendothelioma – The tumor partially fills the lumen of femoral

38. Prominent cytoplasmic vacuolation is seen on high power examination

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.

40. Intravascular papillary endothelial hyperplasia.

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

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

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

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

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

56. Gross : Hemorrhagic appearance of angiosarcoma of lip

57. Angiosarcoma of mediastinal mass : Anastomosing vascular channels

58. vascular Channels are seem to be ,lined by highly atypical endothelial cells

59. Variant
Epithelioid Angiosarcoma
seen in deep soft tissue
Malignant epithelioid cells with abundant eosinophilic or
amhophilic cytoplasm, large vesicular nuclei and prominent
nucleoli

60. Epithelioid angiosarcoma arising form region of seminal vesicle

61. Ulex europaeus lectin 1 reactivity in angiosarcoma

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

63. Cavernous lymphangioma

64. Large cystic hygroma in an infant

65. Lymphangioma of soft tissue showing dilated spaces lined by flattened endothelium.
A scattering of lymphocytes is present in stroma

66. Microscopic appearance of lymphangioma

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

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

73. Glomus tumor

74. Immunoreactivity for smooth muscle in glomus tumor

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