1. Tumors and tumor-like
conditions of blood and
lymph vessels
Sanjay bhardwaj

4. BENIGN TUMORS AND TUMOR-LIKE CONDITIONS
HEMANGIOMAS
Hemangiomas, are the most common tumors of the head and
neck in infancy and childhood, comprising approximately 7% of
all benign soft tissue tumors
A benign skin lesion consisting of dense,
usually elevated masses of dilated blood vessels.

5. Types of Hemangiomas:
Strawberry(capillary) Hemangioma
Cavernous (Deep) Hemangioma
Pyogenic granuloma

6. Capillary Hemangioma
The most common variant
Site : skin, subcutaneous tissues, and mucous
membranes of the oral cavities and lips, liver, spleen,
and kidneys salivary gland and breast.
.

7. The “strawberry type” or juvenile hemangioma [benign
hemangioendothelioma /hyperplastic hemangioma] ,is the
most distinctive and common lesion of the skin of newborns
is extremely common (1 in 200 births), fades at 1 to 3 years
of age and completely regresses by age 7 in 75% to 90% of
cases.

9. Aggregates of closely packed, thin-
walled capillaries, usually blood-filled
and lined by flattened endothelium;
vessels are separated by scant
connective tissue stroma.

10. immunohistochemically
positivity for FVIII, CD31, CD34, FLI-
1, estrogen receptor beta, annexin II

11. Cavernous (Deep) Hemangioma
deeply situated red-blue spongy mass of tissue filled with blood found on 2
out of 100 babies
Deep hemangiomas involve muscle or visceral organs and, are more
difficult to diagnose. Therefore, further diagnostic studies are required.
Brain hemangiomas are most problematic, because they can cause pressure
symptoms or rupture. Cavernous hemangiomas are a component of von Hippel-
Lindau disease

13. A large cavernous hemangioma of the liver in a 28-year-old man.

14. large, cavernous blood-filled vascular
spaces, separated by a modest
connective tissue stroma

15. Pyogenic Granuloma. :
 It is rapidly growing pedunculated red nodule on the skin, or gingival
or oral mucosa; it bleeds easily and is often ulcerated
 associated with history of trauma
 Pregnancy tumor (granuloma gravidarum) is a pyogenic granuloma
that occurs infrequently (1% of patients) in the gingiva of pregnant
women.
These lesions can spontaneously regress.

16. Clinical appearance of typical pyogenic granuloma

17. capillary or vascular lobule

18. Newly formed capillaries embedded in a
loose edematous stroma which has been
infiltrated with chronic inflammatory cells
Fibrosis develops and the small
capillaries disappear, leaving a dense
plug of moderately healthy cellular
fibrous tissue

19. They occur in the back, gluteal region, thigh, and other sites;
sometimes an entire extremity is involved.
The association of varicose veins, (dysplastic) cutaneous
hemangiomas, and soft tissue and bone hypertrophy is known as
Klippel–Trenaunay syndrome.
Large-vessel hemangiomas

20.  composed of veins (venous hemangiomas) or a combination of
veins and arteries (racemose, cirsoid, or arteriovenous hemangiomas).
 Venous hemangiomas of the feet have been seen in patients with
Turner syndrome

21. The mass consists of thick-walled irregular
vascular channels with smooth muscle
walls of varying thickness, thus suggesting
a venous hemangioma
Staining for CD34, a vascular endothelial
marker, reveals strong reactivity in the
flattened luminal-lining endothelial cells,
confirming the tumor’s vascular origin.

22. Skeletal muscle (intramuscular) hemangiomas
venous or cavernous microscopic appearance
these very cellular, with plump nuclei, mitotic figures,
intraluminal papillary projections, and even infiltration of perineurial spaces

23. Masson heMangioMa or Masson lesion/
intravascular papillary endothelial hyperplas
•It represent exuberant organization and recanalization of a thrombus
•Occur in previously normal vessels or in varices, hemorrhoids, hematomas,
pyogenic granulomas, hemangiomas,
•Found in the extremities (particularly the fingers) and the head and neck region,
whereas the type engrafted on a pre existing vascular disorder tends to be in the
trunk.
•It simulates malignant vascular tumors

24. The confinement of the lesion to the vascular lumen and the hyaline core of the
papillae are characteristic features.

25. Spindle cell hemangioma/spindle cell hemangioendothelioma
A benign endothelial neoplasm.
Occur at any age, has a male predominance,occurs
preferentially in the dermisand subcutaneous tissue of the distal extremities,
Some cases have occurred in patients with Maffucci or
Klippel–Trenaunay syndrome

26. characteristic ‘spongy’ low-power appearance

27. cavernous hemangioma-like area Kaposi sarcoma-like area

28. Hobnail hemangioma.
The endothelial cells protrude into the
vessel lumina

29. Intraosseous Hemangioma

30. Clinical presentation : Hemangiomas
On examination, the superficial hemangioma usually consists of a
raised, reddish to purple tumor with a distinct margin.
In contrast, deep subcutaneous hemangiomas often have a deep bluish
hue with normal overlying skin, making diagnosis more difficult.
Both the lesions are firm to palpation and do not pulsate or exhibit any
thrills or bruits.

31. Investigations : Hemangiomas
•Computed tomography (C. T .Scan) and Magnetic resonance imaging ( M. R.I)
imaging techniques are used as diagnostic aids to document the extent of the
deep hemangiomas
"popcorn" pattern in case of cerebellaum cavernous hemengioma

32. •Arteriography:
rarely indicated for the diagnosis of a hemangiomas.
•Biopsy :for final diagnosis

33. Consider Treatment
• Treatment should be
considered if the
hemangioma…. • ulcerates
• bleeds
• causes functional impairment
• causes infection
• grows rapidly and
uncontrollably
• causes psychological problems

34. Treatments of Hemangiomas
Medical
• steroid injection
• interferon alfa-2a
Surgical
• resection
• FPDL
• YAG laser

35. Medical Treatments
Interferon Alfa-2a
benefit in inhibiting angiogenisis and stimulate endothelial cell
prostacyclin formation, which prevents platelet trapping
interferon alfa-2a is administered in daily subcutaneous injections of 1 to
3 million units per square meter of body surface area for an average of 7
months of therapy
acute side effects, which are reversible, include fever, chills, arthralgias
and retinal vasculopathy

36. Surgical Procedures
Resection
surgical excision is occasionally advocated as the primary treatment of
hemangiomas
resection surgically removes all or part of the tissue
indicated as the management of visceral lesions unresponsive to steroids
used for the cosmetic revision of redundant skin remaining after
spontaneous involution of deeper hemangiomas

37. Surgical Procedures
FPDL - flashlight-pumped pulsed dye laser
treatment of choice for superficial strawberry hemangiomas with a
response rate of 60 percent
penetrates to a depth of 1.8mm and has a low risk of scarring
local anesthetic is effective in reducing pain or discomfort and some
bruising may occur
several laser sessions may be needed to achieve optimal improvement

38. Surgical Procedures
YAG Laser
treatment of choice for rapidly growing deep or mixed hemangiomas with
a response rate of 75 percent
penetrates to a depth of 5 to 6 mm, although scar formation is more
frequent than with the FPDL since the laser penetrates deeper into the
skin
requires local or general anesthesia
not recommended in the initial treatment of cutaneous hemangiomas

39. Lymphangiomas
Lymphangiomas are the benign lymphatic analogues of blood vessel hemangiomas
Simple (Capillary) Lymphangioma.
These are composed of small lymphatic channels predominantly occurring in the
head, neck, and axillary subcutaneous tissues. They are slightly elevated or
sometimes pedunculated lesions up to 1 to 2 cm in diameter.

40. Cystically dilated vascular spaces are lined by flattened endothelial cells. Red
blood cells are nearly absent

41. Simple (Capillary) Lymphangioma.

42. Cavernous Lymphangioma (Cystic Hygroma):
o These lesions are typically found in the neck or axilla of children,
and rarely occur in the retroperitonem.
o Cavernous lymphangiomas can occasionally be enormous (up to 15 cm)
and may fill the axilla or produce gross deformities about the neck.

43. Large cystic hygroma in an infant.

44. The image shows lymphatic spaces lined by attenuated endothelium. The cyst
walls contain lymphoid aggregates.

45. Glomus Tumor (Glomangioma)
Benign, painful tumors.
 arising from modified smooth muscle cells of the glomus body, a
specialized arteriovenous structure involved in thermoregulation.
 They are most commonly found in the distal portion of the digits, especially
under the fingernails
. Excision is curative.

46. Glomus tumor lesions are round, slightly elevated, red-blue, firm nodules
(usually <1 cm in diameter)

48. aggregates, nests, and masses of specialized glomus cells intimately associated
with branching vascular channels, all within a connective tissue stroma. Individual
tumor cells are small, uniform, and round or cuboidal, with scant cytoplasm

49. Immunohistochemically, they shows
reactivity for myosin, vimentin, actin,

50. Vascular Malformations/ Vascular ectasias
• Vascular malformations are present at birth and unlike
hemangiomas, do not go through a “rapid proliferation and
they do not “involute”.
• They grow gradualy with the patient.

51. Vascular Malformations - Types
• Vascular malformations may be capillary, venous, arterial, or
combinations of these.
• Approximately 31% of these malformations are found in the
head and neck region.

52. Vascular Malformations types
• Vascular Malformations are divided into two categories:
Low-flow and High-flow lesions.
• Capillary, venous, and lymphatic malformations exhibit “low
flow lesions”.
• Arterial and arterio-venous malformations exhibit “high flow”
and are capable of severe hemorrhage with significant
morbidity.

53. Venous Malformations - Low-flow lesions
• Venous malformations are bluish, soft
and easily compressible, and
auscultation reveals no bruits.
• These malformations can vary from
superficial, localized, mucosal spongy
ectasis to complex invasive lesions that
permeate tissue planes and alter the
regional anatomy.

54. The masses enlarge with physical activity or a dependent position.
Venous malformations can be painful in the morning due to stasis
and microthrombi within the veins.
Venous malformations’ localization is usually in the head and neck.
Venous malformations are the most common vascular anomaly, they
are 40% of all vascular malformations
Venous malformation can be treated with sclerotherapy and surgical
reduction

55. at histological examination showing disorganized muscular wall of veins blending
with surrounding soft tissues

56. Capillary Malformations - Low-flow lesions
Capillary malformation (also known as port-wine stain)/Nevus Flammeus:.
flat, reddish lesions affect the skin of the head and the neck
 They darken with age.
Syndromes associated are: Sturge-Weber syndrome and Klippel-Trenaunay
syndrome
.Capillary malformations can be treated with IPL-(Intensed-pulsed-light)-therapy
or surgical reduction.

58. collections of haphazardly arranged dilated
vessels in the papillary and reticular dermis

59. mononuclear cell infiltrate with occasional
dilated blood vessels within the superficial
dermis.
Spider Telangiectasia.

60. Hereditary Hemorrhagic Telangiectasia (OslerWeber-Rendu Disease).
autosomal dominant
Telangiectasias are Present from birth, over the skin and oral mucous
membranes, the respiratory, gastrointestinal, and urinary tracts.
 These lesions rupture, causing serious epistaxis , GI bleeding, or hematuria.

61. Lymphatic Malformations - Low- flow lesions
• Lymphatic malformations are normally colorless;
however, combined lesions take on the hue of the
additional vessel typ.
• These malformations can become invasive by dissecting
along tissue planes and can cause bony hypertrophy,
distortion or both. As these are lymphatic tissues,
infections may result in rapid enlargement of these lesions.

62. Arterial / Arteriovenous Malformations
• Clinically these lesions appear stained, warm and tender to
palpation. There may be swelling or asymmetry.
• A bruit may be detected.

63. Arteriovenous Malformations

64. Reactive vascular proliferations: Bacillary angiomatosis
Skin lesions are red papules and
nodules,
acute neutrophilic inflammation and
vascular (capillary) proliferation.

65. INTERMEDIATE-GRADE (BORDERLINE) TUMORS
Chronic KS (also called classic or European KS) :
•it is not associated with human immunodeficiency virus (HIV).
•it associated with an underlying second malignancy or altered .
immunity,
• it presents with multiple red to purple skin plaques or nodules,
usually in the distal lower extremitie.
• asymptomatic and remain localized to the skin and subcutaneous
tissue
Kaposi Sarcoma

66. Lymphadenopathic KS (also called African or endemic KS):
 it is also not associated with HIV. No Skin lesions are sparse, and
patients present instead with lymphadenopathy .
 the tumor occasionally involves the viscera and is aggressive.
Transplant-associated KS :
occurs in the setting of solid-organ transplantation
mucosal, and visceral involvement; cutaneous lesions may be
absent.

67. AIDS-associated (epidemic) KS
AIDS-associated KS can involve lymph nodes or viscera
and disseminates widely early in the course of the disease
found in a third of AIDS patients, particularly male homosexuals
Pathogenesis
human herpesvirus-8 (HHV-8) or KS-associated herpesvirus (KSHV)
it is transmitted sexually and by poorly understood nonsexual
route—perhaps including saliva

68. three stages are recognized:
Patches are red to purple macules typically confined to the distal
lower extremities
With time, lesions spread proximally and become larger,
violaceous, raised plaques
Eventually, lesions become nodular and more distinctly neoplastic

69. coalescent red-purple macules and
plaques of the skin
Histologic appearance of nodular form,
demonstrating sheets of plump,
proliferating spindle cells.

70. Elongated spindle cells showing minimal atypia are separated by slits
containing red blood cells

71. Hemangioendothelioma
vascular tumors of an endothelial nature that occupy an intermediate position
between the benign hemangioma and the full-blown angiosarcoma.
The tumor partially fills the lumen of the
femoral vein
Epithelioid hemangioendothelioma
cytoplasm is abundant and eosinophilic,
predominantly vacuolated

72. Malignant endovascular papillary angioendothelioma
(Dabska tumor; papillary intralymphatic angioendothelioma)
papillary tufts that are lined by plump endothelial cells located within dilated
vascular lumina, some of which have a glomeruloid configuration

73. Retiform Hemangioendothelioma
'long arborizing vessels' with features reminiscent of rete-testis ; focal solid
areas composed of spindle and epithelioid cells may be present.

74. MALIGNANT TUMORS
Angiosarcoma
Angiosarcomas are malignant endothelial neoplasms
 Older adults are more commonly affected
 Equal gender predilections
They occur at any site but most often involve skin, soft tissue, breast, and liver.
angiosarcomas are locally invasive and can metastasize readily.
Angiosarcomas are aggressive tumors with current 5-year survival rates of 30%.

75. angiosarcoma of the heart (right ventricle)
large, fleshy masses of red-tan to gray-
white tissue

76. dense clumps of irregular,
moderate anaplastic cells and
distinct vascular lumens
CD31 positivity

77. Hemangiopericytoma
Hemangiopericytomas derived from pericytes-myofibroblast-like cells that are
normally arranged around capillaries and venules.
they can occur as slowly enlarging, painless masses, common on the lower
extremities and in the retroperitoneum..
The tumors may recur after excision, and roughly half will metastasize, usually
hematogenously to lungs, bone, or liver.

78. consist of numerous branching capillary channels and gaping sinusoidal
spaces enclosed within nests of spindle-shaped to round cells.