1. Prof A of colorectal surgery
Hemangioma and
vascular malformation
Walid El Shazly
MD of general surgery,
Prof A of colorectal surgery

2. Hemangioma and vascular malformation
Hemangiomas and vascular tumors
Infantile hemangiomas
Congenital hemangiomas
Intramuscular hemangioma
Non involutional hemangioma
Kaposiform hemangioendothelioma

3. Vascular malformations
High flow
Arteiovenous malformations (AVM)
Arteiovenous fistula (AVF)
Low flow
Capillary malformation (portwine stain)
Venous malformation (cavernous hemangioma)
Lymphatic malformation
Lymphaticovenous malformations
Combined lesions
Klippel Trenaunay syndrome
Parkes Weber syndrome
Proteus syndrome
Maffucci syndrome
Blue Rubber Bleb Nevus syndrome
Cobb syndrome

4. Infantile hemangioma
Infantile hemangioma is a benign neoplasm that commonly develops in neonates within their first few months of life.
Most infantile hemangiomas undergo rapid initial proliferation, with a subsequently plateau in infants aged about 9-10 months;
finally, they become involuted. The involuting phase extends from 1 year until 5 to 7 years of age.

5. Infantile hemangioma
Hemangiomas generally affect the head and neck (80-85%).
The trunk and extremities are less commonly involved.
Most hemangiomas are easily diagnosed without any additional diagnostic tests (eg, MRI).

7. strawbarry birthmark

8. strawbarry birthmark

9. deep haemangioma

12. Congenital hemangioma or Rapidly Involuting Congenital Hemangioma (RICH)
(RICH) is a similar benign tumor (birthmark) to infantile hemangioma, but it differs from infantile hemangioma in that it has a fully developed presentation at birth, with rapid involution and regression.
Overall, congenital hemangioma is a rare entity.

13. Rapidly Involuting Congenital Hemangioma

14. Intramuscular hemangioma
Intramuscular hemangioma is a rare condition;
It differs from infantile hemangioma simply because it occurs in patients older than children.

15. Kaposiform Hemangioendothelioma (KHE)
This is a vascular tumor associated with the Kasabach-Merritt phenomenon (severe coagulopathy due to platelet trapping and spontaneous bleeding).
These tumors are histopathologically distinct from the common hemangiomas of infancy. 
KHE may be present at birth in neonates, or they can develop in infants within the first few months after birth.
An ill-defined purpuric mass is a common presentation. A truncal location is the most common site.
Destructive bone changes in adjacent bones are common. 

16. Kaposiform Hemangioendothelioma (KHE)

17. Vascular malformations
High flow
Arteiovenous malformations (AVM)
Arteiovenous fistula (AVF)
Low flow
Capillary malformation (portwine stain)
Venous malformation (cavernous hemangioma)
Lymphatic malformation
Lymphaticovenous malformations
Combined lesions
Klippel Trenaunay syndrome
Parkes Weber syndrome
Proteus syndrome
Maffucci syndrome
Blue Rubber Bleb Nevus syndrome
Cobb syndrome

19. Portwine stain (Capillary hemangioma)
Venual Malformations (Port Wine Stains) are always present at birth and can range from pale pink to dark purple in color.
In the past these lesions were erroneously called "capillary hemangiomas."
Port Wine Stains occur in 3% of births and occur equally among males and females.

20. Portwine stain

21. Portwine stain

22. Portwine stain

23. Portwine stain

24. Portwine stain

25. Portwine stain

26. Venous malformation
Venous Malformations is an abnormality of the larger, deep venins and is often called a "hemangioma."
Some actually look like hemangiomas but a good history will reveal whether or not the lesion is a hemangioma or vascular malformation.

27. Venous malformation
A venous malformation can be deep or superficial, localized or diffused.
The closer the vessels are to the surface, the deeper the color to the eye. A very deep lesion will have no color but will show a protruding mass.
The jaw, cheek, tongue and lips are common sites for a venous malformation.
These lesions are soft to the touch, the color disappears and empties as the lesion is compressed.
When the child cries or is lying down, the lesion expand, the vessels fill and the color becomes more intense.
The natural history is a slow-steady enlargement.

31. Venous malformation
Regardless of how small it is upon detection, it will grow.
Certain things can cause them to grow more rapidly such as serious sickness, trauma, infection, hormone changes (puberty, pregnancy, menopause).
Partial removal is not recommended since these lesions will just grow back.

32. Lymphatic Malformations
Lymphatic Malformations used to be called cystic hygroma, hemangiolymphangioma, or lymphangiomas.
The lymphatics serve as a collection and transfer system for tissue fluids. When something disturbs this system, a lymphatic malformation is formed.
The excess fluid accumulates and the affected lymphatic vessels enlarge and you see a mass.

33. Cystic Hygroma
Cystic Hygroma

34. Lymphatic-venous malformations
Lymphatic-venous malformations (lymphatic and venous birthmarks)  are low-flow malformations and contain both abnormal lymphatic and venous channels.
In most patients, the lymphatic portion of the malformation (birthmark) appears dominant on imaging studies.
These birthmarks may be scattered in one extremity or may be a focal malformation.

35. Lymphatic-venous malformations

36. Vascular malformations
High flow
Arteiovenous malformations (AVM)
Arteiovenous fistula (AVF)
Low flow
Capillary malformation (portwine stain)
Venous malformation (cavernous hemangioma)
Lymphatic malformation
Lymphaticovenous malformations
Combined lesions
Klippel Trenaunay syndrome
Parkes Weber syndrome
Proteus syndrome
Maffucci syndrome
Blue Rubber Bleb Nevus syndrome
Cobb syndrome

37. Klippel-Trenaunay syndrome
Klippel-Trenaunay syndrome is a slow-flow combined vascular anomaly (capillary-lymphatic-venous malformation) that is typically associated with marked overgrowth of the leg and geographic capillary stains. 
The condition may rarely be associated with hypotrophy.
Anomalous lateral veins, which are typically on the lateral aspect of the thigh, become prominent because of incompetent valves and deep venous anomalies. 

38. Klippel-Trenaunay syndrome

39. Parkes- Weber syndrome
This rare fast-flow combined vascular malformation usually involves a lower limb, and it is usually associated with a geographic stain over the enlarged limb.
Symptoms include cutaneous warmth and a bruit or thrill on clinical examination, all of which are more suggestive of a complex vascular malformation than a simple CM.
MRIs and MRAs show enlarged extremity muscles and bones with an abnormal signal intensity and contrast enhancement pattern; they also show generalized arterial and venous dilatation in the involved extremity.

40. Parkes- Weber syndrome

41. Maffucci’s syndrome
Maffucci’s syndrome, a rare condition, characterized by enchondromas (benign cartilage tumors), bone deformities and venous malformations.
No racial, sexual predilection or familial pattern of inheritance has been shown.
Maffucci syndrome usually manifests early in life, usually around the age of 4 or 5 years.  

42. Proteus syndrome
Proteus syndrome is a rare complex condition, characterized by a variety of cutaneous and subcutaneous lesions including vascular malformations, lipomas, hyperpigmentation, and several types of nevi.
The major clinical features of this rare vascular anomaly include verrucous nevus, lipomas and/or lipomatosis, macrocephaly, asymmetric limbs with partial gigantism of the hands and feet, and cerebriform plantar thickening.
Partial gigantism with limb or digital overgrowth is pathognomonic.

45. Treatment
It is very important to make the appropriate diagnostic work-up in order to decide upon the appropriate treatment method.
A dedicated and well-trained physician is a must in order to make the accurate diagnosis and treatment.
It is very common to see patients who have been misdiagnosed and mistreated (or untreated) for years(50% or higher).

46. Treatment
If the lesion (tumor) is Infantile Hemangioma, there is no need for treatment but close regular follow-ups are necessary to rule out tumor related problems (e.g., compression of the airway) or skin breakdown.
In some patients, medical intervention may be indicated (using steroids either systemically or intralesional injections, or interferon).
The tumor should gradually regress with steroids.

47. Treatment
It is very important to make the appropriate diagnostic work-up in order to decide upon the appropriate treatment method.
If the diagnosis of Kaposiform Hemangioendothelioma (KHE) is made, more aggressive follow-ups (particularly regular check ups for platelets) and pharmacological treatment are needed.
If the patient develops Kasabach-Merritt syndrome transcatheter embolization is the ideal treatment, although some small tumors may be removed surgically.

48. Treatment
Venous malformation (or cavernous  hemangioma) lesions can be treated with sclerotherapy
Depending on the size of the venous malformation lesion, several sessions may be needed.
Surgical removal of venous malformation is feasible for small focal lesions.
Most patients describe "growing back" following surgery, and surgery may cause large tissue necrosis and scars.
Venous malformations ("cavernous hemangioma") generally respond well to sclerotherapy (up to 70-80%).

49. Treatment
Arteriovenous malformations (AVM) need to be treated with transcatheter or percutaneous embolization rather than by surgical excision. 
Lymphatic malformations or cystic hygroma
lesions can also be treated by sclerotherapy, particularly macrocystic (cystic hygroma) and mixed form lymphatic malformations.
Microcystic lymphatic malformations can only be treated with surgical excision (if possible).
Radiofrequency ablation can be used in selected cases.

50. Treatment Klippel-Trenaunay syndrome is
usually managed conservatively.
Superficial varicoid veins can be sclerosed or removed surgically, but it is very important to check the patency of the deep venous system in these patients.
Parkes-Weber syndrome is generally approached in a similar manner to AVM lesions.
Other combined anomalies are also managed conservatively in most cases, and intervention (either surgical or transcatheter such as sclerotherapy or embolization) is used in selected patients if needed.

51. Soft tissue tumors

52. Sebaceous cyst

56. Dermoid cyst

59. Soft tissue sarcomas of adults

60. Soft Tissue Sarcoma Epidemiology
Incidence: 2/ per year
Frequency: 0,8-1% of all malignant tumours
Aetiology: widely unknown

61. Soft Tissue Sarcoma Predisposing Factors
Ionising radiation
Genetic predisposition
Neurofibromatosis
Li-Fraumeni Syndrome
fam. Retinoblastoma
fam. Polyposis coli
Radiation
Increase MFH
Viruses
HIV and kaposi sarcoma
Exposure to chemicals
Phenoxyacetic acid
Chlorophenole
Thorotrast (radioactive)
Vinylchloride
Arsenic
Chron. Lymphatic edema
Stewart-Treves Syndrome

62. Soft Tissue Sarcoma Histologic Classification

63. Soft Tissue Sarcoma Localisation

66. Soft Tissue Sarcoma Stages (UICC)
Stage G T LN M OAS (5a)
IA G1 T1 N0 M0 79
IB G1 T2 N0 M0
IIA G2 T1 N0 M0 65
IIB G2 T2 N0 M0
IIIA G3-4 T1 N0 M0 45
IIIB G3-4 T2 N0 M0
IVA all G all T N1 M0 10
IVB all G all T all N M1

67. Clinical presentation
Asymptomatic mass
Pain and disability
Pressure manifestations
Arterial
Venous
Nerves
Metastases
Local
Blood
Lymphatic

76. Kaposi sarcoma

77. plexiform neurfibromatosis

78. Olacranon bursa

79. Diagnostic work up Tissue diagnosis Imaging FNABC True cut needle
Open biopsy
Incisional
Excisional
Imaging
Tumor CT or MRI
Bone plain or MRI
Lung plain or CT

80. Management of local disease (Surgery)
Tumor resection
Radical or compartmental excision
Wide local excision
Enucleation
Intracapsular
Lymphadenectomy only in epitheliod, angiosarcoma & synovial sarcoma
Amputations
Reconstructions

81. Management of local disease (Radiotherapy)
Should pre operative if
Rapid growth
High grade
Large size
Should post operative in most tumors except G1
Small G2

82. Management of local disease (chemotherapy)
Could be used with radio as pre oprative neoadjuvant
Its use post operative is controversial
Best results with recurrence either loocal or distant

83. Soft Tissue Sarcoma Therapeutic strategy for adults
Local Stage
Metastatic Stage
Radical surgery
non rad. surgery
Surgery
Surgery
+/-
adj. CT/RT CT
+/-
Salv. Op.
neoadj. CT/RT +
Surgery
Exp. therapy

84. Thank you