1. capillary Hemangioma & Its differential Diagnosis
F.Fatemi,MD
Isfahan University Of Medical Sciences

2. Vascular Anomalies (birthmarks)
Overall, are seen in
well above half of the population. 
BUT :
Simple skin discolorations are very common in newborns and do not require diagnostic testing or treatment. 

4. VBF Founded in 1994 by Linda Rozell-Shannon after her daughter Christine was diagnosed with a hemangioma and she found that there was no foundation, no website, no book and very few resources
Linda searched for a surgeon to remove Christine’s hemangioma and found
Dr. Waner.
Together they formed VBF and wrote the first book on the subject “Birthmarks: A Guide to Hemangiomas and Vascular Malformations”

5. 2011 – New Program Brings Hope
VBF establishes the ITEAM.
An international team of surgeons and treatment specialists who volunteer to travel to Russia, India, Asia and Africa to perform free surgeries and to train physicians in those countries to perform future treatments.
One iTEAM trip will conducted every 9 month (based on funding)

6. 2011 and Beyond Realizing the Vision…….
Bringing the iTEAM to underserved countries
Babies With Birthmarks Guidelines established by major medical academies
Conference/Gala in NYC
Increased Awareness & Accessibility to Options
Discover Cause of Birthmarks through Research
Legislation passed to require insurance companies to cover treatment of all children with birthmarks.
No Child Will Remain Untreated!

7. Capillary Hemangioma
Infantile hemangioma
Cong hemangioma

8. Infantile hemangioma
IHM : The most common soft tissue tumor of infancy
Benign endothelial cell neoplasm.
They develop in 4-5% of infants, with the majority of lesions noted within the first several weeks of lifes.
more commonly in Caucasian infants
female: male ratio of 2-5: I

9. Significant numbers of hemangiomas being noted at birth presented as precursor lesions.
Telangiectasias surrounded by a vasoconstricted halo, areas of pallor, pink macules, and blue bruise-like patches are the most common precursor lesions

10. Superficial hemangiomas :
The surface is finely lobulated "strawberry hemangioma“
Superficial and mixed HMs are usually diagnosed based upon their clinical features:

11. Deep hemangiomas :in the deep dermis and/or subcutis.
warm, ill-defined, light blue-purple masses
with minimal or no overlying skin change
making them more difficult to diagnose than superficial or mixed hemangioma.
Presence of dilated veins or telangiectasias overlying a deep hemangioma provides a clue that the lesion is of vascular origin.
Larger deep hemangiomas often have significant arterial blood supply during their peak growth phase.
A thrill may be felt or a bruit auscultated, and high flow can be documented by Doppler ultrasonography.

12. Only MRV can differentiatre these
The most important DD of a deep HMs include:
vascular malformation (particularly venous, lymphatic or combined venous-lymphatic malformations)
The use of CT and MRI scanning is not
particularly helpful in aiding diagnosis, as both IH and vascular malformations reported as haemangiomas
Only MRV can differentiatre these

13. "abortive" or "reticular“ IHM
A small subset of IHM
has little or no growth beyond patches of reticulated erythema with telangiectasias, often superimposed on a background of pallor .
A proliferative component is absent or accounts for <25% of the surface area , often presenting as small red papules at the periphery".
They are GLUT-I-positive and involute similar to classic IHM
These HMs favor the lower body and may develop recalcitrant ulceration or
(if larger and segmental) be associated with LUMBAR syndrome

14. May be part of systemic syndromes (phaces &lumbar)
Less common and more worrisome is:
Larger plaque type
Segmental hemangioma esp on face
May be part of systemic syndromes (phaces &lumbar)

15. congenital hemangioma
-RICH
-NICH

17. This rapidly involuting congenital hemangioma is in a later stage (the child is almost 1 year old); it shows a classic pattern of atrophy left in the wake of rapid resolution
This bright red–violaceous plaque is a rapidly involuting congenital hemangioma (RICH) in an early stage

18. NICH lesions;
They consist of the classic telangiectatic patch with surrounding slight pallor.
unlike infantile HMs,grow proportionately with the child.
The lesions may worsen with maturity and do not involute spontaneously.
The diagnosis is often established
retrospectively after the lesion is
excised because involution fails to
occur.

19. Differential Diagnosis of capillary hemangioma
Most important :
1-Kaposiformhemangioendothelioma
2-Tuffted angioma

20. 1 - Kaposiform hemangioendothelioma
Rare deeply seated , infiltrative vascular tumor
Involve the skin and subcutaneous as well as deep soft tissues, bone and even viscera
Locally aggressive
Expression of mixed blood vascular and lymphatic endothelial markers)
Glut1 (-),CD34+,LYVE1+
likely synonymous in pathogenesis with tufted angioma, with the latter being more superficial in location
Does not spontaneously involute
Complete surgical excision is recommended where feasible
VAC : vincristine /actinomycin /cyclophosphamide ,….

21. 2- Tufted angiomas
Mottled red patches or plaques with superimposed angiomatous papules, typically in congenital forms on limb.
grow slowly over a period of 5 mo to 10Y
GLUT1(-),foci of LYVE1+,but not prominent like KHE.
Eventually stabilize in size, then may persist unchanged, shrink or leave a fibrotic residue.
Rarely, complete spontaneous regression
Complete surgical excision is the treatment of choice for small tufted angiomas.
High dose systemic steroids may be useful.

22. MRI of vascular tumors like KHE & TA shows diffuse soft tissue masses that enhance after contrast administration
If MRI does not confirm the diagnosis of infantile hemangioma or there are concerns regarding a possible malignancy, histologic examination of the tumor is recommended.

23. 3-Multifocal Lymphangioendotheliomatosis with Thrombocytopenia
Multiple congenital & progressive vascular lesions involving the skin and viscera + chronic thrombocytopenia
Skin lesions consist of red brown plaques and they may be numerous
Clinically significant GI bleeding appears universal
Distinctive histopathology : resemble benig Iymph angioendothelioma, with variable expression of Iymphatic associated IHC markers (e.g LYVE-l, podoplanin,D2-40)

24. 4-Infantile Hemangiopericytoma
Usually at birth
single or multiple dermal & subcutaneous nodules
May be large at birth or grow rapidly
Multilobulated tumor with cellular areas of spindle cells and antler-like branching blood vessels, merging with less cellular areas containing plump myofibroblasts
Behaves in a benign fashion, despite frequent focal necrosis and increased mitotic activity

25. 5- Eccrine angiomatous hamartoma (EAH)
A rare, benign condition recognized histologically by increased numbers of eccrine elements, as well as numerous vascular channels.
Patients typically present with a solitary, sometimes enlarging, nodule of the extremities usually appearing at birth or arising during childhood.
When symptomatic, EAH may be associated with hyperhidrosis or pain.

26. 6-Spindle cell hemangioma
The most common type of hemangioma in MS is spindle cell hemangioma
Maffucci syndrome manifests early in life, usually around age 4-5 years, with 25% of cases being congenital.

27. 7- Verrucous hemangioma
uncommon vascular malformation,
mistaken for circumscribed angiokeratoma.
at birth
presents as vascular papules, plaques or nodules of bluish color, with verrucous surface and linear disposition, unilateral and are usually (95%) located in the lower limbs.
Relapse is common after conventional treatment with cryotherapy or electrocoagulation.

28. other neoplasms that may have a vascular appearance due to overlying telangiectasias or a blue to red color :
congenital fibrosarcoma
Rhabdomyosarcoma
Neuroblastoma
primitive neuroectodermal tumor
lymphoblastic lymphoma
dermatofibrosarcoma protuberansinfantile
myofibromatosis, which can present as solitary or multiple red to pink plaques or nodules;
lipoblastoma, presents as an enlarging skin-colored or (less often) erythematous mass;
Nasal glioma, often presenting as a congenital blue-red mass on the bridge of the nose.