Transcript Enlarging Mass on Upper Lip

CASE MANAGEMENT
• JUNE 16, 2014
Dr. Lulubel F. Ilagan
Second year resident
General Data:
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JC
5 months old, female
Filipino
Catholic
Hagonoy, Bulacan
Enlarging Mass on Upper Lip
History of Present Illness
At birth
At 1 month
- reddish,elevated
skin lesion, less
than 5mm with
irregular borders
on the lip, lateral to
the philtrum, left
- size: 1 x 1 cm with
elevation and
thickening
-pediatrician:
hemangioma.
3 months old
- 3.5 x 3 cm,
violet-red,
causing slight
eversion of
the left side of
the upper lip.
- referred to a
hematologist
Example*
PREDNISONE
Upon birth
size
color
character
1 month
2month
4month
History of Present Illness
- Doppler UTZ
- CBC (normal)
- prednisone at
2 mg/kg/day
4 months old
- Followed up
- Decrease in
size to 2.5 x 2.0
cm, lighter in
color
5 months
- Followed up
- Prednisone
stopped due to
ulceration
- Increasing in
size
History of Present Illness
Advised to start
propranolol
Day of
Admission
2d Echo
done
Review of Systems
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General: No weakness, no fever, no weight loss
Skin: No pruritus, no jaundice, no easy bruisability
HEENT: No headache, no dizziness, no hearing loss, no epistaxis, no
hoarseness, no excessive salivation, no blurring of vision
Neck: No pain, no limitation of movement
Respiratory: No cough, no colds, no difficulty of breathing
Cardiovascular: No palpitations, no easy fatiguability, no chest pain
Gastrointestinal:, no diarrhea, no hematemesis, no melena, no
hematochezia
Endocrine: No polyuria, polydipsia, polyphagia
Genitourinary: No discharge, no incontinence, no dysuria
Musculoskeletal: No limitation of movement, no joint pains
Nervous system: No behavioral changes, no seizure
Hematologic: No bleeding, No bruising
Birth and Maternal History
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29-year old, G1P1 (1001)
Prenatal checkup - 2 months AOG; health center
Nonsmoker, nonalcoholic beverage drinker
Ultrasound – 3rd trimester: normal
Second trimester – cough for 5 days
Delivered term, spontaneous vaginal delivery at a
lying-in clinic
Newborn screening - normal
Nutritional History
• Mixed feeding for < 1 month  bottlefed
• Complementary
feeding
4
months,
cereals
• At present: Bonna, 1:2 dilution, 4 oz q3h
• Served daily with mashed potato, banana, or
cereals at least twice/day
• Given multivitamins and ascorbic acid daily.
Immunization History
• BCG: 1 dose
Past Medical History
• No previous admission
• No previous surgical procedure
• No allergies
Growth and Developmental History
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Gross Motor: good head control at 3
months; rolls over at present
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Fine motor: Grasped objects at 4 months
• Adaptive: social smile at 2 months of age.
• Language: cooed at 3 months
Family History
29
27
Father: fish retailer
Mother: housewife
(+) DM – maternal grandmother
(+) HPN – paternal grandfather
(-) vascular lesions
(-) bronchial asthma
Environmental History
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2-bedroom, well lit, well ventilated house
3 other household members
Purified drinking water
Garbage collected 3x times/day
No nearby factories
With exposure to cigarette smoke
Physical Examination
General Survey: Awake, not in cardiorespiratory
distress
Vital Signs: BP: 90/60 CR: 102 RR: 24
Temp 36.2 degrees Celsius
Anthropometrics: Weight: 6.4 kg (z score 0)
Height:65 (z score 0)
Physical Examination
• Skin: Violet-red mass, nontender, 4.5 x 4 cm,
with irregular borders on the upper
lip, lateral to the philtrum
• Head: Open anterior fontanel about 1 x1 cm,
soft and flat, pink palpebral conjunctivae,
anicteric sclerae, no alar flaring, violet-red
mass, nontender,5 x 4 cm, with irregular
borders on the upper lip, lateral to the
philtum, no cervical lymphadenopathy
Physical Examination
Chest: Symmetrical chest expansion, no
retractions, clear breath sounds
Heart: Adynamic precordium, normal rate and
rhythm, no murmurs
Abdomen: Globular, normoactive bowel sounds,
soft, non palpable liver and spleen
Extremities: Warm, pulses full and equal, crt<2
sec
Physical Examination
Neurological Examination
MSE: Calm, appropriately dressed
Cranial Nerves: pupils 2-3mm equally reactive to
light, extra ocular muscles are full and equal,
good masseter tone, can smile with no facial
asymmetry. Patient turns to sound, can turn
head side to side with good gag.
Motor: symmetric movement of all extremities
Reflexes: Deep tendon reflex 2+ on all extremities
Meningeals: No Babinski, no nuchal rigidity
Autonomics: No excessive sweating
Salient Features
• 5 months old, Female
• Violet-red mass on upper lip, increasing in
size
• Treated with hydrocortisone
Working Impression
• Hemangioma, left upper lip
Vascular
lesions
Birthmarks
Nevus
Café-au-lait
Cutaneous
lesions
-Benign acquired
disorders
- Genetic disorders
- Vascular birthmarks
Benign Acquired
disorders
Pyogenic granuloma,
angiokeratoma of
mibelli, spider
angioma
Genetic disorders
Blue-rubber bleb,
Malfucci syndrome,
Osler-Weber-Rendu
disease
Vascular lesions
Malformation
Vascular birthmarks
Tumors
Vascular
tumors
• most common is
hemangioma
Vascular
Malformat
ion
• developmental error
in blood vessel
formation
• do not regress but
slowly enlarges
Vascular
Birthmarks
Vascular Tumors
Hemangioma
Tufted
Angioma
Kaposiform
hemangioend
othelioma
Diffuse
Hemangioma
Vascular Tumors
Hemangioma
Tufted
Angioma
Kaposiform
hemangioend
othelioma
Diffuse
Hemangiomatosis
Numerous hemangiomas are widely distributed
Vascular Tumors
Hemangioma
Tufted
Angioma
Kaposiform
hemangioend
othelioma
-Very aggressive locally
-Solitary, firm and deep purple
-Do not regress spontaneously
-Associated with Kasabach-Meritt syndrome
Diffuse
Hemangioma
Vascular Tumors
Hemangioma
Tufted
Angioma
Kaposiform
hemangioend
othelioma
Diffuse
Hemangioma
- Histologically: “cannonball-like tufts of blood vessels
- Slowly expanding dusky reddish-blue plaque with satellite
lesions (regression not expected) or solitary vascular
nodule
Vascular Tumors
Hemangioma
Tufted
Angioma
Kaposiform
hemangioend
othelioma
Diffuse
Hemangioma
Most common tumor of infancy (5% of all infants)
Proliferation of the vascular endothelium
• J.C.
- Precursor lesion at birth
- Rapid growth
- F>M
Classification
• SUPERFICIAL
- bright red, protuberant, compressible,
sharply demarcated
• DEEP
- more diffuse, less defined; cystic, firm
overlying skin may appear normal or with
bluish hue
PHASE
1. Rapid expansion/ Proliferative phase
2. Stationary phase
3. Involution
- not correlated with size or site
- lesions on the lip seem to persist most
of the time
• Reminder:
• If may literature sources ka, don’t forget to
acknowldege author at the bottom..
• Eg.
Ilagan et al 2010
PATHOPHYSIOLOGY
- Not elucidated
- Proliferation of benign endothelial like cells
that possess histochemical markers
(GLUT-1, Lewis Yantigen, FcyRII and
merosin - present also in placental BV
PATHOPHYSIOLOGY
PLACENTAL THEORY
- explains programmed life cycle
- genetic similarity
ENDOTHELIAL PROGENITOR CELL (EPC)
THEORY
- increased circulating EPC
- Human IH EPC injected to mice
SYNDROMES ASSOCIATED
WITH HEMANGIOMA
PHACE SYNDROME -
GORHAM
posterior fossa brain defects,
hemangioma,
arterial
malformations, cardiac, eye
abnormalities (sternal raphe
defects/ supraumbilical raphe)
- cutaneous hemangiomas with
massive osteolysis
- macrocephaly
lipomas,
hemangiomas of autosomal
dominant inheritance
BANNAYAN-RILEY-RUVALCABA
TREATMENT
• OBSERVE
• INDIVIDUALIZE
- sign of regression:
- Cochrane analysis
“lack of well-designed
blanched or pale gray
- 60% involutes at 5 years old clinical trials and the
absence of US FDA- 90-95% - 9 year old
approved medications
IH, limits ability to
clearly identify the
single best option”.
HIGH RISK INFANTILE HEMANGIOMA
Location
Type
Growth Phase
Periorificial (eyes, nose,
mouth *)
Segmental
Maximal Proliferation
phase (usually 3-6
months)
Central Facial
Multiple
Lumbosacral
Rapidly proliferating
Genital
RATIONALE FOR
TREATMENT
1. To prevent or improve functional
impairment or pain
2. Prevent or improve scarring and/or
disfigurement*
3. To avoid life-threatening complications
TREATMENT
• CORTICOSTEROIDS
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1960s
Prednisone 2-3 mg/kg/day
Regression evident after 2-4 weeks *
Response obtained: taper
TREATMENT
• INTRALESIONAL CORTICOSTEROID
INJECTION
- Triamcinolone
- 1-2 mg/kg
- Bleeding, skin atrophy, skin necrosis,
infection, anaphylaxis, adrenal
suppression
TREATMENT
• VINCRISTINE
- Vinca alkaloid microtubule inhibitor
- 1.0 to 1.5 mg/m2 weekly
- Immunosuppression, neuropathy, alopecia
TREATMENT
• INTERFERON
- antiangiogenic properties
- effective
- Spatstic diplegia (20%), neurotoxicity
TREATMENT
• PULSED DYE LASER
- Very superficial lesions
- Small (<4-5 cm), ulcerated hemangioma *
- Ulceration, scarring
TREATMENT
• TOPICAL THERAPY
- Timolol 0.5% gel
- Superficial IH
• No large clinical trials
TREATMENT
• PROPRANOLOL
• Complications
- 2mg/kg/day *
- Bradycardia,
Hypotension,
- Excellent results
Hypoglycemia
- “exact indications, dosage, length of
treatment, and long-term sequelae have
not been thoroughly investigated”
• After propranolol, ano nangyari? If u plan
to do the time line as seen earlier sa HPI,
you can show it again here to emphasize if
size has decreased..
TREATMENT
• SURGICAL
- Pedunculated or exophytic hemangiomas
in which scarring is highly probable
- Chronically ulcerated hemangiomas
causing pain
- Will lead to disfigurement, bleeding
RECOMMENDATIONS
• Facial hemangioma >/= •5cm
Evaluate for
PHACE
• >/= 5 cutaneous lesions
• Hepatic ultrasound
You may want to add:
• Ano nangyari after propranolol?nagtachy
ba cya? Lumiit ba yung mass?
• When do we rrefer hemangiomas to a
hematologist?
• When does it become alarming? VS
saying to Mom na “observe” lng?
• Pls do not forget to remind your
moderator/reactor
• Be early tom kasi may flagcem.. We cant
help u na if mag8am na..
• Good Luck!