Urgent Maxillectomy of Infants Ali Tawfik

Transcription

Urgent Maxillectomy of Infants
(Melanotic Neuro
Neuro--ectodemral Tumor of infancy )
Review of the Literature and cases Presentation
Ali Tawfik
Abdelwahab Mohamed;
Mohamed; Elsharawy Kamal;
Kamal;
Hazem Emam;
Emam; Waleed Radwan;
Radwan; Ahmed Mousaad
E.N.T. Department,
Faculty of Medicine
Mansoura University, Egypt.
(Melanotic
(
Neuroectodemral Tumor of infancyy )

Melanotic neuroectodermal tumor of infancy is a
rare and mainly
y in children less than 1 year
y
of age.
age
g .
 The
lesion is generally accepted to be of neural
crest origin
i i
28th Alexandria International Combined ORL Congress April 21-23, 2010
Prof. Ali Tawfik
(Melanotic
(
Tumor of the neural crest: Neurofibroma,
 Schwannoma,
 Neuroblastoma,
 Malignant nerve
ner e sheath ttumor,
mor
 Melanoma,
 Melanotic Neuroectodemral Tumor of infancy
 Medulloblastoma,
 Supratentorial primitive neuroectodermal tumor,
 Ewing's sarcoma
Timothy R Gershon ,2006 Department of Pathology,
M
Memorial
i l Sl
Sloan K
Kettering
tt i C
Cancer C
Center,
t N
New Y
York
k
Prof. Ali Tawfik
(Melanotic
(
Melanotic neuroectodermal tumor of infancy (MNTI) : Rare
R
 Mainly benign neoplasm
 Rapid expansile growth
 High recurrence rate
rate.
In the literature the tumor had a variety of names such as:as:
Since the first description by Krompecher in 1918
 Congenital melanocarcinoma, (Krompecher et al., 1918).
 Retinal anlage tumor
tumor, (Clarke BE
BE, Parsons H
H. 1951).
1951)
 Pigmented congenital epulis, (Lurie 1961).
 Melanotic progonoma. (Stowens D, Lin TH., 1974).
Birgit Kruse- Losler et al (2006)
Prof. Ali Tawfik
(Melanotic Neuroectodemral Tumor of infancy )
The literature for the present analysis was selected according to the following criteria:-
I -Inclusion
I l i criteria.
it i
1-These were all publications with the correct diagnosis
2- Histologic confirmation of the described MNTI from 1918 to 2009.
II- Exclusion criteria
1- Uncertain diagnosis
2- Missing of description of the tumor location
3 The lack of histology
3histology.
Prof. Ali Tawfik
(Melanotic
(
The review of the literature revealed : The total number of MNTIs published from 1918 until 2004 includes 355 cases.
cases.
 The additional one case from KruseKruse-Losler et al.
al., (2006
2006))
 One case from Heba Selim etal Department of Oral and Maxillofacial Surgery,
Surgery Ain Shams
University School of Dentistry, Cairo, Egypt (2008
2008))
 3 cases in Mansoura, Egypt (1988
1988--2009
2009))
 All are typical in the age of the patient, location of the lesion, clinical course, and histologic
features..
features
 The total number of MNTIs published from 1918 until 2009 includes 400 cases.
cases.
Prof. Ali Tawfik
(Melanotic
(
Origin of the tumor
Several theories concerning origin of the tumor have been proposed but :There is general agreement that the tumor’s origin is the neural crest. (Borello ED, Gorlin RJ.
1966) , Cutler et al., 1981), (Misugi et al., 1965), (Jones et al., 1990). Bruce,
etal, (1999) and Kruce-
Losler, etal, (2006).
Prof. Ali Tawfik
(Melanotic
(
Sites of the tumor
 92.8% of MNTIs occurred in the head and neck . (Kruce-Losler, etal, (2006).
The maxilla is main site (68%-80%) (Cutler et al., 1981). followed by :
 The
Th skull
k ll (10.8%),
(10 8%)
 Mandible (5.8%),
 Brain (4.3%) (Cutler et al., 1981).
 7.2% occurred in: Epididymis,
 Mediastinum (Misugi K
K. et al
al., 1965).
1965)
 Ovary, (Hameed K, Burslem MRG. 1970).
 Uterus, (Schultz 1957).
 Other sites (Johnson et al
al., 1983)..
Kruce-Losler, etal, (2006).
Prof. Ali Tawfik
(Melanotic
(
This diagnosis is based on
on::
 Clinically presents as a rapidly growing, painless, expansile, partly
pigmented mass, typically in the maxillary region,
 It tends to occur as a single lesion
lesion;; however, multiple lesions have also
been reported
reported.. (Steinberg et al., 1988), (Pontius et al., 1965).
Prof. Ali Tawfik
(Melanotic
(
The histological features include 2 cell populations:
1- Large polygonal epithelioid cells resembling melanocytes,with scattered intracellular brown
granular pigmentation ,( melanin )
2 Smaller
2S ll neuroblast-like
bl t lik round
d cells
ll in
i a stroma
t
off fibrous
fib
tissue
ti
containing
t i i fibroblasts
fib bl t
(Misugi etal, 1965 and Mobsby ,etal 1992).
•
I
Immunohistochemical
hi t h i l staining
t i i in
i MNTIs
MNTI is
i essential
ti l .
Prof. Ali Tawfik
(Melanotic
(
Imaging
g g :• CT scans to reveal hyperdense masses, but hypodense variants have been
reported as well
well..
• MRI shows a hypodense mass,
mass with focal areas of hyperdensity in T1-weighted
images and an isointense mass on T2-weighted images.
images.
(Atkinson GO Jr. 1989 ).,, (Mirich et al., 1991) (Mosby et al., 1992) (Barret et al., 2002). .
Prof. Ali Tawfik
(Melanotic
(
The biologic behavior of MNTI :- rapidly growing and invading
 So needs early diagnosis without unnecessary loss of time
 Late diagnosis may be the reason for difficulty in radical resection,
resection,..
Prof. Ali Tawfik
(Melanotic
(
Malignant behavior
 Has been reported in 5 (3.1%) of the 158 cases of MNTI reviewed by
(Cutler et al 1981).
 The literature review to 1989 increased the total number of malignant MNTIs
to 15 (6.97%
97%) of 215 reported cases, (Kruse
(Kruse--Losler, et al 2006
2006))
Prof. Ali Tawfik
(Melanotic
(
The differential diagnosis of MNTI of Maxilla:1. Neuroblastoma,
2. Ewing’s sarcoma
3. Epithelioid Angiosarcoma
4. Rhabdomyosarcoma,
5 Peripheral neuroepithelioma,
5.
neuroepithelioma
6. Desmoplastic small round cell tumor,
g
melanoma,,
7. Malignant
8. Lymphoma.
G i
Gaiger
d
de Oli
Oliveira
i ett al.,
l 2004
Prof. Ali Tawfik
Surgical excision is the treatment for MNTI
MNTI.
Prof. Ali Tawfik
Recurrence::Recurrence
 The local recurrence after conservative excision was 10
10%
% to 15%
15%.
(Pettinato et al.,
al 1991 ),
) (Kapadia et al.,
al 1993), (Judd et al.,
al 1990), (Hoshina et al.,
al 2000).
2000)
 Recurrences may be caused by incomplete removal of the
primary lesion, dissemination of neoplastic cells during surgery,
and multicentricity
multicentricity.. (Nagase, et al., 1983)
 It might be due to the problem of finding the balance between
radical surgical resection and the preservation of important
anatomic structures
structures..
Kruce-Losler etal
Kruce-Losler,
etal, (2006)
(2006).
Prof. Ali Tawfik
Need
for close followfollow-up, especially within the first 6 months
postoperatively..
postoperatively
Early
detection and treatment of recurrence will avoid further
complications
li ti
and
d may supportt a favorable
f
bl outcome
t
f the
for
th patient
patient.
ti t.
Kruce-Losler, etal, (2006).
Prof. Ali Tawfik
Case 1
Prof. Ali Tawfik
1988
1990
1991
Prof. Ali Tawfik
2001
Case 2
Prof. Ali Tawfik
Prof. Ali Tawfik
2001
2002
2009
2004
2010
Prof. Ali Tawfik
Case 3
Prof. Ali Tawfik
17/5/2009
Prof. Ali Tawfik
17/5/2009
20/5/2009
30/5/2009
2/1/2010
20/4/2010
Prof. Ali Tawfik
Conclusion
 MNTI is a rare and mainly benign neoplasm of early infancy
infancy.
 Rapid expansile growth and a high rate of recurrences.
 The most common site is the maxilla, but it mayy also occur in the mandible, skull, brain,
epididymis, and other rare locations.
 The origin of the tumor is the neural crest.
 The expansive, destructive, and rapid growth of MNTI and its effects on the surrounding
tissues are the most obvious clinical features.
 The definitive diagnosis should be by histopathological and immunohistochemical
 Early diagnosis and radical excision can lead to excellent outcom.
 Needs
N d aggressive
i surgical
i l ttreatment
t
t tto avoid
id th
the llocall recurrence.
 Proper follow up
Prof. Ali Tawfik
Prof. Ali Tawfik

Urgent Maxillectomy of Infants Ali Tawfik

Transcription

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