metastatic retinoblastoma of the mandible in a 4 year ...

VOLUME 1 ISSUE 1, JANUARY- MARCH 2013

METASTATIC RETINOBLASTOMA OF THE MANDIBLE IN A 4 YEAR OLD CHILD - A CASE REPORT Dr. Anshu Sharma* Dr. Anmol Agarwal ** Dr. Sunil Chaudhary*** Dr. Narendra Nath Singh**** *Asst. Prof. Department of Pedodontics and Preventive Dentistry, Teerthanker Mahaveer Dental College and Research Centre Moradabad, **Sr. Lecturer Department of Oral and Maxillofacial Surgery, Sri Sai College of Dental Sciences, Vikarabad *** Prof. ESI Dental College and Hospital, New Delhi ****Prof.& Head, Department of Oral and Maxillofacial pathology, Kothiwal Dental College and Research centre Moradabad.

ABSTRACT Metastatic retinoblastoma of mandible is an extremely rare condition and to our knowledge only 13 cases have been reported. The present case is of a 4 year old child with chief complaint of pain in left mandible with associated lymphadenopathy. Bone marrow aspiration and fine needle aspiration cytology along with Contrast enhanced computed tomography (CECT) were conclusive of retinoblastoma metastasizing into left mandible. Downloaded From IP - 49.206.236.87 on dated 7-Jul-2015

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This is the only report by pedodontist on this malignant lesion, depicts fastest case of mandibular metastasis of all reported cases, and only one with CECT features. This case report signifies the role of pedodontist in the recognition of head and neck malignancies as well as rules out an unusual cause of mandibular pathologies clinically and radiographically.

KEYWORDS: Retinoblastoma, Mandible, Metastasis. INTRODUCTION Retinoblastoma is the most common ocular tumour

pain in left mandible since last seven days. History

seen in children. Disease may involve one or both

revealed that patient had under gone surgical therapy

eyes.1 It usually presents before the age of 3 years in

and chemotherapy for eye tumour 6 months and 20

70% of cases. Metastasis to the mandible from

days back respectively. Clinical examination revealed

retinoblastoma is rare. To our knowledge only 13

closed right eye (Fig 1) and left submandibular

cases of metastasis of retinoblastoma to the mandible

lymph nodes tender on palpation and rubbery in

have been reported in the literature (Table 1).The aim

consistency.

of this case report was to describe an interesting case

radiating towards left mandibular ramus. Intraoral

of metastatic retinoblastoma of left mandible in a

examination revealed intact mandibular teeth with no

four year old child with most rapid metastasis of all

carious exposure and mobility. There was no

reported cases till now i.e. within six months after

intraoral swelling or vestibular obliteration though

surgical

after

marginal ginigiva was inflamed. Bone marrow

Enhanced

aspiration of left mandible showed few abnormal

intervention

chemotherapy

along

and

twenty

with

Contrast

days

Patient had severe continous pain,

Computed Tomography (CECT) findings.

cells showing size variation with hyperchromatic

CASE REPORT

nucleus. Scant cytoplasm were seen singly and in

A 4-year- old- boy reported to the Department of Pedodontics and Preventive Dentistry, because of

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small clusters with an occasional cluster showing moulding of cells. These findings were suggestive of

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metastatic small round cell tumor which was in

found. Twelve (92%) of 13 patients died after the

accordance with results of Fine needle aspiration

metastasis to the mandible. In all the reported cases

cytology of left submandibular lymph nodes. CECT

patient has died except one which shows poor

examination showed large hyper dense mass of the

prognosis and high invasive nature of retinoblastoma.

right globe with large retrobulbar extension (Fig 2)

Radiographically

and outer cortex of the mandibular ramus on left side

appearances. Eight cases of metastatic retinoblastoma

showed erosion with presence of enhancing soft

of the mandible reported ill defined extensive bone

tissue component (Fig 3).On correlation of clinical

destruction

lesions

could

have

variable

on plain radiographs. Merrill and 4

,cytologic and radiographic findings it was diagnosed

Wertheimer

as a case of metastatic retinoblastoma of the

on mesial aspect and apical region of the lower right

mandible.

unerupted permanent second molar and periodontal

DISCUSSION

radiolucency of the lower incisors and canines.

Retinoblastoma (Rb) is the most common intraocular

Pullon and Cohen 5 reported only large developmental

malignancy of children. It is caused by inactivation of

crypts of the left lower first premolar and canine teeth

both copies of a tumor suppressor gene (Rb1), which

with minimal destruction of the mandibular buccal

participates in the control of cell cycling.2,3 Bone

cortex. Displacement of erupted tooth or unerupted

metastasis occurs frequently to the limbs via the

tooth germs by the lesions was reported in five cases.

vascular route. Metastatic retinoblastoma of the jaws

Floating teeth were described in two cases. This is

is very rare. There is not much difference in the

the only case which describes CECT scan findings of

metastasis of retinoblastoma in unilateral or bilateral

retinoblastoma. According to literature available

cases. Though there is slight predilection for higher

though chances of survival of such patients are

incidence of males over females including our case (8

rare.Timely diagnosis and systematic management by

males and 5 females). Prediliction for right side of

expertise could increase life expectancy of an

mandible is higher as compared to left side though in

individual.Paediatric

our case metastasis was on left side. Metastasis has

knowledge of tumors of head and neck and must be

been very rapid in this case just with in 6 months of

able to correlate clinically the pathogenic effects of

surgical and 20 days of chemotherapy. This is an

such malignancies to jaws and associated structures.

unusual presentation since all previously reported

REFERENCES

cases have shown late metastasis except one which

1. Chintagumpala M, Patricia CB, Paysse E, Sharon

metastasized in 7 months. Since the maximum

PE, Hurwitz R. Retinoblastoma: Review of Current

interval

Management. The Oncologist2007;12:1237 1246.

between

the

diagnosis

of

primary

that metastasic retinoblastoma of the mandible may

cancer. J Clin Oncol 1997; 15:3301-3312.

occur even if primary retinoblastoma is treated a long

3 Herwig S, Strauss M. The retinoblastoma protein:

time ago. There was no other metastasis in ten (77%)

A master regulator of cell cycle, differentiation and

of 13 patients when metastasis to the mandible was

apoptosis. Eur J Biochem 1997; 246:581-601.

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Role

of

a

retinoblastoma protein in the pathogenesis of human

ORAL

WGJ.

have

mandible was 8 years, the dentists should remember

OF

Kaelin

should

2.

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WR,

dentist

retinoblastoma and metastatic retinoblastoma of the

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Sellers

reported only an unusual radiolucency

the


4

Merrill

RG,

Wertheimer

FW.

Metastatic

8. Giles AD. Metastatic retinoblastoma of the

retinoblastoma of the mandible. J Oral Surg 1964; 22:

mandible. Br Dent J 1979;147: 275 277.

341 345.

9. Kuo YS, Harn LJ, Wang JS. Metastatic

5. Pullon PA, Cohen DM. Oral metastasis of

retinoblastoma of the mandible. J Formosan Med

retinoblastoma. Oral Surg Oral Med Oral Pathol

Assoc 1981; 80: 542 546.

1974; 37: 583 588.

10. Pandya J, Valverde K, Heon E, Blaser S, Gallie

6. Nishio J, Uchihashi T, Mimura T. Metastatic

BL, Chan HS. Predilection of

retinoblastoma

retinoblastoma metastases for the mandible. Med

of the mandible.

Jpn

J Oral

Maxillofac Surg 1976; 22: 346 353.

Pediatr

Oncol

2002;

38:

271 273

7. King D, Shapiro SD, Beall JC. Metastatic retinoblastoma of the maxilla and mandible. J Dent Child 1976; 43: 347 349.




LEGEND Authors

Sex

Age (years)

Site of primary retinoblastoma

Merrill and Wertheimer 3 Dunlap et al 4

F

9

Both eyes

Interval of metastasis to the mandible (years) 8 years

Site of metastasis

Mandible is the first site of metastasis

Prognosis

Yes

Died

2.5 years

Both mandible Left mandible

F

2.5

Both eyes

Yes

Died

Pullon and Cohen

M

9

Right eye

1.5

Left mandible

Yes

Died

F

6

Right eye

2.7

Left mandible

Yes

Died

M

7

Both eyes

7

No

Died

and

F

8

Right eye

1

Yes

Died

and

M

8

Right eye

2

No

Died

F

9

Both eyes

1.6

Yes

Died

M

10

Left eye

0.7

Yes

Died

Pandya et al 10

M

11

Both eyes

3.7

Yes

Alive

Taguchi et al

M

4

Left eye

0.8

Yes

Died

Sharma A

M

4

Right eye

0.6

Right mandible and maxilla Both mandible Both mandible Right mandible Right mandible Right mandible Left mandible, right maxilla and left maxilla (suspected ) Right mandible

Yes

Died

Nishio et al King et al

6

7

Perriman Figures Perriman Figures Giles 8 Kuo et al

9

Table 1: Metastatic Retinoblastomas to Mandible

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Fig1 Extra oral view showing closed right eye

Fig 2 CECT scan showing hyper dense mass of the right




globe with large retro bulbar extension

Fig.3 - CECT showing eroded left mandibular ramus

ADDRESS FOR CORRESPONDENCE:

SOURCE OF SUPPORT-NIL

Dr Anshu Sharma MDS Assistant Professor Department of Pedodontics and Preventive Dentistry, Teerthanker Mahaveer Dental College and Research Centre Moradabad-244001 Uttar Pradesh Cell: +91-9634143976 Email: [email protected]

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