C Teratoma

http://www.dnbpediatrics.com/

2. 1 day old neonate
Antenatal History of anterior neck swelling
Confirmed postnatally

3. Maternal age 26 years
Second gravida with one alive child
Previous LSCS
No other significant Medical/Surgical issues
Regular follow up while in Antenatal period

4. Clinical assessments normal
Fetus has large neck mass on USG in 37
th
week
Mild Polyhydramnios
Antenatal diagnosis was cystic hygroma

5. 11.01.2011
Fetus with vertical lie
Cephalic presentation
No abnormality detected

6. 8.03.2011
Well defined hypo echoic mass
Dimensions 5.5 X 6 cm
located in cervical region
Lateral to neck vessels

7. Guarded prognosis was explained
Continuation of pregnancy was advised
Mode of delivery was to be decided upon
obstetric indications
Antenatal procedures not feasible

8. FTLSCS at JLNHRC on 19/3/2011 12:50 pm
Baby had massive neck mass
Cried immediately after birth
Had mild respiratory distress in supine position
APGAR score at 1 min –7
5 min -8

9. Birth of high risk newborn anticipated
Instruments for securing airways were ready had the
baby deteriorated further
Kept in thermo neutral environment
Dried and wrapped in warm clothing's
Oro-nasal suctioning were performed
Distress was alleviated in right lateral position

10. Initial stabilization assured in labourroom
Necessary equipments kept ready in NICU
Transported with warm clothes
Airway positioning while transporting assured
Case was informed to pediatric surgeon

11. ›Euthermic
›No other dysmorphicfeatures
›CRT < 2 sec
›Heart rate 122/min
›Respiratory Rate 40/min
›BP 58/32 (40) mm Hg
›Anthropometry

12. ›Respiratory system -B/L breath sounds distinct
equally heard
conducted sounds +
Inspiratorystridor
›CVS –S1S2 +, no murmurs
›Per Abdomen –soft, non distended, non tender
›CNS –NNR present
›Ext genital –female, normal

13. ›Solitary neck mass
›Large –10x8x6 cms
›Midline, encroaching
towards right
›Non pulsatile
›Skin -normal

14. Palpation confirmed
the findings
Temp –normal
Tenderness –absent
firm to hard mass
Not compressible
Well encapsulated

15. Mobile in all the
directions
Not adherent to
underlying structures
Not adherent to skin
Trans illumination-absent
Not pulsatile
No thrills or hum

16. Thermo neutral environment with servo control
Airways management and positioning
Fluid and electrolyte
Nutrition
Respiratory and Hemodynamic monitoring

17. ›Tense cystic hygroma
›Teratoma
›Hemangioma
›Neuorblastoma
›Rhabdomyosarcoma
›Rarer conditions
Congenital thymiccyst
Congenital goitre
Branchialcleft cyst

18. ›Solid mass
›Well encapsulated
›Few areas of cystic degeneration
›Few stipulated calcifications
›No large calibrevessels inside the tumor
›Neck vessels pushed laterally
›Tracheal displacement +

19. ENT consultation
›airway status
›need of elective/emergent intubation
›Intra operative help
ENT Opinion
›No pressure effect on trachea
›No need of emergent intubation
›CT scan
›FNAC

20. FNAC avoided due to
›possible risk of hemorrhage within the mass
leading to airway compromise.
›Non representative areas aspirated
›Limited sensitivity of FNAC

21. ›Origin
›extent
›compression effects
›associated malformations.

22. Localisationof mass
Characterisationof nature of lesion
Airway column assesment
Relationship with major neck vessels

25. 3 mm section thickness plain and post
contrast
Base of skull to diaphragm

32. A large 6.5x6x5 cm sized heterogenous
Mildly enhancing mass lesion wnichis well
encapsulated containing scattered nodular
calcification seen involving neck anteriorly
and on rtside
Supreiorlyuptosubmandibularspace
Inferiorly supraclavicularregion
Displacing airway column on left and
major vessels posteriorlypossibility of
cervical teratoma.Tobe correlated with
clinical and histopathologicalfindings

33. Serum alpha feto-protein on day 2-83,000 ng/ml
Normal range = 100000 to 125 ng/ml from neonatal
to infancy

34. ›Clinical findings -solid mass
›USG -Non vascular
›CT -Heterogenous
-Calcification
›Raised AFP
Cervical Teratoma

35. ›Primary Surgical excision

36. Airway assessment
General anaesthesia

37. Direct larngoscopy Difficult airway

43. Immature cervical teratoma, grade 2

44. Specimen of 6.5x6x3.5
cm received.
O/S-nodular with
retracted capsule.

45. C/S-shows lobulatedgrey
white mass predominantly
solid with multiple small cysts .
Cysts are of varying size from
1mm to1cm diameter filled
with mucinousmaterial.
Few cartilagenousarea,
slimy area & bony spicules
were present in solid part of
the mass.

46. H&E STAIN 40X
Mature cartilage
H&E STAIN 10X
Immature cartilage

47. H&E STAIN 10X
RESPIRATORY EPITHLIAL
CLEFT WITH LINING

48. H&E STAIN 10X SQUAMOUS
EPITHELIUM WITH
KERATINIZATION
H&E STAIN 10X NESTS OF
IMMATURE SQUAMOUS
EPITHELIUM

49. H&E STAIN 10X IMMATURE NEURAL
EPITHELIUM

50. H&E STAIN 10X BLASTEMAL CELLS
H& E STAIN BLASTEMAL
CELLS

52. H & E STAIN 20X MUCIN
PRODUCING GLANDS

53. •Multiple sections studied from tumourshows mature as well as
immature elements derived from all 3 germ layers.
•Mature elements comprise of nests of squamouscells, glands,
mature cartilage, occasional bony tissue, neural tissue &
smooth muscle tissue.
•Immature elements include neuroepithelialelements,
occasional group of blastemalcells & immature cartilage in
myxoidstroma. Mitosis is in the range of 2/10HPF. Normal
thyroid tissue is not seen in the section studied.
•Impression:-ABOVE FEATURES FAVOUR IMMATURE CERVICAL
TERATOMA (Grade –II)

54. 0 Mature solid teratoma
I Abundance of mature tissues, intermixed with loose
mesenchymal tissue with occasional mitoses; immature
cartilage; tooth anlage
IIFewermaturetissues;rarefociofneuroepithelium
withcommon mitoses,notexceedingthree40X
fieldsinanyoneslide
III Few or no mature tissue ; numerous neuroepithelial
elements, merging with a cellular stromaoccupying
≥four 40X fields

55. Greek word –monstrous tumour
Derived from all three embryonic germ layers-ectoderm,
endoderm and mesoderm
Can occur anywhere in the body
Most common location –sacral region
Rarer in adults since most are detected in childhood.
Neonatal period are uncommon and virtually always benign

56. Rare congenital tumoursof neck
Challenging in the neonatal period
Present as massive neck swelling with airway
compression
High perinatalmortality and morbidity rates.
Predominantly of the mature variety

58. Constitute 1.6 to 9.3% of pediatric teratomas, 1per
40,000 births
Global scenario -Over 150 cases reported so far
Indian scenario -4 cases ,1stiiborn, 1 died soon after
birth, 2 surviving
No apparent relationship to the mother's age
No greater odds of occurancein males versus females
No racial or ethnic preference.

59. Exact cause still unknown
Inability of totipotentcells to differentiate into
a complete body or organ
Abnormal development of a conjoined twin
Arises from stem cells within the thyroid gland

60. Novel karyotypicchanges on comparative
genomic hybridization
›1p21.1 amplification
›9p22 deletion
›17q21.33 1-copy gain

61. Rare
›Imperforate anus
›Chondrodystrophiafetalis,
›Hypoplasticleft ventricle with pulmonary
hypoplasia,
›Cystic fibrosis,
›Absence of corpus callosum,
›Arachanoidcyst

62. Based on birth status, age at diagnosis, and the
presence or absence of respiratory distress.
›Group I--stillborn and moribund live newborns
›Group II--newborn with respiratory distress
›Group III--newborn without respiratory distress
›Group IV--children age 1 month to 18 years
›Group V--adults

63. Physical Examination
›Size
›Multiplicity
›Laterality
›Consistency
›Color
›Mobility
›Tenderness
›Fluctuation
›Transilluminationtest

71. USG
MRI

72. Ultrasound –best modality
Asymmetric, well-defined
masses
Large and bulky.
Calcifications
Polyhydramniosin 20 to 40
percent cases
Other fetal abnormalities +

73. Shows mediastinal
involvement
position of the
airway.
Partial / total
Compression

74. Ex uterointrapartum
treatment (EXIT)
procedure / OOPS
procedure
Specifically designed to
preserve uteroplacental
gas exchange to provide
time to secure the airway

75. provides time for:
›Neck dissection
›Clip removal
›Bronchoscopy
›Endotrachealintubation
›Surfactant administration
›Placement of umbilical arterial and venous
catheters

76. Frequent ANCs
Frequent ultrasound exams recommended
to monitor
›amniotic fluid volume,
›tumor size,
›growth and the general health of the fetus
Institutional delivery encouraged
Elective cesarean preferred
Team approach for ex uteromanagement

77. Baseline hemogramand blood biochemistry
USG
CT scan/MRI
FNAC and Biopsy
Thyroid and parathyroid function test
Serum alpha fetoprotienand beta HCG
Transcription factors GATA-4 and GATA-6
Genetic studies

78. Expedient multidisciplinary approach
›Airway management
›Definitive management

79. Primary surgical excision
If malignancy proved then
›Chemotherapy
›Radiotherapy
›Combination therapy

80. Risk for serious thyroid conditions
›Hypoparathyroidisim
›Hypothyroidism
Developmental delay and mental
retardation
Malignant transformation

81. Recurrence
Metastasize to regional lymph nodes
Occurring among siblings (only one
case reported)

82. Recommendations
›AFP levels be obtained
at birth
at 1month,
three-month intervals in infancy and
yearly thereafter, upto3 years of life
›MRI scanning twice a year for the first three
years of life.

83. Airway obstruction at birth
Degree of maturity of tissues
Completeness of resection
Associated anomalies
Mortality is high in untreated infants & low if
treated surgically