Case discussion

1. A rare Ovarian Neoplasm in an
adolescent girl
Dr. Samriddhi Karki
3rd Year Resident
Department of Pathology
13/11/2014

2. Clinical history provided
 13 year old
 06/10/2014
 C/O:
– Fever X 1month
– Abdominal fullness x 20 days
– Pain abdomen x 3 days
 O/E:
– P/A:
• A firm mass corresponding to ~ 36wks of gestation, apparently
arising from the pelvis , extending up to the xiphisternum
 Clinical Diagnosis:
– Right sided Adnexal Mass

3. Investigations:
Hemogram:
• Hb: 10.2gm/dl
•Other parameters: within normal limits
Tumor Markers:
•CA125: 335.8 U/ml (< 35 U/ml)
•Alpha-feto protein: 4.99 IU/ml (0.5-0.6)
•β-hCG: 0.05mIU/ml (<10.0)
USG:
• Large heterogenous mass of varied echogenecity mainly cystic,
measuring 36 x 20 cm
• Impression: Germ cell tumor (Dysgerminoma)
CT scan:
• Well defined large lobulated, predominantly hypoechoeic cystic lesion in
the abdomino-pelvic cavity occupying almost whole abdomen with
cystic and solid component with intralesional calcification and foci of fat
density.
• S/O complex ovarian cyst (Terato-dermoid)

4.  Provisional Diagnosis:
Right sided Ovarian Germ Cell Tumor
 Exploratory Laparotomy done
(12/10/2014)

5. Operative findings
 Ascites  300 ml , straw colored fluid
 Multiple peritoneal deposits <2cm
 Deposits in pouch of douglas, utero-vaginal fold
 Right ovarian mass:
– Capsulated mass
– Smooth surface
– Solid + cystic component
 Deposits on the contralateral ovary
 Uterus normal size
 Deposits in infra colic omentum
 Undersurface of the diaphragm on either side / Liver: Normal
 Deposits in the large and small intestine
 Retroperitoneal + pelvic lymph nodes normal size

6. Specimens sent for
Histopathological examination
1. Right ovarian mass with fallopian tube
2. Contralateral ovarian biopsy
3. Deposits from Pouch of Douglas
4. Peritoneal tissue from anterior abdominal wall
5. Peritoneal tissue from lateral wall of abdomen
6. Peritoneal tissue from descending colon
7. Deposits from outer surface of small intestine
8. Infra colic omentum

7. GROSS
EXAMINATION
15/10/2014
(Biopsy No. S/1238/14)

8. 1. Right ovarian mass with fallopian tube
2. Contralateral ovarian biopsy
3. Deposits from Pouch of Douglas
4. Peritoneal tissue from anterior abdominal wall
5. Peritoneal tissue from lateral wall of abdomen
6. Peritoneal tissue from descending colon
7. Deposits from small bowel
8. Infra colic omentum
… …
. . . …
.. .. .. . . .

12. MICROSCOPIC
EXAMINATION

13.  Heterogenous tumor tissue composed of mixture of
immature and mature tissue, predominantly
immature embryonal type tissues in the form of
neuroectodermal rosettes.
 Immature mesenchyme in the form of loose,
myxoid stroma, with focal differentiation into fat,
osteoid, immature cartilage and rhabdomyoblasts.
 Immature endodermal structure including intestinal
type epithelium with basal vacuolation and blood
vessels also noted.
.
.
. .
.
. . .
.
.
……….
………………
…………………………..

14.  Mature elements are composed of
– Epidermis
– Dermal adnexal structures
– Fragments of bone and cartilage
– Ganglion cells
– Glial tissues
– Intestinal glands
.
………………………………………………………………………………………………………………………………………………………………………..
………………………………………………………………………………………………………………………………………………………………………………………………………….
…..
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….
….
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. ………………………..
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………………………………………………..

15. TERATOMA
• Exclusion of benign solid teratoma.
• Identification of other germ cell
component.
• Determining the grade of the tumor.
Grading based on the extent of
immature tissue within the teratoma

16. Grading of Immature Teratoma
(Gonzales-Crussi)
Grade I:
• Tumors with rare foci of immature neuropeithelial tissue
that occupy less than one low power field (40x) in any
slide
Grade II:
• Tumors with similar elements, occupying 1 to 3 low
power fields in any slide
Grade III:
• Tumors with large amount of immature neuroepithelial
tissue occupying more than 3 low power fields in any
slide.
WHO classification of tumors of the breast and female genital organs

17.  Sections from
– Contralateral Ovary,
– Pouch of Douglas,
– Peritoneal tissue from anterior abdominal
wall/ descending colon,
– Small intestine,
– Infra colic omentum
showed tumor deposits.
..
.
..
. .
. . .
.
..

18. Conclusion:
Immature teratoma - Grade III (WHO)
pT3bNxMx

19. DISCUSSION

21. Introduction
(Greek- root : Teratos: monster )
 The first description of teratoma was made in 1960 by
Thürlbeck and Scully.
 Teratomas are most frequently found in the gonads (ovary
and testes).
 Extragonadal teratomas are rare and arise from midline
structures (thyroid, retroperitoneum, mediastinum,
pericardium , coccyx and brain).
 Very rarely, teratomas are found in other solid (e.g. breast,
parotid gland, liver) and hollow (e.g. oesophagus, stomach,
bladder, uterine cervix) organs.
 Teratomas may be benign, malignant or a component of a
mixed germ cell tumor (GCT).

22.  Immature Teratoma is a malignant germ cell tumor composed
of tissues from three germ layers (ectoderm, mesoderm, and
endoderm), with immature embryonal type structures
(generally) neuroectodermal tissue.
 Less than 1% of ovarian tumors.
 Second most common germ cell tumor.
Outwater EK et al. (2001)
 10-20% cases in first two decades of life with a peak incidence
between 15 and 19 years.
 Results in 30% of the deaths from ovarian cancer in this age
group.
 Rarely occurs in post –menopausal age group.
Netchine I, et al. (2011)

23. Etiology
 Various theories
– Most teratomas (65%) are derived from a single germ
cell after the first meiotic division with subsequent
failure of meiosis II.
 Over expression of p53 gene (Charoenkwan et al.)
 Chromosomal abnormalities (Osterhuis et al.)
– Grade 1and 2 are diploid (90% ), while most grade 3
are aneuploid (66%)
– Lack of 12p amplification

24. Clinical features
 Abdominal pain and distension
 Abnormal uterine bleeding
 Urinary and GIT symptoms
 Anti-NMDA receptor encephalitis [Dabner M et al. (2012)]

25. Investigations
 Tumors markers :
– Alpha feto protein (AFP) can be raised in
50% of cases.
 USG / CT:
– Calcification

26. GROSS
 Usually unilateral; rarely
bilateral (less than 10%)
 Large (6-35 cm)
 Encapsulated with smooth,
glistening outer surface.
Ruptured in almost half of the
cases.
 Variegated , predominantly
solid, fleshy, gray-tan. May be
cystic with hemorrhage and
necrosis
Rosai and Ackerman’s surgical pathology 10th ed. (2011)

27. MICROSCOPIC
 Variable amount of Immature and mature
components
 Immature components:
– Immature ectodermal tissue
• Mainly neuroectodermal rosettes and tubules
– Immature mesenchyme
• Loose myxoid stroma with focal differentiation into
– Immature cartilage
– Immature fat
– Osteoid
– Rhabdomyoblasts
– Immature endodermal tissue
• Hepatic tissue
• Intestinal type epithelium with basal vacuolization
• Embryonic renal tissue with Wilms tumor

28. Neuroepithelial mimicker
 Ameloblastic
epithelium
(neuroepithelial mimic,
which can be a
potential pitfall for an
erroneous diagnosis
of immature
teratoma)

29. Immunohistochemisty
 Immature and mature neural tissue :
– Glial fibrillary acidic protein (GFAP)
– Long chain polysialic acid
 Immature neuroepithelium of high-grade
– Oct 4
Rosai and Ackerman’s surgical pathology 10th ed. (2011); Kwan MY et al. (2004)

30.  To determine the prognosis
 To determine the necessity of adjuvant
treatment
GRADING

31. Treatment
 Preservation of fertility is an important factor.
 Patients with grade I tumours staging surgery
with a unilateral oophorectomy.
 Patients with grade II or III tumors  adjuvant
chemotherapy containing bleomycin, etoposide and
cisplatin in addition to surgery.
 The current combination chemotherapy results in
overall disease free survival rate of >95%.
• Gershenson DM (2007), Pectasides D et al. (2008)

32. Prognosis
 The most important prognostic feature is the grade of
the lesion.
 The 5yr survival rates :
– Grade 1 lesions  82%
– Grade 2 lesions  63%
– Grade 3 lesions  30%
Abiko K et al. (2010)

33. Risk of recurrence
 The only predictor of recurrence is the
presence of histological foci of yolk sac
tumor rather than the grade of the
immature component.
 Most recurrences develop in the first two
years.
WHO classification of tumors of the breast and female genital organs

34. Conclusion
 Morphological spectrum of immature teratoma of
the ovary is varied, complex and offers
diagnostic challenges.
 Histopathological examination is important for
accessing the prognosis and recurrence of the
tumor.
 Early diagnosis associated with immediate
therapy and close follow-up are essential for
long term favourable outcomes.

35. References
1. Rosai J. Female reproductive system- ovary. In: Rosai J, editor. Rosai and Ackerman’s surgical
pathology. 10 th ed. New Delhi: Elsevier; 2011. p. 1587-88.
2. Tavassoli FA, Mooney A. Sex cord-stromal tumors. In: Fattaneh A, Tavassoli FA, Devilee P, editors.
WHO classification of tumors of the breast and female genital organs. Lyon. IARC press; 2003. p. 169-
70.
3. Outwater EK, Siegelman ES, Hunt JL. Ovarian teratomas: tumor types and imaging characteristics.
Radiographics. 2001;21(2):475-90.
4. Chabaud-Williamson M, Netchine I, Fasola S, Larroquet M, Lenoir M, Patte C, et al. Ovarian-sparing
surgery for ovarian teratoma in children. Pediatr Blood Cancer. 2011;57(3):429-34.
5. Gershenson DM. Management of ovarian germ cell tumors. J Clin Oncol 2007;25:2938-43
6. Pectasides D, Pectasides E, Kassanos D. Anti tumour treatment. Germ cell tumors of the ovary.
Cancer Treat Rev 2008;34:427-41
7. Abiko K, Mandai M, Hamanishi J, Matsumura N, Baba T, Horiuchi A, et al. Oct4 expression in
immature teratoma of the ovary: relevance to histologic grade and degree of differentiation. Am J Surg
Pathol. 2010;34(12):1842-8.
8. Kwan MY, Kalle W, Lau GT, Chan JK:Is gliomatosis peritonei derived from the associated ovarian
teratoma? Hum Pathol 2004, 35: 685–688. PubMedAbstract I Publisher Full Text.
9. Thurlbeck WM, Scully RE: Solid teratoma of the ovary. A clinicopathological analysis of 9 cases.
Cancer 1960, 13: 804– 811. PubMed I Publisher Full Text.

36. S/1238/14

37. S/1238/14

38. S/1238/14

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42. H&E x40

43. H & E x100
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44. H & E X40

45. H & E X200

46. H & E X100

47. H & E X100

48. H & E X400

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H & E X100

50. Section from Contralateral ovary
H & E X200

51. Section from POD
H & E X40

52. Peritoneal tissue from anterior abdominal wall
H & E X100

53. Peritoneal tissue from descending colon
H & E X40

54. Deposits form small intestine

55. Deposits from infracolic omentum
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H & E X40

57. GFAP