1 HB Paul H. Kim

2 Outline Case Presentation Case Presentation Teratomas Teratomas OGCT OGCT Mature cystic teratoma Mature cystic teratoma

3 HB 5 yo female 5 yo female 1 wk h/o abd pain, anorexia, subj fever 1 wk h/o abd pain, anorexia, subj fever Dx = flu, bladder infxn, a.appendicitis Dx = flu, bladder infxn, a.appendicitis OSH U/S = acute appendicitis OSH U/S = acute appendicitis PE = +guarding, +RLQ pain PE = +guarding, +RLQ pain WBC ~10, UA (-)ve WBC ~10, UA (-)ve Post-anesthesia exam Post-anesthesia exam –Mobile cystic mass in pelvis thought to be the bladder

4 DDx Acute appendicitis Acute appendicitis Gastrointestinal Bezoar Gastrointestinal Bezoar Ingested FB Ingested FB Ovarian Cystic Teratoma Ovarian Cystic Teratoma Ureterolithiasis Ureterolithiasis

5 Exploratory Laparoscopy

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9 Pathology Right ovary – mature cystic teratoma Right ovary – mature cystic teratoma –Ovary with primary & secondary follicles, cyst wall mix of keratinizing SCC, glial tissue, ependyma, hail follicles, SQ, bone, pigmented retinal cells, no malig cells Appendix – no transmural inflamm Appendix – no transmural inflamm Cytology – peritoneal fluid, no malig cells Cytology – peritoneal fluid, no malig cells

10 Post Op Labs Post Op Labs –Beta HcG < 1 –AFP < 0.8 –LDH 499

TERATOMA 11

12 Teratoma Def: Def: –Embryonal tumors derived from 2 to 3 germ layers, differentiate into identifiable tissues & organs at ectopic locations Most common teratoma = sacrococcygeal Most common teratoma = sacrococcygeal –Teratomas ~3% of pediatric malignancies

13 Many teratomas produce elevated tumor marker Many teratomas produce elevated tumor marker   FP: produced by embroynal liver, intestine, & yolk sac (t ½ 5 d), should decrease to adult lvls by 8 months of age Eg, yolk sac tumors Eg, yolk sac tumors   Hcg: normally made by syncytiotrophoblasts (t ½ 16h), abnormal elevation indicates teratoma with choriocarcinoma Eg, choriocarcinomas Eg, choriocarcinomas

OVARIAN GERM CELL TUMOR 14

15 Ovarian GCT Intro Intro –2/3 all malignant ovarian tumors are GCT Teratoma is most common GCT Teratoma is most common GCT –Teratoma’s are embryonal tumors derived from 2 or 3 germ layers Ovarian teratomas contain 3 germ layers Ovarian teratomas contain 3 germ layers

16 OGCT Dysgerminoma Dysgerminoma Endodermal Sinus Tumor Endodermal Sinus Tumor Embryonal Carcinoma Embryonal Carcinoma Polyembryoma Polyembryoma Choriocarcinoma Choriocarcinoma Teratoma (im, mat, & monodermal) Teratoma (im, mat, & monodermal) Mixed forms Mixed forms Gonadoblastoma Gonadoblastoma

17 Ovarian Germ Cells No DifferentiationDifferentiation DysgerminomaEmbryonal CA Extra Embryonic TissueEmbryonic Tissue Teratoma Endodermal Sinus – Yolk Sac Tumor Choriocarcinoma

18 Incidence / Prevalence Incidence / Prevalence –Varies with age eg, <18 yo GCT comprise 60-74% of ovarian malignancies eg, <18 yo GCT comprise 60-74% of ovarian malignancies –GCT comprise 15-20% ovarian tumors for pts <20 yo & 60% of all ovarian tumors The younger the pt the more likely tumor is malignant The younger the pt the more likely tumor is malignant

19 Ovarian GCT = primordial germ cells Ovarian GCT = primordial germ cells –OGCT can be Malignant or Benign –Median size – 16 cm Common Characteristics Common Characteristics –Occur in young women & girls –Often produce tumor markers –Most common is Dermoid Cyst Dermoid Cyst = Mature Ovarian Teratoma Dermoid Cyst = Mature Ovarian Teratoma

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21 Classification of Ovarian Teratoma Classification of Ovarian Teratoma –Mature cystic or solid, benign cystic or solid, benign –Immature Malignant Malignant –Degree of immaturity depends on cellular differentiation & foci of neuroepithelium –Monodermal / highly specialized Struma Ovarii Struma Ovarii

22 Ovarian Teratoma Ovarian Teratoma –Usually unilateral involvement, but up to 8-10% bilateral

23 Clinical Presentation Clinical Presentation –Abd mass &/or pain - 85% –Ascites – 20% –Rupture – 20% –Torsion – 20% –Fever or vag bleeding - 10%

24 OGCT Markers AFPhCGLDH Dysgerminoma-+/-+ Yolk Sac +-+ Imm Tera +/- +/-+/-+/- Mix GCT +/-+/-+/- ChorioCA-++/- Embry CA +/-++/- Polyembr+/-+_

25 Complications Complications –<1% malignancy –16% torsion –Rupture  granulomatous peritonitis

MATURE CYSTIC TERATOMA 26

27 Mature Cystic Teratoma AKA Dermoid Cysts AKA Dermoid Cysts –Def: tumors that arise from totiopotential primordial germ cells that display all 3 germ layers –Dermoids comprise 38.6% of ovarian neoplasms & 57% pediatric GCT’s –80% dermoids occur during reproductive age

28 Prognosis Dermoid Cyst Prognosis Dermoid Cyst –~50% benign cysts resolve in few month –Malignant transformation is RARE 0.94% transformation into SCCa 0.94% transformation into SCCa –Tangjitgamol et al. Int J Gynecol Cancer 2003 Jul- Aug;13(4):558

29 S/Sx: S/Sx: –Usually asymptomatic, incidental finding –Symptoms include abd pain, abd mass, abnormal uterine bleeding

30 Dx = Imaging Dx = Imaging –US, CT, MRI, Abd XR –Aspiration should NOT be done due to uncertainty of malignancy All germ layers represented on path All germ layers represented on path –Ectodermal, mesodermal, & endodermal

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32 Complications Complications –Torsion Sx emergency Sx emergency –Most common complication of cystic teratoma Occurs more commonly in young due to excessively mobile mesovarium & fallopian tubes Occurs more commonly in young due to excessively mobile mesovarium & fallopian tubes Pain due to veno-occlusion with concurrent arterial perfusion  edema, distention, & hemorrhage Pain due to veno-occlusion with concurrent arterial perfusion  edema, distention, & hemorrhage

33 Complications (cont) Complications (cont) –Rupture –Malignancy Squamous cell most common Squamous cell most common Elevated AFP & HCG Elevated AFP & HCG –Survival is inversely proportional to grade of immature elements & stage of disease

34 Tx = cystectomy Tx = cystectomy –Mature teratomas are cured via Sx resection only –R/O peritoneal seeding since miliary & glial intraperitoneal implants have been obs with mature teratomas

35 Laparoscopy vs. Laparotomy Laparoscopy vs. Laparotomy –Cochrane Review: Laparoscopy had 8.3% Adverse events whereas Laparotomy had 21.7% Laparoscopy had 8.3% Adverse events whereas Laparotomy had 21.7% Laparoscopy has less post-op pain, fewer days in hospital Laparoscopy has less post-op pain, fewer days in hospital

36 References Tangjitgamol et al. Int J Gynecol Cancer 2003 Jul- Aug;13(4):558 Tangjitgamol et al. Int J Gynecol Cancer 2003 Jul- Aug;13(4):558 Lazar EL, Stolar CJ: Evaluation and management of pediatric solid ovarian tumors. Semin Pediatr Surg 7(1):29-34, 1998 Lazar EL, Stolar CJ: Evaluation and management of pediatric solid ovarian tumors. Semin Pediatr Surg 7(1):29-34, 1998 Brown MF, Hebra A, McGeehin K, Ross AJ 3rd: Ovarian masses in children: a review of 91 cases of malignant and benign masses. J Pediatr Surg 28(7):930-3, 1993 Brown MF, Hebra A, McGeehin K, Ross AJ 3rd: Ovarian masses in children: a review of 91 cases of malignant and benign masses. J Pediatr Surg 28(7):930-3, 1993 Liu et al. Sudden onset of RLQ pain after heavy exercise. Am Fam Physician 2008 Aug 1;78(3): , 384 Liu et al. Sudden onset of RLQ pain after heavy exercise. Am Fam Physician 2008 Aug 1;78(3): , 384