Germ Cell Tumors of the Ovary: A Review.

Available online 29 July 2022

Abstract

Ovarian germ cell tumors are a diverse group of benign and malignant neoplasms that occur in a wide age range, but with a predilection for younger age group. The majority are represented by the frequently encountered mature cystic teratomas. Malignant germ cell tumors are uncommon, and in some cases have a characteristic clinical presentation. However, from a histologic standpoint these tumors can sometimes be challenging to diagnosis due to overlapping morphology with epithelial and in some cases sex cord tumors. In these cases, a panel of immunohistochemical stains often facilitates the correct diagnosis. This review article discusses the clinicopathologic findings and pertinent ancillary studies of both common and uncommon germ cell tumors of the ovary.

Section snippetsOverview

Germ cell tumors are second most ovarian common tumors, following surface epithelial tumors and are derived from the pluripotent primordial germ cells. The majority are benign (95%), and the malignant ovarian germ cell tumors are overall rare, accounting for only ∼5% of ovarian cancers. A SEER database study showed that the majority (93%) of patients with malignant ovarian germ cell tumors were less than 40 years of age, and the median age at diagnosis was 22 years.1 While most tumors occur in

References (104)G Cormio et al.Increased human Chorionic Gonadotropin levels five years before diagnosis of an ovarian dysgerminoma

Eur J Obstet Gynecol Reprod Biol

(2018)

JR Brettell et al.Dysgerminoma with syncytiotrophoblastic giant cells presenting as a hydatidiform mole

Gynecol Oncol

(1984)

DS Kapp et al.Pure dysgerminoma of the ovary with elevated serum human chorionic gonadotropin: diagnostic and therapeutic considerations

Gynecol Oncol

(1985)

Y Tatekawa et al.A case of pediatric ovarian dysgerminoma associated with high serum levels and positive immunohistochemical staining of neuron-specific enolase

J Pediatr Surg

(2004)

D Vicus et al.Pure dysgerminoma of the ovary 35 years on: a single institutional experience

Gynecol Oncol

(2010)

P Daher et al.Growing teratoma syndrome: first case report in a 4-year-old girl

J Pediatr Adolesc Gynecol

(2015)

D Perez Montiel et al.The value of SATB2 in the differential diagnosis of intestinal-type mucinous tumors of the ovary: primary vs metastatic

Ann Diagn Pathol

(2015)

V Garcia-Barriola et al.Ovarian ependymoma. A case report

Pathol Res Pract

(2000)

DM Gershenson et al.Conundrums in the management of malignant ovarian germ cell tumors: Toward lessening acute morbidity and late effects of treatment

Gynecol Oncol

(2016)

C Nezhat et al.Familial cystic teratomas: four case reports and review of the literature

J Minim Invasive Gynecol

(2010)

O Solheim et al.Prognostic factors in malignant ovarian germ cell tumours (The Surveillance, Epidemiology and End Results experience 1978-2010)

Eur J Cancer

(2014)

A Ronchi et al.Extragonadal germ cell tumors: Not just a matter of location. A review about clinical, molecular and pathological features

Cancer Med

(2019)

A Stang et al.Gonadal and extragonadal germ cell tumours in the United States, 1973-2007

Int J Androl

(2012)

A Hodgson et al.Somatically Derived Yolk Sac Tumor of the Ovary in a Young Woman

Int J Gynecol Pathol

(2021)

FF Nogales et al.Yolk sac tumours revisited. A review of their many faces and names

Histopathology

(2012)

JT Comerci et al.Mature cystic teratoma: a clinicopathologic evaluation of 517 cases and review of the literature

Obstet Gynecol

(1994)

P Kaatsch et al.Pediatric germ cell tumors from 1987 to 2011: incidence rates, time trends, and survival

Pediatrics

(2015)

WM Thurlbeck et al.Solid teratoma of the ovary. A clinicopathological analysis of 9 cases

Cancer

(1960)

R Rathore et al.Clinicopathological Evaluation of 223 Cases of Mature Cystic Teratoma, Ovary: 25-Year Experience in a Single Tertiary Care Centre in India

J Clin Diagn Res

(2017)

OM Curling et al.Malignant change in benign cystic teratoma of the ovary

Br J Obstet Gynaecol

(1979)

V Ulker et al.Malignant transformation arising from mature cystic teratoma of the ovary: a report of six cases

J Obstet Gynaecol Res

(2012)

OF Garcia-Galvis et al.Malignant Mullerian mixed tumor of the ovary associated with yolk sac tumor, neuroepithelial and trophoblastic differentiation (teratoid carcinosarcoma)

Int J Gynecol Pathol

(2008)

LJ McNeilage et al.Metastatic malignant melanoma arising in a mature ovarian cystic teratoma: a case report and literature review

Int J Gynecol Cancer

(2005)

AM Quadri et al.Malignant transformation in mature cystic teratoma of the ovary: three cases mimicking primary ovarian epithelial tumors

Int J Surg Pathol

(2011)

SH Ueng et al.Ovarian malignant melanoma: a clinicopathologic study of 5 cases

Int J Surg Pathol

(2010)

N Kuroda et al.Compound melanocytic nevus arising in a mature cystic teratoma of the ovary

Pathol Int

(2001)

AA Moulla et al.Rare Skin Adnexal and Melanocytic Tumors Arising in Ovarian Mature Cystic Teratomas: A Report of 3 Cases and Review of the Literature

Int J Gynecol Pathol

(2016)

A Paola et al.A rare melanoma feature with primary ovarian origin: a case report and the literature review

Obstet Gynecol Sci

(2018)

GM Kleinman et al.Primary neuroectodermal tumors of the ovary. A report of 25 cases

Am J Surg Pathol

(1993)

L Liang et al.Primary Glial and Neuronal Tumors of the Ovary or Peritoneum: A Clinicopathologic Study of 11 Cases

Am J Surg Pathol

(2016)

A Tanimoto et al.Teratoid carcinosarcoma of the ovary with prominent neuroectodermal differentiation

Pathol Int

(2001)

ED Euscher et al.Uterine tumors with neuroectodermal differentiation: a series of 17 cases and review of the literature

Am J Surg Pathol

(2008)

L Wheeler et al.Adenocarcinoma With Breast/Adnexal and Upper Gastrointestinal Differentiation Arising in an Ovarian Mature Cystic Teratoma: A Case Report and Review of the Literature

Int J Gynecol Pathol

(2016)

JK McKenney et al.Ovarian mature teratomas with mucinous epithelial neoplasms: morphologic heterogeneity and association with pseudomyxoma peritonei

Am J Surg Pathol

(2008)

R Vang et al.Ovarian mucinous tumors associated with mature cystic teratomas: morphologic and immunohistochemical analysis identifies a subset of potential teratomatous origin that shares features of lower gastrointestinal tract mucinous tumors more commonly encountered as secondary tumors in the ovary

Am J Surg Pathol

(2007)

N Aldaoud et al.The utility of PAX8 and SATB2 immunohistochemical stains in distinguishing ovarian mucinous neoplasms from colonic and appendiceal mucinous neoplasm

BMC Res Notes

(2019)

V Phupong et al.Ovarian teratoma with diffused peritoneal reactions mimicking advanced ovarian malignancy

Arch Gynecol Obstet

(2004)

M Fukushima et al.Peritoneal melanosis secondary to a benign dermoid cyst of the ovary: a case report with ultrastructural study

Int J Gynecol Pathol

(1984)

LM Roth et al.The enigma of struma ovarii

Pathology

(2007)

J CD Prat et al.ZaloudekZaloudek