13

p

: ,

From the Archives ; :: f the AFIP iuchard

P. Moser,

Jr,

Malignant Germ Cell Tumors Ovary: Radiologic-Pathologic Harry M. Brammer Wendelin S. Hayes, Malignant

germ

plasms are

and

tumors

of two immature

These

tumors

are

human tic

can

malignant

malignant with

have

images. levels

sinus

tumor,

germ

cell

a complex

can

suggestive

of these

mature

and

a mix-

tumors,

espe-

teratoma.

predominantly

serum

help

establish

of mature

neoneoplasms

(benign) but

Elevated

USNR MD

ovarian

known

endodermal All

and

gonadotropin

malignant

well

associated

large

or calcifications

existing

be

cross-sectional

chonionic

areas

USA

of the Correlation1

common most

elements.

generally

on

most The

teratoma,

of these

teratoma,

id appearance

the

women.

immature

or more

cially

are

young

dysgerminoma,

tune

MC,

LCDR, MC, USN #{149} James L. Buck, LCDR, MC, #{149} Sheila Sheth, MD #{149} Fattaneb A. Tavassoh

HI,

DO cell

in girls

Col,

teratoma

sol-

a-fetoprotein

and

the

Cys-

do

diagnosis. not

exclude

a co-

neoplasm.

. INTRODUCTION Tumors of germ cell origin are plasms. This group of neoplasms,

the

second which

are

most common group of ovarian neothought to derive from the primitive

germ cells of the embryonic gonad, include dysgerminoma, endodermal sinus tumon, embryonal carcinoma, mature teratoma, immature teratoma, and choniocancinoma. Malignant mixed germ cell tumors, which represent mixtures of two or more

of the

malignant

types,

also

Of the germ cell neoplasms, en, the most common lesion lignant

germ

lignant

cell

tumors,

neoplasms

mons

(i)

in particular

occur.

only mature in this group.

which .

Germ

occur

together

cell

tumors

in much

teratoma All the account

in general

younger

patients

common carcinomas derived from the ovarian than 2 1 years, the majority of ovarian tumors

Abbreviations: Index

AFP

terms:

Ovary,

RadloGraphics I

From

diologic Washington,

Pathology DC;

accepted

tute

ofPathology, The opinions

April

1 21 1

ofRadiology,

(J.L.B.) and the Department

Bethesda, and the

and

852.

human

#{149} Ovary.

National Gynecologic of Radiology

and

constitute the ma5% of ovarian ma-

malignant

than

those

epithelium. are of germ

chorionic

neoplasms,

germ

affected

cell by the

In patients cell origin,

tumore

younger and of these

gonadotropin

852.31

3. 852.329

Naval

1 1 . Address

reprint

the

views

of the

Medical

Center,

requests

ofRadiology. Hopkins

Md (H.M.B.);

toJ.L.B.,

of the

Forces Services

the

Departments

Institute University

Georgetown University Hospital. University Hospital, Baltimore, Md Department

Alaska and Fern Sts, Washington, herein are the private views Department

Bethesda,

and Breast Pathology (FAT.), Armed and Nuclear Medicine, Uniformed

Md (H.M.B.,J.L.B.); Department Department of Radiology, TheJohns

Bldg 54, Rrn M12l, and assertions contained

or as reflecting

HCG

It is by far, howev-

10:715-724

Department

Sciences, (W.S.H.);

ficial

CT,

1990;

the

a-fetoprotein,

is benign. other entities for less than

Na%)’,

the

of Radiologic

Pathology.

DC 20306-6000. of the authors and Department

of the

are

Army,

not

o

Washington, (S.S.). Received Armed

Forces

to be construed

or the

Ra-

of Pathology, of the Health

Department

DC Instias of-

of Dc.

fense. c

RSNA,

1990

715

b.

a. Figure

1. Dysgerminoma of the ovary. (a) Sonogram shows a large echogenic mass arising from the pelvis. (b) Contrast material-enhanced computed tomographic (CT) scan shows a homogeneous mass with attenuation values of soft tissue. (c) Findings from cut gross specimen confirm the almost completely solid radiologic appearance of the tumor. Fine fibrous septa running through the tumor

are

not

more

than

visible

40%

on the

radiologic

arc malignant

images.

(2)

.

Malignant

germ cell tumors account for approximately two-thirds of the ovarian cancers that occur in the first 2 decades of life (3). Although the common ovarian tumors of

epithelial

origin

ly occurring

and

mature

as a dermoid

frequently

discussed

that

Registry

Forces

ington,

DC,

cases,

have

been

of Radiologic

Armed

pathologic

litena-

tumors have been reports (4-7) We of our review of germ cell tumor of .

to the

Pathology

at the

of Pathology, the

past

Wash-

1 5 years.

material,

usually

In all

both

tognaphs of gross specimens and glass of histologic specimens, was available

correlation

with

the radiologic

diagnoses immature nus tumor germ cell

pelvic

been

submitted

Institute during

(usual-

have

in the radiology

tune, malignant germ cell described only in isolated present herein the results the 34 cases of malignant

the ovary

teratoma cyst)

phoslides for

images.

The

included dysgenminoma (n = 10), teratoma (n 8) endodenmal si(n 1 4), and malignant mixed tumor (n 2) Coexisting mature teratoma was present in two cases of endodermal sinus tumor and in all cases of immature teratoma.

In our

,

.

combined

cases,

the time of diagnosis was 4-27 years) All patients .

vic or abdominal

716

U

Ra4ioGrapbics

masses,

U

the average 1 3 .8 years

age at (range,

presented with 19 complained

Brammer

C.

et al

or abdominal

pain,

and

six presented

with fever. Two of the patients with dysgerminoma and one with an endodermal sinus tumor presented while pregnant, and anothen 1 9-year-old patient with dysgenminoma

had

never

tein

(AFP)

menstruated. level

was

The

serum

elevated

a-fetopro-

in three

of five

patients with immature teratoma and in all six patients with endodermal sinus tumor in whom it was tested. In one of three patients with endodenmal sinus tumor ed, the serum human chonionic pin (HCG) level was elevated.

The

average

1 5 5 cm and .

the individual

who were gonadotro-

test-

diameter of the tumors was was approximately the same for types of tumors. The tumor

was unilateral in 32 cases and involved both ovaries in one case each of dysgenminoma and malignant mixed germ cell tumor. Calcifications were present in 1 4 cases (three, dysgerminoma; eight, immature teratoma; two, endodermal sinus tumor; one, malignant mixed germ cell tumor) The internal consistency of the tumor was homogeneously solid in one case (dysgerminoma) greaten .

pdof

,

Volume

10

Number

4

d.

C

Figure

2

Dysgerminoma (D) of the ovary. C cystic spaces, A ascites. Longitudinal (a) and transverse (b) sonograms show a large complex mass. Tumor is predominantly solid, but large cystic spaces are interspersed within the mass. A considerable amount of ascites is present. A coexistent intrauterine pregnancy is seen on the longitudinal sonogram (arrow) (C) CT scan demonstrates solid and cystic components within the tumor and ascites. (d) Findings from cut gross specimen (and histologic specimen [not shown]) confirm the presence of cystic necrosis within the predominantly solid tumor. .

than

50%

solid

oma;

six,

immature

mal cell four

in 29 cases

sinus tumor)

cell

cystic.

The

tumor; .

malignant mixed was completely

appearance

pathologic

accurately

findings

in

DISCUSSION

The

currently

cell tumors the World In a review ovary were

accepted

were

germ

dysgerminomas tune tenatomas

tumors

younger

mature

malignant

malignant

for tumors

classification

of germ

of the ovary was introduced by Health Organization in 1 973 (8). of almost 300 neoplasms of the

in patients

38.6%

1990

one,

No tumor

radiologic

reflected the gross each case.

July

,

,

germ

cell

dysgermin-

1 3 endoder-

sinus tumor; one, malignant mixed germ tumor) and greaten than 50% cystic in cases (two, immature teratoma; one, en-

dodermal

U

(nine,

teratoma;

than

teratomas

germ

cell

cell

tumors

accounted for 25.8%,

19.9%, for

28.6% (2)

in that for 38.7%, endodenmal

malignant 13.5%,

tumors

and

mixed primary

.

for 2 1 %; there were no embryonal carcinomas. In our smaller series, the distnibution of tumor types was similar, except for a larger percentage of endodermal sinus tumors and the additional absence of choniocarcinoma. .

Of the

series, immasinus

germ chonio-

.

Dysgerminoma

Dysgerminoma,

the

homologue

of the

testic-

ular seminoma, in its pure form is not associated with elevation of either the serum AFP or HCG level. Syncytiotrophoblastic giant cells, however, which produce HCG, are present in 5% of dysgerminomas and can dcvate serum HCG levels (9) The tumors are generally solid (Fig 1), but areas of hemor.

rhage

20 years,

and

carcinomas

and

necrosis

can be seen

(Fig

2)

Cal-

.

cification is reported to be rare in pure dysgenminomas (3) but calcification was present in three of our 1 0 cases (Fig 3) The speckled pattern of calcification seen in these dysgerminomas was clearly different from that of the calcification seen in the othen malignant germ cell tumors when they ,

.

Brammer

et al

U

Ra4ioGrapbics

U

717

a.

d.

b.

C

3.

Dysgerminoma of the ovary. (a) Abdominal radiograph reveals a large mass arising from the pelvis and extending to the upper abdomen. (b) Detailed view of the night upper quadrant shows calcifications scattered throughout the cephalic portions of the tumor (arrowheads) (C) CT scan obtained at the level of the kidneys also demonstrates the tumoral calcification (arrowhead) (d) Cut gross specimen shows a large area with an irregular surface, caused by calcifications, as well as small areas of necrosis (arrowheads) Although such calcification is suggestive of a preexisting gonadoblastoma, no clinical or pathologic evidence for gonadoblastoma was noted in this patient. Figure

.

.

.

were

associated

with

mature

presence of calcification oma is strongly suggestive ment

(3)

unusual

neoplasm

curs

U

from

gonadoblastoma germ

718

of dysgerminoma gonadal

cell

and

in phenotypic

Ra4ioGrapbks

teratomas.

stromal

.

The

within dysgerminof the developan

composed

elements, females

#{149} Brammer

with

usually gonadal

et al

(3) The presand underlying .

gonadoblastoma was suggested on clinical examination in one of our cases by the absence of menarche in a 1 9-year-old patient with bilateral calcified dysgerminomas.

a preexisting

Gonadoblastoma,

genesis and Y chromosomes ence of gonadal dysgenesis

of

ocdys-

However,

the

genotype

of that

patient

is un-

known.

Volume

10

Number

4

b

d. Immature teratoma (recently acquired case not included in our original review) (a) Abdomiml radiograph reveals a huge mass containing widely scattered calcifications. (b) CT scan obtained at the level of the kidneys shows the complex nature of the mass. The solid central area of the tumor ( T) conta.ins scattered calcifications and is surrounded by a larger cystic component. (C) The mass seen in the pcIvis posterior to the uterus (*) represents a cul-de-sac metastasis from the primary immature teratoma. Although most penitoneal implants identified at laparotomy were composed of mature glial tissue, areas of immature neural tissue were also found. (d) Gross specimen demonstrates the variegated appearance of the immature teratoma and its associated mature teratomatous elements. C.

Figure

.

4

Immature

.

Teratoma

Immature tenatomas, which have also been called embryonal, malignant, or solid teratomas, contain immature tissues that resemble

those

of the

also wide sent

present variety all three

the predominant nancy

system

July

1990

embryo.

Mature

in most cases of immature germ layers

tissue

elements

are

(3) Although a tissues that nepreis often identified, .

indicative

is primitive neuroectoderm. (grade 0-3) that relates

of maligA grading to the

amount of immature neunoectodermal tissue within the primary tumor has been adopted (1 0). The histologic grade of the metastases has also been shown to affect prognosis (1 1) At times, immature teratoma may be as.

sociated mature

with

multiple

penitoneal

glial tissue (Fig gliomatosisperitonei,

termed ciated with long-term teratomas are typically evations

of serum

Brammer

4).

This

implants

survival not associated

HCG

levels

et al

(1 0),

U

of

condition, is usually asso(3) Immature .

with but

el-

in a

Ra4ioGrapbics

#{149} 719

Figure 5. Immature teratoma of the ovary. C = cystic spaces. (a) Abdominal radiograph shows a large soft-tissue mass displacing the large and small bowels (arrows) Scattered calcifications are seen within the mass (arrowheads). (b) Longitudinal sonogram shows a large complex mass with cystic and solid components. Calcification (arrow) with distal acoustical shadowing (arrowheads) is seen. (C) Cut gross specimen shows a predominantly solid tumor containing a few cystic spaces. The cystic spaces and the teeth (not seen in this section) were caused by a coexisting mature tcratoma. .

a.

review

of the recent

literature,

tumors

had

APP

The

elevated

tumors

50% of these

levels

are usually

(12).

predominantly

sol-

id, but numerous cysts of varying size are also seen (Figs 4 5) Immature teratoma is occasionally composed almost entirely of cysts, which makes the term solid teratoma a poor synonym for this malignant neoplasm. Calcification is seen because of the almost invariable association with mature teratoma. ,

while

moid

is typically

cyst

nodule, sue

scattered the

they

calcified peanance graphs

experience,

720

#{149} RadioGraphics

with

in the mature teratoma

the tumor.

calcifications

more

to a mural

immature may

commonly

Also,

tis-

examination,

sinuses

of the

portion

of patients

rat yolk

also known resemble,

as on

the endodermal sac

with

(1 3).

A high

endodermal

pro-

sinus

tumors are found to have elevated serum AFP levels (1 3 1 4). The typical neoplasm is predominantly solid, but, again, cystic areas are commonly seen (3 1 3) On histologic review, we found that these cystic areas were either epithelial-lined cysts produced by the ,

endodermal

are

al-

sinus

commonly, mas. In the

represent

are fragments

are they

,

of the den-

localized

throughout

though

teeth,

the calcification

the calcifications

associated

microscopic

.

However,

. Endodermal Sinus Tumor Endodenmal sinus tumors yolk sac tumors because

of

cartilage or bone. The resultant apof immature teratoma on radioand cross-sectional images is, in our

cysts latter

tumor

.

itself

(Fig

6) or, less

of coexisting mature teratocase, calcification may also

be identified. As with the other malignant germ cell tumors, endodermal sinus tumor may

be associated

implants

(Fig

with

ascites

and

penitoneal

7).

characteristic.

U

Brammer

et al

Volume

10

Number

4

a. Figure 6. large mass but several

b. Endodermal sinus tumor. (a) Longitudinal sonogram through the middle of the pelvis shows a above the urinary bladder and ascites (A) in the cul-de-sac. The mass is predominantly solid, cystic areas are seen within it. (b) Cut gross specimen shows the minor cystic component (C).

ci. intraabdominal metastases (recently acquired case not included in our original review) (a) CT scan demonstrates a complex mass in the posterior pelvis that was found at surgery to have completely replaced the left ovary. Anterior to the ovarian mass is a mass of higher attenuation (*) , which represents an omental metastasis. A dermoid cyst that contains a large amount of sebum, which has attenuation similar to that of fat, is incidentally seen in the right ovary. Implants of endodermal sinus tumon arc seen on the outside surface of the tumor (arrowhead) (b, C) More cephalic sections from the same examination show extensive ascites and multiple metastatic implants (arrowheads) along the peritoneum. (d) Cut gross specimen of the tumor and its adjacent metastases shows the solid tumor ( T) , which has almost replaced the entire ovary. Residual hemorrhagic corpus luteum (arrowhead) is incidentally seen. C.

Figure

7.

Endodermal

sinus

tumor

with

.

.

July

1990

Brammer

et al

#{149} RadioGrapbics

#{149} 721

.

Malignant

Mixed

Malignant

mixed

composed

of more

Germ

germ

cell

than

Cell

one

with the use of current classification criteria, only 1 5 cases of embryonal carcinoma were identified, 1 1 of which were pure (1 7) The tumors were described as being predomi-

Tumor

tumors

are

those

malignant

germ

.

cell component. It has been stated that mixed tumors account for 8% of the malignant germ cell tumors of the ovary, compared with 40% in the testis (1 1) . There are

differences,

however,

in terminology

nantly solid, with areas of extensive and hemorrhage commonly seen.

els of HCG

and

vated

classification between testicular and ovarian germ cell tumors in regard to teratomas. For example, a combination of endodermal sinus tumor and mature teratoma would be labeled

a (malignant)

mixed

germ

cell

tumor

U

metastasize

and are,

therefore,

in the

not desig-

nated as benign (3) The appearance of a malignant mixed germ cell tumor varies according to its individual constituents, but it is generally a complex, predominantly solid tumor, as are the other germ cell tumors (15,16) (Fig 8). .

Choriocarcinoma (nongestational) cinoma is an extremely S

ovarian rare germ

Primary

choniocarcell tumor,

of well-defined

cifications, tune

which

teratoma,

malignant

cystic

does

neoplasm,

not

exclude

especially

of germ

arising

from

an ectopic

ovarian

preg-

nancy. The nongestational ovarian choniocarcinomas have been described as typically unilateral and predominantly solid with hemorrhage

tional that

contain

nied .

and

Embryonal

bryonal

largest

RadioGrapbks

(1)

germ

choniocarcinoma

by elevated

Embryonal cal to, but the

necrosis

choniocarcinoma, serum

HCG

.

As in gesta-

cell

tumors

are

accompa-

levels

(1).

carcinoma of the ovary is identimuch less common than, the emseries

of the

testis.

of embryonal

U

Brammer

In fact,

carcinoma,

et al

in

a coexisting

when

signifi-

also

cases older

from

epithelial neoplasms (eg, serous on cystadenoma on carcinoma) are

predominantly

cystic

lesions

in most

and generally occur in middle-aged women. Likewise, the other solid

malignant

germ

cell

the olden age of the patients en hand, certain neoplasms

mal cell

origin,

Sertoli-Leydig as predominantly women (3),

such cell

can

and ovar-

thecoma, and be distinguished

tumors

because

of

(3) On the othof gonadal stro.

as granulosa

cell

and

tumors, which may occur solid tumors in younger be difficult to differentiate

from malignant germ cnine effects of these mors, however, often

Carcinoma

carcinoma

a

or cal-

of ma-

cant amounts of solid tumor are present. The common causes of an ovarian mass in girls and young women include functional cysts, endometriosis, tubo-ovarian abscesses, and mature teratoma; however, these are predominantly on completely cystic tumors. The

ian tumors, such as fibnoma, Brennen tumor, can usually

form

spaces

may be suggestive

it is accompanied

a tumor

U

presence

common mucinous

by another

be dc-

SUMMARY

with approximately 50 cases reported in the literature, mainly in patients younger than 20 years (1,3). A germ cell origin of ovarian choniocarcinoma can be established with certainty only in the prepubertal patient on if cell tumor. In patients of reproductive age, pure ovarian choniocarcinoma could represent a metastasis from the uterus on possibly

722

occasionally

(1).

The diagnosis of malignant germ cell tumors of the ovary should be strongly considered whenever a large, predominantly solid, ovarian tumor is discovered in a girl on young woman. Mature tenatoma may coexist with any of the malignant germ cell tumors, particularly immature teratoma. Therefore, the

testis; in the ovary, the same tumor would be labeled endodermal sinus tumor with mature teratoma because testicular mature teratomas

may

and AFP may

necrosis Serum 1ev-

cell tumors. The endogonadal stromal tuhelp distinguish them

from the germ cell neoplasms. Ovarian tonsion, which commonly affects girls and young women, appears as a predominantly solid ovarian lesion and as such may mimic

the appearance of malignant germ mors. The smaller size of the mass

Volume

cell

tu-

and

char-

10

Number

4

a.

b. Figure the

8.

Malignant

ovary.

(a)

soft-tissue

mixed

Radiograph

germ

of the

cell pelvis

tumor

of

shows

a

mass

(arrows) that contains numerous teeth (arrowheads) (b) Longitudinal sonogram shows a complex mass (black arrows) with large cystic areas (C) (larger and more numerous cystic areas were seen in other sections) Several bright echogenic foci (white arrows) with distal acousti.

.

cal

shadowing

bivalved

gross

view, els

extensively

preoperative of AFP

agnostic 30 years

These

in the cases assessment

teratoma,

component

who

tests

present

may

also

of serum

with

an adnexal

1

.

REFERENCES Gershenson mors

2

.

of the

1990

ovary.

and

4.

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tumors Obstet

tu-

in Gyne-

8.

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seen

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on the

in this section

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.

in our

most

tumor logic

not used

were

seen,

(C)

(C) In this

specimen.

mature

ages

The presence of elevated serum levels of AFP and/on HCG may help establish the diagnosis of malignant germ cell tumors, particularly endodenmal sinus tumor or choniocarcinoma. Although these serologic tests were

are

with calcifications. small cystic spaces

spaces

actenistic follicular enlargement seen with ovarian torsion, however, should lead to the proper diagnosis in most cases (18).

(arrowheads)

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Volume

evaluation and adoles-

10

Number

4