Sertoli-Leydig cell tumor

(1)

- 86 - Sertoli-Leydig cell tumor 3-40

75% 40

. 0.2%

low-grade malignancy .

Tumor androgen ,

70-85% .

, , ,

, , .

androgen testosterone, androste-

nedione dehydroepian-

drosterone sulfate . Sertoli-Leydig cell tumor

.

Sertoli-Leydig cell tumor 1%

. 5 Department of Obstetrics and Gynecology, College of Medicine, Hanyang University, Seoul, Korea

Department of Pathology, College of Medicine, Hanyang University, Seoul, Korea Department of Obstetrics and Gynecology, College of Medicine, Wonkwang University,

Gunpo Medical Center

The Sertoli-Leydig cell tumor is a rare sex cord stromal tumor that has low grade malignancy and low bilateral occurrence rate. About two third of recurrence appears within 1 year after the initial treatment and most common recurrence sites are intraperitoneal cavity and retroperitoneal lymph nodes. The frequency of malignant transformation is high in the poor differentiated cell type, retiform pattern, and tumor rupture. In young women with stage I, unilateral salpingo-oophorectomy is performed to preserve uterus and reproductive function. In more advanced stages, hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymph node dissection are performed and occasionally chemotherapy can be combined. We experienced a rare case of malignant metastasis in Sertoli-Leydig cell tumor, and presented with a brief review of literature.

Key Words : Sertoli-Leydig cell tumor, Malignant transformation, Recurrence

(2)

- 87 -

70-90% .

36

Sertoli-Leydig cell tumor .

: , 36

: 2-0-1-2

: 1983

2001 6 2001 11

Sertoli- Leydig cell tumor, intermediate differentiated with heterologous element 2001 11

,

2 Bleomycin, Etoposide, Cisplatin 4 Etoposide, Cisplatin

. 2002 10

(vault) 11×7 cm punch

,

Sertoli-Leydig cell

tumor . 2 Taxol, Epirubicin

palliative chemotherapy

2003 1 .

: 2002 10

11×7 cm

.

: .

: 11×7×8 cm

(internal iliac artery)

, S ,

(Fig. 1).

: WBC 4400/mm², Hb

13.0 g/dL, Hct 37.6%, Platelet 222,000/mm²

, ,

.

CA-125 56.6

.

: punch

.

. Sertoli-Leydig

cell tumor (Fig. 2).

Sertoli-Leydig cell tumor 1905 Pick ,¹ 1930 Meyer

(3)

- 88 - androblastoma arrhenoblastoma

2 WHO androblastoma Sertoli-

Leydig cell tumor .³

Roth Sertoli-Leydig cell tumor 4 .

1) Grade I (well differentiated) (11%)

Sertoli

Reinke crystalloid Leydig .

2) Grade II (Intermediate differentiation) (54%) embryonic testicular sex cord

Sertoli

, well defined tubule mature Leydig immature gonadal mesenchyme .

3) Grade III (poorly differentiated) (13%)

,

atypia . Sertoli

(cord) Leydig

4) Heterologous element (22%)

Intermediate poorly differentiated tumor mucinous epithelium, cartilage, bone, skeletal muscle, smooth muscle, fat

.

Young WHO classification

Sertoli-Leydig cell tumor well differentiated, intermediate differentiated, poorly differentiated,

retiform, mixed 5 .⁸

, Young Scully 207

3 . Young Scully

18%

97.5% I . 2.5%

. well differentiated tumor I poorly differentiated tumor

I

.⁹

Sertoli-Leydig cell tumor 2 ,

, ,

. testosterone

.⁵

testoterone

. 50% Sertoli-Leydig

cell tumor

.^4,5

. Young

Scully 23 well-differentiated

tumor ¹⁰ intermediate

differentiated tumor 11%, poorly differentiated tumor 58%, heterologous element 19%

. retiform pattern tumor rupture

. 64 intermediate poorly differentiated Sertoli-Leydig cell tumor 5

10 92%

.

.⁹

2/3 1

6-7% 5 .

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Sertoli-Leydig cell tumor ,

(4)

- 89 -

, .

I

.⁷ I

, poorly differentiated tumor , intermediate differentiated tumor intraoperative tumor rupture

, mesenchymal heterologous subtype , .

.¹² Adjuvant chemotherapy Cyclophosphamide Cisplatinum

Doxorubicin , Cyclophosphamide

vincristine actinomycin D

.¹³ LHRH agonist LHRH antagonist

.¹³

Sertoli-Leydig cell tumor 1

.

Sertoli-Leydig cell tumor 1% low grade

malignancy . , 2/3 1

. retiform pattern tumor rupture

. I

. I ,

. Sertoli-Leydig cell tumor

. : Sertoli-Leydig cell tumor, ,