abstract
BACKGROUND:
We present the results of the TGM-95 study for gonadal sex-cord stromal tumors (SCT).
METHODS:
Between
1995 and 2005, children (<18 years) with gonadal SCT were
prospectively registered. Primary gonadal resection was recommended
whenever feasible. Patients with disseminated disease or an incomplete
resection received neoadjuvant or adjuvant VIP chemotherapy (etoposide,
ifosfamide, cisplatinum).
RESULTS:
Thirty-eight
children with ovarian SCT were registered. Median age was 10.7y.
Endocrine symptoms were present in 21 cases. The histological diagnoses
were as follows: juvenile (23) and adult (3) granulosa cell tumors,
Sertoli-Leydig cell tumors (11), and mixed germ cell SCT (1). An initial
oophorectomy ± salpingectomy led to complete resection in 23 patients
who did not receive adjuvant treatment; two of them relapsed: one
achieved second complete remission whereas the other
one died of
disease.
Fifteen patients had tumor rupture and/or malignant ascites: 11
received chemotherapy and did not relapse,
four did not receive
chemotherapy and relapsed with a fatal outcome in two cases. With a
median follow-up of 5.9y, the 5-y EFS and OS rates were respectively 85%
and 94%. Eleven patients had localized testicular tumors (median age
0.83y): juvenile granulosa cell tumors (4), Sertoli or Leydig cell
tumors (5) and not otherwise specified SCT (2). Treatment was surgery
alone with an inguinal orchiectomy. None have relapsed (median
follow-up: 5.4y).
CONCLUSIONS:
Childhood
SCT carry favorable prognosis.
In ovarian SCT, surgery should be
complete and non-mutilating. Adjuvant chemotherapy efficiently prevents
recurrences in cases of tumor rupture. In childhood testicular SCT, the
prognosis is excellent with an inguinal orchiectomy, prompting the
debate on testis-sparing surgery.
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