Info

± 5.5

Abbreviation: SD, standard deviation.

From Wu JT, Book L, Sudar K: Serum a-fetoprotein (AFP) levels in normal infants. Pediatr Res 1981;15:50-2, with permission.

Abbreviation: SD, standard deviation.

From Wu JT, Book L, Sudar K: Serum a-fetoprotein (AFP) levels in normal infants. Pediatr Res 1981;15:50-2, with permission.

Table 24-5. Staging Systems for Germ Cell Tumors

Stage Extent of disease Extragonadal

I Complete resection at any site, including coccygectomy for sacrococcygeal site;

negative tumor margins; tumor markers positive but fall to normal or markers negative at diagnosis; lymphadenectomy negative for tumor

II Microscopic residual disease; lymph nodes negative; tumor markers positive or negative

III Gross residual or biopsy only; retroperitoneal nodes negative or positive; tumor markers positive or negative

IV Distant metastases, including liver

Ovarian

I Limited to ovary or ovaries; peritoneal washings negative for malignant cells; no disease beyond ovaries; presence of gliomatosis peritoneia does not result in changing stage I disease to a higher stage; tumor markers normal after appropriate half-life decline (AFP, 5 days; P-hCG, 16 hours)

II Microscopic residual or positive lymph nodes (<2 cm); peritoneal washings negative for malignant cells; presence of gliomatosis peritonei does not result in changing stage II disease to a higher stage; tumor markers positive or negative

III Lymph node involvement (metastatic nodule) >2 cm; gross residual or biopsy only; contiguous visceral involvement (omentum, intestine, bladder); peritoneal washings positive for malignant cells; tumor markers positive or negative

IV Distant metastases, including liver

Testicular

I Limited to testes; complete resection by high inguinal orchiectomy or trans-scrotal orchiectomy with no spill at surgery; no clinical, radiographic, or histologic evidence of disease beyond the testes; tumor markers normal after appropriate half-life decline; patients with normal or unknown tumor markers at diagnosis must have a negative ipsilateral retroperitoneal node dissection to confirm stage I disease

II Trans-scrotal orchiectomy with gross spill of tumor; microscopic disease in scrotum or high in spermatic cord (<5 cm from proximal end); retroperitoneal lymph node involvement (<2 cm); increased tumor markers after appropriate half-life decline

III Retroperitoneal lymph node involvement (>2 cm) but no visceral or extra-abdominal involvement

IV Distant metastases, including liver aPeritoneal implants that contain only mature glial elements with no malignant elements.

and when it arises in extragonadal sites such the as pineal region, anterior mediastinum, and retroperitoneum, it is termed germinoma. Germinomas comprise 10% of ovarian tumors in children and 15% of all germ cell tumors. Germinomas are the most common malignancy found in undescended testes. Ovarian dysgerminomas are sometimes associated with precocious sexual development, but the majority of patients are developmentally normal.

Ovarian Dysgerminoma

Rapid development of signs and symptoms of an abdominal mass is the usual presentation. Abdominal pain is not common unless torsion is present. Seventy-five percent of patients have stage I disease at presentation. Patterns of spread include contiguous extension and metastasis to regional lymph nodes and rarely to liver and lungs.

Surgery

Dysgerminoma is the only germ cell tumor of the ovary in which there is a high incidence of bilateral ovarian involvement (5-10%). Bilateral involvement is particularly common in women with a Y chromosome and gonadal streaks. Conservative surgery consisting of unilateral salpingo-oophorectomy and wedge biopsy of the contralateral ovary and sampling of regional lymph nodes are recommended for patients with the following criteria:

1. Stage I unilateral encapsulated tumor less than 10 cm in diameter

2. Normal contralateral ovary

3. No evidence of retroperitoneal lymph node metastases

4. No ascites; negative cytology of peritoneal washing

5. Normal female 46XX karyotype.

Patients with stage I disease require no further postsurgical therapy. In the past the standard surgical management of patients with stage II and III disease had been total abdominal hysterectomy (TAH) and bilateral salpingo-oophorectomy (BSO) followed by postoperative radiotherapy. However, because the tumor most commonly affects children and young women, a program employing limited surgery, chemotherapy, and/or radiotherapy may be more appropriate. Such an approach preserves as much endocrine and reproductive function as possible without compromising survival, compared to TAH and BSO plus radiotherapy.

Chemotherapy

Dysgerminoma and seminoma (testicular tumor analogous to dysgerminoma) are responsive to combination chemotherapy. Tables 24-6 and 24-7 list the chemotherapy regimens used for the treatment of germ cell tumors.

The primary chemotherapy approach in patients with stage II and III disease, combined with limited surgery, has been effective in children. This approach has the advantage of preserving as much reproductive and endocrine function as possible without compromising long-term survival. It is recommended for pediatric and adolescent patients with 46XX karyotype.

Radiotherapy

Dysgerminoma is the most radiosensitive of the ovarian germ cell tumors. However, radiation therapy is reserved for patients with persistent disease after chemotherapy.

Table 24-6. Various Chemotherapy Regimens for Germ Cell Tumors

<12 months of age (every 3 weeks) >12 months of age (every 3 weeks)

Regimen l (PEB) Cisplatin (P) Etoposide (E) Bleomycin (B)

l.l mg/kg/IV on days l, 2, and S 5.5 mg/kg/IV on days l, 2, and S 0.5 units/kg/IV on day l

Was this article helpful?

0 0

Post a comment