Pediatric Annals

Ovarian Tumors in Childhood

Burton Bronsther, MD; Martin W Abrams, MD

Abstract

No other disease entity presents the diagnostic and therapeutic challenge that is seen in ovarian tumors in children. The prognostic and therapeutic implications demand meticulous diagnosis, as the physical development and future childbearing abilities of these patients depend on careful conservation of ovarian tissue, when indicated, and on total eradication of malignant tissue, when present.

The importance of ovarian tumors is also realized when one understands that 1 per cent of all childhood tumors originate in the ovary. These lesions must be differentiated from such acute and subacute disorders as acute appendicitis, intestinal obstruction, and, occasionally, an abdominal mass, with or without endocrine effects (Figure 1).

1 Abell. M R., et al Ovarian neoplasms in childhood and adolescence. Tumors of non-germ cell origin. Am. J. Obstet Gynecol 93 (1965), 850.

2. Em. S. H.. et al. Cystic and solid ovarian tumors in children: A forty-four year review. J. Pediatr. Surg. 5 (1970), 148.

3. Irons, G. B.. et al. Ovarian teratoma in children. Clin. Pediatr 5 (1966), 157.

4. Thatcher, D. S Ovarian cysts and tumors m children. Surg. Gynecol. Obstet. 117 (1963). 477.

5. Groeber, W. R. Ovarian tumors during infancy and childhood. Am. J Obstet. Gynecol. 86 (1963), 1027.

6. Radman, H. M., et al. Ovarian tumors in children. Am. J. Obstet. Gynecol. 79 (1960), 989

7. Nielsen. 0. V. Ovarian tumors in children. Acta Obstet. Gynecol. Scand. 47 (1968), 119.

8. Borushek, S., et al. Functioning malignant germ cell tumor of the ovary in a 4'/2-year-old girl. Cancer 18 (1965). 1485.

9. Green. G. H. Solid malignant teratoma of the ovary. Am. J. Obstet. Gynecol. 79 (1960), 999.

10. Murray, R. C . and Hofmeister, F. J. Malignant ovarian teratoma in a thirteen-year-old girl. Obstet. Gynecol. 18 (1961), 474.

11. Steckel, R. J., Hendrix, E. L, and Bigelow, R. R. Malignant solid teratoma of the ovary in an 8-year-old child. Obstef. Gynecol. 25 (1965), 249.

12. Schweisguth, O. , et al. Bilateral non functioning thecoma of the ovary in epileptic children under anticonvulsant therapy. Acta Pediatr. Scand. 60 (1971), 6.

13. Goldstein. D. P., et al. Combination chemotherapy in the treatment of germ cell tumors containing choriocarcinoma. Surg. Gynecol. Obstet. 134 (1972), 61.

14. Dewar, M. J. The treatment ot metastatic choriocarcinoma. Aust. N. Z. J. Obstet. Gynecol. 10 (1970), 51.

15. Symposium on choriocarcinoma. Am. J. Med. 49 (1970). 395.

16. Marshall, J. R. Ovarian enlargements in the first year of life: Review of forty-five cases. Ann. Surg. 161 (1965), 372.

1 7. Eberlein. W. R., et al. Ovarian tumors and cysts associated with sexual precocity. J. Pediatr. 57 (1960), 484.

18. Novak, E., and Novak, E. 7exf of Gynecology. Baltimore: The Williams & Wilkins Company, 1956.

19. Niswander, K, R., et al. Precocious pseudopuberty caused by ovarian tumors: Report of three cases. Oosfef. Gynecol. 26 (1965), 381.

20. Abell. M. R., et al. Ovarian neoplasms in childhood: Tumors of germ cell origin. Am. J. Obstet. Gynecol. 92 (1965), 1059.

21 . Boles, E. T., Jr.. et al. Ovarian tumors and cysts in infants and children. Arch. Surg. 83 (1961), 580.

22. Faber, H. K. Meigs syndrome with thecomas of both ovaries in a four-year-old girl. J. Pediatr. 61 (1962), 769.

23. Ehrlich, R. M., et al. Arrhenoblastoma of the ovary in an adolescent. Can. Med. Assoc. J. 99 (1968), 1140.

24. Reis, R. L, et al. Ovarian tumors in infants and children. J. Pediatr. 60 (1962), 96.

25. Perry, R. W. Ovarian tumors of the pediatric patient. Harper Hosp. Bull. 9 (1961). 209.

TABLE 1

TREATMENT OF 20 PATIENTS WITH OVARIAN NEOPLASMS

TABLE 1

TREATMENT…

No other disease entity presents the diagnostic and therapeutic challenge that is seen in ovarian tumors in children. The prognostic and therapeutic implications demand meticulous diagnosis, as the physical development and future childbearing abilities of these patients depend on careful conservation of ovarian tissue, when indicated, and on total eradication of malignant tissue, when present.

The importance of ovarian tumors is also realized when one understands that 1 per cent of all childhood tumors originate in the ovary. These lesions must be differentiated from such acute and subacute disorders as acute appendicitis, intestinal obstruction, and, occasionally, an abdominal mass, with or without endocrine effects (Figure 1).

Figure 1. Five-week-old infant with left-sided mass and intestinal obstruction.

Figure 1. Five-week-old infant with left-sided mass and intestinal obstruction.

This report deals with a series of 20 consecutive patients with ovarian neoplasms seen between 1962 and 1972. The patients varied in age, pathology, and clinical symptoms. Table 1, consisting of factors relating to these 20 patients, tabulates the experience.

Table

TABLE 1TREATMENT OF 20 PATIENTS WITH OVARIAN NEOPLASMS

TABLE 1

TREATMENT OF 20 PATIENTS WITH OVARIAN NEOPLASMS

The patients ranged in age from five weeks to 16 years. Eleven of the tumors occurred on the right side, seven were on the left side, and two were bilateral. Seventeen were benign, and three were malignant (two teratomas, one dysgerminoma). Six tumors presented as an abdominal mass, seven as an acute abdomen, three as intestinal obstruction, and four as an abdominal mass associated with an acute abdomen. One child had associated precocious puberty (secondary to brain-stem involvement with toxoplasmosis), and two had calcific densities within the mass that were noted on preoperative roentgenograms. The pathology noted in this series is seen in Table 2.

Table

TABLE 1TREATMENT OF 20 PATIENTS WITH OVARIAN NEOPLASMS

TABLE 1

TREATMENT OF 20 PATIENTS WITH OVARIAN NEOPLASMS

DIFFERENTIAL DIAGNOSIS

The diagnostic problems with ovarian tumors stem primarily from the tumor's ability to present itself in any quadrant of the abdomen. This variability arises from the tumor's attachment to a rather long and narrow pedicle. Should the pedicle twist and cause infarction of the ovary or fallopian tube, a tender abdominal mass with signs of local peritonitis or abscess formation may be present. If this inflammatory mass abuts against the caecum or sigmoid colon, a clinical picture of intestinal obstruction may be added to the clinical diagnostic problem. A hemorrhagic or ruptured corpus luteum may simulate appendicitis.

Table

TABLE 2PATHOLOGY NOTED IN SERIES DESCRIBED IN TABLE 1

TABLE 2

PATHOLOGY NOTED IN SERIES DESCRIBED IN TABLE 1

The above-mentioned clinical factors caused the following disease entities to be considered in differential diagnosis;

1. Peritonitis

2. Distended bladder

3. Kidney tumor

4. Mesenteric cysts

5. Urachal cyst

6. Liver mass

7. Hydrometrocolpos

8. Appendiceal abscess

9. Intussesception

10. Ascites

TREATMENT

The surgical management of this series was determined by accurate pathologic interpretation of the tissue removed. Simple cysts were excised, saving peripheral ovarian stroma. Infarcted fallopian tubes were excised, and solid tumors, both benign and malignant, were treated by salpingooophorectomy. Dermoid cysts were shelled out, and the contralateral ovary was bisected to rule out the possibility of a second dermoid.

Table

TABLE 3WILLIS' CLASSIFICATION OF OVARIAN TUMORS IN CHILDREN

TABLE 3

WILLIS' CLASSIFICATION OF OVARIAN TUMORS IN CHILDREN

RESULTS

The surgical results in these 20 cases are noted in Table 1. Those children with benign lesions are all alive and well. The three children with malignancies (one ruptured before surgical exploration) are alive and well three, four, and nine years postoperatively. In no case was postoperative radiotherapy or chemotherapy employed.

DISCUSSION

In 1953, Willis classified ovarian tumors in children (Table 3). This classification is based upon strict morphology and enables one to more readily ascertain the proper approach to each patient. Alternative classifications have been rendered by Abell et al.1 and Ein et al.2 Their classifications are based on germ cell derivation and endocrine function. These classifications are not all encompassing and create difficulty in clinical application.

Table

TABLE 4VARIATIONS IN OVARIAN PATHOLOGY

TABLE 4

VARIATIONS IN OVARIAN PATHOLOGY

Table

TABLE 5PERCENTAGE OF MALIGNANCY

TABLE 5

PERCENTAGE OF MALIGNANCY

Brenner cell tumors, theca cell tumors, arrhenoblastomas, and serous and pseudomucinous cystadenomas (benign and malignant) are almost never seen in childhood.

The variations in the ovarian pathology reported by different authors may be seen in Table 4.

The incidence oí malignancy varies from 12.5 to 60 per cent, depending on the reporting author (Table 5).

TERATOMAS

The teratomas make up the largest group of ovarian tumors seen in childhood (Figures 2 and 3). The incidence varies in different series from 22 to 60 per cent.

Twelve per cent of all teratomas3 are bilateral, and the incidence of malignancy varies with the type of teratoma. Dermoids, comprising 80 per cent of all teratomas, have an incidence of malignancy varying from 0.8 to 3 per cent;5·8 solid teratomas, comprising 20 per cent of all teratomas, have an incidence of malignancy of 25 to 50 per cent.5"7

These tumors usually present as mass lesions, with or without pain (depending on the presence or absence of torsion), and rarely have endocrine effects. When they are malignant, the component is usually squamous,3 but it may originate from any of the three germ layers. Choriocarcinoma seen within a teratoma has been reported on various occasions,1,4*8 and may be associated with elevated chorionic gonadotropin, epithelialization of vaginal mucosa, and vaginal bleeding.

The malignant teratomas have a poor prognosis. The mortality varies in different series from 35 to 92 per cent.1·"" The children who manifested metastatic disease usually had symptoms within two years. Our three patients with teratomas are alive and well three, four, and nine years postoperatively.

In the case of benign lesions, the surgical approach is uniformly successful; all authors recommend ovarian cystectomy with salvage of surrounding stroma. The approach for a malignant lesion varies with different authors from oophorectomy1 to salpingo-oophorectomy2,3 to total hysterectomy with bilateral salpingo-oophorectomy.3,12 Surgery is indicated even in the presence of metastatic disease. Proskauer and Noguchi and Peterson12 have reported on several patients in whom peritoneal metastases disappeared after the removal of the primary tumor.

Radiotherapy has occasionally been suggested as adjuvant treatment;2,12 but, because these tumors (with the exception of dysgerminoma) are radioresistant, it is not commonly used.13 Chemotherapy, with methotrexate the most commonly used drug, is of real value. Tumors within which choriocarcinoma has become the malignant force have responded quite consistently to methotrexate.13"15 Metastatic choriocarcinoma has also been reported13,14 to disappear upon administration of methotrexate. Recently, actinomycin D and Cytoxan, when added to the therapeutic armamentarium, have been shown to improve the effects of methotrexate alone.13 The combination of surgery, chemotherapy, and, in rare instances, roentgen therapy has significantly improved the outlook for selected patients with malignant teratomas.

Figure 2. Teratoma of ovary.

Figure 2. Teratoma of ovary.

Figure 3. Infarcted solid teratoma with malignant foci rupture.

Figure 3. Infarcted solid teratoma with malignant foci rupture.

CYSTS

The cystic lesions (Figure 4) other than cystic teratomas (dermoid) comprise 20 to 55 per cent of all ovarian tumors seen in children (Table 6). They usually present as a nontender mass. However, if torsion occurs,6 the cystic lesion may hemorrhage or infarct and cause local peritoneal irritation. It also may abut against local bowel and cause symptoms of intestinal obstruction.16

The follicular cyst is the most common cystic lesion. The incidence varies in different series from 50 to 100 per cent. These are benign lesions and are occasionally associated with precocious puberty.5,17 The most common symptoms are vaginal bleeding, enlarged breasts, early development of pubic hair, and vulvar hypertrophy. The tumor is not palpable in all cases4 and should be treated by ovarian cystectomy, with the surrounding stroma being left in situ.

Children with precocious puberty secondary to follicular cysts have had subsidence of all their symptoms in the postoperative period. The child with precocious puberty in our series had persistence of symptoms because its derivation was from the central nervous system and not the ovary.

Figur· 4. Simple cyst.

Figur· 4. Simple cyst.

The corpus luteum cyst and a group of miscellaneous lesions variously reported as simple unclassified cysts or serous and pseudomucinous cystadenomas are benign and should be treated by simple excision.

Table

TABLE 6SIMPLE CYSTS

TABLE 6

SIMPLE CYSTS

NEOPLASMS OF OVARIAN STROMA

This group comprises 10 to 22 per cent1 of all ovarian tumors. One-third of these tumors are malignant.18 They present as a mass lesion with or without peritoneal signs. In the latter group, when the tumor is malignant, the survival rate is poor, regardless of the method of treatment.1

The granulosa cell tumors, benign or malignant, do cause precocious pseudopuberty. Approximately 95 cases have been reported to date.19 These tumors are not necessarily palpable on abdominal or rectal examination, and are successfully treated with simple salpingooophorectomy.20,21

Theca cell tumors have the lowest incidence of malignant presentation of all neoplasms derived from tissue of ovarian stroma.19 They are associated with precocious puberty. Scattered cases of Meig's syndrome have been reported in conjunction with this lesion.22

Arrhenoblastoma, rarely seen in children, may produce rapid virulization. Obvious evidence of body hair, significant enlargement of the clitoris, and deepening of the voice have been reported within a threemonth period. These tumors are usually not palpable. The elevated 17-ketosteroids reported with this lesion are not supressed with dexamethasone.23 This factor is the key to the diagnosis of this tumor.

Other lesions derived from the ovarian stroma - dysgerminoma (Figures 5 and 6), cystadenomas, cystadenocarcinoma, fibrosarcoma, and other nonspecific embryonal lesions - comprise only a small percentage of this group.

SUMMARY

A presentation of 20 children with ovarian tumors between the ages of five weeks and 15 years has been put forth. The variations in clinical presentation, pathology, and therapy of these tumors have been presented and discussed.

Seventeen children had benign lesions, and three had malignancies. Thirty-five per cent of the lesions were teratomas, 55 per cent cysts, and 10 per cent tumors derived from ovarian stroma. Twelve were rightsided and eight were left-sided. Six tumors presented as abdominal masses, seven as acute abdomens, three as intestinal obstructions, and four as abdominal masses associated with acute abdomens. One child had associated precocious puberty (secondary to brain-stem involvement with toxoplasmosis), and two had calcic densities within the mass that were noted on preoperative roentgenograms.

A review of the American literature and classification of ovarian tumors in childhood are included.

Note: The three children with malignant tumors are alive and well three, four, and nine years postoperatively.

Figure 5. Twelve-year-old with lower abdominal mass (malignant dysgerminoma).

Figure 5. Twelve-year-old with lower abdominal mass (malignant dysgerminoma).

Figur· ß. Malignant dysgerminoma in the left ovary.

Figur· ß. Malignant dysgerminoma in the left ovary.

BIBUOGRAPHY

1 Abell. M R., et al Ovarian neoplasms in childhood and adolescence. Tumors of non-germ cell origin. Am. J. Obstet Gynecol 93 (1965), 850.

2. Em. S. H.. et al. Cystic and solid ovarian tumors in children: A forty-four year review. J. Pediatr. Surg. 5 (1970), 148.

3. Irons, G. B.. et al. Ovarian teratoma in children. Clin. Pediatr 5 (1966), 157.

4. Thatcher, D. S Ovarian cysts and tumors m children. Surg. Gynecol. Obstet. 117 (1963). 477.

5. Groeber, W. R. Ovarian tumors during infancy and childhood. Am. J Obstet. Gynecol. 86 (1963), 1027.

6. Radman, H. M., et al. Ovarian tumors in children. Am. J. Obstet. Gynecol. 79 (1960), 989

7. Nielsen. 0. V. Ovarian tumors in children. Acta Obstet. Gynecol. Scand. 47 (1968), 119.

8. Borushek, S., et al. Functioning malignant germ cell tumor of the ovary in a 4'/2-year-old girl. Cancer 18 (1965). 1485.

9. Green. G. H. Solid malignant teratoma of the ovary. Am. J. Obstet. Gynecol. 79 (1960), 999.

10. Murray, R. C . and Hofmeister, F. J. Malignant ovarian teratoma in a thirteen-year-old girl. Obstet. Gynecol. 18 (1961), 474.

11. Steckel, R. J., Hendrix, E. L, and Bigelow, R. R. Malignant solid teratoma of the ovary in an 8-year-old child. Obstef. Gynecol. 25 (1965), 249.

12. Schweisguth, O. , et al. Bilateral non functioning thecoma of the ovary in epileptic children under anticonvulsant therapy. Acta Pediatr. Scand. 60 (1971), 6.

13. Goldstein. D. P., et al. Combination chemotherapy in the treatment of germ cell tumors containing choriocarcinoma. Surg. Gynecol. Obstet. 134 (1972), 61.

14. Dewar, M. J. The treatment ot metastatic choriocarcinoma. Aust. N. Z. J. Obstet. Gynecol. 10 (1970), 51.

15. Symposium on choriocarcinoma. Am. J. Med. 49 (1970). 395.

16. Marshall, J. R. Ovarian enlargements in the first year of life: Review of forty-five cases. Ann. Surg. 161 (1965), 372.

1 7. Eberlein. W. R., et al. Ovarian tumors and cysts associated with sexual precocity. J. Pediatr. 57 (1960), 484.

18. Novak, E., and Novak, E. 7exf of Gynecology. Baltimore: The Williams & Wilkins Company, 1956.

19. Niswander, K, R., et al. Precocious pseudopuberty caused by ovarian tumors: Report of three cases. Oosfef. Gynecol. 26 (1965), 381.

20. Abell. M. R., et al. Ovarian neoplasms in childhood: Tumors of germ cell origin. Am. J. Obstet. Gynecol. 92 (1965), 1059.

21 . Boles, E. T., Jr.. et al. Ovarian tumors and cysts in infants and children. Arch. Surg. 83 (1961), 580.

22. Faber, H. K. Meigs syndrome with thecomas of both ovaries in a four-year-old girl. J. Pediatr. 61 (1962), 769.

23. Ehrlich, R. M., et al. Arrhenoblastoma of the ovary in an adolescent. Can. Med. Assoc. J. 99 (1968), 1140.

24. Reis, R. L, et al. Ovarian tumors in infants and children. J. Pediatr. 60 (1962), 96.

25. Perry, R. W. Ovarian tumors of the pediatric patient. Harper Hosp. Bull. 9 (1961). 209.

TABLE 1

TREATMENT OF 20 PATIENTS WITH OVARIAN NEOPLASMS

TABLE 1

TREATMENT OF 20 PATIENTS WITH OVARIAN NEOPLASMS

TABLE 2

PATHOLOGY NOTED IN SERIES DESCRIBED IN TABLE 1

TABLE 3

WILLIS' CLASSIFICATION OF OVARIAN TUMORS IN CHILDREN

TABLE 4

VARIATIONS IN OVARIAN PATHOLOGY

TABLE 5

PERCENTAGE OF MALIGNANCY

TABLE 6

SIMPLE CYSTS

10.3928/0090-4481-19751001-05

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