Bilateral By Charles
Ovarian Fibromas in Children
G. Howell, Jr, David A. Rogers, Donna S. Gable, and Greer D. Falls A ugus ta, Georgia
0 The case of bilateral ovarian fibromas occurring in an &year-old black girl is reported. These lesions occur rarely in premenarchal females and may be a manifestation of Nevoid Basal Cell Syndrome. Calcifications are reported to occur rarely in ovarian fibromas but seem to occur frequently in fibromas in children. Management is guided by the benignity of the lesion and consists of surgical excision of the fibroma. Preservation of normal ovarian tissue is recommended with the acknowledged risk of recurrence of the fibroma. 0 1990 by W.B. Saunders Company. INDEX WORDS:
Ovarian fibroma.
IBROMAS of the ovary are uncommon in children and adolescents, accounting for 3% of ovarian neoplasms in this group.’ Fibromas, as a group of tumors, are reported to be unilateral in 90% of cases.’
F
CASE REPORT N.O. is an S-year-old black girl who presented with complaints of abdominal pain, nausea, vomiting, and suprapubic pain with urination. It was reported that these symptoms had occurred approximately 2 weeks prior to this presentation, had resolved spontaneously, and had then recurred. The past medical history and family history were unremarkable. Physical examination was remarkable for mild diffuse abdominal pain, No evidence of secondary sexual characteristic development was noted. A film of the abdomen was obtained (Fig 1) demonstrating multiple spherical calcifications. An ultrasound scan showed a 6 x 5 cm left adnexal mass with a well-defined rim of calcification. At laparotomy a large lesion of the right ovary (Fig 2) was noted. The ovary was torqued several times and was displaced into the left side of the pelvis. The left ovary was remarkable for multiple small nodules. A right salpingo-oophorectomy was performed. The multiple nodules of the left ovary were removed, leaving a rim of normal ovary. On pathological examination, the right adnexal tissue consisted of a firm multinodular maas measuring 9 x 4 x 4 cm. On bisection, the mass consisted of multiple solid fibrous nodules ranging from 5 mm to 3 cm in diameter. There was central hemorrhagic infarction of the larger tumors and a peripheral rim of calcification. Several of the centrally located nodules were completely calcified. The excisional biopsy from the left ovary consisted of a multinodular mass that was composed of fibrous nodules similar to those of the right ovary. Microscopically, the composition of the tumors varied from interlacing bundles of undifferentiated ovarian stroma to slender enlongated
Fig 1. Abdominal scan demonstrating multiple spherical cslcificstions.
spindle cells with a large amount of collagen and focal areas of hyalinization. No mitoses were noted. Residual primordial follicles were present in nonfibromatous areas of both specimens, although they were sparse on the right. Oil red 0 stains were negative for fat. The patient had an uneventful postoperative course. Follow-up ultrasounds have been normal. DISCUSSION
From the Section of Pediatric Surgery, Department of Surgery and Pathology. Medical College of Georgia, Augusta, GA. Address reprint requests to Charles G. Howell, Jr, MD. Section of Pediatric Surgery, Medical College of Georgia. 1459 Laney Walker Blvd. Augusta, GA 30912. o 1990 by W.B. Saunders Company. 0022-3468/90/2506-0028$03.00/O
Ovarian fibromas are common in adults, accounting for 5% of ovarian neoplasms.3 Ovarian neoplasms occur less commonly in children and adolescents, and fibromas account for only 3% of these lesions.’ A review of the literature showed only five previously reported bilateral ovarian fibromas in premenarchal females.48 Two of the cases occurred in children who were subsequently discovered to have Nevoid Basal Cell Carcinoma (Gorlin’s Syndrome).7V8 In both of these patients, the fibromas were the first stigmata of this syndrome to be discovered. Patients with ovarian fibromas usually present with symptoms related to an abdominal mass.’ Generally, these symptoms are chronic and related to the gastrointestinal or genitourinary systems. Rarely, a patient may present with acute symptoms secondary to torsion of the fibroma, as occurred in the case reported here and in others.7*8T’0We used ultrasound in our evaluation of this patient. Ultrasound findings of ovarian fibroma are reported to be distinctive in that they appear as a hypoechoic solid mass.” Whereas other lesions may present a similar picture, the majority of ovarian lesions appear cystic on ultrasound. Calcification of ovarian fibromas has been reported to occur rarely.7*‘2 However, in the majority of cases
690
Journal of Pediatric Surgery, Vol 25. No 6 (June), 1990: pp 690-69 1
691
Fig 2. adnexa.
lntreoperative findings after detorsion of the right
reported in premenarchal females, the fibromas demonstrate either radiographic or pathological evidence of calcification.5~7~*3 In the reports reviewed, the fibromas were managed with excision of only the abnormal tissue with one exception.’ The only recurrences occurred in the two patients with Gorlin’s syndrome. In the case reported here, infarction secondary to torsion required complete excision of the right ovary and salpinx. The fibroma was excised on the left leaving a portion of normal ovarian tissue intact. We recommend that management of this lesion should be excision of the fibromas only, leaving all normal appearing ovarian tissue. The risk of recurrence then requires periodic follow-up.
REFERENCES 1. Breen JL, Maxson WS: Ovarian tumors in children and adolescents. Clin Obstet Gynecol20:607-623, 1977 2. Dockerty MB, Masson JC: Ovarian fibromas: A clinical and pathological study of two hundred and eighty three cases. Am J Obstet GynecoI47:741-752, 1944 3. Griffiths CT, Parker L: Cancer of the ovary, in Knapp RC, Berkowitz TS (eds): Gynecologic Oncology. New York, NY, Macmillan, 1986, pp 3 17-376 4. Martins SM, Klinger OJ: Bilateral ovarian fibromas occurring before the menarche. Am J Obstet Gynecol89:386-391,1964 5. Bower JE, Erickson ER: Bilateral ovarian fibromas in a 5 year old. Am J Obstet Gynecol99:880-882, 1967 6. Tytle T, Rosin D: Bilateral calcified ovarian fibromas. South Med J77:1178-1180, 1984 7. Raggio M, Kaplan AL, Ha&erg JF: Recurrent ovarian fibromas. Obstet Gynecol61:95S96S, 1983 (suppl)
8. Johnson AD, Hebert AA, Esterly NB: Nevoid basal ceil carcinoma syndrome: Bilateral ovarian tibromas in a 3% year old girl. J Am Acad Dermatol 14:371-374, 1986 9. Huffman JW, Dewhurst CJ, Capraro VJ: The Gynecology of Childhood and Adolescence (ed 2). Philadelphia, PA, Saunders, 1981, pp 277-349 10. Charache H: Ovarian tumors in childhood. Arch Surg 79:573580,1959 11. Stephenson WM, Laing FC: Sonography of ovarian fibromas. AJR 144:1239-1240.1985 12. Sotto LS, Postoloff AV, Carr F: A case of calcified ovarian fibroma with ossification. Am J Obstet Gynecol 71:1355-1358, 1956 13. Kraemer BB, Silva EG, Sneige N: Fibrosarcoma of ovary. A new component in the nevoid basal-cell carcinoma syndrome. Am J Surg Path01 8:231-236, 1984
Ovarian Fibromas in Children
G. Howell, Jr, David A. Rogers, Donna S. Gable, and Greer D. Falls A ugus ta, Georgia
0 The case of bilateral ovarian fibromas occurring in an &year-old black girl is reported. These lesions occur rarely in premenarchal females and may be a manifestation of Nevoid Basal Cell Syndrome. Calcifications are reported to occur rarely in ovarian fibromas but seem to occur frequently in fibromas in children. Management is guided by the benignity of the lesion and consists of surgical excision of the fibroma. Preservation of normal ovarian tissue is recommended with the acknowledged risk of recurrence of the fibroma. 0 1990 by W.B. Saunders Company. INDEX WORDS:
Ovarian fibroma.
IBROMAS of the ovary are uncommon in children and adolescents, accounting for 3% of ovarian neoplasms in this group.’ Fibromas, as a group of tumors, are reported to be unilateral in 90% of cases.’
F
CASE REPORT N.O. is an S-year-old black girl who presented with complaints of abdominal pain, nausea, vomiting, and suprapubic pain with urination. It was reported that these symptoms had occurred approximately 2 weeks prior to this presentation, had resolved spontaneously, and had then recurred. The past medical history and family history were unremarkable. Physical examination was remarkable for mild diffuse abdominal pain, No evidence of secondary sexual characteristic development was noted. A film of the abdomen was obtained (Fig 1) demonstrating multiple spherical calcifications. An ultrasound scan showed a 6 x 5 cm left adnexal mass with a well-defined rim of calcification. At laparotomy a large lesion of the right ovary (Fig 2) was noted. The ovary was torqued several times and was displaced into the left side of the pelvis. The left ovary was remarkable for multiple small nodules. A right salpingo-oophorectomy was performed. The multiple nodules of the left ovary were removed, leaving a rim of normal ovary. On pathological examination, the right adnexal tissue consisted of a firm multinodular maas measuring 9 x 4 x 4 cm. On bisection, the mass consisted of multiple solid fibrous nodules ranging from 5 mm to 3 cm in diameter. There was central hemorrhagic infarction of the larger tumors and a peripheral rim of calcification. Several of the centrally located nodules were completely calcified. The excisional biopsy from the left ovary consisted of a multinodular mass that was composed of fibrous nodules similar to those of the right ovary. Microscopically, the composition of the tumors varied from interlacing bundles of undifferentiated ovarian stroma to slender enlongated
Fig 1. Abdominal scan demonstrating multiple spherical cslcificstions.
spindle cells with a large amount of collagen and focal areas of hyalinization. No mitoses were noted. Residual primordial follicles were present in nonfibromatous areas of both specimens, although they were sparse on the right. Oil red 0 stains were negative for fat. The patient had an uneventful postoperative course. Follow-up ultrasounds have been normal. DISCUSSION
From the Section of Pediatric Surgery, Department of Surgery and Pathology. Medical College of Georgia, Augusta, GA. Address reprint requests to Charles G. Howell, Jr, MD. Section of Pediatric Surgery, Medical College of Georgia. 1459 Laney Walker Blvd. Augusta, GA 30912. o 1990 by W.B. Saunders Company. 0022-3468/90/2506-0028$03.00/O
Ovarian fibromas are common in adults, accounting for 5% of ovarian neoplasms.3 Ovarian neoplasms occur less commonly in children and adolescents, and fibromas account for only 3% of these lesions.’ A review of the literature showed only five previously reported bilateral ovarian fibromas in premenarchal females.48 Two of the cases occurred in children who were subsequently discovered to have Nevoid Basal Cell Carcinoma (Gorlin’s Syndrome).7V8 In both of these patients, the fibromas were the first stigmata of this syndrome to be discovered. Patients with ovarian fibromas usually present with symptoms related to an abdominal mass.’ Generally, these symptoms are chronic and related to the gastrointestinal or genitourinary systems. Rarely, a patient may present with acute symptoms secondary to torsion of the fibroma, as occurred in the case reported here and in others.7*8T’0We used ultrasound in our evaluation of this patient. Ultrasound findings of ovarian fibroma are reported to be distinctive in that they appear as a hypoechoic solid mass.” Whereas other lesions may present a similar picture, the majority of ovarian lesions appear cystic on ultrasound. Calcification of ovarian fibromas has been reported to occur rarely.7*‘2 However, in the majority of cases
690
Journal of Pediatric Surgery, Vol 25. No 6 (June), 1990: pp 690-69 1
691
Fig 2. adnexa.
lntreoperative findings after detorsion of the right
reported in premenarchal females, the fibromas demonstrate either radiographic or pathological evidence of calcification.5~7~*3 In the reports reviewed, the fibromas were managed with excision of only the abnormal tissue with one exception.’ The only recurrences occurred in the two patients with Gorlin’s syndrome. In the case reported here, infarction secondary to torsion required complete excision of the right ovary and salpinx. The fibroma was excised on the left leaving a portion of normal ovarian tissue intact. We recommend that management of this lesion should be excision of the fibromas only, leaving all normal appearing ovarian tissue. The risk of recurrence then requires periodic follow-up.
REFERENCES 1. Breen JL, Maxson WS: Ovarian tumors in children and adolescents. Clin Obstet Gynecol20:607-623, 1977 2. Dockerty MB, Masson JC: Ovarian fibromas: A clinical and pathological study of two hundred and eighty three cases. Am J Obstet GynecoI47:741-752, 1944 3. Griffiths CT, Parker L: Cancer of the ovary, in Knapp RC, Berkowitz TS (eds): Gynecologic Oncology. New York, NY, Macmillan, 1986, pp 3 17-376 4. Martins SM, Klinger OJ: Bilateral ovarian fibromas occurring before the menarche. Am J Obstet Gynecol89:386-391,1964 5. Bower JE, Erickson ER: Bilateral ovarian fibromas in a 5 year old. Am J Obstet Gynecol99:880-882, 1967 6. Tytle T, Rosin D: Bilateral calcified ovarian fibromas. South Med J77:1178-1180, 1984 7. Raggio M, Kaplan AL, Ha&erg JF: Recurrent ovarian fibromas. Obstet Gynecol61:95S96S, 1983 (suppl)
8. Johnson AD, Hebert AA, Esterly NB: Nevoid basal ceil carcinoma syndrome: Bilateral ovarian tibromas in a 3% year old girl. J Am Acad Dermatol 14:371-374, 1986 9. Huffman JW, Dewhurst CJ, Capraro VJ: The Gynecology of Childhood and Adolescence (ed 2). Philadelphia, PA, Saunders, 1981, pp 277-349 10. Charache H: Ovarian tumors in childhood. Arch Surg 79:573580,1959 11. Stephenson WM, Laing FC: Sonography of ovarian fibromas. AJR 144:1239-1240.1985 12. Sotto LS, Postoloff AV, Carr F: A case of calcified ovarian fibroma with ossification. Am J Obstet Gynecol 71:1355-1358, 1956 13. Kraemer BB, Silva EG, Sneige N: Fibrosarcoma of ovary. A new component in the nevoid basal-cell carcinoma syndrome. Am J Surg Path01 8:231-236, 1984
