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Cervical Sarcoma Botryoides and Ovarian Sertoli–Leydig Cell Tumor
GYNECOLOGIC ONCOLOGY ARTICLE NO.
67, 102–106 (1997)
GO974807
CASE REPORT Cervical Sarcoma Botryoides and Ovarian Sertoli–Leydig Cell Tumor Pouran Golbang, M.D.,* Afaq Khan, MBBS,† James Scurry, MBBS,†,1 Ian MacIsaac, MBBS,‡ and Robert Planner, MBBS* *Department of Gynecological Oncology, †Department of Pathology and ‡MacIsaac Gynecology and Obstetric Unit, Mercy Hospital for Women, Melbourne 3002, Victoria, Australia Received December 23, 1996
The case of a woman who developed a cervical sarcoma botryoides tumor at age 14 years and a right ovarian Sertoli–Leydig cell tumor with a-fetoprotein production at 27 years is presented. The sarcoma botryoides was a stage 1b, 4-cm, polypoid ectocervical tumor treated by radical hysterectomy and bilateral pelvic lymphadenectomy. The Sertoli–Leydig cell tumor was a stage 1a, 145-g mass removed piecemeal by right oophorectomy. Histologically, it was an intermediate Sertoli–Leydig cell tumor with a heterologous element composed of an endometrioid-like yolk sac tumor which was producing a-fetoprotein. There was no histological similarity between the two tumors. The patient is alive without evidence of disease, 16 years after diagnosis of her sarcoma botryoides and 3 years after her Sertoli–Leydig cell tumor. This is, to our knowledge, the third known association between these two rare gynecological tumors. The basis of the association remains unknown. q 1997 Academic Press
INTRODUCTION
The diagnosis of two or more rare conditions in the same patient is noteworthy because it raises the possibility of a common cause. The association between cervical adenoma malignum and ovarian sex-cord stromal tumor with annular tubules in the Peutz–Jeghers syndrome is an example of a syndrome linking rare tumors of the cervix and ovary [1]. A less well known example is the association between cervical sarcoma botryoides and ovarian Sertoli–Leydig cell tumor, which, to our knowledge, has only been briefly mentioned twice in the literature [2, 3]. Young and Scully in their review of the pathological features of 207 cases of ovarian Sertoli–Leydig cell tumors recorded that 2 patients had a history of cervical sarcoma botryoides [2]. Three years later, 1 To whom correspondence and reprint requests should be addressed at Department of Pathology, Mercy Hospital for Women, Clarendon Street, East Melbourne, 3002, Victoria, Australia. Fax: (03) 9416 1931.
Gyn 4807
/
6d23$$$241
CASE REPORT
In 1979, a 14-year-old schoolgirl presented with abnormal vaginal bleeding since menarche 1 year previously and a lump protruding from the vagina for 7 months. There was no past or family history of gynecological or endocrinological conditions. Physical examination showed a girl with normally developed secondary sex characteristics who had a polypoid fragile mass filling the vagina. After ultrasound, examination under anesthesia and cystoscopy and rectosigmoidoscopy did not show extracervical spread and a biopsy diagnosis of sarcoma botryoides; she was treated with a radical hysterectomy and bilateral pelvic lymphadenectomy. At operation, the ovaries looked normal and were preserved. At 27 years, she represented with pain in right iliac fossa and hirsutism for 3 years. Physical examination showed local tenderness of lower abdomen. Investigation showed a mild elevation of the androgen, dehydroepiandrosterone. A ultrasound scan of the pelvis showed the whole lower abdomen was occupied by a multiseptate mass measuring 12 1 11 1 9.7 cm, with the largest solid area 8 cm in diameter. The ovaries could not be separately identified. At laparotomy, a right ovarian tumor was confirmed, but due to extensive adhesions in the pelvis, it could only be removed piecemeal. Preoperative levels of a-fetoprotein (AFP), human chorionic gonadotrophin, and CA125 were measured on stored serum. AFP was elevated at 213 mg/liter (õ10); the other markers were within normal limits.
102
0090-8258/97 $25.00 Copyright q 1997 by Academic Press All rights of reproduction in any form reserved.
AID
Daya and Scully’s review of 13 cases of cervical sarcoma botryoides refers to apparently the same 2 patients [3]. We report the case of a patient with a cervical sarcoma botryoides diagnosed when she was 14 years old who then developed an ovarian Sertoli-Leydig tumor with heterologous elements at 27 years of age. The basis of the association remains unknown.
09-16-97 06:42:07
goas
AP: GYN
103
CASE REPORT
FIG. 1. Cervical sarcoma botryoides. Cervical stroma is expanded by a low-cellularity tumor with some condensation beneath the mucosa and around blood vessels (hematoxylin and eosin, 140).
Pathology Cervical tumor. The radical hysterectomy specimen showed a 4-cm broad papillary mass on a stalk 2 cm long and 0.6 cm in diameter arising from the ectocervix of the anterior lip, beside the external os. The mass was composed of firm to soft gray tissue with areas of edema and hemorrhage. Tumor was confined to the polypoid mass and did not involve the stalk or remainder of the cervix, corpus, vaginal cuff, or 16 bilateral pelvic lymph nodes. Microscopically, the tumor consisted of a loose population of atypical spindle cells set in an edematous matrix with numerous small blood vessels. The cells formed condensations beneath the ecto- and endocervical epithelium and around endocervical glands and occasional blood vessels (Fig. 1). The tumor cells were generally small with irregular hyperchromatic nuclei and little cytoplasm (Fig. 2), but areas of strap-like cells with eosinophilic cytoplasm, amorphous eosinophilic globules, and large pleomorphic hyperchromatic nuclei were seen, although no cross-striations were identified in any cells. Many of these large cells had degenerate karyorrhectic nuclei. Mitoses were frequent and often atypical, particularly when occurring in the pleomorphic cells. Occasional foci of immature cartilage were identified (Fig. 3). Immunoperoxidase showed patchy cytoplasmic reactivity for desmin- and muscle-specific actin in the noncartilagenous portions of the tumor. A final diagnosis of stage 1b cervical sarcoma botryoides (embryonal rhabdomyosarcoma) with heterologous cartilagenous differentiation was made. Ovarian tumor. The right oophorectomy specimen weighed 145 g and consisted of 7 fragments of solid and cystic tumor ranging in size from 12 1 5 1 2 to 3 1 2 1 0.5 cm. Cut section showed cysts up to several centimeters in diameter separated by spongy, pale solid tissue in which
AID
Gyn 4807
/
6d23$$$242
09-16-97 06:42:07
FIG. 2. Cervical sarcoma botryoides. Mildly atypical spindle cells in a myxoid background (hematoxylin and eosin, 1400).
FIG. 3. Cervical sarcoma botryoides. Island of immature cartilage in a vascular stroma (hematoxylin and eosin, 1100).
goas
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GOLBANG ET AL.
producing endometrioid-like cysts and tubules were interpreted as a heterologous component of yolk sac tumor, endometrioid-like variant. The adjacent ovarian tissue contained normal primordial and developed follicles, follicular cysts, corpora albicantia, and normal cellular stroma. A diagnosis of stage 1a right ovarian Sertoli–Leydig cell tumor of intermediate differentiation with a heterologous component of yolk sac tumor, endometrioid-like variant, with AFP production was made. Follow-up Serum AFP fell to normal levels by 20 days after oophorectomy for Sertoli–Leydig cell tumor. The patient is alive with no evidence of disease 36 months after diagnosis of her Sertoli–Leydig cell tumor and 16 years after diagnosis of sarcoma botryoides. The left ovary is still functioning because serial ultrasound scans have shown normal follicles
FIG. 4. Ovarian Sertoli–Leydig cell tumor. Cysts and tubules with areas of stromal hypercellularity (hematoxylin and eosin, 140).
numerous small locules 0.1 to 1 cm in diameter were present. This solid tissue varied from tan to yellow. Microscopically, the tumor was composed of cysts and tubules and a variably cellular stroma (Fig. 4). Around the cysts and tubules and also lying away from them were clusters of cells with polygonal nuclei and little cytoplasm, resembling mesenchyme, but occasionally containing sex cord elements as well as clumps of Leydig cells with central round nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm (Fig. 5). No Reinke crystals were seen. The cysts and tubules were lined by columnar cells with basal nuclei and abundant eosinophilic cytoplasm, resembling cells of well-differentiated endometrioid carcinoma. Occasional mitoses were noted in all cell types. There was no necrosis. Immunoperoxidase staining for AFP showed cytoplasmic positivity in the endometrioid-like cysts and tubules only. The mesenchyme, sex-cord structures, and Leydig cells were interpreted as a sex-cord stromal tumor of intermediate differentiation, while the AFP-
AID
Gyn 4807
/
6d23$$$242
09-16-97 06:42:07
FIG. 5. Ovarian Sertoli–Leydig cell tumor with endometrioid-like tubules, mesenchyme, and Leydig cells (hematoxylin and eosin, 1400).
goas
AP: GYN
105
CASE REPORT
TABLE 1 Reported Associations between Cervical Sarcoma Botryoides and Ovarian Sertoli–Leydig Tumor Cervical sarcoma botryoides
Sertoli–Leydig cell tumor
Case
Age
Clinical
Pathology
Age
Daya, case 6
23
Vaginal bleeding; polyp, 4 cm; TAH / pelvic nodes
SB
24 or 26 a
Daya, case 9
15
Vaginal bleeding; polyp, 3 cm; polypectomy / chemo
SB with heterologous cartilage
Present case
14
Vaginal bleeding; polyp, 4 cm; radical hysterectomy / pelvic nodes
SB with heterologous cartilage
Clinical
Pathology
Follow-up
NK a
SLCT intermediate differentiation
4 years, SB, A&W; ?SLCT a
16 or 18 a
NK a
SLCT intermediate differentiation
1 year, SB, A&W; ?SLCT a
27
Abdominal pain, hirsutism; 12-cm solid and cystic right ovarian mass; unilateral oophorectomy
SLCT intermediate differentiation with heterologous elements
16 years, SB; 3 years, SLCT; A&W
Note. Abbreviations used: SB, sarcoma botryoides; SLCT, Sertoli–Leydig cell tumor; A&W, alive and well; NK, not known; chemo, chemotherapy. a Young and Scully reported of 207 patients with ovarian Sertoli–Leydig cell tumor who had a past history of cervical sarcoma botryoides 1 and 3 years previously, but do not give information on individual cases.
and serum levels of follicle-stimulating hormone and inhibin are in normal limits for a premenopausal woman. DISCUSSION
The diagnosis of cervical sarcoma botryoides was made on the clinical findings of a soft polypoid tumor in the cervix in a 14-year-old girl with the histological features of atypical spindle cells in a myxoid matrix, cambium layer, cells with eosinophilic cytoplasm, and islands of immature cartilage, supported by positive desmin- and muscle-specific actin on immunoperoxidase. Cross-striations were not found, but have been reported to be seen in only 15% of cases [4]. Foci of immature cartilage have been noted in 45% of cases [3]. Cervical sarcoma botryoides must be distinguished from its more common vaginal counterpart because of its occurrence in an older age group of 12–26, mean 18 years, compared to less than 2 years, and better prognosis. In a recent review 80% of patients were alive with no evidence of disease after a mean follow-up period of 68 months [3]. The diagnosis of ovarian Sertoli–Leydig cell tumor (SLCT) was made based on the presence of a cystic and solid ovarian unilateral tumor in a 27-year-old woman composed of nodular cellular aggregates resembling immature mesenchyme, occasional sex-cord structures, and Leydig cells. There was a heterologous endodermal element which did not resemble the usual mature mucinous cystic tumor, but resembled the endometrioid-like variant of yolk sac tumor of endoderm, an interpretation supported by the finding of AFP production by the tumor
AID
Gyn 4807
/
6d23$$$242
09-16-97 06:42:07
[5]. If the endometrioid-like AFP-producing cysts and tubules were the only component of the tumor, a diagnosis of pure yolk sac tumor, endometrioid-like variant would have been the diagnosis. While AFP is a universal marker for yolk sac tumor, it is also occasionally seen in SLCT, but the value is usually much lower [2]. Interestingly, although not present in this case, immature cartilage and skeletal muscle are seen in 5% of SLCT and provide a potential link between cervical sarcoma botryoides and ovarian SLCT because these elements may be seen in both [6]. SLCT has a good prognosis, with recurrence of intermediate differentiated SLCT occurring in 11% [6]. The clinicopathological findings of the three women with the same association are presented in Table 1. The causes of cervical SB and ovarian SLCT and the reason for their association are not known. We have no hypothesis to link the two conditions together and there is no evidence presented in our or the previously reported cases to indicate what the basis of the association might be. A genetic abnormality, in view of the knowledge that sarcomas in the young may show non-random genetic changes and that Sertoli–Leydig cell tumors are rarely familial, is a possibility. Familial occurrence of SLCT has been documented in six cases and a personal and/or family history of thyroid abnormalities including multinodular goiter and adenoma have also been reported in association with SLCT [7]. While there has been no evidence of an inherited cause of cervical sarcoma botryoides, there has been one case of vaginal sarcoma botryoides reported in a child with multiple congenital abnormalities and bilateral nephroblastomas, suggesting a possible genetic
goas
AP: GYN
106
GOLBANG ET AL.
defect [8]. Unfortunately, cytogenetics were not performed on either tumor or the patient’s blood in our case. ACKNOWLEDGMENTS We are grateful to R. H. Young, M.D., and R. E. Scully, M.D., Department of Pathology, Harvard Medical School, Massachusetts General Hospital, Boston, who reviewed the slides of the ovarian tumor and made the diagnosis of Sertoli–Leydig cell tumor with a heterologous component of yolk sac tumor, endometrioid-like variant.
REFERENCES 1. Young RH, Scully RE: Sex-Cord Stromal, Steroid Cell and Other Ovarian Tumors with Endocrine, Paraendocrine and Paraneoplastic Manifestations, in Kurman RJ (ed): Blaustein’s Pathology of the Female Genital Tract, New York, Springer Verlag, 1994, 4th ed, pp 783–847
AID
Gyn 4807
/
6d23$$$242
09-16-97 06:42:07
2. Young RH, Scully RE: Ovarian Sertoli–Leydig cell tumors: a clinicopathological analysis of 207 cases. Am J Pathol 9:543–569, 1985 3. Daya DA, Scully RE: Sarcoma botryoides of the uterine cervix in young women: the clinicopathological study of 13 cases. Gynecol Oncol 29:290–304, 1988 4. Morales AR, Fine G, Horn RC Jr: Rhabdomyosarcoma: an ultrastructural appraisal. Pathol Annu 7:81–106, 1972 5. Clement PB, Young RH, Scully RE: Endometrioid-like variant of ovarian yolk sac tumor. A clinicopathological analysis of eight cases. Am J Surg Pathol 11:767–778, 1987 6. Zaloudek C, Norris HJ: A clinicopathologic study of 64 intermediate and poorly differentiated Sertoli–Leydig cell tumors of the ovary. Am J Surg Pathol 405–418, 1984 7. Fletcher JA, Gias Z, Donovan K, et al: Ovarian granulosa stromal cell tumors are characterized by trisomy 12: Am J Pathol 138: 515–520, 1991 8. Schmidt WA: Pathology of the Vagina, in Fox H, Wells M (eds): Haines and Taylor Obstetrical and Gynaecological Pathology, New York, Churchill Livingstone, 1995, 4th ed, pp 135–258
goas
AP: GYN
67, 102–106 (1997)
GO974807
CASE REPORT Cervical Sarcoma Botryoides and Ovarian Sertoli–Leydig Cell Tumor Pouran Golbang, M.D.,* Afaq Khan, MBBS,† James Scurry, MBBS,†,1 Ian MacIsaac, MBBS,‡ and Robert Planner, MBBS* *Department of Gynecological Oncology, †Department of Pathology and ‡MacIsaac Gynecology and Obstetric Unit, Mercy Hospital for Women, Melbourne 3002, Victoria, Australia Received December 23, 1996
The case of a woman who developed a cervical sarcoma botryoides tumor at age 14 years and a right ovarian Sertoli–Leydig cell tumor with a-fetoprotein production at 27 years is presented. The sarcoma botryoides was a stage 1b, 4-cm, polypoid ectocervical tumor treated by radical hysterectomy and bilateral pelvic lymphadenectomy. The Sertoli–Leydig cell tumor was a stage 1a, 145-g mass removed piecemeal by right oophorectomy. Histologically, it was an intermediate Sertoli–Leydig cell tumor with a heterologous element composed of an endometrioid-like yolk sac tumor which was producing a-fetoprotein. There was no histological similarity between the two tumors. The patient is alive without evidence of disease, 16 years after diagnosis of her sarcoma botryoides and 3 years after her Sertoli–Leydig cell tumor. This is, to our knowledge, the third known association between these two rare gynecological tumors. The basis of the association remains unknown. q 1997 Academic Press
INTRODUCTION
The diagnosis of two or more rare conditions in the same patient is noteworthy because it raises the possibility of a common cause. The association between cervical adenoma malignum and ovarian sex-cord stromal tumor with annular tubules in the Peutz–Jeghers syndrome is an example of a syndrome linking rare tumors of the cervix and ovary [1]. A less well known example is the association between cervical sarcoma botryoides and ovarian Sertoli–Leydig cell tumor, which, to our knowledge, has only been briefly mentioned twice in the literature [2, 3]. Young and Scully in their review of the pathological features of 207 cases of ovarian Sertoli–Leydig cell tumors recorded that 2 patients had a history of cervical sarcoma botryoides [2]. Three years later, 1 To whom correspondence and reprint requests should be addressed at Department of Pathology, Mercy Hospital for Women, Clarendon Street, East Melbourne, 3002, Victoria, Australia. Fax: (03) 9416 1931.
Gyn 4807
/
6d23$$$241
CASE REPORT
In 1979, a 14-year-old schoolgirl presented with abnormal vaginal bleeding since menarche 1 year previously and a lump protruding from the vagina for 7 months. There was no past or family history of gynecological or endocrinological conditions. Physical examination showed a girl with normally developed secondary sex characteristics who had a polypoid fragile mass filling the vagina. After ultrasound, examination under anesthesia and cystoscopy and rectosigmoidoscopy did not show extracervical spread and a biopsy diagnosis of sarcoma botryoides; she was treated with a radical hysterectomy and bilateral pelvic lymphadenectomy. At operation, the ovaries looked normal and were preserved. At 27 years, she represented with pain in right iliac fossa and hirsutism for 3 years. Physical examination showed local tenderness of lower abdomen. Investigation showed a mild elevation of the androgen, dehydroepiandrosterone. A ultrasound scan of the pelvis showed the whole lower abdomen was occupied by a multiseptate mass measuring 12 1 11 1 9.7 cm, with the largest solid area 8 cm in diameter. The ovaries could not be separately identified. At laparotomy, a right ovarian tumor was confirmed, but due to extensive adhesions in the pelvis, it could only be removed piecemeal. Preoperative levels of a-fetoprotein (AFP), human chorionic gonadotrophin, and CA125 were measured on stored serum. AFP was elevated at 213 mg/liter (õ10); the other markers were within normal limits.
102
0090-8258/97 $25.00 Copyright q 1997 by Academic Press All rights of reproduction in any form reserved.
AID
Daya and Scully’s review of 13 cases of cervical sarcoma botryoides refers to apparently the same 2 patients [3]. We report the case of a patient with a cervical sarcoma botryoides diagnosed when she was 14 years old who then developed an ovarian Sertoli-Leydig tumor with heterologous elements at 27 years of age. The basis of the association remains unknown.
09-16-97 06:42:07
goas
AP: GYN
103
CASE REPORT
FIG. 1. Cervical sarcoma botryoides. Cervical stroma is expanded by a low-cellularity tumor with some condensation beneath the mucosa and around blood vessels (hematoxylin and eosin, 140).
Pathology Cervical tumor. The radical hysterectomy specimen showed a 4-cm broad papillary mass on a stalk 2 cm long and 0.6 cm in diameter arising from the ectocervix of the anterior lip, beside the external os. The mass was composed of firm to soft gray tissue with areas of edema and hemorrhage. Tumor was confined to the polypoid mass and did not involve the stalk or remainder of the cervix, corpus, vaginal cuff, or 16 bilateral pelvic lymph nodes. Microscopically, the tumor consisted of a loose population of atypical spindle cells set in an edematous matrix with numerous small blood vessels. The cells formed condensations beneath the ecto- and endocervical epithelium and around endocervical glands and occasional blood vessels (Fig. 1). The tumor cells were generally small with irregular hyperchromatic nuclei and little cytoplasm (Fig. 2), but areas of strap-like cells with eosinophilic cytoplasm, amorphous eosinophilic globules, and large pleomorphic hyperchromatic nuclei were seen, although no cross-striations were identified in any cells. Many of these large cells had degenerate karyorrhectic nuclei. Mitoses were frequent and often atypical, particularly when occurring in the pleomorphic cells. Occasional foci of immature cartilage were identified (Fig. 3). Immunoperoxidase showed patchy cytoplasmic reactivity for desmin- and muscle-specific actin in the noncartilagenous portions of the tumor. A final diagnosis of stage 1b cervical sarcoma botryoides (embryonal rhabdomyosarcoma) with heterologous cartilagenous differentiation was made. Ovarian tumor. The right oophorectomy specimen weighed 145 g and consisted of 7 fragments of solid and cystic tumor ranging in size from 12 1 5 1 2 to 3 1 2 1 0.5 cm. Cut section showed cysts up to several centimeters in diameter separated by spongy, pale solid tissue in which
AID
Gyn 4807
/
6d23$$$242
09-16-97 06:42:07
FIG. 2. Cervical sarcoma botryoides. Mildly atypical spindle cells in a myxoid background (hematoxylin and eosin, 1400).
FIG. 3. Cervical sarcoma botryoides. Island of immature cartilage in a vascular stroma (hematoxylin and eosin, 1100).
goas
AP: GYN
104
GOLBANG ET AL.
producing endometrioid-like cysts and tubules were interpreted as a heterologous component of yolk sac tumor, endometrioid-like variant. The adjacent ovarian tissue contained normal primordial and developed follicles, follicular cysts, corpora albicantia, and normal cellular stroma. A diagnosis of stage 1a right ovarian Sertoli–Leydig cell tumor of intermediate differentiation with a heterologous component of yolk sac tumor, endometrioid-like variant, with AFP production was made. Follow-up Serum AFP fell to normal levels by 20 days after oophorectomy for Sertoli–Leydig cell tumor. The patient is alive with no evidence of disease 36 months after diagnosis of her Sertoli–Leydig cell tumor and 16 years after diagnosis of sarcoma botryoides. The left ovary is still functioning because serial ultrasound scans have shown normal follicles
FIG. 4. Ovarian Sertoli–Leydig cell tumor. Cysts and tubules with areas of stromal hypercellularity (hematoxylin and eosin, 140).
numerous small locules 0.1 to 1 cm in diameter were present. This solid tissue varied from tan to yellow. Microscopically, the tumor was composed of cysts and tubules and a variably cellular stroma (Fig. 4). Around the cysts and tubules and also lying away from them were clusters of cells with polygonal nuclei and little cytoplasm, resembling mesenchyme, but occasionally containing sex cord elements as well as clumps of Leydig cells with central round nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm (Fig. 5). No Reinke crystals were seen. The cysts and tubules were lined by columnar cells with basal nuclei and abundant eosinophilic cytoplasm, resembling cells of well-differentiated endometrioid carcinoma. Occasional mitoses were noted in all cell types. There was no necrosis. Immunoperoxidase staining for AFP showed cytoplasmic positivity in the endometrioid-like cysts and tubules only. The mesenchyme, sex-cord structures, and Leydig cells were interpreted as a sex-cord stromal tumor of intermediate differentiation, while the AFP-
AID
Gyn 4807
/
6d23$$$242
09-16-97 06:42:07
FIG. 5. Ovarian Sertoli–Leydig cell tumor with endometrioid-like tubules, mesenchyme, and Leydig cells (hematoxylin and eosin, 1400).
goas
AP: GYN
105
CASE REPORT
TABLE 1 Reported Associations between Cervical Sarcoma Botryoides and Ovarian Sertoli–Leydig Tumor Cervical sarcoma botryoides
Sertoli–Leydig cell tumor
Case
Age
Clinical
Pathology
Age
Daya, case 6
23
Vaginal bleeding; polyp, 4 cm; TAH / pelvic nodes
SB
24 or 26 a
Daya, case 9
15
Vaginal bleeding; polyp, 3 cm; polypectomy / chemo
SB with heterologous cartilage
Present case
14
Vaginal bleeding; polyp, 4 cm; radical hysterectomy / pelvic nodes
SB with heterologous cartilage
Clinical
Pathology
Follow-up
NK a
SLCT intermediate differentiation
4 years, SB, A&W; ?SLCT a
16 or 18 a
NK a
SLCT intermediate differentiation
1 year, SB, A&W; ?SLCT a
27
Abdominal pain, hirsutism; 12-cm solid and cystic right ovarian mass; unilateral oophorectomy
SLCT intermediate differentiation with heterologous elements
16 years, SB; 3 years, SLCT; A&W
Note. Abbreviations used: SB, sarcoma botryoides; SLCT, Sertoli–Leydig cell tumor; A&W, alive and well; NK, not known; chemo, chemotherapy. a Young and Scully reported of 207 patients with ovarian Sertoli–Leydig cell tumor who had a past history of cervical sarcoma botryoides 1 and 3 years previously, but do not give information on individual cases.
and serum levels of follicle-stimulating hormone and inhibin are in normal limits for a premenopausal woman. DISCUSSION
The diagnosis of cervical sarcoma botryoides was made on the clinical findings of a soft polypoid tumor in the cervix in a 14-year-old girl with the histological features of atypical spindle cells in a myxoid matrix, cambium layer, cells with eosinophilic cytoplasm, and islands of immature cartilage, supported by positive desmin- and muscle-specific actin on immunoperoxidase. Cross-striations were not found, but have been reported to be seen in only 15% of cases [4]. Foci of immature cartilage have been noted in 45% of cases [3]. Cervical sarcoma botryoides must be distinguished from its more common vaginal counterpart because of its occurrence in an older age group of 12–26, mean 18 years, compared to less than 2 years, and better prognosis. In a recent review 80% of patients were alive with no evidence of disease after a mean follow-up period of 68 months [3]. The diagnosis of ovarian Sertoli–Leydig cell tumor (SLCT) was made based on the presence of a cystic and solid ovarian unilateral tumor in a 27-year-old woman composed of nodular cellular aggregates resembling immature mesenchyme, occasional sex-cord structures, and Leydig cells. There was a heterologous endodermal element which did not resemble the usual mature mucinous cystic tumor, but resembled the endometrioid-like variant of yolk sac tumor of endoderm, an interpretation supported by the finding of AFP production by the tumor
AID
Gyn 4807
/
6d23$$$242
09-16-97 06:42:07
[5]. If the endometrioid-like AFP-producing cysts and tubules were the only component of the tumor, a diagnosis of pure yolk sac tumor, endometrioid-like variant would have been the diagnosis. While AFP is a universal marker for yolk sac tumor, it is also occasionally seen in SLCT, but the value is usually much lower [2]. Interestingly, although not present in this case, immature cartilage and skeletal muscle are seen in 5% of SLCT and provide a potential link between cervical sarcoma botryoides and ovarian SLCT because these elements may be seen in both [6]. SLCT has a good prognosis, with recurrence of intermediate differentiated SLCT occurring in 11% [6]. The clinicopathological findings of the three women with the same association are presented in Table 1. The causes of cervical SB and ovarian SLCT and the reason for their association are not known. We have no hypothesis to link the two conditions together and there is no evidence presented in our or the previously reported cases to indicate what the basis of the association might be. A genetic abnormality, in view of the knowledge that sarcomas in the young may show non-random genetic changes and that Sertoli–Leydig cell tumors are rarely familial, is a possibility. Familial occurrence of SLCT has been documented in six cases and a personal and/or family history of thyroid abnormalities including multinodular goiter and adenoma have also been reported in association with SLCT [7]. While there has been no evidence of an inherited cause of cervical sarcoma botryoides, there has been one case of vaginal sarcoma botryoides reported in a child with multiple congenital abnormalities and bilateral nephroblastomas, suggesting a possible genetic
goas
AP: GYN
106
GOLBANG ET AL.
defect [8]. Unfortunately, cytogenetics were not performed on either tumor or the patient’s blood in our case. ACKNOWLEDGMENTS We are grateful to R. H. Young, M.D., and R. E. Scully, M.D., Department of Pathology, Harvard Medical School, Massachusetts General Hospital, Boston, who reviewed the slides of the ovarian tumor and made the diagnosis of Sertoli–Leydig cell tumor with a heterologous component of yolk sac tumor, endometrioid-like variant.
REFERENCES 1. Young RH, Scully RE: Sex-Cord Stromal, Steroid Cell and Other Ovarian Tumors with Endocrine, Paraendocrine and Paraneoplastic Manifestations, in Kurman RJ (ed): Blaustein’s Pathology of the Female Genital Tract, New York, Springer Verlag, 1994, 4th ed, pp 783–847
AID
Gyn 4807
/
6d23$$$242
09-16-97 06:42:07
2. Young RH, Scully RE: Ovarian Sertoli–Leydig cell tumors: a clinicopathological analysis of 207 cases. Am J Pathol 9:543–569, 1985 3. Daya DA, Scully RE: Sarcoma botryoides of the uterine cervix in young women: the clinicopathological study of 13 cases. Gynecol Oncol 29:290–304, 1988 4. Morales AR, Fine G, Horn RC Jr: Rhabdomyosarcoma: an ultrastructural appraisal. Pathol Annu 7:81–106, 1972 5. Clement PB, Young RH, Scully RE: Endometrioid-like variant of ovarian yolk sac tumor. A clinicopathological analysis of eight cases. Am J Surg Pathol 11:767–778, 1987 6. Zaloudek C, Norris HJ: A clinicopathologic study of 64 intermediate and poorly differentiated Sertoli–Leydig cell tumors of the ovary. Am J Surg Pathol 405–418, 1984 7. Fletcher JA, Gias Z, Donovan K, et al: Ovarian granulosa stromal cell tumors are characterized by trisomy 12: Am J Pathol 138: 515–520, 1991 8. Schmidt WA: Pathology of the Vagina, in Fox H, Wells M (eds): Haines and Taylor Obstetrical and Gynaecological Pathology, New York, Churchill Livingstone, 1995, 4th ed, pp 135–258
goas
AP: GYN