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Multiple neuroectodermal tumors arising in a mature cystic teratoma of the ovary
GYNECOLGGIC
ONCOLOGY
34,
222-225 (1989)
CASE REPORT Multiple Neuroectodermal Tumors Arising in a Mature Cystic Teratoma of the Ovary KARL S. OL~H, M.B., CH.B.,* PETER G. NEEDHAM, *Acting Registrar
in Obstetrics and Gynecology, Hospital, Raddlebarn
fConsultant Obstetrician and Gynecologist, and #Consultant Road, Selly Oak, Birmingham 829 6JD, United Kingdom Received
CASE REPORT A 19-year-old white Caucasian was admitted to the hospital with a 2-day history of severe, constant right iliac fossa pain. There were no other symptoms reported, and she had previously been well. She was taking the combined oral contraceptive pill, and her last withdrawal bleed had been 21 days prior to admission. There was no history of irregular bleeding. The patient was subject to regular pelvic examinations at her family planning clinic, and her last examination, a year prior to admission, had been reported as normal. Clinical examination revealed an area of mild tenderness in the right iliac fossa, and a mass equivalent in size to a 25week gestation arising from the pelvis. The uterus was felt separate from the mass. The presence of a large fluid-filled cyst with solid areas was confirmed on ultrasound. At laparotomy, a 20-cm diameter multilocular cyst was found on the left ovary, with a 6-cm diameter cyst on the right. The appendix was acutely inflamed. A left salpingo-oophorectomy, right ovarian cystectomy, and appendectomy were performed. The patient’s postoperative recovery was uneventful. Histologically, the right ovarian cyst was found to be a typical benign dermoid cyst containing sebaceous material and hair. Macroscopically, the cyst from the left ovary was multiloculated and filled with yellow fluid. The inner walls of many of the loculi were studded with 222 Copyright 0 1989 by Academic Press, Inc. All rights of reproduction in any form reserved.
Pathologist,
Selly Oak
March 29, 1988
Benign cystic teratomas are common, comprising ll-20% of all ovarian tumors. Neural elements can be identified in 38% of teratomas, but the development of a malignant neural neoplasm in an ovarian tumor of this type is rare. Multiple neuroectodermal tumor foci have not been previously described in association with a IUatUR CyStiC teratOnU3 of the ovary. 0 1989 Academic PI~SS, IIIC.
0090-82X3/89 $1.50
FRCS, FRCOG,? AND BERI JONES, FRCPATH.S
small sessile papillary excrescences, with one larger lesion measuring 4 cm in diameter. Microscopically, features typical of a benign dermoid cyst were seen. However, sections from the papillary excrescences contained brain tissue (with neurons in places) which merged with a well-differentiated astrocytic glioma showing cystic transformation (Fig. 1). In addition, there were foci of oligodendrocytoma (Fig. 2) and a compact, undifferentiated small-celled tumor, resembling a medulloblastoma or neuroblastoma (Fig. 3). The tumor did not appear to have breached the cyst wall. The patient was discharged on the seventh postoperative day, and is reviewed regularly in the clinic. The patient remains well 6 years following surgery.
DISCUSSION Benign cystic teratomas (dermoid cysts) are common and comprise 1l-20% of ovarian tumors [l-3]. Solid ovarian teratomas present most commonly in the second decade, while the incidence of benign cystic teratoma peaks in the third and fourth decades. Malignancy arising in these lesions is most commonly seen in the fifth or sixth decades [3], malignant transformation occurring in l-2% of cases [4-91 and forming less than 0.5% of primary ovarian malignant tumors [lo]. Malignant change most commonly involves the epithelial elements in the teratoma with squamous carcinoma being the most common. Most of the remainder are adenocarcinomas [47,9-121. Neural tissue is found in 38% of teratomas [ 131, but the development of a malignant neural neoplasm in an ovarian tumor of this type is extremely rare. A grade 4 astrocytoma arising in a mature cystic teratoma of the
FIG ,. 2.
Foci of oligodendrocytoma. The tumor is very cellular, the majority of the cells being round with distinct cell boundaries ( x 223
224
OLAH, NEEDHAM,
AND JONES
.G. 3. Foci of undifferentiated small-celled tumor. The cells resemble medulloblastoma or neuroblastoma ceils in that they are simall, td or oval cells with little cytoplasm. The cells are irregularly arranged ( x 125).
ovary has been reported [14], and five cases of a neu- foci were found to be present. Such a variety of tumors roblastoma developing in an ovarian mature cystic ter- within a single benign cystic teratoma has not been preatoma are recorded [ 1.5-181.However, in only one case, viously reported. Malignancy developing in one of the elements of a which reported the development of a neuroblastoma, was there any real evidence of malignant change occurring benign cystic teratoma is thought to be associated with in a previously benign teratoma. This is the only report a poor prognosis [10,14,18,19]. In general, neuroectoof a definite tumor mass showing the histological features dermal tumors arising in the central nervous systems do not metastasize to other organs. They may, however, be of a neuroblastoma [ 181. Aguirre and Scully (1982) [19] reported five cases of disseminated by the cerebrospinal fluid to other parts of malignant neural tumors of the ovary, four of which the nervous system. Thus, to avoid direct (transperitoappeared to be primary ovarian neoplasms and one which neal) spread of tumor, every attempt should be made to was possibly a metastasis from an adrenal neuroblas- excise ovarian tumors intact. Transcoelomic spread as toma. Of the four primary ovarian tumors, two were a result of extracapsular extension of tumor or of cyst classified as primitive neuroectodermal neoplasms, with rupture during surgery may result in neuroglial peritoneal no elements within the tumor typical of a benign cystic metastases [20,21], which is a rare, but progressive conteratoma. The remaining two tumors, both classified as dition which responds poorly to chemotherapy 122-241 glioblastoma, were associated with mature nonneural tis- and radiotherapy [25,26]. The long-term survival without sue elements and appeared to have developed in a mature recurrence in this patient is probably a result of the tumor being encapsulated and the cyst being removed intact at cystic teratoma. The case reported is exceptional in that although the operation, In view of the nature of spread of neuroeccyst had the macroscopic and microscopic features of a todermal tumors, the prognosis would be expected to be mature cystic teratoma, multiple neuroectodermal tumor good.
CASE REPORT
ACKNOWLEDGMENT We acknowledge the help of the late Professor W. T. Smith, Professor of Neuropathology at the University of Birmingham, for his analysis of the histopathology.
REFERENCES 1. Peterson, W. F., Prevost, E. C., Edmunds, F. T., Hundley, J. M., Jr., and Morris, F. K. Epidermoid carcinoma arising in benign cystic teratoma, Amer. J. Obstet. Gynecol. 71, 173-189 (1956). 2. Curling, 0. M., Potsides, P. N., and Hudson, C. N. Malignant change in benign cystic teratoma of the ovary, Brit. J. Obstet. Gynecol. 86, 399-402 (1979). 3. Fox, H., and Langley, F. A. Tumours of the ovary, Heinemann, London (1981). 4. Peterson, W. F. Malignant degeneration of benign cystic teratomas of the ovary. Collective review of literature, Obstet. Gynecol. Surv. 12, 793-830 (1957). 5. Kelly, R. R., and Scully, R. E. Cancer developing in dermoid cysts of the ovary, Cancer 14, 989-1000 (l%l). 6. Climie, A. R., and Heath, L. P. Malignant degeneration of benign cystic teratomas of the ovary, Cancer 22, 824-832 (1968). 7. Caruso, P. A., Marsh, M. E., Minkowitz, S., and Karten, G. An intense clinicopathologic study of 305 teratomas of the ovary, Cancer 27, 343-348 (1971). 8. Novak, E. R., and Woodruff, J. D. Gynecologic and obstetric pathology with clinical and endocrine relations, Saunders, Philadelphia, 8th ed., pp. 486-490 (1979). 9. Krumerman, M. S., and Chung, A. Squamous carcinoma arising in benign cystic teratoma of the ovary, Cancer 39, 1237-1242 (1977). 10. Stamp, G. W. H., and McConnell, E. M. Malignancy arising in cystic ovarian teratomas. A report of 24 cases, Brit. J. Obstet. Gynecol. 90, 671-675 (1983). 11. Yakushiji, M., Nishida, T., Sugiyama, T., Mitamura, T., Natsuaki, Y., Nagano, H., Tusunawaki, A., and Kato, T. Malignant degeneration of benign cystic teratomas of the ovary, Acta Obstet. Gynecol. Japan 33, 1095-1098 (1981). 12. Hunter, V., Barnhill, D., Jadwin, D., and Crooks, L. Ovarian mutinous cystadenocarcinoma of low malignant potential associ-
225
ated with a mature cystic teratoma, Gynecol. Oncol. 29, 250-254 (1988). 13. Woodruff, J. D., Protos, P., and Peterson, W. F. Ovarian teratomas: Relationship of histologic and ontogenic factors to prognosis, Amer. J. Obstet. Gynecol. 102, 702-714 (1968). 14. Shirley, R. L., Piro, A. J., and Cracker, D. W. Malignant neural elements in a benign cystic teratoma: A case report, Obstet. Gynecol. 37, 402-407 (1971). 15. McCullough, C. D., and Hardart, F. Neuroblastomatous transformation in a benign cystic teratoma, Obstet. Gynecol. 21, 259-261 (1%3). 16. Mayberger, H. W. Adenocarcinoma of thyroid origin in a benign cystic teratoma, Amer. J. Obstet. Gynecol. 78, 817-820 (1959). 17. Theppisai, R., Shuangshoti, S., and Amatyakul, A. Neuroblastoma arising in metastasizing ovarian teratoma, J. Med. Assoc. Thai. 60, 3%-404 (1977). 18. Reid, H., Van Der Walt, J. D., and Fox, H. Neuroblastoma arising in a mature cystic teratoma of the ovary, J. Clin. Pathol. 36, 6873 (1983). 19. Aguirre, F., and Scully, R. E. Malignant neuroectodermal tumour of the ovary. A distinctive form of monodermal teratoma: Report of five cases, Amer. J. Surg. Pathol. 6, 283-292 (1982). 20. Robboy, S. J., and Scully, R. E. Ovarian teratoma with glial implants on the peritoneum. An analysis of twelve cases, Hum. Parho/. 1, 643-653 (1970). 21. Luesley, D. M., Monypenny, I. J., Fielding, J. W., and Chan, K. K. Gliomatosis peritonei associated with ovarian teratomas. Case report, Brit. J. Obstet. Gynecol. 98, 668-670 (1983). 22. Cangir, A., Smith, J., and Vantys, J. Improved prognosis in children with ovarian cancers following modified V.A.C. chemotherapy, Cancer 43, 1234-1238 (1978). 23. Fort& R. W., and Mathie, I. K. Gliomatosis peritonei caused by ovarian teratoma, J. C&n. Pathof. 22, 348-353 (1%9). 24. Smith, J. P., Rutledge, F., and Sutow, W. W. Malignant gynecologic tumours in children: Current approaches to treatment, Amer. J. Obstet. Gynecol. 116, 261-269 (1973). 25. Noguchi, T. T., and Lonser, E. R. Unusual teratoma of the ovary with implantation in the abdominal cavity, Amer. J. Obstet. Gynecol. 82, 381-385 (1961). 26. Thurlbeck, W. M., and Scully, R. E. Solid teratoma of the ovary: A clinicopathological analysis of nine cases, Cancer 31, 804-811 Wm.
ONCOLOGY
34,
222-225 (1989)
CASE REPORT Multiple Neuroectodermal Tumors Arising in a Mature Cystic Teratoma of the Ovary KARL S. OL~H, M.B., CH.B.,* PETER G. NEEDHAM, *Acting Registrar
in Obstetrics and Gynecology, Hospital, Raddlebarn
fConsultant Obstetrician and Gynecologist, and #Consultant Road, Selly Oak, Birmingham 829 6JD, United Kingdom Received
CASE REPORT A 19-year-old white Caucasian was admitted to the hospital with a 2-day history of severe, constant right iliac fossa pain. There were no other symptoms reported, and she had previously been well. She was taking the combined oral contraceptive pill, and her last withdrawal bleed had been 21 days prior to admission. There was no history of irregular bleeding. The patient was subject to regular pelvic examinations at her family planning clinic, and her last examination, a year prior to admission, had been reported as normal. Clinical examination revealed an area of mild tenderness in the right iliac fossa, and a mass equivalent in size to a 25week gestation arising from the pelvis. The uterus was felt separate from the mass. The presence of a large fluid-filled cyst with solid areas was confirmed on ultrasound. At laparotomy, a 20-cm diameter multilocular cyst was found on the left ovary, with a 6-cm diameter cyst on the right. The appendix was acutely inflamed. A left salpingo-oophorectomy, right ovarian cystectomy, and appendectomy were performed. The patient’s postoperative recovery was uneventful. Histologically, the right ovarian cyst was found to be a typical benign dermoid cyst containing sebaceous material and hair. Macroscopically, the cyst from the left ovary was multiloculated and filled with yellow fluid. The inner walls of many of the loculi were studded with 222 Copyright 0 1989 by Academic Press, Inc. All rights of reproduction in any form reserved.
Pathologist,
Selly Oak
March 29, 1988
Benign cystic teratomas are common, comprising ll-20% of all ovarian tumors. Neural elements can be identified in 38% of teratomas, but the development of a malignant neural neoplasm in an ovarian tumor of this type is rare. Multiple neuroectodermal tumor foci have not been previously described in association with a IUatUR CyStiC teratOnU3 of the ovary. 0 1989 Academic PI~SS, IIIC.
0090-82X3/89 $1.50
FRCS, FRCOG,? AND BERI JONES, FRCPATH.S
small sessile papillary excrescences, with one larger lesion measuring 4 cm in diameter. Microscopically, features typical of a benign dermoid cyst were seen. However, sections from the papillary excrescences contained brain tissue (with neurons in places) which merged with a well-differentiated astrocytic glioma showing cystic transformation (Fig. 1). In addition, there were foci of oligodendrocytoma (Fig. 2) and a compact, undifferentiated small-celled tumor, resembling a medulloblastoma or neuroblastoma (Fig. 3). The tumor did not appear to have breached the cyst wall. The patient was discharged on the seventh postoperative day, and is reviewed regularly in the clinic. The patient remains well 6 years following surgery.
DISCUSSION Benign cystic teratomas (dermoid cysts) are common and comprise 1l-20% of ovarian tumors [l-3]. Solid ovarian teratomas present most commonly in the second decade, while the incidence of benign cystic teratoma peaks in the third and fourth decades. Malignancy arising in these lesions is most commonly seen in the fifth or sixth decades [3], malignant transformation occurring in l-2% of cases [4-91 and forming less than 0.5% of primary ovarian malignant tumors [lo]. Malignant change most commonly involves the epithelial elements in the teratoma with squamous carcinoma being the most common. Most of the remainder are adenocarcinomas [47,9-121. Neural tissue is found in 38% of teratomas [ 131, but the development of a malignant neural neoplasm in an ovarian tumor of this type is extremely rare. A grade 4 astrocytoma arising in a mature cystic teratoma of the
FIG ,. 2.
Foci of oligodendrocytoma. The tumor is very cellular, the majority of the cells being round with distinct cell boundaries ( x 223
224
OLAH, NEEDHAM,
AND JONES
.G. 3. Foci of undifferentiated small-celled tumor. The cells resemble medulloblastoma or neuroblastoma ceils in that they are simall, td or oval cells with little cytoplasm. The cells are irregularly arranged ( x 125).
ovary has been reported [14], and five cases of a neu- foci were found to be present. Such a variety of tumors roblastoma developing in an ovarian mature cystic ter- within a single benign cystic teratoma has not been preatoma are recorded [ 1.5-181.However, in only one case, viously reported. Malignancy developing in one of the elements of a which reported the development of a neuroblastoma, was there any real evidence of malignant change occurring benign cystic teratoma is thought to be associated with in a previously benign teratoma. This is the only report a poor prognosis [10,14,18,19]. In general, neuroectoof a definite tumor mass showing the histological features dermal tumors arising in the central nervous systems do not metastasize to other organs. They may, however, be of a neuroblastoma [ 181. Aguirre and Scully (1982) [19] reported five cases of disseminated by the cerebrospinal fluid to other parts of malignant neural tumors of the ovary, four of which the nervous system. Thus, to avoid direct (transperitoappeared to be primary ovarian neoplasms and one which neal) spread of tumor, every attempt should be made to was possibly a metastasis from an adrenal neuroblas- excise ovarian tumors intact. Transcoelomic spread as toma. Of the four primary ovarian tumors, two were a result of extracapsular extension of tumor or of cyst classified as primitive neuroectodermal neoplasms, with rupture during surgery may result in neuroglial peritoneal no elements within the tumor typical of a benign cystic metastases [20,21], which is a rare, but progressive conteratoma. The remaining two tumors, both classified as dition which responds poorly to chemotherapy 122-241 glioblastoma, were associated with mature nonneural tis- and radiotherapy [25,26]. The long-term survival without sue elements and appeared to have developed in a mature recurrence in this patient is probably a result of the tumor being encapsulated and the cyst being removed intact at cystic teratoma. The case reported is exceptional in that although the operation, In view of the nature of spread of neuroeccyst had the macroscopic and microscopic features of a todermal tumors, the prognosis would be expected to be mature cystic teratoma, multiple neuroectodermal tumor good.
CASE REPORT
ACKNOWLEDGMENT We acknowledge the help of the late Professor W. T. Smith, Professor of Neuropathology at the University of Birmingham, for his analysis of the histopathology.
REFERENCES 1. Peterson, W. F., Prevost, E. C., Edmunds, F. T., Hundley, J. M., Jr., and Morris, F. K. Epidermoid carcinoma arising in benign cystic teratoma, Amer. J. Obstet. Gynecol. 71, 173-189 (1956). 2. Curling, 0. M., Potsides, P. N., and Hudson, C. N. Malignant change in benign cystic teratoma of the ovary, Brit. J. Obstet. Gynecol. 86, 399-402 (1979). 3. Fox, H., and Langley, F. A. Tumours of the ovary, Heinemann, London (1981). 4. Peterson, W. F. Malignant degeneration of benign cystic teratomas of the ovary. Collective review of literature, Obstet. Gynecol. Surv. 12, 793-830 (1957). 5. Kelly, R. R., and Scully, R. E. Cancer developing in dermoid cysts of the ovary, Cancer 14, 989-1000 (l%l). 6. Climie, A. R., and Heath, L. P. Malignant degeneration of benign cystic teratomas of the ovary, Cancer 22, 824-832 (1968). 7. Caruso, P. A., Marsh, M. E., Minkowitz, S., and Karten, G. An intense clinicopathologic study of 305 teratomas of the ovary, Cancer 27, 343-348 (1971). 8. Novak, E. R., and Woodruff, J. D. Gynecologic and obstetric pathology with clinical and endocrine relations, Saunders, Philadelphia, 8th ed., pp. 486-490 (1979). 9. Krumerman, M. S., and Chung, A. Squamous carcinoma arising in benign cystic teratoma of the ovary, Cancer 39, 1237-1242 (1977). 10. Stamp, G. W. H., and McConnell, E. M. Malignancy arising in cystic ovarian teratomas. A report of 24 cases, Brit. J. Obstet. Gynecol. 90, 671-675 (1983). 11. Yakushiji, M., Nishida, T., Sugiyama, T., Mitamura, T., Natsuaki, Y., Nagano, H., Tusunawaki, A., and Kato, T. Malignant degeneration of benign cystic teratomas of the ovary, Acta Obstet. Gynecol. Japan 33, 1095-1098 (1981). 12. Hunter, V., Barnhill, D., Jadwin, D., and Crooks, L. Ovarian mutinous cystadenocarcinoma of low malignant potential associ-
225
ated with a mature cystic teratoma, Gynecol. Oncol. 29, 250-254 (1988). 13. Woodruff, J. D., Protos, P., and Peterson, W. F. Ovarian teratomas: Relationship of histologic and ontogenic factors to prognosis, Amer. J. Obstet. Gynecol. 102, 702-714 (1968). 14. Shirley, R. L., Piro, A. J., and Cracker, D. W. Malignant neural elements in a benign cystic teratoma: A case report, Obstet. Gynecol. 37, 402-407 (1971). 15. McCullough, C. D., and Hardart, F. Neuroblastomatous transformation in a benign cystic teratoma, Obstet. Gynecol. 21, 259-261 (1%3). 16. Mayberger, H. W. Adenocarcinoma of thyroid origin in a benign cystic teratoma, Amer. J. Obstet. Gynecol. 78, 817-820 (1959). 17. Theppisai, R., Shuangshoti, S., and Amatyakul, A. Neuroblastoma arising in metastasizing ovarian teratoma, J. Med. Assoc. Thai. 60, 3%-404 (1977). 18. Reid, H., Van Der Walt, J. D., and Fox, H. Neuroblastoma arising in a mature cystic teratoma of the ovary, J. Clin. Pathol. 36, 6873 (1983). 19. Aguirre, F., and Scully, R. E. Malignant neuroectodermal tumour of the ovary. A distinctive form of monodermal teratoma: Report of five cases, Amer. J. Surg. Pathol. 6, 283-292 (1982). 20. Robboy, S. J., and Scully, R. E. Ovarian teratoma with glial implants on the peritoneum. An analysis of twelve cases, Hum. Parho/. 1, 643-653 (1970). 21. Luesley, D. M., Monypenny, I. J., Fielding, J. W., and Chan, K. K. Gliomatosis peritonei associated with ovarian teratomas. Case report, Brit. J. Obstet. Gynecol. 98, 668-670 (1983). 22. Cangir, A., Smith, J., and Vantys, J. Improved prognosis in children with ovarian cancers following modified V.A.C. chemotherapy, Cancer 43, 1234-1238 (1978). 23. Fort& R. W., and Mathie, I. K. Gliomatosis peritonei caused by ovarian teratoma, J. C&n. Pathof. 22, 348-353 (1%9). 24. Smith, J. P., Rutledge, F., and Sutow, W. W. Malignant gynecologic tumours in children: Current approaches to treatment, Amer. J. Obstet. Gynecol. 116, 261-269 (1973). 25. Noguchi, T. T., and Lonser, E. R. Unusual teratoma of the ovary with implantation in the abdominal cavity, Amer. J. Obstet. Gynecol. 82, 381-385 (1961). 26. Thurlbeck, W. M., and Scully, R. E. Solid teratoma of the ovary: A clinicopathological analysis of nine cases, Cancer 31, 804-811 Wm.