GYNECOLOGIC
ONCOLOGY
38, 135-137 (1990)
CASE REPORT Immature Presacral Teratoma in an Adult Female ALBERTO
LOPES,M.B., CH.B., MRCOG,” SHEILA E. PEARSON, M.B., B.S., MRCOG,” J. TREVOR ROBERTS, MRCP, FRCR,? AND JOHN M. MONAGHAN, FRCS (ED), FRCOG*
*Regional Department of Gynaecological Oncology, Queen Elizabeth Hospital, Gotesheud, Tyne und Weur, and +Regionul Radiotherapy Centre, NeMwstle Generul Hospital, Newcustle, Enxlund Received
August 18, 1989
combined abdominoperineal approach. There was no space between the mass and the pelvic side wall, preventing formal dissection. The gross specimen was a nodular cystic mass 12 x 10 x 8 cm; it was mostly covered by a capsule except over focal areas where it was “torn” surgically. Histologically, it was reported as a grade 2 immature teratoma. The patient made a good recovery but in June 1987 she was found to have a local INTRODUCTION recurrence posterior to the cervix. She was therefore Immature presacral teratomas in adults are extremely referred to the Regional Radiotherapy Unit in Newcastle. A CT scan showed a 2.5cm soft tissue mass to the rare. Cases reported previously have invariably resulted in death [ 1,2]. The present case describes survival in a left of the rectum in a presacral location. A second 2.5 cm mass was seen medial to the left common iliac vessels patient treated using a multidisciplinary approach. and was thought to be an enlarged lymph node. The serum cY-fetoprotein (AFP) was measured at 37 KU/liter CASE REPORT (normal up to 10) and the P-human chorionic gonadoA 24-year-old patient presented in her second preg- tropin (P-hCG) was normal. The patient was treated with nancy with a high head at term. Pelvic examination re- combination chemotherapy consisting of cisplatin, etovealed a smooth round mass posterior to the vagina and poside (VP-16), and bleomycin. However, the tumor conan ultrasound scan suggested this to be a dermoid cyst. tinued to grow and after two courses of treatment had Her first pregnancy 5 years earlier had resulted in a increased to 4 x 6 cm. A repeat serum AFP was still spontaneous vaginal delivery of a 3.2-kg infant. elevated at 37 KU/liter. The patient was therefore reIn January 1987 she required an emergency cesarean ferred to the Regional Gynaecological Oncology Unit at section for failure to progress in labor; the cervix dilated Gateshead for surgery. A laparotomy was performed in to 4 cm and the head remained high. A retroperitoneal September 1987. At operation multiple nodules were presacral mass approximately 7 cm in diameter was found on the cecum and ascending colon, a number of noted; surgical excision was not attempted. Two months which were removed. After extensive dissection, a 3later in March 1987 the mass was just palpable abdom- cm-diameter nodule of tumor was removed from the right inally. Coitus was impossible and the patient complained side of the rectum, but the remainder of the tumor was of low backache radiating to the groins. She had a poor fixed to the pelvic floor and sidewall. The procedure was stream on micturition and her stools were “squashed.” abandoned. Intravenous urography showed lateral displacement of The patient was therefore given external-beam radiothe ureters and an ultrasound showed a mass 11 x 11 therapy to the pelvis to a total dose of 50 Gy over 20 x 6.5 cm posterior to the uterus “suggestive of a fractions. Two weeks following the treatment the tumor had grown in size but her serum AFP was 2 KU/liter, fibroid.” In April 1987 the mass was “shelled” out using a the first time the level had been in the normal range. A case of immature presacral teratoma in an adult female presentingin pregnancyis described.Sheremainsfree of disease 12 months following treatment with a combination of chemotherapy, radiotherapy, and surgery. Serum Lu-fetoproteinlevels appearedto correlate more with tumor “aggression” than with tumor growth. 0 1990 Academic Press, Inc.
135 009@8258/90 $1.50 Copyright 0 1990 by Academic Press, Inc. All rights of reproduction in any form reserved.
136
LOPES ET AL.
Because of the tumor growth she was given second-line chemotherapy consisting of tamoxifen and etretinate orally in combination with adriamycin, vincristine, and ifosfamide. A CT scan performed in February 1988, after five courses, showed a 7-cm-diameter presacral tumor. There was apparent encapsulation though no clear fat plane was seen between it and the left pyriformis muscle or the rectum, suggesting probable invasion. Taking into account the normal serum AFP, possibly suggesting “maturation” of the tumor, the apparent encapsulation of the tumor on CT scan, and a clinical impression that the tumor was now resectable it was decided to attempt further surgery. In March 1988 the presacral tumor was completely excised with a segment of involved rectum. An end-to-end anastomosis was performed using the EEA stapling device and a temporary defunctioning ileostomy. Histology of the specimen, as in that obtained in the second laparotomy, showed a mature solid teratoma; the abundant immature neuroepithelium which was present in the initial specimen had disappeared. The patient made an uneventful postoperative recovery and the ileostomy was closed 3 months later. Twelve months following her last operation she remains free of tumor and with a normal serum AFP level. DISCUSSION Presacral teratomas in the adult are rare. Head et al. [l] in a review of the world literature in 1975 found only 71 documented cases including two of their own. Fiftythree were in females and three of these presented with pelvic dystocia complicating delivery, as in this case. Only 8 of the 71 cases were malignant, with an equal sex distribution; most suffered from local recurrences and eventually died. Lee and Symmonds [2] in a review of the management of presacral tumours in 70 females (age range, 13 days to 79 years) include 28 teratomas. Four of these were malignant and all the patients died within 4 years of the surgery. Teratomas are germ cell tumors composed of derivatives of the three primitive germ layers, ectoderm, mesoderm, and endoderm. They may exhibit varying degrees of maturity and a strong correlation has been recognized between this and the prognosis for the patient. A grading system has been proposed [3] for ovarian teratomas, related to the degree of maturity; this has been used for grading tumors at different sites as in this case. Immature teratomas lack a consistent tumor marker, but occasionally, as here, elevated levels of AFP have been reported [4]. The origin of the AFP has not been satisfactorily explained, some speculating that the immature neural component of the tumor is the source [5]. Multiple chemotherapy regimens have been shown to
result in dramatic improvement in prognosis of patients with teratomas of the ovary [6]. In this case the firstline combination chemotherapy of cisplatin, VP-16, and bleomycin appears to have resulted in histological maturation of the tumor. However, the patient received only two courses of treatment, as the tumor was continuing to grow and the serum AFP level was still elevated at 37 KU/liter. Resection of the tumor was also not possible at this stage. One may only speculate that the radiotherapy and possibly the second-line chemotherapy contributed to the “maturation” process, with serum AFP levels returning to normal after the radiotherapy. This continuing process appears to have made a previously inoperable tumor resectable. The fact that the AFP levels remained normal despite increase in tumor size agrees with the finding of Esterhay et al. [5] that the disease activity in teratomas may not always correlate well with the serum AFP levels. However, as for Esterhay et al. [5], surgical excision with normal AFP levels appears to have resulted in a cure whereas recurrences occurred when AFP levels were elevated initially. Various surgical approaches have been used in the treatment of presacral tumors. In a review [2] of the surgical management of 70 female patients treated at the Mayo Clinic, the abdominal approach, as in this case, was used in 56%, the transsacral in 28%, the abdominoperineal in 9%, and the transperineal in 7%. Generally, the transsacral approach was used in tumors less than 8 cm in size and located over the lower 10 cm of the sacrum and coccyx. In conclusion, immature presacral teratomas are very rare. They require aggressive management with individualization of care. Tumor markers if present may be of value in assessing clearance of aggressive tumor factors. Specialized pathology, radiotherapy, chemotherapy, and surgery have to be combined to achieve any prospect of cure. ACKNOWLEDGMENTS We thank Professor H. Fox for reviewing the histology from the three laparotomies. We also thank Mr. J. Foulds, Consultant Gynaecologist, and Dr. D. C. A. Senadhira, Consultant Pathologist, Bishop Auckland General Hospital, and Mr. M. Higgs, Consultant Surgeon, Queen Elizabeth Hospital, Gateshead, who were involved in the patient’s care.
REFERENCES 1. Head, H. D., Gerstein, J. D., and Muir, R. W. Presacral teratoma in the adult, Amer. Surg. 41, 240-248 (1975). 2. Lee, R. A., and Symmonds, R. E. Presacral tumors in the female: Clinical presentation, surgical management, and results, Obsrer. Gynecol. 71, 216-221 (1988). 3. Thurlbeck, W. M., and Scully, R. E. Solid teratoma of the ovary. A clinicouatholoaical analvsis of 9 cases. Cancer 13.804-811 (1960). .
CASE REPORT 4. Ishiguro, T., Oshida, Y., Tenzaki, T., Ohshima, M., and Suzuki, H. AFP in yolk sac tumor and solid teratoma of the ovary, Cuncrr 48, 2480-2482 (1981). 5. Esterhay, R. J., Shapiro, H. M., Sutherland, J. C., McIntyre, K. R., and Wiernik, P. H. Serum alpha-fetoprotein concentration
137
and tumor growth dissociation in a patient with ovarian teratocarcinema, Cancer 31, 835-839 (1973). 6. Curry, S. L., Smith, J. P., and Gallagher, H. S. Malignant teratoma of the ovary, Prognostic factors and treatment, Amer. J. Ohstet. Gynecol. 131, 845-849 (1978).
ONCOLOGY
38, 135-137 (1990)
CASE REPORT Immature Presacral Teratoma in an Adult Female ALBERTO
LOPES,M.B., CH.B., MRCOG,” SHEILA E. PEARSON, M.B., B.S., MRCOG,” J. TREVOR ROBERTS, MRCP, FRCR,? AND JOHN M. MONAGHAN, FRCS (ED), FRCOG*
*Regional Department of Gynaecological Oncology, Queen Elizabeth Hospital, Gotesheud, Tyne und Weur, and +Regionul Radiotherapy Centre, NeMwstle Generul Hospital, Newcustle, Enxlund Received
August 18, 1989
combined abdominoperineal approach. There was no space between the mass and the pelvic side wall, preventing formal dissection. The gross specimen was a nodular cystic mass 12 x 10 x 8 cm; it was mostly covered by a capsule except over focal areas where it was “torn” surgically. Histologically, it was reported as a grade 2 immature teratoma. The patient made a good recovery but in June 1987 she was found to have a local INTRODUCTION recurrence posterior to the cervix. She was therefore Immature presacral teratomas in adults are extremely referred to the Regional Radiotherapy Unit in Newcastle. A CT scan showed a 2.5cm soft tissue mass to the rare. Cases reported previously have invariably resulted in death [ 1,2]. The present case describes survival in a left of the rectum in a presacral location. A second 2.5 cm mass was seen medial to the left common iliac vessels patient treated using a multidisciplinary approach. and was thought to be an enlarged lymph node. The serum cY-fetoprotein (AFP) was measured at 37 KU/liter CASE REPORT (normal up to 10) and the P-human chorionic gonadoA 24-year-old patient presented in her second preg- tropin (P-hCG) was normal. The patient was treated with nancy with a high head at term. Pelvic examination re- combination chemotherapy consisting of cisplatin, etovealed a smooth round mass posterior to the vagina and poside (VP-16), and bleomycin. However, the tumor conan ultrasound scan suggested this to be a dermoid cyst. tinued to grow and after two courses of treatment had Her first pregnancy 5 years earlier had resulted in a increased to 4 x 6 cm. A repeat serum AFP was still spontaneous vaginal delivery of a 3.2-kg infant. elevated at 37 KU/liter. The patient was therefore reIn January 1987 she required an emergency cesarean ferred to the Regional Gynaecological Oncology Unit at section for failure to progress in labor; the cervix dilated Gateshead for surgery. A laparotomy was performed in to 4 cm and the head remained high. A retroperitoneal September 1987. At operation multiple nodules were presacral mass approximately 7 cm in diameter was found on the cecum and ascending colon, a number of noted; surgical excision was not attempted. Two months which were removed. After extensive dissection, a 3later in March 1987 the mass was just palpable abdom- cm-diameter nodule of tumor was removed from the right inally. Coitus was impossible and the patient complained side of the rectum, but the remainder of the tumor was of low backache radiating to the groins. She had a poor fixed to the pelvic floor and sidewall. The procedure was stream on micturition and her stools were “squashed.” abandoned. Intravenous urography showed lateral displacement of The patient was therefore given external-beam radiothe ureters and an ultrasound showed a mass 11 x 11 therapy to the pelvis to a total dose of 50 Gy over 20 x 6.5 cm posterior to the uterus “suggestive of a fractions. Two weeks following the treatment the tumor had grown in size but her serum AFP was 2 KU/liter, fibroid.” In April 1987 the mass was “shelled” out using a the first time the level had been in the normal range. A case of immature presacral teratoma in an adult female presentingin pregnancyis described.Sheremainsfree of disease 12 months following treatment with a combination of chemotherapy, radiotherapy, and surgery. Serum Lu-fetoproteinlevels appearedto correlate more with tumor “aggression” than with tumor growth. 0 1990 Academic Press, Inc.
135 009@8258/90 $1.50 Copyright 0 1990 by Academic Press, Inc. All rights of reproduction in any form reserved.
136
LOPES ET AL.
Because of the tumor growth she was given second-line chemotherapy consisting of tamoxifen and etretinate orally in combination with adriamycin, vincristine, and ifosfamide. A CT scan performed in February 1988, after five courses, showed a 7-cm-diameter presacral tumor. There was apparent encapsulation though no clear fat plane was seen between it and the left pyriformis muscle or the rectum, suggesting probable invasion. Taking into account the normal serum AFP, possibly suggesting “maturation” of the tumor, the apparent encapsulation of the tumor on CT scan, and a clinical impression that the tumor was now resectable it was decided to attempt further surgery. In March 1988 the presacral tumor was completely excised with a segment of involved rectum. An end-to-end anastomosis was performed using the EEA stapling device and a temporary defunctioning ileostomy. Histology of the specimen, as in that obtained in the second laparotomy, showed a mature solid teratoma; the abundant immature neuroepithelium which was present in the initial specimen had disappeared. The patient made an uneventful postoperative recovery and the ileostomy was closed 3 months later. Twelve months following her last operation she remains free of tumor and with a normal serum AFP level. DISCUSSION Presacral teratomas in the adult are rare. Head et al. [l] in a review of the world literature in 1975 found only 71 documented cases including two of their own. Fiftythree were in females and three of these presented with pelvic dystocia complicating delivery, as in this case. Only 8 of the 71 cases were malignant, with an equal sex distribution; most suffered from local recurrences and eventually died. Lee and Symmonds [2] in a review of the management of presacral tumours in 70 females (age range, 13 days to 79 years) include 28 teratomas. Four of these were malignant and all the patients died within 4 years of the surgery. Teratomas are germ cell tumors composed of derivatives of the three primitive germ layers, ectoderm, mesoderm, and endoderm. They may exhibit varying degrees of maturity and a strong correlation has been recognized between this and the prognosis for the patient. A grading system has been proposed [3] for ovarian teratomas, related to the degree of maturity; this has been used for grading tumors at different sites as in this case. Immature teratomas lack a consistent tumor marker, but occasionally, as here, elevated levels of AFP have been reported [4]. The origin of the AFP has not been satisfactorily explained, some speculating that the immature neural component of the tumor is the source [5]. Multiple chemotherapy regimens have been shown to
result in dramatic improvement in prognosis of patients with teratomas of the ovary [6]. In this case the firstline combination chemotherapy of cisplatin, VP-16, and bleomycin appears to have resulted in histological maturation of the tumor. However, the patient received only two courses of treatment, as the tumor was continuing to grow and the serum AFP level was still elevated at 37 KU/liter. Resection of the tumor was also not possible at this stage. One may only speculate that the radiotherapy and possibly the second-line chemotherapy contributed to the “maturation” process, with serum AFP levels returning to normal after the radiotherapy. This continuing process appears to have made a previously inoperable tumor resectable. The fact that the AFP levels remained normal despite increase in tumor size agrees with the finding of Esterhay et al. [5] that the disease activity in teratomas may not always correlate well with the serum AFP levels. However, as for Esterhay et al. [5], surgical excision with normal AFP levels appears to have resulted in a cure whereas recurrences occurred when AFP levels were elevated initially. Various surgical approaches have been used in the treatment of presacral tumors. In a review [2] of the surgical management of 70 female patients treated at the Mayo Clinic, the abdominal approach, as in this case, was used in 56%, the transsacral in 28%, the abdominoperineal in 9%, and the transperineal in 7%. Generally, the transsacral approach was used in tumors less than 8 cm in size and located over the lower 10 cm of the sacrum and coccyx. In conclusion, immature presacral teratomas are very rare. They require aggressive management with individualization of care. Tumor markers if present may be of value in assessing clearance of aggressive tumor factors. Specialized pathology, radiotherapy, chemotherapy, and surgery have to be combined to achieve any prospect of cure. ACKNOWLEDGMENTS We thank Professor H. Fox for reviewing the histology from the three laparotomies. We also thank Mr. J. Foulds, Consultant Gynaecologist, and Dr. D. C. A. Senadhira, Consultant Pathologist, Bishop Auckland General Hospital, and Mr. M. Higgs, Consultant Surgeon, Queen Elizabeth Hospital, Gateshead, who were involved in the patient’s care.
REFERENCES 1. Head, H. D., Gerstein, J. D., and Muir, R. W. Presacral teratoma in the adult, Amer. Surg. 41, 240-248 (1975). 2. Lee, R. A., and Symmonds, R. E. Presacral tumors in the female: Clinical presentation, surgical management, and results, Obsrer. Gynecol. 71, 216-221 (1988). 3. Thurlbeck, W. M., and Scully, R. E. Solid teratoma of the ovary. A clinicouatholoaical analvsis of 9 cases. Cancer 13.804-811 (1960). .
CASE REPORT 4. Ishiguro, T., Oshida, Y., Tenzaki, T., Ohshima, M., and Suzuki, H. AFP in yolk sac tumor and solid teratoma of the ovary, Cuncrr 48, 2480-2482 (1981). 5. Esterhay, R. J., Shapiro, H. M., Sutherland, J. C., McIntyre, K. R., and Wiernik, P. H. Serum alpha-fetoprotein concentration
137
and tumor growth dissociation in a patient with ovarian teratocarcinema, Cancer 31, 835-839 (1973). 6. Curry, S. L., Smith, J. P., and Gallagher, H. S. Malignant teratoma of the ovary, Prognostic factors and treatment, Amer. J. Ohstet. Gynecol. 131, 845-849 (1978).
