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Primary paraovarian cystadenocarcinoma: Clinical and management aspects and literature review
GYNECOLOGIC
ONCOLOGY
38, 268-272 (19%)
CASE REPORT Primary Paraovarian Cystadenocarcinoma: Clinical and Management Aspects and Literature Review MARCO Gynecological
M. ALTARAS, M.D.,*,’ RICHARD JAFFE, M.D . ,* MARIO CORDUBA, M.D. ,t MICHAEL HOLTZINGER, M.D. ,$: CHARLES BAHARY, M.D.S Oncology
Units, Departments
of Obstetrics and Gynecology *A and fl3, and TDepartment Meir Hospital, Kfar Saba, 44281, Israef
of Pathology,
Sapir Medical
Center,
Received November 28, 1989
Two casesof primary paraovarian-origin serous cystadenocarcinema, one invasive and one of low malignant potential, are presented. Both were diagnosed in postmenopausal women and were initially treated surgically. As of this writing, both women have survived 48 months past their initial diagnosis and have no clinically detectable disease. Clinical aspects and management problems of this fairly recently identified and rare entity are discussed and the literature on primary paraovarian malignant epithelial tumors is reviewed. o IWO Academic press, IIK.
INTRODUCTION Primary malignant paraovarian epithelial tumors (PMTs) are extremely rare and, including the present two cases, only 20 such neoplasms have been documented in the English literature [l-17]. Paraovarian epithelial tumors were initially considered to be of wolffian (mesonephric) origin, but studies by Gardner et al. [3] have revealed that most of these tumors are of mtillerian (paramesonephric) origin. They also have established the histological criteria for determining the origin of these tumors [15]. Whether the origin is wolffian, mtillerian, or mesothelial metaplasia probably is unimportant when management methods and related problems are to be considered. Also, whether these rare neoplasms differ from their ovarian counterparts, in their invasive or lowmalignant-potential (LMP) forms and in their clinical and ’ To whom correspondence and requests for reprints should be addressed. * All authors are also affiliated with the Sackler School of Medicine, University of Tel Aviv.
biological behavior, has yet to be determined. According to the accepted histological criteria, the two cases presented here were found to be of mtillerian origin [3-151. CASE REPORT Case I A 76-year-old Caucasian P,G2 was referred to the gynecological ward in March 1985 for investigation of a pelvic mass and a significant enlargement of her abdomen 3 months prior to admission. General examination raised the possibility of ascites, although liver and spleen were not palpable. Pelvic examination revealed a small uterus, but both adnexae could not be palpated due to a large amount of ascites. A large mobile mass was felt in the pelvis. Ultrasound examination was suggestive of an ovarian multilobular cystic mass. Preoperative investigations were without any pathological findings. At laparotomy, 6 liters of ascitic fluid was aspirated. The abdominal cavity was surveyed and no special findings were detected. Examination of the pelvis revealed a normal-size uterus, with normalappearing ovaries and fallopian tubes. The left adnexa contained a large, multilobular dark-colored cystic mass which was located within the leaves of the broad ligament adjacent to the ovary. No peritoneal implants were identified. Total abdominal hysterectomy (TAH) and bilateral salpingo-oophorectomy (BSO) were performed. The postoperative course was uneventful and the ascites disappeared subsequent to surgery. Cytological assessment of the ascitic fluid revealed no malignancy. Histological examination of the mass !68
0090-8258190 $1.50 Copyright 0 1990by Academic Press,Inc. All rights of reproduction in any form reserved.
269
CASE REPORT
showed a well-differentiated serous cystadenocarcinoma arising in a parovarian cyst, with capsular invasion. She has been regularly followed and was 53 months postdiagnosis, with no clinical evidence of disease, at the time of this writing.
The postoperative course was uneventful. The final histology of the tumor showed an LMP serous cystadenocarcinoma arising from a parovarian cyst. The peritoneal washings were negative for malignancy. As of this writing, she is 51 months postsurgery, with no clinical evidence of disease.
Case 2 A 54-year-old Caucasian, G2P,, was admitted to the gynecological department in July 1985. She had been complaining of a recent enlargement of her abdomen. Her past medical history was not contributive regarding the actual complaints. On admission, she was found to be in general good health, with no noteworthy pathological findings. Pelvic examination revealed a huge cystic mass reaching the lower right rib cage. The uterus was slightly enlarged and neither adnexa could be palpated. On ultrasonic examination, the cystic mass was thought to be of left ovarian origin with several large papillary projections arising from the interior wall. Clinical and laboratory examinations were within normal limits. A laparotomy was performed. No peritoneal effusion was observed. A multilobular cystic smooth mass was found arising from and attached to the right, broad ligament. The right ovary, fallopian tube, adjacent structures, left adnexa, and slightly enlarged uterus were normal in appearance. A survey of the rest of the abdominal cavity revealed no palpable lymph nodes or tumor implants. Following pelvic and abdominal washings, the cystic mass was removed, and TAH, BSO, and an infracolic omentectomy were performed.
FIG.
1.
Atypical
glands showing papillary
PATHOLOGY Case 1 The surgical specimen that was submitted consisted of a normal-appearing left tube and ovary, with an adherent cystic mass mesuring 12 x 9 x 8 cm in its largest dimensions and located within the broad ligament. On cut section, the mass proved to be cystic and partially filled with soft, papillary structures. The lumen contained a turbid, semithick fluid. Microscopic examination of this paraovarian mass (Fig. 1) showed a cuboidal epithelium lining the smooth inner wall zones and an endosalpingeal epithelium overlying the papillary projections. A definite basement membrane was not apparent. In several places, conspicuous foci of foamy histiocytes were noted under the neoplastic epithelium. Some of the papillary structures, with a dense stroma, were covered by epithelium showing prominent heaping-up, some cellular atypia, and few mitotic figures. There also were areas in which definitive invasion of the stroma by atypical glandular structures was observed. Examination of the uterus and right adnexa revealed no noteworthy pathology.
formations
invading
the stroma. H&E,
x
IO.
270
ALTARAS
ET AL.
Although this condition is certainly a rare one, and is cited in textbooks of gynecologic pathology, the clinical A thin-walled cystic tumor measuring 28 x 22 x 15 and management problems have not been documented cm in its largest dimensions and weighing 4300 g was at all, mainly because of the scanty experience gained submitted. The inner surface showed multiple firm papthus far in this field. Therefore, paraovarian cystadeillary projections and budlike excrescences. nocarcinomas, either LMP or invasive forms, have conOn microscopic examination, the cyst wall was found tinually presented a management challenge to the gyneto be lined with cuboidal epithelium, showing occasional cologist. Table 1 summarizes the clinical data on all cases cilia. The tumor arising from the lining epithelium was treated for these neoplasms gathered from the medical papillary, with solid areas composed of glandular strucliterature plus our two cases. tures and cellular stroma. The epithelium of the tumor These data reveal that the diagnosis of PMT was not ranged from cuboidal, to columnar, to secretory, to hobmade for any of the patients prior to surgery. Five of nail-type cells (Fig. 2). these patients underwent ultrasound assessment for a The additional surgical specimen consisted of the palpable pelvic mass, but none were suspected of haruterus and both adnexae. Their histological examination boring a paraovarian tumor, including our own [9,10,13]. revealed no pathological findings. Moreover, in at least three patients, the existence of a PMT was not identified at the time of the tumor’s reDISCUSSION moval. Only the histological diagnosis revealed its presence, and these patients underwent a reexploration to Lennox and Maegher in 1952 opened a new chapter complete surgical treatment and to assess disease status in gynecological oncology by reporting the first case of [9,13,14]. The above data show the extreme difficulty a malignant epithelial tumor of the broad ligament [l]. encountered in the empirical diagnosis of these neoplasms prior to surgery. The second important aspect in these predominantly young patients is the age factor at the time of diagnosis, which might pose critical surgical decision dilemmas, particularly for those patients wishing to preserve their childbearing options or to prevent unnecessary castration procedures. Despite the dearth of published data, the mean age has been found to be 43.1 years, with the youngest patient 13 years old. Our first case, 76 years of age, is the oldest reported patient. A diagnosis was made at 40 years and under for 47.3% (9/19) of the patients. Since the entity is rare, there is no accepted treatment plan or follow-up strategy as there is for their ovarian counterparts. Thus, surgical and additional treatment modalities for the majority of cases were empirically determined. An additional important aspect, in relation to the relatively youthful patient, is the possibility of the coexistence of these tumors with pregnancy, which has been found in 10% (2/20) of the cases [ 14,171. In our first case, the presenting symptom was ascites. Its presence in an amount disproportionate to the relative size of the tumor, with an intact capsule, the absence of tumor cells in the peritoneal effusion, and the disappearance of ascitic fluid following removal of the neoplasm are quite interesting and make this case unique, in both its clinical presentation and its disease course when compared with the other PMTs. Because the peritoneal effusion was submitted for a cytological examination, no diagnostic procedures other than TAH and BSO were employed. FIG. 2. High-power view showing papillary projections or tufts, In our second case, peritoneal washings, diaphragsome of which are detached from the lining. Note the absence of invasion. H&E, x 100. matic wipes, and an infracolic omentectomy were perCase 2
271
CASE REPORT
TABLE 1 Summary of Clinical Data in 20 Cases of Malignant Paraovarian Epithelial Tumors Case No.
Age of patient
Author and year
Pathology Adenoca (serous) Adenoca (clear cell) Adenoca (serous) Adeno (gr.111)
Origin U
I
Lennox (1952)
45
2
Johnston (1957)
37
3
Gardner (1957)
50
Merrill (1959)
70
Hertig (1960) Beck (1962)
40
LMP Adenoca (gr.11) (clear cell)
M W
7
Czernobilsky (1972)
29
LMP (serous)
M
8
Taxy (1976)
41
LMP
W
9
Genadry (1977)
38
LMP (serous)
M
IO
Genadry (1977)
36
LMP (serous)
M
11
Genadry (1977)
28
LMP (serous)
M
12
Genadry (1977)
13
M
13
Clark (1979)
29
14 15
Duvall (1983) Chardraratnam (1983)
52 53
Adenoca (gr.11) (serous) Adenoca (gr.1) (endometrioid) LMP (serous) LMP (serous)
16 17
Yamasaki (1983) Rojansky (1985)
45 40
LMP LMP (serous)
M M
18 19
Seltzer (1988) Altaras (Present Case No. 1) Altaras (Present Case No. 2)
44 76
LMP (mutinous) Adenoca. (gr.1) (serous) LMP (serous)
M M
20
54
-
W U M
M M M
M
Indication for surgery
Treatment
Outcome follow-up
Pain pelvic mass -
TAH + BSO X-ray -
Alive 10 Mt NED -
Pain pelvic mass Pelvic mass ascites Vag. bleeding tumor invasion Adnexal mass pain TAH
Inoperable Xray TAH + BSO
Survived 27 Mt
Adnexal mass Sterilization Adnexal mass Abdominal mass Pain adnexal mass Pelvic mass Accidental finding Lumbal pain Pain mass Pelvic mass Pelvic mass ascites Abdominal mass
Total pelvic exenteration Xray TAH + BSO X-ray TAH + BSO cobalt Adnexectomy Adnexectomy Adnexectomy Adnexectomy TAH + BSO TAH + BSO Adnexectomy TAH + BSO TAH + BSO TAH + BSO TAH + BSO TAH + BSO omentectomy washings
Short Follow-up
Survived 18 Mt
Not mentioned Alive 78 Mt NED Alive 60 Mt NED Alive 60 Mt NED Lost to followup Alive 60 Mt NED Not mentioned Not mentioned Alive 30 Mt NED Alive Alive 12 Mt NED Short follow-up Alive 54 Mt NED Alive 51 Mt NED
Note. LMP, low malignant potential; U, unknown; W, wollfian; M, Mulletian; NED, Nonevidence of disease; Mt, months.
formed as part of the initial surgical treatment. In each i.e., (1) the histological similarities in serous, mutinous, case, we also sought peritoneal implants for sampling endometrioid, and clear cell features encountered in purposes. To the best of our knowledge, these current ovarian- and paraovarian-origin tumors, and (2) their approaches were not employed in the initial surgery of common coelemic epithelium origin in their histogenesis, any of the other published cases. However, we are not we can assume that may be LMP and invasive PMTs convinced that the above procedures are to be consid- with intact capsules can be managed like their ovarian ered as optimal for these PMTs with invasive and LMP counterparts, despite the lack of consensus on clinical features. staging for this entity [8,9,14,17]. Recently, Seltzer et al. A survey of the literature (Table I), including our suggested a similar approach for LMP forms of these cases, indicates that the mean ages for the LMP and tumors [14]. invasive types of PMT are 46 and 4.5years, respectively, Survival rates for the 18 reported patients remain inwhich are almost the same. This mean age, in both forms, conclusive. This is due mainly to the rarity of these cases is similar to the age range of patients with tumors of and the lack of uniformity in both their treatment moovarian origin [18,19]. If we add another two factors, dalities and reporting of follow-up results. Among the
ALTARAS ET AL.
272
seven cases with invasive PMTs, only one survived more than 5 years, two died of the disease, and two were alive at the time of this writing. No data were available on the survival of the remaining two. In addition, only 3 of 11 patients with LMP PMTs were found to survive more than 5 years, and no deaths were reported in this group. When comparing our findings with the scanty literature, one should recall that most of the cases used individual management. Thus, there are no bona fide guidelines for modern management methods and follow-up modalities. The data are minimal, and it is difficult to draw conclusions on the clinical and biological behavior of these tumors. It is hoped that a general consensus on management criteria will be reaches to aid the physician in treatment decisions when such rare entities are encountered. REFERENCES 1. Lennox, B . , and Meagher, D. J . Paraovarian carcinoma: A hitherto undescribed tumor, .l. Obstet. Gynaecol. Brit. Emp. 59, 783-785 (1952). 2. Johnston, J. W., Kemodle, J. R., Saunders, C. L., and Burlington, N. C. Malignant mesonephroma of the broad ligament: A case report, Amer. J. Obstet. Gynecol. 74, 1272-1274 (1957). 3. Gardner, G. H., Green, R. R., and Peckham, B. Tumors of the broad ligament, Amer. J. Obstet. Gynecol. 73, 536-555 (1957). 4. Merrill, J. A. Carcinoma of the broad ligament, Obstet. Gynecol. 13, 472-476 (1959). 5. Czemobilsky, B., and Lancet, M. Broad ligament adenocarcinoma of mullerian origin, Obstet. Gynecol. 40, 238-242 (1972). 6. Hertig, A. T., and Gore, H. Tumor of the vulva, vagina and uterus, in Atlas of tumor pathology, Armed Forces Institute of Pathology, Washington, DC, p. 274 (1960).
7. Taxy, J. B., and Battifora, H. Female adnexal tumor of probable Wollfian origin, Cancer 37, 2349-2354 (1976).
8. Genadry, R., Parmley, T., and Woodruff, J. D. The origin and clinical behavior of the paraovarian tumor, Amer. J. Obstet. Gynecol. 129, 873-880 (1977). 9. Clark, J. E., Wood, H., Jaffurs, W. J., and Fabro, S. Endometrioid-type cystadenocarcinoma arising in the mesosalpinx, Obstet. Gynecol. 54, 656-658 (1979). 10. Yamasaki, K., Watanabe, Y., and Imaoawa, K. Papillary tumor of the broad ligament, Acta Pathol. Japan. 33, 979-984 (1983). 11. Chandraratnam, E., and Leong, A. S-Y. Papillary serous cystadenoma of borderline malignancy arising in a paraovarian paramesonephric cyst: Light microscopic and ultrastructural observations, Histopathology 7, 601-611 (1983). Duvall, E., and Survis, J. A. Borderline tumors of the broad lig12. ament: Case report, Bit. J. Obstet. Gynaecol. 90, 372-375 (1983). 13. Rojansky, N., Ophir, E., Sharony, A., Spira, H., and Suprun, A. Broad ligament adenocarcinoma: Its origins and clinical behavior. A literature review and report of a case, Obstet. Gynecol. Surv. 40, 665-671 (1985). 14. Seltzer, V. L., Molho, L., Fougner, A., et al. Paraovarian cystadenocarcinoma of low malignant potential, Gynecol. Oncol. 30, 216-221 (1988). 15. Gardner, G. H., Greene, R. L., and Peckham, B. M. Normal and cystic structures of the broad ligament, Amer. J. Obstet. Gynecol. 55, 517-539 (1948). 16. McKay, D. G. The origin of ovarian tumors, Clin. Obstet. Gynecol. 5, 1181 (1962). 17. Beck, J. S., and Scott, L. A. Malignant mesonephroma arising in a congenital mesonephric duct cyst of the broad ligament, J. Pathol. Bacterial. 83, 557-560 (1962). 18. Lim-Tan, S. K., Cajigas, H. E., and Scully, R. E. Ovarian cystectomy for serous borderline tumors: A follow-up study of 35 cases. Obstet. Gynecol. 72, 775-781 (1988). 19. Annual report on the results of treatment in gynecological cancer, FIG0 20, 115 (1988).
ONCOLOGY
38, 268-272 (19%)
CASE REPORT Primary Paraovarian Cystadenocarcinoma: Clinical and Management Aspects and Literature Review MARCO Gynecological
M. ALTARAS, M.D.,*,’ RICHARD JAFFE, M.D . ,* MARIO CORDUBA, M.D. ,t MICHAEL HOLTZINGER, M.D. ,$: CHARLES BAHARY, M.D.S Oncology
Units, Departments
of Obstetrics and Gynecology *A and fl3, and TDepartment Meir Hospital, Kfar Saba, 44281, Israef
of Pathology,
Sapir Medical
Center,
Received November 28, 1989
Two casesof primary paraovarian-origin serous cystadenocarcinema, one invasive and one of low malignant potential, are presented. Both were diagnosed in postmenopausal women and were initially treated surgically. As of this writing, both women have survived 48 months past their initial diagnosis and have no clinically detectable disease. Clinical aspects and management problems of this fairly recently identified and rare entity are discussed and the literature on primary paraovarian malignant epithelial tumors is reviewed. o IWO Academic press, IIK.
INTRODUCTION Primary malignant paraovarian epithelial tumors (PMTs) are extremely rare and, including the present two cases, only 20 such neoplasms have been documented in the English literature [l-17]. Paraovarian epithelial tumors were initially considered to be of wolffian (mesonephric) origin, but studies by Gardner et al. [3] have revealed that most of these tumors are of mtillerian (paramesonephric) origin. They also have established the histological criteria for determining the origin of these tumors [15]. Whether the origin is wolffian, mtillerian, or mesothelial metaplasia probably is unimportant when management methods and related problems are to be considered. Also, whether these rare neoplasms differ from their ovarian counterparts, in their invasive or lowmalignant-potential (LMP) forms and in their clinical and ’ To whom correspondence and requests for reprints should be addressed. * All authors are also affiliated with the Sackler School of Medicine, University of Tel Aviv.
biological behavior, has yet to be determined. According to the accepted histological criteria, the two cases presented here were found to be of mtillerian origin [3-151. CASE REPORT Case I A 76-year-old Caucasian P,G2 was referred to the gynecological ward in March 1985 for investigation of a pelvic mass and a significant enlargement of her abdomen 3 months prior to admission. General examination raised the possibility of ascites, although liver and spleen were not palpable. Pelvic examination revealed a small uterus, but both adnexae could not be palpated due to a large amount of ascites. A large mobile mass was felt in the pelvis. Ultrasound examination was suggestive of an ovarian multilobular cystic mass. Preoperative investigations were without any pathological findings. At laparotomy, 6 liters of ascitic fluid was aspirated. The abdominal cavity was surveyed and no special findings were detected. Examination of the pelvis revealed a normal-size uterus, with normalappearing ovaries and fallopian tubes. The left adnexa contained a large, multilobular dark-colored cystic mass which was located within the leaves of the broad ligament adjacent to the ovary. No peritoneal implants were identified. Total abdominal hysterectomy (TAH) and bilateral salpingo-oophorectomy (BSO) were performed. The postoperative course was uneventful and the ascites disappeared subsequent to surgery. Cytological assessment of the ascitic fluid revealed no malignancy. Histological examination of the mass !68
0090-8258190 $1.50 Copyright 0 1990by Academic Press,Inc. All rights of reproduction in any form reserved.
269
CASE REPORT
showed a well-differentiated serous cystadenocarcinoma arising in a parovarian cyst, with capsular invasion. She has been regularly followed and was 53 months postdiagnosis, with no clinical evidence of disease, at the time of this writing.
The postoperative course was uneventful. The final histology of the tumor showed an LMP serous cystadenocarcinoma arising from a parovarian cyst. The peritoneal washings were negative for malignancy. As of this writing, she is 51 months postsurgery, with no clinical evidence of disease.
Case 2 A 54-year-old Caucasian, G2P,, was admitted to the gynecological department in July 1985. She had been complaining of a recent enlargement of her abdomen. Her past medical history was not contributive regarding the actual complaints. On admission, she was found to be in general good health, with no noteworthy pathological findings. Pelvic examination revealed a huge cystic mass reaching the lower right rib cage. The uterus was slightly enlarged and neither adnexa could be palpated. On ultrasonic examination, the cystic mass was thought to be of left ovarian origin with several large papillary projections arising from the interior wall. Clinical and laboratory examinations were within normal limits. A laparotomy was performed. No peritoneal effusion was observed. A multilobular cystic smooth mass was found arising from and attached to the right, broad ligament. The right ovary, fallopian tube, adjacent structures, left adnexa, and slightly enlarged uterus were normal in appearance. A survey of the rest of the abdominal cavity revealed no palpable lymph nodes or tumor implants. Following pelvic and abdominal washings, the cystic mass was removed, and TAH, BSO, and an infracolic omentectomy were performed.
FIG.
1.
Atypical
glands showing papillary
PATHOLOGY Case 1 The surgical specimen that was submitted consisted of a normal-appearing left tube and ovary, with an adherent cystic mass mesuring 12 x 9 x 8 cm in its largest dimensions and located within the broad ligament. On cut section, the mass proved to be cystic and partially filled with soft, papillary structures. The lumen contained a turbid, semithick fluid. Microscopic examination of this paraovarian mass (Fig. 1) showed a cuboidal epithelium lining the smooth inner wall zones and an endosalpingeal epithelium overlying the papillary projections. A definite basement membrane was not apparent. In several places, conspicuous foci of foamy histiocytes were noted under the neoplastic epithelium. Some of the papillary structures, with a dense stroma, were covered by epithelium showing prominent heaping-up, some cellular atypia, and few mitotic figures. There also were areas in which definitive invasion of the stroma by atypical glandular structures was observed. Examination of the uterus and right adnexa revealed no noteworthy pathology.
formations
invading
the stroma. H&E,
x
IO.
270
ALTARAS
ET AL.
Although this condition is certainly a rare one, and is cited in textbooks of gynecologic pathology, the clinical A thin-walled cystic tumor measuring 28 x 22 x 15 and management problems have not been documented cm in its largest dimensions and weighing 4300 g was at all, mainly because of the scanty experience gained submitted. The inner surface showed multiple firm papthus far in this field. Therefore, paraovarian cystadeillary projections and budlike excrescences. nocarcinomas, either LMP or invasive forms, have conOn microscopic examination, the cyst wall was found tinually presented a management challenge to the gyneto be lined with cuboidal epithelium, showing occasional cologist. Table 1 summarizes the clinical data on all cases cilia. The tumor arising from the lining epithelium was treated for these neoplasms gathered from the medical papillary, with solid areas composed of glandular strucliterature plus our two cases. tures and cellular stroma. The epithelium of the tumor These data reveal that the diagnosis of PMT was not ranged from cuboidal, to columnar, to secretory, to hobmade for any of the patients prior to surgery. Five of nail-type cells (Fig. 2). these patients underwent ultrasound assessment for a The additional surgical specimen consisted of the palpable pelvic mass, but none were suspected of haruterus and both adnexae. Their histological examination boring a paraovarian tumor, including our own [9,10,13]. revealed no pathological findings. Moreover, in at least three patients, the existence of a PMT was not identified at the time of the tumor’s reDISCUSSION moval. Only the histological diagnosis revealed its presence, and these patients underwent a reexploration to Lennox and Maegher in 1952 opened a new chapter complete surgical treatment and to assess disease status in gynecological oncology by reporting the first case of [9,13,14]. The above data show the extreme difficulty a malignant epithelial tumor of the broad ligament [l]. encountered in the empirical diagnosis of these neoplasms prior to surgery. The second important aspect in these predominantly young patients is the age factor at the time of diagnosis, which might pose critical surgical decision dilemmas, particularly for those patients wishing to preserve their childbearing options or to prevent unnecessary castration procedures. Despite the dearth of published data, the mean age has been found to be 43.1 years, with the youngest patient 13 years old. Our first case, 76 years of age, is the oldest reported patient. A diagnosis was made at 40 years and under for 47.3% (9/19) of the patients. Since the entity is rare, there is no accepted treatment plan or follow-up strategy as there is for their ovarian counterparts. Thus, surgical and additional treatment modalities for the majority of cases were empirically determined. An additional important aspect, in relation to the relatively youthful patient, is the possibility of the coexistence of these tumors with pregnancy, which has been found in 10% (2/20) of the cases [ 14,171. In our first case, the presenting symptom was ascites. Its presence in an amount disproportionate to the relative size of the tumor, with an intact capsule, the absence of tumor cells in the peritoneal effusion, and the disappearance of ascitic fluid following removal of the neoplasm are quite interesting and make this case unique, in both its clinical presentation and its disease course when compared with the other PMTs. Because the peritoneal effusion was submitted for a cytological examination, no diagnostic procedures other than TAH and BSO were employed. FIG. 2. High-power view showing papillary projections or tufts, In our second case, peritoneal washings, diaphragsome of which are detached from the lining. Note the absence of invasion. H&E, x 100. matic wipes, and an infracolic omentectomy were perCase 2
271
CASE REPORT
TABLE 1 Summary of Clinical Data in 20 Cases of Malignant Paraovarian Epithelial Tumors Case No.
Age of patient
Author and year
Pathology Adenoca (serous) Adenoca (clear cell) Adenoca (serous) Adeno (gr.111)
Origin U
I
Lennox (1952)
45
2
Johnston (1957)
37
3
Gardner (1957)
50
Merrill (1959)
70
Hertig (1960) Beck (1962)
40
LMP Adenoca (gr.11) (clear cell)
M W
7
Czernobilsky (1972)
29
LMP (serous)
M
8
Taxy (1976)
41
LMP
W
9
Genadry (1977)
38
LMP (serous)
M
IO
Genadry (1977)
36
LMP (serous)
M
11
Genadry (1977)
28
LMP (serous)
M
12
Genadry (1977)
13
M
13
Clark (1979)
29
14 15
Duvall (1983) Chardraratnam (1983)
52 53
Adenoca (gr.11) (serous) Adenoca (gr.1) (endometrioid) LMP (serous) LMP (serous)
16 17
Yamasaki (1983) Rojansky (1985)
45 40
LMP LMP (serous)
M M
18 19
Seltzer (1988) Altaras (Present Case No. 1) Altaras (Present Case No. 2)
44 76
LMP (mutinous) Adenoca. (gr.1) (serous) LMP (serous)
M M
20
54
-
W U M
M M M
M
Indication for surgery
Treatment
Outcome follow-up
Pain pelvic mass -
TAH + BSO X-ray -
Alive 10 Mt NED -
Pain pelvic mass Pelvic mass ascites Vag. bleeding tumor invasion Adnexal mass pain TAH
Inoperable Xray TAH + BSO
Survived 27 Mt
Adnexal mass Sterilization Adnexal mass Abdominal mass Pain adnexal mass Pelvic mass Accidental finding Lumbal pain Pain mass Pelvic mass Pelvic mass ascites Abdominal mass
Total pelvic exenteration Xray TAH + BSO X-ray TAH + BSO cobalt Adnexectomy Adnexectomy Adnexectomy Adnexectomy TAH + BSO TAH + BSO Adnexectomy TAH + BSO TAH + BSO TAH + BSO TAH + BSO TAH + BSO omentectomy washings
Short Follow-up
Survived 18 Mt
Not mentioned Alive 78 Mt NED Alive 60 Mt NED Alive 60 Mt NED Lost to followup Alive 60 Mt NED Not mentioned Not mentioned Alive 30 Mt NED Alive Alive 12 Mt NED Short follow-up Alive 54 Mt NED Alive 51 Mt NED
Note. LMP, low malignant potential; U, unknown; W, wollfian; M, Mulletian; NED, Nonevidence of disease; Mt, months.
formed as part of the initial surgical treatment. In each i.e., (1) the histological similarities in serous, mutinous, case, we also sought peritoneal implants for sampling endometrioid, and clear cell features encountered in purposes. To the best of our knowledge, these current ovarian- and paraovarian-origin tumors, and (2) their approaches were not employed in the initial surgery of common coelemic epithelium origin in their histogenesis, any of the other published cases. However, we are not we can assume that may be LMP and invasive PMTs convinced that the above procedures are to be consid- with intact capsules can be managed like their ovarian ered as optimal for these PMTs with invasive and LMP counterparts, despite the lack of consensus on clinical features. staging for this entity [8,9,14,17]. Recently, Seltzer et al. A survey of the literature (Table I), including our suggested a similar approach for LMP forms of these cases, indicates that the mean ages for the LMP and tumors [14]. invasive types of PMT are 46 and 4.5years, respectively, Survival rates for the 18 reported patients remain inwhich are almost the same. This mean age, in both forms, conclusive. This is due mainly to the rarity of these cases is similar to the age range of patients with tumors of and the lack of uniformity in both their treatment moovarian origin [18,19]. If we add another two factors, dalities and reporting of follow-up results. Among the
ALTARAS ET AL.
272
seven cases with invasive PMTs, only one survived more than 5 years, two died of the disease, and two were alive at the time of this writing. No data were available on the survival of the remaining two. In addition, only 3 of 11 patients with LMP PMTs were found to survive more than 5 years, and no deaths were reported in this group. When comparing our findings with the scanty literature, one should recall that most of the cases used individual management. Thus, there are no bona fide guidelines for modern management methods and follow-up modalities. The data are minimal, and it is difficult to draw conclusions on the clinical and biological behavior of these tumors. It is hoped that a general consensus on management criteria will be reaches to aid the physician in treatment decisions when such rare entities are encountered. REFERENCES 1. Lennox, B . , and Meagher, D. J . Paraovarian carcinoma: A hitherto undescribed tumor, .l. Obstet. Gynaecol. Brit. Emp. 59, 783-785 (1952). 2. Johnston, J. W., Kemodle, J. R., Saunders, C. L., and Burlington, N. C. Malignant mesonephroma of the broad ligament: A case report, Amer. J. Obstet. Gynecol. 74, 1272-1274 (1957). 3. Gardner, G. H., Green, R. R., and Peckham, B. Tumors of the broad ligament, Amer. J. Obstet. Gynecol. 73, 536-555 (1957). 4. Merrill, J. A. Carcinoma of the broad ligament, Obstet. Gynecol. 13, 472-476 (1959). 5. Czemobilsky, B., and Lancet, M. Broad ligament adenocarcinoma of mullerian origin, Obstet. Gynecol. 40, 238-242 (1972). 6. Hertig, A. T., and Gore, H. Tumor of the vulva, vagina and uterus, in Atlas of tumor pathology, Armed Forces Institute of Pathology, Washington, DC, p. 274 (1960).
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