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Parovarian cystadenocarcinoma of low-malignant potential
GYNECOLOGIC
ONCOLOGY
30, 2 16-22 1 (1988)
Parovarian Cystadenocarcinoma of Low-Malignant Potential VICKI L. SELTZER, M.D.,*.’ LAURA MOLHO, M.D.,f ARTHUR FOUGNER, PETER HONG, M.D.,* BEATA KERESZTI, M.D. ,* MELANIE GERO, M.D. MARK SPITZER, M.D.*
M.D.,* ,i AND
*Department of Obstetrics and Gynecology and TDepartment of Pathology, Queens Hospital Center, Afjliation of the Long Island Jewish Medical Center and the State University of New York at Stony Brook, Jamaica, New York 11432 Received September 8, 1986 In the world’s literature, only 16 patients with parovarian malignancies have been described, five of which have been of low-malignant potential. All but one of these low-malignant potential lesions have been serous tumors. This is the second low-malignant potential parovarian tumor to be described with mutinous and serous components as well as hobnail cells. These tumors occur almost exclusively in young women, yet they are so rare that no treatment plan has been developed. We propose that until more data becomes available, these low-malignant potential lesions be treated as are their ovarian counterparts with staging laparotomy and surgery which conserves reproductive functioning being appropriate for women who meet the rigid clinical and histologic criteria for a very early potentially nonaggressive tumor, and complete extirpative surgery for those who do not. o 1988Academic Press. Inc.
INTRODUCTION
Epithelial ovarian tumors of low-malignant potential are a well-defined histologic and biologic entity comprising approximately 15-25% of all epithelial cancers [l]. On the other hand, parovarian neoplasms are much less common than ovarian lesions, and there have been only five reports in the literature of parovarian tumors of low-malignant potential [2-41. These have been predominantly serous. This paper reports the sixth low-malignant potential parovarian tumor, and the second with mutinous and serous components as well as hobnail cells. CASE REPORT
The patient is a 44-year-old Hispanic female, G5, P 4-O-O-4,who was admitted to the hospital on February 4, 1985, following an uncomplicated antepartum course. She had a normal spontaneous delivery of a term male infant, Apgar 910, and was scheduled to undergo an elective tubal ligation the following day. The patient’s past medical history was significant for hypothyroidism of 9 years ’ To whom reprint requests should be addressed at Queens Hospital Center, 82-68 164th Street, Jamaica, NY 11432. 216 OO90-8258/B$1.50 Copyright All rights
0 1988 by Academic Press, Inc. of reproduction in any form reserved.
LMP
PAROVARIAN
CYSTADENOCARCINOMA
217
duration, for which she took Synthyroid 0.1 mg daily, a vein stripping 11 years prior to admission, and a penicillin allergy. On February 5, 1985 the patient was taken to the operating room for a bilateral tubal ligation. This was performed through an infraumbilical incision. The left fallopian tube was ligated using a modified Pomeroy procedure. The right fallopian tube could not be traced to the fimbria and was thought to be wrapped over a large mass. Laparotomy was then initiated through a vertical incision, and an 8 x 6.5 x 6-cm cystic mass was found in the right broad ligament. The ovaries on both sides were normal. Adhesions were lysed, and a right salpingectomy and removal of the mass was performed. Upon careful gross and histologic examination of the mass, it was found to be separate from the ovary and the fallopian tube, the latter being stretched over the mass. A histologic diagnosis of borderline parovarian tumor with serous, mutinous, and clear cell components was made. Therefore, the patient had a second laparotomy performed 5 weeks later, at which time a total abdominal hysterectomy, left salpingo-oophorectomy, right oophorectomy, partial omentectomy, and careful abdominal exploration with peritoneal washings and multiple biopsies were performed. There was no evidence of disease in these specimens. The patient was discharged from the hospital in satisfactory condition and remains well. PATHOLOGY
The right fallopian tube was found to be entirely normal, but stretched over a separate cystic parovarian mass measuring 8 x 6.5 x 6 cm, with which it did not communicate. The mass was also completely separate from the ovary. The outer surface of the cyst wall was smooth (Fig. 1). The inner surface contained multiple friable papillations and contained copious yellow mutinous material. There were two additional benign parovarian cysts, measuring 2.5 cm. and 1.5 cm. respectively, not communicating with the larger cyst, and each containing clear fluid. The large parovarian cyst was lined predominantly by columnar, focally ciliated cells and had many complex papillary projections extending into the lumen (Fig. 2). These were comprised of epithelial cells, some with a hobnail pattern and others containing mucin (Figs. 3 and 4). Psammoma bodies were present (Fig. 5). There was focal stratification as well as detached cellular tufts and cytologic atypia. Rare mitoses were present. Multiple sections were taken, failing to demonstrate invasion of the cyst wall. The cyst wall contained smooth muscle. All addition1 specimens (uterus, cervix, ovaries, fallopian tubes, liver biopsy, omentum, multiple intraperitoneal biopsies, and peritoneal washings) showed no evidence of disease. DISCUSSION
Parovarian cysts arise in the mesosalpinx, between the ovarian hilum and the fallopian tube. They may be of mesothelial, paramesonephric, or mesonephric origin [3]. Parovarian cysts of clinical significance are considerably less frequent than ovarian cysts, and malignant parovarian lesions are exceedingly rare. In a review of 140 parovarian lesions from the Gynecologic Pathology Laboratory at
218
SELTZER ET AL.
FIG. 1. External surface of main cyst with adjacent benign smaller cyst and fallopian tube stretched over the mass.
Johns Hopkins, only four malignancies were demonstrated, three of which were low-malignant potential serous lesions [3]. The first parovarian cancer was described by Lennox in 1952 [5]. Since that time, 16 parovarian malignancies have been reported, five of which have been predominantly serous low-malignant potential lesions. This is the second lowmalignant potential parovarian tumor ever described which contains mutinous and serous components as well as hobnail cells. Low-malignant potential tumors have a histologic appearance (and presumably a biologic behavior) between the clearly benign and frankly malignant lesions. Histologic criteria for these lesions include stratification of the epithelial lining of the papillae, formation of tufts arising from the papillae, nuclear and cellular atypia, epithelial pleomorphism, mitotic activity, and the absence of stromal invasion. Little is known about the biologic behavior of the low-malignant potential parovarian tumor, since these lesions are so rare. Therefore, the decision regarding therapy was extrapolated from our therapeutic regimen for low-malignant potential ovarian lesions. As many as 40% of patients who have LMP ovarian tumors may have disease which is more extensive than Stage IA [6-81. The E-year survival for women with borderline ovarian tumors has been reported to be 73%. Therefore, it is
LMP
FIG. 2. Detail tufts (100X).
of complex
PAROVARIAN
papillary
219
CYSTADENOCARCINOMA
projections
with
pseudostratification
and detached
cellular
essential that these women have complete staging laparotomies to make certain that the disease has not spread. For women with advanced disease or for those in whom reproductive potential is no longer desired a total abdominal hysterectomy and bilateral salpingo-oophorectomy is performed. However, most of these lesions are found in young women, the youngest reported patient being 13 [3]. Perhaps, as is the case with ovarian neoplasms, patients who meet certain clinical and histologic criteria can be treated with preservation of their reproductive function, if they strongly desire more children, and if they understand that no definite answers regarding therapy are available. If the parovarian lesion has no external excrescences nor adhesions and can be shelled out without rupture. if there is no ascites, if there are no other structures involved (as demonstrated by multiple biopsies and careful staging laparotomy), if the lesion is low grade, and if the woman is young, reliable and very interested in future fertility, it may be acceptable to treat her without a hysterectomy. If the woman does not meet every one of these criteria, or if she does not desire future fertility, more aggressive surgery is probably advisable. The appropriate therapy for this unusual lesion has not been fully defined, since a minimal amount of follow-up data is available on the few cases which have been reported. Most patients appear to have been treated with a total abdominal hysterectomy and bilateral salpingo-oophorectomy, which was the
220
SELTZER ET AL.
FIG. 3. Cellular atypia with hobnail cells (250x).
FIG. 4. High-power detain of mucin-containing cells. This was mucicarmine positive (250x).
LMP
PAROVARIAN
CYSTADENOCARCINOMA
FIG. 5. Portion of tumor with psammoma bodies (100x ).
treatment utilized for our patient. We speculate that for a small subset of parovarian malignancies, which meet similar criteria to those which have been outlined regarding ovarian malignancies, surgery which conserves reproductive function may be appropriate. The expected prognosis for our patient is probably good. However, she will require appropriate surveillance over many years, since we expect that as is the case with low-malignant potential ovarian tumors, this lesion may have a propensity for late recurrence. REFERENCES I. Richart, R. M., Naus, G. J., and Seltzer, V. Diagnosing tumors of low malignant potential. Contemp. Obstet. Gynecol. 25, 143-158 (1985). 2. Duvall, G., and Survis, J. A. Borderline tumour of the broad ligament-case report, Brit. J. Obstet. Gynecol.90,
372-375 (1983).
3. Genadry, R., Parmley, T., and Woodruff, J. D. The origin and clinical behavior of the parovarian tumor, Amer. J. Obstet. Gynecol. 129, 873-880 (1977). 4. Yamazaki, K., Watanabe, Y., and Imagawa, K. Papillary tumor of the broad ligament, Acta Pathol.
Jpn. 33(5), 979-984 (1983).
5. Lennox, B.. and Meagher, D. J. Parovarian carcinoma, J. Obstet. Gynecol. 59, 783-785 (1952). 6. Nikrui. N. Survey of clinical behavior of patients with borderline epithelial tumors of the ovary, Gynecol.
Oncol. 12, 107-119 (1981).
7. Aure, J.. Hoeg, K., and Kolstad, P. Clinical and histologic studies of ovarian carcinoma, Obstet. Gynecol. 37, 1-9 (1971). 8. Katzenstein, A. A., Mazur, M. T.. and Morgan, T. E. Proliferative serous tumors of the ovary, Amer. J. Surg. Pathol. 2, 339-355 (1978).
ONCOLOGY
30, 2 16-22 1 (1988)
Parovarian Cystadenocarcinoma of Low-Malignant Potential VICKI L. SELTZER, M.D.,*.’ LAURA MOLHO, M.D.,f ARTHUR FOUGNER, PETER HONG, M.D.,* BEATA KERESZTI, M.D. ,* MELANIE GERO, M.D. MARK SPITZER, M.D.*
M.D.,* ,i AND
*Department of Obstetrics and Gynecology and TDepartment of Pathology, Queens Hospital Center, Afjliation of the Long Island Jewish Medical Center and the State University of New York at Stony Brook, Jamaica, New York 11432 Received September 8, 1986 In the world’s literature, only 16 patients with parovarian malignancies have been described, five of which have been of low-malignant potential. All but one of these low-malignant potential lesions have been serous tumors. This is the second low-malignant potential parovarian tumor to be described with mutinous and serous components as well as hobnail cells. These tumors occur almost exclusively in young women, yet they are so rare that no treatment plan has been developed. We propose that until more data becomes available, these low-malignant potential lesions be treated as are their ovarian counterparts with staging laparotomy and surgery which conserves reproductive functioning being appropriate for women who meet the rigid clinical and histologic criteria for a very early potentially nonaggressive tumor, and complete extirpative surgery for those who do not. o 1988Academic Press. Inc.
INTRODUCTION
Epithelial ovarian tumors of low-malignant potential are a well-defined histologic and biologic entity comprising approximately 15-25% of all epithelial cancers [l]. On the other hand, parovarian neoplasms are much less common than ovarian lesions, and there have been only five reports in the literature of parovarian tumors of low-malignant potential [2-41. These have been predominantly serous. This paper reports the sixth low-malignant potential parovarian tumor, and the second with mutinous and serous components as well as hobnail cells. CASE REPORT
The patient is a 44-year-old Hispanic female, G5, P 4-O-O-4,who was admitted to the hospital on February 4, 1985, following an uncomplicated antepartum course. She had a normal spontaneous delivery of a term male infant, Apgar 910, and was scheduled to undergo an elective tubal ligation the following day. The patient’s past medical history was significant for hypothyroidism of 9 years ’ To whom reprint requests should be addressed at Queens Hospital Center, 82-68 164th Street, Jamaica, NY 11432. 216 OO90-8258/B$1.50 Copyright All rights
0 1988 by Academic Press, Inc. of reproduction in any form reserved.
LMP
PAROVARIAN
CYSTADENOCARCINOMA
217
duration, for which she took Synthyroid 0.1 mg daily, a vein stripping 11 years prior to admission, and a penicillin allergy. On February 5, 1985 the patient was taken to the operating room for a bilateral tubal ligation. This was performed through an infraumbilical incision. The left fallopian tube was ligated using a modified Pomeroy procedure. The right fallopian tube could not be traced to the fimbria and was thought to be wrapped over a large mass. Laparotomy was then initiated through a vertical incision, and an 8 x 6.5 x 6-cm cystic mass was found in the right broad ligament. The ovaries on both sides were normal. Adhesions were lysed, and a right salpingectomy and removal of the mass was performed. Upon careful gross and histologic examination of the mass, it was found to be separate from the ovary and the fallopian tube, the latter being stretched over the mass. A histologic diagnosis of borderline parovarian tumor with serous, mutinous, and clear cell components was made. Therefore, the patient had a second laparotomy performed 5 weeks later, at which time a total abdominal hysterectomy, left salpingo-oophorectomy, right oophorectomy, partial omentectomy, and careful abdominal exploration with peritoneal washings and multiple biopsies were performed. There was no evidence of disease in these specimens. The patient was discharged from the hospital in satisfactory condition and remains well. PATHOLOGY
The right fallopian tube was found to be entirely normal, but stretched over a separate cystic parovarian mass measuring 8 x 6.5 x 6 cm, with which it did not communicate. The mass was also completely separate from the ovary. The outer surface of the cyst wall was smooth (Fig. 1). The inner surface contained multiple friable papillations and contained copious yellow mutinous material. There were two additional benign parovarian cysts, measuring 2.5 cm. and 1.5 cm. respectively, not communicating with the larger cyst, and each containing clear fluid. The large parovarian cyst was lined predominantly by columnar, focally ciliated cells and had many complex papillary projections extending into the lumen (Fig. 2). These were comprised of epithelial cells, some with a hobnail pattern and others containing mucin (Figs. 3 and 4). Psammoma bodies were present (Fig. 5). There was focal stratification as well as detached cellular tufts and cytologic atypia. Rare mitoses were present. Multiple sections were taken, failing to demonstrate invasion of the cyst wall. The cyst wall contained smooth muscle. All addition1 specimens (uterus, cervix, ovaries, fallopian tubes, liver biopsy, omentum, multiple intraperitoneal biopsies, and peritoneal washings) showed no evidence of disease. DISCUSSION
Parovarian cysts arise in the mesosalpinx, between the ovarian hilum and the fallopian tube. They may be of mesothelial, paramesonephric, or mesonephric origin [3]. Parovarian cysts of clinical significance are considerably less frequent than ovarian cysts, and malignant parovarian lesions are exceedingly rare. In a review of 140 parovarian lesions from the Gynecologic Pathology Laboratory at
218
SELTZER ET AL.
FIG. 1. External surface of main cyst with adjacent benign smaller cyst and fallopian tube stretched over the mass.
Johns Hopkins, only four malignancies were demonstrated, three of which were low-malignant potential serous lesions [3]. The first parovarian cancer was described by Lennox in 1952 [5]. Since that time, 16 parovarian malignancies have been reported, five of which have been predominantly serous low-malignant potential lesions. This is the second lowmalignant potential parovarian tumor ever described which contains mutinous and serous components as well as hobnail cells. Low-malignant potential tumors have a histologic appearance (and presumably a biologic behavior) between the clearly benign and frankly malignant lesions. Histologic criteria for these lesions include stratification of the epithelial lining of the papillae, formation of tufts arising from the papillae, nuclear and cellular atypia, epithelial pleomorphism, mitotic activity, and the absence of stromal invasion. Little is known about the biologic behavior of the low-malignant potential parovarian tumor, since these lesions are so rare. Therefore, the decision regarding therapy was extrapolated from our therapeutic regimen for low-malignant potential ovarian lesions. As many as 40% of patients who have LMP ovarian tumors may have disease which is more extensive than Stage IA [6-81. The E-year survival for women with borderline ovarian tumors has been reported to be 73%. Therefore, it is
LMP
FIG. 2. Detail tufts (100X).
of complex
PAROVARIAN
papillary
219
CYSTADENOCARCINOMA
projections
with
pseudostratification
and detached
cellular
essential that these women have complete staging laparotomies to make certain that the disease has not spread. For women with advanced disease or for those in whom reproductive potential is no longer desired a total abdominal hysterectomy and bilateral salpingo-oophorectomy is performed. However, most of these lesions are found in young women, the youngest reported patient being 13 [3]. Perhaps, as is the case with ovarian neoplasms, patients who meet certain clinical and histologic criteria can be treated with preservation of their reproductive function, if they strongly desire more children, and if they understand that no definite answers regarding therapy are available. If the parovarian lesion has no external excrescences nor adhesions and can be shelled out without rupture. if there is no ascites, if there are no other structures involved (as demonstrated by multiple biopsies and careful staging laparotomy), if the lesion is low grade, and if the woman is young, reliable and very interested in future fertility, it may be acceptable to treat her without a hysterectomy. If the woman does not meet every one of these criteria, or if she does not desire future fertility, more aggressive surgery is probably advisable. The appropriate therapy for this unusual lesion has not been fully defined, since a minimal amount of follow-up data is available on the few cases which have been reported. Most patients appear to have been treated with a total abdominal hysterectomy and bilateral salpingo-oophorectomy, which was the
220
SELTZER ET AL.
FIG. 3. Cellular atypia with hobnail cells (250x).
FIG. 4. High-power detain of mucin-containing cells. This was mucicarmine positive (250x).
LMP
PAROVARIAN
CYSTADENOCARCINOMA
FIG. 5. Portion of tumor with psammoma bodies (100x ).
treatment utilized for our patient. We speculate that for a small subset of parovarian malignancies, which meet similar criteria to those which have been outlined regarding ovarian malignancies, surgery which conserves reproductive function may be appropriate. The expected prognosis for our patient is probably good. However, she will require appropriate surveillance over many years, since we expect that as is the case with low-malignant potential ovarian tumors, this lesion may have a propensity for late recurrence. REFERENCES I. Richart, R. M., Naus, G. J., and Seltzer, V. Diagnosing tumors of low malignant potential. Contemp. Obstet. Gynecol. 25, 143-158 (1985). 2. Duvall, G., and Survis, J. A. Borderline tumour of the broad ligament-case report, Brit. J. Obstet. Gynecol.90,
372-375 (1983).
3. Genadry, R., Parmley, T., and Woodruff, J. D. The origin and clinical behavior of the parovarian tumor, Amer. J. Obstet. Gynecol. 129, 873-880 (1977). 4. Yamazaki, K., Watanabe, Y., and Imagawa, K. Papillary tumor of the broad ligament, Acta Pathol.
Jpn. 33(5), 979-984 (1983).
5. Lennox, B.. and Meagher, D. J. Parovarian carcinoma, J. Obstet. Gynecol. 59, 783-785 (1952). 6. Nikrui. N. Survey of clinical behavior of patients with borderline epithelial tumors of the ovary, Gynecol.
Oncol. 12, 107-119 (1981).
7. Aure, J.. Hoeg, K., and Kolstad, P. Clinical and histologic studies of ovarian carcinoma, Obstet. Gynecol. 37, 1-9 (1971). 8. Katzenstein, A. A., Mazur, M. T.. and Morgan, T. E. Proliferative serous tumors of the ovary, Amer. J. Surg. Pathol. 2, 339-355 (1978).