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Lymphoma of ovary with stromal luteinization, presenting as secondary amenorrhea
GYNECOLOGIC
ONCOLOGY
45, 69-75
(1992)
CASE REPORT Lymphoma of Ovary with Stromal Luteinization, Presenting as Secondary Amenorrhea KHUSHBAKHAT Department
RAI MITTAL,
ANDREW BLECHMAN,
of Pathology and Department of Obstetrics Stanley and Rita Kaplan Cancer Center,
M. ALBA GRECO,FLORESALFONSO, AND RITA DEMOP~ULOS
and Gynecology, New York New York University School
University Medical of Medicine, New
Center and Bellevue Hospital, York, New York 10016
and the
ReceivedSeptember 20, 1991 A case of primary lymphoma of the ovary with extensive stromalluteinization is presented.Immunohistochemical markers that work satisfactorily in fixed tissuesalloweda diagnosisof B cell lymphoma to be madeon formalin-fixed tissuesections.Immunohistochemical analysisof frozen tissueand genotypic analysis confirmed the diagnosisof B cell lymphoma. By electron microscopy two populations of cells, including large atypical lymphoid cellsand luteinized stromalcells,were identified. Presentation with amenorrheaand resumptionof menstrualperiods after removal of the tumor suggested the possibilityof functional activity in the luteinized stromalcells. Stromal luteinization has not been previously describedin primary ovarian lymphoma. 0 WI2 Academic
Press, Inc.
INTRODUCTION Lymphoma rarely presents as primary ovarian tumor, with less than 50 well-documented cases reported [l]. Stromal luteinization is a frequent finding in metastatic carcinoma to ovary [2] and has been known to occur in many other ovarian tumors [3]. Stromal luteinization has not been reported in primary lymphoma of ovary [4-61. We report a case of lymphoma of ovary with prominent stromal luteinization, presenting as secondary amenorrhea. CASE REPORT The patient is a 31-year-old para 1031, gravida 4 woman who was being followed for secondary amenorrhea of 2 year duration. A right adnexal mass was detected on physical examination. The tumor appeared solid and confined to the right ovary on pelvic ultrasound and a CT scan. No lymph node, liver, or spleen enlargement was seen. The serum estradiol, prolactin, follicle-stimulating
hormone, luteinizing hormone, and /+HCG levels were in the normal range. A right salpingo-oophorectomy and omentectomy were performed. At surgery, the left fallopian tube, the uterus, and the omentum were of normal appearance. The left ovary was small and atrophic without evidence of functional cysts. Following a diagnosis of lymphoma the patient underwent a staging workup for possible extraovarian disease. This included an upper G.I. series, barium enema, bone marrow biopsy, and CT scan of chest, abdomen, and pelvis. There was no evidence of extraovarian disease. The patient’s HIV status was negative. Postoperatively the patient received chemotherapy consisting of Adriamycin, vincristine, cytoxan, and prednisone. The patient resumed normal menstrual periods 2 months after removal of the tumor. She is disease free at a follow-up period of 5 months. Gross findings. The right ovary was completely replaced by a solid, lobulated, encapsulated, and firm tumor, measuring 14 x 10 x 6 cm and weighing 460 g. The cut surface was variegated light grayish-white with scattered foci of fresh hemorrhage (Fig. 1). The right fallopian tube and the omentum were not involved by the tumor. Microscopic findings. The tumor was composed of large cells with markedly irregular and cleaved as well as noncleaved nuclei (Fig. 2). The cytoplasm was lightly eosinophillic. Mitotic activity averaged 1 mitosis/HPF. The cells in most areas were arranged in nodules separated by fibrous septae (Fig. 3.). In some areas tumor cells were arranged in a diffuse pattern (Fig. 3). A lymphoplasmacytic infiltrate was present in the fibrous septae and in the tumor itself (Fig. 4). Although occasional bior trinucleate tumor cells were seen, multinucleated cells
69
GIl90-8258/92$1.50 Copyright 0 1992byAcademic Press, Inc. All rightsof reproduction in anyformreserved.
70
MI-I-I-AL ET AL.
nuclei. Crystalloids of Reinke were not seen. The luteinized stromal cells or edema was not present in small residual ovarian tissue that was uninvolved by the tumor. The left fallopian tube and the omentum were negative for tumor on microscopic examination. Immunohistochemical studies. A PAS stain was negative in tumor cells. A methyl green-pyronin stain was weakly positive in tumor cells. Immunoperoxidase
FIG. 1. Gross appearance of the cut surface of the tumor showing a variegated appearance.
of syncytiotrophoblastic type were not seen. A prominent feature was the presence of nests of luteinized stromal cells (Figs. 5a and 5b) throughout the tumor. The nests of luteinized stromal cells were usually physically separated from the tumor cells and were located in edematous areas present amidst the tumor. These stromal cells had abundant eisinophillic cytoplasm and small uniform round
studies on formalin-@ed
tissue.
The tumor cells were positive for leukocyte common antigen (LCA) and for L-26 (Fig. 6). L-26 is a pan-B cell marker that works satisfactorily in formalin-fixed tissues [7]. No immunoreactivity for UCHL (a pan-T cell marker), cytokeratin, vimentin, HCG, and S-100 was seen in the tumor cells. Reactive lymphocytic infiltrate in the tumor stained for LCA, L-26, and UCHL. The luteinized stromal cells stained only for vimentin. No testosterone immunoreactivity was seen in the luteinized stromal cells on an immunoperoxidase stain for testosterone. Zmmunoperoxidase studies on frozen tissue. Cell marker analysis was performed on snap-frozen tissue. The malignant cells stained for leukocyte common antigen and pan-B markers (BL9 and Bl). The small lymphocytes were predominantly T cells. Gene rearrangement studies. Gene rearrangement studies were performed on DNA extracted from frozen tissue. The DNA was cut in all instances with three restriction enzymes (EcoRI, BarnI, and HindIII) and in turn
FIG. 2. Light microscopic appearance of the tumor cells, showing markedly pleomorphic convoluted nuclei (hematoxylin and eosin,
x
400).
71
CASE REPORT
FIG.
3. Low-power
light microscopic appearance of the tumor, showing a mixed nodular and diffuse pattern (hematoxylin and eosin,
hybric lized to Jh, Jk, c-myc, and Bei-2 probes (Oncor, MD). sou lthern blot analysis showed gene rearrangements of immu noglobulin gene heavy and light chains (Jh and Jk)
x
20).
and rearrangement of c-myc gene. The B&2 gene was in its germline configuration. These results established the monoclonality of this tumor and confirmed the im munophenotypic diagnosis of B cell lymphoma.
FIG. 4. A lymphoplasmacytic infiltrate was seen in the fibrous septae separating the tumor cells (hematoxylin and eosin, X2@)).
72
x
MIT-I-AL
ET AL.
FIG. 5. Nests of luteinized stromal cells, with abundant cytoplasm and round uniform nuclei (a, top; b, top right) (hematoxylin and eosin, 200 and x 400).
Ultrastructural findings. Electron microscopic studies showed tumor cells with convuluted nuclei, a few mitochondria, short strands of rough endoplasmic reticulum, and abundant ribosomes. Some tumor cells contained lipid droplets. The cell membranes were irregular with many projections. No intercellular junctions were seen (Fig. 7). The stromal cells had abundant lipid droplets
and many mitochondria (Fig. 8). On the basis of the light microscopic, immunohistochemical, immunoperoxidase, and ultrastructural findings a diagnosis of lymphoma was made. The lymphoma was classified as malignant lymphoma, follicular, predominantly large cell type, with diffuse areas. The luteinized stromal cells were felt to be reactive in origin.
73
CASE REPORT
FIG. 6. Immunoperoxidase
staining for L-26, showing positive staining in tumor cells. The cystic follicle to the left is negative (x200).
DISCUSSION Although malignant lymphoma is seen to involve the ovaries in up to 25% of cases at autopsy [5], less than 50 well-documented cases of lymphoma presenting as an ovarian tumor are on record [l]. We were able to characterize the case presented here as a B cell lymphoma with the newly available antibodies that work well in formaldehyde-fixed tissues [7].
FIG.
Stromal luteinization is usually seen in cases with primary common epithelial tumors of the ovary, with metastatic carcinoma, and less commonly with germ cell tumors such as dysgerminoma [8-lo]. A marked degree of stromal luteinization can be seen in malignant lymphoma of the ovary, as exemplified by the case presented here. Recently, stromal luteinization was briefly described and illustrated in a case of lymphoma that relapsed in the ovary [ 111. If an ovarian tumor
7. Electron microscopic appearance of the tumor cells, showing a lack of intercellular
hridges (x 12,600).
MITTAL
ET AL.
FIG. 8. Electron microscopic appearance of the stromal cells, showing numerous lipid droplets in the cytoplasm (x98.50).
shows a component of the luteinized cells or cells resembling interstitial cells of the testes, possibility of lymphoma should be considered in the differential diagnosis. Because of the architecture of the tumor under low-power microscope, dysgerminoma was considered in the differential diagnosis in this case. Differentiation of lymphoma from dysgerminoma can be difficult based on light microscopic features alone. Markedly convoluted nuclei, as seen in the present case, should suggest a diagnosis of lymphoma. Dysgerminoma usually has relatively uniform round nuclei, although marked nuclear pleomorphism may be seen in the anaplastic variant [12]. Syncytiotrophoblastic giant cells and noncaseating granulomas are present in a small minority of dysgerminomas, but are not seen in lymphoma of ovary. Use of immunohistochemical, immunoperoxidase, and ultrastructural studies allows a clear distinction between these two tumors to be made. The mechanism by which stromal luteinization occurred in the present case is unclear. Possible mechanisms include physical presence of the tumor cells in close proximity to the stromal cells or biochemical changes that induce stromal luteinization [2,3]. Since luteinized stromal cells were usually physically separated from the lymphoma cells, -a biochemical rather than a physical change appears to be responsible for stromal luteinization in this case. This biochemical stimulus may take the form of luteinizing hormone- or gonadotropin-like substances, or may be a nonspecific change caused by the presence of a tumor in the vicinity. The serum luteinizing hormone levels were normal in this patient, and no immunoreactive p-human chorionic gonadotropin was observed in the tumor cells. Clusters of luteinized stromal cells have been
described in over one-third of cases of massive ovarian edema (13). The luteinized stromal cells in the presently reported case were almost always in the edematous stromal areas, suggesting a pathogenesis similar to that seen in massive ovarian edema. Edema and stromal luteinization were not seen in small areas of ovarian tissue uninvolved by the tumor. The foci of stromal luteinization in this case may be a consequence of local ischemia and stromal edema caused by the presence of a tumor in the vicinity. Presence of amenorrhea prior to surgery, the atrophic appearance of the left ovary, and resumption of menstrual cycles after surgery suggests the possibility of androgenic functional activity in the luteinized stromal cells. Although no immunoreactivity to testosterone was seen on an immunoperoxidase stain, this does not rule out the production of other androgenic hormones or precursors. Rare cases in which lymphoma of ovary has been apparently cured by surgery alone show that lymphoma can be a primary tumor in the ovary [14]. However, 5-year survival for lymphoma confined to ovary at presentation is in the 60% range [4]. Clearly, at least a third of the patients presenting with lymphoma apparently confined to an ovary have undetected tumor outside of the ovary at the time of presentation. Lymphoma of ovary should be treated with systemic chemotherapy even if spread beyond the ovary is not detected by the staging procedures [14]. Reported 5-year survival is 33% when both ovaries are involved and 23% when disease has spread beyond the ovary [4]. Fox et al. [14] found B cell type, slow onset of symptoms, absence of systemic disease, low-stage disease, and unilateral ovarian involvement to be favorable prognostic indicators for
CASE REPORT
lymphoma of ovary, all of which characterized tient’s tumor.
this pa7.
ACKNOWLEDGMENT We are thankful to Maria Mittal for preparing this manuscript.
8.
REFERENCES 9. Talerman, A. Nonspecific tumors of the ovary, in Blaustein’s pathology of the female genital tract (R. B. Kurman, Ed.), SpringerVerlag, New York, 3rd ed., pp. 733-736 (1987). Scully, R. E., and Richardson, G. S. Luteinization of the stroma of metastatic cancer involving the ovary and its endocrine significance, Cancer 14, 827-840(1961). Scully, R. E. Ovarian tumors with functioning stroma, in Haines and Taylor’s gynecological obstetrical pathology (H. Fox, Ed.), Churchill Livingstone, Edinburgh, 3rd ed., (1983). Osborne, B. M., and Robboy, S. J. Lymphomas or leukemia presenting as ovarian tumors. Cancer 52, 1933-1943 (1983). 5. Charlton, I., Norris, H. J., and King, F. M. Malignant reticuloendothelial disease involving the ovary as a primary manifestation: A series of 19 lymphomas and 1 granulocytic sarcoma, Cancer 34, 397-407 (1974). 6. Paladugu, R. R., Bearman, R. M., and Rappaport, H., Malignant
10.
11.
12. 13. 14.
75
lymphoma with primary manifestation in the gonad: A clinicopathologic study of 38 patients, Cancer 45, 561-571 (1980). Davey, F. R., Elghetany, M. T., and Kurec, A. S. Immunophenotyping of hematologic neoplasms in paraffin-embedded tissue sections, Am. .I. Clin. Pathol. 93, 517-526 (1990). Scully, R. E. Tumors of the ovary and maldeveloped gonads, in Atlas of tumor pathology, Armed Forces Institute of Pathology, Washington, DC, fascicle 16, 2nd series, pp. 353-363 (1979). Lash, R. H., and Hart, W. R. Intestinal adenocarcinoma metastatic to the ovaries. A clinicopathologic evaluation of 22 cases, Am. J. Surg. Pathol. 11, 114-121 (1987). Ulbright, T. M., Roth, L. M., and Stehman, F. B. Secondary ovarian neoplasia. A clinicopathologic study of 35 cases, Cancer 53, 1164-1174 (1984). Ferry, J. A., and Young, R. H. Malignant lymphoma, pseudolymphoma and hematopoietic disorders of the female genital tract (review), Pathol. Ann. 26(l), 227-263 (1991). Gillepsie, J. J., and Arnold, L. K. Anaplastic dysgerminoma, Cancer 42, 1886-1889 (1978). Roth, L. M., Deaton, R. L., and Sternberg, W. H. Massive ovarian oedema, Am. J. Surg. Pathol. 3, 11-21 (1979). Fox, H., Langley, F. A., Govan, A. D. T., Hills, A. S., and Bennett, M. H. Malignant lymphoma presenting as an ovarian tumor: A clinicopathological analysis of 34 cases, Br. J. Obstet. Gynaecol. 95, 386-390 (1988).
ONCOLOGY
45, 69-75
(1992)
CASE REPORT Lymphoma of Ovary with Stromal Luteinization, Presenting as Secondary Amenorrhea KHUSHBAKHAT Department
RAI MITTAL,
ANDREW BLECHMAN,
of Pathology and Department of Obstetrics Stanley and Rita Kaplan Cancer Center,
M. ALBA GRECO,FLORESALFONSO, AND RITA DEMOP~ULOS
and Gynecology, New York New York University School
University Medical of Medicine, New
Center and Bellevue Hospital, York, New York 10016
and the
ReceivedSeptember 20, 1991 A case of primary lymphoma of the ovary with extensive stromalluteinization is presented.Immunohistochemical markers that work satisfactorily in fixed tissuesalloweda diagnosisof B cell lymphoma to be madeon formalin-fixed tissuesections.Immunohistochemical analysisof frozen tissueand genotypic analysis confirmed the diagnosisof B cell lymphoma. By electron microscopy two populations of cells, including large atypical lymphoid cellsand luteinized stromalcells,were identified. Presentation with amenorrheaand resumptionof menstrualperiods after removal of the tumor suggested the possibilityof functional activity in the luteinized stromalcells. Stromal luteinization has not been previously describedin primary ovarian lymphoma. 0 WI2 Academic
Press, Inc.
INTRODUCTION Lymphoma rarely presents as primary ovarian tumor, with less than 50 well-documented cases reported [l]. Stromal luteinization is a frequent finding in metastatic carcinoma to ovary [2] and has been known to occur in many other ovarian tumors [3]. Stromal luteinization has not been reported in primary lymphoma of ovary [4-61. We report a case of lymphoma of ovary with prominent stromal luteinization, presenting as secondary amenorrhea. CASE REPORT The patient is a 31-year-old para 1031, gravida 4 woman who was being followed for secondary amenorrhea of 2 year duration. A right adnexal mass was detected on physical examination. The tumor appeared solid and confined to the right ovary on pelvic ultrasound and a CT scan. No lymph node, liver, or spleen enlargement was seen. The serum estradiol, prolactin, follicle-stimulating
hormone, luteinizing hormone, and /+HCG levels were in the normal range. A right salpingo-oophorectomy and omentectomy were performed. At surgery, the left fallopian tube, the uterus, and the omentum were of normal appearance. The left ovary was small and atrophic without evidence of functional cysts. Following a diagnosis of lymphoma the patient underwent a staging workup for possible extraovarian disease. This included an upper G.I. series, barium enema, bone marrow biopsy, and CT scan of chest, abdomen, and pelvis. There was no evidence of extraovarian disease. The patient’s HIV status was negative. Postoperatively the patient received chemotherapy consisting of Adriamycin, vincristine, cytoxan, and prednisone. The patient resumed normal menstrual periods 2 months after removal of the tumor. She is disease free at a follow-up period of 5 months. Gross findings. The right ovary was completely replaced by a solid, lobulated, encapsulated, and firm tumor, measuring 14 x 10 x 6 cm and weighing 460 g. The cut surface was variegated light grayish-white with scattered foci of fresh hemorrhage (Fig. 1). The right fallopian tube and the omentum were not involved by the tumor. Microscopic findings. The tumor was composed of large cells with markedly irregular and cleaved as well as noncleaved nuclei (Fig. 2). The cytoplasm was lightly eosinophillic. Mitotic activity averaged 1 mitosis/HPF. The cells in most areas were arranged in nodules separated by fibrous septae (Fig. 3.). In some areas tumor cells were arranged in a diffuse pattern (Fig. 3). A lymphoplasmacytic infiltrate was present in the fibrous septae and in the tumor itself (Fig. 4). Although occasional bior trinucleate tumor cells were seen, multinucleated cells
69
GIl90-8258/92$1.50 Copyright 0 1992byAcademic Press, Inc. All rightsof reproduction in anyformreserved.
70
MI-I-I-AL ET AL.
nuclei. Crystalloids of Reinke were not seen. The luteinized stromal cells or edema was not present in small residual ovarian tissue that was uninvolved by the tumor. The left fallopian tube and the omentum were negative for tumor on microscopic examination. Immunohistochemical studies. A PAS stain was negative in tumor cells. A methyl green-pyronin stain was weakly positive in tumor cells. Immunoperoxidase
FIG. 1. Gross appearance of the cut surface of the tumor showing a variegated appearance.
of syncytiotrophoblastic type were not seen. A prominent feature was the presence of nests of luteinized stromal cells (Figs. 5a and 5b) throughout the tumor. The nests of luteinized stromal cells were usually physically separated from the tumor cells and were located in edematous areas present amidst the tumor. These stromal cells had abundant eisinophillic cytoplasm and small uniform round
studies on formalin-@ed
tissue.
The tumor cells were positive for leukocyte common antigen (LCA) and for L-26 (Fig. 6). L-26 is a pan-B cell marker that works satisfactorily in formalin-fixed tissues [7]. No immunoreactivity for UCHL (a pan-T cell marker), cytokeratin, vimentin, HCG, and S-100 was seen in the tumor cells. Reactive lymphocytic infiltrate in the tumor stained for LCA, L-26, and UCHL. The luteinized stromal cells stained only for vimentin. No testosterone immunoreactivity was seen in the luteinized stromal cells on an immunoperoxidase stain for testosterone. Zmmunoperoxidase studies on frozen tissue. Cell marker analysis was performed on snap-frozen tissue. The malignant cells stained for leukocyte common antigen and pan-B markers (BL9 and Bl). The small lymphocytes were predominantly T cells. Gene rearrangement studies. Gene rearrangement studies were performed on DNA extracted from frozen tissue. The DNA was cut in all instances with three restriction enzymes (EcoRI, BarnI, and HindIII) and in turn
FIG. 2. Light microscopic appearance of the tumor cells, showing markedly pleomorphic convoluted nuclei (hematoxylin and eosin,
x
400).
71
CASE REPORT
FIG.
3. Low-power
light microscopic appearance of the tumor, showing a mixed nodular and diffuse pattern (hematoxylin and eosin,
hybric lized to Jh, Jk, c-myc, and Bei-2 probes (Oncor, MD). sou lthern blot analysis showed gene rearrangements of immu noglobulin gene heavy and light chains (Jh and Jk)
x
20).
and rearrangement of c-myc gene. The B&2 gene was in its germline configuration. These results established the monoclonality of this tumor and confirmed the im munophenotypic diagnosis of B cell lymphoma.
FIG. 4. A lymphoplasmacytic infiltrate was seen in the fibrous septae separating the tumor cells (hematoxylin and eosin, X2@)).
72
x
MIT-I-AL
ET AL.
FIG. 5. Nests of luteinized stromal cells, with abundant cytoplasm and round uniform nuclei (a, top; b, top right) (hematoxylin and eosin, 200 and x 400).
Ultrastructural findings. Electron microscopic studies showed tumor cells with convuluted nuclei, a few mitochondria, short strands of rough endoplasmic reticulum, and abundant ribosomes. Some tumor cells contained lipid droplets. The cell membranes were irregular with many projections. No intercellular junctions were seen (Fig. 7). The stromal cells had abundant lipid droplets
and many mitochondria (Fig. 8). On the basis of the light microscopic, immunohistochemical, immunoperoxidase, and ultrastructural findings a diagnosis of lymphoma was made. The lymphoma was classified as malignant lymphoma, follicular, predominantly large cell type, with diffuse areas. The luteinized stromal cells were felt to be reactive in origin.
73
CASE REPORT
FIG. 6. Immunoperoxidase
staining for L-26, showing positive staining in tumor cells. The cystic follicle to the left is negative (x200).
DISCUSSION Although malignant lymphoma is seen to involve the ovaries in up to 25% of cases at autopsy [5], less than 50 well-documented cases of lymphoma presenting as an ovarian tumor are on record [l]. We were able to characterize the case presented here as a B cell lymphoma with the newly available antibodies that work well in formaldehyde-fixed tissues [7].
FIG.
Stromal luteinization is usually seen in cases with primary common epithelial tumors of the ovary, with metastatic carcinoma, and less commonly with germ cell tumors such as dysgerminoma [8-lo]. A marked degree of stromal luteinization can be seen in malignant lymphoma of the ovary, as exemplified by the case presented here. Recently, stromal luteinization was briefly described and illustrated in a case of lymphoma that relapsed in the ovary [ 111. If an ovarian tumor
7. Electron microscopic appearance of the tumor cells, showing a lack of intercellular
hridges (x 12,600).
MITTAL
ET AL.
FIG. 8. Electron microscopic appearance of the stromal cells, showing numerous lipid droplets in the cytoplasm (x98.50).
shows a component of the luteinized cells or cells resembling interstitial cells of the testes, possibility of lymphoma should be considered in the differential diagnosis. Because of the architecture of the tumor under low-power microscope, dysgerminoma was considered in the differential diagnosis in this case. Differentiation of lymphoma from dysgerminoma can be difficult based on light microscopic features alone. Markedly convoluted nuclei, as seen in the present case, should suggest a diagnosis of lymphoma. Dysgerminoma usually has relatively uniform round nuclei, although marked nuclear pleomorphism may be seen in the anaplastic variant [12]. Syncytiotrophoblastic giant cells and noncaseating granulomas are present in a small minority of dysgerminomas, but are not seen in lymphoma of ovary. Use of immunohistochemical, immunoperoxidase, and ultrastructural studies allows a clear distinction between these two tumors to be made. The mechanism by which stromal luteinization occurred in the present case is unclear. Possible mechanisms include physical presence of the tumor cells in close proximity to the stromal cells or biochemical changes that induce stromal luteinization [2,3]. Since luteinized stromal cells were usually physically separated from the lymphoma cells, -a biochemical rather than a physical change appears to be responsible for stromal luteinization in this case. This biochemical stimulus may take the form of luteinizing hormone- or gonadotropin-like substances, or may be a nonspecific change caused by the presence of a tumor in the vicinity. The serum luteinizing hormone levels were normal in this patient, and no immunoreactive p-human chorionic gonadotropin was observed in the tumor cells. Clusters of luteinized stromal cells have been
described in over one-third of cases of massive ovarian edema (13). The luteinized stromal cells in the presently reported case were almost always in the edematous stromal areas, suggesting a pathogenesis similar to that seen in massive ovarian edema. Edema and stromal luteinization were not seen in small areas of ovarian tissue uninvolved by the tumor. The foci of stromal luteinization in this case may be a consequence of local ischemia and stromal edema caused by the presence of a tumor in the vicinity. Presence of amenorrhea prior to surgery, the atrophic appearance of the left ovary, and resumption of menstrual cycles after surgery suggests the possibility of androgenic functional activity in the luteinized stromal cells. Although no immunoreactivity to testosterone was seen on an immunoperoxidase stain, this does not rule out the production of other androgenic hormones or precursors. Rare cases in which lymphoma of ovary has been apparently cured by surgery alone show that lymphoma can be a primary tumor in the ovary [14]. However, 5-year survival for lymphoma confined to ovary at presentation is in the 60% range [4]. Clearly, at least a third of the patients presenting with lymphoma apparently confined to an ovary have undetected tumor outside of the ovary at the time of presentation. Lymphoma of ovary should be treated with systemic chemotherapy even if spread beyond the ovary is not detected by the staging procedures [14]. Reported 5-year survival is 33% when both ovaries are involved and 23% when disease has spread beyond the ovary [4]. Fox et al. [14] found B cell type, slow onset of symptoms, absence of systemic disease, low-stage disease, and unilateral ovarian involvement to be favorable prognostic indicators for
CASE REPORT
lymphoma of ovary, all of which characterized tient’s tumor.
this pa7.
ACKNOWLEDGMENT We are thankful to Maria Mittal for preparing this manuscript.
8.
REFERENCES 9. Talerman, A. Nonspecific tumors of the ovary, in Blaustein’s pathology of the female genital tract (R. B. Kurman, Ed.), SpringerVerlag, New York, 3rd ed., pp. 733-736 (1987). Scully, R. E., and Richardson, G. S. Luteinization of the stroma of metastatic cancer involving the ovary and its endocrine significance, Cancer 14, 827-840(1961). Scully, R. E. Ovarian tumors with functioning stroma, in Haines and Taylor’s gynecological obstetrical pathology (H. Fox, Ed.), Churchill Livingstone, Edinburgh, 3rd ed., (1983). Osborne, B. M., and Robboy, S. J. Lymphomas or leukemia presenting as ovarian tumors. Cancer 52, 1933-1943 (1983). 5. Charlton, I., Norris, H. J., and King, F. M. Malignant reticuloendothelial disease involving the ovary as a primary manifestation: A series of 19 lymphomas and 1 granulocytic sarcoma, Cancer 34, 397-407 (1974). 6. Paladugu, R. R., Bearman, R. M., and Rappaport, H., Malignant
10.
11.
12. 13. 14.
75
lymphoma with primary manifestation in the gonad: A clinicopathologic study of 38 patients, Cancer 45, 561-571 (1980). Davey, F. R., Elghetany, M. T., and Kurec, A. S. Immunophenotyping of hematologic neoplasms in paraffin-embedded tissue sections, Am. .I. Clin. Pathol. 93, 517-526 (1990). Scully, R. E. Tumors of the ovary and maldeveloped gonads, in Atlas of tumor pathology, Armed Forces Institute of Pathology, Washington, DC, fascicle 16, 2nd series, pp. 353-363 (1979). Lash, R. H., and Hart, W. R. Intestinal adenocarcinoma metastatic to the ovaries. A clinicopathologic evaluation of 22 cases, Am. J. Surg. Pathol. 11, 114-121 (1987). Ulbright, T. M., Roth, L. M., and Stehman, F. B. Secondary ovarian neoplasia. A clinicopathologic study of 35 cases, Cancer 53, 1164-1174 (1984). Ferry, J. A., and Young, R. H. Malignant lymphoma, pseudolymphoma and hematopoietic disorders of the female genital tract (review), Pathol. Ann. 26(l), 227-263 (1991). Gillepsie, J. J., and Arnold, L. K. Anaplastic dysgerminoma, Cancer 42, 1886-1889 (1978). Roth, L. M., Deaton, R. L., and Sternberg, W. H. Massive ovarian oedema, Am. J. Surg. Pathol. 3, 11-21 (1979). Fox, H., Langley, F. A., Govan, A. D. T., Hills, A. S., and Bennett, M. H. Malignant lymphoma presenting as an ovarian tumor: A clinicopathological analysis of 34 cases, Br. J. Obstet. Gynaecol. 95, 386-390 (1988).