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Solid ovarian teratoma with peritoneal and abdominal wall implants, progressive in vivo maturation, and probable cure
GYNECOLOGIC
ONCOLOGY
3, 308-3 I3 (1975)
Solid Ovarian Teratoma with Peritoneal and Abdominal Wall Implants, Progressive in vivo Maturation, and Probable Cure FRED BENJAMIN, M.D., AND EDWARDA RORAT, M.D. The Department of Obstetrics 8s Gynecology und Pathology, Queens Hospital Center Afiliate of the Long Islund Jeivish-Hillside Medical Center, Jamaica, New York, and the State University of New York crt Stony Brook Received July 28, 1975 Progressive in viva maturation from immature to mature tissue occurred in a case of solid ovarian tumor with peritoneal and abdominal wall implants. Apparent cure occurred after conservative surgery without irradiation or chemotherapy. The history and therapy of such tumors is discussed.
Despite peritoneal implants and subsequent recurrence in the pelvis and abdominal wall, progressive maturation of a solid ovarian tumor occurred with ultimate spontaneous cure. The surgery was conservative and incomplete, and no radiotherapy or chemotherapy was administered. This case is of interest both from the standpoint of the therapy and the biology of such tumors. CASE REPORT First Admission
V. T., a 16-yr-old, unmarried black female, Para 0, was admitted to Queens Hospital Center on July 27, 1966, because of a large (approximately lo-cm diameter) left adnexal mass. This was considered to be an ovarian tumor. At laparotomy, although the uterus and right tube and ovary were normal, the left ovary was completely replaced by a tumor, predominantly solid with a small cystic area, and a left salpingo-ooporectomy was done. No free fluid nor gross metastases were noted. The right ovary was not biopsied. Pathology. Grossly (Fig. l), the primary tumor, measuring 12 X 9 X 7 cm was a completely encapsulated predominantly solid mass, with a cystic space, containing hair and sebaceous material. The solid portion was polypoid and cauliflowerlike in appearance, soft and focally spongy in consistency. Microscopically, sections of the primary solid mass revealed predominantly mature tissues with foci of embryonal neuroepithelial rosettes (Fig. 2), with nuclear atypism and mitotic activity, resembling neuroblastoma. Other areas revealed malignant mesenchyme and foci of necrosis. The mature elements, including the cyst, revealed typical benign teratoma. This mass was graded 2 out of 3, according to the grading of Thurlbeck and Scully [ 11. 308 Copyright 0 1975 by Academic Press, Inc. All rights of reproduction in any form reserved.
SOLID OVARIAN
TERATOMA
309
FIG. 1. Partially opened solid ovarian teratoma, composed predominantly of polypoid mass. Lower quadrant shows cyst, filled with matted hair.
Second Admission
The patient returned to the hospital 1 yr later (July 1967) complaining of a mass in the surgical scar. There was a 4 X 5 X 3-cm hard mass in the center of the Pfannensteil incision, somewhat attached to the symphysis pubis. The mass
310
BENJAMIN
FIG. 2. Embryonal
neuroepithelial
AND
RORAT
rosettes with numerous mitoses (X 250).
was enucleated locally, the peritoneal cavity not opened. The patient was discharged without further therapy. Pathology. The specimen, removed from the surgical scar, was grossly predominantly solid, interspersed with small cysts. Microscopically, the tumor was composed of the same type of tissue as the first specimen, but the immature neuroeipthelium and mesenchyme were more sparse.
SOLID
OVARIAN
TERATOMA
311
Third Admission Fourteen months later (September 1968), the patient presented again with a 6 x 8 X S-cm mass occupying most of the right half of the Pfannensteil incision and extending towards the symphysis pubis, the mons pubis, and into the deeper planes of the abdominal wall.. Pelvic examination was negative except for a small fixed mass in the culdesac. IVP, cystoscopy, bone survey, barium enema, and serum chemistries did not reveal evidence of distant metastases. The abdomen and pelvis were explored. A mass, which extended to the symphysis pubis and the urinary bladder, was removed together with a flap of urinary bladder. Some small implants were found and excised from the omentum, right ovary, and right mesosalpinx. A fixed mass that could not be removed was found in the culdesac. A biopsy of this mass was taken. Following an uneventful recovery the patient was discharged without further therapy. Pathology. All the aforementioned biopsies revealed the same picture microscopically, i.e., exclusively mature elements: epidermis with skin appendages (Fig. 3), intestinal mucosa, cartilage, respiratory epithelium, adipose tissue, and mature glial tissue. No immature or malignant elements were identified. Eight-Year Follow-up Following her last operation in 1968, the patient was repeatedly examined and found to be in excellent health and menstruating normally. The last physical examination in December 1974 revealed a woman in excellent health who was attending college. The pelvic examination was normal: the uterus was anteverted, normal in size and mobile; the adnexa were not palpable or tender; there was no residual palpable tumor in the culdesac. COMMENT
The reported incidence of solid teratomas of the ovary has been 2 : 1000 of all ovarian tumors [ 11. These lesions are predominantly solid in consistency, containing varying amounts of completely mature and/or embryonal tissue. Therefore, they have been graded according to the quantity of embryo& tissue found in each: Grades O-3 [ 11. The lower the grade, the better the prognosis. There have been several case reports of solid ovarian teratomas, with peritoneal implants composed predominantly or entirely of glial tissue, who survived [2]. Such reports indicate that the presence of metastases, when composed of glial tissue, does not necessarily mean a poor prognosis and that in such cases conservative treatment may be sufficient. The present case illustrates the following: (a) The primary tumor in 1966 was composed of moderate quantities of embryonal tissue (Grade 2). Successive biopsies from 1966 to 1968 revealed progressive maturation of the tumor tissue. (b) The final biopsies showed totally mature metastatic implants composed of neuroepithelium, epidermis with skin appendages, intestinal mucosa, respiratory epithelium, and cartilage. Other cases in the literature [2] revealed only glial tissue in implants seen at follow-up. (c) The inoperable mass in the culdesac noted in 1968 had completely regressed in 1973 without any treatment. (d) The patient was alive and well and, as far as can be determined, free of disease, 8 yr
312
BENJAMIN
AND
FIG. 3. Omental implant, composed of keratinizing tive tissue, with four sweat glands (X25).
RORAT
squamous epithelium
with underlying
connec-
after the first appearance of the tumor and 6 yr after the occurrence of metastases. It is not possible to know whether the implants were first immature like the primary tumor and then matured or whether mature tissue was transferred through a possible microscopic defect in the capsule of the primary tumor.
SOLID OVARIAN
TERATOMA
313
The progressive in viva maturation of the tumor and the regression of the culdesac implant raise interesting questions about the biology of these tumors. There is an interesting and growing literature on the remarkable phenomenon of in vivo maturation, and hence cure, of neuroblastoma [3, 41. This tendency for spontaneous growth control in this type of tumor seems to have been activated in some instances by partial or complete removal of the primary tumor [ 5, 61. The world literature now contains a number of well-documented cases of spontaneous regression of cancer [7]. Although the most frequent one is neuroblastoma, spontaneous regression of other cancers, especially hypernephroma and choriocarcinoma, has also been documented [7]. Possible factors that may be responsible for progressive maturation and spontaneous regression of cancer include: endocrine influences, unusual sensitivity to usually inadequate therapy, fever and/or infection, allergic or immune reactions, interference with maturation of the tumor, and removal of the carcinogenic agent. The case reported here raises questions about the management of solid ovarian teratomas. Treatment of such tumors with mature implants in the peritoneum has varied from simple removal of the involved ovary to radical surgery, followed by postoperative irradiation or chemotherapy. This case, as well as others in the literature [ 21, suggests that, particularly if glial tissue or any mature tissue is involved, it may be justified to spare the contralateral ovary (the tumor is usually unilateral) in a young woman and that no therapy is indicated for mature peritoneal implants. REFERENCES 1. Thurlbeck, W. M., and Scully, R. E. Solid teratoma of the ovary: A Clinicopathological analysis of 9 cases, Cancer 13, 804 (1960). 2. Robboy, S. J., and Scully, R. E. Ovarian teratoma with glial implants in the peritoneum: An analyses of 12 cases, Hum. Pathol. 1, 643 (1970). 3. Gross, R. E., Farber, S., and Martin, L. W. Neuroblastoma sympatheticum, pediatrics 23, 1179 (1959). 4. Former, J., Nicastri, A., and Murphy, M. L. Neuroblastoma: natural history and results of treating 133 cases, Ann. Surg. 167, 132 (1968). 5. Koop, C. E., and Hemandez, J. R. Neuroblastoma: experience with 100 cases in children, Surgery 56, 726 (1964). 6. Koop, E. E., Kiesemetter, W. B., and Horn, R. C., Jr. Neuroblastoma in childhood: Survival after major surgical insult to tumor, Surgery 38, 272 (1955). 7. Everson, T. C. Spontaneous regression of cancer, Ann. N.Y. Acad. Sci. 114, 721 (1964).
ONCOLOGY
3, 308-3 I3 (1975)
Solid Ovarian Teratoma with Peritoneal and Abdominal Wall Implants, Progressive in vivo Maturation, and Probable Cure FRED BENJAMIN, M.D., AND EDWARDA RORAT, M.D. The Department of Obstetrics 8s Gynecology und Pathology, Queens Hospital Center Afiliate of the Long Islund Jeivish-Hillside Medical Center, Jamaica, New York, and the State University of New York crt Stony Brook Received July 28, 1975 Progressive in viva maturation from immature to mature tissue occurred in a case of solid ovarian tumor with peritoneal and abdominal wall implants. Apparent cure occurred after conservative surgery without irradiation or chemotherapy. The history and therapy of such tumors is discussed.
Despite peritoneal implants and subsequent recurrence in the pelvis and abdominal wall, progressive maturation of a solid ovarian tumor occurred with ultimate spontaneous cure. The surgery was conservative and incomplete, and no radiotherapy or chemotherapy was administered. This case is of interest both from the standpoint of the therapy and the biology of such tumors. CASE REPORT First Admission
V. T., a 16-yr-old, unmarried black female, Para 0, was admitted to Queens Hospital Center on July 27, 1966, because of a large (approximately lo-cm diameter) left adnexal mass. This was considered to be an ovarian tumor. At laparotomy, although the uterus and right tube and ovary were normal, the left ovary was completely replaced by a tumor, predominantly solid with a small cystic area, and a left salpingo-ooporectomy was done. No free fluid nor gross metastases were noted. The right ovary was not biopsied. Pathology. Grossly (Fig. l), the primary tumor, measuring 12 X 9 X 7 cm was a completely encapsulated predominantly solid mass, with a cystic space, containing hair and sebaceous material. The solid portion was polypoid and cauliflowerlike in appearance, soft and focally spongy in consistency. Microscopically, sections of the primary solid mass revealed predominantly mature tissues with foci of embryonal neuroepithelial rosettes (Fig. 2), with nuclear atypism and mitotic activity, resembling neuroblastoma. Other areas revealed malignant mesenchyme and foci of necrosis. The mature elements, including the cyst, revealed typical benign teratoma. This mass was graded 2 out of 3, according to the grading of Thurlbeck and Scully [ 11. 308 Copyright 0 1975 by Academic Press, Inc. All rights of reproduction in any form reserved.
SOLID OVARIAN
TERATOMA
309
FIG. 1. Partially opened solid ovarian teratoma, composed predominantly of polypoid mass. Lower quadrant shows cyst, filled with matted hair.
Second Admission
The patient returned to the hospital 1 yr later (July 1967) complaining of a mass in the surgical scar. There was a 4 X 5 X 3-cm hard mass in the center of the Pfannensteil incision, somewhat attached to the symphysis pubis. The mass
310
BENJAMIN
FIG. 2. Embryonal
neuroepithelial
AND
RORAT
rosettes with numerous mitoses (X 250).
was enucleated locally, the peritoneal cavity not opened. The patient was discharged without further therapy. Pathology. The specimen, removed from the surgical scar, was grossly predominantly solid, interspersed with small cysts. Microscopically, the tumor was composed of the same type of tissue as the first specimen, but the immature neuroeipthelium and mesenchyme were more sparse.
SOLID
OVARIAN
TERATOMA
311
Third Admission Fourteen months later (September 1968), the patient presented again with a 6 x 8 X S-cm mass occupying most of the right half of the Pfannensteil incision and extending towards the symphysis pubis, the mons pubis, and into the deeper planes of the abdominal wall.. Pelvic examination was negative except for a small fixed mass in the culdesac. IVP, cystoscopy, bone survey, barium enema, and serum chemistries did not reveal evidence of distant metastases. The abdomen and pelvis were explored. A mass, which extended to the symphysis pubis and the urinary bladder, was removed together with a flap of urinary bladder. Some small implants were found and excised from the omentum, right ovary, and right mesosalpinx. A fixed mass that could not be removed was found in the culdesac. A biopsy of this mass was taken. Following an uneventful recovery the patient was discharged without further therapy. Pathology. All the aforementioned biopsies revealed the same picture microscopically, i.e., exclusively mature elements: epidermis with skin appendages (Fig. 3), intestinal mucosa, cartilage, respiratory epithelium, adipose tissue, and mature glial tissue. No immature or malignant elements were identified. Eight-Year Follow-up Following her last operation in 1968, the patient was repeatedly examined and found to be in excellent health and menstruating normally. The last physical examination in December 1974 revealed a woman in excellent health who was attending college. The pelvic examination was normal: the uterus was anteverted, normal in size and mobile; the adnexa were not palpable or tender; there was no residual palpable tumor in the culdesac. COMMENT
The reported incidence of solid teratomas of the ovary has been 2 : 1000 of all ovarian tumors [ 11. These lesions are predominantly solid in consistency, containing varying amounts of completely mature and/or embryonal tissue. Therefore, they have been graded according to the quantity of embryo& tissue found in each: Grades O-3 [ 11. The lower the grade, the better the prognosis. There have been several case reports of solid ovarian teratomas, with peritoneal implants composed predominantly or entirely of glial tissue, who survived [2]. Such reports indicate that the presence of metastases, when composed of glial tissue, does not necessarily mean a poor prognosis and that in such cases conservative treatment may be sufficient. The present case illustrates the following: (a) The primary tumor in 1966 was composed of moderate quantities of embryonal tissue (Grade 2). Successive biopsies from 1966 to 1968 revealed progressive maturation of the tumor tissue. (b) The final biopsies showed totally mature metastatic implants composed of neuroepithelium, epidermis with skin appendages, intestinal mucosa, respiratory epithelium, and cartilage. Other cases in the literature [2] revealed only glial tissue in implants seen at follow-up. (c) The inoperable mass in the culdesac noted in 1968 had completely regressed in 1973 without any treatment. (d) The patient was alive and well and, as far as can be determined, free of disease, 8 yr
312
BENJAMIN
AND
FIG. 3. Omental implant, composed of keratinizing tive tissue, with four sweat glands (X25).
RORAT
squamous epithelium
with underlying
connec-
after the first appearance of the tumor and 6 yr after the occurrence of metastases. It is not possible to know whether the implants were first immature like the primary tumor and then matured or whether mature tissue was transferred through a possible microscopic defect in the capsule of the primary tumor.
SOLID OVARIAN
TERATOMA
313
The progressive in viva maturation of the tumor and the regression of the culdesac implant raise interesting questions about the biology of these tumors. There is an interesting and growing literature on the remarkable phenomenon of in vivo maturation, and hence cure, of neuroblastoma [3, 41. This tendency for spontaneous growth control in this type of tumor seems to have been activated in some instances by partial or complete removal of the primary tumor [ 5, 61. The world literature now contains a number of well-documented cases of spontaneous regression of cancer [7]. Although the most frequent one is neuroblastoma, spontaneous regression of other cancers, especially hypernephroma and choriocarcinoma, has also been documented [7]. Possible factors that may be responsible for progressive maturation and spontaneous regression of cancer include: endocrine influences, unusual sensitivity to usually inadequate therapy, fever and/or infection, allergic or immune reactions, interference with maturation of the tumor, and removal of the carcinogenic agent. The case reported here raises questions about the management of solid ovarian teratomas. Treatment of such tumors with mature implants in the peritoneum has varied from simple removal of the involved ovary to radical surgery, followed by postoperative irradiation or chemotherapy. This case, as well as others in the literature [ 21, suggests that, particularly if glial tissue or any mature tissue is involved, it may be justified to spare the contralateral ovary (the tumor is usually unilateral) in a young woman and that no therapy is indicated for mature peritoneal implants. REFERENCES 1. Thurlbeck, W. M., and Scully, R. E. Solid teratoma of the ovary: A Clinicopathological analysis of 9 cases, Cancer 13, 804 (1960). 2. Robboy, S. J., and Scully, R. E. Ovarian teratoma with glial implants in the peritoneum: An analyses of 12 cases, Hum. Pathol. 1, 643 (1970). 3. Gross, R. E., Farber, S., and Martin, L. W. Neuroblastoma sympatheticum, pediatrics 23, 1179 (1959). 4. Former, J., Nicastri, A., and Murphy, M. L. Neuroblastoma: natural history and results of treating 133 cases, Ann. Surg. 167, 132 (1968). 5. Koop, C. E., and Hemandez, J. R. Neuroblastoma: experience with 100 cases in children, Surgery 56, 726 (1964). 6. Koop, E. E., Kiesemetter, W. B., and Horn, R. C., Jr. Neuroblastoma in childhood: Survival after major surgical insult to tumor, Surgery 38, 272 (1955). 7. Everson, T. C. Spontaneous regression of cancer, Ann. N.Y. Acad. Sci. 114, 721 (1964).