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Embryonal rhabdomyosarcoma (botryoid type) of the cervix: A case report and review
GYNECOLOGIC
ONCOLOGY
13, 115-119
(1982)
CASE REPORT Embryonal Rhabdomyosarcoma (Botryoid Type) of the Cervix: A Case Report and Review ANTON ORTNER, M.D.,’ GRUNTERWEISER, M.D., HELMUT HAAS, M.D., ROSMARIERESCH, M.D., AND OTTO DAPUNT, M.D. Departments
qf Obstetrics
and Gynecology
und Pathology Innsbruck. Austriu
and Pediatrics,
University
of Innsbruck,
Accepted May 8, 1981
A case report of embryonal rhabdomyosarcoma originating from the cervix in a 3-yearold girl is presented and a brief review of the literature on this topic given. The histopathology of the tumor is discussed and the effectiveness of a less aggressive surgical approach and chemotherapy is demonstrated in this particular case.
INTRODUCTION
Embryonal rhabdomysarcoma is a rare tumor, especially originating from the cervix. The mode of therapy has changed in recent years from ultraradical surgical treatment to a less aggressive surgical approach followed by irradiation and chemotherapy to control the residual tumor. The purpose of this report is to describe the case history of an embryonal rhabdomyosarcoma (botryoid type) originating from the cervix, to discuss the histogenesis and morphology of the tumor, and to demonstrate the effectiveness of a reasonable surgical approach and chemotherapy in this particular case. CASE REPORT
W.R., a 3&year-old girl had a history of intermittent vaginal spotting for 4 months. Family history and pediatric physical tests were normal. Gynecological examination revealed a polypoid mass protruding into the vaginal orifice (Fig. 1) and originating from the posterior lip of the uterine cervix. There was no further palpable mass in the pelvis. The cytological sample (Figs. 2 and 3) showed the generally spindle-shaped tumor cells singly or in loose sheets. The cytoplasm was abundant and cyanophilic without cross-striation at least on light microscopic view. The nuclei were frequently eccentrically located and showed no marked ’ To whom reprint requests should be addressed at: Department of Obstetrics and Gynecology, University of Innsbruck. Anichstrasse 35. A-6020 Innsbruck, Austria.
0090-8258/82/010115-05$01.00/O Copyright 0 1982 by Academic Press. Inc. All rights of reproduction in any form reserved.
116
ORTNER
ET AL.
EMBRYONAL
RHABDOMYOSARCOMA
117
anaplastic changes. Histological sections of the avulsed polypoid tumor showed mostly spindle-shaped tumor cells with abundant elongated cytoplasm beneath a normal squamous or endocervical epithelium (Fig. 4). The tumor cells were classified as rhabdomyoblasts as demonstrated in Fig. 5. Pathological diagnosis of embryonal rhabdomyosarcoma (botryoid type) was reported. Total abdominal hysterectomy with vaginal cuff and left salpingo-oophorectomy was our surgical procedure. There was no residual tumor on macroscopic view of the surgical specimen. Histological examination of the surgical specimen (No. 23069) revealed sarcoma botryoides orginating from the posterior lip of the uterine cervix, growing beneath the normal epithelium into the uterine cavity. Histological sections showed the polypoid tumor which was partly covered either with normal low squamous or with normal cervical epithelium. The spindle- or ovalshaped tumor cells showed no marked nuclear pleomorphism in the edematous neoplastic tissue. No infiltration of the myometrium and the vaginal wall or spreading into vessels was found. According to the classification of the Intergroup Rhabdomyosarcoma Study [l] our patient had to be classified in clinical stage I. Irradiation was omitted because of the localized disease and polychemotherapy started with vincristine, actinomycin D, and cyclophosphamide. Chemotherapy was performed according to the regimen of the Intergroup Rhabdomyosarcoma Study: vincristine-1.5 mg/m’ iv once a week during the first 12 weeks, then every 3 months for 2 years; actinomycin D-O.450 mg/m’/ day over 5 days every 3 months for 2 years; oral cyclophosphamide-2.5 mg/kg/ day for 2 years. Due to bone marrow depression cyclophosphamide had to be reduced to 2 mg/kg every other day after the first 10 weeks. Chemotherapy was stopped in May 1980. At the time of this writing the child is in complete remission for 31 months. The course so far was uneventful. DISCUSSION The incidence of botryoid sarcoma originating from the cervix is probably very rare. The true incidence is difficult to estimate for several reasons. First, knowledge is based on individual case reports. Second, this tumor is classified either in the group of Muellerian tumors or in the group of rhabdomyosarcomas, and numerous synonyms were used in the past [2]. The primary site of origin is not always exactly stated in the literature. During childhood this tumor is reported to originate mainly from the vagina, while the cervix is the primary disease site in the adult. Daniel and co-workers [3] reported the vagina as the site of origin in all of FIG. I.
Polypoid tumor mass protruding into the vaginal orifice.
FIG.
2. Cytologic sample of botryoid sarcoma cells (Pap, 250 x ).
FIG.
3. Cytologic sample of botryoid sarcoma cells: note the racket cell type (Pap 250x).
FIG. 4. Histologic section of embryonal rhabdomyosarcoma (H&E IO x ). FIG.
5. Histologic section of embryonal rhabdomysarcoma. (Masson 160x ).
118
ORTNER ET AL.
their 13 cases. The cervix was involved secondarily in a few cases. Four out of seven cases of sarcoma botryoides arose from the cervix in Taylor’s [4] series, but only one of these cases was during childhood. Novak [5] stressed the distal part of the Muellerian duct as the main source of this tumor. Typical extragenital localizations are the urinary tract, the bile ducts, and the head and neck region. On clinical examination the tumor appears as a soft, pink, polypoid mass. The denomination “botryoid” refers to the multiple polypoid structures of the gross specimen and was introduced by Pfannenstiel in 1892 [6]. Hemorrhagic vaginal discharge may be the first symptom before the tumor protrudes through the vaginal introitus. The malignant potential of such a lesion is not obvious on macroscopic view but because of the rarity of benign polyps in very young girls every such lesion must be biopsied in this age group. The recommendations of the Intergroup Rhabdomyosarcoma Study [l] are widely used today for clinical staging. The clinical course of rhabdomyosarcoma is very progressive. Sulser [7] reported only 3 cases with 5 years survival in his series of 46 rhabdomyosarcomas from various sites. The prognosis depends both on location of the tumor and on its clinical stage. According to Sulser [7] and Rivard and co-workers [8] the mean duration of survival is 1 year. Yet recently published papers on this subject demonstrate a better survival rate with adequate therapy and much better quality of life. Contrary to the pleomorphic rhabdomyosarcoma which tends to metastasize early, the embryonal (botryoid) rhabdomyosarcoma infiltrates locally and tends to recur locally. Histogenesis
and Histopathology
Two main theories about the histogenesis of botryoid sarcoma are discussed in the literature. Some authors [5,9,10] classify this tumor under the heading of mixed mesodermal tumors while others [3,1 l-141 describe this tumor as a special form of embryonal rhabdomyosarcoma. The latter view facilitates explaining the histogenesis of this tumor in extragenital localization. Further various mesodermal types of cells were absent in most cases reported in the literature. The histologic pattern of botryoid sarcoma is fairly equal to that of embryonal rhabdomysarcoma. The spindle- or polygonal-shaped tumor cells spread beneath an intact epithelium in a loose arrangement. The demonstration of rhabdomyoblasts is of diagnostic value [7]. According to Carlson [15] rhabdomyoblasts are mononuclear cells with myofilaments demonstrable with the electron microscope. On light microscopic view these cells are spindle-shaped and their cytoplasm sometimes filled with eosinophilic granules. The oval vesicular nuclei often show one prominent nucleolus. For diagnostic purposes the Masson-Goldner (Trichrom) staining which shows the cytoplasm in bright red color is very helpful. Cross-striation is demonstrable only in 30-50% of the cases with light microscopy. REFERENCES 1. Maurer, H. M., ef ul. The Intergroup Rhabdomyosarcoma Study, a preliminary report, Cancer 40, 2015-2026 (1977). 2. Hilgers, R. D., Malkasian, G. D., Jr., and Soule, E. H. Embryonal rhabdomyosarcoma (botryoid type) of the vagina, Amer. J. Obsret. Gynecol. 107, 484-502 (1970).
EMBRYONAL
RHABDOMYOSARCOMA
119
3. Daniel, W. W., Koss, L. G.. and Brunschwig, A. Sarcoma botryoides of the vagina, Canc,er 12, 74-84 (1959). 4. Taylor, C. W. Mesodermal mixed tumours of the female genital tract, J. Obsrer. Gwmrcol. Brit. Emp. 65, 177-188 (1958). 5. Novak, E. R., and Woodruff, J. D. Novak’s gynecologic and obstetric pathology, Saunders, Philadelphia/London/Toronto, 8th ed., pp. 76-78 (1979). 6. Pfannenstiel, J. Das traubige Sarcom der Cervix uteri, Virchows Arch. Parhol. Ana/. 127, 305-337 (1892). 7. Sulser, H. Das Rhabdomyosarkom. Virchows Arch. Purhol. Anui. ffistol. 379, 35-71 (1978). 8. Rivard, G., Ortega, J., Hittle, R., Nitschke, R.. and Karon, M. Intensive chemotherapy as primary treatment for rhabdomyosarcoma of the pelvis. Cuncer 36, 1593-1597 (1975). 9. Robbins, St. L. Puthologic basis of diseuse, Saunders, Philadelphia/London/Toronto, pp. 1213-1214 (1974). 10. Hafez, E. S. E., and Evans, T. N. The human vagina, North-Holland, Amsterdam/New York/ Oxford, pp. 376-377 (1978). Il. Haines, M., and Taylor, C. W. Gynaecological pathology, Churchill-Livingstone, Edinburgh/ London/New York, pp. 295-297 (1975). 12. Horn, R. C., Jr., and Enterline, H. T. Rhabdomyosarcoma-A clinicopathological study and classification of 39 cases, Cancer 11, 181-199 (1958). 13. Stout, A. P., and Lattes, R. Tumors of soft tissues, in Atlas oftumor pathology, Armed Forces Institute of Pathology, Washington D. C., Fast. I, Ser. 2, pp. 138-144 (1967). 14. Talerman, A. Sarcoma botryoides presenting as a polyp on the labium majus, Cancer 32,994-999 (1973). 15. Carlson, B. M. The regeneration of skeletal muscle--A review. Amer. J. Anur. 137, 119-IS0 (1973).
ONCOLOGY
13, 115-119
(1982)
CASE REPORT Embryonal Rhabdomyosarcoma (Botryoid Type) of the Cervix: A Case Report and Review ANTON ORTNER, M.D.,’ GRUNTERWEISER, M.D., HELMUT HAAS, M.D., ROSMARIERESCH, M.D., AND OTTO DAPUNT, M.D. Departments
qf Obstetrics
and Gynecology
und Pathology Innsbruck. Austriu
and Pediatrics,
University
of Innsbruck,
Accepted May 8, 1981
A case report of embryonal rhabdomyosarcoma originating from the cervix in a 3-yearold girl is presented and a brief review of the literature on this topic given. The histopathology of the tumor is discussed and the effectiveness of a less aggressive surgical approach and chemotherapy is demonstrated in this particular case.
INTRODUCTION
Embryonal rhabdomysarcoma is a rare tumor, especially originating from the cervix. The mode of therapy has changed in recent years from ultraradical surgical treatment to a less aggressive surgical approach followed by irradiation and chemotherapy to control the residual tumor. The purpose of this report is to describe the case history of an embryonal rhabdomyosarcoma (botryoid type) originating from the cervix, to discuss the histogenesis and morphology of the tumor, and to demonstrate the effectiveness of a reasonable surgical approach and chemotherapy in this particular case. CASE REPORT
W.R., a 3&year-old girl had a history of intermittent vaginal spotting for 4 months. Family history and pediatric physical tests were normal. Gynecological examination revealed a polypoid mass protruding into the vaginal orifice (Fig. 1) and originating from the posterior lip of the uterine cervix. There was no further palpable mass in the pelvis. The cytological sample (Figs. 2 and 3) showed the generally spindle-shaped tumor cells singly or in loose sheets. The cytoplasm was abundant and cyanophilic without cross-striation at least on light microscopic view. The nuclei were frequently eccentrically located and showed no marked ’ To whom reprint requests should be addressed at: Department of Obstetrics and Gynecology, University of Innsbruck. Anichstrasse 35. A-6020 Innsbruck, Austria.
0090-8258/82/010115-05$01.00/O Copyright 0 1982 by Academic Press. Inc. All rights of reproduction in any form reserved.
116
ORTNER
ET AL.
EMBRYONAL
RHABDOMYOSARCOMA
117
anaplastic changes. Histological sections of the avulsed polypoid tumor showed mostly spindle-shaped tumor cells with abundant elongated cytoplasm beneath a normal squamous or endocervical epithelium (Fig. 4). The tumor cells were classified as rhabdomyoblasts as demonstrated in Fig. 5. Pathological diagnosis of embryonal rhabdomyosarcoma (botryoid type) was reported. Total abdominal hysterectomy with vaginal cuff and left salpingo-oophorectomy was our surgical procedure. There was no residual tumor on macroscopic view of the surgical specimen. Histological examination of the surgical specimen (No. 23069) revealed sarcoma botryoides orginating from the posterior lip of the uterine cervix, growing beneath the normal epithelium into the uterine cavity. Histological sections showed the polypoid tumor which was partly covered either with normal low squamous or with normal cervical epithelium. The spindle- or ovalshaped tumor cells showed no marked nuclear pleomorphism in the edematous neoplastic tissue. No infiltration of the myometrium and the vaginal wall or spreading into vessels was found. According to the classification of the Intergroup Rhabdomyosarcoma Study [l] our patient had to be classified in clinical stage I. Irradiation was omitted because of the localized disease and polychemotherapy started with vincristine, actinomycin D, and cyclophosphamide. Chemotherapy was performed according to the regimen of the Intergroup Rhabdomyosarcoma Study: vincristine-1.5 mg/m’ iv once a week during the first 12 weeks, then every 3 months for 2 years; actinomycin D-O.450 mg/m’/ day over 5 days every 3 months for 2 years; oral cyclophosphamide-2.5 mg/kg/ day for 2 years. Due to bone marrow depression cyclophosphamide had to be reduced to 2 mg/kg every other day after the first 10 weeks. Chemotherapy was stopped in May 1980. At the time of this writing the child is in complete remission for 31 months. The course so far was uneventful. DISCUSSION The incidence of botryoid sarcoma originating from the cervix is probably very rare. The true incidence is difficult to estimate for several reasons. First, knowledge is based on individual case reports. Second, this tumor is classified either in the group of Muellerian tumors or in the group of rhabdomyosarcomas, and numerous synonyms were used in the past [2]. The primary site of origin is not always exactly stated in the literature. During childhood this tumor is reported to originate mainly from the vagina, while the cervix is the primary disease site in the adult. Daniel and co-workers [3] reported the vagina as the site of origin in all of FIG. I.
Polypoid tumor mass protruding into the vaginal orifice.
FIG.
2. Cytologic sample of botryoid sarcoma cells (Pap, 250 x ).
FIG.
3. Cytologic sample of botryoid sarcoma cells: note the racket cell type (Pap 250x).
FIG. 4. Histologic section of embryonal rhabdomyosarcoma (H&E IO x ). FIG.
5. Histologic section of embryonal rhabdomysarcoma. (Masson 160x ).
118
ORTNER ET AL.
their 13 cases. The cervix was involved secondarily in a few cases. Four out of seven cases of sarcoma botryoides arose from the cervix in Taylor’s [4] series, but only one of these cases was during childhood. Novak [5] stressed the distal part of the Muellerian duct as the main source of this tumor. Typical extragenital localizations are the urinary tract, the bile ducts, and the head and neck region. On clinical examination the tumor appears as a soft, pink, polypoid mass. The denomination “botryoid” refers to the multiple polypoid structures of the gross specimen and was introduced by Pfannenstiel in 1892 [6]. Hemorrhagic vaginal discharge may be the first symptom before the tumor protrudes through the vaginal introitus. The malignant potential of such a lesion is not obvious on macroscopic view but because of the rarity of benign polyps in very young girls every such lesion must be biopsied in this age group. The recommendations of the Intergroup Rhabdomyosarcoma Study [l] are widely used today for clinical staging. The clinical course of rhabdomyosarcoma is very progressive. Sulser [7] reported only 3 cases with 5 years survival in his series of 46 rhabdomyosarcomas from various sites. The prognosis depends both on location of the tumor and on its clinical stage. According to Sulser [7] and Rivard and co-workers [8] the mean duration of survival is 1 year. Yet recently published papers on this subject demonstrate a better survival rate with adequate therapy and much better quality of life. Contrary to the pleomorphic rhabdomyosarcoma which tends to metastasize early, the embryonal (botryoid) rhabdomyosarcoma infiltrates locally and tends to recur locally. Histogenesis
and Histopathology
Two main theories about the histogenesis of botryoid sarcoma are discussed in the literature. Some authors [5,9,10] classify this tumor under the heading of mixed mesodermal tumors while others [3,1 l-141 describe this tumor as a special form of embryonal rhabdomyosarcoma. The latter view facilitates explaining the histogenesis of this tumor in extragenital localization. Further various mesodermal types of cells were absent in most cases reported in the literature. The histologic pattern of botryoid sarcoma is fairly equal to that of embryonal rhabdomysarcoma. The spindle- or polygonal-shaped tumor cells spread beneath an intact epithelium in a loose arrangement. The demonstration of rhabdomyoblasts is of diagnostic value [7]. According to Carlson [15] rhabdomyoblasts are mononuclear cells with myofilaments demonstrable with the electron microscope. On light microscopic view these cells are spindle-shaped and their cytoplasm sometimes filled with eosinophilic granules. The oval vesicular nuclei often show one prominent nucleolus. For diagnostic purposes the Masson-Goldner (Trichrom) staining which shows the cytoplasm in bright red color is very helpful. Cross-striation is demonstrable only in 30-50% of the cases with light microscopy. REFERENCES 1. Maurer, H. M., ef ul. The Intergroup Rhabdomyosarcoma Study, a preliminary report, Cancer 40, 2015-2026 (1977). 2. Hilgers, R. D., Malkasian, G. D., Jr., and Soule, E. H. Embryonal rhabdomyosarcoma (botryoid type) of the vagina, Amer. J. Obsret. Gynecol. 107, 484-502 (1970).
EMBRYONAL
RHABDOMYOSARCOMA
119
3. Daniel, W. W., Koss, L. G.. and Brunschwig, A. Sarcoma botryoides of the vagina, Canc,er 12, 74-84 (1959). 4. Taylor, C. W. Mesodermal mixed tumours of the female genital tract, J. Obsrer. Gwmrcol. Brit. Emp. 65, 177-188 (1958). 5. Novak, E. R., and Woodruff, J. D. Novak’s gynecologic and obstetric pathology, Saunders, Philadelphia/London/Toronto, 8th ed., pp. 76-78 (1979). 6. Pfannenstiel, J. Das traubige Sarcom der Cervix uteri, Virchows Arch. Parhol. Ana/. 127, 305-337 (1892). 7. Sulser, H. Das Rhabdomyosarkom. Virchows Arch. Purhol. Anui. ffistol. 379, 35-71 (1978). 8. Rivard, G., Ortega, J., Hittle, R., Nitschke, R.. and Karon, M. Intensive chemotherapy as primary treatment for rhabdomyosarcoma of the pelvis. Cuncer 36, 1593-1597 (1975). 9. Robbins, St. L. Puthologic basis of diseuse, Saunders, Philadelphia/London/Toronto, pp. 1213-1214 (1974). 10. Hafez, E. S. E., and Evans, T. N. The human vagina, North-Holland, Amsterdam/New York/ Oxford, pp. 376-377 (1978). Il. Haines, M., and Taylor, C. W. Gynaecological pathology, Churchill-Livingstone, Edinburgh/ London/New York, pp. 295-297 (1975). 12. Horn, R. C., Jr., and Enterline, H. T. Rhabdomyosarcoma-A clinicopathological study and classification of 39 cases, Cancer 11, 181-199 (1958). 13. Stout, A. P., and Lattes, R. Tumors of soft tissues, in Atlas oftumor pathology, Armed Forces Institute of Pathology, Washington D. C., Fast. I, Ser. 2, pp. 138-144 (1967). 14. Talerman, A. Sarcoma botryoides presenting as a polyp on the labium majus, Cancer 32,994-999 (1973). 15. Carlson, B. M. The regeneration of skeletal muscle--A review. Amer. J. Anur. 137, 119-IS0 (1973).