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Vulvar sarcoma: A report of four cases
GYNECOLOGIC
ONCOLOGY
46,
384-390 (1992)
CASE REPORT Vulvar Sarcoma: A Report of Four Cases YOUNES N. BAKRI,* Division
of Gynecologic
Oncology,
*Departments
M. AKHTAR,~ M. EL-SENOUSSI,$
AND
of Obstetrics and Gynecology, +Pathology, and Research Centre, Riyadh, Saudi Arabia
R.
WIERZBICKI$
and SOncology,
King Faisal Specialist Hospital
Received November 18. 1991
amycin, and DTIC were given, followed by wide local excision. She returned to Riyadh and received radiation comas are presented. Two patients had leiomyosarcoma in astherapy to the pelvis and vulva which was completed in sociation with pregnancy, a third patient had a leiomyosarcoma December 1982. Chemotherapy was resumed in January with epithelioid elements, and a fourth had alveolar rhabdo1983. myosarcoma. The natural history of the disease in the three leioIn January 1986, examination under anesthesia and myosarcomas (including the case with epithelioid elements) was characterized by an indolent protracted course and frequent local multiple vulvar and pelvic biopsies revealed no evidence recurrence, followed by distant fatal metastases.The patient with of disease. Follow-up in April 1987 manifested negative alveolar rhabdomyosarcoma is alive-with-disease. Surgery, che- chest X ray. However, in October 1987 chest X ray remotherapy, and radiotherapy achieved palliation rather than vealed multiple bilateral pulmonary metastases and local cure. Pregnancy did not seem to infhrence the prognosis. o wu right vulvar recurrence. She died at home in early 1988. The clinical profiles of four patients with primary vulvar sar-
Academic
Rcs.Inc.
INTRODUCTION
Case 2
Primary vulvar sarcomas constitute l-3% of all malignant vulvar neoplasms, less than 30 cases of all histologic types were reported until 1990,5 of which were associated with pregnancy [l]. Knowledge of the disease is limited to case reports and collective reviews rather than large series [2-51. The purpose of this report is to describe the clinical profiles of vulvar sarcomas in 4 patients.
CASE REPORTS Case I A 29-year-old patient, gravida 6 para 6, was well until she noticed a lump in the right labium majus, which slowly increased in size until it became 9 cm in largest diameter when first excised in late 1978. Pathology revealed leiomyosarcoma (Fig. 1). She underwent four excisions for local recurrence in the 3-year period between 1978-1981. First excision for recurrence was done 6 weeks after normal vaginal birth. In 1982 she was transferred to M. D. Anderson Hospital where two courses of Cytoxan, Adri-
The patient, a 41-year-old gravida 10 para 10, was well until mid 1981 when she noticed a lump in the left labium majus, which gradually increased in size. In March 1982 she had a normal vaginal birth but the vulvar mass remained without treatment until May 1982 when excision of a left vulvar lesion 10 x 8 cm was performed. A second local resection for recurrence was done in November 1982 and a third in February 1983. Histopathology revealed leiomyosarcoma grade III. In June 1985 a fourth local recurrence and resection of a 6-cm lesion was done, but chest X ray this time revealed multiple bilateral pulmonary metastases. She was asymptomatic then, and for that reason a decision was made to observe without further therapy. She developed chest pains in December 1986 for which palliative irradiation was given, with improvement of chest symptoms. Condition deteriorated in late 1987 and palliative DTIC was given, but in May 1988 she had paraplegia secondary to metastatic Tr fracture. She died in late 1988 (42 months after developing pulmonary metastases).
384 0090-8235~92$4.00 Copyright 0 1992 by Academic Press, Inc. All rights of reproduction in any form reserved.
CASE REPORT
385
Z. 1. High grade leiomyosarcoma featuring intertwining bundles of spindle-shaped cells with several mitoses and nuclear pleomoq bhism (Case 1). Hematoxylin and eosin stain, 200x.
of a lump in the left labium majus. The initial lesion was excised in 1979 and repeated excisions were done for The patient, a 17-year-old woman, was first seen at recurrence in 1981 and 1982; pathology material and recKFSH in October 1985 because of vulvar mass. She was ords of these excisions were not available. On admission well until 1977 when at age 9, she noticed the appearance to KFSH she complained of difficulty in walking and voiding because of a mass-pressure effect. Chest X ray revealed multiple bilateral pulmonary metastases. A wide local excision of a left vulvar 15 x lo-cm mass (Fig. 2) was performed, and pathologic examination of the mass revealed leiomyosarcoma, grade I, with focal epithelioid and mutinous changes (Figs. 3 and 4). Chemotherapy with vincristine, Adriamycin, and Cytoxan was started in November 1985 and was switched to vindesine, actinomycin D, and DTIC in February 1986. Patient died of metastases in June 1986. Case 3
Case 4
FIG. 2. Left vulvar mass lesion leiomyosarcoma (Case 3).
The patient, a 20-year-old unmarried woman, was well until November 1990 when she noticed a left vulvar swelling, followed a month later by a swelling of the left groin and pain in the left thigh. Upon referral to our hospital in March 1991 she was normal at general physical ex-
386
BAKRI ET AL,
FIG. 3. Bundles of spindle-shaped cells from well-differentiated leiomyosarcoma (Case 3). Hemotoxylin and eosin stain, 150x .
amination and had a left labium majus, vulvar mass 8 x 10 cm which was firm and nontender, together with 5 x 3 cm left inguinal lymph node enlargement. Workup revealed normal blood chemistries but CT of the pelvis and abdomen revealed extensive retroperitoneal paraaortic and pelvic lymph nodal enlargement with left hydroureter. She underwent a wide local excision of the vulvar mass and removal of grossly enlarged left inguinal lymph nodes. Histopathology revealed alveolar rhabdomyosarcoma of the left labium majus (Fig. 5) with metastases to inguinal lymph nodes. Adjuvant chemotherapy incorporating cyclophosphamide, Adriamycin, and vincristine (CAV) was given. Laparotomy for suspected left pelvic retroperitoneal mass after five CAV courses revealed fibrosis only, with microscopic metastatic involvement of the left internal iliac nodes. Paraaortic and common iliac nodes were negative. Postoperative pelvic external beam irradiation was started in February 1992. DISCUSSION The natural history of vulvar sarcoma is not clearly understood; this is related to the rarity of the vulvar
involvement by primary sarcomatous neoplasms, various histologic types, and the limited number of case reports, which make it difficult to draw meaningful conclusions, particularly because most reported cases had short-term follow-up at the time they were published. This is in contradistinction to soft tissue sarcomas originating from extragenital sites, mainly extremities, where the natural history of the disease is well known. Among published reports, the M. D. Anderson Hospital [3,6] and the University of Michigan Medical Center [4], managed the largest series of patients with vulvar sarcoma, and experienced the longest follow-up. Ulbright et al. [7] described 2 cases of vulvar epithelioid sarcomas and concluded that the disease, when originating from the vulva, manifests a more aggressive behavior than extragenital epithelioid sarcoma; however, our Case 3 who had leiomyosarcoma with epithelioid changes together with the 2 other cases of leiomyosarcoma had a behavior similar to extragenital disease, with indolent protracted course preceding fatal metastatic outcome. Two of our patients and 5 of the 30 cases reported in the literature had the disease in association with pregnancy, making it a relatively common association when compared with vulvar carcinomas; this
CASE REPORT
FIG. 4. An area within the tumor from Case 3, featuring epithelial cells in a myxoid background. Occasional multinucleated tumor cells are also present.
can be explained by the fact that sarcomas usually affect a younger age while vulvar carcinomas usually affect an older postmenopausal group. Cases reported in association with pregnancy were of variable histologic types and included 2 fibrosarcomas, 1 reticulum cell vs rhabdomysarcoma, 1 mixed mesodermal tumor, and 1 leiomyosarcoma [l]. In our 2 patients with leiomyosarcoma, pregnancy did not seem to influence survival. Unfortunately, almost all reports of vulvar sarcoma in association with pregnancy are weakened by the short-term follow-up and no valid conclusions could be drawn as to how pregnancy influences the disease outcome. In Case 4 the referral diagnosis and even our intraoperative frozen-section microscopic diagnosis were mistaken for poorly differentiated adenocarcinoma. This case supports the conclusion of Copeland et al. [6] that awareness of the typical microscopic picture of alveolar rhabdomyosarcoma is important for early diagnosis and treatment. Table 1 summarizes the clinical profiles of reported cases of vulvar sarcoma who had 5 years or more of follow-up. We excluded casespublished with short-term follow-up. Perhaps
personal communication with publishing authors or institutions in order to obtain updates on the status of those patients whose case reports were already published in peer-reviewed literature may yield long-term follow-up information on a number of patients adequate enough to help draw meaningful conclusions. Davos and Abel1 [5] believe that inadequate initial treatment leads to frequent local recurrences and recommend wide local excision or radical vulvectomy early in the disease, with bilateral lymph node resection for those sarcomas that have a potential for lymphogenous metastases. We believe, however, that the treatment should be individualized, to take into consideration the factors of tumor histologic type and grade, tumor size, location, and extent of locaregional and distant metastases. Review of the published cases with adequate follow-up information (Table 1) perhaps does not supply convincing evidence that radical surgery improves the final disease outcome. Although it is difficult and perhaps invalid to draw any conclusion from such a small number of reviewed cases, it is perhaps reasonable to recommend local excision with adequate surgical mar-
388
BAKRI
ET AL.
TABLE 1 Clinical Profiles of Patients with Vulvar Sarcoma and Long-Term Follow-up Survival Reference
Histology
Age
Location
Size (cm)
Initial
surgery
Present study Leiomyosarcoma grade III Leiomyosarcoma grade III Leiomyosarcoma with epithelioid elements
29 Right labium majus 41 Left labium majus I7 Left labium majus
DiSaia et al. Leiomyosarcoma
64 Labium majus
S x 6
Leiomyosarcoma
57 Labmm majus
5 x 6
Local excision, 1957
Leiomyosarcoma
56 Labium majus
2 x 3
Local excision, I948
Rhabdomyosarcoma Rhabdomyosarcoma
28 Labium majus 44 Labium majus
6
Neurotibrosarcoma
25 Labium majus 18 Lahtum majus
131
DiSaia et af. Alveolar rhabdomyo13.61 sarcoma Copeland et Alveolar al. (61 rhabdomyosalcoma Alveolar rhabdomyosarcoma Alveolar rhabdomyosarcoma Alveolar rhabdomyosarcoma Ulbright et Epithehoid sarcoma af.171
Hall et al.
PI
Epithelioid sarcoma Epithelioid sarcoma
I5 Vulva
13 Perineum
Y X Y
Local excision, 197R 10 x X Local exciston, 1982 15 X IO Loctal excision, 1979 Local excision, 1947
Local excision, 1%7 1 Radical vulvectomy lnguinal lymphadenectomy, 1963 1.5 x 2.5 Radical vulvectomy, 1961 10 x 8 Incisional biopsy with gross residual disease ? Complete local excision ?
14 Perineum and left buttock
?
20 Perineum and perianal
?
55 Right labium majus
<5
55 Right labium majus 31 Left antero lateral vulva
<5
Initial biopsy with gross residual disease Complete excision
Incisional biopsy with gross restdual disease Local excision
Surgery for persistence or recurrence
Other therapy
Local excision x 4; Chemotherapy, 1978, 1979. 19w). 1981 radiotherapy Local excision X 3; Chemotherapy, 982, 1983, 1985 radiotherapy Local excision x 3; Chemotherapy 1981, 1982, 19XS Local excision, 1957 Radical vulvectomy. partial vaginectomy. and removal of contents of ischiorectal fossa, 1958 Abdominal perineal. partial vagmectomy, and radical vulvectomy. 1957 Multiple local excisions l948- 195R Radical vulvectomy. 1958 Excision left vaginal wall recurrence, 1961 -
Chemotherapy
Status Died Died Died
Died
Period 10 years 7.5 years 9 yea* IS
Metastases Pulmonary Pulmonary Pulmonary
Pulmonary years
-
Chemotherapy
Died
7.5 years
Liver
Died
16
Pulmonary YEiS
Chemotherapy -
-
Died
9
Alive
months 7.5 years
-
Alwe
9
-
Pulmonary
yean -
Right pelvic mass incision, recurrent vulvar lesion excision -
Intra-arterial chemotherapy, radiotherapy Radiotherapy
Died
4
Widespread months
Alive
12 years
Chemotherapy, radiotherapy
Died
7
-
Chemotherapy, radiotherapy
Alive
7.5 years
-
Chemotherapy, radiotherapy
Died
3
Radical vufvectomy and bilateral lymphadenectomy, 8 months after initial surgery; posterior pelvic exenteration 2 months after vulvectomy
-
Widespread months -
Vertebral months
Died
15
Pulmonary months
Chemotherapy
Wide local excision
-
Died
Pulmonary
8 months
?
Local excision, 196g
Excision left labium majus and groin, 1974; wide local excision and groin nodes dissection. 1974; palliative excision and debridement, 3978
Chemotherapy, radiotherapy
Died
Pulmonary
II years
TABLE
l-Continued Survival
Reference
Histology
Davos and Abel1 [5]
Leiomyosarcoma
Age
Location
84 Posterior fourchette and perineal b-3
Leiomyosarcoma kiomyosarcoma Leiomyosarcoma
Posterior fourchette Posterior 35 fourchette Barth01 in 49 gland area
Fibrosarcoma Dermatotibro sarcoma protuberans Malignant hemangioperi cytoma Malignant mesothelioma
Right labium majus Labium majus 38 Clitoreal area 16 Left labium majus 38
Embryonal stromal sarcoma
54
50
28
Left labium majus
Size (cm)
?
? ? ?
? 6 6 3x5
?
Initial
surgery
Surgery for persistence or recurrence
-
Local excision, 1945
Local excision, lY48 Local excision, 1970 Local excision, 1965
Local excision, 1936 Wide local excision, 1953 Local excision, 1941 Local excision, 1970
Local excision, 1967
Other therapy
Status Died
Period 6
Metastases Disseminated
months
Hemivulvectomy, 1949; hysterectomy and excision, vaginal mass, 1957
9 -
Died
years
Disseminated
years
-
5 Re-excision, 1970 Wide local excision, 1967 Re-excision, 1936; simple vulvectomy, 1936; wide local excision and bilateral inguinal node dissection, 1937
Alive 9 -
Alive
-
Died
16
Wide local excision, 1942
Radical vulvectomy and inguinal lymphadenectomy, 1967; wide local excision, 1967; resection pulmonary metastasis, 1968
years
months Disseminated 20
Alive
years
Alive
years 5
-
Alive
-
Alive
years
6 years
FIG. 5. Vulvar tumor in Case 4 showing tumor cells lining alveolar rhabdomyosarcoma. Hematoxylin and eosin stain, 150x . 389
-
19
-
BAKRI ET AL.
gins for patients with low-grade histologic types such as dermatofibrosarcoma protuberance and cases of small lesion size, while reserving the more radical surgery together with lymphnodal resection for casesof higher grade histological types and cases of large tumor size. The role of adjuvant chemotherapy and/or radiation therapy in the treatment of the locoregional disease remains to be established. REFERENCES 1. Kuller, J. A., Zucker, P. K., and Peng, T. C. Vulvar leiomyosarcoma in pregnancy, Am. 1. Obstet. Gynecol. 162, 164-166 (1990). 2. Tavassoh, F. A., and Norris, H. J. Smooth muscle tumors of the vulva, Obstet. Gynecol. 53, 213-217 (1979).
3. DiSaia, P. J., Rutlege, F., and Smith, J. P. Sarcoma of the vulva: Report of 12 patients, Obstet. Gynecof. 38, RIO-184 (1971). 4. Leake, J. F., Buscema, J., Cho, K. R., and Currie, J. L. Dermatofibrosarcoma protuberans of the vulva, Gynecol. Oncol. 4, 245249 (1990). 5. Davos, I., and Abell, M. R. Soft tissue sarcomas of the vulva, Gynecol. Oncol. 4, 70-86 (1976). 6. Copeland, L. J., Sneige, N., Stringer, C. A., Gershenson, D. M., Saul, P. B., and Kavahagh, J. J. Alveolar rhabdomyosarcoma of the female genitalia, Cancer 56, 849-855 (1985). 7. Ulbright, T. M., Brokaw, S. A., Stehman, F. B., and Roth, L. M. Epithelioid sarcoma of the vulva, evidence suggesting a more aggressive behavior than extra-genital epithelioid sarcoma, Cancer 52, 1462-1469 (1983). 8. Hall, D. J., Grimes, M. M., and Goplerud, D. R. Epithelioid sarcoma of the vulva, Gynecol. Oncol. 9, 237-246 (1980).
ONCOLOGY
46,
384-390 (1992)
CASE REPORT Vulvar Sarcoma: A Report of Four Cases YOUNES N. BAKRI,* Division
of Gynecologic
Oncology,
*Departments
M. AKHTAR,~ M. EL-SENOUSSI,$
AND
of Obstetrics and Gynecology, +Pathology, and Research Centre, Riyadh, Saudi Arabia
R.
WIERZBICKI$
and SOncology,
King Faisal Specialist Hospital
Received November 18. 1991
amycin, and DTIC were given, followed by wide local excision. She returned to Riyadh and received radiation comas are presented. Two patients had leiomyosarcoma in astherapy to the pelvis and vulva which was completed in sociation with pregnancy, a third patient had a leiomyosarcoma December 1982. Chemotherapy was resumed in January with epithelioid elements, and a fourth had alveolar rhabdo1983. myosarcoma. The natural history of the disease in the three leioIn January 1986, examination under anesthesia and myosarcomas (including the case with epithelioid elements) was characterized by an indolent protracted course and frequent local multiple vulvar and pelvic biopsies revealed no evidence recurrence, followed by distant fatal metastases.The patient with of disease. Follow-up in April 1987 manifested negative alveolar rhabdomyosarcoma is alive-with-disease. Surgery, che- chest X ray. However, in October 1987 chest X ray remotherapy, and radiotherapy achieved palliation rather than vealed multiple bilateral pulmonary metastases and local cure. Pregnancy did not seem to infhrence the prognosis. o wu right vulvar recurrence. She died at home in early 1988. The clinical profiles of four patients with primary vulvar sar-
Academic
Rcs.Inc.
INTRODUCTION
Case 2
Primary vulvar sarcomas constitute l-3% of all malignant vulvar neoplasms, less than 30 cases of all histologic types were reported until 1990,5 of which were associated with pregnancy [l]. Knowledge of the disease is limited to case reports and collective reviews rather than large series [2-51. The purpose of this report is to describe the clinical profiles of vulvar sarcomas in 4 patients.
CASE REPORTS Case I A 29-year-old patient, gravida 6 para 6, was well until she noticed a lump in the right labium majus, which slowly increased in size until it became 9 cm in largest diameter when first excised in late 1978. Pathology revealed leiomyosarcoma (Fig. 1). She underwent four excisions for local recurrence in the 3-year period between 1978-1981. First excision for recurrence was done 6 weeks after normal vaginal birth. In 1982 she was transferred to M. D. Anderson Hospital where two courses of Cytoxan, Adri-
The patient, a 41-year-old gravida 10 para 10, was well until mid 1981 when she noticed a lump in the left labium majus, which gradually increased in size. In March 1982 she had a normal vaginal birth but the vulvar mass remained without treatment until May 1982 when excision of a left vulvar lesion 10 x 8 cm was performed. A second local resection for recurrence was done in November 1982 and a third in February 1983. Histopathology revealed leiomyosarcoma grade III. In June 1985 a fourth local recurrence and resection of a 6-cm lesion was done, but chest X ray this time revealed multiple bilateral pulmonary metastases. She was asymptomatic then, and for that reason a decision was made to observe without further therapy. She developed chest pains in December 1986 for which palliative irradiation was given, with improvement of chest symptoms. Condition deteriorated in late 1987 and palliative DTIC was given, but in May 1988 she had paraplegia secondary to metastatic Tr fracture. She died in late 1988 (42 months after developing pulmonary metastases).
384 0090-8235~92$4.00 Copyright 0 1992 by Academic Press, Inc. All rights of reproduction in any form reserved.
CASE REPORT
385
Z. 1. High grade leiomyosarcoma featuring intertwining bundles of spindle-shaped cells with several mitoses and nuclear pleomoq bhism (Case 1). Hematoxylin and eosin stain, 200x.
of a lump in the left labium majus. The initial lesion was excised in 1979 and repeated excisions were done for The patient, a 17-year-old woman, was first seen at recurrence in 1981 and 1982; pathology material and recKFSH in October 1985 because of vulvar mass. She was ords of these excisions were not available. On admission well until 1977 when at age 9, she noticed the appearance to KFSH she complained of difficulty in walking and voiding because of a mass-pressure effect. Chest X ray revealed multiple bilateral pulmonary metastases. A wide local excision of a left vulvar 15 x lo-cm mass (Fig. 2) was performed, and pathologic examination of the mass revealed leiomyosarcoma, grade I, with focal epithelioid and mutinous changes (Figs. 3 and 4). Chemotherapy with vincristine, Adriamycin, and Cytoxan was started in November 1985 and was switched to vindesine, actinomycin D, and DTIC in February 1986. Patient died of metastases in June 1986. Case 3
Case 4
FIG. 2. Left vulvar mass lesion leiomyosarcoma (Case 3).
The patient, a 20-year-old unmarried woman, was well until November 1990 when she noticed a left vulvar swelling, followed a month later by a swelling of the left groin and pain in the left thigh. Upon referral to our hospital in March 1991 she was normal at general physical ex-
386
BAKRI ET AL,
FIG. 3. Bundles of spindle-shaped cells from well-differentiated leiomyosarcoma (Case 3). Hemotoxylin and eosin stain, 150x .
amination and had a left labium majus, vulvar mass 8 x 10 cm which was firm and nontender, together with 5 x 3 cm left inguinal lymph node enlargement. Workup revealed normal blood chemistries but CT of the pelvis and abdomen revealed extensive retroperitoneal paraaortic and pelvic lymph nodal enlargement with left hydroureter. She underwent a wide local excision of the vulvar mass and removal of grossly enlarged left inguinal lymph nodes. Histopathology revealed alveolar rhabdomyosarcoma of the left labium majus (Fig. 5) with metastases to inguinal lymph nodes. Adjuvant chemotherapy incorporating cyclophosphamide, Adriamycin, and vincristine (CAV) was given. Laparotomy for suspected left pelvic retroperitoneal mass after five CAV courses revealed fibrosis only, with microscopic metastatic involvement of the left internal iliac nodes. Paraaortic and common iliac nodes were negative. Postoperative pelvic external beam irradiation was started in February 1992. DISCUSSION The natural history of vulvar sarcoma is not clearly understood; this is related to the rarity of the vulvar
involvement by primary sarcomatous neoplasms, various histologic types, and the limited number of case reports, which make it difficult to draw meaningful conclusions, particularly because most reported cases had short-term follow-up at the time they were published. This is in contradistinction to soft tissue sarcomas originating from extragenital sites, mainly extremities, where the natural history of the disease is well known. Among published reports, the M. D. Anderson Hospital [3,6] and the University of Michigan Medical Center [4], managed the largest series of patients with vulvar sarcoma, and experienced the longest follow-up. Ulbright et al. [7] described 2 cases of vulvar epithelioid sarcomas and concluded that the disease, when originating from the vulva, manifests a more aggressive behavior than extragenital epithelioid sarcoma; however, our Case 3 who had leiomyosarcoma with epithelioid changes together with the 2 other cases of leiomyosarcoma had a behavior similar to extragenital disease, with indolent protracted course preceding fatal metastatic outcome. Two of our patients and 5 of the 30 cases reported in the literature had the disease in association with pregnancy, making it a relatively common association when compared with vulvar carcinomas; this
CASE REPORT
FIG. 4. An area within the tumor from Case 3, featuring epithelial cells in a myxoid background. Occasional multinucleated tumor cells are also present.
can be explained by the fact that sarcomas usually affect a younger age while vulvar carcinomas usually affect an older postmenopausal group. Cases reported in association with pregnancy were of variable histologic types and included 2 fibrosarcomas, 1 reticulum cell vs rhabdomysarcoma, 1 mixed mesodermal tumor, and 1 leiomyosarcoma [l]. In our 2 patients with leiomyosarcoma, pregnancy did not seem to influence survival. Unfortunately, almost all reports of vulvar sarcoma in association with pregnancy are weakened by the short-term follow-up and no valid conclusions could be drawn as to how pregnancy influences the disease outcome. In Case 4 the referral diagnosis and even our intraoperative frozen-section microscopic diagnosis were mistaken for poorly differentiated adenocarcinoma. This case supports the conclusion of Copeland et al. [6] that awareness of the typical microscopic picture of alveolar rhabdomyosarcoma is important for early diagnosis and treatment. Table 1 summarizes the clinical profiles of reported cases of vulvar sarcoma who had 5 years or more of follow-up. We excluded casespublished with short-term follow-up. Perhaps
personal communication with publishing authors or institutions in order to obtain updates on the status of those patients whose case reports were already published in peer-reviewed literature may yield long-term follow-up information on a number of patients adequate enough to help draw meaningful conclusions. Davos and Abel1 [5] believe that inadequate initial treatment leads to frequent local recurrences and recommend wide local excision or radical vulvectomy early in the disease, with bilateral lymph node resection for those sarcomas that have a potential for lymphogenous metastases. We believe, however, that the treatment should be individualized, to take into consideration the factors of tumor histologic type and grade, tumor size, location, and extent of locaregional and distant metastases. Review of the published cases with adequate follow-up information (Table 1) perhaps does not supply convincing evidence that radical surgery improves the final disease outcome. Although it is difficult and perhaps invalid to draw any conclusion from such a small number of reviewed cases, it is perhaps reasonable to recommend local excision with adequate surgical mar-
388
BAKRI
ET AL.
TABLE 1 Clinical Profiles of Patients with Vulvar Sarcoma and Long-Term Follow-up Survival Reference
Histology
Age
Location
Size (cm)
Initial
surgery
Present study Leiomyosarcoma grade III Leiomyosarcoma grade III Leiomyosarcoma with epithelioid elements
29 Right labium majus 41 Left labium majus I7 Left labium majus
DiSaia et al. Leiomyosarcoma
64 Labium majus
S x 6
Leiomyosarcoma
57 Labmm majus
5 x 6
Local excision, 1957
Leiomyosarcoma
56 Labium majus
2 x 3
Local excision, I948
Rhabdomyosarcoma Rhabdomyosarcoma
28 Labium majus 44 Labium majus
6
Neurotibrosarcoma
25 Labium majus 18 Lahtum majus
131
DiSaia et af. Alveolar rhabdomyo13.61 sarcoma Copeland et Alveolar al. (61 rhabdomyosalcoma Alveolar rhabdomyosarcoma Alveolar rhabdomyosarcoma Alveolar rhabdomyosarcoma Ulbright et Epithehoid sarcoma af.171
Hall et al.
PI
Epithelioid sarcoma Epithelioid sarcoma
I5 Vulva
13 Perineum
Y X Y
Local excision, 197R 10 x X Local exciston, 1982 15 X IO Loctal excision, 1979 Local excision, 1947
Local excision, 1%7 1 Radical vulvectomy lnguinal lymphadenectomy, 1963 1.5 x 2.5 Radical vulvectomy, 1961 10 x 8 Incisional biopsy with gross residual disease ? Complete local excision ?
14 Perineum and left buttock
?
20 Perineum and perianal
?
55 Right labium majus
<5
55 Right labium majus 31 Left antero lateral vulva
<5
Initial biopsy with gross residual disease Complete excision
Incisional biopsy with gross restdual disease Local excision
Surgery for persistence or recurrence
Other therapy
Local excision x 4; Chemotherapy, 1978, 1979. 19w). 1981 radiotherapy Local excision X 3; Chemotherapy, 982, 1983, 1985 radiotherapy Local excision x 3; Chemotherapy 1981, 1982, 19XS Local excision, 1957 Radical vulvectomy. partial vaginectomy. and removal of contents of ischiorectal fossa, 1958 Abdominal perineal. partial vagmectomy, and radical vulvectomy. 1957 Multiple local excisions l948- 195R Radical vulvectomy. 1958 Excision left vaginal wall recurrence, 1961 -
Chemotherapy
Status Died Died Died
Died
Period 10 years 7.5 years 9 yea* IS
Metastases Pulmonary Pulmonary Pulmonary
Pulmonary years
-
Chemotherapy
Died
7.5 years
Liver
Died
16
Pulmonary YEiS
Chemotherapy -
-
Died
9
Alive
months 7.5 years
-
Alwe
9
-
Pulmonary
yean -
Right pelvic mass incision, recurrent vulvar lesion excision -
Intra-arterial chemotherapy, radiotherapy Radiotherapy
Died
4
Widespread months
Alive
12 years
Chemotherapy, radiotherapy
Died
7
-
Chemotherapy, radiotherapy
Alive
7.5 years
-
Chemotherapy, radiotherapy
Died
3
Radical vufvectomy and bilateral lymphadenectomy, 8 months after initial surgery; posterior pelvic exenteration 2 months after vulvectomy
-
Widespread months -
Vertebral months
Died
15
Pulmonary months
Chemotherapy
Wide local excision
-
Died
Pulmonary
8 months
?
Local excision, 196g
Excision left labium majus and groin, 1974; wide local excision and groin nodes dissection. 1974; palliative excision and debridement, 3978
Chemotherapy, radiotherapy
Died
Pulmonary
II years
TABLE
l-Continued Survival
Reference
Histology
Davos and Abel1 [5]
Leiomyosarcoma
Age
Location
84 Posterior fourchette and perineal b-3
Leiomyosarcoma kiomyosarcoma Leiomyosarcoma
Posterior fourchette Posterior 35 fourchette Barth01 in 49 gland area
Fibrosarcoma Dermatotibro sarcoma protuberans Malignant hemangioperi cytoma Malignant mesothelioma
Right labium majus Labium majus 38 Clitoreal area 16 Left labium majus 38
Embryonal stromal sarcoma
54
50
28
Left labium majus
Size (cm)
?
? ? ?
? 6 6 3x5
?
Initial
surgery
Surgery for persistence or recurrence
-
Local excision, 1945
Local excision, lY48 Local excision, 1970 Local excision, 1965
Local excision, 1936 Wide local excision, 1953 Local excision, 1941 Local excision, 1970
Local excision, 1967
Other therapy
Status Died
Period 6
Metastases Disseminated
months
Hemivulvectomy, 1949; hysterectomy and excision, vaginal mass, 1957
9 -
Died
years
Disseminated
years
-
5 Re-excision, 1970 Wide local excision, 1967 Re-excision, 1936; simple vulvectomy, 1936; wide local excision and bilateral inguinal node dissection, 1937
Alive 9 -
Alive
-
Died
16
Wide local excision, 1942
Radical vulvectomy and inguinal lymphadenectomy, 1967; wide local excision, 1967; resection pulmonary metastasis, 1968
years
months Disseminated 20
Alive
years
Alive
years 5
-
Alive
-
Alive
years
6 years
FIG. 5. Vulvar tumor in Case 4 showing tumor cells lining alveolar rhabdomyosarcoma. Hematoxylin and eosin stain, 150x . 389
-
19
-
BAKRI ET AL.
gins for patients with low-grade histologic types such as dermatofibrosarcoma protuberance and cases of small lesion size, while reserving the more radical surgery together with lymphnodal resection for casesof higher grade histological types and cases of large tumor size. The role of adjuvant chemotherapy and/or radiation therapy in the treatment of the locoregional disease remains to be established. REFERENCES 1. Kuller, J. A., Zucker, P. K., and Peng, T. C. Vulvar leiomyosarcoma in pregnancy, Am. 1. Obstet. Gynecol. 162, 164-166 (1990). 2. Tavassoh, F. A., and Norris, H. J. Smooth muscle tumors of the vulva, Obstet. Gynecol. 53, 213-217 (1979).
3. DiSaia, P. J., Rutlege, F., and Smith, J. P. Sarcoma of the vulva: Report of 12 patients, Obstet. Gynecof. 38, RIO-184 (1971). 4. Leake, J. F., Buscema, J., Cho, K. R., and Currie, J. L. Dermatofibrosarcoma protuberans of the vulva, Gynecol. Oncol. 4, 245249 (1990). 5. Davos, I., and Abell, M. R. Soft tissue sarcomas of the vulva, Gynecol. Oncol. 4, 70-86 (1976). 6. Copeland, L. J., Sneige, N., Stringer, C. A., Gershenson, D. M., Saul, P. B., and Kavahagh, J. J. Alveolar rhabdomyosarcoma of the female genitalia, Cancer 56, 849-855 (1985). 7. Ulbright, T. M., Brokaw, S. A., Stehman, F. B., and Roth, L. M. Epithelioid sarcoma of the vulva, evidence suggesting a more aggressive behavior than extra-genital epithelioid sarcoma, Cancer 52, 1462-1469 (1983). 8. Hall, D. J., Grimes, M. M., and Goplerud, D. R. Epithelioid sarcoma of the vulva, Gynecol. Oncol. 9, 237-246 (1980).