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Leiomyosarcoma of the vulva
GYNECOLOGIC
ONCOLOGY
10,
350-355 (1980)
CASE REPORT Leiomyosarcoma
of the Vulva
PIERREAUDET-LAPOINTE, M.D., FRCS(C),*al FRANCOISPAQUIN, M.D., FRCP(C),t MARIE-JOSEEGUEFURD, M.D.,t ALBERT CHARBONNEAU, M.D.,* FRANCISMETHOT, M.D.,* AND GUY MORAND, M.D., FRCS(C)S *Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, and TDepartment of Pathology, Hdpital Notre-Dame et Universit 6 de Mont&al, Montkal, and SDepartment of Obstetrics and Gynecology, Hbpital St-E&be, Joliette, Quebec, Canada Received April 19, 1979 Leiomyosarcoma of the vulva is a rare disease and 15cases only have been reported in the literature. On account of this rarity, we report a case that we had the opportunity to treat recently and we present a brief review of the literature.
Sarcomas of the vulva are rare tumors. In fact, only about 100 cases have been reported in the world literature (8). They represent between 1.5 and 3% of the malignant neoplasms of the vulva [3-5,7,111, which constitute from 3.5 to 5% of all cancers of the female genital tract [3, I I, 13,161. We recently had the opportunity to treat a leiomyosarcoma of the vulva and it is the case we will now describe. A.M. (H.N.D. 666-541) is a 48-year-old woman, G,, P5, A,, who noticed in July 1976, a nodule in her left labium majus. The unpainful mass had grown rapidly and about 2 months after, measured 2 x 4 cm. At that time, she underwent simple tumorectomy. In January 1978, she noticed local recurrence of the tumor which at that time was extending to the left inguinal region and measured 7 x 2 cm, and again she had a tumorectomy. In July 1978, the patient had a second recurrence and was then referred to our hospital where investigation was completed. Examination at that time showed a patient to be in excellent physical condition. There was diffuse edema of the vulva at the site of the previous surgery and a 3-cm lump was palpable in the left labium majus. Lymphography, I.V.P., bone scan, hepatic scan, barium enema, complete blood count, SMAC, urine analysis and culture, coagulation tests, lung X ray, left prescalenic node biopsy were all negative. On August 2, 1978, we operated on the patient. The surgery consisted of a radical vulvectomy with bilateral inguinal lymph node resection. The postopera1 Address reprint requests to Pierre Audet-Lapointe, M.D., FRCS(C), Head, Division of Gynecology-Oncology, Department of Obstetrics and Gynecology, Hopitat Notre-Dame, C.P. Box 1560, Montreal, P.Q., Canada H2L 4K8. 350 0090-8258/80/060350-06$0 1.00/O Copyright All rights
@ 1980 by Academic Ress, Inc. of reproduction in any form reserved.
LEIOMYOSARCOMA
FIG. 1.
Proliferation
of fusiform
OF THE VULVA
cells grouped in bundles. HPS, x 95.
351
AUDET-LAPOINTE
352
ET AL.
--FIG.
~
2. Malignant-appearing nuclei with two mitotic figures in the field. HPS,
X
380.
LEIOMYOSARCOMA
OF THE VULVA
FIG. 3. Electron photomicrograph of smooth muscle tumor cells.
353
x
8500.
354
AUDET-LAPOINTE
ET AL.
tive period was complicated by a little necrosis dehiscence of the skin over the pubis, which was corrected on August 29, by a cutaneous graft and this graft healed completely. Two years after surgery, the patient is still free of disease. The specimens of both tumorectomies and radical vulvectomy showed a illdefined mesenchymal tumor made of fusiform cells with elongated nuclei, and fairly abundant fibrillated acidophilic cytoplasm (Fig. 1). The cells were grouped in bundles oriented in all directions. Nuclei.were hyperchromatic and the mitotic count showed 1.S mitoses per high-power field (Fig.2). The electron microscopic study demonstrated that the tumor cells contained in their cytoplasm myofibrils and dense bodies; the cytoplasmic membrane showed micropinocytic vesicles and a segmental basal lamina (Fig. 3). Both the histology and the electron microscopy were consistent with a leiomyosarcoma grade 1. Leiomyosarcomas of the vulva are very rare and account for about 1% of malignant tumors of the vulva [19,20]: only 15 such cases have been reported [4,5,14,19,20]. Fibrosarcomas [5,6, 11,211, lymphomas [2,11,16,20,2 I], rhabdomyosarcomas [5,20], neurofibrosarcomas [S,l I], and epithelioid sarcomas [4,7,15,19] have also been published. Sarcomas of the vulva occur most frequently in the majus labium, followed in frequency by the clitoris and the periurethral region [1,4,5,9]. They appear at a younger age, third and fourth decade [3,10], as compared to the carcinomas, sixth and seventh decade [9,16]. Parity does not seem to be a predisposing factor [8,12]. Patients generally complain of a nodule which most frequently has a benign looking appearance and only later on, will the lesion begin to infiltrate the skin and the deep fascias with secondary signs and symptoms. Leiomyosarcoma has a tendency to reappear locally and therefore the initial treatment should be radical surgery. Indeed after recurrence, the frequency of metastatic dissemination, mainly hematogenous, are much higher. Radiotherapy does not seem to alter the evolution of the lesion [ 11,16- 181,and in most cases chemotherapy was very deceptive. Only DiSaia [5] using triple chemotherapy (vincristine, actinomycine D, and cyclophosphamide) seemed to achieve some good results in controlling metastases. REFERENCES 1. Blaustein, A. Pathology of the female genital tract, Springer-Verlag, New York (1977). 2. Buckingham, J. C., and McClure, J. H. Reticulum cell sarcoma of the vulva: Report of a case, Obstet. Gynecol. 6, 138-143 (1955). 3. Cramer, D. W., and Cutler, S. J. lncidnece and histopathology of malignancies of the female genital organs in the United States, Amer. J. Obstet. Gynecof. 118, 443-460 (1974). 4. Davos, I., and Abel), M. R. Soft tissue sarcoma of the vulva, Gynecol. Oncol. 4, 70-86 (1976). 5. DiSaia, P.J., Rutledge, R., and Smith, J.P. Sarcoma of the vulva, Obstet. Gynecol. 38, 180-184 (1971). 6. Folsome, C. E. Benign and malignant tumours of the vulva, J. Amer. Med. Assoc. 114, 1499- 1503 (1940). 7. Gallup, D. G., Abell, M. R., and Morhey, G. W. Epithelioid sarcoma of the vulva, Obstet. Gynecol. 48, 145-175 (1976). 8. Gompel, C., and Silverberg, S. Pathology in gynecology and obstetrics, Lippincott, Philadelphia, 2nd ed. (1977). 9. Hertig. A. T., and Gore H. Tumors of the female sex organs, Parr 2, AFIP, Washington, D.C. (1960).
LEIOMYOSARCOMA
OF THE VULVA
355
IO. Kehrer, E. De vulva and ilre erkrankunger, in Handbach dn gynakalogie, Vol. V, Munich, 3rd ed. (1929). I I. Lundwall, F. Cancer of vulva: A clinical review, Acta Radio/. Suppl. 208, l-326 (1961). 12. Novak, E., Seegar, G. A., and Jones, H. W. Novak’s textbook ofgynecology, Williams&Wilkins, Baltimore, 9th ed. (1976). 13. Palmer, J. P., Sadugor, M.G., and Reinhard, M. C. Carcinoma of the vulva, Surg. Gynecol. Obstet. 88, 435-440 (1949). 14. Phelan, J. T., Sherer, W., and Mesa, P. Malignant smooth-muscle tumours (leiomyosarcomas) of soft tissue origin, N. Engl. J. Med. 226, 1027-1030 (1962). IS. Piver, S., Tsukada, Y., and Barlow, J. Epithelioid sarcoma of the vulva, Obstet. Gynecol. 40, 839-842 (1972). 16. Ramioul, H., Damiean-Gillet, and Groulard, J. Un nouveau cas de sarcome vulvaire (reticuloendotheliome malin), Gynaecologia 133, 74-78 (1952). 17. Smith, F. R., and Pollack, R. S. Carcinoma of the vulva, Surg. Gynecol. Obstet. 84,78-84 (1947). 18. Taussig, F. J. Sarcoma of the vulva, Amer. J. Obstet. Gynecol. 33, 1017-1026 (1937). 19. Tavassoli, F. A., and Norris, H. J. Smooth muscle tumors of the vulva, Obster. Gynecol. 53, 213-217 (1979). 20. Witt, M. J. Sarcoma of the vulva, Proc. Roy. Sot. Med., 60, 998-999 (1%7). 21. Woodruff, J. D., and Brack, C. B. Unusual malignancies of the vulva-urethral regions, Obsfet. Gynecol. 12, 677-686 (1958).
ONCOLOGY
10,
350-355 (1980)
CASE REPORT Leiomyosarcoma
of the Vulva
PIERREAUDET-LAPOINTE, M.D., FRCS(C),*al FRANCOISPAQUIN, M.D., FRCP(C),t MARIE-JOSEEGUEFURD, M.D.,t ALBERT CHARBONNEAU, M.D.,* FRANCISMETHOT, M.D.,* AND GUY MORAND, M.D., FRCS(C)S *Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, and TDepartment of Pathology, Hdpital Notre-Dame et Universit 6 de Mont&al, Montkal, and SDepartment of Obstetrics and Gynecology, Hbpital St-E&be, Joliette, Quebec, Canada Received April 19, 1979 Leiomyosarcoma of the vulva is a rare disease and 15cases only have been reported in the literature. On account of this rarity, we report a case that we had the opportunity to treat recently and we present a brief review of the literature.
Sarcomas of the vulva are rare tumors. In fact, only about 100 cases have been reported in the world literature (8). They represent between 1.5 and 3% of the malignant neoplasms of the vulva [3-5,7,111, which constitute from 3.5 to 5% of all cancers of the female genital tract [3, I I, 13,161. We recently had the opportunity to treat a leiomyosarcoma of the vulva and it is the case we will now describe. A.M. (H.N.D. 666-541) is a 48-year-old woman, G,, P5, A,, who noticed in July 1976, a nodule in her left labium majus. The unpainful mass had grown rapidly and about 2 months after, measured 2 x 4 cm. At that time, she underwent simple tumorectomy. In January 1978, she noticed local recurrence of the tumor which at that time was extending to the left inguinal region and measured 7 x 2 cm, and again she had a tumorectomy. In July 1978, the patient had a second recurrence and was then referred to our hospital where investigation was completed. Examination at that time showed a patient to be in excellent physical condition. There was diffuse edema of the vulva at the site of the previous surgery and a 3-cm lump was palpable in the left labium majus. Lymphography, I.V.P., bone scan, hepatic scan, barium enema, complete blood count, SMAC, urine analysis and culture, coagulation tests, lung X ray, left prescalenic node biopsy were all negative. On August 2, 1978, we operated on the patient. The surgery consisted of a radical vulvectomy with bilateral inguinal lymph node resection. The postopera1 Address reprint requests to Pierre Audet-Lapointe, M.D., FRCS(C), Head, Division of Gynecology-Oncology, Department of Obstetrics and Gynecology, Hopitat Notre-Dame, C.P. Box 1560, Montreal, P.Q., Canada H2L 4K8. 350 0090-8258/80/060350-06$0 1.00/O Copyright All rights
@ 1980 by Academic Ress, Inc. of reproduction in any form reserved.
LEIOMYOSARCOMA
FIG. 1.
Proliferation
of fusiform
OF THE VULVA
cells grouped in bundles. HPS, x 95.
351
AUDET-LAPOINTE
352
ET AL.
--FIG.
~
2. Malignant-appearing nuclei with two mitotic figures in the field. HPS,
X
380.
LEIOMYOSARCOMA
OF THE VULVA
FIG. 3. Electron photomicrograph of smooth muscle tumor cells.
353
x
8500.
354
AUDET-LAPOINTE
ET AL.
tive period was complicated by a little necrosis dehiscence of the skin over the pubis, which was corrected on August 29, by a cutaneous graft and this graft healed completely. Two years after surgery, the patient is still free of disease. The specimens of both tumorectomies and radical vulvectomy showed a illdefined mesenchymal tumor made of fusiform cells with elongated nuclei, and fairly abundant fibrillated acidophilic cytoplasm (Fig. 1). The cells were grouped in bundles oriented in all directions. Nuclei.were hyperchromatic and the mitotic count showed 1.S mitoses per high-power field (Fig.2). The electron microscopic study demonstrated that the tumor cells contained in their cytoplasm myofibrils and dense bodies; the cytoplasmic membrane showed micropinocytic vesicles and a segmental basal lamina (Fig. 3). Both the histology and the electron microscopy were consistent with a leiomyosarcoma grade 1. Leiomyosarcomas of the vulva are very rare and account for about 1% of malignant tumors of the vulva [19,20]: only 15 such cases have been reported [4,5,14,19,20]. Fibrosarcomas [5,6, 11,211, lymphomas [2,11,16,20,2 I], rhabdomyosarcomas [5,20], neurofibrosarcomas [S,l I], and epithelioid sarcomas [4,7,15,19] have also been published. Sarcomas of the vulva occur most frequently in the majus labium, followed in frequency by the clitoris and the periurethral region [1,4,5,9]. They appear at a younger age, third and fourth decade [3,10], as compared to the carcinomas, sixth and seventh decade [9,16]. Parity does not seem to be a predisposing factor [8,12]. Patients generally complain of a nodule which most frequently has a benign looking appearance and only later on, will the lesion begin to infiltrate the skin and the deep fascias with secondary signs and symptoms. Leiomyosarcoma has a tendency to reappear locally and therefore the initial treatment should be radical surgery. Indeed after recurrence, the frequency of metastatic dissemination, mainly hematogenous, are much higher. Radiotherapy does not seem to alter the evolution of the lesion [ 11,16- 181,and in most cases chemotherapy was very deceptive. Only DiSaia [5] using triple chemotherapy (vincristine, actinomycine D, and cyclophosphamide) seemed to achieve some good results in controlling metastases. REFERENCES 1. Blaustein, A. Pathology of the female genital tract, Springer-Verlag, New York (1977). 2. Buckingham, J. C., and McClure, J. H. Reticulum cell sarcoma of the vulva: Report of a case, Obstet. Gynecol. 6, 138-143 (1955). 3. Cramer, D. W., and Cutler, S. J. lncidnece and histopathology of malignancies of the female genital organs in the United States, Amer. J. Obstet. Gynecof. 118, 443-460 (1974). 4. Davos, I., and Abel), M. R. Soft tissue sarcoma of the vulva, Gynecol. Oncol. 4, 70-86 (1976). 5. DiSaia, P.J., Rutledge, R., and Smith, J.P. Sarcoma of the vulva, Obstet. Gynecol. 38, 180-184 (1971). 6. Folsome, C. E. Benign and malignant tumours of the vulva, J. Amer. Med. Assoc. 114, 1499- 1503 (1940). 7. Gallup, D. G., Abell, M. R., and Morhey, G. W. Epithelioid sarcoma of the vulva, Obstet. Gynecol. 48, 145-175 (1976). 8. Gompel, C., and Silverberg, S. Pathology in gynecology and obstetrics, Lippincott, Philadelphia, 2nd ed. (1977). 9. Hertig. A. T., and Gore H. Tumors of the female sex organs, Parr 2, AFIP, Washington, D.C. (1960).
LEIOMYOSARCOMA
OF THE VULVA
355
IO. Kehrer, E. De vulva and ilre erkrankunger, in Handbach dn gynakalogie, Vol. V, Munich, 3rd ed. (1929). I I. Lundwall, F. Cancer of vulva: A clinical review, Acta Radio/. Suppl. 208, l-326 (1961). 12. Novak, E., Seegar, G. A., and Jones, H. W. Novak’s textbook ofgynecology, Williams&Wilkins, Baltimore, 9th ed. (1976). 13. Palmer, J. P., Sadugor, M.G., and Reinhard, M. C. Carcinoma of the vulva, Surg. Gynecol. Obstet. 88, 435-440 (1949). 14. Phelan, J. T., Sherer, W., and Mesa, P. Malignant smooth-muscle tumours (leiomyosarcomas) of soft tissue origin, N. Engl. J. Med. 226, 1027-1030 (1962). IS. Piver, S., Tsukada, Y., and Barlow, J. Epithelioid sarcoma of the vulva, Obstet. Gynecol. 40, 839-842 (1972). 16. Ramioul, H., Damiean-Gillet, and Groulard, J. Un nouveau cas de sarcome vulvaire (reticuloendotheliome malin), Gynaecologia 133, 74-78 (1952). 17. Smith, F. R., and Pollack, R. S. Carcinoma of the vulva, Surg. Gynecol. Obstet. 84,78-84 (1947). 18. Taussig, F. J. Sarcoma of the vulva, Amer. J. Obstet. Gynecol. 33, 1017-1026 (1937). 19. Tavassoli, F. A., and Norris, H. J. Smooth muscle tumors of the vulva, Obster. Gynecol. 53, 213-217 (1979). 20. Witt, M. J. Sarcoma of the vulva, Proc. Roy. Sot. Med., 60, 998-999 (1%7). 21. Woodruff, J. D., and Brack, C. B. Unusual malignancies of the vulva-urethral regions, Obsfet. Gynecol. 12, 677-686 (1958).