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Sarcoma of the vulva
SARCOMA OF THE VULVA* PHINEAS BERNSTEIN, M.D.
NEW YORK CITY
T
HE high mortality and the certain fata outcome represented by a vuIvar sarcoma is we11 known. The case here described is unusual in that the patient is aIive and we11 four years after the tumor was removed. The absence of metastasis over this Iong period as we11 as the rarity of
this maIignancy warrant of facts which foIIow:
the presentation
S. K., an obese, 45 year old Russian born housewife, was admitted to the gynecoIogic ward, January 2, 1934. She had had five normaI pregnancies. Menses were always norma1 before the onset of the menopause one vear prior to admission. For sixteen years the patient had observed the slow growth of a nodule in the Ieft Iabium, unti1 it reached the size of a Iarge grapefruit. There had been periods of two to five years during which no enrargement occurred; in the intervaIs, however, the size increased and the mass became inflamed and uIcerated. Recently she had experienced difflcuIty in walking because of the tumor. There were no other complaints or symptoms. Examination. A firm, nodular, grapefruitsized mass was found broadIy attached to the Ieft vulva. (Fig. I.) The clitoris at the upper margin of attachment was concealed behind the mass. The fIattened Ieft labia1 fold surmounted the anteromedia1 surface. The overIying skin was tense, movable, and ulcerated on the inner aspect. The inguinal gIands were not enIarged. The genera1 physica findings were normal. Wassermann and Kahn tests were negative. Blood pressure was 128/78, hemoglobin 84 per cent, sedimentation rate seventy minutes. A diagnosis of fibroIipoma was made. Operation and Findings. Under gas, oxygen and ether anesthesia a IongitudinaI incision through the skin was made 2 inches IateraI to the IabiaI edge. The tumor was removed by both bIunt and sharp dissection. Its fossa compIeteIy covered the descending ramus of the pubis, reaching the vagina1 wall medialIy. The tumor and its capsure were removed compIeteIy * From the GynecologicaI
Service
after several Iarge vessels were Iigated, and the skin was then closed. (Operation was done by Dr. U. .J. SaImon.) Pathologic Report. The specimen was described as grapefruit-sized (IO X 8 cm.), moderateIy edematous, soft and pIiabIe, and gray-white in coIor. The capsule was thick and injected and contained several areas of hemorrhage. I arge gaping blood vessels were noted. When sectioned, the cut surface appeared glossy, edematous and white. It was smooth throughout except for several smaI1 Ioculi and a discrete, round yeIIow area, containing tinv, coalescent, irreguIar elevations, punctate ;n character. Microscopic sections were reported by Dr. PauI KIemperer as polymorphous ceI1 scarcoma of the vulva. Postoperatively, x-ray radiation, totaIIing 1200 R units was given directIy over the Ieft vuIva. It is now four years since operation and no compIaints referabIe to the origina condition have been reported by the patient. The chest, peIvic bones and Iower spine, recentIy x-rayed, reveaIed no metastasis.
DISCUSSION The literature contains about IOO reports of cases of malignancies of this category. The mortaIity is very high. FraenkeI’O states that the incidence of 0.01 per cent, inclusive of meIanosarcoma. E. Kehrer, in the Veit-StoeckeI Handbuch,13 coIIected from the Iiterature up to 1929 seventy-seven vuIvar sarcomata, excIuding melanosarcoma. In 62 per cent they arose from the Iabium majus, in I I .4 per cent from the Iabia minora. Sarcomata have aIso been found in the round Iigain BarthoIin ment,lg in fibromata,15116 eIephantiasis.5 gIands,2p12 and in vuIvar TaussigsI beIieves that many are derived from the fibrofatty substance of the vuIva rather than the fibrous eIement aIone.
of the Mt. Sinai Hospital, 591
New York City.
592
American Journd of Surgery
Bernstein-Sarcoma
HistoIogicaIIy, the type described are spindIe,” round,32,33 giant,2g and poIymorphous ceI1. lg Myxosarcoma,23’31 fibro,4 Iipofibro, 14130 and Iymphosarcoma,31 are
of Vulva
SEPTEhlBEH. ,939
may spread IocaIfy in the earIy stages. They differ from carcinoma and melanosarcoma in that the Iymphatic vesseIs are rareIy invoIved unti1 very late.
FIG. I. Sarcoma of left vdva.
reported. EndotheIiaI,6 peritheIiaI,25126,3 and angiomatous varieties are mentioned. BIair BeII,l in twenty-one cases, found the myxosarcoma most common. In a group of twenty-six coIIected cases, R. T. Frank6 states there were ten myxosarcomata, four fibro-, six spindle-, three poIymorphous, and one each of peri, endotheIia1, and angiosarcoma. Taussig31 feeIs that many of the round ceII sarcomata wouId now be caIIed Iymphosarcomata, in view of the recent changes in the maIignant tumor cIassification. The age incidence is agreed to be between 30 and 50 years. The average age of incidence is 40 years. However, H. S. Morgan20 reports one case in a chiId of sixteen years. R. T. Frank8 states that these tumors resembIe fibromyomata unti1 uIceration and infiltration takes pIace. They begin as smaI1 peduncuIated or sessiIe growth, and
LocaI metastases are found both earlx and Iate. Nebesky, 21 found extension in four cases of urethra1 sarcoma, secondariIy to the vuIva, vagina and inguina1 gIands. B. P. Watson’s case,31 was simiIar. Distant metastases are the rule, however, especially and aIso Iate in the after operation disease. Maas and 01shausen15 have reported a case with five year freedom from recurrence and Rhomberg24 an even Ionger period. The prognosis is always grave, since the disease is usuaIIy fata because of metastases to vita1 organs. As regards recurrence after excision, the prognosis is aIso bad. In five instances, E. Kehrer13 reported that gestation exercised no influence upon the growth of this tumor. Diagnosis is rareIy made cIinicaIIy. The microscopic picture is aIways necessary and fina1.
NEW SERIES VOL.XLV, No. 3
Bernstein-Sarcoma
Treatment. EarIy excision offers the only hope of cure. Recurrent Iocal Iesions shouId be repeatedIy excised. The vaIue of radium and x-ray seems quite questionabIe. SurgicaI procedures empIoyed are usuaIIy simpIe and not attended by serious sequelae. SUMMARY I. Sarcoma of the vuIva is a rare disease : about IOO cases are reported in the entire Iiterature. 2. The mortaIity is exceedingIy high. 3. A brief rC.sumi: of the characteristics of this maIignancy is given. 4. The case presented is unusua1 in that four years after operation, there are (I) no compIaints referabIe to the original tumor; (2) x-rays revea1 no metastasis, (3) there are no nodes paIpabIe externaIIy. The patient is cIinicaIIy cured. 5. A survey of the Iimited Iiterature and a brief discussion of the cIinica1 course of the disease is aIso given. REFERENCES I. BELL, B. J. Obst. e?? Gynaec. hit. 2. 3. 4. 5. 6. 7.
Emp.,
12: 275,
1907. BLUHM. Arch. j. Gynaek., 71: I, 1904. CARBONE. Un case di sarcoma deIIa v&a a tipo periteIiaIe. Arch. obstetr., 13: 84, 1926. CARUSO. Sot. ital. di ostet. e ginec., vo1. 2, 1896. Zentralbl. j. Gyniik., 20: 908, 1896. CHROBAK. Tumor der Klitoris (EIephantiasis). Wien. klin. Wcbnscbr., 1895. DELLE CHIAGE. Ann. d. ostet. e ginec., 29: 451, 1907. FLATAU. Zentralbl. j. Gyniik., 27: ggo, rgo3.
of VuIva
American ~~~~~~~of
surgery 593
8.
FRANK, ROBERT. GynecoIogicaI and Obstetrica PathoIogy. New York, 1922. D. AppletonCentury Co., p. 125. g. FRANCKE. Vircbow’s Arch., 154: 363. (First case.) IO. FRAENKEL. Zetttratbf. j. Cyniik., 28: 1025, 1904. I I. HARTMANN.Zentralb1.j. Gyniik., vol. 32, no. 2, 1908. 12. HOFMEIER. Schroeder’s Handbuch d. Frauenkrankheiten. 14th ed., 1908, p. 65. 13. KEHRER, E. In Veit-StoeckeI. Handbuch der GyniikoIogie 5, Part I, pp. 496-503. (Literature.) 14. KLEEBERG.Petersburg med. Ztscbr., 15: Part 2, 1869. 15. LICHTENSTEIN.Gyniik. Ges., Leipzig, 1908. 16. LICHTENSTEIN.Beitrag zur Frage der Kontagiositlt med. des CondyIoma acuminata. Miincben. Wcbnscbr., p. 270, 1922. 17. LYNCK, FRANK. PeIvic NeonIasms. New York. 1024. _ _ D. Appleton-Century Co., pp. 3641. d. Carcinoma u. 18. MAAS. Ueber d. MaIignitat Sarkome an der ausseren weiblichen Genitalien. Inaug-Diss., HaIIe, 1887. 19. MALY. Arch. f. GynZik., 76: 175, 1905. 20. MORGAN, H. S. Am. J. Obst. u Gynec., 15: 861-865 (June) 1928. 21. NEBESKY. Arch. j. Gyniik., 93: 539, 191 I. 22. NETZER. Zentralbl., 41: 413, 1925. (Literature on sarcoma vulvae.) 23. ROBB, H. Myxosarcoma of clitoris. Jobns Hopkins Hosp. Rep., 2: 231, rgoo. 24. RHOMBERG.Zentralbl. j. Gyn?ik., 39: 780, 1915. cIitoris. Arch. j. 25. SCHMIDLECHNER. Carcinoma Gyntik., 74: 195, 1904. 26. SCHMIDLECHNER.Arch. f, GynGk., 74: 200, rgo4. 27. SCHMIDT,H. R. SpindeIzeIIensarkom der weibIichen Urethra. Zentralbl. j. Gynaek., 50: 3 I 22, 1926. 28. SHAW, A. F. B. J. Patb. fl Baet., 43: 277. 1936. 29. SZILI. Beitr. z. klin. Cbir., 31: 734. 30. TAUSSIG, F. J. Diseases of the Vulva. New York, 1930. D. Appleton-Century Co., p. 176. 31. TAUSSIG, B. P. Am. J. Obst. @ Gynec., 33: 1017
(June)
1937.
32. WATSON, B. F. Am. J. Obst., 6g: 4, 1914. (Literature has 52 undoubted cases.) 33. VON WINCKEL. PathoIogie der weiblichen Sexualorgane. Leipzig, 1878. 34. VON WINCKEL. PathoIogie der weibIichen SexuaIorgane. 1881, p. 275. (CoIIected 54 cases.)
NEW YORK CITY
T
HE high mortality and the certain fata outcome represented by a vuIvar sarcoma is we11 known. The case here described is unusual in that the patient is aIive and we11 four years after the tumor was removed. The absence of metastasis over this Iong period as we11 as the rarity of
this maIignancy warrant of facts which foIIow:
the presentation
S. K., an obese, 45 year old Russian born housewife, was admitted to the gynecoIogic ward, January 2, 1934. She had had five normaI pregnancies. Menses were always norma1 before the onset of the menopause one vear prior to admission. For sixteen years the patient had observed the slow growth of a nodule in the Ieft Iabium, unti1 it reached the size of a Iarge grapefruit. There had been periods of two to five years during which no enrargement occurred; in the intervaIs, however, the size increased and the mass became inflamed and uIcerated. Recently she had experienced difflcuIty in walking because of the tumor. There were no other complaints or symptoms. Examination. A firm, nodular, grapefruitsized mass was found broadIy attached to the Ieft vulva. (Fig. I.) The clitoris at the upper margin of attachment was concealed behind the mass. The fIattened Ieft labia1 fold surmounted the anteromedia1 surface. The overIying skin was tense, movable, and ulcerated on the inner aspect. The inguinal gIands were not enIarged. The genera1 physica findings were normal. Wassermann and Kahn tests were negative. Blood pressure was 128/78, hemoglobin 84 per cent, sedimentation rate seventy minutes. A diagnosis of fibroIipoma was made. Operation and Findings. Under gas, oxygen and ether anesthesia a IongitudinaI incision through the skin was made 2 inches IateraI to the IabiaI edge. The tumor was removed by both bIunt and sharp dissection. Its fossa compIeteIy covered the descending ramus of the pubis, reaching the vagina1 wall medialIy. The tumor and its capsure were removed compIeteIy * From the GynecologicaI
Service
after several Iarge vessels were Iigated, and the skin was then closed. (Operation was done by Dr. U. .J. SaImon.) Pathologic Report. The specimen was described as grapefruit-sized (IO X 8 cm.), moderateIy edematous, soft and pIiabIe, and gray-white in coIor. The capsule was thick and injected and contained several areas of hemorrhage. I arge gaping blood vessels were noted. When sectioned, the cut surface appeared glossy, edematous and white. It was smooth throughout except for several smaI1 Ioculi and a discrete, round yeIIow area, containing tinv, coalescent, irreguIar elevations, punctate ;n character. Microscopic sections were reported by Dr. PauI KIemperer as polymorphous ceI1 scarcoma of the vulva. Postoperatively, x-ray radiation, totaIIing 1200 R units was given directIy over the Ieft vuIva. It is now four years since operation and no compIaints referabIe to the origina condition have been reported by the patient. The chest, peIvic bones and Iower spine, recentIy x-rayed, reveaIed no metastasis.
DISCUSSION The literature contains about IOO reports of cases of malignancies of this category. The mortaIity is very high. FraenkeI’O states that the incidence of 0.01 per cent, inclusive of meIanosarcoma. E. Kehrer, in the Veit-StoeckeI Handbuch,13 coIIected from the Iiterature up to 1929 seventy-seven vuIvar sarcomata, excIuding melanosarcoma. In 62 per cent they arose from the Iabium majus, in I I .4 per cent from the Iabia minora. Sarcomata have aIso been found in the round Iigain BarthoIin ment,lg in fibromata,15116 eIephantiasis.5 gIands,2p12 and in vuIvar TaussigsI beIieves that many are derived from the fibrofatty substance of the vuIva rather than the fibrous eIement aIone.
of the Mt. Sinai Hospital, 591
New York City.
592
American Journd of Surgery
Bernstein-Sarcoma
HistoIogicaIIy, the type described are spindIe,” round,32,33 giant,2g and poIymorphous ceI1. lg Myxosarcoma,23’31 fibro,4 Iipofibro, 14130 and Iymphosarcoma,31 are
of Vulva
SEPTEhlBEH. ,939
may spread IocaIfy in the earIy stages. They differ from carcinoma and melanosarcoma in that the Iymphatic vesseIs are rareIy invoIved unti1 very late.
FIG. I. Sarcoma of left vdva.
reported. EndotheIiaI,6 peritheIiaI,25126,3 and angiomatous varieties are mentioned. BIair BeII,l in twenty-one cases, found the myxosarcoma most common. In a group of twenty-six coIIected cases, R. T. Frank6 states there were ten myxosarcomata, four fibro-, six spindle-, three poIymorphous, and one each of peri, endotheIia1, and angiosarcoma. Taussig31 feeIs that many of the round ceII sarcomata wouId now be caIIed Iymphosarcomata, in view of the recent changes in the maIignant tumor cIassification. The age incidence is agreed to be between 30 and 50 years. The average age of incidence is 40 years. However, H. S. Morgan20 reports one case in a chiId of sixteen years. R. T. Frank8 states that these tumors resembIe fibromyomata unti1 uIceration and infiltration takes pIace. They begin as smaI1 peduncuIated or sessiIe growth, and
LocaI metastases are found both earlx and Iate. Nebesky, 21 found extension in four cases of urethra1 sarcoma, secondariIy to the vuIva, vagina and inguina1 gIands. B. P. Watson’s case,31 was simiIar. Distant metastases are the rule, however, especially and aIso Iate in the after operation disease. Maas and 01shausen15 have reported a case with five year freedom from recurrence and Rhomberg24 an even Ionger period. The prognosis is always grave, since the disease is usuaIIy fata because of metastases to vita1 organs. As regards recurrence after excision, the prognosis is aIso bad. In five instances, E. Kehrer13 reported that gestation exercised no influence upon the growth of this tumor. Diagnosis is rareIy made cIinicaIIy. The microscopic picture is aIways necessary and fina1.
NEW SERIES VOL.XLV, No. 3
Bernstein-Sarcoma
Treatment. EarIy excision offers the only hope of cure. Recurrent Iocal Iesions shouId be repeatedIy excised. The vaIue of radium and x-ray seems quite questionabIe. SurgicaI procedures empIoyed are usuaIIy simpIe and not attended by serious sequelae. SUMMARY I. Sarcoma of the vuIva is a rare disease : about IOO cases are reported in the entire Iiterature. 2. The mortaIity is exceedingIy high. 3. A brief rC.sumi: of the characteristics of this maIignancy is given. 4. The case presented is unusua1 in that four years after operation, there are (I) no compIaints referabIe to the original tumor; (2) x-rays revea1 no metastasis, (3) there are no nodes paIpabIe externaIIy. The patient is cIinicaIIy cured. 5. A survey of the Iimited Iiterature and a brief discussion of the cIinica1 course of the disease is aIso given. REFERENCES I. BELL, B. J. Obst. e?? Gynaec. hit. 2. 3. 4. 5. 6. 7.
Emp.,
12: 275,
1907. BLUHM. Arch. j. Gynaek., 71: I, 1904. CARBONE. Un case di sarcoma deIIa v&a a tipo periteIiaIe. Arch. obstetr., 13: 84, 1926. CARUSO. Sot. ital. di ostet. e ginec., vo1. 2, 1896. Zentralbl. j. Gyniik., 20: 908, 1896. CHROBAK. Tumor der Klitoris (EIephantiasis). Wien. klin. Wcbnscbr., 1895. DELLE CHIAGE. Ann. d. ostet. e ginec., 29: 451, 1907. FLATAU. Zentralbl. j. Gyniik., 27: ggo, rgo3.
of VuIva
American ~~~~~~~of
surgery 593
8.
FRANK, ROBERT. GynecoIogicaI and Obstetrica PathoIogy. New York, 1922. D. AppletonCentury Co., p. 125. g. FRANCKE. Vircbow’s Arch., 154: 363. (First case.) IO. FRAENKEL. Zetttratbf. j. Cyniik., 28: 1025, 1904. I I. HARTMANN.Zentralb1.j. Gyniik., vol. 32, no. 2, 1908. 12. HOFMEIER. Schroeder’s Handbuch d. Frauenkrankheiten. 14th ed., 1908, p. 65. 13. KEHRER, E. In Veit-StoeckeI. Handbuch der GyniikoIogie 5, Part I, pp. 496-503. (Literature.) 14. KLEEBERG.Petersburg med. Ztscbr., 15: Part 2, 1869. 15. LICHTENSTEIN.Gyniik. Ges., Leipzig, 1908. 16. LICHTENSTEIN.Beitrag zur Frage der Kontagiositlt med. des CondyIoma acuminata. Miincben. Wcbnscbr., p. 270, 1922. 17. LYNCK, FRANK. PeIvic NeonIasms. New York. 1024. _ _ D. Appleton-Century Co., pp. 3641. d. Carcinoma u. 18. MAAS. Ueber d. MaIignitat Sarkome an der ausseren weiblichen Genitalien. Inaug-Diss., HaIIe, 1887. 19. MALY. Arch. f. GynZik., 76: 175, 1905. 20. MORGAN, H. S. Am. J. Obst. u Gynec., 15: 861-865 (June) 1928. 21. NEBESKY. Arch. j. Gyniik., 93: 539, 191 I. 22. NETZER. Zentralbl., 41: 413, 1925. (Literature on sarcoma vulvae.) 23. ROBB, H. Myxosarcoma of clitoris. Jobns Hopkins Hosp. Rep., 2: 231, rgoo. 24. RHOMBERG.Zentralbl. j. Gyn?ik., 39: 780, 1915. cIitoris. Arch. j. 25. SCHMIDLECHNER. Carcinoma Gyntik., 74: 195, 1904. 26. SCHMIDLECHNER.Arch. f, GynGk., 74: 200, rgo4. 27. SCHMIDT,H. R. SpindeIzeIIensarkom der weibIichen Urethra. Zentralbl. j. Gynaek., 50: 3 I 22, 1926. 28. SHAW, A. F. B. J. Patb. fl Baet., 43: 277. 1936. 29. SZILI. Beitr. z. klin. Cbir., 31: 734. 30. TAUSSIG, F. J. Diseases of the Vulva. New York, 1930. D. Appleton-Century Co., p. 176. 31. TAUSSIG, B. P. Am. J. Obst. @ Gynec., 33: 1017
(June)
1937.
32. WATSON, B. F. Am. J. Obst., 6g: 4, 1914. (Literature has 52 undoubted cases.) 33. VON WINCKEL. PathoIogie der weiblichen Sexualorgane. Leipzig, 1878. 34. VON WINCKEL. PathoIogie der weibIichen SexuaIorgane. 1881, p. 275. (CoIIected 54 cases.)