Inr. 1. Radiation 0 Pergmon
Oncology Press Inc.,
Biol. Phys., Vol. 4, pp. 705-708 1978. Printed in the U S.A.
0 Current Concepts
03@-3016/78/0801-0705/$02.00/0
in Cancer
SARCOMAS RAFFAELE
OF THE BREAST LATTES, M.D.
Professor of Surgical Pathology, College of Physicians and Surgeons, Columbia University, Sarcomas
of the breast
The term, sarcoma of the breast, without any further qualifications can be misleading because it has been applied to a miscellaneous spectrum of lesions which vary not only in their histologic characteristics but also in clinical behavior. For instance, if in a survey of “sarcoma” of the breast, one should include all cases diagnosed as cystosarcoma phyllodes he would find that (1) the overall incidence of mammary sarcomas becomes quite high, and (2) their overall clinical malignancy is conversely quite low, since the majority of lesions labeled with that term behave as local, relatively harmless conditions. To give another example, especially in the older age group some carcinomas of the breast may exhibit areas of spindle cell, cartilagenous or osseous metaplasia. “.I6 To classify these tumors as fibrosarcoma or osteogenic or chondrosarcoma leads only to confusion and consequently to improper treatment since they behave not as sarcomas, but as carcinomas potentially able to metastasize as such to the axillary lymph nodes. Even the so-called carcinosarcomas should not be included because they are either purely epithelial with malignancies pseudosarcomatous metaplasia or possibly but more rarely admixtures of carcinoma and sarcoma.‘6 Finally locally invasive tumors of the fibrohistiocytic group, the so-called dermatofibrosarcoma protuberans, can occur in the dermis of the mammary region, as well as elsewhere. These, however, are not primary tumors of the breast. but of the overlying tissues. After these preliminary remarks. I shall list and discuss briefly, the clinicopathologic correlations of those lesions which we feel can be rightly included under the heading of sarcoma of the breast. Sarcomas
arising
New York, U.S.A.
in cystosarcoma
This is the reason for including malignant cystosarcomas in this discussion. As a rule, these tumors feature a highly cellular proliferation of immature mesenchymal cells originating in the stroma of a fibroepithelial tumor, generally of the intracanalicular fibroadenoma type. This proliferation may have the pattern of malignant fibrosarcoma or more rarely that of liposarcoma, osteogenic sarcoma, rhabdomyosarcoma, or a mixture of malignant mesenchymal tissues (malignant mesenchymoma). When these tumors metastasize they follow, as a rule, the hematogenous route, so that radical mastectomy with axillary dissection is not indicated.2.9”.‘9 It has been mentioned before that the majority of cystosarcoma phyllodes have an essentially benign course. This is true in the experience of most authors who have analyzed large series, even though the incidence of clinical malignancy reported in different articles varies considerably, most probably in function of the selection of cases and the histological criteria used in diagnosing the lesion. For instance, in the series reported from our department by Lester and Stout in 1954,‘j there were five metastasizing tumors of a total of 58 cystosarcomas. However, only 36 of these 58 were local patients treated in Columbia Presbyterian Medical Center and of these, only one had metastases. This is probably the true incidence of metastases, since the other 22 patients with four metastases were pre-selected, being outside consultations. The same probably can be said for the relatively high incidence of metastases in the series reported from Memorial Hospital for Cancer and Allied Diseases in New York City by Treves and SunderlandZ5 (special referrals to a cancer hospital). It might be interesting to note that after the series published by Lester and Stout,” an additional 267 cases of cystosarcoma were entered into our diagnostic files. Of these, 126 were local Columbia-Presbyterian Medical Center patients, 22 of whom had local recurrences and 5 had metastases; in the
phyllodes
In the rare instances in which cystosarcoma phyllodes exhibits a malignant course, it is its stroma which becomes malignant and behaves as a sarcoma. 705
706
Radiation Oncology 0 Biology 0 Physics
all outside consultations, remaining 141 patients, there were 10 distant metastases and 14 local recurrences. Probably many of the cases that recur locally are just instances in which the original excision was resulting in persistence and further incomplete. growth. It is to be noted here, that many cystosarcomas are incompletely encapsulated, so that very limited excisions will leave behind some of the residual tumor tissue. This is proved because in many of these recurrences, the histology is still that of a fibroepithelial tumor rather than of a pure sarcoma.’ There is no doubt, however, that some local recurrences are the result of a local aggressive behavior of the sarcomatous component of the original tumor. Whether the malignant behavior of cystosarcoma phyllodes can be predicted with some degree of accuracy from the histologic findings is still by and large an unsettled question. In our department2.9.‘5.‘9.25 the experience has been that the majority of those cystosarcomas exhibiting a “sarcomatous” stroma were cured by adequate local excision, while at least one with a fulminating clinical course did not show convincing histologic malignancy. We feel that malignancy should be suspected especially when the tumor, besides showing a sarcomatous-like stroma, also exhibits rapid growth and lack of true encapsulation, with local aggressive behavior. Occasionally, the sarcomatous component of a cystosarcoma can be extremely aggressive, leading to repeated local recurrences and eventually invading through the chest wall, but without true embolic metastases. Sarcomas of the breast proliferation
not associated
with epithelial
The usual types of soft tissue sarcomas occasionally can present as primary breast tumors, the socalled “stromal sarcomas” of the breast.’ It is probable that some of the reported cases were cystosarcoma phyllodes in which at the time of the diagnosis it was not possible to establish with certainty the origin of the sarcoma in a preexisting fibroepithelial tumor.“.*’ Besides these cases, however, there is no doubt that fibrosarcomas, liposarcomas,‘2~‘7~24 leio and rhabdomyosarcomas7.22 malignant hemangioendotheliomas or angiosarcomas,’ malignant lymphomas’.“.‘4,‘” (and Lattes et al., unpublished data), and occasionally more unusual variants4.‘* may occur as primary breast tumors, as stated earlier. An accurate diagnosis can be established only by accurate pathologic study of adequate biopsy material. The prognosis varies with the histologic type and is not different from that of histologically similar tumors arising elsewhere. It depends on timely clinical and pathologic recognition of the nature of the lesion and on adequate treatment
July-August 1978, Volume 4, No. 7 and No. 8 that must consist of wide, well-planned, surgical excision, with the possible exception of the primary malignant lymphomas which will be discussed later. Axillary lymph node dissection is not indicated since, as a rule, these tumors do not metastasize via the lymphatics. As with the soft tissue sarcomas elseinadequate surgical excision may lead to where, further local seeding of the tumor with recurrences which may result in an inoperable lesion even when the tumor type is not one likely to metastasize.‘.4.h An idea of the respective incidence or rather rarity of the different types of sarcomas of the breast can be obtained from Haagensen’ and others.4~R~‘c~‘x.2’ Here, it seems justified to discuss in some detail two special groups, which are important as pathologic entities and as clinical problems. Malignant
hemangioendothelioma
(angiosarcoma)
of
the breast.
This highly malignant tumor, one of the rare soft tissue sarcomas, is perhaps relatively more frequent in the breast as compared to other regions of the body. It may present clinically as a rapidly growing mass and grossly as a poorly circumscribed spongy, hemorrhagic tissue. The histologic diagnosis generally presents no problem; however, some angiosarcomas of the breast may look deceptively benign and a diagnosis of benign hemangioma of the mammary gland should be looked upon with skepticism. The accepted treatment is wide local removal which has often been achieved with radical mastectomy. However, in 35 cases from our files there was no instance of regional lymph node metastasis. Similarly no lymph node metastases were recorded in a series et al.’ These of ten cases reported by Steingazner tumors are practically uniformly fatal, and the majority of the patients die with generalized blood-borne metastases. There is no good evidence that these tumors are ever preceded by benign angiomas or vascular malformations. Malignant
lymphoma
of the breast
Relatively little has been written on this subject, but there is no doubt that malignant lymphoma not only can present clinically as an apparently primary breast tumor, but that at least in some instances it is acceptable as a primary solitary breast lesion. Those are the instances in which local treatment alone (surgery or radiation therapy or both) has resulted in “clinical cures”. long-duration Briefly, and with the exclusion of those cases in which the mammary involvement is obviously but a manifestation of a systemic lymphomatous disease, this is a condition which can occur at any age, including adolescence and in young adult females, can be unilateral or bilateral, and generally features rapid
Sarcomas of the breast 0 R.
and diffuse growth. It can be mistaken for acute mastitis.13 The clinical course is usually rapid and often fatal. Several years ago, with the collaboration of Wermenlinger and Longstreth (unpublished data), we reviewed the clinical pathologic correlations in a series of 38 cases recorded in our files as apparently primary lymphomas of the breast. (There are now about 80 other well-documented cases reported in the available literature). The results of this analysis can be summarized as follows. The ages varied from 14 to 85, with 14 patients younger than 40. Bilateral involvement occurred nine times and of these nine patients, three were pregnant and three were puerperal. In 15 cases, there was confirmed axillary lymph node involvement. The size of the presenting tumors varied, but it is noteworthy that some reached a very large size, up to 15 cm in diameter. These larger tumors were also those that grew more rapidly and had a worse prognosis. The histologic types included 28 so-called reticulum cell sarcomas, that is, malignant lymphomas of histiocytic type, 5 lymphocytic lymphosarcomas, 3 mixed lymphocytic and histiocytic type, and 2 giant follicular (nodular) lymphomas. It is interesting that at times, in a diffuse lymphosarcoma the low power pattern can simulate a follicular lymphoma because of a persistence of the breast lobule outlines, which are packed with tumor cells. the differential diagnosis between Occasionally, and undifferentiated “reticulum cell sarcoma” present difficulties even to carcinoma can experienced pathologists. Of these cases, 33 had adequate follow-up and 6 were living apparently free of disease 1, 3, 4, 8,9 and 10 years, respectively, from diagnosis. Of the others, 6 were alive, but with persistent tumor, and 21 died of disease, 17 of them within 1 year of diagnosis. There was no obvious
LATTES
707
correlation between histological type and prognosis. Only one of all these patients subsequently developed signs years
of lymphocytic leukemia: this happened five after radical mastectomy. The treatment used in
these cases varied from surgery alone (simple or radical mastectomy), to surgery followed by irradiation, to irradiation alone. At variance with what has been stated elsewhere, this study suggested that probably irradiation alone is adequate to control the local disease. In fact, the two longest survivors (9 and IO years) were treated with irradiation alone. None of the patients treated with surgery alone were among the survivors. In those who died, the disease often had a fulminating course, involving either several lymphatic stations or distant areas such as the central nervous system. We have no data regarding the possible role of chemotherapy in these lesions, all of which were treated more than I5 years ago. Summary
Sarcoma of the breast is rare. Rather than one single clinical pathologic entity, it is a family of diseases and its prognosis and treatment depend on the diagnostic criteria used and on the different histologic types. The usual varients of soft tissue sarcoma can occur in the breast either in the stroma of cystosarcoma phyllodes or directly in the mammary tissue. Their behavior is analogous to that of soft tissue sarcomas elsewhere. When they metastasize they do not go to the regional lymph nodes but travel via the hematogenous routes. Therefore, the surgical treatment should consist of adequate wide excision. Axillary dissection is generally not indicated. The two most malignant types of sarcoma of the breast are the malignant hemangioendothelioma (or angiosarcoma) and the malignant lymphoma.
REFERENCES 1. Berg.
2.
3.
4.
5.
6. 7.
J.W.. De Cosse, J.J.. Fracchia, A.A., Farrow, J.: Stromal Sarcoma of the Breast: A unified approach to connective tissue sarcomas other than cystosarcoma phyllodes. Cancer 15: 418-424, 1%2. Blichert-Toft, M.. Hansen, J.P.. Hansen O.H., SchrGdt. T.: Clinical course of cystosarcoma phyllodes related to histologic appearance. Surg. Gynecol. & Ohsret. 140: 929-932, 1975. Bird, C.C.: A breast sarcoma containing rhabdomyosarcomatous and other metaplastic elements. J. J. Path. 101: 286289, 1970. Botham. R.J.. McDonald, J.R.. Clagett, O.T.: Sarcoma of the mammary gland. Surg. Gynecol. Ohstet. 107: 55-61, 1961. De Cosse. J.J.. Berg, G.F., Fracchia, A.A., Farrow, J.H.: Primary lymphosarcoma of the breast. Cancer 15: 1264-1268. 1%2. Haagensen. C.D.: Diseases of the breast. 2nd Edn. Philadelphia, Saunders, 1971, pp. 227-249, 292-325. Hajdu, S.I., Espinosa, M.H.. Robbins. G.F.: Recurrent cystosarcoma phyllodes. Cancer 38: 1402-1406. 1976.
8. Hill, R.P., Stout, A.P.: Sarcoma of the breast. Arch. Surg. 44: 723-759. 1942. 9. Hoover, H.C., Trestioreanu, A., Ketcham. A.S.: Metastatic cystosarcoma phyllodes in an adolescent girl. Ann. Surg. 181: 279-282, 1975. 10. Huvos, A.G., Lucas. J.C., Foote, F.W.: Metaplastic breast carcinoma. N.Y. State J. Med. 1078-1082, I May, 1973. Il. Jackson, A.V.: Metastasizing liposarcoma of the breast arising in a fibroadenoma. J. Path. Bad. 83: 582-584, 1%2. 12. Jackson, A.V.: Metastasizing liposarcoma of the Breast arising in a fibroadenoma. .I. Path. Bact. 83: 582-584, l%2. 13. Kay, S.: Lymphosarcoma of the female mammary gland. Arch. Path. 60: 575-579, 1955. 14. Lawler, M.R.. Richie. R.E.: Reticulum cell sarcoma of the breast. Cancer 20: 1438-1446, l%7. 15. Lester, J., Stout, A.P.: Cystosarcoma phyllodes. Cancer 7: 335-353, 1954. 16. McDivitt. R.W.. Stewart. F.W., Berg. J.W.: Tumors of
708
17. IS. 19.
20.
21.
Radiation Oncology 0 Biology 0 Physics
the Breast. 2nd series, Fascicle 2, Washington, D.C. Armed Forces Institute of Pathology, 1967, pp. 117132. Menon, M., Van Velthoven. M.: Liposarcoma of the breast. Arch. Path. 98: 37&372, 1974. Norris, H.J., Taylor, H.: Sarcomas and related mesenchymal tumors of the breast. Cancer 22: 22-28, l%S. Norris, H.J.. Taylor, H.B.: Relationship of histologic features to behavior of cystosarcoma phyllodes. Cancer 20: 2090-2099, l%7. Oberman, H.A.: Primary lymphoreticular neoplasms of the breast. Surg. Gynecol. & Ohstet. 123: 1047-1051. l%6. Oberman, H.A.: Sarcomas of the breast. Cancer 18: 1233-1243. l%S.
July-August
1978, Volume 4, No. 7 and No. 8
22. Pardo-Mindau. J., Garcia-Julian, G., Altuna, M.E.: Leiomyosarcoma of the breast. Am. J. C/in. Path. 62: 477-480, 1974. 23. Steinganzer, L.C., Enzinger, F.M., Taylor, H.B.: Hemangiosarcoma of the breast. Cancer 18: 352-361, l%5. 24. Stout, A.P., Bernanke, M.: Liposarcoma of the female mammary gland. Surg. Gynecol. & Obstet. 83: 216-218, 1946. 25. Treves, N.. Sunderland, D.A.: Cystosarcoma phyllodes of the breast. A malignant and a benign tumor. Clinicopathologic study of 77 cases. Cancer 4: 1286-1332. 1951.
Oncology Press Inc.,
Biol. Phys., Vol. 4, pp. 705-708 1978. Printed in the U S.A.
0 Current Concepts
03@-3016/78/0801-0705/$02.00/0
in Cancer
SARCOMAS RAFFAELE
OF THE BREAST LATTES, M.D.
Professor of Surgical Pathology, College of Physicians and Surgeons, Columbia University, Sarcomas
of the breast
The term, sarcoma of the breast, without any further qualifications can be misleading because it has been applied to a miscellaneous spectrum of lesions which vary not only in their histologic characteristics but also in clinical behavior. For instance, if in a survey of “sarcoma” of the breast, one should include all cases diagnosed as cystosarcoma phyllodes he would find that (1) the overall incidence of mammary sarcomas becomes quite high, and (2) their overall clinical malignancy is conversely quite low, since the majority of lesions labeled with that term behave as local, relatively harmless conditions. To give another example, especially in the older age group some carcinomas of the breast may exhibit areas of spindle cell, cartilagenous or osseous metaplasia. “.I6 To classify these tumors as fibrosarcoma or osteogenic or chondrosarcoma leads only to confusion and consequently to improper treatment since they behave not as sarcomas, but as carcinomas potentially able to metastasize as such to the axillary lymph nodes. Even the so-called carcinosarcomas should not be included because they are either purely epithelial with malignancies pseudosarcomatous metaplasia or possibly but more rarely admixtures of carcinoma and sarcoma.‘6 Finally locally invasive tumors of the fibrohistiocytic group, the so-called dermatofibrosarcoma protuberans, can occur in the dermis of the mammary region, as well as elsewhere. These, however, are not primary tumors of the breast. but of the overlying tissues. After these preliminary remarks. I shall list and discuss briefly, the clinicopathologic correlations of those lesions which we feel can be rightly included under the heading of sarcoma of the breast. Sarcomas
arising
New York, U.S.A.
in cystosarcoma
This is the reason for including malignant cystosarcomas in this discussion. As a rule, these tumors feature a highly cellular proliferation of immature mesenchymal cells originating in the stroma of a fibroepithelial tumor, generally of the intracanalicular fibroadenoma type. This proliferation may have the pattern of malignant fibrosarcoma or more rarely that of liposarcoma, osteogenic sarcoma, rhabdomyosarcoma, or a mixture of malignant mesenchymal tissues (malignant mesenchymoma). When these tumors metastasize they follow, as a rule, the hematogenous route, so that radical mastectomy with axillary dissection is not indicated.2.9”.‘9 It has been mentioned before that the majority of cystosarcoma phyllodes have an essentially benign course. This is true in the experience of most authors who have analyzed large series, even though the incidence of clinical malignancy reported in different articles varies considerably, most probably in function of the selection of cases and the histological criteria used in diagnosing the lesion. For instance, in the series reported from our department by Lester and Stout in 1954,‘j there were five metastasizing tumors of a total of 58 cystosarcomas. However, only 36 of these 58 were local patients treated in Columbia Presbyterian Medical Center and of these, only one had metastases. This is probably the true incidence of metastases, since the other 22 patients with four metastases were pre-selected, being outside consultations. The same probably can be said for the relatively high incidence of metastases in the series reported from Memorial Hospital for Cancer and Allied Diseases in New York City by Treves and SunderlandZ5 (special referrals to a cancer hospital). It might be interesting to note that after the series published by Lester and Stout,” an additional 267 cases of cystosarcoma were entered into our diagnostic files. Of these, 126 were local Columbia-Presbyterian Medical Center patients, 22 of whom had local recurrences and 5 had metastases; in the
phyllodes
In the rare instances in which cystosarcoma phyllodes exhibits a malignant course, it is its stroma which becomes malignant and behaves as a sarcoma. 705
706
Radiation Oncology 0 Biology 0 Physics
all outside consultations, remaining 141 patients, there were 10 distant metastases and 14 local recurrences. Probably many of the cases that recur locally are just instances in which the original excision was resulting in persistence and further incomplete. growth. It is to be noted here, that many cystosarcomas are incompletely encapsulated, so that very limited excisions will leave behind some of the residual tumor tissue. This is proved because in many of these recurrences, the histology is still that of a fibroepithelial tumor rather than of a pure sarcoma.’ There is no doubt, however, that some local recurrences are the result of a local aggressive behavior of the sarcomatous component of the original tumor. Whether the malignant behavior of cystosarcoma phyllodes can be predicted with some degree of accuracy from the histologic findings is still by and large an unsettled question. In our department2.9.‘5.‘9.25 the experience has been that the majority of those cystosarcomas exhibiting a “sarcomatous” stroma were cured by adequate local excision, while at least one with a fulminating clinical course did not show convincing histologic malignancy. We feel that malignancy should be suspected especially when the tumor, besides showing a sarcomatous-like stroma, also exhibits rapid growth and lack of true encapsulation, with local aggressive behavior. Occasionally, the sarcomatous component of a cystosarcoma can be extremely aggressive, leading to repeated local recurrences and eventually invading through the chest wall, but without true embolic metastases. Sarcomas of the breast proliferation
not associated
with epithelial
The usual types of soft tissue sarcomas occasionally can present as primary breast tumors, the socalled “stromal sarcomas” of the breast.’ It is probable that some of the reported cases were cystosarcoma phyllodes in which at the time of the diagnosis it was not possible to establish with certainty the origin of the sarcoma in a preexisting fibroepithelial tumor.“.*’ Besides these cases, however, there is no doubt that fibrosarcomas, liposarcomas,‘2~‘7~24 leio and rhabdomyosarcomas7.22 malignant hemangioendotheliomas or angiosarcomas,’ malignant lymphomas’.“.‘4,‘” (and Lattes et al., unpublished data), and occasionally more unusual variants4.‘* may occur as primary breast tumors, as stated earlier. An accurate diagnosis can be established only by accurate pathologic study of adequate biopsy material. The prognosis varies with the histologic type and is not different from that of histologically similar tumors arising elsewhere. It depends on timely clinical and pathologic recognition of the nature of the lesion and on adequate treatment
July-August 1978, Volume 4, No. 7 and No. 8 that must consist of wide, well-planned, surgical excision, with the possible exception of the primary malignant lymphomas which will be discussed later. Axillary lymph node dissection is not indicated since, as a rule, these tumors do not metastasize via the lymphatics. As with the soft tissue sarcomas elseinadequate surgical excision may lead to where, further local seeding of the tumor with recurrences which may result in an inoperable lesion even when the tumor type is not one likely to metastasize.‘.4.h An idea of the respective incidence or rather rarity of the different types of sarcomas of the breast can be obtained from Haagensen’ and others.4~R~‘c~‘x.2’ Here, it seems justified to discuss in some detail two special groups, which are important as pathologic entities and as clinical problems. Malignant
hemangioendothelioma
(angiosarcoma)
of
the breast.
This highly malignant tumor, one of the rare soft tissue sarcomas, is perhaps relatively more frequent in the breast as compared to other regions of the body. It may present clinically as a rapidly growing mass and grossly as a poorly circumscribed spongy, hemorrhagic tissue. The histologic diagnosis generally presents no problem; however, some angiosarcomas of the breast may look deceptively benign and a diagnosis of benign hemangioma of the mammary gland should be looked upon with skepticism. The accepted treatment is wide local removal which has often been achieved with radical mastectomy. However, in 35 cases from our files there was no instance of regional lymph node metastasis. Similarly no lymph node metastases were recorded in a series et al.’ These of ten cases reported by Steingazner tumors are practically uniformly fatal, and the majority of the patients die with generalized blood-borne metastases. There is no good evidence that these tumors are ever preceded by benign angiomas or vascular malformations. Malignant
lymphoma
of the breast
Relatively little has been written on this subject, but there is no doubt that malignant lymphoma not only can present clinically as an apparently primary breast tumor, but that at least in some instances it is acceptable as a primary solitary breast lesion. Those are the instances in which local treatment alone (surgery or radiation therapy or both) has resulted in “clinical cures”. long-duration Briefly, and with the exclusion of those cases in which the mammary involvement is obviously but a manifestation of a systemic lymphomatous disease, this is a condition which can occur at any age, including adolescence and in young adult females, can be unilateral or bilateral, and generally features rapid
Sarcomas of the breast 0 R.
and diffuse growth. It can be mistaken for acute mastitis.13 The clinical course is usually rapid and often fatal. Several years ago, with the collaboration of Wermenlinger and Longstreth (unpublished data), we reviewed the clinical pathologic correlations in a series of 38 cases recorded in our files as apparently primary lymphomas of the breast. (There are now about 80 other well-documented cases reported in the available literature). The results of this analysis can be summarized as follows. The ages varied from 14 to 85, with 14 patients younger than 40. Bilateral involvement occurred nine times and of these nine patients, three were pregnant and three were puerperal. In 15 cases, there was confirmed axillary lymph node involvement. The size of the presenting tumors varied, but it is noteworthy that some reached a very large size, up to 15 cm in diameter. These larger tumors were also those that grew more rapidly and had a worse prognosis. The histologic types included 28 so-called reticulum cell sarcomas, that is, malignant lymphomas of histiocytic type, 5 lymphocytic lymphosarcomas, 3 mixed lymphocytic and histiocytic type, and 2 giant follicular (nodular) lymphomas. It is interesting that at times, in a diffuse lymphosarcoma the low power pattern can simulate a follicular lymphoma because of a persistence of the breast lobule outlines, which are packed with tumor cells. the differential diagnosis between Occasionally, and undifferentiated “reticulum cell sarcoma” present difficulties even to carcinoma can experienced pathologists. Of these cases, 33 had adequate follow-up and 6 were living apparently free of disease 1, 3, 4, 8,9 and 10 years, respectively, from diagnosis. Of the others, 6 were alive, but with persistent tumor, and 21 died of disease, 17 of them within 1 year of diagnosis. There was no obvious
LATTES
707
correlation between histological type and prognosis. Only one of all these patients subsequently developed signs years
of lymphocytic leukemia: this happened five after radical mastectomy. The treatment used in
these cases varied from surgery alone (simple or radical mastectomy), to surgery followed by irradiation, to irradiation alone. At variance with what has been stated elsewhere, this study suggested that probably irradiation alone is adequate to control the local disease. In fact, the two longest survivors (9 and IO years) were treated with irradiation alone. None of the patients treated with surgery alone were among the survivors. In those who died, the disease often had a fulminating course, involving either several lymphatic stations or distant areas such as the central nervous system. We have no data regarding the possible role of chemotherapy in these lesions, all of which were treated more than I5 years ago. Summary
Sarcoma of the breast is rare. Rather than one single clinical pathologic entity, it is a family of diseases and its prognosis and treatment depend on the diagnostic criteria used and on the different histologic types. The usual varients of soft tissue sarcoma can occur in the breast either in the stroma of cystosarcoma phyllodes or directly in the mammary tissue. Their behavior is analogous to that of soft tissue sarcomas elsewhere. When they metastasize they do not go to the regional lymph nodes but travel via the hematogenous routes. Therefore, the surgical treatment should consist of adequate wide excision. Axillary dissection is generally not indicated. The two most malignant types of sarcoma of the breast are the malignant hemangioendothelioma (or angiosarcoma) and the malignant lymphoma.
REFERENCES 1. Berg.
2.
3.
4.
5.
6. 7.
J.W.. De Cosse, J.J.. Fracchia, A.A., Farrow, J.: Stromal Sarcoma of the Breast: A unified approach to connective tissue sarcomas other than cystosarcoma phyllodes. Cancer 15: 418-424, 1%2. Blichert-Toft, M.. Hansen, J.P.. Hansen O.H., SchrGdt. T.: Clinical course of cystosarcoma phyllodes related to histologic appearance. Surg. Gynecol. & Ohsret. 140: 929-932, 1975. Bird, C.C.: A breast sarcoma containing rhabdomyosarcomatous and other metaplastic elements. J. J. Path. 101: 286289, 1970. Botham. R.J.. McDonald, J.R.. Clagett, O.T.: Sarcoma of the mammary gland. Surg. Gynecol. Ohstet. 107: 55-61, 1961. De Cosse. J.J.. Berg, G.F., Fracchia, A.A., Farrow, J.H.: Primary lymphosarcoma of the breast. Cancer 15: 1264-1268. 1%2. Haagensen. C.D.: Diseases of the breast. 2nd Edn. Philadelphia, Saunders, 1971, pp. 227-249, 292-325. Hajdu, S.I., Espinosa, M.H.. Robbins. G.F.: Recurrent cystosarcoma phyllodes. Cancer 38: 1402-1406. 1976.
8. Hill, R.P., Stout, A.P.: Sarcoma of the breast. Arch. Surg. 44: 723-759. 1942. 9. Hoover, H.C., Trestioreanu, A., Ketcham. A.S.: Metastatic cystosarcoma phyllodes in an adolescent girl. Ann. Surg. 181: 279-282, 1975. 10. Huvos, A.G., Lucas. J.C., Foote, F.W.: Metaplastic breast carcinoma. N.Y. State J. Med. 1078-1082, I May, 1973. Il. Jackson, A.V.: Metastasizing liposarcoma of the breast arising in a fibroadenoma. J. Path. Bad. 83: 582-584, 1%2. 12. Jackson, A.V.: Metastasizing liposarcoma of the Breast arising in a fibroadenoma. .I. Path. Bact. 83: 582-584, l%2. 13. Kay, S.: Lymphosarcoma of the female mammary gland. Arch. Path. 60: 575-579, 1955. 14. Lawler, M.R.. Richie. R.E.: Reticulum cell sarcoma of the breast. Cancer 20: 1438-1446, l%7. 15. Lester, J., Stout, A.P.: Cystosarcoma phyllodes. Cancer 7: 335-353, 1954. 16. McDivitt. R.W.. Stewart. F.W., Berg. J.W.: Tumors of
708
17. IS. 19.
20.
21.
Radiation Oncology 0 Biology 0 Physics
the Breast. 2nd series, Fascicle 2, Washington, D.C. Armed Forces Institute of Pathology, 1967, pp. 117132. Menon, M., Van Velthoven. M.: Liposarcoma of the breast. Arch. Path. 98: 37&372, 1974. Norris, H.J., Taylor, H.: Sarcomas and related mesenchymal tumors of the breast. Cancer 22: 22-28, l%S. Norris, H.J.. Taylor, H.B.: Relationship of histologic features to behavior of cystosarcoma phyllodes. Cancer 20: 2090-2099, l%7. Oberman, H.A.: Primary lymphoreticular neoplasms of the breast. Surg. Gynecol. & Ohstet. 123: 1047-1051. l%6. Oberman, H.A.: Sarcomas of the breast. Cancer 18: 1233-1243. l%S.
July-August
1978, Volume 4, No. 7 and No. 8
22. Pardo-Mindau. J., Garcia-Julian, G., Altuna, M.E.: Leiomyosarcoma of the breast. Am. J. C/in. Path. 62: 477-480, 1974. 23. Steinganzer, L.C., Enzinger, F.M., Taylor, H.B.: Hemangiosarcoma of the breast. Cancer 18: 352-361, l%5. 24. Stout, A.P., Bernanke, M.: Liposarcoma of the female mammary gland. Surg. Gynecol. & Obstet. 83: 216-218, 1946. 25. Treves, N.. Sunderland, D.A.: Cystosarcoma phyllodes of the breast. A malignant and a benign tumor. Clinicopathologic study of 77 cases. Cancer 4: 1286-1332. 1951.
