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Retroperitoneal Angiomyosarcoma: A Case Report
Vol. 106, December Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1971 by The Williams & Wilkins Co.
RETROPERI'l;'ONEAL ANGIOMYOSARCOMA: A CASE REPORT BALFOUR M. MOUNT,* STEVEN I. HAJDUt
AND
WILLET F. WHITMORE, JR.
From the Urology Service, Department of Surgery and the Department of Pathology, Memorial Hospital for Cancer and Allied Disease, New York, New York
Angiosarcoma and leiomyosarcoma are uncommon tumors of mesodermal origin. The occurrence of both of these elements in a pelvic tumor seems to be unique. CASE REPORT
M. B., M H 61-63-53, a 55-year-old woman, was well until May 1966 when spasms of left inguinal pain occurred. These were relieved with aspirin but they persisted with some increase in severity over the ensuing years. Finally a left inguinal exploration was undertaken on February 17, 1970. The preoperative diagnosis was inguinal hernia. Biopsy of enlarged external iliac nodes exposed at the inguinal exploration demonstrated a metastatic malignant tumor which defied further classification. The patient was referred to this hospital. Physical examination in March 1970 revealed a welldeveloped white woman in apparent good health. Blood pressure was 140/100. Significant findings were limited to the abdomen, which was slightly indurated in the ·lower quadrants, and the pelvis, which demonstrated a cystocele, urethrocele and a firm, fixed left suprapubic mass closely applied to the left superior pubic and ischial rami. Rectal examination was unremarkable. Femoral pulses were strong bilaterally without peripheral edema or evidence of venous or lymphatic obstruction. Routine hematology, urinalysis, electrocardiogram, chest x-ray, serum glutamic oxaloacetic transaminase, electrolytes, serum proteins, blood sugar, blood urea nitrogen, alkaline phosphatase and urine cytology were normal, as were an upper gastrointestinal series, gallbladder series, barium enema, skeletal survey and mammogram. Medial deviation of the pelvic portion of the left ureter was seen on an otherwise normal excretory urogram (IVP) while lymphangiography demonstrated left obturator, iliac and para-aortic lymphadenopathy (fig. 1). Cystoscopy was negative.
On March 24 laparotomy demonstrated left para-aortic and left iliac lymphadenopathy and a smooth, firm, fixed vascular mass in the left iliac fossa closely applied to the left obturator internus muscle and to the adjacent superior pubic and ischial rami and left iliac vessels. The obturator and iliac nodes were most extensively involved with decreasing involvement at more superior levels. The gross appearance of the lymphadenopathy was unusual. The lymph nodes were enlarged, reddened and unusually vascular. They had a finely lobulated appearance due to multiple small translucent cystic structures, 1 to 3 mm. in diameter, which exuded lymph, blood or a combination thereof when punctured. There was rich network of grossly visible, dilated vascular channels, both hemic and lymphatic, surrounding the major vessels between adjacent lymph nodes. These channels also exhibited the cystic structures associated with the lymph nodes themselves. Some of these channels bled freely when transected. A tedious.left pelvic dissection was carried out, removing all gross neoplasm from the left pelvis along with the overlying peritoneum. A left salpingo-oophorectomy, hysterectomy and resection of the left lateral wall of the bladder to which the mass was adherent were undertaken. A left groin dissection and a para-aortic lymphadenectomy above the inferior mesenteric artery were considered but not carried out because of the magnitude of the pelvic procedure and pending pathological report on the unusually vascular tumor. Frozen section during the operative procedure was uninformative. Convalescence was uneventful and the left inguinal pain disappeared. The patient is cu~·rently receiving irradiation to the para-aortic lymph nodes and pelvis.
Accepted for publication December 1970. * Current address: McGill University, Montreal, Quebec, Canada.. t Requests for reprints: 444 East 68th Street, New York, New York 10021. 837
GROSS AND MICROSCOPIC PATHOLOGY
The pertinent portion of the gross specimen consisted of an aggregate of hemorrhagic lymph nodes and fibroadipose tissue measuring 15 by 7 by 5 cm. A 6 by 3 cm. segment of the bladder was attached to this mass by fibrous tissue.
838
MOUNT, HAJDU AND WHITMORE
Fm. 1. A, IVP demonstrates medial deviation of lower third of left ureter by pelvic mass. B, lymphangiography demonstrates abnormal left obturator, iliac and para-aortic lymph nodes. More superior lymph nodes appear to be less involved than those in pelvis.
Fm. 2. A and B, proliferating small, medium and large vascular channels. Note bizarre, atypical smooth muscle fibers and sheets of proliferating malignant endothelial cells. H & E, reduced from X 140. The uterus, cervix, fallopian tubes and ovaries showed no gross or microscopic abnormality. Histologically, the tumor was composed mainly of small and medium-sized blood vessels diffusely infiltrating fibroadipose tissue and hyperplastic lymph nodes. The prominent proliferation of malignant endothelial cells of blood vessels and of atypical smooth muscle cells in their walls could be recognized in most sections (fig. 2). Pleomorphic and bizarre mitotic cells were observed frequently. Not rarely, elongated atypical smooth muscle cells attempted to form avascular solid areas. Malignant vascular channels and sheets of malignant endothelial cells could be seen in tumor nodules metastatic to lymph nodes (fig. 3).
DISCUSSION
A variety of malignant neoplasms may arise from the constituent cells of blood vessels (the endothelial cell, the smooth muscle cell and the pericyte). Such tumors include angiosarcoma, endothelioma, hemangiopericytoma, Kaposi's sarcoma and leiomyosarcoma. These tumors are uncommon and their occurrence in the retroperitoneal region is rare. Leiomyosarcoma occurring in major vessels was considered theoretically reasonable by Stout in 1943 but he hastened to add that "the evidence that such a neoplastic type exists is scanty". 1 1 Stout, A. P.: Hemangio-endothelioma; tumor of blood vessels featuring vascular endothelial cells. Ann. Surg., 118: 445, 1943.
RETROPERITONEAL ANGIOMYOSARCOMA
839
FIG. 3. Angiomyosarcoma metastatic to lymph node. Note proliferating large vascular channels adjacent to clusters of malignant endothelial cells. H & E, reduced from Xl40.
However, since then several investigators have recorded cases of leiomyosarcoma arising from large blood vessels. In 1960 Thomas and Fine reviewed the literature and collected 27 case reports of primary tumors in veins and added 2 more cases. 2 Of these 29 cases, 28 tumors arose from the media and 1 from the endothelium. Sixteen were considered malignant and 12 benign. Of the 16 cases of sarcoma only 3 had demonstrated metastases: a giant cell sarcoma of the superior vena cava, a "sarcoma" of the right pulmonary vein and a leiomyosarcoma of the inferior vena cava. In the cases reviewed by Thomas and Fine the most common sites for vascular leiomyosarcomas (in pure form) were the inferior vena cava, femoral vein, saphenous vein and pulmonary vein with solitary examples occurring in other veins. To our knowledge, an apparent primary site in the vessels of the side wall of the pelvis as in our case has not been reported previously. Leiomyosarcomas of non-vascular origin not infrequently occur in the retroperitoneal region. Ackerman lists them as second in frequency to tumors of fatty origin. 3 However, the occurrence of leiomyosarcoma in conjunction with the angiosarcomatous component suggests a vascular origin in our case and renders the lesion unique. 2 Thomas, M.A. and Fine, G.: Leiomyosarcoma of veins: report of 2 cases and review of the li terature. Cancer, 13: 96, 1960. 3 Ackerman, L. V.: Tumors of the retroperitoneum, mesentery and peritoneum. In: Atlas of Tumor Pathology. Washington, D. C.: Armed Forces Institute of Pathology, sec. VI, fasc. 23 and 24, 1954.
Angiosarcomas in the retroperitoneal region are extremely rare. Ackerman states "we have not seen a single example" while in 1943 Stout was able to find only 3 cases in his review of the literature. At this hospital there are approximately 20 admissions annually for primary retroperitoneal tumors. Our case represents the first admission for angiosarcoma in the retroperitoneal region. In 1963 Y annopoulos and Stout collected 44 non-lymphomatous primary solid tumors of the mesentery including 5 leiomyosarcomas and 5 hemangiomatous tumors. 4 None had demonstrable metastases. The thesis that the tumor arose in the vessels of the left pelvic side wall is supported by the large left pelvic vascular mass, the initial left inguinal symptoms, the pattern of lymphatic spread (with the most involved nodes in the pelvis) and the absence of demonstrable disease elsewhere. The histological features indicate a vascular tumor of angiosarcomatous and leiomyosarcomatous components. The extensive local disease precluded accurate identification of the precise vessel of ongm. No clear relationship between this tumor and the renal angiomyolipoma, a tumor which also contains vascular and smooth muscle elements, can be defined. Angiomyolipomas are uncommon new growths of uncertain histogenesis which occur in the kidney as isolated lesions and in asso4 Yannopoulos, K. and Stout, A. P.: Primary solid tumors of the mesentery. Cancer, 16: 914,
1963.
840
MOUNT, HAJDU AND WHITMORE
ciation with tuberous sclerosis. 5 They are usually benign but sometimes seem to be malignant histologically. Metastases are uncommon and have been reported only when associated with tuberous sclerosis. 6 The clinical and histological features in our case, that is normal kidneys on IVP and negative history for tuberous sclerosis, make it highly unlikely that this tumor represents metastases from an occult primary renal tumor. Radical excision of the tumor was attempted because of the long antecedent history and the patient's general good health as well as the un-
usual gross features and uncertain nature of the tumor. Stout has emphasized the fact that sarcomas vary in prognosis and natural history, depending on cell type, anatomic site and the patient's age. 7 Thus an accurate prognosis is impossible in the present case. The 4-year history suggests a slow growth pattern and favors aggressive attempts at palliation. The roles of chemotherapy and irradiation have not been established.
6 Hajdu, S. I. and Foote, F. W., Jr.: Angiomyolipoma of the kidney: report of 27 cases and review of the literature. J. Urol., 102: 396, 1969. 6 Tweeddale, D. N., Dawe, C. J., McDonald, J. R. and Culp, 0. S.: Angiolipoleiomyoma of the kidney; report of a case with observations on histogenesis. Cancer, 8: 759, 1955.
An angiomyosarcoma of the pelvis with lymphatic-borne metastases in a 59-year-old woman is reported.
SUMMARY
7 Stout, A. P.: Pathological aspects of soft part sarcomas. Ann. N.Y. Acad. Sci., 114: 1041, 1964.
THE JOURNAL OF UROLOGY
Copyright© 1971 by The Williams & Wilkins Co.
RETROPERI'l;'ONEAL ANGIOMYOSARCOMA: A CASE REPORT BALFOUR M. MOUNT,* STEVEN I. HAJDUt
AND
WILLET F. WHITMORE, JR.
From the Urology Service, Department of Surgery and the Department of Pathology, Memorial Hospital for Cancer and Allied Disease, New York, New York
Angiosarcoma and leiomyosarcoma are uncommon tumors of mesodermal origin. The occurrence of both of these elements in a pelvic tumor seems to be unique. CASE REPORT
M. B., M H 61-63-53, a 55-year-old woman, was well until May 1966 when spasms of left inguinal pain occurred. These were relieved with aspirin but they persisted with some increase in severity over the ensuing years. Finally a left inguinal exploration was undertaken on February 17, 1970. The preoperative diagnosis was inguinal hernia. Biopsy of enlarged external iliac nodes exposed at the inguinal exploration demonstrated a metastatic malignant tumor which defied further classification. The patient was referred to this hospital. Physical examination in March 1970 revealed a welldeveloped white woman in apparent good health. Blood pressure was 140/100. Significant findings were limited to the abdomen, which was slightly indurated in the ·lower quadrants, and the pelvis, which demonstrated a cystocele, urethrocele and a firm, fixed left suprapubic mass closely applied to the left superior pubic and ischial rami. Rectal examination was unremarkable. Femoral pulses were strong bilaterally without peripheral edema or evidence of venous or lymphatic obstruction. Routine hematology, urinalysis, electrocardiogram, chest x-ray, serum glutamic oxaloacetic transaminase, electrolytes, serum proteins, blood sugar, blood urea nitrogen, alkaline phosphatase and urine cytology were normal, as were an upper gastrointestinal series, gallbladder series, barium enema, skeletal survey and mammogram. Medial deviation of the pelvic portion of the left ureter was seen on an otherwise normal excretory urogram (IVP) while lymphangiography demonstrated left obturator, iliac and para-aortic lymphadenopathy (fig. 1). Cystoscopy was negative.
On March 24 laparotomy demonstrated left para-aortic and left iliac lymphadenopathy and a smooth, firm, fixed vascular mass in the left iliac fossa closely applied to the left obturator internus muscle and to the adjacent superior pubic and ischial rami and left iliac vessels. The obturator and iliac nodes were most extensively involved with decreasing involvement at more superior levels. The gross appearance of the lymphadenopathy was unusual. The lymph nodes were enlarged, reddened and unusually vascular. They had a finely lobulated appearance due to multiple small translucent cystic structures, 1 to 3 mm. in diameter, which exuded lymph, blood or a combination thereof when punctured. There was rich network of grossly visible, dilated vascular channels, both hemic and lymphatic, surrounding the major vessels between adjacent lymph nodes. These channels also exhibited the cystic structures associated with the lymph nodes themselves. Some of these channels bled freely when transected. A tedious.left pelvic dissection was carried out, removing all gross neoplasm from the left pelvis along with the overlying peritoneum. A left salpingo-oophorectomy, hysterectomy and resection of the left lateral wall of the bladder to which the mass was adherent were undertaken. A left groin dissection and a para-aortic lymphadenectomy above the inferior mesenteric artery were considered but not carried out because of the magnitude of the pelvic procedure and pending pathological report on the unusually vascular tumor. Frozen section during the operative procedure was uninformative. Convalescence was uneventful and the left inguinal pain disappeared. The patient is cu~·rently receiving irradiation to the para-aortic lymph nodes and pelvis.
Accepted for publication December 1970. * Current address: McGill University, Montreal, Quebec, Canada.. t Requests for reprints: 444 East 68th Street, New York, New York 10021. 837
GROSS AND MICROSCOPIC PATHOLOGY
The pertinent portion of the gross specimen consisted of an aggregate of hemorrhagic lymph nodes and fibroadipose tissue measuring 15 by 7 by 5 cm. A 6 by 3 cm. segment of the bladder was attached to this mass by fibrous tissue.
838
MOUNT, HAJDU AND WHITMORE
Fm. 1. A, IVP demonstrates medial deviation of lower third of left ureter by pelvic mass. B, lymphangiography demonstrates abnormal left obturator, iliac and para-aortic lymph nodes. More superior lymph nodes appear to be less involved than those in pelvis.
Fm. 2. A and B, proliferating small, medium and large vascular channels. Note bizarre, atypical smooth muscle fibers and sheets of proliferating malignant endothelial cells. H & E, reduced from X 140. The uterus, cervix, fallopian tubes and ovaries showed no gross or microscopic abnormality. Histologically, the tumor was composed mainly of small and medium-sized blood vessels diffusely infiltrating fibroadipose tissue and hyperplastic lymph nodes. The prominent proliferation of malignant endothelial cells of blood vessels and of atypical smooth muscle cells in their walls could be recognized in most sections (fig. 2). Pleomorphic and bizarre mitotic cells were observed frequently. Not rarely, elongated atypical smooth muscle cells attempted to form avascular solid areas. Malignant vascular channels and sheets of malignant endothelial cells could be seen in tumor nodules metastatic to lymph nodes (fig. 3).
DISCUSSION
A variety of malignant neoplasms may arise from the constituent cells of blood vessels (the endothelial cell, the smooth muscle cell and the pericyte). Such tumors include angiosarcoma, endothelioma, hemangiopericytoma, Kaposi's sarcoma and leiomyosarcoma. These tumors are uncommon and their occurrence in the retroperitoneal region is rare. Leiomyosarcoma occurring in major vessels was considered theoretically reasonable by Stout in 1943 but he hastened to add that "the evidence that such a neoplastic type exists is scanty". 1 1 Stout, A. P.: Hemangio-endothelioma; tumor of blood vessels featuring vascular endothelial cells. Ann. Surg., 118: 445, 1943.
RETROPERITONEAL ANGIOMYOSARCOMA
839
FIG. 3. Angiomyosarcoma metastatic to lymph node. Note proliferating large vascular channels adjacent to clusters of malignant endothelial cells. H & E, reduced from Xl40.
However, since then several investigators have recorded cases of leiomyosarcoma arising from large blood vessels. In 1960 Thomas and Fine reviewed the literature and collected 27 case reports of primary tumors in veins and added 2 more cases. 2 Of these 29 cases, 28 tumors arose from the media and 1 from the endothelium. Sixteen were considered malignant and 12 benign. Of the 16 cases of sarcoma only 3 had demonstrated metastases: a giant cell sarcoma of the superior vena cava, a "sarcoma" of the right pulmonary vein and a leiomyosarcoma of the inferior vena cava. In the cases reviewed by Thomas and Fine the most common sites for vascular leiomyosarcomas (in pure form) were the inferior vena cava, femoral vein, saphenous vein and pulmonary vein with solitary examples occurring in other veins. To our knowledge, an apparent primary site in the vessels of the side wall of the pelvis as in our case has not been reported previously. Leiomyosarcomas of non-vascular origin not infrequently occur in the retroperitoneal region. Ackerman lists them as second in frequency to tumors of fatty origin. 3 However, the occurrence of leiomyosarcoma in conjunction with the angiosarcomatous component suggests a vascular origin in our case and renders the lesion unique. 2 Thomas, M.A. and Fine, G.: Leiomyosarcoma of veins: report of 2 cases and review of the li terature. Cancer, 13: 96, 1960. 3 Ackerman, L. V.: Tumors of the retroperitoneum, mesentery and peritoneum. In: Atlas of Tumor Pathology. Washington, D. C.: Armed Forces Institute of Pathology, sec. VI, fasc. 23 and 24, 1954.
Angiosarcomas in the retroperitoneal region are extremely rare. Ackerman states "we have not seen a single example" while in 1943 Stout was able to find only 3 cases in his review of the literature. At this hospital there are approximately 20 admissions annually for primary retroperitoneal tumors. Our case represents the first admission for angiosarcoma in the retroperitoneal region. In 1963 Y annopoulos and Stout collected 44 non-lymphomatous primary solid tumors of the mesentery including 5 leiomyosarcomas and 5 hemangiomatous tumors. 4 None had demonstrable metastases. The thesis that the tumor arose in the vessels of the left pelvic side wall is supported by the large left pelvic vascular mass, the initial left inguinal symptoms, the pattern of lymphatic spread (with the most involved nodes in the pelvis) and the absence of demonstrable disease elsewhere. The histological features indicate a vascular tumor of angiosarcomatous and leiomyosarcomatous components. The extensive local disease precluded accurate identification of the precise vessel of ongm. No clear relationship between this tumor and the renal angiomyolipoma, a tumor which also contains vascular and smooth muscle elements, can be defined. Angiomyolipomas are uncommon new growths of uncertain histogenesis which occur in the kidney as isolated lesions and in asso4 Yannopoulos, K. and Stout, A. P.: Primary solid tumors of the mesentery. Cancer, 16: 914,
1963.
840
MOUNT, HAJDU AND WHITMORE
ciation with tuberous sclerosis. 5 They are usually benign but sometimes seem to be malignant histologically. Metastases are uncommon and have been reported only when associated with tuberous sclerosis. 6 The clinical and histological features in our case, that is normal kidneys on IVP and negative history for tuberous sclerosis, make it highly unlikely that this tumor represents metastases from an occult primary renal tumor. Radical excision of the tumor was attempted because of the long antecedent history and the patient's general good health as well as the un-
usual gross features and uncertain nature of the tumor. Stout has emphasized the fact that sarcomas vary in prognosis and natural history, depending on cell type, anatomic site and the patient's age. 7 Thus an accurate prognosis is impossible in the present case. The 4-year history suggests a slow growth pattern and favors aggressive attempts at palliation. The roles of chemotherapy and irradiation have not been established.
6 Hajdu, S. I. and Foote, F. W., Jr.: Angiomyolipoma of the kidney: report of 27 cases and review of the literature. J. Urol., 102: 396, 1969. 6 Tweeddale, D. N., Dawe, C. J., McDonald, J. R. and Culp, 0. S.: Angiolipoleiomyoma of the kidney; report of a case with observations on histogenesis. Cancer, 8: 759, 1955.
An angiomyosarcoma of the pelvis with lymphatic-borne metastases in a 59-year-old woman is reported.
SUMMARY
7 Stout, A. P.: Pathological aspects of soft part sarcomas. Ann. N.Y. Acad. Sci., 114: 1041, 1964.