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Aggressive angiomyxoma first diagnosed as levator hernia
Aggressive angiomyxoma first diagnosed as levator hernia Gregory P. Sutton, MD: Robert E. Rogers, MD: Lawrence M. Roth, MD: and Clarence E. Ehrlich, MD" IndianapolIS, Indiana When a 32·year-old woman was first seen, physical findings suggested she had a large levator hernia, but at the time of surgical resection an aggressive angiomyxoma was found . (AM J GaSTET GVNECOL 1989;161 :73-5.)
Key words: Angiomyxoma, levator hernia Aggressive angiomyxoma is a benign but locally aggressive neoplasm seen almost exclusively in the pelvis or perineum of premenopausal women. I 2 The tumor in this case was initially diagnosed as a levator sling hernia. A pelvic computerized axial tomogram helped to clarify the diagnosis and to direct surgical excision.
Case report I. W., a 32-year-old white woman (para 2-0-0-2) was referred to Indiana University Medical Center because of a large, soft mass of the left vulva and buttock. The patient noticed asymmetry of her left buttock shortly after she was uneventfully delivered of her second child in November 1981. On standing, the mass protruded into the left buttock and was accompanied by a progressive traction sensation in the lower abdomen. She had no associated bowel complaints but did have progressive urinary frequency. She was thought to have a levator or perineal hernia, and was referred for evaluation in June 1987. The patient was a healthy-appearing postal worker whose physical examination results were normal. On pelvic examination a 6 x 8 em protuberant soft mass was identified arising from the anterior left buttock and labium majus (Fig. 1). The mass could be easily reduced through a palpable 7 em defect in the left midlevator plate and gave the impression of a hernia. The uterus was retrodisplaced, and there was a mass sensation on bimanual examination. A computerized tomogram showed an 11 x 8 cm well-demarcated mass that extended from the pelvis to the left buttock. There was a central, cleft-like hypodense area, but no fat density was observed (Fig. 2). An intravenous pyelogram demonstrated moderate hy-
From the Departments of Obstetncs and Gynecology' and Pathology,' Indiana UnIVeTSlty School of Medtcme. Supported In part by the Amencan Cancer Soczety Career Development Award 86-74. ReceIVed for publication September 7, 1988; reVISed December 13, 1988; accepted january II , 1989. Repnnt requests: Gregory P. Sutton, MD, ASSOCiate Professor and Chief, G.vnecologlc Oncology, Department of Obstetrics and G.vnecology, Indiana UniveTSlty School oj Medlcme, 926 W. Michigan St .. Indianapolis, IN 46223.
Fig. 1. Appearance of mass distortmg left labium majus and buttock.
dronephrosis on the right side, marked displacement of the bladder to the right, and deviation of the left ureter across the midline of the pelvis (Fig. 3). A biopsy specimen obtained through the left side of the vagina showed myxomatous and vascular components diagnostic of aggressive angiomyxoma, and the next day the large mass was completely resected using a combined abdominoperineal approach. The reproductive organs were preserved. The patient did well and had no evidence of disease 1 year postoperatively. Estrogen and progesterone receptor content were determined with a dextran-coated charcoal assay. The estrogen receptor level was 29.4 femtomoles/mg of protein and the progesterone receptor level was 5.2 femtomoles/mg of protein; both were intermediate levels. Pathology Gross and microscopic findings. On gross examination the specimen was soft, easily compressible, and somewhat fleshy in consistency. The cut surface of the tumor was circumscribed, soft, gray-white, homogeneous, gelatinous, and moist (Fig. 4). Histologically, the tumor was circumscribed and was
73
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Sutton et al.
July 1989 Am J Obstet Gynecol
Fig. 2. Computerized tomography shows rightward displacement of bladder by heterogenous mass.
sional small inconspicuous nucleolus, but lacking mitoses. Interspersed throughout the myxomatous matrix were small to medium-sized vessels, some of which were thin-walled and resembled capillaries, whereas others had thick muscular walls. In some areas around medium-sized muscular blood vessels, slips of smooth muscle were observed within the myxomatous stroma. No small nerves were noted within the tumor.
Fig. 3. Intravenous pyelogram demonstrates marked displacement of bladder and left ureter and right hydronephrosis.
confined by a condensation of eosinophilic stromal material and tumor cells. Two distinct components were seen. The first, a loosely textured myxomatous component, contained bipolar to multipolar mesenchymal cells with small uniform vesicular nuclei with an occa-
Comment This case was, for the most part, a typical aggressive angiomyxoma, although it was better circumscribed than many of the reported cases. Although multiple small nerves have been found within the myxoid stroma of several of the tumors, this case is the first in which fascicles of smooth muscle cells were present focally within the stroma. The ultrastructural features of the neoplasm were similar to those of the previously studied cases. Steeper and Rosai' coined the term "aggressive angiomyxoma" in 1983 to describe nine cases of a benign but locally infiltrative neoplasm that arose in the pelvis or perineum of women who ranged from 21 to 38 years of age. The next year Begin and associates 2 reported nine additional cases including two tumors that arose in male patients and one in a 63-year-old woman. These masses were alternatively diagnosed as Bartholin cysts or neoplasms, vaginal prolapse, or hernias of the pelvic floor or obturator fossa. The history and physical fiudings in the current case were strongly suggestive of a levator hernia. Computed tomographic findings, however, suggested the diagnosis of aggressive angiomyxoma. These
Aggressive angiomyxoma diagnosed as levator hernia
VoIUJll(, 161
:'\umbcr I
75
Fig. 4. Cut surface of neoplasm was homogeneous. moist, and gelatinous.
findings underscore the importance of radiographic evaluation before operation for suspected perineal or levator hernias. Local infiltration of these tumors is characteristic, although they may be clearly demarcated in some areas. Infiltration into skeletal muscle and fat has been observed. Local recurrences at intervals from 9 to 180 months were reported in 10 of the 18 cases reported by Steeper et al. I and Begin et al! All were thought to be a result of an inadequate primary excision. No metastases or deaths have been described. Aggressive angiomyxoma must be distinguished from a variety of similar neoplasms including myxoid lipoma and liposarcoma, soft tissue myxoma, nerve sheath tumor, myxoid neurofibroma, embryonal rhabdomyosarcoma, malignant fibrous histiocytoma, and fibromatosis with focal myxoid areas.
Vulvar, pelvic, or perineal masses that are soft and slow growing must be evaluated with the diagnosis of aggressive angiomyxoma in mind so that adequate extirpative surgery may be performed and recurrences obviated. The presence of estrogen and progesterone receptor protein suggests a possible role for hormonal therapy in those cases that cannot be completely removed . REFERENCES 1. Steeper TA, Rosai J. Aggressive angiomyxoma of the female pelvis and perineum. Am J Surg Pathol 1983;7:46375. 2. Begin LR, Clement PB, Kirk ME, et al. Aggressive angiomyxoma of pelvic soft parts : a clinicopathologic study of nine cases. Hum Pathol 1985;16:621-8.
Key words: Angiomyxoma, levator hernia Aggressive angiomyxoma is a benign but locally aggressive neoplasm seen almost exclusively in the pelvis or perineum of premenopausal women. I 2 The tumor in this case was initially diagnosed as a levator sling hernia. A pelvic computerized axial tomogram helped to clarify the diagnosis and to direct surgical excision.
Case report I. W., a 32-year-old white woman (para 2-0-0-2) was referred to Indiana University Medical Center because of a large, soft mass of the left vulva and buttock. The patient noticed asymmetry of her left buttock shortly after she was uneventfully delivered of her second child in November 1981. On standing, the mass protruded into the left buttock and was accompanied by a progressive traction sensation in the lower abdomen. She had no associated bowel complaints but did have progressive urinary frequency. She was thought to have a levator or perineal hernia, and was referred for evaluation in June 1987. The patient was a healthy-appearing postal worker whose physical examination results were normal. On pelvic examination a 6 x 8 em protuberant soft mass was identified arising from the anterior left buttock and labium majus (Fig. 1). The mass could be easily reduced through a palpable 7 em defect in the left midlevator plate and gave the impression of a hernia. The uterus was retrodisplaced, and there was a mass sensation on bimanual examination. A computerized tomogram showed an 11 x 8 cm well-demarcated mass that extended from the pelvis to the left buttock. There was a central, cleft-like hypodense area, but no fat density was observed (Fig. 2). An intravenous pyelogram demonstrated moderate hy-
From the Departments of Obstetncs and Gynecology' and Pathology,' Indiana UnIVeTSlty School of Medtcme. Supported In part by the Amencan Cancer Soczety Career Development Award 86-74. ReceIVed for publication September 7, 1988; reVISed December 13, 1988; accepted january II , 1989. Repnnt requests: Gregory P. Sutton, MD, ASSOCiate Professor and Chief, G.vnecologlc Oncology, Department of Obstetrics and G.vnecology, Indiana UniveTSlty School oj Medlcme, 926 W. Michigan St .. Indianapolis, IN 46223.
Fig. 1. Appearance of mass distortmg left labium majus and buttock.
dronephrosis on the right side, marked displacement of the bladder to the right, and deviation of the left ureter across the midline of the pelvis (Fig. 3). A biopsy specimen obtained through the left side of the vagina showed myxomatous and vascular components diagnostic of aggressive angiomyxoma, and the next day the large mass was completely resected using a combined abdominoperineal approach. The reproductive organs were preserved. The patient did well and had no evidence of disease 1 year postoperatively. Estrogen and progesterone receptor content were determined with a dextran-coated charcoal assay. The estrogen receptor level was 29.4 femtomoles/mg of protein and the progesterone receptor level was 5.2 femtomoles/mg of protein; both were intermediate levels. Pathology Gross and microscopic findings. On gross examination the specimen was soft, easily compressible, and somewhat fleshy in consistency. The cut surface of the tumor was circumscribed, soft, gray-white, homogeneous, gelatinous, and moist (Fig. 4). Histologically, the tumor was circumscribed and was
73
74
Sutton et al.
July 1989 Am J Obstet Gynecol
Fig. 2. Computerized tomography shows rightward displacement of bladder by heterogenous mass.
sional small inconspicuous nucleolus, but lacking mitoses. Interspersed throughout the myxomatous matrix were small to medium-sized vessels, some of which were thin-walled and resembled capillaries, whereas others had thick muscular walls. In some areas around medium-sized muscular blood vessels, slips of smooth muscle were observed within the myxomatous stroma. No small nerves were noted within the tumor.
Fig. 3. Intravenous pyelogram demonstrates marked displacement of bladder and left ureter and right hydronephrosis.
confined by a condensation of eosinophilic stromal material and tumor cells. Two distinct components were seen. The first, a loosely textured myxomatous component, contained bipolar to multipolar mesenchymal cells with small uniform vesicular nuclei with an occa-
Comment This case was, for the most part, a typical aggressive angiomyxoma, although it was better circumscribed than many of the reported cases. Although multiple small nerves have been found within the myxoid stroma of several of the tumors, this case is the first in which fascicles of smooth muscle cells were present focally within the stroma. The ultrastructural features of the neoplasm were similar to those of the previously studied cases. Steeper and Rosai' coined the term "aggressive angiomyxoma" in 1983 to describe nine cases of a benign but locally infiltrative neoplasm that arose in the pelvis or perineum of women who ranged from 21 to 38 years of age. The next year Begin and associates 2 reported nine additional cases including two tumors that arose in male patients and one in a 63-year-old woman. These masses were alternatively diagnosed as Bartholin cysts or neoplasms, vaginal prolapse, or hernias of the pelvic floor or obturator fossa. The history and physical fiudings in the current case were strongly suggestive of a levator hernia. Computed tomographic findings, however, suggested the diagnosis of aggressive angiomyxoma. These
Aggressive angiomyxoma diagnosed as levator hernia
VoIUJll(, 161
:'\umbcr I
75
Fig. 4. Cut surface of neoplasm was homogeneous. moist, and gelatinous.
findings underscore the importance of radiographic evaluation before operation for suspected perineal or levator hernias. Local infiltration of these tumors is characteristic, although they may be clearly demarcated in some areas. Infiltration into skeletal muscle and fat has been observed. Local recurrences at intervals from 9 to 180 months were reported in 10 of the 18 cases reported by Steeper et al. I and Begin et al! All were thought to be a result of an inadequate primary excision. No metastases or deaths have been described. Aggressive angiomyxoma must be distinguished from a variety of similar neoplasms including myxoid lipoma and liposarcoma, soft tissue myxoma, nerve sheath tumor, myxoid neurofibroma, embryonal rhabdomyosarcoma, malignant fibrous histiocytoma, and fibromatosis with focal myxoid areas.
Vulvar, pelvic, or perineal masses that are soft and slow growing must be evaluated with the diagnosis of aggressive angiomyxoma in mind so that adequate extirpative surgery may be performed and recurrences obviated. The presence of estrogen and progesterone receptor protein suggests a possible role for hormonal therapy in those cases that cannot be completely removed . REFERENCES 1. Steeper TA, Rosai J. Aggressive angiomyxoma of the female pelvis and perineum. Am J Surg Pathol 1983;7:46375. 2. Begin LR, Clement PB, Kirk ME, et al. Aggressive angiomyxoma of pelvic soft parts : a clinicopathologic study of nine cases. Hum Pathol 1985;16:621-8.