ARCHIVES OF ANDROLOGY 48:101 106 (2002)

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AGGRESSIVE ANGIOMYXOMA OF THE SCROTUM

F.-P. CHUANG
S.-T. WU
S.-S. LEE
H.-I. CHEN
S.-Y. CHANG
D.-S. YU
G.-H. SUN
Division of Urology, Department of Surgery, Tri-Service General Hospital,
National Defense Medical Center, Taipei, Taiwan, Republic of China
Aggressive angiomyxoma is a rare and nonmetastasizing soft tissue tumor of the pelvis and perineum and occurs almost exclusively in adult females. It in ltrates locally and has a high risk of local
recurrence. Recommended treatment of the symptomatic patient is wide excision with tumor-free
margins and close postoperative monitoring. Herein, a case of aggressive angiomyxoma in an adult
male is described, which arose in the scrotum over 12 months. The tumor showed an intermediate
signal on T1-weighted MRI images. Contrast-enhanced T1-weighted images showed good enhancement. Wide excision of the tumor was performed. The surgical specimen measured 7 5 5 cm in
size and weighed 80 g. The tumors surface was smooth and had a gelatinous cut surface. Grossly, it
was encapsulated with a pleura-like membrane and had a nger-like projection. Microscopically,
sections showed many walled vessels of various sizes, collagen brils, a loose myxoid background,
and spindle stroma cells. MRI and CT showed the angiomatous and myxomatous nature of the
tumor wall. To the authors knowledge, this is the rst report to describe MRI ndings in scrotal
angiomyxoma.
Keywords

angiomyxoma, scrotum

Aggressive angiomyxoma is a rare mesenchymal soft tumor of the pelvis and perineum
and occurs almost exclusively in adult women. The tumors are characterized by local
in ltration, a gelatinous cut surface, frequent recurrence, no metastasis, slow growth, large
size, and nger-like projections, and are occasionally encapsulated [3, 7, 9]. Aggressive
angiomyxoma can be confused with malignant neoplasms and need to be distinguished
from malignant myxoid neoplasms. Aggressive angiomyxoma (AAM) was rst recognized by Steeper and Rosai in 1983 [8]. Reports of aggressive angiomyxoma in males
have nevertheless been rare, with only 9 cases arising in the scrotum reported in the
English-language literature [3]. Herein, a case of aggressive adult scrotal angiomyxom a
and its associated MRI ndings are described.

Address correspondence to: Guang-Huan Sun, Division of Urology, Department of Surgery, Tri-Service
General Hospital, No. 325, Section 2, Cheng-Kung Road, Neihu 114, Taipei, Taiwan, ROC.

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CASE REPORT
A 24-year-old man presented with a right scrotal mass that had slowly increased in
volume over the preceding year. No history of scrotal trauma, voiding di culties, or other
genitourinar y disease was elicited. On physical examination, the right scrotal mass measured 6 5 5 cm in size and was elastic in nature and nontender. Tumor markers, including a-FP and b-hCG, were normal. Ultrasonographi c examination showed it to be a
large, solid mass with homogeneous echogenicity and minimal calci cation over the right
side of the scrotum immediately below the right testis. The right testis was upwardly
displaced by the tumor. T1-weighted MRI images of the scrotum showed an intermediate
signal mass around 5 4 cm in area over the right half of the scrotum, below the right
testis, pushing the testis superolaterally. Postcontrast, the mass exhibited good vascular
perfusion, with a heterogenous signal and minimal free signal over the central portion
(Figures 1 and 2). The clinical impression was that of a paratesticular tumor and a wide
excision was performed.
During surgery, a mass was found between the spermatic fascia and the skin, which
displaced the testis upward. The boundary of the tumor was well circumscribed but the
originating organ of the tumor could not be determined. The surgical specimen measured
7 5 5 cm in size and weighed 80 g. The tumor had a smooth surface and was encapsulated with a pleura-like membrane, had a nger-like projection, and its cut surface
was gelatinous (Figure 3). Microscopically, sections of the mass showed many walled

Figure 1. T1-weighted scrotal MRI showed an intermediate signal mass around 5 4 cm in area over
the right side of the scrotum, lying below the superolaterally displaced testis. The mass showed good
peripheral enhancement, heterogenous signal, and minimal free signal, over the central portion (arrow).

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Aggressi ve Scrotal Angiomyxoma

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Figure 2. Postcontrast T1-weighted MRI showed good vascular perfusion, heterogenous signal, and
minimal free signal over the central portion (arrow).

Figure 3. The surgical specimen measured 7 5 5 cm and weighed 80 g. It had a smooth surface,
was encapsulated with a pleura-like membrane, had a nger-like projection, and its cut surface was
gelatinous.

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Figure 4. Microscopically, the sections show many walled vessels of various sizes, collagen brils, a
loose myxoid background, and spindle stroma cells (H&E, 200).

vessels of various sizes, collagen brils, a loose myxoid background, and spindle stroma
cells (Figure 4). Immunohistochemically , the spindle stroma cells were positive for vimentin but negative for S-100 and myosin. No further recurrence and no distant metastases were identi ed in the 26 months following surgery.

DISCUSSION
Since 1983, when Steeper and Rosai rst recognized aggressive angiomyxoma, around
50 cases have been reported [2, 6, 8]. AAM occurs almost exclusively in the vulva, vagina,
pelvic oor, and perineum of women. In males, aggressive angiomyxoma tends to originate from the scrotum, spermatid cord, inguinal tissues, perianally, perineally, and from
the pelvis. The tumor typically presents as an asymptomatic mass in females, and is
sometimes confused with Bartholins gland cysts or perineal hernias [3].
Grossly, AAM is a grayish-colored, nger-like tumor with a smooth surface but is
occasionally encapsulated. The tumor is soft and has a gelatinous cut surface or glistens
[6]. The characteristic histological ndings are ill-de ned margins, prominent vasculature
of small to medium caliber with thin and thick walls, abundant myxoid matrix, spindle
to stellate broblast-like cells, cytologically bland nuclei, few mitoses, and variable
brosis [2]. The thick-walled vessels are therefore useful for di erentiating between other
soft tumors of the pelvis, including myxoid neuro broma, myxoma, myxolipoma,

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angiomyo broblastoma, spindle cell lipoma, myxoid leiomyoma, myxoid leiomyosarcoma, myxoid malignant brous histiocytoma, myxoid liposarcoma, and Botryoid rhabdomyosarcoma [3]. Immunohistochemically , the spindle stroma cells in AAM are positive
for vimentin and variably positive for muscle-speci c actin [7]. Aggressive angiomyxom a
tends to grow very slowly, and patients tend to demonstrate a prolonged period of disease
progression, with most patients being essentially asymptomatic.
Preoperatively, diagnosis is di cult and error prone [6]. AAM has been described as
exhibiting low attenuation on CT. One report has described the tumor as appearing cystic
on both sonography and CT [4, 5, 10]. From MR imaging and CT ndings, regions that
enhance gradually with high signal intensity on T2-weighted MRI probably represent
myxoid components, whereas regions that enhance immediately with low signal intensity
on T2-weighted MRI probably represent angiomatous components. For this reason, typical imaging studies show an irregular enhancement pattern after contrast administration
[4], as in the case described herein.
AAM tends to in ltrate and has a high risk of local recurrence. The earliest recurrence
has been reported as appearing 9 months after surgery and the latest, 14 years after surgery
[7]. That frequent recurrence (37%) may be attributable to incomplete tumor resection.
Wide-margin excision is therefore justi able for the prevention of recurrence [6]. Accordingly, imaging procedures that can clearly delineate the anatomical extent of the tumor three dimensionally are essential to surgical planning. Few cases involving MRI for
the assessment of tumor invasion have been described. MRI can provide sagittal, coronal,
and axial images and is a valuable tool for the assessment of tumor involvement and for
surgical planning [1]. In the present case, no recurrence was found in the 26 months following surgery, but long-term follow-up remained necessary because of the risk of late
recurrence. To date, no distant metastases have been found.
In summary, AAM is a benign tumor for which the mainstays of treatment are wide
excision with tumor-free margins and close postoperative monitoring [2].
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