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Penis and scrotum
Non-neoplastic lesions of scrotum
Aggressive angiomyxoma
Editors: Antonio Cubilla, M.D. and Alcides Chaux, M.D. (see Author/Reviewers page)
Revised: 22 May 2010, last major update May 2010
Copyright: (c) 2002-2010, PathologyOutlines.com, Inc.
Definition
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● Infiltrative, hypocellular, bland myxoid lesion of perineum and pelvis, much less common in males than females (Am J Clin Pathol 1995;104:391)
Epidemiology
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● Usually adults, but pediatric cases have been reported (Pediatr Dev Pathol 2003;6:187)
Clinical features
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● Slow-growing painless mass, months to years of duration
● Infiltration of deep tissues may occur
● May simulate an inguinal hernia (Asian J Androl 2007;9:723)
● High incidence of local recurrence (Int J Gynecol Cancer 2010;20:303)
Case reports
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● 64 year old man with clinical hydrocele (Case Report Med 2009;2009:157624)
Clinical images
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Marked swelling of skin, Intraoperative scrotal mass
resembling lymphedema
Gross description (Macroscopy)
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● Irregular and infiltrative borders
● Gelatinous, mucoid cut surface
● Tumor size 3-60 cm (usually >5 cm)
Gross images
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Gelatinous cut surface Vulvar tumor
Testis
Ill-defined mass of myxoid tissue fills the paratesticular region and extends to the scrotal skin (top). Testis is at bottom (AFIP image courtesy of Dr. J. C. Iezzoni, Charlottesville, VA)
Micro description (Histopathology)
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● Hypocellular, stellate/spindle stromal cells with delicate cytoplasmic processes
● Prominent myxoid (mucicarmine positive) to collagenous stroma
● Abundant small to medium-sized blood vessels, many with hypertrophy / hyalinization (Am J Surg Pathol 1992;16:1059)
● Extravasation of erythrocytes is common
● No atypia or significant mitotic activity
Micro images
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Thick walled vessels, myxoid to collagenous stroma Delicate vessels and myxoid stroma
CD34+ Masson trichrome stains collagen fibers
Spermatic cord
Spindle cells in Variable sized vessels Perivascular spindled cells
myxoid matrix
Vimentin+ CD34+ Scattered PR+
Vulva
Other images: vulvar tumor #1; #2; #3; #4
Positive stains
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● Vimentin, CD34
● Variable ER, PR (Ann Diagn Pathol 2006;10:197) and androgen receptors (Int J Urol 2003;10:672)
Negative stains
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● Desmin, S100, CD68
● Muscle specific actin and alpha smooth muscle actin are negative or variable
Molecular / cytogenetics
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● HMGA2 rearrangements in 1/3 (Genes Chromosomes Cancer 2007;46:981)
Differential Diagnosis
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● Superficial (cutaneous) angiomyxoma: well-defined borders, nodular/multinodular pattern of growth
● Angiomyofibroblastoma: hypercellular areas, plump neoplastic cells, perivascular accentuation of stromal cells, edematous (not myxoid) stroma
Additional references
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End of Penis and scrotum > Non-neoplastic lesions of scrotum > Aggressive angiomyxoma
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