Penis and scrotum
Inflammatory lesions
Peyronie disease


Topic Completed: 1 February 2010

Revised: 25 October 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Peyronie disease[TI]

Alcides Chaux, M.D.
Antonio L. Cubilla, M.D.
Page views in 2018: 465
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Cite this page: Chaux A, Cubilla AL, Arora K. Peyronie disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/penscrotumpeyronie.html. Accessed December 7th, 2019.
Definition / general
  • Dense focal fibrosis of dermis and Buck fascia of penile shaft between corpora cavernosa and tunica albuginea, usually causing curvature towards side of lesion and restricting movement of these structures during erection
Terminology
  • Also called "induratio penis plastica", penile fibromatosis
Epidemiology
Palmar fibromatosis (Dupuytren's contracture)
  • 75% are men
  • 50% are bilateral, 10% also have plantar disease, 1 - 4% have penile fibromatosis

Plantar fibromatosis (Ledderhose's disease)
  • Common in boys < 10 years old and teenagers
  • Associated with palmar and penile fibromatosis, also continuous phenobarbital treatment for epilepsy (Epilepsia 2008;49:1965)
  • Usually NOT associated with contractures

Penile fibromatosis (Peyronie's)
Etiology
  • Considered a variant of superficial fibromatosis which preferentially affects the tunica albuginea
  • May be related to microtrauma (coital, urethral instrumentation), which releases fibroblast related cytokines
  • May arise secondary to urethritis as a sclerosing inflammatory process
  • Etiology may be related to Parc protein (BJU Int 2010;106:1706) or Wnt2 (J Sex Med 2012;9:1430)
Clinical features
  • May be associated with carcinoid syndrome, Dupuytren contracture
  • May be associated with beta blockers, thiazides (J Sex Med 2010;7:1529), hypertension, diabetes and immune reactions
  • Fibrosis produces an abnormal curvature of the penis, which may cause pain during erection and intercourse
  • In some cases, nodules adherent to tunica albuginea are noted over the mid dorsal line
  • May be clinically mimicked by epithelioid sarcoma (Int J Impot Res 2003;15:378)
Case reports
Treatment
Clinical images

AFIP images

Peyronie disease



Images hosted on other servers:

Various images

Preoperative

Gross description
  • Irregular areas of fibrosis in dermis and fascia of penile shaft, mainly related to and in contact with tunica albuginea
  • Occasionally nodular areas of fibrosis may predominate
Gross images

AFIP images

Tunica albuginea
surrounding both
corpora cavernosa
is thickened

Microscopic (histologic) description
  • Dense fibrous nodules, similar to those in Dupuytren contracture, fibromatosis and other desmoplastic conditions involving myofibroblasts
  • More dense and less cellular than other types of superficial fibromatosis
  • Perivascular lymphoid infiltrate in early stages of disease
  • Fibrotic tunica albuginea with extension of fibrosis to corpus cavernosum
  • Abnormal vessels with venous leakages
  • Calcification or ossification may occur

Palmar fibromatosis (Dupuytren's contracture)
  • Proliferative phase:
    • Uniform, plump, immature spindle cells (myofibroblasts and fibroblasts) with bland nuclei and indistinct nucleoli
    • Moderate collagen and elongated vessels
  • Older lesions:
    • More dense collagen, less cellularity
    • Variable mitotic figures
    • Occasional attachment to dermis or cartilaginous metaplasia
    • Usually no infiltration of surrounding tissue beyond subcutis

Plantar fibromatosis (Ledderhose's disease)
  • Proliferative phase:
    • Hypercellular collection of uniform, plump, immature spindle cells with bland nuclei and indistinct nucleoli
    • Moderate collagen and elongated vessels
    • Variable multinucleated giant cells (Am J Surg Pathol 2002;26:244)
  • Older lesions:
    • Denser collagen, less cellularity
    • Often prominent chronic inflammation, variable mitotic figures and hemosiderin

Penile fibromatosis (Peyronie's)
  • Disorganization of collagen of tunica albuginea with formation of nodules, often hyalinizing fibrosis, perivascular lymphocytic infiltrate in 1/3, linear band of calcification in 1/4 (J Urol 1997;157:282)
Microscopic (histologic) images

AFIP images

Peyronie disease

Whole mount shows
focal thickening of
tunica albuginea
and calcification



  Superficial fibromatosis - other sites:

Mitotic figures
may be present
in cellular regions
but are never atypical

Nodule of variably cellular fibroblastic tissue

Electron microscopy description
Palmar fibromatosis (Dupuytren's contracture)
  • Fibroblasts and myofibroblasts

Plantar fibromatosis (Ledderhose's disease)
  • Fibroblasts and myofibroblasts

Penile fibromatosis (Peyronie's)
  • Penile plaques are composed of collagen fibrils, amorphous particulate material and fibroblasts (Int J Urol 1997;4:274)
Differential diagnosis
Palmar fibromatosis (Dupuytren's contracture)
  • Fibrosarcoma: single large mass of deep soft tissue with intersecting bundles of cells whose nuclei have abnormal chromatin
  • Epithelioid sarcoma: common in hands, but some cells have distinctive epithelioid appearance with abundant bright eosinophilic cytoplasm, also necrosis, keratin+, CD34+
  • Desmoid tumors: rare in hand, dominant mass infiltrates skeletal muscle

Plantar fibromatosis (Ledderhose's disease)
  • Calcifying aponeurotic fibroma: plump or epithelioid fibroblasts palisading around cartilage and spotty calcification
  • Desmoid fibromatosis: rare in feet, infiltrates skeletal muscle, > 3 cm, often beta-catenin+
  • Fibrosarcoma: single large mass of deep soft tissue with intersecting bundles of cells with abnormal chromatin, herringbone pattern
  • Monophasic synovial sarcoma: uniformly hypercellular, often staghorn vascular pattern or ropy collagen

Penile fibromatosis (Peyronie's)
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