Skin nonmelanocytic tumor

Fibrous, fibrohistiocytic and myofibroblastic neoplasms

Angiofibroma / fibrous papule



Minor changes: 11 October 2021

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PubMed Search: Angiofibroma[TI] OR fibrous papule[TI] review[PT]

See Also: Soft tissue - cellular angiofibroma

Said Albahra, M.D.
Gregory A. Hosler, M.D., Ph.D.
Page views in 2020: 18,754
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Cite this page: Albahra S, Hosler GA. Angiofibroma / fibrous papule. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticangiofibroma.html. Accessed December 3rd, 2021.
Definition / general
  • Benign fibrohistiocytic tumor consisting of dermal dendritic cells
Essential features
  • Common benign lesion composed of stellate, factor XIIIa positive stromal cells
  • Most common variant referred to as fibrous papule when present on the central face
Terminology
  • Solitary angiofibroma on the central face referred to as fibrous papule
  • Multiple angiofibromatous lesions in tuberous sclerosis referred to as adenoma sebaceum
  • On the penis, referred to as pearly penile papules
ICD coding
  • ICD-O: 9160/0 - angiofibroma, NOS
  • ICD-10: D21.9 - benign neoplasm of connective and other soft tissue, unspecified
  • ICD-10: D23.30 - other benign neoplasm of skin of unspecified part of face
Epidemiology
Sites
  • Central face, particularly on the nose
Pathophysiology
  • Unknown at this time
Etiology
Clinical features
  • Dome shaped, skin colored papules measuring a few millimeters
  • Asymptomatic
Diagnosis
  • Diagnosis can be made on biopsy
Case reports
Treatment
  • For cosmetic purposes, can be removed by excisional / shave biopsy or electrosurgery
Clinical images

Images hosted on other servers:
Fibrous papule of the nose Various images

Fibrous papule of the nose

Tuberous sclerosis angiofibromas

Tuberous sclerosis angiofibromas

Microscopic (histologic) description
  • Slightly raised dermal lesion composed of collagenous stroma with increased vasculature
  • Increased stromal cells with varying morphology
    • Cells can be plump, spindle shaped, stellate or multinucleate
  • Mitotic figures are rare
  • Epidermis uninvolved but can appear flattened or atrophic
  • Histologic variants exist:
Microscopic (histologic) images

Contributed by Gregory A. Hosler, M.D., Ph.D.
Fibrous papule, superficial shave

Fibrous papule, superficial shave

Fibrous papule, classic Fibrous papule, classic

Fibrous papule, classic

Hypercellular variant Hypercellular variant

Hypercellular variant


Giant hypercellular variant Giant hypercellular variant

Giant hypercellular variant

Clear cell variant Clear cell variant

Clear cell variant

Pigmented variant Pigmented variant

Pigmented variant



Contributed by Hillary Rose Elwood, M.D.
Fibrous papule with multinucleate cells

Fibrous papule with multinucleate cells

Fibrous papule, classic Fibrous papule, classic

Fibrous papule, classic

Clear cell variant

Clear cell variant

Virtual slides

Images hosted on other servers:
Cutaneous angiofibroma

Cutaneous angiofibroma

Positive stains
Negative stains
Electron microscopy description
Videos

Fibrous papule (angiofibroma)
by Dr. Gardner

Sample pathology report
  • Skin, nasal tip, shave biopsy:
    • Fibrous papule
Differential diagnosis
  • Adenoma sebaceum (angiofibroma of tuberous sclerosis):
    • Fewer bizarre dermal stromal cells than in fibrous papule
    • Vessels are smaller and likely to show more concentric fibrosis
  • Pearly penile papules:
    • Absence of pilosebaceous follicles
    • Multiple in number and located on the penis
  • Angioma:
    • Proliferation of ectatic vessels
    • Lacks stellate stromal cells
  • Fibrosing / sclerotic nevus:
    • Lacks stellate cells and cellular stroma
    • S100 positive
  • Pleomorphic fibroma:
    • Pleomorphic cells can resemble those occasionally present in fibrous papule
    • More commonly located on the truck and extremities
    • Lacks vascularity
  • Scar:
    • Horizontal orientation of fibroblasts with vertically oriented blood vessels
Board review style question #1

Which of the following is most commonly expressed in this lesion from the nasal ala of a 45 year old woman?

  1. Cytokeratin AE1 / AE3
  2. Factor XIIIa
  3. MART1
  4. NKI-C3
  5. S100
Board review style answer #1
B. Factor XIIIa. The spindle cells of fibrous papule express factor XIIIa. Although NKI-C3 may be positive in the clear cell variant of fibrous papule, it is negative in classic variant shown here. S100, MART1 and cytokeratins are negative.

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Reference: Angiofibroma / fibrous papule
Board review style question #2
Multiple facial fibrous papules are associated with what hereditary condition?

  1. Brooke-Spiegler syndrome
  2. Cowden syndrome
  3. Gardner syndrome
  4. Reed syndrome
  5. Tuberous sclerosis
Board review style answer #2
E. Tuberous sclerosis. Multiple facial fibrous papules are classically associated with the autosomal dominant neurocutaneous syndrome of tuberous sclerosis. Angiofibromatous lesions found in patients with this syndrome are referred to as adenoma sebaceum (a misnomer, as there is no adenomatous proliferation of sebaceous glands). Other hereditary conditions also associated with multiple facial fibrous papules include multiple endocrine neoplasia type I (MEN 1), neurofibromatosis II and Birt-Hogg-Dubé.

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Reference: Angiofibroma / fibrous papule
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