Skin nonmelanocytic tumor

Fibrous, fibrohistiocytic and myofibroblastic neoplasms

Multinucleate cell angiohistiocytoma


Resident / Fellow Advisory Board: Farres Obeidin, M.D.
Editorial Board Member: Jonathan D. Ho, M.B.B.S., D.Sc.
Simon F. Roy, M.D.
Jennifer M. McNiff, M.D.

Topic Completed: 7 October 2021

Minor changes: 7 October 2021

Copyright: 2021, PathologyOutlines.com, Inc.

PubMed Search: Multinucleate cell angiohistiocytoma

Simon F. Roy, M.D.
Jennifer M. McNiff, M.D.
Page views in 2020: 61
Page views in 2021 to date: 728
Cite this page: Roy SF, McNiff JM. Multinucleate cell angiohistiocytoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticmultinucleatecellangio.html. Accessed December 3rd, 2021.
Definition / general
  • Uncommon cutaneous lesion
  • Vascular and fibrohistiocytic lineage are hypothesized
  • Characterized by odd multinucleated fibroblasts, superficial fibrosis and thickening of superficial papillary dermal vessels
Essential features
  • Presence of odd multinucleated fibroblasts
  • Presence of superficial parallel fibrosis
  • Presence and thickening of superficial papillary dermal vessels
  • Absence of perifollicular fibrosis
ICD coding
  • ICD-10: D23.9 - other benign neoplasm of skin, unspecified
  • ICD-11: 2F23 - benign dermal fibrous or fibrohistiocytic neoplasms
Epidemiology
Sites
  • Dorsal hands or fingers most frequent; when multiple lesions are found, they are most likely to affect the hands or fingers (J Cutan Pathol 2019;46:563)
  • Lower extremities
  • Trunk or back
  • Head and neck (rare, most often these are fibrous papules with multinucleated stromal cells, not multinucleate cell angiohistiocytoma)
Pathophysiology
  • Currently unknown
  • Role for increased mast cells had been postulated but when mast cell counts were compared with counts of normal skin, no difference was found (J Cutan Pathol 2019;46:563)
  • Role for fibroblasts, given the increased intralesional elastic fibers in generalized multinucleate cell angiohistiocytoma, has been postulated (J Dermatol 2021;48:114)
  • Role for estrogen receptor alpha overexpression has been proposed (Am J Dermatopathol 2010;32:655)
Etiology
  • Unknown
Clinical features
Diagnosis
  • Made on H&E stain with light microscopy in conjunction with clinical findings
Prognostic factors
Case reports
Treatment
Clinical images

Contributed by Simon F. Roy, M.D. and Jennifer M. McNiff, M.D.

Erythematous papules on the trunk

Flesh colored papules

Gross description
Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Simon F. Roy, M.D. and Jennifer M. McNiff, M.D.
Superficial papillary dermal fibrosis

Superficial papillary dermal fibrosis

Prominent papillary dermal vessels

Prominent papillary dermal vessels

Odd multinucleated cells

Odd multinucleated cells

Superficial dermal vessels

Superficial dermal vessels

Positive stains
Negative stains
Sample pathology report
  • Skin, left dorsal hand, punch biopsy:
    • Multinucleate cell angiohistiocytoma (see comment)
    • Comment: Sections show superficial parallel fibrosis, thickened and abundant papillary dermal vessels and stellate multinucleated cells consistent with multinucleate cell angiohistiocytoma.
Differential diagnosis
  • Fibrous papule of the nose:
    • Can also show prominent superficial papillary dermal vessels and multinucleated cells but the fibrosis is not parallel to the epidermis
    • Shows concentric perifollicular fibrosis in most instances, unlike multinucleate cell angiohistiocytoma
    • Much more commonly seen on the face / nose (J Cutan Pathol 2019;46:563)
  • Dermatofibroma:
    • More common on lower extremities
    • Atrophic vascular variant of dermatofibroma may mimic multinucleate cell angiohistiocytoma
    • Fibrosis in dermatofibroma is more storiform, rather than parallel to the epidermis
    • May also show multinucleated cells and factor XIIIa positivity
    • Follicular induction and beta catenin positivity in underlying dermal fibroblasts is frequent in dermatofibroma but absent in multinucleate cell angiohistiocytoma (J Cutan Pathol 2019;46:563)
  • Telangiectasia macularis eruptive perstans:
    • Eosinophils are frequent in the inflammatory infiltrate of telangiectasia macularis eruptive perstans but very rare in multinucleate cell angiohistiocytoma
    • Multinucleated cells in telangiectasia macularis eruptive perstans are slightly different, showing < 3 nuclei, contrarily to multinucleate cell angiohistiocytoma
    • Mast cell counts are elevated in telangiectasia macularis eruptive perstans compared with multinucleate cell angiohistiocytoma or normal skin (J Cutan Pathol 2019;46:563)
Board review style question #1

There are multiple red papules on the dorsal hands with multinucleated stromal cells, thickened superficial papillary dermal vessels and superficial dermal fibrosis arrayed in parallel to the epidermis. What is the best diagnosis?

  1. Dermatofibroma
  2. Fibrous papule
  3. Multinucleate cell angiohistiocytoma (MCAH)
  4. Telangiectasia macularis eruptive perstans (TMEP)
Board review style answer #1
C. Multinucleate cell angiohistiocytoma (MCAH)

Comment Here

Reference: Multinucleate cell angiohistiocytoma
Board review style question #2

Which histologic feature is absent in multinucleate cell angiohistiocytoma?

  1. Concentric perifollicular fibrosis
  2. Multinucleated stromal cells
  3. Prominent and thickened superficial papillary dermal vessels
  4. Superficial dermal fibrosis arrayed in parallel
Board review style answer #2
A. Concentric perifollicular fibrosis. Concentric perifollicular fibrosis is rather a feature of fibrous papule of the nose and is the main histopathological criteria to distinguish these entities, along with knowledge of the anatomical site.

Comment Here

Reference: Multinucleate cell angiohistiocytoma
Back to top
Image 01 Image 02