Skin nonmelanocytic tumor

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Sweat gland derived (apocrine & eccrine glands)

Hidradenoma papilliferum



Last author update: 3 January 2022
Last staff update: 24 June 2022 (update in progress)

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PubMed Search: Hidradenoma papilliferum [title]

Bitania Wondimu, M.D.
Oliver Hsinju Chang, M.D.
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Cite this page: Wondimu B, Chang OH. Hidradenoma papilliferum. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocytichidradenomapap.html. Accessed December 6th, 2022.
Definition / general
  • Hidradenoma papilliferum is a benign dermal papulonodular tumor almost always found in the vulva / perianal region of women
Essential features
  • Benign neoplasm of modified anogenital mammary-like glands, predominantly found in the vulva / perianal region of women
  • Histopathology features include characteristic dermal maze-like growth with papillary / glandular / cyst-like architecture, often with apocrine differentiation
Terminology
  • Also referred to as papillary hidradenoma
ICD coding
  • ICD-O: 8405/0 - papillary hidradenoma / hidradenoma papilliferum
  • ICD-10: D23.5 - other benign neoplasm of skin of trunk
  • ICD-10: D28.0 - benign neoplasm of vulva
Epidemiology
Sites
  • Vulva or perianal region
  • Rarely involves ectopic sites (head, neck, chest, abdomen) (Indian J Pathol Microbiol 2018;61:287)
    • 60% of ectopic HP is present in head and neck region, including eyelid, forehead, face, external auditory canal, post auricular region
    • Thought to arise from modified apocrine glands in eyelids / external auditory canal or heterotopic apocrine glands
    • Rare case reports of sebaceous differentiation in ectopic HP (Pathology 2019;51:362)
Pathophysiology
Etiology
  • Unknown
Clinical features
Diagnosis
Prognostic factors
  • Generally excellent prognosis and curative with complete excision
  • Can very rarely transform into malignant hidradenocarcinoma papilliferum or aggressive adenosquamous carcinoma (Gynecol Oncol 1989;35:395)
Case reports
Treatment
Clinical images

Images hosted on other servers:
Hidradenoma papilliferum of anus

Hidradenoma papilliferum of anus

Eyelid papule

Eyelid papule

Left areola erythematous papule

Left areola erythematous papule

External auditory canal

External auditory canal

Gross description
  • Solid, skin colored papule, usually < 1 - 2 cm
Microscopic (histologic) description
  • Well circumscribed dermal maze-like or arborizing proliferation (rarely connected to epidermis), with a combination of papillary, cystic or glandular architecture
  • 2 types of epithelia:
  • Entrapment of epithelial cells in connective tissue may mimic infiltrative pattern (Arch Gynecol Obstet 2011;284:1015)
  • May have connection to the epidermis with associated dermal plasma cell infiltrate (mimics syringocystadenoma papilliferum) (Am J Dermatopathol 2016;38:598)
  • Stromal changes: dense stromal plasma cell infiltrate, sclerosis, hyperplasia of myofibroblast-like cells, foamy macrophages
  • Metaplasia occasionally present
    • Most commonly oxyphilic (eosinophilic, granular cytoplasm, conspicuous nucleoli, occasional pleomorphism), oftentimes leading to misdiagnosis of malignancy (Am J Dermatopathol 2005;27:102)
    • Rarely squamous or mucinous metaplasia
  • Remnants of anogenital mammary-like glands (AGMLG) often present
  • Mitotic rate can be variable and sometimes high but high mitotic rate does not correlate with an aggressive outcome (Am J Dermatopathol 2006;28:322)
  • Rare case reports with sebaceous differentiation (in association with ectopic HP) and extramammary Paget disease (J Dermatol 2006;33:256, Am J Dermatopathol 2016;38:598)
Microscopic (histologic) images

Contributed by Bitania Wondimu, M.D.
Well circumscribed dermal lesion

Well circumscribed dermal lesion

Cystic lesion

Cystic lesion

Columnar and myoepithelial layers

Columnar and myoepithelial layers

Apocrine differentiation

Apocrine differentiation


Tubulopapillary architecture

Tubulopapillary architecture

CK7 highlights columnar cells

CK7 highlights columnar cells

SMA highlights myoepithelial layer

SMA highlights myoepithelial layer

p63 highlights myoepithelial layer

p63 highlights myoepithelial layer

Virtual slides

Images hosted on other servers:

Vulvar lesion

Vulvar biopsy

Negative stains
  • S100 (will highlight myoepithelial cells), HMWK
Molecular / cytogenetics description
  • Described to harbor mutations in genes involved in PI3K/ATK/MAPK signaling pathways (Pathology 2019;51:362)
  • Non-PI3K/AKT mutated cases display mutations in various other genes (PIK3CA PIK3R1, SYNE1, AR, IL6ST, PDGFRB, KMT2C, AR, BTK, DST, KAT6A, BRD3, RNF213, USP9X, ADGRB3, MAGI1, and IL7R) involved in PI3K/AKT signaling
  • Rare cases described mutations in BRAF, APC, ERBB4 (Genes Chromosomes Cancer 2016;55:113)
Videos

Diagnostic pearls of hidradenoma papilliferum

Sample pathology report
  • Skin, vulva, biopsy:
    • Diagnosis: hidradenoma papilliferum (see comment)
    • Comment: The biopsy contains a sharply circumscribed, maze-like glandular and papillary architecture without connection to overlying epithelium. The glands are lined by an inner layer of cuboidal cells and an outer layer of myoepithelial cells. Mitotic figures are rare. The morphologic features are most consistent with hidradenoma papilliferum.
Differential diagnosis
Board review style question #1

The lesion shown above is found in the vulva. What is the diagnosis?

  1. Hidradenocarcinoma
  2. Hidradenoma papilliferum
  3. Syringocystadenoma papilliferum
  4. Tubular apocrine adenoma
Board review style answer #1
B. Hidradenoma papilliferum

Comment Here

Reference: Hidradenoma papilliferum
Board review style question #2

A 57 year old woman develops a lesion on her vulva. Excision of the lesion shows a glandular and papillary proliferation (shown above). Which of the following is true concerning this lesion?

  1. Higher mitotic activity indicates worse prognosis
  2. It is often associated with a plasma cell infiltrate
  3. It is positive for EMA and GCDFP-15
  4. Malignant transformation occurs frequently
Board review style answer #2
C. It is positive for EMA and GCDFP-15

Comment Here

Reference: Hidradenoma papilliferum
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