Skin nonmelanocytic tumor

Adnexal tumors

Sweat gland derived (apocrine & eccrine glands)

Eccrine spiradenoma


Resident / Fellow Advisory Board: Farres Obeidin, M.D.
Tien-Anh Tran, M.D.
Na’im Fanaian, M.D.

Last author update: 1 November 2021
Last staff update: 24 June 2022 (update in progress)

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PubMed Search: Eccrine spiradenoma

Tien-Anh Tran, M.D.
Na’im Fanaian, M.D.
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Cite this page: Tran TA, Fanaian N. Eccrine spiradenoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticeccrinespiradenoma.html. Accessed December 7th, 2022.
Definition / general
  • Benign (multi) nodular solid tumor showing eccrine differentiation
Essential features
  • Benign neoplasm, excision is curative
  • Predilection for head and neck
  • Usually asymptomatic but can be painful, belonging to the painful skin tumors in the acronym BLEND TAN EGG (blue rubber bleb nevus, leiomyoma, eccrine spiradenoma, neuroma, dermatofibroma, tufted angioma, angiolipoma, neurilemmoma, endometrioma, granular cell tumor, glomus tumor) (Indian J Dermatol Venereol Leprol 2019;85:231)
  • Association with the Brooke-Spiegler syndrome
  • Multiple dark nests of cells confined to dermis (blue balls in the dermis) composed of 2 epithelial cell types: small dark peripheral cells and larger pale central cells, with intratumoral lymphocytes
Terminology
  • Spiradenoma
  • Eccrine spiradenoma
ICD coding
  • ICD-0: 8403/0 - eccrine spiradenoma
  • ICD-10: D23 - other benign neoplasms of skin
Epidemiology
Sites
  • Head / face and neck
  • Other sites (extremities and trunk) less common
Clinical features
  • Commonly presenting as solitary nodule
  • Rarely as multiple lesions in combination with other types of adnexal tumors as part of the Brooke-Spiegler syndrome
  • Predilection site: head and neck area, less commonly trunk and extremities
  • Usually asymptomatic, although often cited as a painful skin tumors in the acronym BLEND TAN EGG (blue rubber bleb nevus, leiomyoma, eccrine spiradenoma, neuroma, dermatofibroma, tufted angioma, angiolipoma, neurilemmoma [schwannoma], endometrioma, granular cell tumor, glomus tumor) (Indian J Dermatol Venereol Leprol 2019;85:231)
  • Firm, round and smooth surface nodule
  • Skin colored, gray-pink, reddish or blue
  • Small, usually 1 - 3 cm in size, may be larger, rare giant variants
Diagnosis
Prognostic factors
  • Benign neoplasm
  • Malignant transformation is a very rare phenomenon with the malignant component classified into basal cell adenocarcinoma-like, low grade pattern, basal cell adenocarcinoma-like, high grade pattern, invasive adenocarcinoma, NOS, sarcomatoid carcinoma / carcinosarcoma and malignant epithelial myoepithelial carcinoma (Am J Surg Pathol 2009;33:705, Int J Surg Pathol 2020;28:427)
Case reports
Treatment
  • Complete excision is curative
Clinical images

Contributed by Nicole Riddle, M.D. and Mark R. Wick, M.D.

Scalp

Missing Image

Breast skin



Images hosted on other servers:

Erythematous scalp nodule

Brooke-Spiegler
syndrome, multiple
face and
scalp nodules

Supraorbital spiradenoma with malignant transformation

Auricular nodule

Gross description
  • Usually less than 1 cm, rarely can be larger
  • Gray-pink or blue nodule
  • Either uninodular or multinodular tumor centered within the dermis
  • Extension into the subcutaneous tissue is common
  • Usually not connected to epidermis (Arch Craniofac Surg 2017;18:211)
Frozen section description
  • Not typically submitted for frozen section
  • Histologic features described below
  • Might be mistaken for basal cell carcinoma at the time of Mohs surgery (Wien Med Wochenschr 2018;168:218)
Microscopic (histologic) description
  • Circumscribed uninodular or multinodular basophilic tumor (see Figure 1)
    • Mostly centered in the dermis (blue balls)
    • Some tumors are encapsulated (see Figure 2)
  • Extension into the subcutis is common
  • 2 cell populations in the neoplastic nodules: centrally located large pale cells and small dark basaloid cells at the periphery (see Figure 3)
  • Neoplastic cells are arranged in trabecular, reticular or solid fashion
  • Intratumoral lymphocytes as an integral component of the tumor (see Figure 3)
  • Tubular / ductal structures (see Figure 4) and PAS positive basement membrane material (see Figure 5)
  • Stroma between the nests of epithelial cells is often edematous with prominent vascularity (see Figure 6); may be hyalinized (see Figure 7)
  • Vessels may be widely dilated (see Figure 7) / hemangiopericytoma-like
  • Many T lymphocytes and Langerhans cells often scattered throughout lobules
  • Mitoses extremely rare
  • Morphologic variations:
    • Architectural variations:
    • Cytologic variations:
      • Squamous metaplasia (see Figure 12)
      • Clear cell changes / metaplasia (see Figure 13)
      • Mucinous metaplasia and sebaceous differentiation
    • Stromal variation: edematous, myxoid, lipomatous metaplasia, osseous metaplasia, myoid metaplasia
Microscopic (histologic) images

Contributed by Tien-Anh Tran, M.D.

Multinodular

Encapsulated

2 cell populations and lymphocytes

Dilated ducts

Basement membrane


Fibrovascular stroma

Hyalizined stroma

Cystic change

Adenomatous changes

Adenomyoepithelioma


Cylindroma-like morphology

Squamous metaplasia

Clear cells

S100

SMA

Virtual slides

Images hosted on other servers:

Spiradenoma

Spiradenoma

Cytology description
  • Rare case reports describing the fine needle aspiration (FNA) cytologic findings of spiradenoma
  • Tight multilayered clusters of uniform benign cuboidal epithelial cells along with spindle shaped myoepithelial cells and occasional lymphocytes (J Lab Physicians 2014;6:130)
  • Prominent basement membrane deposition with an irregular tubular and nesting growth pattern and bland basaloid cells (Acta Cytol 1990;34:275)
Cytology images

Images hosted on other servers:

Cells with hyaline

Tight clusters of cells

Positive stains
Electron microscopy description
  • Lobules separated by amorphous / fibrillar material
  • Large pale cell has mitochondria, vesicles, glycogen in cytoplasm (J Invest Dermatol 1962;38:289)
Molecular / cytogenetics description
Videos

Spiradenoma: 5 minute pathology pearls

Spiradenoma and cylindroma: sweat gland tumor pathology

Spiradenoma for beginners

Sample pathology report
  • Skin, left cheek, excision:
    • Spiradenoma, completely excised with negative surgical margins
    • Negative for malignancy
Differential diagnosis
  • Cylindroma:
    • Closely related tumor with morphologic overlap but generally more abundant eosinophilic material around clusters of dark cells with jigsaw pattern and usually lack of lymphocytes
  • Poroma:
    • Only basaloid cells, absence of the second pale cell population and intratumoral lymphocytes
  • Spiradenocarcinoma / malignant spiradenoma / carcinoma ex spiradenoma:
    • Infiltrative growth and nuclear atypia, arising from spiradenoma (both components identified)
Board review style question #1

Which of the following is true about this cutaneous lesion?

  1. Mitoses are very rare
  2. Most commonly involves the extremities
  3. Typically presents as multiple nodules
  4. Usually not often painful
Board review style answer #1
A. Mitoses are very rare in spiradenoma

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Reference: Eccrine spiradenoma
Board review style question #2
Which of the following is true of spiradenoma?

  1. Composed of nests of a single cell type, forming dark nests in the dermis
  2. Even with complete excision, local recurrence is typical
  3. Intratumoral lymphocytes are a common component
  4. Usually centered in the epidermis
Board review style answer #2
C. Intratumoral lymphocytes are a common component

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Reference: Eccrine spiradenoma
Board review style question #3
Spiradenoma is an adnexal tumor which commonly occurs in what hereditary syndrome?

  1. Birt-Hogg-Dube syndrome
  2. Brooke-Spiegler syndrome
  3. Cowden syndrome
  4. Tuberous sclerosis
Board review style answer #3
B. Brooke-Spiegler syndrome

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Reference: Eccrine spiradenoma
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