Breast
Other nonneoplastic
Collagenous spherulosis


Minor changes: 17 November 2020

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PubMed Search: Collagenous spherulosis [title] breast

Iskender Sinan Genco, M.D.
Sabina Hajiyeva, M.D.
Page views in 2019: 5,198
Page views in 2020 to date: 5,796
Cite this page: Genco IS, Hajiyeva S. Collagenous spherulosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/breastcollageneousspherulosis.html. Accessed November 25th, 2020.
Definition / general
Essential features
  • Benign proliferation with intraluminal pink basement membrane material (spherule) surrounded by myoepithelial cells
Terminology
  • Collagenous spherulosis
  • Simple spherulosis
  • Mucinous spherulosis
  • Adenoid cystic hyperplasia (not recommended due to confusion with adenoid cystic carcinoma)
ICD coding
    ICD-10:
  • N60.8 - other benign mammary dysplasias
  • N60.9 - unspecified benign mammary dysplasia

    ICD11:
  • GB20.Y - other specified benign breast disease
  • GB20.Z - benign breast disease, unspecified
Epidemiology
Sites
  • No specific site in the breast
Pathophysiology
  • Unclear
  • Accumulation of basement membrane material in spaces
Etiology
  • Unknown
Clinical features
Diagnosis
  • Based on histologic examination of removed tissue
Radiology description
  • May present as radiologic or rarely as architectural distortion, density or mass (Pathology 2017;49:181)
Prognostic factors
Case reports
Treatment
  • No treatment required
Gross description
  • No specific gross features (usually not discernable from background breast tissue)
Microscopic (histologic) description
  • Round spaces filled with acellular pink basement membrane material (spherules) (Pathology 2017;49:181)
  • Spherules measure 20 - 100 μm in diameter (Hoda: Rosen's Diagnosis of Breast Pathology by Needle Core Biopsy, 4th Edition, 2017)
  • Center of the spherules may be transparent but thin rim of basement membrane material always present
  • Radiating stellate fibrils or microcalcifications may be seen in the spherules
  • Spherules surrounded by small oval / spindle myoepithelial cells with interspersed cuboidal luminal cells
  • Myoepithelial cells are uniformly present at the periphery of involved glands
  • Majority associated with benign proliferative lesions, mostly intraductal papilloma
Microscopic (histologic) images

Contributed by Iskender Sinan Genco, M.D. and Sabina Hajiyeva, M.D.
Cribriform architecture

Cribriform architecture

Multiple ducts

Multiple ducts

2 different sized ducts

2 different sized ducts

Cribriform architecture

Cribriform architecture

Partial involvement Partial involvement

Partial involvement


Adjacent apocrine metaplasia

Adjacent apocrine metaplasia

Columnar cell changes

Columnar cell changes

Smooth muscle myosin heavy chain

Smooth muscle myosin heavy chain

CD10

CD10

KIT / CD117

KIT / CD117

In situ carcinoma

In situ carcinoma


ER

ER

CK5/6

CK5/6

Adjacent invasive carcinoma

Adjacent invasive carcinoma

Smooth muscle actin (SMA)

Smooth muscle actin

Smooth muscle myosin heavy chain (SMMHC)

Smooth muscle myosin heavy chain

Calponin

Calponin


p63

p63

PAS PAS

PAS

Cytology description
Cytology images

Contributed by Mark R. Wick, M.D.
FNA

FNA

Negative stains
Electron microscopy description
Videos

Breast calcifications

Sample pathology report
  • Breast, left, 3 o'clock, stereotactic core biopsy:
    • Lobular carcinoma in situ (LCIS) involving collagenous spherulosis, with microcalcifications (see comment)
    • Comment: Immunohistochemical stain for E-cadherin is negative in LCIS, supporting the diagnosis. Controls are appropriate.
Differential diagnosis
  • Atypical ductal hyperplasia:
    • Some, but not all, features of low grade ductal carcinoma in situ (see above)
    • Negative for CK5/6
  • Board review style question #1

    H&E


    A premenopausal woman had a stereotactic breast core biopsy for calcifications. Microscopic examination revealed fibrocystic changes with microcalcifications as well as the microscopic finding shown above (H&E). Regarding this entity, which of the following statements is true?

    1. Comprised of a clonal (neoplastic) proliferation
    2. Epithelial cells interspersed between spaces are negative for ER
    3. Flattened (myoepithelial) cells around spaces are positive for CD10
    4. Luminal epithelial cells are positive for KIT / CD117
    5. Surgical excision should be performed
    Board review answer #1
    C. The flattened (myoepithelial) cells around spaces are positive for CD10. The picture shows slightly expanded ducts with spaces filled with eosinophilic material. The spaces are surrounded by small oval / spindle myoepithelial cells with interspersed cuboidal luminal cells, features consistent with collagenous spherulosis. Collagenous spherulosis is a benign finding and does not require treatment. Adenoid cystic carcinoma is the main differential diagnosis which shows MYB-NFIB fusion gene, is negative for ER and PR, positive for KIT / CD117 and should be surgically excised.

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    Reference: Collagenous spherulosis
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