Vulva, vagina & female urethra

Other tumors

Mixed tumor of vagina


Editorial Board Member: C. Blake Gilks, M.D.
Deputy Editor-in-Chief: Jennifer A. Bennett, M.D.
Adam Lechner, B.M.
Carlos Parra-Herran, M.D.

Last author update: 17 August 2022
Last staff update: 17 August 2022

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PubMed Search: Mixed tumor vagina

Adam Lechner, B.M.
Carlos Parra-Herran, M.D.
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Cite this page: Lechner A, Parra-Herran C. Mixed tumor of vagina. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/vaginaspindlecellep.html. Accessed December 9th, 2022.
Definition / general
Essential features
  • Rare, benign tumor commonly of the lower posterior vaginal wall (near hymenal ring)
  • Well circumscribed, unencapsulated, detached from overlying epithelium
  • Predominantly bland spindle cells (cytokeratin+, SMA+, S100-) with sparsely admixed glands / squamous islands
  • Recurrence following complete excision is rare
Terminology
  • Mixed tumor of vagina is the term currently endorsed by the World Health Organization classification of female genital tract tumors
  • The term spindle cell epithelioma (SCE) is not recommended
ICD coding
  • ICD-O: 8011/0 - epithelioma, benign
  • ICD-10: D28.1 - benign neoplasm of vagina
Epidemiology
Sites
Pathophysiology
Clinical features
Diagnosis
  • Diagnosis is made by histopathologic examination, either via biopsy or excision of the lesion
Radiology description
  • Ovoid, well circumscribed
  • T1 isointense, T2 hyperintense relative to adjacent vaginal wall; homogenous enhancement and restricted diffusion (Clin Imaging 2018;50:181)
Prognostic factors
Case reports
Treatment
  • Local excision
Clinical images

Images hosted on other servers:

Posterior vaginal wall tumor

Gross description
Microscopic (histologic) description
  • Well circumscribed but unencapsulated with a pushing border
  • Located just deep (< 3 mm) to the vaginal mucosa but without connection to overlying epithelium (seen as a Grenz-like zone of loose connective tissue in between)
  • Biphasic population of predominantly spindle cells with a minor squamous or glandular component and numerous small vessels (Am J Surg Pathol 1993;17:509)
  • Spindle cell (major) component:
    • Variably cellular spindle cell component
    • Hypocellular areas of fibroblastic type cells
    • Bland cytology, minimal mitotic activity
    • Cytoplasm is usually indistinct but can appear eosinophilic
    • Condensed extracellular matrix forms eosinophilic hyaline globules
  • Glandular / squamoid (minor) component:
    • More often seen at the periphery of the lesion
    • Simple glands lined by cuboidal to columnar epithelium
    • Glandular epithelium may resemble endocervical epithelium with intracytoplasmic mucin
    • Squamous metaplasia is common, resulting in nests / morules and cords of plump squamous cells with glycogenated cytoplasm (Am J Surg Pathol 1993;17:509, Pathol Res Pract 2012;208:424)
Microscopic (histologic) images

Contributed by Brooke Howitt, M.D.
Separation from squamous epithelium

Separation from squamous epithelium

Cytologically bland spindle cells

Cytologically bland spindle cells

Discrete nests

Discrete nests

Pancytokeratin

Pancytokeratin

Positive stains
Negative stains
Electron microscopy description
  • Spindle cells show prominent tonofilaments with associated desmosomes (Am J Surg Pathol 1993;17:509)
  • Myoepithelial features (dense myofibrils, pinocytotic vesicles, basal laminar investitures) are not seen
Sample pathology report
  • Vagina, local excision:
    • Benign mixed tumor of vagina, 1.5 cm, margins are uninvolved by tumor (see comment)
    • Comment: Immunostaining shows AE1 / AE3, CD10, SMA positivity in lesional cells. S100 and SOX10 are negative. The cytomorphology and immunohistochemical profile are consistent with the above diagnosis.
Differential diagnosis
  • Carcinosarcoma of vagina:
    • Infiltrative border
    • Overtly sarcomatous mesenchymal component
    • Malignant glandular component (usually high grade morphologically)
  • Müllerian adenosarcoma:
    • Cambium layer
    • Mitotic activity (≥ 2 per 10 high power fields)
    • Leaf-like (phyllodes-like) architecture
    • Periglandular stromal condensation
    • Atypical / frankly malignant spindle cell component
  • Tubulosquamous polyp:
    • Anatomically more superior
    • Thought to arise from mesonephric rests
    • Epithelial elements with squamoid and glandular appearance are discrete but are not associated with a surrounding spindle cell population
    • GATA3+ and NKX3.1+ epithelium, CD10- and cytokeratin- stroma (Am J Surg Pathol 2007;31:1013)
  • Metastatic endometrial stromal sarcoma:
    • Infiltrative border
    • Usually epithelial elements are absent
    • Previous history of the disease
  • Metastatic / primary spindle cell carcinoma:
    • Infiltrative border and marked atypia
    • Overlying mucosal dysplasia or better differentiated squamous cell carcinoma components (if primary)
  • Synovial sarcoma:
Board review style question #1

A 31 year old woman presents with a 2 cm solid mass protruding from the vagina. A local resection is performed and shows a submucosal, well circumscribed but unencapsulated lesion composed of predominantly bland spindle cells with sparse glands and squamous metaplasia (see image shown above). Electron microscopy of the spindle cells shows abundant tonofilaments and desmosomes but no pinocytotic vesicles. Which of the following is true regarding these spindle cells?

  1. They are derived from mesonephric rests
  2. They are derived from normal myoepithelial cells found in the vagina
  3. They are likely keratin+, CD10+ and SMA+
  4. They are the malignant component of this biphasic tumor
Board review style answer #1
C. They are likely keratin+, CD10+ and SMA+. This lesion represents a benign spindle cell epithelioma (mixed tumor of vagina). Electron microscopy of the spindle cells of this lesion shows epithelial cell features and a lack of contractile elements. A differential diagnosis of this lesion is a tubulosquamous polyp, which is thought to be derived from mesonephric rests. Myoepithelium does not normally occur in the vagina; a multipotent progenitor cell is thought to give rise to this lesion.

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Reference: Mixed tumor of vagina
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