Anus & perianal area
Premalignant
Buschke-Löwenstein tumor


Minor changes: 5 January 2021

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PubMed Search: Buschke-Löwenstein tumor anus

Lewis A. Hassell, M.D.
Page views in 2020: 602
Page views in 2021 to date: 85
Cite this page: Nguyen PN, Hassell LA. Buschke-Löwenstein tumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/anusbuschkelowenstein.html. Accessed January 23rd, 2021.
Definition / general
Essential features
  • Condylomatous squamous lesion without high grade dysplasia, with only low risk HPV types present, forming an exophytic mass, usually greater than 3 cm
Terminology
ICD coding
  • ICD-O: None
  • ICD-11: 1A95.0 - Anal warts
Epidemiology
Sites
  • Anogenital region (involves both inside and outside of anal canal)
  • Urinary bladder, genitalia, oropharynx
Pathophysiology
  • Similar to the process in other skin and mucosal HPV induced tumors, viral DNA is incorporated into cellular DNA following exposure, redirecting cellular processes into dysregulated cellular proliferation (IARC Monogr Eval Carcinog Risks Hum 2007;90:1)
Etiology
Clinical features
Diagnosis
  • Diagnosis is often based on clinical course, radiology and histopathology
Radiology description
  • Preoperative CT / MRI is often required to evaluate local extension
Radiology images

Images hosted on other servers:

Tumor invading the rectum

Extensive pelvic infiltration of tumor

Pelvic structures infiltrated by tumor

Tumor expands along the endopelvis

Tumor involves the inguinal area

Prognostic factors
Case reports
Treatment
  • Preferred initial therapy: complete excision with wide margins
  • Other treatment strategies: podofilox cream, topical 5-fluorouracil, regional radiation, CO2 laser or a combined chemoradiation regimen (J Obstet Gynaecol 2020;40:582)
Clinical images

Contributed by Philippe Simon, M.D. and Andrew L. Atkinson, M.D.
Bulky tumor of vulva and anus

Bulky tumor of vulva and anus

Large exophytic tumor of vulva and perineum

Large exophytic tumor of vulva and perineum

Gross description
  • Size: up to 13 cm (Histopathology 2017;70:938)
  • Large, exophytic, cauliflower-like mass
  • May show fistula, bleeding, ulceration, superficial infection
Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Lewis A. Hassell, M.D. (source: Juan Rosai Slide Collection)
Well formed papillae with fibrovascular cores

Well formed papillae with fibrovascular cores

Hyperkeratosis with parakeratosis

Hyperkeratosis with parakeratosis

Koilocytes and fungal elements

Koilocytes and fungal elements

Koilocytes with perinuclear clearing

Koilocytes with perinuclear clearing

Chronic inflammation and vascular changes in stroma

Chronic
inflammation and
vascular changes
in stroma

Virtual slides

Images hosted on other servers:

Anal giant condyloma

Buschke-Löwenstein tumor

Positive stains
Negative stains
  • p16 (focal / patchy or negative)
  • p53 (wild type pattern)
  • Ki67 (confined to basal zone)
Videos

Giant condyloma

Sample pathology report
  • Anal canal and perianal skin, complete excision:
    • Buschke-Löwenstein tumor / giant condyloma acuminatum (see synoptic report)

Synoptic report
Tumor summary Anus
Procedure Complete excision
Tumor site Perianal skin
Tumor size 5 cm
Histologic type Giant condyloma acuminatum
Margins Free, 0.5 cm to radial
Extent Anal canal, below the pectinate line
Tumor invasion Not identified
Examined sections 05 blocks
Lymph nodes, # sampled 10
Lymph nodes, # involved 0
Ancillary study result
  • p16
  • Ki67

Patchy, 5% of cells
Restricted to parabasal layers
Reference: College of American Pathologists: Definition of Synoptic Reporting [Accessed 18 November 2020]
Differential diagnosis
  • Condyloma acuminatum:
    • Histologically, Buschke-Löwenstein tumor resembles condyloma acuminatum
    • Clinical findings help differentiate condyloma from Buschke-Löwenstein tumor (large size - usually > 3 cm, locally expansive and displacement of surrounding tissues)
  • Verrucous carcinoma:
  • Squamous cell carcinoma:
    • Marked cytologic atypia with stromal invasion
    • p16: diffusely positive
  • Squamous papilloma of anus:
Board review style question #1
Which of the following features best distinguishes Buschke-Löwenstein tumor from verrucous squamous carcinoma?

  1. HPV ISH positivity
  2. p53 immunostatus
  3. Patient age less than 50 years
  4. Pushing invasion
  5. Size greater than 5 cm
Board review style answer #1
A. HPV ISH positivity. Buschke-Löwenstein tumor is an HPV related neoplasm whereas verrucous carcinoma is not, so HPV ISH staining (positive) would be expected with Buschke-Löwenstein tumor but not with verrucous carcinoma. Buschke-Löwenstein tumor is most often due to a low risk HPV type, such as HPV 6 or 11. Invasive or displacing type growth can be seen in either lesion.

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Reference: Buschke-Löwenstein tumor
Board review style question #2

Lymphadenopathy seen in conjunction with a bulky anovaginal tumor characterized by parakeratosis, superficial koilocytosis, HPV ISH positivity and minimal squamous atypia as seen in this image is most likely due to

  1. Associated lymphoma or other immune deficiency inducing state
  2. Fatty infiltration of the lymph nodes
  3. Metastasis from a well differentiated invasive component of the tumor
  4. Netherton syndrome
  5. Reactive lymphadenopathy related to tumor associated infection
Board review style answer #2
E. Reactive lymphadenopathy related to tumor associated infection. Buschke-Löwenstein tumor is frequently associated with fistulae and perirectal or peritumoral abscess formation, which can lead to regional lymphadenopathy. Metastasis is very uncommon. While immunocompromise may predispose to development of Buschke-Löwenstein tumor, the cause is infrequently due to a lymphoproliferative disorder that would enlarge the lymph nodes. Netherton syndrome is not primarily associated with adenopathy.

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Reference: Buschke-Löwenstein tumor
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