Thread by @TristanRutland7, Vulva #Gynpath #dermpath #surgpath #pathtwitter Question: Based on the initial images, what is your [...]

Tristan D Rutland

TristanRutland7

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Vulva

#Gynpath #dermpath #surgpath #pathtwitter

Question:

Based on the initial images, what is your impression?

Immunohistochemistry:

Epithelium and stroma: ER /PR

Diagnosis:

Fibroadenoma

(Old case)

True or false?

These tumours are thought to arise from ectopic breast tissue.

1/ Original thought to arise from ectopic breast tissue, it is now thought they arise from anogenital mammary-like glands (which are normal structures).

2/ Other lesions such as hidradenoma papilliferum are thought to arise from these.
https://pubmed.ncbi.nlm.nih.gov/21169735/

Lesions of anogenital mammary-like glands: an update - PubMed

Long considered to be ectopic breast tissue representing the caudal remnants of the milk ridges, anogenital mammary-like glands are nowadays thought to represent a normal constituent of the anogeni...

https://pubmed.ncbi.nlm.nih.gov/21169735/

Question:

Which of the following are also thought to arise from anogenital mammary-like glands (AGMLG)?

3/ Some other tumours that are associated with these glands are phyllodes tumour, chondroid syringoma & adenocarcinoma of AGMLG.

4/ Interesting, I cannot find any info on PLAG1 rearrangements in chondroid syringoma/pleomorphic adenomas of the vulva.
https://pubmed.ncbi.nlm.nih.gov/22532914/

Pleomorphic adenoma of the vulva, clinical reminder of a rare occurrence - PubMed

Pleomorphic adenoma, also known as mixed tumor, is a benign tumor which typically presents as a painless and persistent mass. The majority of pleomorphic adenomas involve the salivary glands, most...

https://pubmed.ncbi.nlm.nih.gov/22532914/

True or false?

Lactational change can be seen in these tumours.

5/ Fibroadenomas have been described with numerous morphological changes such as:

”Pseudoangiomatous stromal hyperplasia”

Lactational change

Usual ductal hyperplasia

Multinucleate stromal giant cells
https://pubmed.ncbi.nlm.nih.gov/20035149/

Fibroadenoma and phyllodes tumors of anogenital mammary-like glands: a series of 13 neoplasms in 12...

The authors present a series of 13 fibroepithelial neoplasms involving anogenital mammary-like glands, all occurring in 12 female patients, whose age at diagnosis ranged from 30 to 51 years (mean, 38...

https://pubmed.ncbi.nlm.nih.gov/20035149/

Last question:

True or false?

Fibroadenomas of the vulva are benign.

6/ These tumours are very rare, usually occurring in women their reproductive years, but cases outside have been reported.

7/ They are generally considered benign, but rare cases of recurrent tumours have been documented. https://pubmed.ncbi.nlm.nih.gov/30732503/

Recurrent vulvar breast fibroadenoma: presentation of a rare clinical condition - PubMed

Vulvar fibroadenoma is an ectopic mammary-like fibroepithelial tumor of undetermined pathogenesis. Surgery is usually the primary option of treatment for this condition. Recurrent vulvar fibroadenoma...

https://pubmed.ncbi.nlm.nih.gov/30732503/

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