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  Table of Contents    
LETTER TO EDITOR  
Year : 2021  |  Volume : 64  |  Issue : 4  |  Page : 863-865
Benign phyllodes tumor of the vulva


1 Department of Pathology, Kempegowda Institute of Medical Sciences, Bangalore, Karnataka, India
2 Department of Obstetrics and Gynaecology, Kempegowda Institute of Medical Sciences, Bangalore, Karnataka, India
3 Department of Pathology, Kempegowda Institute of Medical Sciences; Division of Molecular Medicine at St John's Research Institute, Bangalore, Karnataka, India

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Date of Submission01-Apr-2020
Date of Decision19-May-2020
Date of Acceptance23-Jun-2020
Date of Web Publication20-Oct-2021
 

How to cite this article:
Venkatesh K, Jayanthy T, Patil S. Benign phyllodes tumor of the vulva. Indian J Pathol Microbiol 2021;64:863-5

How to cite this URL:
Venkatesh K, Jayanthy T, Patil S. Benign phyllodes tumor of the vulva. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 Nov 28];64:863-5. Available from: https://www.ijpmonline.org/text.asp?2021/64/4/863/328551




Dear Editor,

Phyllodes tumor is a well illustrated neoplasm of the breast having characteristic histomorphological features with leaf like protrusions and slit like ducts. Proliferative lesions occurring in the breast such as fibrocystic disease, fibroadenoma, lactating adenoma, phyllodes tumor and adenocarcinoma have been found in extra mammary sites as the axilla, anal region and vulva. Origin of the mammary tissue in these locations is elusive with one theory stating that they are rudiments of embryonic milk lines or mammary ridges which can give rise to tumors. The second theory put forth by Van Der Putte states that lesions resembling breast tumors develop from mammary-like glands (MLG) in the vulva, as mammary ridges in human embryos do not extend up to anogenital region.[1] There are only 18 cases of phyllodes tumor of the vulva reported in literature and most of them were benign along with a few borderline and malignant tumors.[2]

A 45-year-old female presented with a mass lesion in the left labium majus. It was gradually increasing in size since one year and painful since 15 days. Patient was para six with 5 living children and had one abortion. All were normal vaginal deliveries. The last child birth was 16 years back and then she was tubectomised. She had regular menstrual cycles and was on treatment for hypertension since one year. There was no history of breast lump and hormonal therapy. Physical examination showed tubectomy scar and both breasts did not have any lump. Vulva showed a pedunculated mass arising from left side labium majus [Figure 1]a. The mass was soft to firm, freely mobile and non tender with focal ulceration of the overlying skin covered with minimal slough. Per speculum examination showed mild cystocele and second degree uterine descent.

Vulval mass was excised and sent for histopathological examination with differential diagnoses of fibrolipoma and Hamartoma. The mass received in the lab was soft to firm, nodular, measured 7.0 × 4.5 × 3.0 cm covered with skin having an ulcer measuring 1.0 cm. The cut surface was grey white with slit like spaces, tiny cysts, mucoid and gelatinous areas. A smaller bit labeled as the tissue from the stalk of the main lesion measured 4.0 × 2.0 × 1.5 cm, was covered with hairy skin [Figure 1]b and [Figure 1]c. Multiple bits were processed and stained with Haematoxylin and Eosin (H&E). Tissue sections from the peduncle showed normal skin with adnexal structures. Sections from the main mass showed a neoplasm with biphasic morphology having intracanalicular pattern of slit like ducts with “leaf like architecture” and spindle celled stroma. Ducts were lined by bilayered epithelium with inner cuboidal to columnar cells and outer basal cells [Figure 1]d. The stroma was variably cellular composed of spindle cells with tapered nuclei in a collagenized background. There was no cellular atypia and no mitoses seen in the stromal cells [Figure 1]e, and [Figure 2]a and [Figure 2]b. The overlying skin showed an ulcer covered with inflammatory exudate. Immunohistochemistry (IHC) of the tumor tissue showed strong positivity for Estrogen Receptor (ER), Progesterone Receptor (PR) and Gross Cystic Disease Fluid Protein-15 (GCDFP -15) [Figure 2]c, [Figure 2]d, [Figure 2]e, [Figure 2]f. A diagnosis of benign phyllodes tumor of the vulva was made by routine histopathology which was substantiated by IHC. Patient has no recurrence of the tumor on follow up for four years.
Figure 1: (a) Pedunculated mass in the left labium majus; (b) Gross specimen with skin covered nodular mass and separated peduncle; (c) Grey white cut surface with slit like spaces; (d) Microphotograph of the tumor showing typical “leaf-like” architecture (H and E, × 100) ; (e) Spindle celled stroma with collagen bands (H and E, × 100)

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Figure 2: (a) Microphotograph of the tumor with ducts lined by bilayered epithelium (H and E, × 100); (b) Spindle celled stroma with no atypia and mitosis (H&E stain, × 400); (c) Ductal cells showing ER positivity with IHC (× 400); (d) Ductal epithelial cells positive for PR (× 100); (e) Ductal epithelium showing GCDFP-15 Positivity (× 400); (f) Apical snouts of ductal cells are positive for GCDFP-15 immunostaining (× 400)

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   Discussion Top


Proliferative lesions occurring in the breast have been found in extra mammary sites such as the axilla, anus, prostate, seminal vesicle, and vulva. Phyllodes tumor of breast was first described by Johannes Muller in 1838, it comprises less than 1% of primary breast tumors and diagnosed by typical leaf like architecture with biphasic pattern.[3] The two components include the ducts lined by bilayered epithelium and the spindle celled stroma. The ducts may have cystic dilatations, epithelium exhibiting pseudo stratification and papillary proliferations. The stromal cells have elongated tapering nuclei in a collegenized background.[4]

Lee S and Nodit L reviewed 13 masses diagnosed as phyllodes tumor of the vulva reported by 10 authors from 1993 to 2013, and found that the patient's age ranged from 17 to 69 years. Majority of these tumors were located in the labium majus and other locations included interlabial sulcus, mons pubis, and periclitoral sites. Tumor size ranged between 0.7 to 6.6 cm, majority were well circumscribed, freely mobile and non tender. Grossly they showed grey white to tan cut surface with cleft like spaces and small cysts having papillary structures. Ulceration of the overlying skin was rare.[5] In the case reported here, the tumor was larger in size and had ulceration of the covering skin.

The differential diagnosis of phyllodes tumor of the vulva includes other biphasic tumors such as fibroadenoma, papillary hidradenoma and chondroid syringoma.[6] Phyllodes tumors of the vulva are classified as benign, borderline, or malignant, based on a semi-quantitative assessment of cellularity, pleomorphism and mitotic activity of the stromal cells similar to the counterpart in breast.

Interestingly, immunohistochemical markers specific for breast tumors are positive in phyllodes tumor of the vulva; the epithelial cells show nuclear positivity for ER and PR. The marker which is always positive in ducts with apocrine change is GCDFP-15. The other IHC markers include SMA and S100 for myoepithelial cells, the stroma shows positivity for CD34, Vimentin, SMA and up to 15% are positive for Ki67.

Local recurrence is known with phyllodes tumor as even a benign tumor can recur if not excised completely. Hence an accurate diagnosis of phyllodes tumor of the vulva is essential warranting a strict clinical follow up of the patient.

In conclusion, phyllodes tumor of the vulva is a rare tumor with controversial histogenesis and presents with characteristic histomorphology. As the site of occurrence is unusual, positivity for IHC markers specific for mammary tissue contributes for confirmation of the diagnosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgements

The authors acknowledge the technical staff of the lab and OT for their support.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
van der Putte SC. Mammary-like glands of the vulva and their disorders. Int J Gynecol Pathol 1994;13:150-60.  Back to cited text no. 1
    
2.
Kilitci A, Arıoz O. Primary benign phyllodes tumor of the vulva: case report and review of literature. Eur J Breast Health 2019;15:196-9.  Back to cited text no. 2
    
3.
Moulla A, Hunt L, Shaikh H, Datta S. Phyllodes tumor in the vulva. Breast J 2017;23:476-8.  Back to cited text no. 3
    
4.
Heffernan TP, Sarode VR, Hoffman B, Lea J. Recurrent phyllodes tumor of the vulva: A case report with review of diagnostic criteria and differential diagnosis. Int J Gynecol Pathol 2010;29:294-7.  Back to cited text no. 4
    
5.
Lee S, Nodit L. Phyllodes tumor of vulva: a brief diagnostic review. Arch Pathol Lab Med 2014;138:1546-50.  Back to cited text no. 5
    
6.
Kazakov DV, Spagnolo DV, Stewart CJ, Thompson J, Agaimy A, Magro G, et al. Fibroadenoma and phyllodes tumors of anogenital mammary-like glands: a series of 13 neoplasms in 12 cases, including mammary-type juvenile fibroadenoma, fibroadenoma with lactation changes, and neurofibromatosis-associated pseudoangiomatous stromal hyperplasia with multinucleated giant cells. Am J Surg Pathol 2010;34:95-103.  Back to cited text no. 6
    

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Correspondence Address:
Kusuma Venkatesh
#28, 2nd Cross, Maruthi Seva Nagar, Banasawadi Moad, Bangalore - 560 033, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_292_20

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