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CASE REPORT
Year : 2020  |  Volume : 11  |  Issue : 1  |  Page : 37-39

Vulvovaginal fibroepithelial stromal polyp with myxoid stroma: A diagnostic dilemma on histology – Report of two cases


Department of Pathology, Institute of Medical Sciences and SUM Hospital, Bhubaneshwar, Odisha, India

Date of Submission18-Jul-2020
Date of Acceptance14-Oct-2020
Date of Web Publication23-Dec-2020

Correspondence Address:
Dr. Nibedita Sahoo
Department of Pathology, Institute of Medical Sciences and SUM Hospital, Bhubaneshwar, Odisha
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mjmsr.mjmsr_27_20

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  Abstract 


Genital stromal tumors are a rare and unique subset of soft-tissue tumors encountered in the vulvovaginal and inguinoscrotal region and this group includes fibroepithelial stromal polyp, superficial (cervicovaginal) myofibroblastoma, cellular angiofibroma, mammary-type myofibroblastoma, angiomyofibroblastoma, superficial angiomyxoma, and deep/aggressive angiomyxoma. The relative rarity and overlapping histomorphological and immunohistochemical features result in a diagnostic difficulty for the pathologists. Herein, we describe two cases of soft-tissue tumors in the vulvovaginal region presenting as polypoidal mass with detailed histopathology and immunohistochemical workup.

Keywords: Aggressive angiomyxoma, fibroepithelial polyp, genital stromal tumor, immunohistochemistry


How to cite this article:
Ghosh U, Mohapatra D, Sahoo N. Vulvovaginal fibroepithelial stromal polyp with myxoid stroma: A diagnostic dilemma on histology – Report of two cases. Muller J Med Sci Res 2020;11:37-9

How to cite this URL:
Ghosh U, Mohapatra D, Sahoo N. Vulvovaginal fibroepithelial stromal polyp with myxoid stroma: A diagnostic dilemma on histology – Report of two cases. Muller J Med Sci Res [serial online] 2020 [cited 2021 Jan 18];11:37-9. Available from: https://www.mjmsr.net/text.asp?2020/11/1/37/304591




  Introduction Top


Genital stromal tumors are a rare and unique subset of soft-tissue tumors encountered in the vulvovaginal and inguinoscrotal region. This group includes fibroepithelial stromal polyp (FESP), superficial (cervicovaginal) myofibroblastoma, cellular angiofibroma, mammary-type myofibroblastoma, angiomyofibroblastoma, superficial angiomyxoma, and aggressive angiomyxoma (AA). The relative rarity, overlapping histomorphological and immunohistochemical features result in a diagnostic difficulty for the pathologists.[1],[2] Some specific tumors have predilection to these sites, while other tumors can occur at any site. With this background, herein, we describe two cases of soft-tissue tumors in the vulvovaginal region presenting as polypoidal mass with detailed histopathology and immunohistochemical workup.


  Case Reports Top


Case 1

A 21-year-old female presented to the gynecological outpatient department (OPD) with a painful vulval swelling which had been present and gradually increasing in size for the past 10 months and had also been causing difficulty in micturition. There was no history of any discharge, bleeding, or sexual difficulty. Menstrual cycle was also normal. Local examination revealed a well-circumscribed pedunculated fleshy polypoidal mass, measuring 5 cm × 4 cm, which was soft in consistency. Her blood reports were completely normal. No radiological investigation was done.

With a clinical diagnosis of fibroepithelial polyp, surgical excision of the mass was done and sample was sent for histopathological analysis.

The gross specimen was a skin-covered polypoidal structure, measuring 2.7 cm × 1.8 cm × 0.5 cm, with a stalk measuring 0.8 cm in length. Cut surface was soft, homogeneous with glistening myxoid areas [Figure 1]a.
Figure 1: (a and b) Gross pictures of case 1 and case 2

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Microsections revealed a polypoidal tumor covered with thinned-out epidermis and focal acanthosis. The subepidermal stroma was myxoid and embedded within the stroma were haphazardly arranged spindle to stellate cells having ill defined cytoplasm [Figure 2]. Admixed few bi- and multinucleated giant cells having wreath-like nuclei were also noted. Other notable features included the presence of many thin- and thick-walled blood vessels with medial hypertrophy in some. There is focal increased cellularity around the thick-walled vessels, along with extravasation of red blood cells. No increased mitosis or necrosis was noted in the sections studied.
Figure 2: Low magnification depicting thick- and thin-walled vassels and myxoid stroma (a), higher magnification showing extravassated red blood cells (b), bland spindle and stellate cells over myxoid stroma (c), masson trichome stain (d) highlighting the fine collagen fibrils (blue in color), immunopositivity for estrogen receptor (e), desmin (f)

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Case 2

A 25-year-old female presented to the gynecology OPD complaining of something hanging from her vagina. On examination, there was a polypoidal mass on her labia majora measuring 4 cm × 4 cm, which was completely painless and present for 4 months. With a clinical diagnosis of lipoma, excision of the mass was done.

The gross received was a polypoidal structure with the largest dimension of 4 cm and attached stalk measuring 0.6 cm in length. Cut surface was similar to the previous case, i.e., soft, myxoid, and glistening [Figure 1]b.

Histopathology revealed ulcerated epidermis covered with granulation tissue and adjoining epithelium displayed irregular acanthosis, hyperkeratosis, parakeratosis, and focal papillomatosis [Figure 2]b. Underlying stroma was myxoid, admixed with thick- and thin-walled vessels. Other histomorphological features like the previous case were identified, but multimucleated giant cells were noted.

On immunohistochemistry (IHC), both the cases revealed focal positivity for estrogen receptor (ER), progesterone receptor (PR), and desmin for the spindle cells [Figure 2]e and [Figure 2]f.


  Discussion Top


The vulvovaginal mesenchymal lesions are thought to arise from the specialized subpeithelial stroma of the lower female genital tract extending from the cervix to the vulva, which is highly vascular, variably myxoid, and contains atypical multinucleate fibroblast-like cells. Due to common histogenesis, these lesions show overlapping histomorphological features, but vigilant observation and some immunohistochemical stains help in the correct diagnosis. The entities included in these lesions are FESP, superficial angiomyxoma, deep/AA, angiomyofibroblastoma, and cellular angiofibroma.

Angiomyxomas are classified either as superficial/cutaneous myxoma or as deep/aggressive. Superficial angiomyxoma though has a predilection for the trunk, lower extremities, and head-and-neck region, it can arise in the genital region and grow as a polypoidal or papulonodular lesion. However, they show uniform hypocellularity and delicate thin-walled capillaries, lacking the thick-walled blood vessels which were present in our case.

AA is a deep-seated infiltrative tumor which may blend imperceptibly with the surrounding soft tissue and has a tendency to locally recur if excised incompletely.[3],[4] Due to the presence of vessels of varying caliber, admixed uniformly over an extensive hypocellular myxoid stroma, our case mimicked with AA. These tumors can be polypoidal, occasional case reports also describe it as pedunculated polyp.[5] However, typical morphology of AA is uniform bland spindle cells in a hypocellular myxoid stroma and radiating myoid fibers from the thick-walled vessels, which were absent in our cases.

Angiomyofibroblastoma and cellular angiofibroma are usually subcutaneous circumscribed nodules and angiomyofibroblastoma shows the perivascular arrangement of epithelioid cells around thin-walled blood vessels.[1],[2]

FESPs are polypoidal lesions of the vulvovaginal region in the reproductive age group, which can be solitary and rarely multiple.[1] These lesions are usually small with large thick-walled centrally located vessels. However, large polyps can have an edematous, myxoid stroma with spindle-to-stellate-shaped cells and sometimes, multinucleated cells with wreath-like nuclei are seen. Unlike most of the other vulvovaginal lesions, FESP lacks uninvolved grenz zone. Absence of the grenz zone, presence of spindle-to-stellate-shaped cells, and multinucleated cells in one of our case concluded the final diagnosis of FESP.

FESP, otherwise known as cellular pseudosarcomatous FESP, pseudosarcoma botryoides, or mesodermal stromal polyp, is a benign polypoidal growth occurs generally in young-age women to middle-age women in their reproductive years; however, they can present at any age.[1],[6],[7] They occur most commonly in the vagina, but they can also occur in the vulva and less commonly, the cervix. It can exhibit worrisome morphological features, especially in pregnancy like hypercellularity and nuclear atypia, including atypical mitosis of stromal cells and can mimic malignancy.

The specialized subepithelial mesenchymal cells of lower female genital tract are hormone responsive and exhibit positive immunohistochemical stain for ER and PR.[1],[2] This zone is also positive for desmin, CD34, and CD99. For this to distinguish between various mesenchymal lesions, IHC plays a minor role. Our cases also displayed varying expression of ER, PR, and desmin. The edematous stroma of FESP bringing a differential of AA can be ruled out by IHC marker HMGA2 which is expressed in AA.[2]

The diagnostic difficulty faced in our case was the relatively larger size, myxoid stroma, and uniform distribution of the vasculature, mimicking as AA. As the vulvovaginal mesenchymal lesions share a common age group of presentation, overlapping morphology and immunophenotype, extensive grossing accompanied by proper histomorphological evaluation is needed for a definite diagnosis which will predict the clinical course and proper management of the patients.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Nucci MR, Fletcher CD. Vulvovaginal soft tissue tumours: Update and review. Histopathology 2000;36:97-108.  Back to cited text no. 1
    
2.
McCluggage WG. A review and update of morphologically bland vulvovaginal mesenchymal lesions. Int J Gynecol Pathol 2005;24:26-38.  Back to cited text no. 2
    
3.
Steeper TA, Rosai J. Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm. Am J Surg Pathol 1983;7:463-75.  Back to cited text no. 3
    
4.
Ribaldone R, Piantanida P, Surico D, Boldorini R, Colombo N, Surico N. Aggressive angiomyxoma of the vulva. Gynecol Oncol 2004;95:724-8.  Back to cited text no. 4
    
5.
Deshmukh BD, Kulkarni MP, Momin YA, Sulhyan KR. Aggressive angiomyxoma of vulva: A case report and review of literature. Arch Med Health Sci 2015;3:88-90.  Back to cited text no. 5
  [Full text]  
6.
Nucci MR, Young RH, Fletcher CD. Cellular pseudosarcomatous fibroepithelial stromal polyps of the lower female genital tract: An underrecognized lesion often misdiagnosed as sarcoma. Am J Surg Pathol 2000;24:231-40.  Back to cited text no. 6
    
7.
Ostör AG, Fortune DW, Riley CB. Fibroepithelial polyps with atypical stromal cells (pseudosarcoma botryoides) of vulva and vagina. A report of 13 cases. Int J Gynecol Pathol 1988;7:351-60.  Back to cited text no. 7
    


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  [Figure 1], [Figure 2]



 

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