Cellular pseudosarcomatous fibroepithelial stromal polyp of the endocervix

Cellular pseudosarcomatous fibroepithelial stromal polyp of the endocervix

Pathology ISSN: 0031-3025 (Print) 1465-3931 (Online) Journal homepage: https://www.tandfonline.com/loi/ipat20 Cellular pseudosarcomatous fibroepithe...

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Pathology

ISSN: 0031-3025 (Print) 1465-3931 (Online) Journal homepage: https://www.tandfonline.com/loi/ipat20

Cellular pseudosarcomatous fibroepithelial stromal polyp of the endocervix Leonardo D. Santos, Alfred Ng & Yu‐Min Tan To cite this article: Leonardo D. Santos, Alfred Ng & Yu‐Min Tan (2004) Cellular pseudosarcomatous fibroepithelial stromal polyp of the endocervix, Pathology, 36:4, 376-378 To link to this article: https://doi.org/10.1080/00313020410001721609

Published online: 06 Jul 2009.

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Pathology (2004), 36(4), August

tract11 and genitourinary tract.12 FSPs are easily recognised as benign when they are hypocellular and the lesional cells are bland.9 A recently described subset of FSP designated as cellular pseudosarcomatous fibroepithelial stromal polyp (CPFSP) exhibits bizarre, worrisome cytomorphological features that are different from the usual FSPs.9 We report a case of endocervical CPFSP that mimics sarcoma because of the presence of cellular pleomorphism, hypercellularity and mitoses, including abnormal forms. A 15-year-old girl consulted her physician when she discovered a painless lump protruding from her vagina for more than a month. At times, the mass totally moved inside the vagina and she could not palpate it. There was no associated bleeding or discharge. She had never been sexually active. There was no history of instrumentation, vaginal trauma or surgery. The clinical impression was a vaginal polyp. On examination under anaesthesia, the lump was attached to the endocervix. The hymen was unremarkable. A twist-avulsion procedure was performed. Clinical follow-up revealed there was no tumour recurrence or malignant transformation. Grossly, the tan soft polyp measured 1961369 mm. On sectioning, it showed a few cystic spaces measuring up to 4 mm, which contained mucus material. There was no evidence of necrosis or haemorrhage. Histologically, the cervical polyp, which was focally ulcerated, was lined by an endocervical epithelium with areas of squamous metaplasia and short papillary-like projections (Fig. 1). The oedematous to focally haemorrhagic stroma showed variable cellularity. Poorly defined, patchy hypercellular areas limited beneath the mucosa were present and contained loose aggregates of hyperchromatic, pleomorphic and mitotically active large cells. There were five mitoses per 10 HPFs, including tri- and multipolar abnormal mitotic forms. The lesional cells varied from polygonal to multinucleated irregular cells with inconspicuous to abundant pale cytoplasm. There were also scattered stellate, bizarre giant and spindle cells. Anisonucleosis and nuclear membrane irregularity were prominent. A few cells showed coarse chromatin. Nucleoli were small or indistinct. Many degenerate cells exhibited smudged chromatin and indistinct cell borders (Fig. 2A,B). The stromal collagen was loose and wispy. Occasional

Cellular pseudosarcomatous fibroepithelial stromal polyp of the endocervix Sir, Fibroepithelial stromal polyps (FSPs) are unusual benign lesions of the vagina,1–4 and rarely are found in the vulva,5 endometrium, cervix,6–10 nose, oral cavity, gastrointestinal

Fig. 1 The surface of the endocervical cellular pseudosarcomatous fibroepithelial stromal polyp is covered focally by papillary-like processes. An area of superficial ulceration is present (H&E, original magnification, 620).

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B

Fig. 2 Within the patchy hypercellular areas (A) abnormal mitotic figures (arrows) and (B) pleomorphic, degenerate, hyperchromatic stromal cells are prominent features (H&E, original magnification, 6400).

dilated endocervical glands and inflammatory cells, composed of lymphocytes, neutrophils, plasma cells and mast cells were present, as well as dilated, thin-walled vessels and a few bundles of smooth muscle. The lesional cells were strongly positive for vimentin (clone V9, 1:150 dilution; Dako, Denmark), oestrogen receptor (ER; clone 6F11, 1:100 dilution; Novocastra, UK) and progesterone receptor (PR; clone PgR 636, 1:500 dilution; Dako). A few cells were positive for CD34 (clone QBend 10, 1:50 dilution; Dako), smooth muscle actin (SMA; clone 1A4, 1:75 dilution; Dako) and desmin (clone D33, 1:100 dilution; Dako). There was negative staining for low molecular weight cytokeratin (clone CAM5.2, 1:3 dilution; Becton-Dickinson, USA), pankeratin (clone AE1/ AE3, 1:100 dilution; Dako), epithelial membrane antigen (EMA; clone E29, 1:150 dilution; Dako), S100 protein (polyclonal, 1:500 dilution; Dako), myogenin (clone F5D, 1:50 dilution; Dako), myoglobin (polyclonal, 1:3 dilution; Dako), muscle-specific actin (clone HHF-35, 1:40 dilution; Dako), and macrophage (CD68, clone PG-M1, 1:200 dilution; Dako) and histiocyte/myeloid markers (clone MAC 387, 1:75 dilution; Dako). The proliferation index (clone Ki67, 1:100 dilution; Dako) was approximately 30% (Fig. 3). FSPs, also known as mesodermal stromal polyps,8 are benign lesions3,4,8,9 that can occur at any age and in a wide variety of sites including the breast, and gastrointestinal,11 genitourinary12 and genital tracts.1–10 CPFSPs, a recently

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described subset of FSPs, have been reported involving the cervix in only seven cases; most, if not all, originated from the ectocervix.9 CPFSPs are characterised by at least three of the four cytomorphological features: marked hypercellularity, marked cytological pleomorphism, mitotic counts of more than five mitoses per 10 HPFs, and the presence of atypical mitoses.9 The presence of these striking histological features can lead to a misdiagnosis of sarcoma.9 In comparison, FSPs are easily recognised as benign when the abnormal stromal cells are few and bland.9 Many FSPs contained some bizarre stromal cells, but only occasional cases exhibited focal hypercellularity and scattered normal or abnormal mitoses. Because of the atypical stromal cells, they were called pseudosarcomatous botryoides.3,4,6,9 The aetiopathogenesis of CPFSPs is not well understood. They are believed to be not a true neoplasm but rather a reactive hyperplastic process involving the distinctive subepithelial myxoid stroma of the cervix.9 Reactive process is favoured because the collection of abnormal stromal cells of CPFSPs lacks an identifiable lesional margin. The stellate, polygonal and multinucleated stromal cells of CPFSPs are also seen in the subepithelial stroma of normal cervix, vagina, vulva and breast, suggesting that these polyps may represent a proliferation of stromal cells normally found at these organs.9 Immunohistochemically and ultrastructurally the stromal cells show fibroblastic and myofibroblastic differentiation.4 The positive reaction for ER and PR supports the concept that these lesions are hormonally responsive.9 Differential diagnoses include the conventional endocervical polyps and sarcoma. The typical endocervical polyps, which are the most common new growths of the uterine cervix, are not true neoplasms; rather, they are focal, hyperplastic processes containing bland glandular epithelial and stromal cells. Botryoid embryonal rhabdomyosarcoma (BER) and endometrial stromal sarcoma (ESS) may appear as endocervical polypoid lesions. BER occurs in a younger age group, with a history of rapid growth. The tumour shows greater mitotic activity, a cambium layer, and the cells show skeletal muscle differentiation histologically, immunohistochemically and ultrastructurally.4 Low-grade ESS is characteristically very vascular and the vessels typically resemble spiral arterioles. In addition, thick bands of hyalinised collagen in a starburst pattern and the presence of dot-like perinuclear staining pattern with desmin and/or keratin are characteristics of low-grade ESS.9 Awareness of the bizarre cytomorphological features exhibited by CPFSPs is crucial in their accurate recognition as a benign lesion, thus avoiding misdiagnosis of sarcoma and unnecessary surgical overtreatment. The treatment of choice is local excision.4 The previously reported seven cases of CPFSPs were all alive and well. On follow-up 12 months after treatment, our patient showed no evidence of recurrence or malignant transformation. Leonardo D. Santos* Alfred Ng{ Yu-Min Tan*

Fig. 3 The proliferation index of the pseudosarcomatous area is high (ABC method, Ki67, original magnification, 6400).

*Department of Anatomical Pathology, South Western Area Pathology Service, Liverpool Hospital, Liverpool, NSW and

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{Department of Obstetrics and Gynaecology, BankstownLidcombe Hospital, NSW, Australia Contact Dr L. D. Santos. E-mail: leonardo.santos@ swsahs.nsw.gov.au 1. Miettinen M, Wahlstrom T, Vesterinen E, Saksela E. Vaginal polyps with pseudosarcomatous features. A clinicopathologic study of seven cases. Cancer 1983; 51: 1148–51. 2. Hartmann C-A, Sperling M, Stein H. So-called fibroepithelial polyps of the vagina exhibiting an unusual but uniform profile characterized by expression of desmin and steroid hormone receptors but no muscle-specific actin or macrophage markers. Am J Clin Pathol 1990; 93: 604–8. 3. Nucci MR, Fletcher CDM. Fibroepithelial stromal polyps of vulvovaginal tissue. From the banal to the bizarre. Pathol Case Rev 1998; 3: 151–7. 4. Nielsen GP, Young RH. Mesenchymal tumors and tumor-like lesions of the female genital tract: a selective review with emphasis on recently described entities. Int J Gynecol Pathol 2001; 20: 105–27.

Pathology (2004), 36(4), August 5. Aranha J, Bartolo E, Dessai S, Goncalves JCA. Fibroepithelial polyp of the vulva. Am J Dermatopathol 1998; 20: 610. 6. Elliott GB, Reynolds HA, Fidler HK. Pseudo-sarcoma botryoides of cervix and vagina in pregnancy. J Obstet Gynaecol Br Commonw 1967; 74: 728–33. 7. Cachaza JA, Caballero JJL, Fernandez JA, Salido E. Endocervical polyp with pseudosarcomatous pattern and cytoplasmic inclusions: an electron microscopic study. Am J Clin Pathol 1986; 85: 633–5. 8. Varga Z, Caduff R. Fibroepithelial polyp of the uterine cervix. Histopathology 1999; 34: 375–6. 9. Nucci MR, Young RH, Fletcher CDM. 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. 10. Kommoss F. Miscellaneous mesenchymal tumours and tumour-like lesions of the uterus. Histopathology 2002; 41 (S2): 27–31. 11. Shekitka KM, Helwig EB. Deceptive bizarre stromal cells in polyps and ulcers of the gastrointestinal tract. Cancer 1991; 67: 2111–7. 12. Blank C, Lissmer J, Kaneti. Fibroepithelial polyp of the renal pelvis. J Urol 1987; 137: 962–3.

DOI: 10.1080/00313020410001721609