Vaginal Solitary Fibrous Tumor

Iyengar, Pratibha; Ismiil, Nadia; Gerber, David; Khalifa, Mahmoud A.

doi:10.1097/01.lgt.0000230205.73365.5e

Cited by 13 publications

(10 citation statements)

References 13 publications

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“…In our series, patients presented over a wide age range, averaging 55 years, similar to previous reports 2,8–23,34–53. Clinical presentation varied by site.…”

Section: Discussionsupporting

confidence: 87%

“…The classic histologic features of SFT include a so-called “patternless” arrangement of ovoid to spindled cells in a variably collagenous stroma, scattered dense collagen bundles, and thin-walled branching vessels 1,4,6,7,23. These features were seen at least focally in all our tumors and in previously reported FSFTs 9–22,34–52. Most neoplasms demonstrate alternating cellular and hypocellular areas, although uniformly cellular tumors are not uncommon,4,6 48% in our series.…”

Section: Discussionsupporting

confidence: 55%

“…FSFTs have been reported most commonly in the vulva, followed by the uterine corpus, and infrequently at other locations 9–22,34–52. In our series, they were seen throughout the gynecologic tract with greater representation in the upper tract than previously reported, particularly within the ovary and fallopian tube/paratubal soft tissue 22,34,42,44,49,51.…”

Section: Discussionsupporting

confidence: 50%

“…One patient with an ovarian tumor had a mild elevation in serum CA-125, causing concern for an epithelial neoplasm. Previously reported FSFTs had similar presentations,9–22,34–52 with a few exceptions. Tumors of the uterine corpus and cervix occasionally presented with abnormal bleeding 38,50.…”

Section: Discussionmentioning

confidence: 69%

“…The discovery of a recurrent NAB2 - STAT6 gene fusion and STAT6 immunohistochemistry further aided diagnosis of this specific entity 31–33. Despite increased awareness and recognition of SFTs, studies in the female genital tract (FSFTs) are limited to one series of 25 tumors and sporadic case reports, most describing vulvar tumors 9–22,34–53. Our study is the first with a preponderance of nonvulvar tumors.…”

Section: Discussionmentioning

confidence: 93%

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Solitary Fibrous Tumors of the Female Genital Tract

Devins

Young

Croce

et al. 2021

American Journal of Surgical Pathology

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We report 27 solitary fibrous tumors of the female genital tract emphasizing nonvulvar locations, variant histology, and prognostic factors. The patients ranged from 25 to 78 years (most were over 40), and tumors occurred in the vulva (7), vagina (2), cervix (2), corpus (6), fallopian tube/paratubal soft tissue (5), and ovary (5). They ranged from 1.5 to 39 (mean = 10.5) cm and were typically solid, but 4 were predominantly cystic. All had a haphazard arrangement of spindled to ovoid cells, with most demonstrating alternating cellular and hypocellular areas and prominent vessels, but 13 lacked hypocellular areas, and 7 had focal diffuse growth with inconspicuous vasculature. Other patterns included corded (8), fascicular (5), trabecular (1), and nested (1). Microcysts (6), myxoid background (8), hyalinization (8), lipomatous differentiation (2), and multinucleated cells (6) were also present, and 10 tumors had necrosis. Vasculature included thin-walled branching "staghorn" (27), thick-walled (7), and hyalinized vessels (5) or dilated anastomosing vascular channels (3). Nuclear atypia ranged from mild (19), moderate (7), to severe (1), and mitoses from 0 to 24/10 HPF (mean = 4). STAT6 was positive in all 25 tumors tested. One tumor showed dedifferentiation; the remainder were classified as benign (19) or malignant (7) based on mitotic rate (univariate stratification model) and as low risk ( 14), intermediate risk (8), or high risk (4) based on the Demicco multivariate risk stratification score. Follow-up (median = 23 mo) was available for 16 patients. Six tumors recurred (2 intermediate risk, 3 high risk, and the dedifferentiated tumor), 5 in the abdomen; the dedifferentiated tumor metastasized to the lung. Multivariate risk stratification was superior to univariate classification, as 5 "benign" tumors were reclassified as intermediate risk using the multivariate model; of these, 2 recurred, and 1 patient died of disease. Upper female genital tract tumors occurred in older patients, were larger, and more frequently classified as high risk compared with those of the lower tract. A trend toward increased cellularity was also seen in the upper tract tumors. Only size (P = 0.04), necrosis (P = 0.04), and Demicco score (P = 0.01) independently correlated with recurrence. Female genital tract solitary fibrous tumors demonstrate a wide range of variant morphologies and occur in diverse sites in addition to the vulva. Tumors were often misdiagnosed as other neoplasms; thus, awareness of solitary fibrous tumors occurring at these sites is crucial in prompting staining for STAT6 to establish this diagnosis. The Demicco risk stratification system effectively predicts behavior.

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“…In our series, patients presented over a wide age range, averaging 55 years, similar to previous reports 2,8–23,34–53. Clinical presentation varied by site.…”

Section: Discussionsupporting

confidence: 87%

Section: Discussionsupporting

confidence: 55%

Section: Discussionsupporting

confidence: 50%

Section: Discussionmentioning

confidence: 69%

Section: Discussionmentioning

confidence: 93%

See 3 more Smart Citations

Solitary Fibrous Tumors of the Female Genital Tract

Devins

Young

Croce

et al. 2021

American Journal of Surgical Pathology

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Cytomorphologic features of malignant solitary fibrous tumor with mediastinal involvement sampled by endoscopic and endobronchial ultrasound‐guided fine‐needle aspiration: A comparison of two cases

Hupp

Najmuddin

Dinçer

et al. 2019

Diagnostic Cytopathology

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Solitary fibrous tumor (SFT) is an uncommon fibroblastic neoplasm with considerable risk of local recurrence. SFT is histologically characterized by bland spindled-to-epithelioid cells in alternating hyper-and hypocellular zones, a "patternless pattern," ectatic "staghorn" vessels with variable edematous perivascular stroma, and thick ropey collagen.Cytologically, smears are variably cellular with spindled-to-epithelioid cells with oval nuclei, wispy cytoplasm, multiple inconspicuous nucleoli, and occasional nuclear pseudoinclusions. Small vessels and bare/stripped nuclei are generally present while mild atypia is not uncommon. STAT6 nuclear expression is the most useful immunohistochemical stain and is the product of a NAB2-STAT6 gene fusion. SFTs with mediastinal involvement may be diagnostically challenging due to proximity to vital structures and anticipated patient risks. Endobronchial and endoscopic ultrasound-guided fineneedle aspiration (EBUS/EUS-FNA) are minimally-invasive tissue sampling methods that provide diagnostic material while minimizing patient risk, and the mediastinum is accessible by both procedures. Small aspirate samples and SFT nonspecific features can compound the diagnostic difficulty, although familiarity with the cytologic, morphologic, immunophenotypic, and genetic features of SFTs assist the pathologist in confirming the diagnosis. Pathologists must also be aware of high-risk SFT features to ensure appropriate therapy and management. Case #1 describes a recurrent mediastinal SFT with high-risk features sampled by EUS-FNA. Case #2 describes a primary diagnosis of mediastinal SFT with malignant behavior made on an EBUS-FNA specimen. K E Y W O R D S endoscopic ultrasound, fine-needle aspiration, mediastinum, solitary fibrous tumor, STAT6

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