Solitary Fibrous Tumors of the Female Genital Tract: A Study of 27 Cases Emphasizing Nonvulvar Locations, Variant Histology, and Prognostic Factors.

Autor: Devins KM; Massachusetts General Hospital, Harvard Medical School, Boston, MA., Young RH; Massachusetts General Hospital, Harvard Medical School, Boston, MA., Croce S; Department of Pathology, Bergonié Institute, Bordeaux, France., Burandt E; Department of Pathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany., Bennett JA; Department of Pathology, University of Chicago, Chicago, IL., Pesci A; Department of Pathology, IRCCS Sacred Heart Hospital, Negrar-Verona., Zannoni GF; Department of Pathology, Catholic University of the Sacred Heart, Rome, Italy., Ip PPC; Department of Pathology, University of Hong Kong, Pokfulam, Hong Kong., Nielsen GP; Massachusetts General Hospital, Harvard Medical School, Boston, MA., Oliva E; Massachusetts General Hospital, Harvard Medical School, Boston, MA.
Jazyk: angličtina
Zdroj: The American journal of surgical pathology [Am J Surg Pathol] 2022 Mar 01; Vol. 46 (3), pp. 363-375.
DOI: 10.1097/PAS.0000000000001829
Abstrakt: 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.
Competing Interests: Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.
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Databáze: MEDLINE