Solitary Fibrous Tumors of the Female Genital Tract: A Study of 27 Cases Emphasizing Nonvulvar Locations, Variant Histology, and Prognostic Factors.
Journal
The American journal of surgical pathology
ISSN: 1532-0979
Titre abrégé: Am J Surg Pathol
Pays: United States
ID NLM: 7707904
Informations de publication
Date de publication:
01 03 2022
01 03 2022
Historique:
pubmed:
6
11
2021
medline:
8
3
2022
entrez:
5
11
2021
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 34739418
doi: 10.1097/PAS.0000000000001829
pii: 00000478-202203000-00008
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
363-375Informations de copyright
Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.
Déclaration de conflit d'intérêts
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.
Références
Klemperer P, Rabin C. Primary neoplasm of the pleura: a report of five cases. Arch Pathol. 1931;11:385–412.
Demicco EG, Wagner MJ, Maki RG, et al. Risk assessment in solitary fibrous tumors: validation and refinement of a risk stratification model. Mod Pathol. 2017;30:1433–1442.
Salas S, Resseguier N, Blay JY, et al. Prediction of local and metastatic recurrence in solitary fibrous tumor: construction of a risk calculator in a multicenter cohort from the French Sarcoma Group (FSG) database. Ann Oncol. 2017;28:1979–1987.
Demicco EG, Park MS, Araujo DM, et al. Solitary fibrous tumor: a clinicopathological study of 110 cases and proposed risk assessment model. Mod Pathol. 2012;25:1298–1306.
Goldman SM, Davidson AJ, Neal J. Retroperitoneal and pelvic hemangiopericytomas: clinical, radiologic, and pathologic correlation. Radiology. 1988;168:13–17.
Pasquali S, Gronchi A, Strauss D, et al. Resectable extra-pleural and extra-meningeal solitary fibrous tumours: a multi-centre prognostic study. Eur J Surg Oncol. 2016;42:1064–1070.
Smith SC, Gooding WE, Elkins M, et al. Solitary fibrous tumors of the head and neck: a multi-institutional clinicopathologic study. Am J Surg Pathol. 2017;41:1642–1656.
Hasegawa T, Matsuno Y, Shimoda T, et al. Extrathoracic solitary fibrous tumors: their histological variability and potentially aggressive behavior. Hum Pathol. 1999;30:1464–1473.
Biedrzycki OJ, Singh N, Habeeb H, et al. Solitary fibrous tumor of the female genital tract a case report and review of the literature. Int J Gynecol Pathol. 2007;26:259–264.
Burnett LA, Christopherson WA, Olawaiye AB. Vulvar hemangiopericytoma: a case report and literature review. Gynecol Oncol Case Rep. 2012;2:146–149.
Fukunaga M. Atypical solitary fibrous tumor of the vulva. Int J Gynecol Pathol. 2000;19:164–168.
He Y, Yang KX, Yang F, et al. Primary solitary fibrous tumor of the vulva: a case report. J Reprod Med. 2010;55:452–456.
Lee DH. Solitary fibrous tumor arising in the mons pubis: a case report. Eur J Gynaecol Oncol. 2016;37:423–425.
Nag G, Rao SR. A rare mesenchymal neoplasm at unusual location: solitary fibrous tumor of vulva. Gynecol Oncol Rep. 2015;12:52–54.
Nielsen GP, O’Connell JX, Dickersin GR, et al. Solitary fibrous tumor of soft tissue: a report of 15 cases, including 5 malignant examples with light microscopic, immunohistochemical, and ultrastructural data. Mod Pathol. 1997;10:1028–1037.
Pearre DC, Federspiel JJ, Grumbine FC. Solitary fibrous tumor of the vulva resulting in spinal metastasis: a case report. Gynecol Oncol Rep. 2017;22:97–99.
Rekhi B, Bapat P, Jindal A. A rare case of a solitary fibrous tumor of vulva, displaying NAB2ex6-STAT6ex17 fusion. Indian J Pathol Microbiol. 2021;64:177–179.
Taki M, Baba T, Mandai M, et al. Solitary fibrous tumor arising slowly in the vulva over 10 years: case report and review. J Obstet Gynaecol Res. 2012;38:884–888.
Tandon N. Solitary fibrous tumor of the vulva: case report of a rare entity and review of literature. Int J Gynecol Pathol. 2021;40:234–239.
Tardio JC, Machado I, Alemany I, et al. Solitary fibrous tumor of the vulva: report of 2 cases, including a de novo dedifferentiated solitary fibrous tumor diagnosed after molecular demonstration of NAB2-STAT6 gene fusion. Int J Gynecol Pathol. 2018;37:547–553.
Vaysse C, Escourrou G, Motton S, et al. Solitary fibrous tumor of the vulva: a case report. Gynecol Obstet Fertil. 2011;39:e49–e51.
Yang EJ, Howitt BE, Fletcher CDM, et al. Solitary fibrous tumour of the female genital tract: a clinicopathological analysis of 25 cases. Histopathology. 2018;72:749–759.
England DM, Hochholzer L, McCarthy MJ. Localized benign and malignant fibrous tumors of the pleura: a clinicopathologic review of 223 cases. Am J Surg Pathol. 1989;13:640–658.
Mosquera JM, Fletcher CD. Expanding the spectrum of malignant progression in solitary fibrous tumors: a study of 8 cases with a discrete anaplastic component—is this dedifferentiated SFT? Am J Surg Pathol. 2009;33:1314–1321.
Stout AP, Murray MR. Localized pleural mesothelioma: investigation of its characteristics and histogenesis by the method of tissue culture. Arch Pathol. 1942;34:951–964.
Stout AP, Murray MR. Hemangiopericytoma: a vascular tumor featuring Zimmermann’s pericytes. Ann Surg. 1942;116:26–33.
Gengler C, Guillou L. Solitary fibrous tumour and haemangiopericytoma: evolution of a concept. Histopathology. 2006;48:63–74.
Bhargava R, Shia J, Hummer AJ, et al. Distinction of endometrial stromal sarcomas from ‘hemangiopericytomatous’ tumors using a panel of immunohistochemical stains. Mod Pathol. 2005;18:40–47.
Vallat-Decouvelaere AV, Dry SM, Fletcher CD. Atypical and malignant solitary fibrous tumors in extrathoracic locations: evidence of their comparability to intra-thoracic tumors. Am J Surg Pathol. 1998;22:1501–1511.
van de Rijn M, Lombard CM, Rouse RV. Expression of CD34 by solitary fibrous tumors of the pleura, mediastinum, and lung. Am J Surg Pathol. 1994;18:814–820.
Chmielecki J, Crago AM, Rosenberg M, et al. Whole-exome sequencing identifies a recurrent NAB2-STAT6 fusion in solitary fibrous tumors. Nat Genet. 2013;45:131–132.
Koelsche C, Schweizer L, Renner M, et al. Nuclear relocation of STAT6 reliably predicts NAB2-STAT6 fusion for the diagnosis of solitary fibrous tumour. Histopathology. 2014;65:613–622.
Robinson DR, Wu YM, Kalyana-Sundaram S, et al. Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing. Nat Genet. 2013;45:180–185.
Zubor P, Kajo K, Szunyogh N, et al. A solitary fibrous tumor in the broad ligament of the uterus. Pathol Res Pract. 2007;203:555–560.
Wakami K, Tateyama H, Kawashima H, et al. Solitary fibrous tumor of the uterus producing high-molecular-weight insulin-like growth factor II and associated with hypoglycemia. Int J Gynecol Pathol. 2005;24:79–84.
Vadmal MS, Pellegrini AE. Solitary fibrous tumor of the vagina. Am J Dermatopathol. 2000;22:83–86.
Strickland KC, Nucci MR, Esselen KM, et al. Solitary fibrous tumor of the uterus presenting with lung metastases: a case report. Int J Gynecol Pathol. 2016;35:25–29.
Sidebotham EL, DeLair D, Comerci JT, et al. Pediatric radical abdominal trachelectomy for solitary fibrous tumor of the uterine cervix. Gynecol Oncol. 2009;115:302–305.
Reyhan A. Solitary fibrous tumor of the vagina with potentially malignant features: a case report and review of the literature. Turk Patoloji Derg. 2018;34:186–189.
Rekhi B, Bapat P, Shetty O. A rare case of a vaginal solitary fibrous tumor, presenting as a cystic mass, showing NAB2ex4-STAT6ex2 fusion and STAT6 immunostaining. Int J Gynecol Pathol. 2019;38:21–26.
Rahimi K, Shaw PA, Chetty R. Solitary fibrous tumor of the uterine cervix. Int J Gynecol Pathol. 2010;29:189–192.
Porter E, McGregor A, Brown L, et al. Solitary fibrous tumour of round ligament mimicking a leiomyoma. J Obstet Gynaecol. 2008;28:463–464.
Nowakowski A, Kozlowski W, Wlodarczyk D, et al. A case of a large solitary fibrous tumour of the uterine cervix. BMC Womens Health. 2014;14:3.
Meena M, Jindal T, Duggal R, et al. Solitary fibrous tumor of the ovary. J Obstet Gynaecol Res. 2018;44:1177–1180.
Iyengar P, Ismiil ND, Gerber D, et al. Vaginal solitary fibrous tumor: a case report with recurrence after incomplete excision. J Low Genit Tract Dis. 2007;11:50–54.
Hasegawa T, Matsuno Y, Shimoda T, et al. Frequent expression of bcl-2 protein in solitary fibrous tumors. Jpn J Clin Oncol. 1998;28:86–91.
Dvorak O, Dvorakova E, Laco J, et al. Solitary fibrous tumor of endometrium—a case report. Ceska Gynekol. 2013;78:302–305.
Chu PW, Liu JY, Peng YJ, et al. Solitary fibrous tumor of the uterus. Taiwan J Obstet Gynecol. 2006;45:350–352.
Chen S, Zheng Y, Chen L, et al. A broad ligament solitary fibrous tumor with Doege-Potter syndrome. Medicine. 2018;97:e12564.
Casanova J, Vizcaino JR, Pinto F, et al. Abdominal mass mimicking a leiomyoma: malignant uterine solitary fibrous tumor. Gynecol Oncol Case Rep. 2012;2:143–145.
Berzal-Cantalejo F, Montesinos-Carbonell M, Montesinos-Carbonell ML, et al. Solitary fibrous tumor arising in the fallopian tube. Gynecol Oncol. 2005;96:880–882.
Akiyama Y, Nabeshima K, Koita H, et al. Solitary fibrous tumor of the vagina. Pathol Int. 2000;50:327–331.
Zou Z, Xiao S, Xue M. Giant solitary fibrous tumor of vagina: a case report and literature review. Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2018;43:816–820.
Balduyck B, Lauwers P, Govaert K, et al. Solitary fibrous tumor of the pleura with associated hypoglycemia: Doege-Potter syndrome: a case report. J Thorac Oncol. 2006;1:588–590.
Doege KW. Fibro-sarcoma of the mediastinum. Ann Surg. 1930;92:955–960.
Feasel P, Al-Ibraheemi A, Fritchie K, et al. Superficial solitary fibrous tumor: a series of 26 cases. Am J Surg Pathol. 2018;42:778–785.
Guillou L, Gebhard S, Coindre JM. Orbital and extraorbital giant cell angiofibroma: a giant cell-rich variant of solitary fibrous tumor? Clinicopathologic and immunohistochemical analysis of a series in favor of a unifying concept. Am J Surg Pathol. 2000;24:971–979.
Demicco EG, Harms PW, Patel RM, et al. Extensive survey of STAT6 expression in a large series of mesenchymal tumors. Am J Clin Pathol. 2015;143:672–682.
Machado I, Morales GN, Cruz J, et al. Solitary fibrous tumor: a case series identifying pathological adverse factors-implications for risk stratification and classification. Virchows Arch. 2020;476:597–607.
Bongiovanni M, Viberti L, Pecchioni C, et al. Steroid hormone receptor in pleural solitary fibrous tumours and CD34+ progenitor stromal cells. J Pathol. 2002;198:252–257.
Carretta A, Bandiera A, Melloni G, et al. Solitary fibrous tumors of the pleura: Immunohistochemical analysis and evaluation of prognostic factors after surgical treatment. J Surg Oncol. 2006;94:40–44.
Piperi E, Rohrer MD, Pambuccian SE, et al. Vascular solitary fibrous tumor with “floret” cells or giant cell angiofibroma? A lingual example highlighting the overlapping characteristics of these entities and positive immunoreaction for estrogen and progesterone receptors. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009;107:685–690.
Bell SW, Kempson RL, Hendrickson MR. Problematic uterine smooth muscle neoplasms. A clinicopathologic study of 213 cases. Am J Surg Pathol. 1994;18:535–558.
Oliva E, Young RH, Clement PB, et al. Cellular benign mesenchymal tumors of the uterus. A comparative morphologic and immunohistochemical analysis of 33 highly cellular leiomyomas and six endometrial stromal nodules, two frequently confused tumors. Am J Surg Pathol. 1995;19:757–768.
Rizeq MN, van de Rijn M, Hendrickson MR, et al. A comparative immunohistochemical study of uterine smooth muscle neoplasms with emphasis on the epithelioid variant. Hum Pathol. 1994;25:671–677.
Reyes C, Karamurzin Y, Frizzell N, et al. Uterine smooth muscle tumors with features suggesting fumarate hydratase aberration: detailed morphologic analysis and correlation with S-(2-succino)-cysteine immunohistochemistry. Mod Pathol. 2013;27:1020–1027.
Bennett JA, Weigelt B, Chiang S, et al. Leiomyoma with bizarre nuclei: a morphological, immunohistochemical and molecular analysis of 31 cases. Mod Pathol. 2017;30:1476–1488.
Oliva E, Clement PB, Young RH. Endometrial stromal tumors: an update on a group of tumors with a protean phenotype. Adv Anat Pathol. 2000;7:257–281.
Lewis N, Soslow RA, Delair DF, et al. ZC3H7B-BCOR high-grade endometrial stromal sarcomas: a report of 17 cases of a newly defined entity. Mod Pathol. 2018;31:674–684.
McCluggage WG, Sumathi VP, Maxwell P. CD10 is a sensitive and diagnostically useful immunohistochemical marker of normal endometrial stroma and of endometrial stromal neoplasms. Histopathology. 2001;39:273–278.
Chu P, Arber DA. Paraffin-section detection of CD10 in 505 nonhematopoietic neoplasms. Frequent expression in renal cell carcinoma and endometrial stromal sarcoma. Am J Clin Pathol. 2000;113:374–382.
Chu PG, Arber DA, Weiss LM, et al. Utility of CD10 in distinguishing between endometrial stromal sarcoma and uterine smooth muscle tumors: an immunohistochemical comparison of 34 cases. Mod Pathol. 2001;14:465–471.
Chiang S, Lee CH, Stewart CJR, et al. BCOR is a robust diagnostic immunohistochemical marker of genetically diverse high-grade endometrial stromal sarcoma, including tumors exhibiting variant morphology. Mod Pathol. 2017;30:1251–1261.
Argani P, Kao YC, Zhang L, et al. BCOR overexpression in renal malignant solitary fibrous tumors: a close mimic of clear cell sarcoma of kidney. Am J Surg Pathol. 2019;43:773–782.
Dockerty MB, Masson JC. Ovarian fibromas: a clinical and pathologic study of 283 cases. Am J Obstet Gynecol. 1944;47:741–752.
Irving JA, Alkushi A, Young RH, et al. Cellular fibromas of the ovary: a study of 75 cases including 40 mitotically active tumors emphasizing their distinction from fibrosarcoma. Am J Surg Pathol. 2006;30:929–938.
He H, Luthringer DJ, Hui P, et al. Expression of CD56 and WT1 in ovarian stroma and ovarian stromal tumors. Am J Surg Pathol. 2008;32:884–890.
McCluggage WG, Singh N, Kommoss S, et al. Ovarian cellular fibromas lack FOXL2 mutations: a useful diagnostic adjunct in the distinction from diffuse adult granulosa cell tumor. Am J Surg Pathol. 2013;37:1450–1455.
Zhao C, Vinh TN, McManus K, et al. Identification of the most sensitive and robust immunohistochemical markers in different categories of ovarian sex cord-stromal tumors. Am J Surg Pathol. 2009;33:354–366.
Devins KM, Young RH, Watkins JC. Sclerosing stromal tumor: a clinicopathologic study of 100 cases of a distinctive benign ovarian stromal tumor typically occurring in the young. Histopathology. 2021. In press.
Nucci MR, Granter SR, Fletcher CD. Cellular angiofibroma: a benign neoplasm distinct from angiomyofibroblastoma and spindle cell lipoma. Am J Surg Pathol. 1997;21:636–644.
McCluggage WG, Ganesan R, Hirschowitz L, et al. Cellular angiofibroma and related fibromatous lesions of the vulva: report of a series of cases with a morphological spectrum wider than previously described. Histopathology. 2004;45:360–368.
Flucke U, van Krieken JH, Mentzel T. Cellular angiofibroma: analysis of 25 cases emphasizing its relationship to spindle cell lipoma and mammary-type myofibroblastoma. Mod Pathol. 2011;24:82–89.
McMenamin ME, Fletcher CD. Mammary-type myofibroblastoma of soft tissue: a tumor closely related to spindle cell lipoma. Am J Surg Pathol. 2001;25:1022–1029.
Diebold M, Soltermann A, Hottinger S, et al. Prognostic value of MIB-1 proliferation index in solitary fibrous tumors of the pleura implemented in a new score—a multicenter study. Respir Res. 2017;18:210.
Gold JS, Antonescu CR, Hajdu C, et al. Clinicopathologic correlates of solitary fibrous tumors. Cancer. 2002;94:1057–1068.
Hanau CA, Miettinen M. Solitary fibrous tumor: histological and immunohistochemical spectrum of benign and malignant variants presenting at different sites. Hum Pathol. 1995;26:440–449.
Demicco EG, Griffin AM, Gladdy RA, et al. Comparison of published risk models for prediction of outcome in patients with extrameningeal solitary fibrous tumour. Histopathology. 2019;75:723–737.
Reisenauer JS, Mneimneh W, Jenkins S, et al. Comparison of risk stratification models to predict recurrence and survival in pleuropulmonary solitary fibrous tumor. J Thorac Oncol. 2018;13:1349–1362.
The WHO Classification of Tumours Editorial Board. WHO Classification of Tumours Soft Tissue and Bone Tumours, 5th ed. Lyon, France: IARC Press; 2020.