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Vulvar Pathology pp 439–489Cite as

Tumors of Smooth Muscle, of Skeletal Muscle, and of Unknown Origin and Tumor-Like Conditions of the Vulva

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Abstract

This chapter analyzes a variety of benign and malignant vulvar mesenchymal tumors and tumorlike conditions. Smooth muscle tumors arising in the vulva have a unique set of criteria for predicting behavior; the guidelines for assessing smooth muscle tumors in the uterus, deep soft tissues, and skin each differ significantly and are not applicable to the vulva. Skeletal muscle tumors arising in the vulvovaginal region—rhabdomyoma and rhabdomyosarcoma—are distinguished by their composition of mature skeletal muscle elements. Recognizing the histopathologic spectrum of rhabdomyosarcoma is key to effectively selecting cases for molecular testing. With respect to the tumors of unknown origin discussed herein—epithelioid sarcoma, Ewing sarcoma, and synovial sarcoma—ancillary studies have become a staple of diagnosis, especially for those affecting unusual body sites like the vulva. Two tumorlike conditions—Langerhans cell histiocytosis and traumatic neuroma—are also covered. Langerhans cell histiocytosis exhibits a polymorphous clinical picture and typically affects the vulva as part of a multisystem process. Despite advances in elucidating the molecular underpinnings of this disease, treatment for vulvar cases continues to be ad hoc. Traumatic neuromas of the vulva are rare and differ from those occurring at other body sites in their unique clinical history.

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Notes

  1. 1.

    Histiocytoid is used in place of histiocytic because although dendritic cells are considered histiocytes (together with macrophages), common usage today of “histiocyte” connotes a monocyte-derived tissue macrophage.

  2. 2.

    One case was apparently published twice under different names [252, 253], both of which were counted in the totals by Asher [250] and Sumathi [254].

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Authors and Affiliations

  1. Department of Pathology, The University of Chicago Medical Center, Chicago, IL, USA

    Kristen M. Paral M.D.

  2. Department of Medicine, The University of Chicago Medicine, Chicago, IL, USA

    Christopher R. Shea M.D.

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  1. Kristen M. Paral M.D.
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Correspondence to Christopher R. Shea M.D. .

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  1. Department of Pathology, Harvard Medical School, Massachusetts General Hospital, Boston, Massachusetts, USA

    Mai P. Hoang

  2. Department of Pathology, Duke University Medical Center, Durham, North Carolina, USA

    Maria Angelica Selim

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Paral, K.M., Shea, C.R. (2015). Tumors of Smooth Muscle, of Skeletal Muscle, and of Unknown Origin and Tumor-Like Conditions of the Vulva. In: Hoang, M., Selim, M. (eds) Vulvar Pathology. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1807-2_15

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