Genital rhabdomyoma of the urethra in an infant girl
Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA.Human pathology (Impact Factor: 2.77). 10/2011; 43(4):597-600. DOI: 10.1016/j.humpath.2011.06.012
Extracardiac rhabdomyomas are rare benign entities that usually occur in the head and neck region. Although genital rhabdomyoma is known to occur in the lower genital tract of young and middle-aged women, involvement of the anatomically adjacent urethra by rhabdomyoma is exceedingly rare. We present a case of genital rhabdomyoma arising from the urethra of an infant girl. The tumor was characterized by the submucosal presence of mature-appearing rhabdomyoblastic cells containing conspicuous cross-striations, with the cells set in a collagenous stroma. Necrosis and mitoses were absent. Skeletal muscle differentiation of the tumor cells was supported by positive immunohistochemical staining for desmin and myogenin. To our knowledge, this is the first case of urethral genital-type rhabdomyoma in a child.
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ABSTRACT: To investigate the clinicopathologic characteristics, differential diagnosis and biological behavior of extracardiac rhabdomyoma. Nine cases of extracardiac rhabdomyoma diagnosed between January of 1997 and July of 2014 were reviewed. The clinical, pathologic and immunohistochemical profiles were evaluated. There were 5 males and 4 females at diagnosis with age ranging from 2 years and three months to 59 years (mean, 37.6 years). Sites included the head and neck region (7 cases), chest (1 case ) and vagina wall (1 case). Clinically, most cases manifested as a subcutaneous nodule or as a submucosal polypoid lesion with a mean diameter of 3.2 cm. Histologically, 4 were adult-type rhabdomyoma characterized by tightly packed large round or polygonal rhabdomyoblasts with abundant eosinophilic to clear cytoplasm; 3 were myxoid variant of fetal rhabdomyoma composed of immature myofibrils, spindled and primitive mesenchymal cells embedded in a myxoid background, 1 was an intermediate form of fetal rhabdomyoma consisting of densely arranged differentiated myoblasts with little myxoid stroma; 1 was a genital rhabdomyoma composed of elongated or strap-like myoblasts scattered in loose fibrous connective tissue. By immunohistochemistry, they showed diffuse and strong positivity for desmin, MSA and myoglobin with variable expression of myogenin. A case of intermediate type also stained for α-smooth muscle actin. Follow up data (2 months∼17 years) showed local recurrence in one patient 6 months after surgery. Rhabdomyoma is a distinctively rare benign mesenchymal tumor showing skeletal muscle differentiation, which may occassionally recur if incompletely excised. Familiarity with its clinical and morphological variants is essential to avoid misdiagnosing this benign lesion as embryonal rhabdomyosarcoma.
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ABSTRACT: Objectives: The female urethra may become involved by a variety of conditions, which may be challenging to providers who treat women. Mass-like urethral lesions need to be distinguished from other lesions arising from the anterior (ventral) vagina. Methods: A literature review was conducted. A Medline search was used, using the terms urethral neoplasms, urethral diseases, and female. Results: Reported lesions of the urethra were reviewed. Many of these lesions can be mistaken for each other clinically. Conclusions: A review of the scope of urethral lesions is presented to assist in developing a differential diagnosis if a patient with a urethral lesion is encountered. For practitioners who care for women, this will encourage communication and consultation where appropriate.
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