Primary vaginal leiomyosarcomas (pvLMS) are rare, recurrent tumours accounting for ca. 2% of all vaginal cancers. The etiology is still unknown, the prognosis is poor and there is no consensus guideline on its management. Diagnosis is usually made during the 5th decade due to the presence of a vaginal mass or nodule [1-2]. Current medical literature reports about 200 cases (PubMed®); only 3 studies have considered the ultrastructure [2-4]. Herein a pvLMS is presented and discussed. A nodular, 25 x 23 x 28 mm-mass, infiltrating the urethra but not the rectovaginal septum, was widely excised from the superior vaginal wall of a 58-year-old previously hysterectomized woman. Macroscopic images and MRI were performed. Iliac lymph nodes and HMB45 were negative. The sample was fixed and prepared for light microscopy, transmission (TEM) and scanning (SEM) electron microscopy. Semithin sections showed a storiform pattern of spindle shaped cells with blunt-ended nuclei. Cells arranged in interwoven fascicles within a dense and richly vascularised stroma (neoangiogenesis). Some atypic mitotic figures and focal necrosis were seen. SEM evidenced a dense collagenous stroma with numerous microvessels. TEM showed neoplastic and pleomorphic cells with complex cytoplasm projections containing paranuclear crowds of dilated mitochondria, free ribosomes and a well-developed rough endoplasmic reticulum. Nuclei were large, mostly hyperchromatic, usually indented, with prominent nucleoli and nucleolonema. The dense intercellular space contained dense bundles of collagen fibers. A high and reactive endothelium lined blood vessels. After 4 follow-ups, the patient is fine and without recurrence. Best outcomes occur when the tumour is small, localized, and can be removed surgically with wide, clear margins, as it was for this case. As there are different kinds of LMS, biopsy followed by immunohistochemistry and electron microscopy still represents a good diagnostic choice. References [1] Umeadi et al. (2008) Vaginal leiomyosarcoma. J Obstet Gynaecol 28(5): 553-554. [2] Tobon et al. (1973) Primary leiomyosarcoma of the vagina. Light and electron microscopic observations. Cancer 32(2): 450-457. [3] Akhtar et al. (1978) Primary leiomyosarcoma of the vagina: light and electron microscopic study of a case with review of literature. Tex Med 74(9): 67-71. [4] Rastogi et al. (1984) Primary leiomyosarcoma of the vagina: a study of five cases. Gynecol Oncol 18(1): 77-86.

Primary vaginal leiomyosarcoma, a rare tumour: case report and review / Rosemarie, Heyn; Corrado, Giacomo; Battaglione, Ezio; Vizza, Enrico. - In: ITALIAN JOURNAL OF ANATOMY AND EMBRYOLOGY. - ISSN 2038-5129. - ELETTRONICO. - 122:1 Suppl.(2017), pp. 110-110. (Intervento presentato al convegno 71° Congresso della Società Italiana di Anatomia e Istologia tenutosi a Taormina) [10.13128/IJAE-21502].

Primary vaginal leiomyosarcoma, a rare tumour: case report and review

Rosemarie Heyn
;
Ezio Battaglione
Software
;
2017

Abstract

Primary vaginal leiomyosarcomas (pvLMS) are rare, recurrent tumours accounting for ca. 2% of all vaginal cancers. The etiology is still unknown, the prognosis is poor and there is no consensus guideline on its management. Diagnosis is usually made during the 5th decade due to the presence of a vaginal mass or nodule [1-2]. Current medical literature reports about 200 cases (PubMed®); only 3 studies have considered the ultrastructure [2-4]. Herein a pvLMS is presented and discussed. A nodular, 25 x 23 x 28 mm-mass, infiltrating the urethra but not the rectovaginal septum, was widely excised from the superior vaginal wall of a 58-year-old previously hysterectomized woman. Macroscopic images and MRI were performed. Iliac lymph nodes and HMB45 were negative. The sample was fixed and prepared for light microscopy, transmission (TEM) and scanning (SEM) electron microscopy. Semithin sections showed a storiform pattern of spindle shaped cells with blunt-ended nuclei. Cells arranged in interwoven fascicles within a dense and richly vascularised stroma (neoangiogenesis). Some atypic mitotic figures and focal necrosis were seen. SEM evidenced a dense collagenous stroma with numerous microvessels. TEM showed neoplastic and pleomorphic cells with complex cytoplasm projections containing paranuclear crowds of dilated mitochondria, free ribosomes and a well-developed rough endoplasmic reticulum. Nuclei were large, mostly hyperchromatic, usually indented, with prominent nucleoli and nucleolonema. The dense intercellular space contained dense bundles of collagen fibers. A high and reactive endothelium lined blood vessels. After 4 follow-ups, the patient is fine and without recurrence. Best outcomes occur when the tumour is small, localized, and can be removed surgically with wide, clear margins, as it was for this case. As there are different kinds of LMS, biopsy followed by immunohistochemistry and electron microscopy still represents a good diagnostic choice. References [1] Umeadi et al. (2008) Vaginal leiomyosarcoma. J Obstet Gynaecol 28(5): 553-554. [2] Tobon et al. (1973) Primary leiomyosarcoma of the vagina. Light and electron microscopic observations. Cancer 32(2): 450-457. [3] Akhtar et al. (1978) Primary leiomyosarcoma of the vagina: light and electron microscopic study of a case with review of literature. Tex Med 74(9): 67-71. [4] Rastogi et al. (1984) Primary leiomyosarcoma of the vagina: a study of five cases. Gynecol Oncol 18(1): 77-86.
2017
71° Congresso della Società Italiana di Anatomia e Istologia
04 Pubblicazione in atti di convegno::04d Abstract in atti di convegno
Primary vaginal leiomyosarcoma, a rare tumour: case report and review / Rosemarie, Heyn; Corrado, Giacomo; Battaglione, Ezio; Vizza, Enrico. - In: ITALIAN JOURNAL OF ANATOMY AND EMBRYOLOGY. - ISSN 2038-5129. - ELETTRONICO. - 122:1 Suppl.(2017), pp. 110-110. (Intervento presentato al convegno 71° Congresso della Società Italiana di Anatomia e Istologia tenutosi a Taormina) [10.13128/IJAE-21502].
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1112535
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