Recurrent vaginal epithelioid leiomyosarcoma; a case report from Botswana and review of the literature

• Published in: African journal of reproductive health 2021-02, Vol.25 (1), p.161-168
• Main Authors: Benti, Tadele Melese, Mercy, Nassali, Eshetu, Alemayehu B, Iwuh, Ibe Enyeribe, Kassa, Mamo Woldu
• Format: Article
• Language: English
• Subjects: Botswana; Care and treatment; Case reports; Development and progression; Diseases; epithelioid leiomyosarcoma; Estrogen; Estrogens; Histochemistry; Histopathology; Hysterectomy; Leiomyosarcoma; Lymphatic system; léiomyosarcome épithélioïde; masse vaginale; Medical prognosis; Metastasis; Oophorectomy; Patients; Progesterone; Radiation therapy; radiotherapy; radiothérapie; Relapse; Sarcoma; sarcome vaginal; Vagina; vaginal mass; vaginal sarcoma
• ISSN: 1118-4841; 1118-4841
• DOI: 10.29063/ajrh2021/v25i1.17

Primary vaginal leiomyosarcoma is a rare gynecological malignancy. The clinical presentation is a benign looking well circumscribed mobile mass which might however occasionally present with distant metastases. Post treatment recurrence is common, and the clinical course is often unpredictable. Primary surgical management plus radiotherapy is the commonly practiced treatment of choice. We report a case of primary vaginal epithelioid leiomyosarcoma that recurred twice after local surgical resection. The patient subsequently underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy for a recurrent vaginal leiomyosarcoma. Histopathology of the vaginal mass revealed an epithelioid leiomyosarcoma of the vagina. She also received a course of adjuvant radiotherapy. The patient was free of recurrence at 3 year follow up. Vaginal mass must be evaluated with a high index of suspicion for malignancy. Local surgical resection alone is insufficient for primary vaginal leiomyosarcoma. Surgical resection with adjuvant radiotherapy offers better treatment outcomes with decreased risk of recurrence. Empirical oophorectomy in patients with completed family size might confer additional benefit in preventing disease recurrence in resource limited settings where testing for estrogen and progesterone receptor status of the tumour is unavailable. Due to the unpredictable course of the disease, lifelong patient follow-up is critical for better outcomes. (Afr J Reprod Health 2021; 25[1]: 161-168). Le léiomyosarcome vaginal primaire est une tumeur maligne gynécologique rare. La présentation clinique est une masse mobile bien circonscrite d'aspect bénin qui peut cependant occasionnellement présenter des métastases à distance. La récidive après le traitement est courante et l'évolution clinique est souvent imprévisible. La prise en charge chirurgicale primaire associée à la radiothérapie est le traitement de choix couramment pratiqué. Nous rapportons un cas de léiomyosarcome épithélioïde vaginal primitif récidivant deux fois après une résection chirurgicale locale. La patiente a ensuite subi une hystérectomie abdominale totale avec salpingo-ovariectomie bilatérale pour un léiomyosarcome vaginal récidivant. L'histopathologie de la masse vaginale a révélé un léiomyosarcome épithélioïde du vagin. Elle a également reçu un cours de radiothérapie adjuvante. Le patient était sans récidive à 3 ans de suivi. La masse vaginale doit être évaluée avec un indice de suspicion él