Abstract

Uterine leiomyosarcoma (uLMS) is a rare and aggressive malignancy with poor clinical outcomes. Even when localized, uLMS is associated with high rates of local and distant recurrences that are usually fatal. Common sites of recurrence are lung, liver, pelvic lymph nodes, and vertebral and long bones, though atypical patterns of recurrence have been described. Among them, intracranial recurrence appears as a rare finding, almost exceptional in skull and dura. We describe the case of a solitary skull metastasis from uLMS in a 39-year-old woman, which represents the third reported case of skull recurrence in literature. After multidisciplinary discussion, the patient underwent surgery and received adjuvant radiotherapy. After 4 months, she is currently alive, without evidence of extracranial disease. This case highlights the importance of suspecting and recognizing atypical and extremely rare metastasis to this region. We encourage the need for large case series in order to provide further information about cranial recurrences of uLMS taking into account the paucity of data currently available in literature and the frequently unpredictable behavior of this rare and highly lethal disease.

Highlights

  • Uterine leiomyosarcoma is a rare malignancy accounting for 1–3% of all uterine cancers and approximately for 65% of all uterine sarcomas (US) [1]

  • Histological exam showed the presence of spindle cells with moderate pleomorphism, 15 mitotic figures per 10 HPF (Figure 2A), and strong positivity for smooth muscle actin (SMA) (Figure 2B), vimentin, and desmin in Abbreviations: cGy, centigray; CT, computed tomography; HPF, high power fields; MDT, multidisciplinary team; MRI, magnetic resonance imaging; OS, overall survival; Uterine leiomyosarcoma (uLMS), uterine leiomyosarcoma; US, uterine sarcoma

  • Diagnosis of skull recurrence of uLMS requires a high index of suspicion

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Summary

BACKGROUND

Uterine leiomyosarcoma (uLMS) is a rare malignancy accounting for 1–3% of all uterine cancers and approximately for 65% of all uterine sarcomas (US) [1]. The array of tools currently available in imaging techniques and the prompt detection in high-risk patients has allowed the identification of more atypical patterns of uLMS recurrence, such as heart, parotid gland, gastrointestinal tract, brain, and skull [8,9,10]. Clinical and imaging follow up showed no evidence of recurrence until June 2019, when a total body computed tomography (CT) scan revealed an osteolytic lesion in the right temporal-occipital region, 8 mm in diameter and positive to bone scintigraphy. Histological exam showed the presence of spindle cells with moderate pleomorphism, 15 mitotic figures per 10 HPF (Figure 2A), and strong positivity for smooth muscle actin (SMA) (Figure 2B), vimentin, and desmin in Abbreviations: cGy, centigray; CT, computed tomography; HPF, high power fields; MDT, multidisciplinary team; MRI, magnetic resonance imaging; OS, overall survival; uLMS, uterine leiomyosarcoma; US, uterine sarcoma. The patient is currently alive, without evidence of extracranial disease

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