Leiomyosarcoma of the Nasal Cavity

Abstract

Leiomyosarcoma (LMS) in the sinonasal tract (SNT) is a rarity that has been firstly described in 1958. Since then, there have been only a few articles about this entity. Most of the data available about LMS in the SNT is derived from case reports. We believe that our case will support the data set and help guiding the management of this rare condition. A 84-year-old female presented with nasal airway obstruction on the left side. She experienced several episodes of epistaxis from her left nostril, what made her to seek medical care. A rhinoscopy revealed an obstructing mass in the left nasal cavity. Computed tomography (CT) scan of the paranasal sinuses revealed a homogenous mass occupying the left nasal cavity, bone destruction of the left middle, and inferior nasal turbinates. An infiltration of the left nasolacrimal duct was also present. The patient refused to undergo open surgery and the mass was removed during an endoscopic approach. The histopathological analysis combined with immunohistochemistry was consistent with LMS. The resection margins were positive for tumor cells. A staging with CT-neck-thorax, abdomen ultrasound, and MRI of the head ruled out metastases. She underwent a second endoscopic tumor resection surgery with positive resection margins and obtained adjuvant radiotherapy. On 9 months of follow-up, there was no recurrence or metastases.

Endoscopic Treatment of Sinonasal Leiomyosarcoma: A Case Report in Light of the Literature

“…Sinonasal leiomyosarcoma is a rare aggressive sarcoma that represents only 2% to 3% of non-epithelial neoplasia of the nasal cavity, paranasal sinuses and nasopharynx. 1 Its most frequent localization is the nasal cavity. 2 An association with previous therapeutic irradiation and hereditary retinoblastoma has been described.…”

“…2 An association with previous therapeutic irradiation and hereditary retinoblastoma has been described. [1][2][3] The diagnosis of sinonasal leiomyosarcoma is often difficult, as patients often present late, owing to the non-specific symptoms and non-specific clinical and radiological findings. 4,5 The presenting symptoms depend on the localization of the leiomyosarcoma within the sinonasal tracts.…”

“…4 The mainstay of treatment is complete surgical extirpation to avoid recurrence. [1][2][3][4] We report the case of a 49-year-old Japanese man who presented with left nasal bleeding. Complete resection with endoscopic surgery was performed.…”

“…Sinonasal leiomyosarcoma is a rare type of tumor. 1 Leiomyosarcoma is an aggressive mesenchymal tumor that shows smooth muscle differentiation by histology and immunophenotype. 2 Common affected sites are uterus, skin and retroperitoneum, with only around 3% in the head and neck region.…”

“…2 Common affected sites are uterus, skin and retroperitoneum, with only around 3% in the head and neck region. 1,2 Clinically, sinonasal leiomyosarcoma is associated with nasal obstruction and nasal bleeding. 3 Histologically, leiomyosarcoma is made up of spindle cells forming fascicular, storiform, or whorled patterns showing immunoreactivity for α smooth muscle actin and desmin.…”