1

Introduction

Leiomyosarcoma is a rare disease of the nasal cavity. Soft tissue sarcomas account for 1% of malignant neoplasms of the head and neck; leiomyosarcoma accounts for 3% of these tumors. . As compared to uterine and gastrointestinal variants, tumors involving the head and neck are more aggressive and have a worse prognosis . Fifty-three cases of sinonasal leiomyosarcoma have been reported in literature to date. Of those, none address the non-contiguous growth pattern that can be seen in this type of tumor. Patients commonly lack early symptoms and present when the tumor has grown large enough to cause congestion, obstruction, epistaxis or pain.

2

Case reports

An 83 year old female was referred for evaluation of an incidental right nasal mass, noted on computed tomography during a workup for syncope. When interviewed, she reported facial pain for over one year, unilateral nasal congestion, intermittent epistaxis, and right sided epiphora. Fiberoptic exam was significant for a firm, smooth, and friable nasal mass seen along the right inferior nasal cavity. The CT scan showed a large heterogeneously enhancing mass occupying the right nasal cavity involving the inferior turbinate and lateral nasal wall.

Biopsy in the operating room revealed low to intermediate grade leiomyosarcoma, with areas of necrosis, and approximately 2 mitotic figures per 10 high power fields. Immunohistochemistry studies stained positive for muscle specific actin (MSA) and desmin and negative for pan-keratin (AE1/AE3), CD117, CD34 and S-100 protein.

The patient underwent endoscopic medial maxillectomy and infratemporal fossa dissection to resect the tumor with clear margins. Adjuvant therapy was not recommended by the Multidisciplinary Tumor Board after consideration of the clinical characteristics and review of the available literature.

She was followed regularly for tumor recurrence. Four years after her definitive resection, she reported ipsilateral epiphora and a smooth soft tissue lesion in the anterior nasal cavity was seen adjacent to resection site. This lesion was biopsied and showed recurrent leiomyosarcoma. PET/CT showed a right nasal cavity tumor with suspected involvement of the anterior maxillary sinus, adjacent lacrimal bone, and adjacent remnant anterior ethmoid. There was dehiscence of the lamina papyracea with tumor pushing against but not invading the periorbita. The patient underwent a midfacial degloving to perform a right sided extraoral infrastructure maxillectomy, orbit sparing procedure, with excision of the involved orbital floor. A synchronous non-contiguous lesion was seen on the right nasal septum, and was biopsy proven as a separate leiomysoarcoma; this was resected with clear margins. Due to the recurrent nature of the tumor, as well as the identification of a non-contiguous lesion, post-operative radiotherapy of 35 Gy was recommended and completed to cover the nasal cavities and adjacent maxilla.

Upon evaluation one year after surgery and post-operative radiation, the patient complained of worsening left unilateral epistaxis and nasal obstruction. She presented with a rapidly growing non-contiguous lesion involving the left nasal vestibule, in a separate site than her other lesions. This was biopsied and showed recurrent leiomyosarcoma. The patient underwent an endoscopic-assisted partial rhinectomy with clear margins. Given her prior treatment, and after discussion with the patient about further options, no further adjuvant treatment was pursued. Currently, she continues to be tumor free for sixteen months.

2

Case reports

An 83 year old female was referred for evaluation of an incidental right nasal mass, noted on computed tomography during a workup for syncope. When interviewed, she reported facial pain for over one year, unilateral nasal congestion, intermittent epistaxis, and right sided epiphora. Fiberoptic exam was significant for a firm, smooth, and friable nasal mass seen along the right inferior nasal cavity. The CT scan showed a large heterogeneously enhancing mass occupying the right nasal cavity involving the inferior turbinate and lateral nasal wall.

Biopsy in the operating room revealed low to intermediate grade leiomyosarcoma, with areas of necrosis, and approximately 2 mitotic figures per 10 high power fields. Immunohistochemistry studies stained positive for muscle specific actin (MSA) and desmin and negative for pan-keratin (AE1/AE3), CD117, CD34 and S-100 protein.

The patient underwent endoscopic medial maxillectomy and infratemporal fossa dissection to resect the tumor with clear margins. Adjuvant therapy was not recommended by the Multidisciplinary Tumor Board after consideration of the clinical characteristics and review of the available literature.

She was followed regularly for tumor recurrence. Four years after her definitive resection, she reported ipsilateral epiphora and a smooth soft tissue lesion in the anterior nasal cavity was seen adjacent to resection site. This lesion was biopsied and showed recurrent leiomyosarcoma. PET/CT showed a right nasal cavity tumor with suspected involvement of the anterior maxillary sinus, adjacent lacrimal bone, and adjacent remnant anterior ethmoid. There was dehiscence of the lamina papyracea with tumor pushing against but not invading the periorbita. The patient underwent a midfacial degloving to perform a right sided extraoral infrastructure maxillectomy, orbit sparing procedure, with excision of the involved orbital floor. A synchronous non-contiguous lesion was seen on the right nasal septum, and was biopsy proven as a separate leiomysoarcoma; this was resected with clear margins. Due to the recurrent nature of the tumor, as well as the identification of a non-contiguous lesion, post-operative radiotherapy of 35 Gy was recommended and completed to cover the nasal cavities and adjacent maxilla.

Upon evaluation one year after surgery and post-operative radiation, the patient complained of worsening left unilateral epistaxis and nasal obstruction. She presented with a rapidly growing non-contiguous lesion involving the left nasal vestibule, in a separate site than her other lesions. This was biopsied and showed recurrent leiomyosarcoma. The patient underwent an endoscopic-assisted partial rhinectomy with clear margins. Given her prior treatment, and after discussion with the patient about further options, no further adjuvant treatment was pursued. Currently, she continues to be tumor free for sixteen months.