Leiomyosarcoma of the maxillary sinuses: report of two cases

Ear, Nose & Throat Journal, Feb, 2004 by Kishore Chandra Prasad, Thokur Bhasker Alva, Urmila Khadilkar, Damodara Madhu

Abstract

Leiomyosarcoma is a malignant smooth-muscle tumor that has a predilection for the gastrointestinal tract and the female genital tract. It is locally fast-spreading and highly aggressive, and the prognosis is poor. We report two cases of leiomyosarcoma of the maxilla in patients who sought treatment for maxillary swelling, nasal obstruction, and epistaxis; one patient also had orbital involvement and cervical lymph node metastasis. Both patients underwent radical surgery followed by chemo- and/or radiotherapy. The patient with orbital involvement died shortly thereafter, but the other was disease-free at 18 months.

Introduction

Smooth-muscle tumors are rare in the head and neck. Most arise in the gastrointestinal tract and in the female genital tract, perhaps because of the preponderance of smooth muscles at these sites. Leiomyosarcomas of the head and neck are believed to originate in the tunica media of the blood vessels or in pluripotential mesenchymal cells. Clinically, these tumors are very aggressive, and the prognosis is poor. Leiomyosarcomas of the paranasal sinus are unusual and distinct entities. We report two cases of leiomyosarcoma of the maxillary sinus. Both cases demonstrated the aggressive and highly malignant nature of these tumors.

Case reports

Patient 1. A 27-year-old woman sought treatment for a 6-month history of painful swelling over the right maxillary area that was accompanied by nasal obstruction, epistaxis, proptosis, and loose teeth.

Clinical examination revealed that the diffuse swelling measured 5 x 4 cm and involved the orbit (figure 1). The tumor extended inferiorly to the angle of the mouth, laterally to the front of the tragus, and medially to the dorsum of the nose. It obliterated the nasolabial fold and involved the skin at the root of the nose. The skin over the swelling appeared to be stretched. Movement of the right eyeball was restricted. Intraorally, the swelling destroyed the hard palate and alveolus. On anterior rhinoscopy, the mass could be seen filling the entire nasal cavity. Findings on postnasal examination were normal. Examination of the neck revealed that multiple cervical lymph nodes at levels I and II were hard, nontender, and mobile.

[FIGURE 1 OMITTED]

We arrived at a clinical diagnosis of a malignancy of the right maxilla with orbital infiltration and metastasis to the cervical lymph nodes. Computed tomography (CT) revealed a large soft-tissue density in the right maxillary sinus that had destroyed all of the maxillary walls except the posterior wall and that had involved the orbit (figure 2). There was no intracranial extension.

[FIGURE 2 OMITTED]

Analysis of the biopsy specimen revealed that the tumor tissue was made up of fascicles of spindle-shaped cells with eosinophilic cytoplasm and oval to elongated and blunt-ended vesicular nuclei (figure 3). Mitosis was conspicuous. Areas of necrosis, fibrosis, hyalinization, and inflammatory infiltrates were seen. The reticulin stain showed abundant reticulin fibers around individual tumor cells. The van Gieson's and Masson's trichrome stains showed smooth-muscle differentiation in the spindle cells. These features were those of a leiomyosarcoma.

[FIGURE 3 OMITTED]

The patient underwent radical maxillectomy with orbital exenteration and radical neck dissection. A temporary obturator was inserted. The patient underwent chemo- and radiotherapy for 3 days, but died of aspiration pneumonia.

Patient 2. A 42-year-old woman reported a 2-month history of swelling over the left maxillary region, nasal obstruction, epistaxis, and loose teeth and a 1-month history of swelling in the left upper jaw. The jaw swelling arose after she had had three teeth extracted by a dentist.

Clinical examination revealed that the diffuse swelling over the left maxillary area measured 3 x 2 cm. The swelling extended superiorly to the infraorbital region, inferiorly to the angle of the mouth, laterally to the malar bone, and medially to the ala of the left nostril, where it obliterated the left nasolabial fold. The skin over the swelling was normal. The same swelling was seen intraorally, and it had destroyed the hard palate and extended to the alveolus, where it had obliterated the gingivobuccal sulcus. The surface of the mass in the oral cavity was smooth, and the mucous membrane was intact. Anterior rhinoscopy revealed that a smooth mass in the nasal cavity had filled the left nostril. Findings on postnasal examination were normal. The orbit and the movement of the left eyeball were also normal. There were no palpable lymph nodes.

We arrived at a clinical diagnosis of a malignancy of the left maxilla without orbital infiltration. CT showed a large soft-tissue density in the maxillary sinus that had destroyed the anterior floor and the medial wall of the left maxillary sinus (figure 4). Biopsy analysis identified the lesion as a leiomyosarcoma. The patient underwent a total maxillectomy followed by radiotherapy, and she received a permanent prosthesis. At the 18-month follow-up, she was symptom-free (figure 5).