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.
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.
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.
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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.
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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.
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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).
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The first case of maxillary leiomyosarcoma was reported in 1958. (1) Since then, only about 65 cases have been reported throughout the world. (2)
Incidence. Leiomyosarcomas account for 6.5% of all soft-tissue sarcomas, and only 3% of them arise in the head and neck. (3) When they occur in the sinonasal tract, the most common sites are the nasal cavity, the maxillary sinus, and the ethmoid sinus, in that order.
Clinical features. Leiomyosarcomas of the sinonasal tract are more common in men than in women. (4) The average age at diagnosis is 50 years. (5) Initial symptoms in order of decreasing frequency include nasal obstruction, epistaxis, facial pain, and facial swelling. Sinonasal tract leiomyosarcoma is characterized as locally aggressive rather than as having high metastatic potential. (1) Any degree of orbital invasion, which occurred in our patient 1, is disastrous. Leiomyosarcoma with regional lymph node involvement is rare, but cervical node metastasis has been reported in 15% of leiomyosarcomas that arise in the oral cavity. (6)
Histopathology. On histologic examination, the tumor is made up of interlacing fascicles of spindle-shaped cells that have elongated, blunt-ended nuclei and eosinophilic cytoplasm. Numerous mitotic figures are present. The cytoplasm of the tumor cells stains red in Masson's trichrome medium. (7)
Radiology. On CT, leiomyosarcomas appear as bulky masses, and they are frequently associated with extensive necrotic or cystic changes. These tumors do not contain calcifications, but they do cause frank bony destruction, as occurred in both of our patients. On magnetic resonance imaging, leiomyosarcomas show intermediate enhancement with contrast and intermediate to high signal intensity on T2-weighted imaging. (3)
Differential diagnosis. Depending on the patient's age and the location of the tumor, the differential diagnosis includes malignant schwannoma, malignant fibrous histiocytoma, fibrosarcoma, amelanotic melanoma, malignant lymphoma, squamous cell carcinoma, extramedullary plasmacytoma, metastatic tumor, glandular tumor, chondroma, chondrosarcoma, osteogenic sarcoma, and inverted papilloma. (3,6)
Treatment. Although regional lymph node involvement is rare, when it does occur, resection of the primary tumor with wide margins and neck dissection is the treatment of choice. Recent studies show that combination therapy with mesna, doxorubicin, ifosfamide, and dacarbazine or combination therapy with cyclophosphamide, doxorubicin, vincristine, and dacarbazine is effective against soft-tissue sarcomas.
The clinical features of leiomyosarcoma in our 2 patients differed in that patient 1 exhibited orbital involvement and cervical lymph node metastasis. Her prognosis was very poor and despite radical surgery and postoperative chemo- and radiotherapy, she died of complications. Patient 2, who had less extensive disease, responded well to treatment.
(1.) Konrad HR. Nasal neoplasms. In: English GM, ed. Otolaryngology. Philadelphia: J.B. Lippincott, 1993:1-11.
(2.) Ortega JM, Gomez-Angulo JC, Aragones P, et al. [Leiomyosarcoma of the paranasal sinuses with intracranial involvement: Report of a clinical case and review of the literature]. Neurocirugia (Astur) 2001;12:331-7.
(3.) Tanaka H, Westesson PL, Wilbur DC. Leiomyosarcoma of the maxillary sinus: CT and MRI findings. Br J Radiol 1998;71: 221-4.
(4.) Carran RL, Myers EN. Neoplasms of the nose and paranasal sinuses. In: Bailey BJ, ed. Head and Neck Surgery: Otolaryngology. 2nd ed. Vol. 2. Philadelphia: Lippincott-Raven, 1998:1450.
(5.) Rice DH, Stanley RB. Surgical therapy of tumors of the nasal cavity, ethmoid sinus and maxillary sinus. In: Thawley SE, Panje WR, eds. Comprehensive Management of Head and Neck Tumors. 2nd ed. Vol. 1. Philadelphia: W.B. Saunders, 1999:571.
(6.) Sumida T, Hamakawa H, Otsuka K, Tanioka H. Leiomyosarcoma of the maxillary sinus with cervical lymph node metastasis. J Oral Maxillofac Surg 2001;59:568-71.
(7.) Izumi K, Maeda T, Cheng J, Saku T. Primary leiomyosarcoma of the maxilla with regional lymph node metastasis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995;80:310-19.
From the Department of Otolaryngology (Dr. Prasad, Dr. Alva, and Dr. Madhu) and the Department of Pathology (Dr. Khadilkar), Kasturba Medical College, Mangalore, India.
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