Leiomyosarcoma

Abstract

Maxillary leiomyosarcoma is an uncommon tumor, and the occurrence of its metastasis to the spine is exceedingly rare. We report the case of a patient with maxillary leiomyosarcoma who developed a spinal metastasis 18 months following a total maxillectomy. Our patient represents only the third case of metastasis to the spine that has been reported in the literature, and the only reported case of metastasis to the iliac bone.

Introduction

Leiomyosarcoma, a malignant lesion of smooth-muscle origin, accounts for approximately 7% of all soft-tissue sarcomas. (1) Most of these tumors occur in the uterus, gastrointestinal tract, and retroperitoneum. The rarity of this neoplasm in the oral cavity can be attributed to the paucity of smooth muscle at this site. Those lesions that do develop are believed to arise from the tunica media or from pluripotential undifferentiated mesenchymal cells. (2, 3)

Leiomyosarcomas that arise in the maxilla are very rare, and to our knowledge, only 15 cases of metastatic leiomyosarcoma have been reported in the literature. (4) Among these previously reported cases, only two involved metastasis to the spine. In this article, we report a new case of spinal metastasis of maxillary leiomyosarcoma.

Case report

A 41-year-old man came to the otolaryngology clinic with a 7-month history of a slowly progressive and painful swelling on the left side of his hard palate. On examination, the swelling was found to be firm, nontender, and approximately 3 x 2cm in size. The overlying mucosa was intact, and no bleeding or ulceration was present. No cervical neck nodes were palpable. The remainder of the physical examination revealed no other abnormality.

Analysis of a punch biopsy of the mass revealed the presence of interlaced fascicles of spindle cells, eosinophilic cytoplasm, and blunt-ended hyperchromatic nuclei. Abundant mitoses (9/high-power field), moderate nuclear hyperchromatism, and pleomorphism were also seen. No necrosis was observed. To confirm the likely diagnosis of leiomyosarcoma, immunohistochemistry was performed for cytoskeletal proteins such as actin, myosin, and desmin; these proteins have been widely used in the differential diagnosis of sarcomas. (5, 6) The results were conclusive.

Computed tomography of the head and neck identified a 5 x 3 x 2-cm mass that involved the maxilla and hard palate and extended into the pterygopalatine fossa, the posterior wall of the maxillary sinus, and the left nasal cavity. Inversion of the left inferior turbinate was noted. No muscular invasion or lymphadenopathy was documented.

A left total maxillectomy was performed, and the specimen was sent for repeat confirmatory histopathology. The maxillary defect was filled with a split-thickness skin graft obtained from the right thigh. The patient had a 6-day hospital stay and experienced no postoperative complications. Postdischarge management included radiotherapy over a 2-month period and regular follow-up visits every 4 weeks.

Exactly 18 months following his surgery, the patient came to the orthopedics clinic complaining of lower back pain of 45 days' duration and numbness on the lateral border of his left foot of 15 days' duration. The back pain radiated to both buttocks; the pain worsened when the patient was sitting and abated when he was lying down. Since his surgery, the patient had experienced no trauma or weight loss. No history of urinary or bowel symptoms was documented.

On examination, the patient's ankle reflex on his left side was absent, and there was a diminished sensation over his S1 dermatome. During a straight-leg-raising test, the patient was able to lift his left leg only 40[degrees]. The results of the examination of his right leg were normal.

Magnetic resonance imaging (MRI) of the lumbosacral region detected extensive metastatic disease to the vertebral and iliac bones (figure). No evidence of any nerve compression was seen. Findings on repeated chest x-rays were normal.

The patient was referred to an oncologist. After multiple courses of chemotherapy, the patient was free of disease at the most recent follow-up.

Discussion

Oral leiomyosarcomas are extremely rare. Affected patients have ranged in age from 15 to 85 years, and the disease has a predilection for males. (4) The diagnosis of leiomyosarcoma of the oral cavity can be difficult because of its similarities to other sarcomas that are made up of spindle cells. Therefore, it is essential that immunohistochemical techniques be employed to accurately diagnose sarcomas. Reactivity to desmin and muscle-specific actin in spindle-cell sarcomas confirms a diagnosis of leiomyosarcoma. Further confirmation can be obtained by electron microscopy. (7)

Several theories have been proposed to explain the origin of leiomyosarcoma of the oral cavity, and the most commonly agreed-upon tissue of origin is the vascular wall. (8) Surgical resection with adequate margins of uninvolved tissue is the recommended treatment. (9) Although many tumors appear to be well circumscribed, the high incidence of local recurrence and the persistence of leiomyosarcomas despite initial wide resection illustrate the infiltrative and aggressive nature of this malignancy. The postoperative course is unpredictable, and long-term follow-up is mandatory.

Metastasis to regional lymph nodes is relatively rare in cases of oral leiomyosarcoma, whereas pulmonary and hepatic metastases have been reported frequently. (1) Our patient represents only the third case of metastasis to the spine that has been reported in the literature, and the only reported case of metastasis to the iliac bone.

References

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(2.) Kratochvil FJ III, MacGregor SD, Budnick SD, et al. Leiomyosarcoma of the maxilla. Report of a case and review of the literature. Oral Surg Oral Med Oral Pathol 1982;54:647-55.

(3.) Martin-Hirsch DP, Habashi S, Benbow EW, Farrington WT. Post-irradiation Ieiomyosarcoma of the maxilla. J Laryngol Otol 1991;105:1068-71.

(4.) Wertheimer-Hatch L, Hatch GF III, Hatch KF, et al. Tumors of the oral cavity and pharynx. World J Surg 2000;24:395-400.

(5.) Miettinen M, Lehto VP, Ekblom P, et al. Leiomyosarcoma of the mandible: Diagnosis as aided by immunohistochemical demonstration of desmin and laminin. J Oral Pathol 1984;13:373-81.

(6.) Krishnan V, Miyaji CM, Mainous EG. Leiomyosarcoma of the mandible: A case report. J Oral Maxillofac Surg 1991;49:652-5.

(7.) Gabbiani G, Kapanci Y, Barazzone P, Franke WW. Immunochemical identification of intermediate-sized filaments in human neoplastic cells. A diagnostic aid for the surgical pathologist. Am J Pathol 1981;104:206-16.

(8.) Savastano G, Palombini L, Muscariello V. Erra S. Leiomyosarcoma of the maxilla: A case report. J Oral Maxillofac Surg 1998;56:1101-3.

(9.) Wile AG, Evans HL, Romsdahl MM. Leiomyosarcoma of soft tissue: A clinicopathologic study. Cancer 198l;48:1022-32.

From the Department of Surgery, Aga Khan University Hospital, Karachi, Pakistan.

Reprint requests: Mubasher Ikram, FCPS, ENT-Head and Neck Surgery, Department of Surgery, Aga Khan University Hospital, Stadium Rd., Karachi, 74800 Pakistan. Phone: 92-21-4859-4770; fax: 92-21-493-4294 or 92-21-493-2095; e-mail: mubasher.ikram@aku.edu

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