P Shukla, P Gupta, M Pant, N Husain, D Gupta, S Bisht, S Gupta, J Verma
leiomyosarcoma, metastasis, oral cavity, renal
P Shukla, P Gupta, M Pant, N Husain, D Gupta, S Bisht, S Gupta, J Verma. Bilateral Renal Metastases In Oral Leiomyosarcoma: A Case Report. The Internet Journal of Radiology. 2009 Volume 11 Number 1.
A primary leiomyosarcoma in the oral cavity with bilateral renal metastasis in a 22-year-old female is reported. The tumour started during pregnancy and progressed to form a large unresectable mass post delivery. CT scan
Leiomyosarcoma account for 5-10% of soft tissue sarcomas. They are principally tumors of adult life and are more common in women than in men. Primary oral leiomyosarcoma is a rare entity with unusual bone location because of paucity of smooth muscle in that site. Isolated renal metastasis in nonrenal sarcomas is extremely rare. We report a histologically proven case of leiomyosarcoma of maxilla with bilateral renal metastasis, which were visualized in CT and diagnosed by CT guided fine needle aspiration cytology (FNAC).
Leiomyosarcoma (LMS) of the oral cavity is a very rare tumor that is associated with aggressive clinical behavior and low survival. They are best treated surgically, early and aggressively. 1
The incidence of LMS among sarcomas of oral & maxillofacial region was found to be 6.25% by Yamaguchi et al2 in a study of 32 cases of oral and maxillofacial sarcomas. Oral LMS have been found to have a peak incidence in the 3rd decade and 6th-7th decade with no gender predilection. Females have a higher incidence in the third decade whereas males have an even age distribution 3. Estrogen receptor positivity has also been reported in some leiomyosarcomas raising the possibility of hormonal responsiveness. In an Immunohistochemical study of estrogen receptor (ER) and progesterone receptor (PR) expression in uterine and extrauterine leiomyosarcomas, it was
Leiomyosarcomas are rare in the maxilla due to minimal smooth muscle in the region. In several studies of oral LMS, 50% -70% of the cases arose from the jaws (1,3,6). Most of the cases appeared to be associated with neurovascular structures of the facial skeleton as evidenced by the imaging studies 3. In our case, though the clinical examination and imaging results indicated a maxillary origin of the tumour, but the exact origin could not be ascertained due to advanced local disease and unresectability of the tumour at the time of presentation.
Distant metastasis seen in 39% cases of oral LMS, occurred mostly to the lungs and some cases showed cervical lymph node metastasis1. The case presented above had no metastasis to the lung as evidenced by chest X-ray and had no enlarged lymph nodes. A case of maxillary LMS with spinal metastasis has been reported in the literature 7. We could find no report in literature of renal metastasis from oral LMS.
Although metastases to the kidney have been reported to occur in 7-20% of patients with cancer at autopsy8, the diagnosis of metastases to the kidney in patients without evidence of a disseminated nonrenal malignancy is rare. In a review of 100 consecutive patients with nonrenal malignancies diagnosed with renal masses at presentation or follow-up, a total of 19 patients were found with metastases to the kidney which included those with primary tumors of the lung, lymphoma, esophagus, head and neck, breast, colon, pancreas, extremity sarcoma, testis and myelodysplasia9. Other primary neoplasms which may metastasize to the kidney include carcinoma of the stomach, melanoma and leukaemia. Metastases to the kidneys are frequently small, asymptomatic and detected only at autopsy. Renal metastases may present as multiple or bilateral small renal masses or, less commonly, as a solitary lesion. Our case presented with bilateral renal masses in the presence of a nonrenal malignancy, which was very suggestive of metastasis to the kidney. We further confirmed the presence of spindle cell neoplasm in CT guided FNAC from the renal lesions.
The diagnosis of soft tissue leiomyosarcoma is based on the morphologic features mainly a spindle cell neoplasm with intersecting bundles, blunt ended nuclei, varying degrees of anaplasia and mitoses with the support of IHC, chiefly Smooth muscle actin and desmin 10. The case presented, fulfilled these criteria confirming the diagnosis of leiomyosarcoma.
Surgical resection, hemimandibulectomy or hemimaxillec¬tomy, partial maxillectomy, are the preferred initial treat¬ments for cases of leiomyosarcoma primary in the jawbones. Regional lymph node resection is recommended if there is clinical evidence of lymphadenopathy. In the present case as surgical resection was not possible chemotherapy was instituted. The patient has received three cycles of anthracycline based chemotherapy and is planned for further chemotherapy. Some regression in the size of the lesion has been observed.