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Journal of Cancer Research and Therapeutics, Vol. 7, No. 2, April-June, 2011, pp. 185-188 Case Report Myxofibrosarcoma of the infratemporal space Arvind Krishnamurthy1, Anitha Vaidhyanathan1, Urmila Majhi2 1 Department of Surgical Oncology, Cancer Institute (WIA), 36, Sardar Patel Road, Adyar, Chennai - 600 020, India Code Number: cr11043 PMID: 21768709 Abstract A 42-year-old lady described a three-month history of a left-sided, painless swelling below the left earlobe. She presented to us after an acute exacerbation - with complaints of aching pain over the area for the past ten days. Computed tomography (CT) revealed a heterodense mass lesion measuring 48 x 53 mm at its greatest dimension, arising from the left infratemporal fossa. A preoperative trucut biopsy of the lesion showed features of a spindle cell lesion with fibromyxoid stroma. Resection of the infratemporal mass, a left-sided posterior segmental mandibulectomy with infratemporal clearance was performed. The tumor was histopathologically and immunohistochemically diagnosed as a myxofibrosarcoma-Grade II. She received 56 Gy/30 fractions postoperatively. The patient's postoperative clinical course was uneventful, and she remains free of disease 26 months after treatment. We review our experience and to the best of our knowledge, document the first case of myxofibrosarcoma to arise in the infratemporal space. Keywords: Head and neck sarcomas, infratemporal space, myxofibrosarcoma Introduction Myxofibrosarcoma has been documented to share ultrastructural phenotypes with that of normal fibroblasts and has been classified as a distinct fibroblastic sarcoma. [1],[2] Commonly seen in the extremities and trunk, and rarely in the retroperitoneum and head and neck, [1],[2],[3],[4],[5],[6],[7] it has been known to grow slowly and painlessly, with patients presenting with acute exacerbation of symptoms. [1] We present a case wherein a patient presented with myxofibrosarcoma in the left infratemporal space. To the best of our knowledge, such a presentation of a myxofibrosarcoma has not been reported before. Case Report A 42-year-old lady presented with a complaint of swelling below the left earlobe which had increased in size over three months. She presented in August 2008 after an acute exacerbation - with complaints of aching pain over the area for the past ten days. Clinical examination revealed a firm, well-circumscribed swelling measuring 7 x 5 cm in the left parotid region. The mass was nodular on palpation and painful on pressure. Otorhinolaryngological findings were otherwise normal; there was no evidence of cervical lymphadenopathy or any systemic symptoms. Facial nerve function remained unaffected. Computed tomography (CT) revealed a heterodense mass lesion measuring 48 x 53 mm at its greatest dimension, arising from the infratemporal fossa, involving the masticator space, pushing the parotid space laterally and the parapharyngeal space medially, with destruction of the ascending ramus and extension into the skull base [Figure - 1]A,B. Her chest skiagram was normal. A preoperative trucut biopsy of the lesion showed features of a spindle cell lesion with fibromyxoid stroma. The patient was taken up for surgery, intraoperatively the lesion was visible in the left infratemporal space eroding the ascending ramus of the mandible and displacing the parotid laterally. A superficial parotidectomy was performed, preserving all branches of the facial nerve. The tumor was resected en bloc along with the posterior segment of the mandible [Figure - 2]A,B. Macroscopically, the tumor mass was a well-circumscribed grey-white gelatinous nodular mass measuring 6.5 x 4.5 x 4 cm, with the adherent posterior segment of the left hemi-mandible. Microscopy of the tumor mass revealed a multilobulated tumor mass composed of strands and sheets of polygonal to spindle-shaped cells with moderate cytoplasm and vesicular nuclei separated by a myxoid stroma with foci of chondroid and osteoid. Mitosis observed was 0-2/ hpf [high power field] Infiltration of bone at the periphery was also evident. The superficial lobe of the parotid showed normal salivary gland tissue with reactive changes. Immunohistochemical studies showed that the tumor cells expressed strong immunoreactivity for epithelial membrane antigen (EMA), keratin, smooth muscle actin, S100, and vimentin. Labeling index of Ki-67 was at 40% [Figure - 3]A,B. On the basis of the histopathological and immunohistochemical findings, the tumor was diagnosed as a myxofibrosarcoma-Grade II. As the tumor had eroded the mandible, she was advised postoperative radiotherapy. She received external beam radiotherapy using Cobalt 60 to a total dose of 56 Grey in 30 fractions. The course of treatment was uneventful. She is currently on regular review and free of disease for the past 26 months [Figure - 4]. Discussion Myxofibrosarcomas of the head and neck are extremely rare; there have been a few isolated case reports of tumors occurring in the sphenoid sinus, mandible, parotid, and hypopharynx. The reported peak tumor incidence is between the sixth and eighth decade, with equal sex predilection. Mentzel separated myxofibrosarcoma into two groups: superficial (dermal/subcutaneous) and deep (intramuscular/subfascial). [2] While the superficial group of tumors infiltrate and spread extensively in a longitudinal manner, the tumors arising in the intramuscular/subfascial plane were noted to form a single discrete mass. Clinically, the full extent of the tumor is difficult to determine as these tumors lack a pseudocapsule and display multiple gelatinous nodules with non-palpable spread along muscle bundles, infiltrating fascial planes as well as superficial tissues beyond the epicenter of the primary tumor. [4],[5],[7],[8],[9] Wada reported a peculiar growth pattern with extension of tumor tissue along vascular and fascial planes, travelling the distance of the entire thigh without the characteristic "nodular" appearance. [9] Manoso states that a diffuse spreading growth pattern considerably reduced the disease-free survival as compared with centrifugal nodular type of growth pattern. [4] In confirmation, Kaya in their magnetic resonance imaging (MRI) evaluation documented 80.9% of tumors to show multidirectional signal spreading across fascial planes, and this pattern was confirmed as tumor infiltration histologically. [5] This accounts for the difficulty in complete resection, propensity for local recurrence, tumor progression and increased metastases. These tumors have been subdivided into three or four grades based on the degree of cellularity, nuclear pleomorphism and mitotic activity. [2],[3] The tendency of myxofibrosarcoma to be of progressively higher grade in recurrences by becoming gradually more cellular, more pleomorphic, more mitotically active, and more necrotic seems to be a distinctive feature and is in contrast to most other sarcomas. [2] Surgery in the form of wide excision aimed at achieving negative margins is the main modality of treatment. Mentzel reviewed 75 patients, with an average follow-up of 45 months, with local recurrences documented at 55%. The authors associate higher mortality with recurrences occurring within 12 months of the original resection. Twenty-three percent of their patients developed distant metastases. [2] Huang documented local recurrence in 57%, with 42% of recurrent cases showing tumor progression and eventually distant metastases. The five-year recurrence-free survival and metastasis-free survival were 41% and 90% respectively. [3] Lin documented a significant association between margin status and local recurrence-free survival. [6] Furthermore, as reported by Lin and Huang, tumor necrosis >10%, tumor size>5 cm,<75% of myxoid area and high mitotic counts >20/10 hpf were significant prognostic factors. [3],[6] More recently, Sanfilippo′s [7] review of 158 patients documented five-year overall survival at 77% and that size, histological grade and status of surgical margins were significant independent predictors of survival. While local recurrences were predicted by the quality of surgical margins, the distant metastases were observed based on histological grade. The authors further go on to suggest that the use of radiotherapy had only a limited value in reducing local recurrences. Considering the above facts, wide-margin surgical excision and possible re-excision of recurrent lesions is considered the mainstay of therapy for disease control; the role of adjuvant radiotherapy is yet to be clearly defined. References
Copyright 2011 - Journal of Cancer Research and Therapeutics The following images related to this document are available:Photo images[cr11043f3.jpg] [cr11043f4.jpg] [cr11043f2.jpg] [cr11043f1.jpg] |
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