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Radiation-induced undifferentiated high-grade pleomorphic sarcoma (malignant fibrous histiocytoma) of the mandible: Report of a case arising in the background of long-standing osteomyelitis with a review of the literature

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Abstract

We report a rare case of radiation-induced undifferentiated high-grade pleomorphic sarcoma (UPS) (malignant fibrous histiocytoma, MFH) in the right mandible of a 44-year-old woman. The patient had suffered from osteomyelitis of the same region of the mandible for several years, which was considered to be due to radiotherapy for a malignant lymphoma in her right neck 19 years before. The tumor appeared as an exophytic and invasive growth in the molar region of the mandible. Histopathologically, the tumor consisted of an interlacing proliferation of vimentin-immunopositive spindle-shaped fibroblastic cells with bizarre nuclei with high Ki-67 labeling scores, and tumor cells showed storiform patterns mixed with pleomorphic cells. Taking the history of radiation into consideration, we diagnosed the lesion as radiation-induced MFH/UPS. Including the present case, there have been only 14 documented cases of radiation-induced UPS in the jawbone, and this is the first UPS case arising in the follow-up period of long-standing osteomyelitis.

Introduction

Undifferentiated pleomorphic sarcoma (UPS), also known as malignant fibrous histiocytoma (MFH), pleomorphic type, is one of the most common soft tissue sarcomas of mesenchymal origin. It is histopathologically characterized by the interlacing or storiform proliferation of constituent anaplastic fibroblastic cells intermingled with bizarre multinucleated giant cells. UPS affects most of the soft tissue of extremities of adults in their later life, with a male predominance [8], [9]. It also arises in the bone, and such a bony counterpart is designated as undifferentiated high-grade pleomorphic sarcoma (also abbreviated as UPS in this report) in the new WHO classification [24]. One of the most established etiologic factors for UPS is ionizing radiation for cancers, especially for breast carcinoma, malignant lymphoma, cervical carcinoma, and brain tumors [10]. Radiotherapy has also been an important modality of treatment for head and neck neoplasms, including squamous cell carcinoma [6] and nasopharyngeal carcinoma (lymphoepithelial carcinoma) [30]. In 1989, the cumulative incidence of sarcomas including UPS following radiation therapy was reported to range from 0.03% to 0.3% [1], while higher incidences of 1 to 3% were reported in 1998 [8]. A similar tendency of rising incidences –from 0.06% to 0.17% between 1960 and 2010 – has been reported from a single institution [28]. The rise in the rate of incidence is likely attributable to successful radiotherapies for malignancies in recent years, which have resulted in prolonged survival but at the same time increased the risk of eventual postradiation malignancies [8], [22], [28]. Among radiation-induced sarcomas of the oral region, osteosarcoma of the jawbones seems to be most common, followed by UPS, chondrosarcoma, rhabdomyosarcoma, and leiomyosarcoma in soft parts as well as in bones [19], [20], [21], [22], [26], [28]. In terms of UPS of the jawbone, 13 previous cases have been documented in the literature [16], [17], [19], [20], [24], [26], [27], [28]. However, clinical details about the developing processes of those cases have not always been reported.

In this report, we describe a rare case of radiation-induced UPS in the mandible after a 7-month follow-up period of osteomyelitis, which was diagnosed 19 years after radiation treatment for cervical malignant lymphoma. This is the first case report of radiation-induced UPS secondarily to osteomyelitis of the jawbone. Based on our review of the literature, clinicopathological features of the radiation-induced UPS in the oral region are discussed.

Section snippets

Materials and methods

For histopathological examination, surgical specimens were fixed in formalin and decalcified with decalcified with Planck Rychlo's solution containing 8.5% hydrochloric acid and 5% formic acid. The jawbone specimen was frontally cut into 6-mm-thick slices (totally 13 slices), routinely processed, and embedded in paraffin. Serial 5-μm-thick sections were prepared from 14 cut surfaces form the 13 slices, and one set of them was stained with hematoxylin and eosin (HE), and the other sets were used

Case report

A 44-year-old woman was referred to the Department of Oral and Maxillofacial Surgery, Niigata University Hospital, complaining of swelling and pain in the right side of her lower jaw. Nineteen years earlier the patient had developed malignant lymphoma (non-Hodgkin) of her right neck and had received external irradiation (70 Gy) and combination chemotherapy (Table 1), which led to long-standing osteomyelitis. The patient had suffered from hypoesthesia in the right side of the lower lip to mentum

Discussion

Relative frequencies of sarcomas among patients treated with radiation therapy, which have been estimated to be between 0.03% and 1.0%, vary from institute to institute and are dependent on primary disease types [1], [20]. When restricted to nasopharyngeal carcinoma, the primary therapy for which is radiation, frequencies tend to increase in the range from 0.10% to 0.68% [4], [5], [15], [28], [30]. In 1948, when radiation therapy had been in use for treating both benign and malignant lesions

Acknowledgment

The authors wish to thank Professor Kiyohiro Sakae, Kagoshima University for his helpful discussion.

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