IJHNS Fibrosarcoma developing in the Parotid10.5005/jp-journals-10001-1233 Gland: An Unusual Presentation CASE REPORT Fibrosarcoma developing in the Parotid Gland: An Unusual Presentation 1Shagufta Qadri, 2Nishat Afroz, 3Divya Rabindranath, 4Shakeba Qadri ABSTRACT soft-part sarcoma.2 Fibrosarcoma of the head and neck Primary sarcomas of the salivary glands occur rarely with few represents 5% of all fibrosarcomas. Microscopically, cases having been reported. Fibrosarcoma is a rare type of fibrosarcoma shows an intense proliferation of spindle primary sarcoma with a poor prognosis and low life expectancy. shaped cells, varying a little in their size and shape Microscopically, these tumors resemble their counterparts and arranged in parallel bands which partly cross each encountered in other areas. They can be easily confused with 3 spindle cell (sarcomatoid) carcinomas, myoepithelial carcino- other at acute angles (herring bone pattern). They show mas or malignant melanomas. We present an unusual case of a considerable morphological and histopathological a primary fibrosarcoma that arose in the right parotid gland of overlapping with spindle cell (sarcomatoid) carcinomas, a 50 years male, who presented with a large nodular mass in myoepithelial carcinomas and malignant melanomas. the right preauricular region. Computed tomography (CT) scan revealed a tumor arising from the right parotid gland. A near total Hence, immunohistochemistry is mandatory to diffe- parotidectomy was performed and the histopathology report rentiate fibrosarcoma from the above-mentioned tumors. showed neoplastic spindle cells predominantly arranged in a Malignant spindle cell tumors within the salivary ‘herring bone’ pattern. On immunohistochemistry, the tumor gland is a diagnostic challenge for the pathologist. was positive for vimentin and was negative for pancytokeratin and S-100, which lead to a firm diagnosis of fibrosarcoma. Through this report, the authors are elucidating the existence of fibrosarcoma in the parotid gland and simul- Keywords: Fibrosarcoma, Parotid gland, Salivary gland, Sarcoma. taneously emphasizing the need for a high index of suspi- cion to rule out other lesions with a similar appearance in How to cite this article: Qadri S, Afroz N, Rabindranath D, Qadri S. Fibrosarcoma developing in the Parotid Gland: An order to reach the correct diagnosis and form an effective Unusual Presentation. Int J Head Neck Surg 2015;6(2):99-102. plan of management. Nil Source of support: CASE REPORT Conflict of interest: None A 50 years Indian male presented to the Outpatient Clinic of the Department of Surgery with a large mass INTRODUCTION in the right preauricular region for the past 8 months, Parotid malignancies represent 1 to 3% of all head and which progressively increased from a small nodule to the 1 neck cancers, while sarcomas comprise only 0.3 to 1.5% of present size. No history of ionizing radiation exposure 2 all major salivary gland neoplasms. The most frequently to the head and neck area could be elicited. The patient described sarcomas of the parotid gland are rhabdomyo- consulted a surgeon 5 months previously when the size of sarcoma and malignant fibrous histiocytoma (MFH), the swelling was 3 × 4 cm. Fine needle aspiration cytology although less commonly a variety of other tumors can (FNAC) was performed and the smears were suggestive occur, including leiomyosarcoma, malignant peripheral of spindle cell neoplasm. Subsequently, excisional biopsy nerve sheath tumor (MPNST), angiosarcoma, osteo- was advised, but the patient refused to undergo surgery. sarcoma, fibrosarcoma, liposarcoma, hemangiopericy- Local examination revealed a 9 × 8 cm, fungating mass in toma, Kaposi sarcoma synovial sarcoma, and alveolar the right preauricular region with an irregular, nodular surface, slightly tender to touch, firm to hard in consisten- cy, immobile and fixed to the underlying tissue. Cervical 1Senior Resident, 2Professor, 3,4Resident lymphadenopathy was absent and no sign of facial nerve 1-3Department of Pathology, Jawaharlal Nehru Medical College involvement was seen. Fine needle aspiration cytology Aligarh, Uttar Pradesh, India smears were reviewed and cytology revealed a highly cel- 4Department of Maxillofacial Surgery, Sir Ziauddin Dental College lular smear with relatively monomorphic spindle-shaped Aligarh, Uttar Pradesh, India cells with hyperchromatic, fusiform and tapering nuclei Corresponding Author: Shagufta Qadri, Senior Resident with scanty, elongated and poorly delimited cytoplasm Department of Pathology, Jawaharlal Nehru Medical College enmeshed with stromal element (Fig. 1). Aligarh, Uttar Pradesh, India, Phone: 919897532879, e-mail: Computed tomography (CT) scan revealed a hypoatte- [email protected] nuated mass measuring 9 × 8 cm, with ill-defined margins International Journal of Head and Neck Surgery, April-June 2015;6(2):99-102 99 Shagufta Qadri et al arising from right parotid gland along with the involve- carcinosarcoma (malignant mixed tumor) and MPNST ment of bone (Fig. 2). respectively, which led to the diagnosis of fibrosarcoma. Patient underwent a near total parotidectomy sparing Postoperative period was uneventful. Currently, the the facial nerve and the resected specimen was submitted patient is in follow-up. He is doing well 15 months after for histopathological examination. Gross examination surgery. showed an unencapsulated, nodular mass partially covered with skin showing surface ulceration and funga- DISCUSSION tion. Areas of hemorrhage and necrosis were also noted Tumors of salivary gland originate a diverse group of (Fig. 3). Cut-section revealed greyish-white, firm nodular neoplasms, and they may present significant challenges areas with accompanying foci of hemorrhage and necrosis. in the diagnosis as well as in their management. These Extensive histopathological examination of the tumor, tumors are rare with an overall incidence in the Western revealed sweeping fascicles of neoplastic spindle cells world of approximately 2.5 to 3.0 cases per 100,000 per with moderate degree of pleomorphism that exhibited year.5 Malignant salivary gland tumors account for 0.5% frequent ‘herring bone’ pattern and entrapment of few of all malignancies and approximately 3 to 5% of all head non-neoplastic acini and ducts within the tumor (Fig. 4). and neck cancers.5,6 The most common age of presentation The tumor also revealed high mitotic activity (18/10 hpf) for malignant tumors is in the sixth or seventh decade of with frequent atypical forms (Fig. 5). Immunohisto- life.7, 8 Ionizing radiation has been suggested as a cause chemical analysis showed only vimentin positivity (Fig. 6) of salivary gland cancer but the etiology of most salivary while pancytokeratin (Fig. 7) and S-100 (Fig. 8) were gland cancers is still unknown.6, 7,9,10 Certain occupations negative, that helped in excluding the possibility of are thought to be associated with an increased risk for Fig. 1: Fine needle aspiration cytology smear showing high Fig. 2: Computed tomography showing an ill-defined mass cellularity with monomorphic spindle cell population (H&E: 400×) protruding from the right parotid gland Fig. 3: Gross appearance demonstrating an irregular, nodular, Fig. 4: Sweeping fascicles of mature spindle cells exhibiting fungating mass, partially covered with skin with surface frequent herringbone pattern and normal acini and ducts entrapped ulceration within the tumor (H&E: 100×) 100 IJHNS Fibrosarcoma developing in the Parotid Gland: An Unusual Presentation Fig. 5: Spindle-shaped cells with hyperchromatic, fusiform Fig. 6: Vimentin immunostaining showing cytoplasmic positivity nuclei with scanty, fibrillary cytoplasm and a high mitotic activity in the spindle cells (vimentin: 400×) (H&E: 400×) Fig. 7: Tumor cells showing negative immunostaining for Fig. 8: S100 negativity of the neoplastic spindle cells pancytokeratin (CK: 100×) (S100: 100×) salivary gland cancers including rubber products manu- an intermediate grade of prognosis and rare metastasis, facturing, asbestos mining, plumbing, and some types whereas adult fibrosarcoma is a diagnosis of exclusion of wood works.7 The majority of the malignant tumors with a profound metastasizing potential and grave of salivary glands are epithelial in origin, whereas non prognosis. Grossly, adult fibrosarcoma appears as a well epithelial tumors constitute less than 5%, of which nearly circumscribed mass with firm white to tan cut surface. 90% are benign.11 Microscopy shows compact spindle cells arranged in Primary sarcomas of the parotid are an even rarer short fascicles which split and merge, giving the appea- group of malignant salivary gland tumors arising from rance known as ‘herring bone’ pattern in a background the mesenchymal component of salivary gland and com- of variable collagenous stroma. The individual cells are prises only 0.3 to 1.5% of all salivary gland neoplasms.2 spindle to polygonal in shape with fibrillary eosinophilic The two most common type of primary sarcomas of cytoplasm and plump hyperchromatic nuclei. the salivary gland have been reported to be malignant Certain conditions should be ruled out before reaching schwannoma and fibrosarcoma.4 Involvement of the a diagnosis of fibrosarcoma of the parotid. Firstly, in order parotid salivary glands are more common than the sub- to rule out the possibility of metastatic sarcoma, four basic mandibular glands.4 The mean age of occurrence has criteria should be met are as follows: been reported to be 42 years for men and 38