Carcinosarcoma of the parotid gland: A case report and short review of literature. R Patnayak, A Jena, G Raju, S Uppin, I Satish Rao, C Sundaram

Transcription

1 ISPUB.COM The Internet Journal of Oncology Volume 5 Number 1 Carcinosarcoma of the parotid gland: A case report and short review of literature R Patnayak, A Jena, G Raju, S Uppin, I Satish Rao, C Sundaram Citation R Patnayak, A Jena, G Raju, S Uppin, I Satish Rao, C Sundaram. Carcinosarcoma of the parotid gland: A case report and short review of literature. The Internet Journal of Oncology Volume 5 Number 1. Abstract Background: Carcinosarcomas are very rare true malignant mixed tumors of salivary gland. Majority is seen in the parotid glands. Chondrosarcoma and osteosarcoma elements are the most common sarcomatous element whereas moderately or a poorly differentiated ductal adenocarcinoma is the most common carcinomatous element seen. Case details: We report a parotid lesion in a 47-year-old female, composed of ductal type adenocarcinoma admixed with chondrosarcomatous and malignant spindle cell elements. Conclusion: The case is of interest because of its rarity and the initial diagnosis of benign tumor by Fine needle aspiration cytology. This case is a carcinosarcoma, which combines both features of ductal type adenocarcinoma and malignant spindle cell tumors along with chondrosarcomatous elements. In addition, a short review of literature is presented. INTRODUCTION Malignant mixed tumour of salivary gland comprises 3 different clinical entities-carcinoma arising in benign mixed tumour (carcinoma ex pleomorphic adenoma), carcinosarcoma and metastasizing mixed malignant tumour. It constitutes approximately 12% of malignant salivary gland tumors, 6.2% of all mixed tumors and 3.6% of all salivary gland neoplasms 1. Carcinosarcoma is a very rare true malignant mixed tumor of salivary gland, which is an aggressive, high-grade neoplasm with multiple episodes of recurrences and metastasis. 1 Computerized tomography (CT) scan of the swelling showed enhancing hyper-dense lesion in parotid gland with popcorn calcification (Fig.1). CASE DETAILS A 47-year-old female presented with history of swelling in right parotid area for 25 years, which was increasing in size for past 1 month. Previous history of operation was not available. The swelling was 5.0x5.0 cm, hard, immobile and slightly tender. The patient had facial palsy at presentation. 1 of 6

2 Figure 1 Figure 1: CECT showing enhancing hyperdense lesion in the right parotid gland with popcorn calcification Figure 2 Figure 2: An area showing chondromatous differentiation. (H&E; X100) Fine needle aspiration cytology (FNAC) of the parotid gland done previously in another institution was reported as pleomorphic adenoma. Right total parotidectomy was done and the tumor was found to be fixed posteriorly to mastoid and to infiltrate the facial nerve trunk and was resected in piecemeal. The other component intermingled with these was the malignant ductal component (Fig.3, 4). Figure 3 Figure 3: Admixture of glandular and mesenchymal component. (H&E; X100) PATHOLOGY The tumor was received in multiple soft tissue bits, measuring 7.5x7.0x4.0 cm and weighing 40 grams. The external surface of the lesion was nodular and at places well circumscribed. The cut surface showed normal salivary gland along with a gray white lesion, measuring 3.5x3.0x3.0 cm, which had a gritty sensation while taking sections. Regional lymph nodes were not involved. Multiple histological sections showed a lesion comprised of a bimodal population of both epithelial and mesenchymal component. The predominant mesenchymal component was the malignant spindle cells, arranged in interlacing fascicles along with a chondrosarcomatous component (Fig.2). 2 of 6

3 Figure 4 Figure 4: Higher magnification of Figure 3, showing malignant glandular and mesenchymal component. (H&E; X200) carcinosarcoma 11, 14, 22. In the literature, the most common epithelial components are ductal adenocarcinoma or squamous cell carcinoma, whereas the most common mesenchymal components are chondrosarcoma. Other sarcomas that have been reported include fibrosarcoma, leiomyosarcoma, osteosarcoma, malignant fibrous histiocytoma and rarely liposarcoma, rhabdomyosarcoma or the combination of these sarcomas and also myoepithelial malignant proliferation 5, 6, 8, 9, 12, 13, 18, 19, 20, 29. Also recently the combination of epithelial- myoepithelial carcinoma and pleomorphic sarcoma and large-cell neuroendocrine carcinoma with rhabdomyosarcoma has been described 17, 18. (Table-1) Figure 5 Table 1: Carcinosarcoma of Salivary Glands: A Literature Review Both the components showed pleomorphism, hyperchromasia of nuclei and presence of mitotic figures. However no areas of necrosis identified. The lesion showed entrapped nerve twigs and was also seen infiltrating the normal salivary gland. Multiple sections showed no evidence of pleomorphic adenoma. DISCUSSION Kirklin et al of the Mayo Clinic first described mixed carcinoma and sarcoma of the parotid gland in King in 1967 first used the term true malignant mixed tumor (carcinosarcoma), which means a tumor consisting of both carcinomatous and sarcomatous elements 3. There are 3 distinct histological types of malignant mixed tumor: carcinoma ex pleomorphic adenoma; metastasizing mixed tumors; and carcinosarcomas, which are true malignant mixed tumors. Carcinosarcoma of the salivary gland is an extremely rare tumor 4, 5, 6, 14, 21, 29. By definition, it is a biphasic tumor composed of malignant epithelial and malignant mesenchymal elements. In most tumors epithelial and mesenchymal components are admixed with each other, as seen in the present case. Gnepp 6 summarized 43 cases of carcinosarcoma of the salivary glands published in the literature. The majority of these tumors (33%) are from parotid glands followed by submandibular glands (19%) and palate (14%) 6. There is no sex predominance. The mean age at presentation is 58 years with a range of years 27. Many cases are seen to arise in a pre-existing benign mixed tumor 6, 14, 12, 25. Few articles have described the cytologic features of In our case, the malignant mesenchymal components are undifferentiated spindle cell elements and chondrosarcomatous elements and these are intermingled with malignant ductal component. The differential diagnosis always includes benign mixed tumor and primary versus metastatic sarcomas. Multiple sections taken in the current tumor however did not show any evidence of pleomorphic adenoma. The histogenesis and pathogenesis true malignant mixed 3 of 6

4 tumors of the salivary glands are controversial. According to some authors, carcinosarcoma and pleomorphic adenoma of the salivary gland may share a common precursor, possibly a myoepithelial cell 6, 7, 23, 24. On the other hand, it has been shown that in some carcinosarcomas both components arise de novo. 6, 7, 9, 10. Immunohistochemically cytokeratin and EMA are epithelial markers whereas vimentin and s-100 are sarcomatous mesenchymal markers 12, 30. Kwon and Gu, based on their study where both components did not show any immunoreactivity for smooth muscle actin, a marker for myoepithelial cells hypothesized that the primitive mesenchymal cells may have given rise to different kinds of sarcoma 12. Gotte K et al also favored the hypothesis of common stem cell monoclonal origin of carcinosarcoma that could be the myoepithelial cell and an inactivated tumor suppressor gene on chromosome 17 other than p Carcinosarcoma is an aggressive, high-grade neoplasm and requires aggressive therapy, including radical surgery, with and without radiation therapy and chemotherapy. Carcinosarcoma of the parotid arising after irradiation to the resection site of a pleomorphic adenoma is also described 22, 26. Recurrent disease develops in approximately two thirds of patients and metastases in about half 15. The routes of metastases are hematogenous and lymphatic. Lung is described as the most common site of metastases. Cervical and hilar lymph node metastases are also common 6, 8. There is a good correlation between stage and local extension of the tumor and prognosis. Facial nerve palsy and grade of malignancy are important prognostic factors 16. CONCLUSION The case reported in this article is a carcinosarcoma, which combines both features of ductal type adenocarcinoma and malignant spindle cell tumors along with chondrosarcomatous elements. It is reported because of its rarity. Post-operatively the patient received radiotherapy and is doing well after a follow-up of two years. ACKNOWLEDGMENTS The authors wish to thank senior technician Mrs. Nirmala and Hema for their help. References 1. Gnepp DR., El-Mofty SK. Salivary glands. In: Damjanov I, Linder J, Eds Anderson's pathology.10th ed. Missouri: Mosby, 1996: Kirklin JW, McDonald JR, Harrington SW, New GB. Parotid tumors: histopathology, clinical behavior, and end results. Surg Gynecol Obstet 1951; 92: King O Jr. Carcinosarcoma of accessory salivary gland: first report of a case. Oral Surg Oral Med Oral Pathol 1967; 23: Ellis GL, Auclair PL. Tumors of the Salivary Glands. Washington, DC: Armed Forces Institute of Pathology; Atlas of Tumor Pathology; 3rd series, fascicle Alvarez-Canas C, Rodilla IG True malignant mixed tumor (carcinosarcoma) of the parotid gland. Report of a case with Immunohistochemical study. Oral Surg Oral Med Oral Pathol Oral RadiolEndod.1996Apr; 81(4): Gnepp DR. Malignant mixed tumors of the salivary glands: a review. Pathol Ann 1993; 28: Bleiweiss IJ, Huvos AG, Lara J, Strong EW. Carcinosarcoma of the submandibular salivary gland: immunohistochemical findings. Cancer 1992; 69: Stephen J, Batsakis JG, Luna MA, von der Heyden U, Byers RM. True malignant mixed tumors (carcinosarcoma) of salivary glands. Oral Surg Oral Med Oral Pathol1986; 61: Grenko RT, Tytor M, Boeryd B. Giant-cell tumor of the salivary gland with associated carcinosarcoma. Histopathology 1993; 23: Latkovich P, Johnson RL. Carcinosarcoma of the parotid gland: report of a case with cytohistologic and immunohistological findings. Arch Pathol Lab Med 1998; 122: de la Torre M, Larsson E. Fine-needle aspiration cytology of carcinosarcoma of the parotid gland: cytohistological and immunohistochemical findings. Diagn Cytopathol 1995; 12: Meeae Y. Kwon, Mai Gu. True Malignant Mixed Tumor (Carcinosarcoma) of Parotid Gland with Unusual Mesenchymal Component- A Case Report and Review of the Literature. Archives of Pathology and Laboratory Medicine: Vol. 125, No. 6, pp Gandour-Edwards RF, Donald PJ, Vogt PJ, Munn R, Min KW. Carcinosarcoma (malignant mixed tumor) of the parotid: report of a case with a pure rhabdomyosarcoma component. Head Neck 1994; 16: Kim T, Yoon GS, Kim O, Gong GG. Fine needle aspiration diagnosis of malignant mixed tumor (carcinosarcoma) arising in pleomorphic adenoma of the salivary gland: a case report. Acta Cytol 1998; 42: LiVolsi VA, et al. Malignant mixed tumors arising in salivary glands. I. Carcinomas arising in benign mixed tumors: a clinicopathologic study. Cancer. 1977; 39: Peterson D. et al. Malignant parotid tumors in 110 consecutive patients: treatment results and prognosis. Laryngoscope 1992; Manuel S, Mathews A, Chandramohan K, Pandey M. Carcinosarcoma of the parotid gland with epithelialmyoepithelial carcinoma and pleomorphic sarcoma components. Br J Oral Maxillofac Surg Dec; 40(6): Ueo T, Kaku N, Kashima K, Daa T, Kondo Y, Yoshida K, Suzuki M, Yokoyama S. Carcinosarcoma of the parotid gland: an unusual case with large-cell neuroendocrine carcinoma and rhabdomyosarcoma. APMIS Jun; 113(6): Vajda K, Bogi I True malignant mixed tumors of parotid gland. Orv Heti 1995; 136: Mardi K, Sharma J. True malignant mixed tumor (carcinosarcoma) of parotid gland: a case report. Indian J Pathol Microbiol Jan; 47(1): Gogas J, Markopoulos C, Karydakis V, Gogas G, Delladetsima J Carcinosarcoma of the submandibular 4 of 6

5 salivary gland. Eur J Surg Oncol.1999 Jun; 25(3): Granger JK, Houn H-Y. Malignant mixed tumor (carcinosarcoma) of parotid gland diagnosed by fine-needle aspiration biopsy. Diagn Cytopathol 1991; 7: Carson HJ, Tojo DP, Chow JM, Hammadeh R, Raslan WF. Carcinosarcoma of salivary glands with unusual stromal components: report of two cases and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995; 79: Bocklage T, Feddersen R. Unusual mesenchymal and mixed tumors of the salivary gland: an immunohistochemical and flow cytometric analysis of three cases. Arch Pathol Lab Med 1995; 119: Lopez JI, Ballestin C, Garcia-Prats MD, De Agustin P. Carcinosarcoma of the parotid gland: immunohistochemical study of a case. Histopathology 1994; 25: Spraggs PDR, Rose DSC, Grant HR, Gallimore AP. Post-irradiation carcinosarcoma of the parotid gland. J Laryngol Otol 1994; 108: Gnepp DR (2001) Diagnostic Surgical Pathology of the Head and Neck.W.B. Saunders: Philadelphia 28. Gotte K, Riedel F, Coy JF, Spahn V, Hormann K.Salivary gland carcinosarcoma: immunohistochemical, molecular genetic and electron microscopic findings. Oral Oncol Jul; 36(4): Sironi M, Isimbaldi G, Claren R, Delpiano C, Di Nuovo F, Spinelli M. Carcinosarcoma of the parotid gland: cytological, clinicopathological and immunohistochemical study of a case. Pathol Res Pract. 2000; 196(7): Yamashita T, Kameda N, Katayama K, Hiruta N, Nakada M, Takeda Y True malignant mixed tumor of the submandibular gland. Acta Pathol Jpn Feb; 40(2): of 6

6 Author Information Rashmi Patnayak, M.D. Amitabh Jena, M.S. G.S.N. Raju, M.S., M.Ch. Shantveer G. Uppin, MD I. Satish Rao, MD C. Sundaram, MD 6 of 6