Background: Carcinosarcoma of the salivary gland is a rare aggressive malignant tumor, composed of a mixture of carcinomatous and sarcomatous components. The most common metastatic sites include the lungs, bones, and central nervous system. Case presentation: This report describes a rare case of carcinosarcoma of the parotid gland with an osteosarcoma as sarcomatous component in a 72-year-old man who had a history of low anterior resection for rectal cancer. Six months after parotidectomy, he presented abdominal pain as a symptom of abdominal metastasis by the sarcomatous component. At that time, the possibility of abdominal metastasis was overlooked because of the history of abdominal surgery. After several days of conservative treatment, emergency laparotomy was done. However, he died of acute respiratory distress syndrome. Conclusions: Awareness of the possibility of abdominal metastasis by salivary carcinosarcoma may help in managing patients with a history of abdominal surgery. Keywords: Carcinosarcoma, Parotid gland, Metastasis, Osteosarcoma, Abdominal cavity Background components of salivary gland carcinosarcomas include Carcinosarcoma, also called true malignant mixed squamous cell carcinoma and adenocarcinoma, whereas tumor, is an aggressive malignant neoplasm of the saliv- malignant mesenchymal components include chondro- ary gland. It is an extremely rare malignancy, comprising sarcoma, fibrosarcoma, leiomyosarcoma, osteosarcoma, 0.04 to 0.16% of all salivary gland tumors and 0.4% of all and liposarcoma, in that order of frequency . The malignant salivary gland neoplasms. The mean age at most common metastatic sites include the lungs, bones, presentation is in the sixth to seventh decade of life, but and central nervous system [2–4]. can range from 14 to 87 years [1–3]. Although de novo To date, 10 cases of carcinosarcoma of salivary glands carcinosarcomas can occur in the salivary gland, many with osteosarcomatous element have been published, of patients have a history of long standing or recurrent which only lung metastasis have been found [7–16]. We pleomorphic adenomas, a condition described as carci- describe a case of carcinosarcoma arising in the parotid nosarcoma ex pleomorphic adenoma [4–6]. Most of gland with an osteosarcomatous component which me- these lesions arise in the major salivary glands, with two tastasized to abdominal cavity; to our knowledge, this is thirds in the parotid glands . Patients typically present the first patient so described. with a rapidly growing mass. These tumors are composed of a mixture of distinct Case presentation carcinomatous and sarcomatous components with either A 72-year-old man presented with a mass in the right component capable of metastasis. Malignant epithelial parotid area that had become rapidly enlarged for 2 months. His medical history included a subtotal gastrec- tomy for a gastric ulcer 20 years earlier and low anterior * Correspondence: firstname.lastname@example.org resection for moderately differentiated adenocarcinoma of Department of Pathology, Chungbuk National University Hosipital, 776, 1Sunhwan-ro, Seowon-gu, Cheongju 28644, South Korea the rectum (Stage T1N0) 1 year earlier. Preoperative © The Author(s). 2018 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Woo and Son World Journal of Surgical Oncology (2018) 16:103 Page 2 of 6 computed tomography (CT) showed a relatively Following surgery, the patient received postoperative well-defined heterogeneous enhancing solid lesion with radiation therapy. Six months later, he complained of calcification in the superficial lobe of the right parotid abdominal pain. Abdominal CT showed a distension of gland, with no indications of metastasis to the regional small bowel with luminal narrowing and localized high lymph nodes (Fig. 1). density material in the abdominal cavity (Fig. 4). The Fine-needle aspiration specimen showed that the lesion was regarded as mechanical obstruction with tumor contained many clusters of malignant epithelial postoperative adhesion because the patient had under- cells and scattered atypical spindle cells on a necrotic gone previous abdominal surgery for rectal adenocarcin- background. Under suspicion of a malignant tumor, the oma. After conservative management for several days, patient underwent total parotidectomy. Gross examin- he experienced severe abdominal pain and an increased ation of the specimen revealed a multifocal, ill-defined, heart rate, and an emergency laparotomy was performed. grayish-white, and heterogeneous solid tumor, accom- Multiple hard calcified masses were observed in the panied by calcification and measuring 3 × 2.5 cm. Micro- abdominal walls, omentum, and mesentery, and mass scopically, the tumor was composed of two malignant excision with small bowel resection was done. On gross components, carcinoma (Fig. 2a, b) and sarcoma examination, bulky, gritty, and hemorrhagic mass (Fig. 2c), with multifocal invasion (> 1.5 mm) of the adhered to the mesentery was identified (Fig. 5). Micro- capsule and adjacent tissues on a background of scopically, the tumors were identified as osteogenic sar- pre-existing pleomorphic adenoma (Fig. 2d). The carcin- comas, with histologic features identical to those of the oma component of the tumor consisted of squamous osteosarcomatous component of the carcinosarcoma of cell carcinoma and poorly differentiated adenocarcin- the parotid gland (Fig. 6a). The tumor cells were positive oma, whereas the sarcoma component consisted mainly for vimentin on immunohistochemical staining (Fig. 6b). of osteosarcoma, characterized by neoplastic bone and Because of its rapid development over 6 months and no severe cellular anaplasia. Many mitotic figures and nec- history of osteosarcoma at any sites, we concluded that rotic foci were observed. The external resection margin the abdominal osteosarcoma was metastatic from the showed tumor involvement. Immunohistochemical carcinosarcoma of the parotid gland. The patient was staining showed that the carcinoma cells were positive postoperatively admitted to the intensive care unit and for cytokeratin AE1/AE3 (Fig. 3a) and the osteosarcoma died of acute respiratory distress syndrome (ARDS) component was diffusely positive for vimentin (Fig. 3b). caused by aspiration pneumonia. Both components showed some degree of nuclear im- munoreactivity for p53 (Fig. 3c, d). Discussion and conclusions Malignant mixed tumors of the salivary gland can describe three distinct histologic entities: carcinoma ex pleomorphic adenoma (the most common), metastasiz- ing mixed tumor, and carcinosarcoma . More than 99% of these tumors arise from pleomorphic adenomas, with only 0.2% being primary carcinosarcomas or true malignant mixed tumors . Two hypotheses have been advanced to explain the histogenesis of carcinosar- coma. The convergence hypothesis suggests that these tumors are multiclonal and are derived from two or more types of mesenchymal and epithelial stem cells. The divergence hypothesis suggests that these tumors are monoclonal, with a single totipotent stem cell differ- entiating into epithelial and mesenchymal cells . Surgery followed by radiation therapy may aid in the treatment of salivary carcinosarcoma. Despite treatment, over half of these patients die of local recurrence and/or metastasis and the average survival of these patients is 3.6 years [2, 7]. Only 10 cases of carcinosarcoma with osteosarcoma as the sarcomatous component have been reported. The clinicopathological features and outcomes of the re- Fig. 1 A heterogeneous enhancing solid lesion with calcification (arrow) ported cases are detailed in Table 1. Our reviews exhib- was identified in the superficial lobe of right parotid gland on neck CT ited a male predominance (72.7%) with an age range of Woo and Son World Journal of Surgical Oncology (2018) 16:103 Page 3 of 6 Fig. 2 a The tumor cells showed features of squamous cell carcinoma, which were composed of abundant pink cytoplasm, moderate atypia, and focal keratinization (magnification, × 100). b The poorly differentiated adenocarcinoma components were consisted of solid nests of poorly differentiated tumor cells with ovoid nuclei and prominent nucleoli (magnification, × 100). c The sarcoma component consisted mainly of osteosarcoma, characterized by neoplastic bone and severe cellular anaplasia (magnification, × 100). d In pleomorphic adenoma component, the tubular and acinar structures formed by epithelial cells were compressed by abundant hyalinized eosinophilic matrix (magnification, × 100) 35 to 83 years (median, 64 years). All cases had an obstruction with high density material in the abdominal adenocarcinoma as the carcinomatous component, and cavity found on CT were regarded as an effect of his three cases also showed squamous cell carcinoma ele- abdominal surgery for rectal adenocarcinoma 1 year ments. The sarcomatous components other than osteo- earlier. Therefore, the management was only conserva- sarcoma were chondrosarcomas, rhabdomyosarcomas, tive, without surgery, and the possibility of metastasis and fibrosarcomas. Of the 11 cases, 8 cases occurred in from the salivary malignancy was overlooked. Later, the parotid gland. All patients underwent surgical resec- rapid growth of the metastatic tumor resulted in an tion and 6 of them received radiotherapy and/or chemo- emergency laparotomy. Abdominal metastases of the therapy. Distant metastasis occurred in five cases, of mesentery, omentum, and peritoneum were observed. which four cases involved the lung. In the present case, The patient experienced postoperative complications the sarcomatous component was an osteosarcoma, and and died of ARDS. the carcinomatous components were adenocarcinoma We described a case of salivary gland carcinosarcoma and squamous cell carcinoma. The tumor metastasized harboring an osteosarcomatous component with abdom- to the abdominal cavity by the osteosarcomatous com- inal metastasis consisted entirely of the sarcomatous ponent alone, which has not been reported to date. element. Awareness of the possibility of abdominal me- The present patient underwent surgery and adjuvant tastasis by the sarcomatous component of salivary carci- radiotherapy. After 6 months, he complained of abdom- nosarcoma may help in managing patients with a history inal pain. At that time, the signs of small bowel of abdominal surgery. Woo and Son World Journal of Surgical Oncology (2018) 16:103 Page 4 of 6 Fig. 3 Immunohistochemistry showed that carcinomatous components were positive for cytokeratin AE1/AE3 (a) and sarcomatous components were positive for vimentin (b). Both components showed some degree of nuclear immunoreactivity for p53 (c carcinoma component; d sarcoma component) (magnification, all × 100) Fig. 4 A localized high density material (arrow) in the abdominal Fig. 5 Bulky, gritty, and hemorrhagic masses (arrow) adhered to the cavity was presented in abdominal CT small intestinal mesentery were observed Woo and Son World Journal of Surgical Oncology (2018) 16:103 Page 5 of 6 Fig. 6 a Microscopically, the abdominal mass showed the osteosarcoma (magnification, × 100). b The immunostaining for vimentin was diffuse positive (magnification, × 100) Table 1 Review of previously reported cases of carcinosarcoma of the salivary glands with osteosarcoma components Reference Age (years)/sex Location Histologic type Histologic type Metastasis/ Histologic type of Treatment Outcome of carcinoma of sarcoma site metastatic tumor Garner et al. 57/F Parotid gland Adenocarcinoma Osteosarcoma, No (−) Left parotidectomy Local recurrence 1989  chondrosarcoma and radical neck at 6 weeks and dissection died Yamashita et al. 52/M Submandibular Adenocarcinoma, Osteosarcoma, No (−) Excision and right No recurrence 1990  gland squamous cell chondrosarcoma, modified neck for 5 months carcinoma fibrosarcoma dissection with chemotherapy Bleiweiss et al. 64/M Submandibular Adenocarcinoma Osteosarcoma No (−) Partial Local recurrence 1992  gland mandibulectomy at 4 months with radical neck dissection de la Torre et al. 83/F Parotid gland Adenocarcinoma Osteosarcoma, Yes/lung NR Wide excision Local recurrence 1995  chondrosarcoma and lung metastasis, died after 1 year Carson et al. 51/F Parotid gland Adenocarcinoma Osteosarcoma No (−) Total Local recurrence 1995  parotidectomy at 7 months with chemotherapy and died at 9 months Gogas et al. 77/M Submandibular Salivary duct Osteosarcoma. Yes/lung NR Wide excision with Lung metastasis 1999  gland carcinoma chondrosarcoma, radiotherapy and at 3 months rhabdomyosarcoma chemotherapy Sironi et al. 77/M Parotid gland Squamous cell Osteosarcoma Yes/NR NR Right total Metastasis at 2000  carcinoma, parotidectomy 3 months adenocarcinoma, with radical neck dissection Mardi et al. 59/M Parotid gland Adenocarcinoma Osteosarcoma, NA NA NA NA 2004  chondrosarcoma Jang et al. 67/M Parotid gland Adenocarcinoma Osteosarcoma Yes/lung Osteosarcoma Left parotidectomy Lung metastasis 2011  with radiotherapy at 5 months Jha et al. 35/M Parotid gland Adenocarcinoma Osteosarcoma No (−) Total radical No recurrence 2017  parotidectomy for 12 months with radiotherapy Present case 72/M Parotid gland Adenocarcinoma, Osteosarcoma Yes/abdominal Osteosarcoma Total Metastasis at squamous cell cavity parotidectomy 6 months and carcinoma with radiotherapy died NA, not available, NR not reported Woo and Son World Journal of Surgical Oncology (2018) 16:103 Page 6 of 6 Abbreviations 15. Jang SM, Jun YJ, Han H, Jang KS, Paik SS. Parotid gland carcinosarcoma with CT: Computed tomography; ARDS: Acute respiratory distress syndrome osteosarcoma as a sarcomatous component: a case report with fine needle aspiration cytologic findings. Korean J Pathol. 2011;45:412–6. Availability of data and materials 16. Jha V, Kolte S, Goyal S, Mandal AK. Osteosarcoma arising in carcinosarcoma All findings of this case report are based on diagnostic examinations de novo parotid gland in a young man: an unusual case with review of performed during patient hospitalization. The publication of these data was literature. J Clin Diagn Res. 2017;11:ED08–10. authorized by Chungbuk National University Hospital. Data sharing is not 17. Owaki S, Kitano H, Hanada M, Asada Y, Sugihara H, Moritani S, Banba M. applicable to this article as no datasets were generated or analyzed in the Carcinosarcoma of the submandibular gland: an autopsy case. Auris Nasus present report. Larynx. 2003;30:439–42. 18. 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Published: Jun 2, 2018