Report of a rare case of carcinosarcoma of the maxillary sinus with sternal metastasis

June 8, 2014
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Abstract

Carcinosarcoma is a highly aggressive and infiltrative tumor. A finding of this tumor in a paranasal sinus is exceedingly rare. We describe the case of a 61-year-old man who presented with a mass on the left side of his face. The mass was excised via a total maxillectomy with a modified radical neck dissection. Histologic analysis identified a mixture of carcinomatous and sarcomatous components. Within 1 month of surgery, the patient developed a sternal metastasis, and he died within a short period of time. The aggressive nature of this tumor and its metastases demand early diagnosis and prompt treatment.


Introduction

Carcinosarcoma is a rare malignant mixed tumor that contains both carcinomatous and sarcomatous elements. It can occur in the epithelium of any part of the body. The carcinomatous component is usually an adenocarcinoma. The sarcomatous component is made up of either nondescript undifferentiated spindle cells or pleomorphic cells that are capable of mesenchymal differentiation into bone (osteoid sarcoma), cartilage (chondroid sarcoma), or muscle (rhabdoid sarcoma).1

Carcinosarcoma of the sinonasal tract is extremely rare.1 We describe a case of carcinosarcoma of the left maxillary sinus that later metastasized to the sternum.


Case report

A 61-year-old Malaysian man was referred to our ENT department by the dental service for evaluation of a swelling on the left side of his face. While the swelling had been present for more than 10 years, its size had rapidly increased over the previous 6 months. The patient also reported associated loss of weight and appetite. He had no complaints of diplopia, epistaxis, or nasal blockage. He did not smoke cigarettes or abuse alcohol.

On examination, the mass extended from below the left eye to the cheek; it measured 6 x 8 cm. The swelling was irregular in shape, nontender, and fixed to underlying structures. The overlying skin was intact. Anterior rhinoscopy showed a mass in the left nasal cavity. In the oral cavity, a fungating tumor was noted on the left half of the hard palate; it extended across the midline. Examination of the neck revealed multiple left-sided enlarged lymph nodes in the submandibular and submental areas. Findings on the ophthalmology examination were normal.

A biopsy sample obtained from the left antrum was reported as an epithelial tumor. Computed tomography (CT) obtained from the base of the skull to the neck revealed a heterogeneously enhancing 5.2 x 6.6 x 6.5-cm mass at the left maxillary sinus (figure 1). The mass extended into the left orbit, where it displaced the inferior rectus muscle and globe superiorly. The infratemporal fossa was involved. Inferiorly, the mass extended to the left alveolar ridge of the maxilla and the hard palate. The left maxillary sinus was expanded, and destruction of the anterior, lateral, and medial walls was noted. Destruction of the left zygomatic bone was also seen. The mass encroached upon the left nasal cavity and abutted the nasal septum. The lymph nodes at left submandibular and submental levels II and III were enlarged. Chest x-ray and abdominal ultrasonography did not find any evidence of distant metastasis.


Figure 1. CT shows the heterogeneously enhancing primary tumor in the left maxillary sinus. The mass extends into the left orbit, where it has displaced the inferior rectus muscle and globe.

The diagnosis was discussed with the patient, and he agreed to undergo a total maxillectomy. He requested preservation of his left eye. He was scheduled for surgery within 2 weeks, and a preparatory tracheostomy was performed. At surgery, a modified radical neck dissection followed by a total left maxillectomy was performed. A transfacial approach to the maxillary sinus was taken via a Weber-Fergusson incision. The maxillary tumor was removed en bloc. The anterior wall of the maxillary antrum had been invaded by the tumor mass. The orbital floor was eroded, but the inferior rectus muscle appeared to be intact. Inferiorly, the tumor had devoured the hard palate.

The histopathology report indicated that 9 of 18 lymph nodes showed evidence of metastasis. The primary tumor on the maxilla was confirmed to be a carcinosarcoma, as it was composed of a mixture of carcinomatous and sarcomatous components (figure 2). The carcinomatous component demonstrated an island of malignant cells with glandular differentiation. The sarcomatous component contained cells that resembled spindle-shaped cells. Immunostaining was positive for vimentin and cytokeratin and negative for desmin, smooth-muscle actin, factor VIII, CD34, sarcomeric actin, and c-kit.


Figure 2. Histopathology of the primary tumor cells shows the mixture of carcinomatous and sarcomatous components.

Within 1 month of surgery, the patient developed a new swelling, which was located over the anterior chest wall at the upper sternum (figure 3). The 10 x 12-cm mass was firm and fixed to underlying bone. It had grown rapidly. Findings on fine-needle aspiration cytology were consistent with metastatic carcinoma. CT of the thorax confirmed the presence of the mass at the sternal notch with erosion of the upper part of the manubrium (figure 4).


Figure 3. The metastatic mass is seen at the upper sternum.


Figure 4. CT shows the heterogeneous metastasis at the sternal notch. Note the erosion of the upper part of the manubrium.

The patient consulted an oncologist, and he was scheduled for chemotherapy followed by radiotherapy. However, his physical status quickly deteriorated, and he died before chemotherapy could be instituted.


Discussion

The term spindle cell carcinoma is used to describe a carcinoma with no definite mesenchymal differentiation.2 It is a biphasic, true malignant mixed tumor in which both the epithelial and stromal components are malignant.3 When the transitions between the two cell populations are obvious-that is, when there is a hybrid epithelial-mesenchymal cell type-the term sarcomatoid carcinoma is preferred.4

The sociodemographic characteristics of patients with carcinosarcoma are similar to those of patients with squamous cell carcinoma.4 Carcinosarcomas generally occur during the fifth and sixth decades of life, there is a strong male preponderance, and their incidence is closely associated with cigarette smoking and alcohol consumption. Another risk factor is a history of irradiation to the site. However, with the exception of his sex, our patient had none of these risk factors.

In the head and neck, the most common sites of primary carcinosarcomas are the larynx, particularly the glottis, and the oral cavity. Less common sites include the oropharynx, hypopharynx, nasal cavity, and sinuses. As mentioned, carcinosarcoma of the sinonasal tract is extremely rare.1 Our patient's tumor was located in the left maxillary sinus.

The histogenesis of carcinosarcoma is still controversial. No definitive evidence exists to show whether this tumor arises (1) from two distinct carcinoma and sarcoma clones or (2) from a single malignant clone that is capable of both epithelial and mesenchymal differentiation. Sometimes the transition between carcinomatous and sarcomatous components can be observed in a single patient, and some of the spindle cells are immunoreactive to the epithelial markers. This suggests that carcinosarcomas may develop from a totipotent cell that is capable of both epithelial and mesenchymal differentiation.2

Carcinosarcoma is the tumor appropriately described in the epithelial-mesenchymal transition (EMT) theory. EMT is a type of epithelial plasticity that is characterized by a long-lasting morphologic and molecular change in epithelial cells, which occurs as a result of transdifferentiation toward the mesenchymal type of cells.3 Through EMT, the malignant epithelial cells that transdifferentiate into myofibroblasts lose their malignant phenotype, but they still retain the desmoplastic stroma that is essential for tumor proliferation and metastasis. In general, carcinosarcomas, which very likely exemplify EMT, are highly infiltrative and metastasizing, and their behavior is similar to that of poorly differentiated carcinomas. Metastases of carcinosarcoma usually contain squamous cells or a combination of squamous cells and spindle cells; rarely are they made up of spindle cells only.5

Our thorough search of the literature found no previous report of a carcinosarcoma metastatic to bone. Our patient's sternal metastasis was composed of squamous cells.

The prognosis for patients with spindle cell carcinoma (or carcinosarcoma) is similar to that for patients with conventional squamous cell carcinoma. Survival depends largely on the location and stage of the tumor; patients with glottic spindle cell carcinoma have a relatively good prognosis, while those with a spindle cell carcinoma of the oral cavity or paranasal sinuses have a poor prognosis. Reported 5-year survival rates range between 63 and 94%; overall mortality is 30 to 34%.5

Wide surgical excision, alone or with radical neck dissection, is the most successful treatment for spindle cell carcinoma. Radiotherapy is generally considered to be less effective.5 Gale and Zidar reported a case of true carcinosarcoma of the maxillary sinus in a patient who died of a tumor recurrence despite intensive irradiation, chemotherapy, and total maxillectomy.6 At autopsy, the recurrent tumor was found to consist entirely of osteosarcoma cells. Their case suggests that radiation and chemotherapy are effective against the carcinomatous component but not the sarcomatous component.

In conclusion, even though squamous cell carcinoma accounts for most malignant tumors of the paranasal sinuses, we should always consider the possibility of a rare neoplasm such as carcinosarcoma because treatment modalities are different for the different types of tumor. Squamous cell carcinoma of the maxillary sinus might respond well to radio- and chemotherapy, but spindle cell carcinoma does not. Wide excision with radical neck dissection appears to be the best treatment option for patients with spindle cell carcinoma.

The length of time between presentation and treatment has an effect on prognosis; a delay in treatment could adversely affect the patient's outcome. Rapid growth and extensive local destruction are the prominent features of carcinosarcoma, so early diagnosis and prompt institution of aggressive treatment are important.


Acknowledgment

We thank Dr. Faizah bt. Ahmad of the Pathology Department at the General Hospital of Malacca for providing us with the histologic slide.


From the Department of Otorhinolaryngology (Dr. Cheong) and the Department of Pathology (Dr. Rahayu), University Malaya Medical Centre, Kuala Lumpur, Malaysia; the Department of Otorhinolaryngology, Cyberjaya University College of Medical Sciences, Selangor, Malaysia (Dr. Halim); and the Department of Otorhinolaryngology (Dr. Khir) and the Department of Pathology (Dr. Noorafidah), Sultanah Fatimah Specialist Hospital, Johor, Malaysia. The case described in this article occurred at the Sultanah Fatimah Specialist Hospital.
Corresponding author: Dr. Jack P. Cheong, Department of Otorhinolaryngology, University of Malaya, 50603, Kuala Lumpur, Malaysia. Email: jewel_pein2003@yahoo.com

References

  1. Furuta Y, Nojima T, Terakura N ,et al. A rare case of carcinosarcoma of the maxillary sinus with osteosarcomatous differentiation. Auris Nasus Larynx 2001; 28 (Suppl): S127 - 9.
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  4. Shindo ML, Stanley RB Jr., Kiyabu MT. Carcinosarcoma of the nasal cavity and paranasal sinuses. Head Neck 1990; 12 (6): 516-19.
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Ear Nose Throat J. 2014 June;93(6):E1