Liposarcoma of Maxilla- A Rare Case Report.

INTRODUCTION

Sarcomas of the head and neck region account for less than 10% of soft tissue sarcomas, and comprise less than 1% of head and neck malignancies. Approximately 80% of sarcomas arise from soft tissue, with the remaining originating from bone or cartilage. Head and neck sarcomas typically occur more frequently in men.

CASE REPORT

Our patient was a 30 year old male who presented with a ulceroproliferative mass over the soft palate increasing in size over past 6 months. Punch biopsy of the lesion was suggestive of carcinosarcoma. MRI PNS was suggestive of 38 × 39 × 33 mm sized lobulated lesion involving soft palate on right side, crossing midline and displaces uvula. The lesion involved right side of hard palate, right retromolar trigone with abutment of right medial pterygoid. It was also suggestive of bilateral sub centimetric nodes involving bilateral level 2 neck nodes. The patient received 3 cycles neoadjuvant chemotherapy Ifosfamide and Doxorubicin and patient had no subjective response to chemotherapy with subjective increase in the size of lesion. MRI PNS post neoadjuvant chemotherapy was suggestive of 46 × 46 × 41 mm lobulated lesion involving right side of the soft palate crossing midline over to the left, involving right posterior hard palate and right retromolar trigone, tonsillar fossa and TL sulcus. CECT thorax was negative for lung metastasis. Our patient was a 30 year old male who presented with a ulceroproliferative mass over the soft palate increasing in size over past 6 months. Punch biopsy of the lesion was suggestive of carcinosarcoma. MRI PNS was suggestive of 38x39x33mm sized lobulated lesion involving soft palate on right side, crossing midline and displaces uvula. The lesion involved right side of hard palate, right retromolar trigone with abutment of right medial pterygoid. It was also suggestive of bilateral sub centimetric nodes involving bilateral level 2 neck nodes. The patient received 3 cycles neoadjuvant chemotherapy Ifosfamide and Doxorubicin and patient had no subjective response to chemotherapy with subjective increase in the size of lesion. MRI PNS post neoadjuvant chemotherapy was suggestive of 46 × 46 × 41 mm lobulated lesion involving right side of the soft palate crossing midline over to the left, involving right posterior hard palate and right retromolar trigone, tonsillar fossa and TL sulcus. CECT thorax was negative for lung metastasis.

DISCUSSION

The patient underwent wide local excision with medial maxillectomy via a bilateral Weber-Ferguson incision with Diffenbach extension and sent for frozen. The margins were negative and free of tumor. Bilateral modified neck dissection type 3 was done for lymph node clearance. A bilateral temporalis flap was done for reconstruction of the defect with a temporary obturator placement. Sarcomas of the sinonasal region may present a diagnostic challenge, as their location in the sinuses or nasal cavity may lead to presenting symptoms such as epistaxis, nasal congestion, or sinus pain and pressure that may be attributed to more benign causes such as chronic sinusitis, sinonasal polyposis, or allergic rhinitis. The less common sinonasal sarcoma subtypes may present a diagnostic challenge as their histopathological characteristics may overlap, especially with variations in tumor grade or with dedifferentiation. Additionally, the ideal treatment modality may present a therapeutic challenge, as the response to radiation and/or chemotherapy may vary according to the sarcoma subtype. Surgery is considered to be the mainstay in the management of carcinosarcomas of head and neck. Based on the evidence presented herein, elective neck dissection should be considered to treat squamous cell carcinomas of the maxillary palate, ginigiva, and alveolus.