Abstract
Background
Pleomorphic liposarcoma (PL) is an exceedingly rare tumor of the head and neck. This aggressive liposarcoma variant portends a poorer prognosis compared to more typical sarcomatous tumors.
Methods
Multi-institutional study including two cases of PL, the first case occurring in the post-auricular region and the second in the cheek with later recurrence within the parotid bed.
Results
Both patients were treated surgically with wide local excision. One patient required reoperation to obtain negative margins. The other patient underwent a total parotidectomy with neck dissection for recurrent intraparotid nodal disease. Both patients underwent adjuvant radiotherapy. No patient had evidence of recurrent disease with minimum one year follow-up.
Conclusion
Although the literature is sparse on this disease, wide surgical extirpation with negative margins and adjuvant radiation is recommended. Increased reports of this pathology can be helpful in providing practitioners with experienced-based information that can aid in early detection and treatment. We present the first case series of PL of the head and neck in the literature.
1
Introduction
Pleomorphic liposarcomas (PLs) are an exceedingly rare type of tumor in the head and neck region and can present variably depending on the subsite within the head and neck region. Although rare, as head and neck surgeons, it is important to have an understanding of the presentation, evaluation and management of these tumors. In this care series, we present two cases of PL arising in different head and neck subsites along with a general overview of the current literature on the subject. To our knowledge, this is the first series of head and neck pleomorphic liposarcomas in the English literature.
3
Results
3.1
Case presentation #1
A 60-year-old male with a history of human immunodeficiency virus (HIV) presented to the office with a 1-year history of a left post-auricular mass, which he feels had grown in the past several months. The patient reported no associated symptoms aside from a transient episode of pain, which resolved without intervention. He denied infectious or neurosensory symptoms. The patient has no history of tobacco or alcohol abuse.
The patient underwent computed tomography (CT) of the neck with intravenous contrast, which showed a 3.2 cm poorly defined, rim enhancing cystic mass without underlying mastoid involvement. Fig. A displays a representative axial image from this patient.
The patient was taken to the operating room for resection of the post-auricular mass, including periosteum of the temporal/occipital bone along with portions of the sternocleidomastoid muscle, trapezius, and sub-occipital fat. There was no evidence of bony erosion at the time of surgery. Preliminary analysis of the specimen showed a malignant spindle cell neoplasm with a significant liposarcomatous component. MDM2 staining was negative. Final pathologic diagnosis was pleomorphic liposarcoma.
The patient then underwent a staging PET-CT scan which showed no evidence of locoregional or distant disease. As the final pathology from the first surgery demonstrated focal involvement of the periosteum overlying the temporal bone, the patient was taken back to the operating room for a revision procedure including wide resection of the postauricular region including the scar, subcutaneous margins, and a drill-out of the outer cortex of the temporal bone. The subsequent defect was reconstructed with a superiorly based trapezius myocutaneous flap.
The patient’s post-operative course was uneventful. He later underwent adjuvant radiation. He continues to be free of disease at one year follow-up.
3.2
Case presentation #2
A 56-year-old male presented to the office with a progressively growing subcutaneous mass of the left cheek. An excisional biopsy was performed on this specimen and found to be a PL. Following diagnosis of malignancy, a repeat wide local excision was performed to obtain adequate oncologic margins. No adjuvant therapy was performed and the patient did well in the interim.
Six months following surgery, the patient returned to the office with an ipsilateral mass in the tail of the parotid gland. A core needle biopsy was performed, revealing metastatic PL. A total parotidectomy with levels II–V selective neck dissection was performed, revealing cervical metastasis in a 1.1 cm single intraparotid lymph node without extracapsular spread. Fig. B shows a photograph of the malignant focus in the intraparotid lymph node biopsy. Fig. C shows a magnified view of the pathology. There was no residual disease in the parenchyma of the parotid gland other than the involved intraparotid node and the remaining lymphadenectomy specimen was free of disease as well.
