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Andrea Bianco MD1,2, Gioconda Porras MD1, Esperanza García MD1, Mariana Sánchez R MD1,3, Gabriela Agostini MD1,41Clínica El Avila, 2Hospital Dr. Leopoldo Manrique Terrero, 3Hospital Dr. Domingo Luciani, 4Hospital Universitario Dr. Peset

1 ,2, 3 Caracas – Venezuela, 4 Valencia - España

INTRODUCTION

CASE REPORT

CONCLUSIONSDISCUSSION

REFERENCES

ABSTRACT

CONTACT

LIPOSARCOMA OF THE NASOPHARYNX: A case report

Andrea Bianco, MDClinica El Avilaandreabianco7@gmail.comPhone: +584123360419

A 29 year old female with a two years history of nasal congestion, nasal voice and oral breathing. High-resolutioncomputed tomography (CT), as well as magnetic resonanceimaging (MRI), showed a massin nasopharynx with no patterns of invasion into thesurrounding tissues. She wastreated only with complete exceresis of the lesion. Subsequent tissue samplingand histopathologydemonstrated featuresconsistent with welldifferentiated liposarcoma. After fourteen month fromsurgery the patient wasdisease free. We only found four cases reported on literature. Therefore with this case wewant to reinforce the notionthat while this is anuncommon entity, liposarcomashould be still considered as part of the differentialdiagnosis in patients with rarecases of nasal obstructivesymptoms

Liposarcoma, described by Virchow in 1856, accounts 15% of all soft tissue sarcomatous tumours in the body. Only1.8-6.2% occur in the head and neck region, mostly affecting the neck, larynx and hypopharynx. It has a peakocurrence between the 5th and 7th decade, a slight male preponderance and there are no known association withethnicity or geography. In the reported cases of nasopharyngeal lesions, as well as in our case, the main symptomswere nasal obstruction that did not improve with medical treatment. Most authors agree that the imaging modality of choice is MRI, showing a fatty, high intensity on T1 weighted sequences and intermediate signal on T2 weightedsequences, with signal attenuation following fat saturation. However, CT is still recommended, especially when data regarding bony or cartilage erosion is required.

On histology, the characteristic cell is the lipoblast. These cells have a characteristic hyperchromatic “chicken claw”shaped nucleus that is indented by cytoplasmic fat globules. The current classification system accepted by the WorldHealth Organization for liposarcomas consists of five subtypes: well differentiated, myxoid, dedifferentiated, round-cell and pleomorphic. The latter three subtypes are considered to represent the poorly differentiated high-grade liposarcomas. It represents diagnostic challenges because it could not be diagnosticated by an intraoperative frozensamples.

Prognosis of liposarcoma is influenced by three factors: histologic variant, adequacy of surgical escisión and locationof the tumor, having been the most important the histologic grade. Well differentiated and myxoid liposarcomas mayrecur locally, especially if surgical escision is incomplete, but they do not metastasize. In contrast, pleomorphic and round cell liposarcomas are much more aggressive tumors, 80% of the patients with round cell tumors died turingfollow-up. The role of tumor size in prognosis is unclear.

A 29 year-old female presented to our practice with2 years history of nasal congestion, nasal voice and oral breathing that did not improve with medical treatment. Nasopharyngeal endoscopy revealed a grayish right-sided mass, situated at the posterior wall of the nasopharynx. High-resolution computedtomography (CT), as well as magnetic resonanceimaging (MRI), showed a mass, measuringapproximately 3x3 cm, with no patterns of invasioninto the surrounding tissues. The lesion showedheterogeneous and predominantly high signalintensity on T1 weighted imaging and low signalintensity on the fat-saturated T2 weightedsequences. The tumor was completely resectedthrough an intranasal approach with grossly clearmargins. The patient had an uneventful post-operative recovery. The histopathologicalexamination showed a well differentiated liposarcomawith clean margins. Inmunoperoxidase stains werepositive for S100 protein. Six months later the patientunderwent surgery for recurrent tonsillitis, performingtonsillectomy, curettage of the posterior wall of thenasopharynx and cervical lymph node excision biopsywithout signs of recurrence. Fourteen monthsafter original surgery there are still no signs of recurrence on follow-up endoscopy and MRI.

Liposarcoma is a soft tissue malignant tumor thatexceptionally develops in nasopharynx, with only fourcases having been described in the literature. Thenatural history and therefore prognosis is largelydeterminate by histologic grade. Treatment of choiceis surgical excision with the wider possible marginsfollowed by postoperative radiotherapy orchemotherapy in selected cases.

Liposarcomas, are soft-tissue tumors that commonlyaffect the retroperitoneum. In head and neck regioncomprise 1.8-6.2% of all cases. In nasopharynx are exceptionally rare. We report our experience with onecase of well differentiated liposarcoma in nasopharynx and discuss the diagnostic challengesposed by a rare entity in this anatomical location.

- Chakraborty, E. E. Kassel, and E. Yu, “Nasopharyngeal liposarcoma MRI imaging features and a review of the literature,” The British journal of radiology, vol. 80, no. 955, pp. e145–146, 2007.- Golledge J, Fisher C, Rhys- Evans Ph. Head and Neck Liposarcoma. Cancer 1995; 76: 1051-58- Piles Galdon, A; et al. Liposarcoma Cervical. A propósito de un caso y revisión de la literatura. Acta Otorrinolaringol Esp 2002; 53: 617-620- S. Gritli, K. Khamassi, A. Lachkhem et al., “Head and neck liposarcomas. A 32 years experience,” AurisNasus Larynx, vol. 37, no. 3, pp. 347–351, 2010- Papacharalampous, G: et al. Liposarcoma of the Nasopharynx: Diagnosis and Management of a Rare Diagnostic Entity- B. Nageris, M. Feinmesser, I. Brama, and R. Feinmesser, “Li- posarcoma of the nasopharynx: a case report,” Ear, Nose and Throat Journal, vol. 70, no. 8, pp. 520–522, 1991- C. H. Knowles and P. H. Huggill, “Liposarcoma: with report of a case in a child,” The Journal of Pathologyand Bacteriology, vol. 68, no. 1, pp. 235–245, 1954C. H. Knowles and P. H. Huggill, “Liposarcoma: withreport of a case in a child,” The Journal of Pathology and Bacteriology, vol. 68, no. 1, pp. 235–245, 1954

Provided that nasopharyngeal liposarcomas are exceedingly rare, there are very limited reports in theliterature with regard to the management of suchmalignancies. Most therapeutic protocols and experience are basically based on treatment strategiesinvolved in liposarcomas of the head and neck oranother anatomic regions. It is primarily a surgicaldisease, so wide surgical excision alone is consideredthe main goal for low grade liposarcomas. Sometimesradical tumor excision is not possible, mainly due toanatomical limitations correlated with the site of thelesion, which increases the rate of local recurrence in low-grade lesions or distant metastases in high-grade lesions such as round-cell or pleomorphicliposarcomas. In that cases, most authors agree thatradiation therapy as adjuvant treatment attempt toreduce the risk of local recurrence. The role of chemotherapy is less defined; some authors supportthe use of neoadjuvant chemotherapy regimens in selected patients with high-grade tumors

In fact, all cases of nasopharyngeal liposarcomas, wererelated with various difficulties, affecting treatmentstrategy. Our case, was the first case treated only withcomplete excision along wide margins being free of disease after fourteen months.