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Parapharyngeal Liposarcoma: A Rare Case
Report and Review of the Literature
Jawad Hasnaoui*, Said Anajar, Reda Abada, Sami Rouadi, Mohammed Roubal and Mohammed Mahtar
ENT Department, Face and Neck Surgery, Hospital August, University Hospital Centre, Morrocco
Submission: February 23, 2017; Published: March 09, 2017
*Corresponding author: Jawad Hasnaoui, ENT Department, Face and Neck Surgery, Hospital August, 20’1953, University Hospital Centre IBN OCHD, 6, Street lahssen ELAARJOUN quartier des hopitaux, Casablanca, Morroco, Tel:212675 601663; Email:firstname.lastname@example.org
How to cite this article: Jawad H, Said A, Reda A, Sami R, Mohammed R, Mohammed M. Parapharyngeal Liposarcoma: A Rare Case Report and Review
002 of the Literature. Glob J Oto 2017; 5(1): 555651. DOI: 10.19080/GJO.2017.05.555651
Liposarcomas rarely occur in the parapharyngeal space and only a few case reports exist. We report a case of a 31-year-old female with a liposarcoma arising in the parapharyngeal space. We removed the tumour surgically utilising a cervical approach. The histological diagnosis was well-differentiated liposarcoma. There is no recurrence after 6 month of follow up. The diagnosis of liposarcoma is based on clinical, radiological and histological arguments. CT and/or MRI evaluation of the head and neck is very important, although histological confirmation is critical. For curative therapy of liposarcoma, surgical excision remains the dominant modality. Although a wide surgical margin is important to prevent local recurrence, wide excision is often difficult in the head and neck region. The benefits of adjuvant therapy are still not proven. Long-term follow up will be necessary because delayed local recurrences are not rare.
Parapharyngeal liposarcoma is a very rare malignant tumor that often causes nonspecific clinical symptoms, including progressive dysphagia, globus sensation and/or respiratory disturbances [1,2]. They usually occur in the lower extremities and retroperitoneum, and rarely occur in the head and neck region . The parapharyngeal space is a very rare site for liposarcomas, and only a few case reports exist. We present a case of a 31-year-old female with a liposarcoma arising in the parapharyngeal space. Through our case, we describe the different steps that led to the diagnosis of liposarcoma, as well as a review of the literature.
A 31-year-old north African woman presented with a 3-month history of swelling in the right side of his mouth and neck. He also complained of dysphagia, and odynophagia without any weight loss. On physical examination, he was found to have a bulging asymmetry of his right soft palate and tonsil. He also had a painful soft and smooth mass which was 7 cm in size under the right mandible with intact skin but no palpable adenopathy (Figure 1). The radiological examination (CT) revealed a low-density regular solid lesion on the posterior wall of the oropharynx and laryngopharynx, which descended to the thyroid cartilage and extended to the right parapharyngeal space and sternocleidomastoid muscle (Figure 2). The patient underwent transcervical excision. The submandibular gland wasremoved and the posterior belly of digastric muscle was resected to improve access to the parapharyngeal space. Intra-operative inspection revealed a lobulated lipomatous mass which measured 7 x 7 x 5 cm (Figure 3). Pathological examination of the tumour revealed a well-differentiated liposarcoma. Facial nerve function was normal after surgery, and the patient had no recurrence after 6 months of follow up.
Parapharyngeal liposarcomas are extremely rare, and only
a few cases have been described in the literature. They are very
slow-growing tumors and do not cause symptoms until they
reach a large size [1,3]. They typically occur between the fourth
and sixth decades of life and exhibit a male preponderance.
Liposarcomas do not develop from lipomas but rather from
primitive mesenchymal cells . The mechanisms responsible
for liposarcoma development are very complicated and are
not fully understood. The most common symptoms include an
abnormal sensation in the throat, noisy breathing, hoarseness,
dyspnea, dysphagia and obstructive sleep apnea . In our case,
the first symptoms were dysphagia and odynophagia without
dyspnea, which delayed the diagnosis. The reason for the
consultation was the appearance of the mass which developed 2
months after appearance of the symptoms.
The diagnosis of liposarcoma is based on clinical,
radiological and histological arguments. From the anatomical
site and radiological findings of the tumour, we considered
lymphangioma, salivary gland tumour, neurinoma and dermoidcyst as principal differential diagnosis. CT and/or MRI evaluation
of the head and neck is very important, although histological
confirmation is critical. The CT and MRI findings indicated an
adipose prominent solid mass with nodular or patchy nonadipose
components, and these are consistent with liposarcoma
. To confirm the diagnosis of liposarcoma, a surgical removal
of the tumour is usually necessary. The margin status of our case
was microscopically clear. The patient had no recurrence after 6
months of follow up. Liposarcomas are considered radiosensitive
tumors, although the efficacy of radiation therapy in patients
with head and neck liposarcoma is controversial. In areas where
wide local excision would be contraindicated given anatomic
limitations, radiation may play a role in therapy for liposarcomas
. In our case, the margins of resection are clear, the benefits
of adjuvant therapy after surgery are still not proven. We opted
for long-term follow up will be necessary because delayed local
recurrences are not rare.
We present a rare case of liposarcoma arising in the
parapharyngeal space. Although wide local excision remains the
mainstay for treatment for liposarcoma, taking a wide surgical
margin is often difficult in the head and neck region. The benefits
of adjuvant therapy are still not proven. Long-term follow up will
be necessary because delayed local recurrences are not rare.