Keywords
sarcoma - synovial - deglutition disorders
Introduction
Synovial sarcoma is a malignant tumor of pluripotent mesenchymal cells,[1]
[2]
[3]
[4] most commonly found in periarticular areas, with few cases occurring in the head
and neck.[1]
[2]
[3]
[4]
[5] The first synovial sarcoma was described by Jernstrom in 1954.[6] In this article, we present a case of synovial sarcoma in the posterolateral wall
of the oropharynx. This case report was authorized by the ethics committee by the
number 229.577.
Case Report
The patient, a 23-year-old woman, was admitted to the otorhinolaryngology clinic at
our hospital with a history of dysphagia and difficulty of breathing for 8 months,
resulting in progressive worsening and emergence of snoring, muffled voice, and local
pain. She denied fever episodes, smoking, and alcohol consumption. Physical examination
revealed an oropharyngeal tumor in the left posterolateral wall near the base of the
tongue. Rhinoscopy and otoscopy confirmed the diagnosis, with no changes. Videolaryngoscopy
([Fig. 1]) showed a rounded submucosal tumor with intact mucosa, starting at the left posterolateral
wall, touching the epiglottis down to the left, narrowing the light of the entire
pharynx, preventing full view of the glottis. Cervical magnetic resonance imaging
([Figs. 2], [3], and [4]) showed a solid cystic lesion in the submucosal left posterolateral wall of the
oropharynx of relatively delimited contour, reducing the airway and rejecting the
left piriform sinus. The mass measured ∼5.3 × 3.9 × 2.2 cm in its greatest axis. Its
left lateral region was predominately solid, showing heterogeneous impregnation of
paramagnetic contrast, and measured 2.1 × 2.0 cm, with the cleavage plane delimited
by prevertebral muscles. Lymph nodes measured up to 1.3 cm in internal high jugular
chains and submandibular (IB and IIA) on the right. Laboratory tests were unchanged.
Fig. 1 Preoperative laryngoscopy showing the mass at the hypopharynx.
Fig. 2 Nuclear magnetic resonance, axial view.
Fig. 3 Nuclear magnetic resonance, coronal view.
Fig. 4 Nuclear magnetic resonance, sagittal view.
Endoscopic pharyngectomy was used to excise the tumor. Pathologic examination revealed
a synovial sarcoma with positive margins, and Mohs technique was proposed for edge
control. A full pharyngectomy with ipsilateral modified radical neck dissection was
proposed, with the possibility of full laryngeal pharyngectomy depending on surgical
margins obtained intraoperatively by freezing anatomy. During surgery, the margins
came initially committed, and after expansion, the margins were disease-free. It was
possible to obtain free surgical margins without the need for total laryngectomy.
The pharynx was reconstructed with a microvascularized forearm flap. We also performed
the proposed neck dissection, tracheostomy, and passage of a nasogastric tube for
nutrition. The neck dissection was disease-free.
Postoperatively, the patient's condition was stable. Upon follow-up with an oncologist,
chemotherapy and radiation were given. A gastrostomy was performed for nutrition,
as oral feeding was impossible. At swallowing endoscopy, there was saliva aspiration
due to lack of mobility of the pharyngeal muscles. The patient is already in speech
therapy. All mobility of the larynx was preserved. Within ∼1 year postoperatively,
the patient continues with locoregional control without recurrence and performs pharyngoesophageal
dilatations to treat actinic stenosis. Return to oral feeding is in progress. The
tracheostomy tube is closed and will be removed as soon as oral feeding is stabilized.
Discussion
Despite its name, synovial sarcoma rarely originates in the synovial membranes.[7] It is most commonly found in the vicinity of large joints. Occurrence in the head
and neck, a location poor in synovial tissue, is uncommon.[8] Synovial sarcoma comprises 8 to 10% of all soft tissue sarcoma,[1]
[3]
[5]
[7]
[9] and it is estimated that ∼3 to 10% of sarcomas occur in the head and neck.[4]
[5]
[10] These tumors can be located in the prevertebral space from the base of the skull
to the hypopharynx, retropharyngeal and parapharyngeal spaces, and anterior neck along
the edges of the sternocleidomastoid muscle, as well as sites in the oropharynx and
larynx.[8]
[10] Synovial sarcomas show a male predominance (3:2) and predilection for patients between
25 and 36 years of age.[4]
[11]
[12]
This type of tumor usually appears as an asymptomatic mass until it acquires sufficient
volume to cause compressive effects on neighboring structures.[1] It can be a painless mass or associated with symptoms such as pain, earache, sore
throat, and bleeding. There are two subtypes: monophasic and biphasic. For diagnosis,
it is important to include laryngoscopy and head, neck, and chest computed tomography
to define the local extent of disease and metastasis.[4]
Resection with negative margins remains the foundation of therapy,[7]
[12] which is not so easy to achieve in the head and neck.[4]
[5] Local excision is followed by high recurrence rates (60 to 90%), usually within
2 years.[4]
[5]
[9] Metastasis to regional lymph nodes of synovial sarcomas in the head and neck is
not seen often, although there can be clinically enlarged lymph nodes.[5] Most metastasis originate from hematogenous dissemination, although up to 20% spread
through the lymphatics to regional lymph nodes.[4]
[8] Cervical lymph node dissection is not routinely performed, except in the presence
of enlarged lymph nodes.[8]
[10] Hematogenous dissemination is the harbinger of death. Pre- and postoperative radiotherapy,
with or without chemotherapy, may be responsible for the increase in survival statistics
over the last 2 to 3 decades, but studies are still controversial and inconclusive.[4]
[7]
[9]
Despite relatively slow growth, long-term survival for synovial sarcomas from anywhere,
including the head and neck, is not good.[5] Synovial sarcoma in the head and neck has an aggressive nature and a reserved prognosis.[3]
[8] Several factors have been evaluated for prognosis, and the most significant were
tumor size and deep extension at the time of primary treatment,[9] with survival varying inversely with these two factors.[5] The age at diagnosis seems to have involvement with prognosis,[9] although the relative youth of patients with synovial sarcoma of the head and neck
does not seem to give a significantly better prognosis.[5]
Final Comments
Synovial sarcoma is a rare malignant tumor which treatment is essentially surgical
resection with high rates of relapse. Although traditional treatment for hypopharynx
tumors has been laryngectomy, it can be modified for sarcomas at this area. In these
cases, most important are the border's microscopic control and lack of lymphatic metastasis.
Radiation therapy with or without chemotherapy appears to be responsible for the increased
survival in recent decades. It is important for the otolaryngologist and head and
neck surgeon to be familiar with this aggressive tumor, which carries high mortality
and morbidity. The appropriate early diagnosis and treatment can improve the prognosis
and survival of patients.
