A M E R I C A N J O U R N A L O F O T O L A R Y N G O L O G Y – H E A D A N D N E C K M E D I C I N E A N D S U R G E R Y 3 4 ( 2 0 1 3 ) 5 4 5 – 5 4 7
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Case reports
Solitary fibrous tumor of the hypopharynx: Case report andliterature review☆,☆☆,★
Christopher F. Thompson, MDa,⁎, Sunita M. Bhuta, MDb, Elliot Abemayor, MD, PhDa
a Department of Head and Neck Surgery, University of California, Los Angeles, CAb Department of Pathology and Laboratory Medicine, University of California, Los Angeles, CA
A R T I C L E I N F O
☆ Presentation at the Triological Society Co☆☆ No financial funding or support.★ No Disclosures.⁎ Corresponding author. UCLA Department of
Solitary fibrous tumors are rare mesenchymal neoplasms that are increasingly beingdescribed in the head and neck. Clinical presentations may include compression by thesetumors on vital surrounding structures. While malignant transformation is rare, treatmententails wide local excision. We present the case of a 74 year-old female with an increasinglyenlarging symptomatic hypopharyngeal solitary fibrous tumor that was found on carotidduplex ultrasound. Transoral surgical excision resulted in relief of symptoms. Treatmentoptions are discussed and a literature review of this uncommon disorder presented.
Solitary fibrous tumor (SFT) is an uncommon, slow-growing,mesenchymal neoplasm that was initially described in 1931arising from the pleura [1–4]. Since this first case, SFTs havebeen described in the abdominal cavity, extremities, retro-peritoneum, and head and neck region [1]. The first reportedcase in the upper aerodigestive tract was in 1991, and there areless than 200 reported cases in the head and neck, with themost common sites being the oral cavity and paranasalsinuses [3,5–7]. To our knowledge, we present the 3rd reportedcase of a SFT arising from the hypopharynx, with successfultreatment using transoral surgery (Table 1) [8,9].
mbined Sections Meeting
Head and Neck Surgery, 1
cla.edu (C.F. Thompson).
er Inc. All rights reserved
2. Case report
A 74-year-old female with a one-year history of progressivedysphagia and dysphonia presented to our tertiary academicmedical center with a hypopharyngeal mass. This mass wasincidentally found on a carotid duplex ultrasound of theneck. An outside biopsy was inconclusive. An MRI wassignificant for a 4 × 2 × 2 cm hypopharyngeal mass compres-sing the airway medially and abutting the carotid arterylaterally (Fig. 1).
She was then taken to the operating room and underwentmicrosuspension direct laryngoscopy and biopsy. Intraopera-tive frozen sections were nondiagnostic. Therefore, transoral
, Scottsdale, AZ, January 24–26, 2013.
0833 Le Conte Ave, CHS 62–142, Los Angeles, CA 90095. Tel.: +1 323
Mussak et al. 4.5 × 4 × 3.2 Progressive Dysphagia, 30 lb wt. loss Lateral pharyngotomy 18Hanna et al. 4.1 × 3 × 1.2 Progressive Dysphagia Transoral 9Present Study 4 × 2 × 2 Progressive dysphagia, dysphonia Transoral 9
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CO2 laser debulking was undertaken. The tumor was denselyadherent to the lateral hypopharyngeal wall. Final pathologyrevealed low grade spindle cell neoplasm consistent with SFT(Figs. 2 and 3). Nine-months postoperatively, she is asymp-tomatic with no mucosal irregularities on flexible laryngos-copy. She has elected conservative management with closeclinical and radiologic follow-up.
3. Discussion
Solitary fibrous tumors are slowly enlarging neoplasms thatare incidentally found on imaging or present with localcompression on nearby structures. Despite this neoplasm'srarity in the head and neck region, cases have been reported inthe soft tissues of the neck, salivary glands, oral cavity,pharynx, paranasal sinuses, and scalp [1,6,7]. As shown inTable 1, we present the third reported hypopharyngeal SFThighlighting the importance of including SFT in the differen-tial diagnosis of enlarging neck masses [8,9].
Depending on tumor location, patients often present withprogressive discomfort, unilateral nasal obstruction, changein vision, worsening dysphagia, hoarseness, or shortness ofbreath [6]. Our patient presented with right neck fullness andone-year history of mild progressive dysphagia and dyspho-nia. Despite the size and location of the mass, she denied anyshortness of breath or difficulty breathing.
Fig. 1 – Coronal T1-weighted MRI revealing a well-definedenhancing mass in the right hypopharynx.
Radiological imaging is critical in the diagnostic work-up,as SFTs cannot be diagnosed based on clinical symptoms orphysical exam alone. Computed tomography (CT) or mag-netic resonance imaging (MRI) can be used. Both CT and MRIscans usually reveal homogenously enhancing masses withdistinct borders (Fig. 1) [1]. Since SFTs are slow growing, CTscans often demonstrate expansile bony remodeling, espe-cially when located in the paranasal sinuses. On both T1 andT2 weighted MRIs, SFTs are isointense to brain tissue ornearby musculature [1].
Definitive biopsy is critical for confirming diagnosis. H&Eexamination of a typical SFT reveals spindle cells in apatternless pattern, with amorphous areas of collagen andhaphazardly arranged cells (Fig. 2) [1]. Immunohistochemistryis significant for the presence of CD34, CD99 and Bcl-2, withCD34 being the most sensitive and specific marker of SFT andsignifying mesenchymal origin (Fig. 3) [1,6]. Rarely, malignantfeatures can be seen, such as focal necrosis, hypercellularityand increased mitoses, which can increase the otherwise lowrisk of metastases [8,10].
Treatment for all SFTs is surgery. Chemotherapy andradiation therapy have not been shown to have significantroles as adjuvant treatments, but radiation can be consideredin cases of SFTswithmalignant features, especially if negativemargins were not obtained [1,10].
Although our case is only the 3rd reported hypopharyngealSFT, it illustrates the importance of including SFT in thedifferential diagnosis for any patient with an enlarging neckmass or progressive, insidious symptoms in the upper
Fig. 2 – Hematoxylin and eosin stain at high magnification(×20) revealing small spindle shaped cells individually sepa-rated by thin bands of collagen fibers arranged haphazardly.Note the thin walled ectatic vessel in the center.
Fig. 3 – CD34 is the most sensitive and specific marker forsolitary fibrous tumor as seen in this photomicrograph (×20).
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aerodigestive tract. Moreover, since “negative margins” in theupper aerodigestive tract can often mean significant mucosaldefects and negative functional outcomes, our case under-scores the possibility that more modern, less invasive toolssuch as transoral laser excision could have a significant role toplay in treating this unusual disorder. Our patient has doneextremely well post-operatively and is asymptomatic. Closelong-term follow-up with endoscopy and radiological imagingto rule out local recurrence is critical.
4. Conclusion
Solitary fibrous tumor of the hypopharynx is especiallyrare. Clinical presentation results from local compression
and includes progressive dysphagia. Our case highlightsthe effectiveness of a transoral approach for SFT excisionwith excellent functional outcomes and no evidence ofdisease recurrence.
R E F E R E N C E S
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