Adult Alveolar Soft Part Sarcoma of the Head and Neck: A Report of Two Cases and Literature Review

Abstract

Background. Alveolar soft part sarcomas (ASPS) of the head and neck are rare, aggressive soft-tissue malignancies. This study describes the clinical course and management of two patients presenting with ASPS in very rare head and neck locations, the larynx and parotid gland. Methods. We identified two patients presenting with ASPS of the head and neck and treated at the University of North Carolina. We compared our results to the literature from 1987 to 2013. Results. Patient ages at diagnosis were 27 and 39 with presenting symptoms of hoarseness and parotid swelling, respectively. Mean follow-up was 87 months. All patients received surgical resection and adjuvant radiotherapy. There were no recurrences or evidence of distant metastatic spread during the series. Disease-free survival time for the patients was 4 months and 168 months, respectively. Conclusions. Our study suggests that a combined-modality approach is important in the treatment of ASPS of the head and neck even in these rare locations. Continued research into new therapies is necessary to improve historically poor outcomes.

Alveolar Soft Part Sarcoma of Hypopharynx; A Case Report

“…28,31,32 The involvement of pharynx with ASPS is uncommon compared to other organs (tissues) at the head and neck. In the larynx, cases occur in the false vocal cord 5,8 and true vocal cord. 2 A case of large ASPS occupying the hemilarynx was also reported in a 23-year-old male.…”

Primary Alveolar Soft Part Sarcoma of Cheek: Report of a Case and Review of the Literature

“…Primary ASPS of the cheek is exceedingly rare. Although approximately 15 such cases have been reported to date, only this case was confirmed to have type 2 ASPSCR1 (exon 7)-TFE3 (exon 5) fusion transcription (Table 1) [4][5][6][7][8][9][10][11][12].…”

“…In contrast, general ASPS usually presents as a painless and slow-growing mass that rarely causes functional impairment, and it is considered as a high-grade sarcoma by definition [1]. Previous studies have reported that the cheek ASPS sometimes exhibited well-circumscribed margins (4/10, 40%) and round contours (4/10, 40%), while general ASPS had ill-defined margins and lobulated or irregular contours [1,[4][5][6][7][8][9][10][11][12][13]18]. In the cheek ASPS, the mean size was 3.7 cm (range 1.2-10 cm) and mean age was 33.7 years (range 5-57 years), whereas, in general ASPS, the mean size was 6.5 cm (range 1.2-24 cm) and mean age was 25 years (range 1-78 years) [1,[4][5][6][7][8][9][10][11][12].…”

“…Previous studies have reported that the cheek ASPS sometimes exhibited well-circumscribed margins (4/10, 40%) and round contours (4/10, 40%), while general ASPS had ill-defined margins and lobulated or irregular contours [1,[4][5][6][7][8][9][10][11][12][13]18]. In the cheek ASPS, the mean size was 3.7 cm (range 1.2-10 cm) and mean age was 33.7 years (range 5-57 years), whereas, in general ASPS, the mean size was 6.5 cm (range 1.2-24 cm) and mean age was 25 years (range 1-78 years) [1,[4][5][6][7][8][9][10][11][12]. The median size of lingual ASPS was 2.5 cm and that of orbital ASPS was 2.85 cm [19,20].…”

“…In the head and neck region, the most commonly affected sites are the tongue and orbit. Approximately 15 cases of ASPS of the cheek have been reported to date (Table 1) [4][5][6][7][8][9][10][11][12]. The presence of ASPSCR1-TFE3 fusion gene was confirmed by reverse transcriptionpolymerase chain reaction (RT-PCR) in only one of those cases, but the fusion type was unknown [8].…”