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A rare cause of dysphagia: Pyriform sinus atypical lipomatous tumor.

Atypical lipomatous tumor (ALT) and well-differentiated liposarcoma (WDL) are rare liposarcoma subtypes that are histologically identical but distinguished by the 2002 WHO classification: ALTs occur in surgically amenable locations, whereas WDLs do not. This distinction exists because these tumors do not tend to recur after complete surgical removal, despite their infiltrative growth pattern and locally aggressive behavior. (1)

A 60-year-old male nonsmoker presented for evaluation of several months of throat irritation and a sense of something stuck in his throat. Physical examination was normal, except flexible endoscopy revealed a benign-appearing, pedunculated mass with normal-appearing mucosa extending from the posterolateral pharyngeal wall onto the arytenoid tower. Computed tomography showed a benign-appearing mass within the left hypo-pharynx effacing the pyriform sinus.

The patient underwent transoral robotic surgical (TORS) excision of the mass. It was 5 cm in size, pedunculated andbilobed (figure 1, A). It was excised, leaving an approximately I-cm mucosal defect. The specimen was soft and pink (figure 1, B). Microscopic examination revealed a relatively circumscribed, adipocytic proliferation arranged in lobules of mature-appearing adipose tissue with prominent intervening fibrous septae. Random distribution of enlarged hyperchromatic cells with mild nuclear atypia and occasional multi-nucleation involved both the adipose and stromal components. Scarce lipoblasts and a rare hibernoma-like nodule were appreciated. Neither necrosis nor mitotic activity was identified. Cells were immunoreactive for MDM2 and p16, and nonreactive for p53, consistent with ALT (figure 2). Three months postoperatively, the patient had no evidence of recurrence.

ALT rarely presents in the laryngopharynx. (1-3) In 2009, Davis et al reviewed all head and neck sarcomas at the University of Texas M.D. Anderson Cancer Center over a 60-year period (1945-2005) and found 30 head and neck liposarcomas, of which only 7 were ALT/WDLs. (4) Three of these were in the upper aerodigestive tract. In 1999, Mandell et al compiled reports of eight hypopharyngeal liposarcomas from three studies, all of which were ALT/WDLs; they had a strong male predominance (7/8) and a size ranging from 2 to 14.5 cm. (3) In 2010, Shi et al reported five ALTs in the laryngopharynx: two in the hypopharynx and three in the larynx, ranging from 2.0 to 5.0 cm. (2) Dysphagia, dysphonia, and globus sensation were typical presenting symptoms.

Immunohistochemistry staining of MDM-2, a negative regulator of the p53 tumor suppressor, and p16 have emerged as markers for ALT/WDL. A 2017 study of 101 ALTs showed that dual p16 and MDM2 testing yielded a 100% positive predictive value and a 100% negative predictive value when MDM2+/p16+andMDM2-/p16-staining were observed for ALT-WDL, respectively. (5)

Surgical excision in the hypopharynx can be challenging because of inadequate access and visualization. Classic approaches include lateral pharyngotomy or direct suspension microlaryngoscopy. (6) Radiation is not recommended as primary therapy for hypopharyngeal ALTs but has been offered as adjunctive therapy. (3,4) If TORS continues to show a low recurrence rate for ALTs, it will further differentiate them from WDLs.

Yoseph A. Kram, MD; James M. McCann, DMD, MS; Joseph Golden, MD; Eric Wirtz, MD

From the Department of Otolaryngology, Tripler Army Medical Center, Hawaii. Disclaimer: The views expressed in this article are those of the author) and do not reflect the official policy or position of the Department of the Army, Department of Defense, or the United States government.

References

(1.) Dei Tos AP, Pedeutour F. Atypical lipomatous tumour/well differentiated liposarcoma. In: Fletcher C, Unni KK, Mertens F (eds). Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon, France: IARC Press; 2002:35-7.

(2.) Shi HY, Wei LX, Wang HT, Sun L. Clinicopathological features of atypical lipomatous tumors of the laryngopharynx. J Zhejiang Univ Sei B 2010;11(12):918-22.

(3.) Mandell DL, Brandwein MS, Woo P, et al. Upper aerodigestive tract liposarcoma: Report on four cases and literature review. Laryngoscope 1999;109(8):1245-52.

(4.) Davis EC, Balio MT, Luna MA, et al. Liposarcoma of the head and neck: The University of Texas M.D. Anderson Cancer Center experience. Head Neck 2009;31(l):28-36.

(5.) Kammerer-Jacquet SF, Thierry S, Cabillic F, et al. Differential diagnosis of atypical lipomatous tumor/well-differentiated liposarcoma and dedifferentiated liposarcoma: Utility of p16 in combination with MDM2 and CDK4 immunohistochemistry. Hum Pathol 2017;59:34-40.

(6.) Wenig BM, Heffner DK. Liposarcomas of the larynx and hypopharynx: A clinicopathologic study of eight new cases and a review of the literature. Laryngoscope 1995;105(7 Pt l):747-56.

Caption: Figure 1. A: Intraoperative image shows the pedunculated, bilobed tumor in the left hypopharynx. B: The 5-cm excised tumor is soft and pink with a fatty appearance.

Caption: Figure 2. The tumor is positive for pl6 on immunohistochemical staining.
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Title Annotation:DYSPHAGIA CLINIC
Author:Kram, Yoseph A.; McCann, James M.; Golden, Joseph; Wirtz, Eric
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Date:Apr 1, 2018
Words:779
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