PATHOLOGY RESEARCH AND PRACTICE
© Urban & Fischer Verlag
Laryngeal Liposarcoma: Report of a Case M. J. Mestre de Juan and M. J. Fernandez-Acenero Department of Surgical Pathology, Hospital de M6stoles, Madrid, Spain
Summary Laryngeal and hypopharyngeal liposarcomas are fairly rare tumors, with only 30 convincing cases reported to date. These tumors usually arise in the supraglottic area, and only two cases have been reported to affect the true vocal cord. They behave in an indolent fashion with multiple local recurrences and only rarely cause the patient's death. Our case highlights the natural history of this entity. A 62 year-old man presented because of airway obstruction. A CT scan discovered a large 5 em polypoid mass in the right aryepiglottic fold. The patient had already undergone three previous operations for the same reason in another clinic, with histopathological diagnoses of fibrovascular polyps. In the last recurrence, only a careful search for lipoblasts in the surgical specimen allowed us to identify this lesion as a low-grade well-differentiated liposarcoma. Complete resection was impossible in this case, despite total laryngectomy. Key words: Larynx - Liposarcoma - Lipoma - Hypopharynx - Soft tissue tumors
Laryngeal and hypopharyngealliposarcomas are exceedingly rare, and to date only 30 cases have been reported in the world literature [5, 9]. We report another case and review the main clinical and pathological features of this tumor.
Case Report A 62-year-old man with a history of heavy smoking and chronic laryngitis presented with airway obstruction that had developed over the last 4 months. Although a laryngeal polyp had been removed from the left true vocal cord 12 years before, he underwent three operaPathol. Res. Pract. 195: 125-128 (1999)
tions over the last four years for the same complaints (airway obstruction) in another clinic. Polypoid lesions arising in the right supraglottic area were removed and histopathologically diagnosed as fibrovascular polyps. The preoperative CT scan performed in our patient showed a huge 5 em mass in the right aryepiglottic fold probably extending to and affecting the ipsilateral false vocal cord. A biopsy of the mass was obtained with the use of endoscopic techniques. We received a pedunculated polypoid mass excised from the aryepiglottic fold with a size of 3 em in largest diameter. On section, an intact epithelium covered a soft yellow tissue. The tumor was localized in the submucosa, composed of fat lobules with varying amounts of fibrous tissue and showed a myxoid matrix with a plexiform capillary network (Figs. 1 and 2). The adipocytes were of different size with scattered lipoblasts (Fig. 3). Mitoses were scant. Immunohistochemistry on formalin-fixed paraffinembedded tissue revealed positivity of the tumor cells only for S-lOO protein. As the surgical margin was affected, a supraglottic laryngectomy was performed. The huge 5 em yellow mass present in the surgical specimen (Fig. 4) also infiltrated into the distal resection margin. As a consequence, totallaringectomy was performed. The study of the latter specimen revealed tumor infiltration of the posterior margin. Three months after laryngectomy, the patient was again operated in another clinic for removal of the tumor left in the previous surgery. His postoperative course was uneventful and he received no further treatment. Today, 14 months after laryngectomy, he is alive and well. Address for correspondence: Department of Surgical Pathology, Hospital de M6stoles, c/Rio Jucar sin. M6stoles. E - 28935 Madrid (Spain). Tel.: ++34-91-6648640, E-mail: [email protected]
M. J. Mestre de Juan and M. J. Fernandez-Acefiero
Fig. I. Myxoid areas with a plexiform capill ary network in some areas of the tumor (H & E x 100).
Fig. 2. Foci of mature adipocytes intermingled with some myxoid area s (H & E xIOO).
Fig. 3. High power view of a lipoblast (H & EX400).
Laryngeal Liposarcoma . 127
Fig. 4. Supraglottic laryngectomy. Note the huge mass in the right aryepiglottic fold, covered by normal mucosa. The tumorreached the resection margin.
Discussion Laryngeal and hypopharyngealliposarcomas are exceedingly rare tumors [1, 3J. Having reviewed the 30 cases reported to date, we think that the typical clinical picture is as follows [8, 9J: These tumors are more common in men, and only three cases have been reported in women (male to female ratio 9: 1). Age ranged between 25 and 81 years, but they seem to occur more commonly in middle-aged persons (fifth to seventh decades of life). The most frequent complaints at presentation is airway obstruction or dysphagia, although some patients present because of hoarseness or throat discomfort [7, 8]. Almost all the reported cases affected the supraglottic area. There are only two reports of tumors affecting the true vocal cords [2, 9J, but tumors infiltrating into the subglottic area have not yet been described so far. The reasons for this tendency to affect the supraglottic area and for the increased incidence in males are unknown, and to date no etiologic factors have been identified for these lesions.
Histologically, most of these lesions are low grade tumors (either well differentiated or myxoid liposarcomas), with only few reports describing high grade tumors (pleomorphic [6J or round-cell [4J liposarcomas). The main differential diagnosis is lipoma and myxoid chondrosarcomas of this area. Lipomas are usually well-demarcated, encapsulated lesions which have no tendency to infiltrate into the surrounding structures, show no lipoblasts and allow a simple excision without recurrences. Immunohistochemistry is not useful for the differential diagnosis of these tumors, because both lipomas and chondrosarcomas are positive for S-IOO protein. Therefore, we must find the typical histological features of liposarcoma (mainly the presence of lipoblasts) and make sure that there are no chondromatous areas. The biological behavior of these tumors is uniform in most cases. The well-differentiated tumors tend to recur after incomplete excision but fail to show metastases. Only the high grade tumors have spread to the skin and the bone, causing the patient's death [2, 4J. The recommended treatment is wide surgical resection of the lesion. There appears to be no justification for the use of radiotherapy or chemoradiation therapy in these patients, because these adjuvant techniques do not improve the results obtained by complete resection alone. Our case fulfills most of the forementioned characteristics of this entity. We find this case interesting, because our patient had undergone three previous operations with histological diagnoses of fibrovascular polyps. We should keep in mind the extreme difficulty in achieving a correct diagnosis of well differentiated liposarcomas because lipoblasts are rare in some cases. This diagnosis should be considered in soft tissue tumors of the larynx- or hypopharynx that recur several times after incomplete resection. Benign tumors arising in this area have little tendency to recur, and this fact should force us to look carefully for the presence of myxoid areas, nuclear pleomorphism or lipoblasts diagnostic of liposarcoma. In summary, our case highlights the importance of considering every lipomatous recurrence of the larynx or hypopharynx as a potential well-differentiated liposarcoma.
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