- Email: [email protected]
Leiomyosarcoma of the hypopharynx: A case report
Auris Nasus Larynx 35 (2008) 304–307 www.elsevier.com/locate/anl
Leiomyosarcoma of the hypopharynx: A case report Toshitaka Takahashi, Ryuzaburo Higo *, Hiroto Nakata, Masashi Sugasawa Department of Otolaryngology, Head and Neck Surgery, Saitama Medical University, Morohongo 38, Moroyama-cho, Iruma-gun, Saitama 350-0495, Japan Received 30 November 2006; accepted 17 October 2007 Available online 6 February 2008
Abstract Cases of leiomyosarcoma in the head and neck are very rare with only four reports of a leiomyosarcoma originating in the hypopharynx. A rare case of leiomyosarcoma of the hypopharynx is described. A 62-year-old woman, who had experienced slight difficulty of swallowing and hoarseness, visited our hospital. A smooth-surfaced tumor was found extending from the postcricoid to an area in the left pyriform sinus, and the vocal cord on the left side was fixed. Imaging studies, including FDG-PET CT imaging showed the tumor in the postcricoid, but no distinguishable distant metastases. A total laryngectomy with a left-lobe thyroidectomy was performed. Since no metastases of the regional lymph nodes were found by imaging, a neck dissection was not performed. Dense infiltration by spindle-shaped and/or round-like cells was observed histologically as the predominant growth pattern. In immunohistochemical staining, the tumor was positive for desmin and muscle actin, while it was negative for myoglobin, myogenin, cytokeratin, and S-100 protein. Electron microscopic examination did not show Z banding and sarcomeres. Those features lead to the diagnosis of a leiomyosarcoma. No recurrence has been found at 1 year after the surgery. Immunohistochemical staining and electron microscopic examination are essential for a definite diagnosis of leiomyosarcoma. # 2007 Elsevier Ireland Ltd. All rights reserved. Keywords: Leiomyosarcoma; Hypopharynx; Immunohistochemical staining; Electron microscopic examination
1. Introduction Leiomyosarcoma is a malignant tumor derived from smooth muscle that accounts for 5–6% of all soft tissue sarcomas [1]. Leiomyosarcoma usually occurs in soft tissue of the extremities and trunk; only 3% of leiomyosarcomas are in the head and neck [2]. In the head and neck region, most leiomyosarcomas occur in the nasal cavity and paranasal sinuses, mouth, and larynx, while only four cases of leiomyosarcoma of the hypopharynx have been reported. We describe the fifth reported case of leiomyosarcoma in the hypopharynx.
2. Case report A 62-year-old woman, who had experienced slight difficulty of swallowing for 2 years, visited our hospital. She * Corresponding author. Tel.: +81 49 276 1253; fax: +81 49 295 8061. E-mail address: [email protected] (R. Higo).
also complained of hoarseness of several months in duration. An endoscopic laryngeal examination revealed a smoothsurfaced tumor extending from the postcricoid to an area of the left pyriform sinus, and the vocal cord on the left side was fixed (Fig. 1). CT imaging showed a non-uniform contrast enhanced tumor in the postcricoid region, and T1weighted MRI imaging of the tumor was high intensity with a relatively clear border (Fig. 2). FDG-PET CT imaging (2deoxy-2-[(18)F]-fluoro-D-glucose positron emission tomography, combined with CT images) revealed relatively high standardized uptake values (SUV: 3.8) corresponding to the tumor, but no distinguishable distant metastases. A biopsy performed under microlaryngoscopic surgery resulted in a diagnosis of spindle cell sarcoma. Since the tumor was mainly located in the postcricoid region, a total laryngectomy with a left-lobe thyroidectomy was performed. Neck dissection was not performed, because no regional lymph node metastases were found on sequential imaging studies, such as CT, MRI, and FDG-PET CT. Histopathological examination of the resected material showed dense infiltration by spindle-shaped and/or round-
0385-8146/$ – see front matter # 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.anl.2007.10.002
T. Takahashi et al. / Auris Nasus Larynx 35 (2008) 304–307
305
Fig. 1. The smooth-surfaced tumor can be seen extending from the postcricoid to an area in the left pyriform sinus (arrow). The vocal cord on the left side is fixed (left, inspiration; right, phonation).
like cells as the predominant growth pattern (Fig. 3). On immunohistochemical staining, the tumor was positive for desmin, and muscle actin (Fig. 4), while it was negative for myoglobin, myogenin, cytokeratin, and S-100 protein. Electron microscopic examination did not show Z banding and sarcomeres, which implied a structure of striated muscle. The tumor was diagnosed as a leiomyosarcoma. Since the surgical margins were negative, no postsurgical treatment was given. The patient is being followed up with no recurrence at 1 year after the surgery.
3. Discussion Cases of leiomyosarcoma originating in the head and neck region are very rare. Leiomyosarcoma of the hypopharynx has been reported only four times in the English medical literature [3–6]. The rarity of a leiomyosarcoma in the hypopharynx can be attributed to the limited smooth muscle tissue in the hypopharynx. A possible candidate for the origin of leiomyosarcoma in the hypopharynx is the smooth muscle surrounding blood vessels. Among the four cases presented in these literatures, two were located in the posterior wall of the hypopharynx, one in the pyriform sinus, and one in the postcricoid. The age at onset was 39–65 years (mean: 55.4 years). The initial symptoms are, first, dysphagia, and second, hoarseness. No metastasis of the regional lymph nodes at the first examinations were reported in any of these four cases. A definitive diagnosis must be confirmed by not only H&E staining, but also immunohistochemical staining and electron microscopic examination, to differentiate a leiomyosarcoma from a leiomyoma, rhabdomyosarcoma,
and spindle cell carcinoma [7,8]. Leiomyosarcoma is positive for vimentin and smooth muscle actin, and it is usually positive for desmin [9,10]. This tumor is negative for cytokeratin, which is useful for differentiating it from a spindle cell carcinoma [11]. The tumor is usually, but not always, negative for S-100 protein [10]. Rhabdomyosarcoma is positive for myogenin, as a marker of skeletal muscle. Leiomyosarcoma is occasionally positive for myogenin, which is noteworthy [10]. On electron microscopy, identification of myofilaments and confirmation of the formation of a basal lamina is important. Z banding and sarcomeres, which imply a striated-muscle structure, are not present in a leiomyosarcoma through electron microscopy. Surgical treatment is the first option for leiomyosarcoma [7,10,12]. It has been reported that no prophylactic neck dissection is usually required, because lymph node metastasis does not generally accompany leiomyosarcoma [7,12]. Surgery was performed in all of the four cases of leiomyosarcoma in the hypopharynx reported in the literature; however, different surgical approaches were employed. Moreover, this small number of cases cannot be seen as establishing a standard therapy for the hypopharynx. Although the efficacy of adjuvant radiotherapy is unknown for leiomyosarcoma of the head and neck, prospective randomized trials of soft tissue sarcomas of the extremities have shown that combined therapy of surgery and radiation is more effective than surgical treatment alone [13–15]. According to these reports, combined therapy of complete surgery and postoperative radiation was recommended [8]; however, the cause of death in leiomyosarcoma has been reported to be due to distant metastasis [10]. Further studies on preventing distant metastasis in leiomyosarcoma are needed.
306
T. Takahashi et al. / Auris Nasus Larynx 35 (2008) 304–307
Fig. 3. H&E staining: dense infiltration by spindle-shaped and/or roundlike cells can be seen with no fissuration, a small number of irregular-sized nuclei, and condensed chromatin.
Fig. 4. Immunohistochemical staining: positive for alpha-smooth muscle actin.
Fig. 2. Upper: CT imaging. The non-uniform tumor (lesion) can be seen at the postcricoid. Lower: T1-weighted MRI. The high-signal image shows a relatively clear border.
References [1] Barnes L. Tumors and tumorlike lesions of the soft tissues. In: Barnes L, editor. Surgical pathology of the head and neck. 3rd ed., New York, NY: Marcel Dekker; 1985. p. 725–880. [2] Goldberg SH, Hanft K, Ossakow SJ. Pathologic quiz case 1. Leiomyosarcoma. Arch Otolaryngol Head Neck Surg 1988;114:1330–2. [3] Fuller AM, Van Vliet PD, Lillie JC, Devine KD. Pharyngeal leiomyosarcoma with fever of unknown origin. Arch Otolaryngol 1966;84:96–8.
[4] Mankodi RC, Shah SS, Kanvinde MS, Joshi JS. Pharyngeal leiomyosarcoma. J Laryngol Otol 1970;84:327–30. [5] Glover GW, Park WW. Pharyngeal leiomyosarcoma. J Laryngol Otol 1971;85:1031–8. [6] Tommerup B, Mogensen C, Nielsen AL. A leiomyosarcoma of the hypopharynx. J Laryngol Otol 1985;99:605–8. [7] Kainuma K, Kikukawa M, Itoh T, Osawa M, Watanabe M. Leiomyosarcoma of the larynx: emergency tracheostomy. J Laryngol Otol 2001;115:570–2. [8] Akcam T, Oysul K, Birkent H, Gerek M, Yetiser S. Leiomyosarcoma of the head and neck: report of two cases and review of the literature. Auris Nasus Larynx 2005;32:209–12. [9] Marioni G, Staffieri C, Marino F, Staffieri A. Leiomyosarcoma of the larynx: critical analysis of the diagnostic role played by immunohistochemistry. Am J Otolaryngol 2005;26:201–6. [10] Montgomery E, Goldblum JR, Fisher C. Leiomyosarcoma of the head and neck: a clinicopathological study. Histopathology 2002;40:518–25. [11] McKiernan DC, Watters GW. Smooth muscle tumours of the larynx. J Laryngol Otol 1995;109:77–9.
T. Takahashi et al. / Auris Nasus Larynx 35 (2008) 304–307 [12] Mindell RS, Calcaterra TC, Ward PH. Leiomyosarcoma of the head and neck: a review of the literature and report of two cases. Laryngoscope 1975;85:904–10. [13] Pisters PW, Harrison LB, Leung DH, Woodruff JM, Casper ES, Brennan MF. Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. J Clin Oncol 1996;14:859–68.
307
[14] Yang JC, Chang AE, Baker AR, Sindelar WF, Danforth DN, Topalian SL, et al. Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 1998;16:197–203. [15] Suit HD, Mankin HJ, Wood WC, Gebhardt MC, Harmon DC, Rosenberg A, et al. Treatment of the patient with stage M0 soft tissue sarcoma. J Clin Oncol 1988;6:854–62.
Leiomyosarcoma of the hypopharynx: A case report Toshitaka Takahashi, Ryuzaburo Higo *, Hiroto Nakata, Masashi Sugasawa Department of Otolaryngology, Head and Neck Surgery, Saitama Medical University, Morohongo 38, Moroyama-cho, Iruma-gun, Saitama 350-0495, Japan Received 30 November 2006; accepted 17 October 2007 Available online 6 February 2008
Abstract Cases of leiomyosarcoma in the head and neck are very rare with only four reports of a leiomyosarcoma originating in the hypopharynx. A rare case of leiomyosarcoma of the hypopharynx is described. A 62-year-old woman, who had experienced slight difficulty of swallowing and hoarseness, visited our hospital. A smooth-surfaced tumor was found extending from the postcricoid to an area in the left pyriform sinus, and the vocal cord on the left side was fixed. Imaging studies, including FDG-PET CT imaging showed the tumor in the postcricoid, but no distinguishable distant metastases. A total laryngectomy with a left-lobe thyroidectomy was performed. Since no metastases of the regional lymph nodes were found by imaging, a neck dissection was not performed. Dense infiltration by spindle-shaped and/or round-like cells was observed histologically as the predominant growth pattern. In immunohistochemical staining, the tumor was positive for desmin and muscle actin, while it was negative for myoglobin, myogenin, cytokeratin, and S-100 protein. Electron microscopic examination did not show Z banding and sarcomeres. Those features lead to the diagnosis of a leiomyosarcoma. No recurrence has been found at 1 year after the surgery. Immunohistochemical staining and electron microscopic examination are essential for a definite diagnosis of leiomyosarcoma. # 2007 Elsevier Ireland Ltd. All rights reserved. Keywords: Leiomyosarcoma; Hypopharynx; Immunohistochemical staining; Electron microscopic examination
1. Introduction Leiomyosarcoma is a malignant tumor derived from smooth muscle that accounts for 5–6% of all soft tissue sarcomas [1]. Leiomyosarcoma usually occurs in soft tissue of the extremities and trunk; only 3% of leiomyosarcomas are in the head and neck [2]. In the head and neck region, most leiomyosarcomas occur in the nasal cavity and paranasal sinuses, mouth, and larynx, while only four cases of leiomyosarcoma of the hypopharynx have been reported. We describe the fifth reported case of leiomyosarcoma in the hypopharynx.
2. Case report A 62-year-old woman, who had experienced slight difficulty of swallowing for 2 years, visited our hospital. She * Corresponding author. Tel.: +81 49 276 1253; fax: +81 49 295 8061. E-mail address: [email protected] (R. Higo).
also complained of hoarseness of several months in duration. An endoscopic laryngeal examination revealed a smoothsurfaced tumor extending from the postcricoid to an area of the left pyriform sinus, and the vocal cord on the left side was fixed (Fig. 1). CT imaging showed a non-uniform contrast enhanced tumor in the postcricoid region, and T1weighted MRI imaging of the tumor was high intensity with a relatively clear border (Fig. 2). FDG-PET CT imaging (2deoxy-2-[(18)F]-fluoro-D-glucose positron emission tomography, combined with CT images) revealed relatively high standardized uptake values (SUV: 3.8) corresponding to the tumor, but no distinguishable distant metastases. A biopsy performed under microlaryngoscopic surgery resulted in a diagnosis of spindle cell sarcoma. Since the tumor was mainly located in the postcricoid region, a total laryngectomy with a left-lobe thyroidectomy was performed. Neck dissection was not performed, because no regional lymph node metastases were found on sequential imaging studies, such as CT, MRI, and FDG-PET CT. Histopathological examination of the resected material showed dense infiltration by spindle-shaped and/or round-
0385-8146/$ – see front matter # 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.anl.2007.10.002
T. Takahashi et al. / Auris Nasus Larynx 35 (2008) 304–307
305
Fig. 1. The smooth-surfaced tumor can be seen extending from the postcricoid to an area in the left pyriform sinus (arrow). The vocal cord on the left side is fixed (left, inspiration; right, phonation).
like cells as the predominant growth pattern (Fig. 3). On immunohistochemical staining, the tumor was positive for desmin, and muscle actin (Fig. 4), while it was negative for myoglobin, myogenin, cytokeratin, and S-100 protein. Electron microscopic examination did not show Z banding and sarcomeres, which implied a structure of striated muscle. The tumor was diagnosed as a leiomyosarcoma. Since the surgical margins were negative, no postsurgical treatment was given. The patient is being followed up with no recurrence at 1 year after the surgery.
3. Discussion Cases of leiomyosarcoma originating in the head and neck region are very rare. Leiomyosarcoma of the hypopharynx has been reported only four times in the English medical literature [3–6]. The rarity of a leiomyosarcoma in the hypopharynx can be attributed to the limited smooth muscle tissue in the hypopharynx. A possible candidate for the origin of leiomyosarcoma in the hypopharynx is the smooth muscle surrounding blood vessels. Among the four cases presented in these literatures, two were located in the posterior wall of the hypopharynx, one in the pyriform sinus, and one in the postcricoid. The age at onset was 39–65 years (mean: 55.4 years). The initial symptoms are, first, dysphagia, and second, hoarseness. No metastasis of the regional lymph nodes at the first examinations were reported in any of these four cases. A definitive diagnosis must be confirmed by not only H&E staining, but also immunohistochemical staining and electron microscopic examination, to differentiate a leiomyosarcoma from a leiomyoma, rhabdomyosarcoma,
and spindle cell carcinoma [7,8]. Leiomyosarcoma is positive for vimentin and smooth muscle actin, and it is usually positive for desmin [9,10]. This tumor is negative for cytokeratin, which is useful for differentiating it from a spindle cell carcinoma [11]. The tumor is usually, but not always, negative for S-100 protein [10]. Rhabdomyosarcoma is positive for myogenin, as a marker of skeletal muscle. Leiomyosarcoma is occasionally positive for myogenin, which is noteworthy [10]. On electron microscopy, identification of myofilaments and confirmation of the formation of a basal lamina is important. Z banding and sarcomeres, which imply a striated-muscle structure, are not present in a leiomyosarcoma through electron microscopy. Surgical treatment is the first option for leiomyosarcoma [7,10,12]. It has been reported that no prophylactic neck dissection is usually required, because lymph node metastasis does not generally accompany leiomyosarcoma [7,12]. Surgery was performed in all of the four cases of leiomyosarcoma in the hypopharynx reported in the literature; however, different surgical approaches were employed. Moreover, this small number of cases cannot be seen as establishing a standard therapy for the hypopharynx. Although the efficacy of adjuvant radiotherapy is unknown for leiomyosarcoma of the head and neck, prospective randomized trials of soft tissue sarcomas of the extremities have shown that combined therapy of surgery and radiation is more effective than surgical treatment alone [13–15]. According to these reports, combined therapy of complete surgery and postoperative radiation was recommended [8]; however, the cause of death in leiomyosarcoma has been reported to be due to distant metastasis [10]. Further studies on preventing distant metastasis in leiomyosarcoma are needed.
306
T. Takahashi et al. / Auris Nasus Larynx 35 (2008) 304–307
Fig. 3. H&E staining: dense infiltration by spindle-shaped and/or roundlike cells can be seen with no fissuration, a small number of irregular-sized nuclei, and condensed chromatin.
Fig. 4. Immunohistochemical staining: positive for alpha-smooth muscle actin.
Fig. 2. Upper: CT imaging. The non-uniform tumor (lesion) can be seen at the postcricoid. Lower: T1-weighted MRI. The high-signal image shows a relatively clear border.
References [1] Barnes L. Tumors and tumorlike lesions of the soft tissues. In: Barnes L, editor. Surgical pathology of the head and neck. 3rd ed., New York, NY: Marcel Dekker; 1985. p. 725–880. [2] Goldberg SH, Hanft K, Ossakow SJ. Pathologic quiz case 1. Leiomyosarcoma. Arch Otolaryngol Head Neck Surg 1988;114:1330–2. [3] Fuller AM, Van Vliet PD, Lillie JC, Devine KD. Pharyngeal leiomyosarcoma with fever of unknown origin. Arch Otolaryngol 1966;84:96–8.
[4] Mankodi RC, Shah SS, Kanvinde MS, Joshi JS. Pharyngeal leiomyosarcoma. J Laryngol Otol 1970;84:327–30. [5] Glover GW, Park WW. Pharyngeal leiomyosarcoma. J Laryngol Otol 1971;85:1031–8. [6] Tommerup B, Mogensen C, Nielsen AL. A leiomyosarcoma of the hypopharynx. J Laryngol Otol 1985;99:605–8. [7] Kainuma K, Kikukawa M, Itoh T, Osawa M, Watanabe M. Leiomyosarcoma of the larynx: emergency tracheostomy. J Laryngol Otol 2001;115:570–2. [8] Akcam T, Oysul K, Birkent H, Gerek M, Yetiser S. Leiomyosarcoma of the head and neck: report of two cases and review of the literature. Auris Nasus Larynx 2005;32:209–12. [9] Marioni G, Staffieri C, Marino F, Staffieri A. Leiomyosarcoma of the larynx: critical analysis of the diagnostic role played by immunohistochemistry. Am J Otolaryngol 2005;26:201–6. [10] Montgomery E, Goldblum JR, Fisher C. Leiomyosarcoma of the head and neck: a clinicopathological study. Histopathology 2002;40:518–25. [11] McKiernan DC, Watters GW. Smooth muscle tumours of the larynx. J Laryngol Otol 1995;109:77–9.
T. Takahashi et al. / Auris Nasus Larynx 35 (2008) 304–307 [12] Mindell RS, Calcaterra TC, Ward PH. Leiomyosarcoma of the head and neck: a review of the literature and report of two cases. Laryngoscope 1975;85:904–10. [13] Pisters PW, Harrison LB, Leung DH, Woodruff JM, Casper ES, Brennan MF. Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. J Clin Oncol 1996;14:859–68.
307
[14] Yang JC, Chang AE, Baker AR, Sindelar WF, Danforth DN, Topalian SL, et al. Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 1998;16:197–203. [15] Suit HD, Mankin HJ, Wood WC, Gebhardt MC, Harmon DC, Rosenberg A, et al. Treatment of the patient with stage M0 soft tissue sarcoma. J Clin Oncol 1988;6:854–62.