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Well-differentiated, pedunculated liposarcoma of the hypopharynx
G Model
ARTICLE IN PRESS
ANORL-691; No. of Pages 3
European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2017) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
Case report
Well-differentiated, pedunculated liposarcoma of the hypopharynx C. Eyermann ∗ , T. Raguin , P. Hemar , C. Debry Service d’ORL et de chirurgie cervico-faciale, HUS, hôpital de Hautepierre, avenue Molière, 67000 Strasbourg, France
a r t i c l e
i n f o
Keywords: Well-differentiated liposarcoma Hypopharyngeal neoplasms Hypopharynx Otorhinolaryngological surgical procedures
a b s t r a c t Introduction: Liposarcoma of the hypopharynx is extremely rare, as only 28 cases have been reported in the literature. The cardinal symptom of liposarcoma is progressively worsening dysphagia. Case report: The authors report the case of a 71-year-old man who presented with dysphagia, marked weight loss over several weeks and an episode of exteriorization of a solid mass from the mouth during an episode of vomiting. Imaging revealed a fat density intra-oesophageal mass. Panendoscopy and upper gastrointestinal endoscopy visualized the pedunculated tumour in the left piriform sinus, which was able to be exteriorized via the mouth. The tumour was then resected endoscopically at its hypopharyngeal insertion pedicle. Histological examination of the operative specimen concluded on well-differentiated benign liposarcoma. Discussion: Well-differentiated liposarcoma is the most common form of liposarcoma, but is only exceptionally reported in the hypopharynx. The main symptoms are related to compression of adjacent structures. Imaging findings are nonspecific. Only histological examination can distinguish liposarcoma from other benign oesophageal tumours. Standard treatment consists of wide, complete resection, which is not always possible in the neck. Long-term follow-up of these patients is essential in order to rapidly detect recurrence. © 2017 Elsevier Masson SAS. All rights reserved.
1. Introduction Liposarcoma is the most common form of soft tissue sarcoma. It is usually localized in the retroperitoneum and extremities, and is uncommon in the neck [1], and extremely rare in the hypopharynx: only 28 cases have been reported in the international literature since 1979 [2,3]. The cardinal symptom of this disease is progressively worsening dysphagia. We report a case of well-differentiated, pedunculated liposarcoma of the hypopharynx, in which a diagnosis of oesophageal polyp was initially proposed.
2. Case report A 71-year-old man, with a history of hypertension and noninsulin-dependent diabetes, consulted for progressively worsening dysphagia to solids, responsible for weight loss of 15 kg over several weeks. He also described an episode of vomiting with very brief expulsion of a solid mass from the mouth.
∗ Corresponding author. E-mail address: [email protected] (C. Eyermann).
An upper gastrointestinal contrast study demonstrated dilatation of the proximal two-thirds of the oesophagus, suggesting an intraluminal tumour rather than extrinsic compression. Contrast-enhanced neck and chest computed tomography (CT) and magnetic resonance imaging (MRI) revealed a 16-centimetre long, circumscribed adipose tumour of the oesophagus with a left posterolateral implantation base, compatible with the diagnosis of oesophageal lipoma (Fig. 1). Panendoscopy and upper gastrointestinal endoscopy were performed, revealing an intra-oesophageal tumour, extending as far as 35 cm from the dental arch, allowing it to be expelled from the mouth (Appendix 1). The tumour was sectioned with a scalpel at the base of its pedicle, situated in the left piriform sinus, via the hypopharyngoscope. The macroscopic appearance of the tumour was that of a polyp with a smooth surface, measuring 10 × 5 × 4 cm comprising a 5-cm long pedicle (Fig. 2). Histological examination concluded on well-differentiated liposarcoma, composed of mature adipocytes and CD34+, HMGA2+ spindle cells, with positive MDM2 gene amplification. Follow-up endoscopy performed 4 months later revealed no signs of recurrence. Six months after the first operation, the patient was asymptomatic. Follow-up imaging and endoscopy will be performed at 1 year.
http://dx.doi.org/10.1016/j.anorl.2017.07.001 1879-7296/© 2017 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Eyermann C, et al. Well-differentiated, pedunculated liposarcoma of the hypopharynx. European Annals of Otorhinolaryngology, Head and Neck diseases (2017), http://dx.doi.org/10.1016/j.anorl.2017.07.001
G Model ANORL-691; No. of Pages 3
ARTICLE IN PRESS C. Eyermann et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2017) xxx–xxx
2
Fig. 1. Well circumscribed fat density tumour following the course of the oesophagus with a hypopharyngeal implantation base: a: neck and chest MRI, T2 Fat-Sat sequence, frontal section; b: neck and chest MRI, T2 Fat-Sat sequence, sagittal section; c: contrast-enhanced neck and chest CT scan, frontal section; d: contrast-enhanced neck and chest CT scan, sagittal section.
Fig. 2. a and b: intraoperative views after extraction of the tumour by the mouth.
3. Discussion Liposarcomas are malignant soft tissue tumours with adipocyte differentiation. They mainly arise in the deep soft tissues of the limbs (especially the thigh) and retroperitoneum, in the form of a painless, slowly growing mass. They have very rarely been described in the head and neck [1,4,5]. They are classified
into 5 histological groups: well-differentiated, myxoid or round cells, pleiomorphic, poorly differentiated and mixed [6]. Welldifferentiated liposarcomas are by far the most common form (40–45% of liposarcomas). Liposarcoma of the hypopharynx is an extremely rare entity. According to a review of the literature conducted by Corvino et al. in 2016, only 28 cases of liposarcoma of the hypopharynx had been
Please cite this article in press as: Eyermann C, et al. Well-differentiated, pedunculated liposarcoma of the hypopharynx. European Annals of Otorhinolaryngology, Head and Neck diseases (2017), http://dx.doi.org/10.1016/j.anorl.2017.07.001
G Model ANORL-691; No. of Pages 3
ARTICLE IN PRESS C. Eyermann et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2017) xxx–xxx
reported in the international literature [2], mostly arising from the piriform sinus, as in the case reported here. Clinically, the main symptoms are related to compression of adjacent anatomical structures: the most characteristic feature is progressively worsening dysphagia with weight loss that may be present for months before the diagnosis. Episodes of expulsion of the tumour from the mouth, and even asphyxiation due to upper airway obstruction, have sometimes been described [7,8]. The unusual feature of the case reported here is the large size of the liposarcoma, which was 15 cm long, while the mean length reported in the literature is 7.60 cm [2]. Hypopharyngeal liposarcoma is usually polypoid and pedunculated, which can be suggestive of a diagnosis of benign oesophageal polyp on the imaging assessment. On computed tomography and MRI, liposarcoma presents as a fat density mass with regular contours. The fat signal is more intense with the increasing degree of differentiation of the tumour [2,5]. On imaging, well-differentiated liposarcoma of the hypopharynx can be easily confused with other benign tumours, especially giant oesophageal polyp, corresponding to various histological diagnoses (lipoma, fibroma, fibrolipoma, myxolipoma, leiomyoma, fibrosarcoma or leiomyosarcoma). These various oesophageal and hypopharyngeal adipose tumours can only be distinguished by histological examination. The standard treatment of well-differentiated liposarcoma is surgical resection [2]. Lymph node dissection is not recommended, as well-differentiated liposarcomas do not metastasize [1,9]. Wide resection is rarely possible in the neck without inducing severe functional sequelae. Radiotherapy may be indicated in the case of incomplete resection or inoperable tumours [1,4,5]. There are two surgical approaches to the treatment of hypopharyngeal liposarcoma: open neck incision (lateral pharyngotomy) or endoscopy. Endoscopic laser or scalpel resection allows a reduction of postoperative morbidity (decreased length of hospital stay, rapid resumption of feeding, no need for tracheotomy), but only allows limited resection with surgical margins that are difficult to evaluate. Lateral pharyngotomy allows wide resection with good control of surgical margins, but with a potentially more complicated postoperative course (risk of fistulas, need for drainage, later resumption of feeding) [10]. The main risk associated with liposarcoma is local recurrence, especially in the case of incomplete resection [9], independently of the technique used [2,10]. However, well-differentiated liposarcomas are slowly growing, low-grade malignant tumours with a 5-year survival rate of 100% despite recurrences [1]. Loss of differentiation of the liposarcoma [2,5] should be suspected in the case of repeated recurrences.
3
Long-term follow-up of these patients by clinical, endoscopic or even radiological surveillance for at least 5 years is essential [2]. 4. Conclusion Liposarcoma of the hypopharynx is a rare entity that must be considered in a context of benign tumour of the oesophagus, presenting in the form of progressively worsening dysphagia. A preoperative histological diagnosis allows the largest possible primary resection, by endoscopic or open surgery. Clinical, endoscopic and radiological surveillance is essential due to the high potential for local recurrence. Disclosure of interest The authors declare that they have no competing interest. Appendix 1. Supplementary data Supplementary data (Video) associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/ j.anorl.2017.07.001. References [1] Golledge J, Fisher C, Rhys-Evans PH. Head and neck liposarcoma. Cancer 1995;76(6):1051–8. [2] Corvino A, Riva G, Sensini M, Garzaro M, Pecorari G. Liposarcomas of the hypopharynx: a systematic review of the literature. J Health Soc Sci 2016;1(1):57–66. [3] Frey-Schlottmann ML, Stiens R. Liposarkom im Hypopharynxbereich. Arch Otorhinolaryngol 1979;223(2–4):419–22. [4] Gritli S, Khamassi K, Lachkhem A, et al. Head and neck liposarcomas: a 32 years experience. Auris Nasus Larynx 2010;37(3):347–51. [5] Nouri H, Hassani R, Aderdour L, Raji A. Liposarcome bien différencié de l’hypopharynx. Ann Fr Otorhinolaryngol Pathol Cervicofac 2011;128(3):162–4. [6] World Health Organization, International Agency for Research on Cancer. WHO classification of tumours of soft tissue and bone, 4th ed. Lyon: IARC Press; 2006 [Volume 5]. [7] Bergmark R, Jung D, Vakharia K, Gray ST, Lin DT, Rosenberg AE. Pathology quiz case 1. Liposarcoma of the hypopharynx. Arch Otolaryngol Head Neck Surg 2012;138(3):313–5. [8] Takiguchi G, Nakamura T, Otowa Y, et al. Successful resection of giant esophageal liposarcoma by endoscopic submucosal dissection combined with surgical retrieval: a case report and literature review. Surg Case Rep 2016;2(1):90. [9] McCulloch TM, Makielski KH, McNutt MA. Head and neck liposarcoma. A histopathologic reevaluation of reported cases. Arch Otolaryngol Head Neck Surg 1992;118(10):1045–9. [10] Luna-Ortiz K, Campos-Ramos E, Carmona-Luna T, Mohar-Betancourt A, FerrariCarballo T. Laser resection of liposarcoma of the hypopharynx. Med Oral Patol Oral Cirugia Bucal 2009;14(5):E252–6.
Please cite this article in press as: Eyermann C, et al. Well-differentiated, pedunculated liposarcoma of the hypopharynx. European Annals of Otorhinolaryngology, Head and Neck diseases (2017), http://dx.doi.org/10.1016/j.anorl.2017.07.001
ARTICLE IN PRESS
ANORL-691; No. of Pages 3
European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2017) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
Case report
Well-differentiated, pedunculated liposarcoma of the hypopharynx C. Eyermann ∗ , T. Raguin , P. Hemar , C. Debry Service d’ORL et de chirurgie cervico-faciale, HUS, hôpital de Hautepierre, avenue Molière, 67000 Strasbourg, France
a r t i c l e
i n f o
Keywords: Well-differentiated liposarcoma Hypopharyngeal neoplasms Hypopharynx Otorhinolaryngological surgical procedures
a b s t r a c t Introduction: Liposarcoma of the hypopharynx is extremely rare, as only 28 cases have been reported in the literature. The cardinal symptom of liposarcoma is progressively worsening dysphagia. Case report: The authors report the case of a 71-year-old man who presented with dysphagia, marked weight loss over several weeks and an episode of exteriorization of a solid mass from the mouth during an episode of vomiting. Imaging revealed a fat density intra-oesophageal mass. Panendoscopy and upper gastrointestinal endoscopy visualized the pedunculated tumour in the left piriform sinus, which was able to be exteriorized via the mouth. The tumour was then resected endoscopically at its hypopharyngeal insertion pedicle. Histological examination of the operative specimen concluded on well-differentiated benign liposarcoma. Discussion: Well-differentiated liposarcoma is the most common form of liposarcoma, but is only exceptionally reported in the hypopharynx. The main symptoms are related to compression of adjacent structures. Imaging findings are nonspecific. Only histological examination can distinguish liposarcoma from other benign oesophageal tumours. Standard treatment consists of wide, complete resection, which is not always possible in the neck. Long-term follow-up of these patients is essential in order to rapidly detect recurrence. © 2017 Elsevier Masson SAS. All rights reserved.
1. Introduction Liposarcoma is the most common form of soft tissue sarcoma. It is usually localized in the retroperitoneum and extremities, and is uncommon in the neck [1], and extremely rare in the hypopharynx: only 28 cases have been reported in the international literature since 1979 [2,3]. The cardinal symptom of this disease is progressively worsening dysphagia. We report a case of well-differentiated, pedunculated liposarcoma of the hypopharynx, in which a diagnosis of oesophageal polyp was initially proposed.
2. Case report A 71-year-old man, with a history of hypertension and noninsulin-dependent diabetes, consulted for progressively worsening dysphagia to solids, responsible for weight loss of 15 kg over several weeks. He also described an episode of vomiting with very brief expulsion of a solid mass from the mouth.
∗ Corresponding author. E-mail address: [email protected] (C. Eyermann).
An upper gastrointestinal contrast study demonstrated dilatation of the proximal two-thirds of the oesophagus, suggesting an intraluminal tumour rather than extrinsic compression. Contrast-enhanced neck and chest computed tomography (CT) and magnetic resonance imaging (MRI) revealed a 16-centimetre long, circumscribed adipose tumour of the oesophagus with a left posterolateral implantation base, compatible with the diagnosis of oesophageal lipoma (Fig. 1). Panendoscopy and upper gastrointestinal endoscopy were performed, revealing an intra-oesophageal tumour, extending as far as 35 cm from the dental arch, allowing it to be expelled from the mouth (Appendix 1). The tumour was sectioned with a scalpel at the base of its pedicle, situated in the left piriform sinus, via the hypopharyngoscope. The macroscopic appearance of the tumour was that of a polyp with a smooth surface, measuring 10 × 5 × 4 cm comprising a 5-cm long pedicle (Fig. 2). Histological examination concluded on well-differentiated liposarcoma, composed of mature adipocytes and CD34+, HMGA2+ spindle cells, with positive MDM2 gene amplification. Follow-up endoscopy performed 4 months later revealed no signs of recurrence. Six months after the first operation, the patient was asymptomatic. Follow-up imaging and endoscopy will be performed at 1 year.
http://dx.doi.org/10.1016/j.anorl.2017.07.001 1879-7296/© 2017 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Eyermann C, et al. Well-differentiated, pedunculated liposarcoma of the hypopharynx. European Annals of Otorhinolaryngology, Head and Neck diseases (2017), http://dx.doi.org/10.1016/j.anorl.2017.07.001
G Model ANORL-691; No. of Pages 3
ARTICLE IN PRESS C. Eyermann et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2017) xxx–xxx
2
Fig. 1. Well circumscribed fat density tumour following the course of the oesophagus with a hypopharyngeal implantation base: a: neck and chest MRI, T2 Fat-Sat sequence, frontal section; b: neck and chest MRI, T2 Fat-Sat sequence, sagittal section; c: contrast-enhanced neck and chest CT scan, frontal section; d: contrast-enhanced neck and chest CT scan, sagittal section.
Fig. 2. a and b: intraoperative views after extraction of the tumour by the mouth.
3. Discussion Liposarcomas are malignant soft tissue tumours with adipocyte differentiation. They mainly arise in the deep soft tissues of the limbs (especially the thigh) and retroperitoneum, in the form of a painless, slowly growing mass. They have very rarely been described in the head and neck [1,4,5]. They are classified
into 5 histological groups: well-differentiated, myxoid or round cells, pleiomorphic, poorly differentiated and mixed [6]. Welldifferentiated liposarcomas are by far the most common form (40–45% of liposarcomas). Liposarcoma of the hypopharynx is an extremely rare entity. According to a review of the literature conducted by Corvino et al. in 2016, only 28 cases of liposarcoma of the hypopharynx had been
Please cite this article in press as: Eyermann C, et al. Well-differentiated, pedunculated liposarcoma of the hypopharynx. European Annals of Otorhinolaryngology, Head and Neck diseases (2017), http://dx.doi.org/10.1016/j.anorl.2017.07.001
G Model ANORL-691; No. of Pages 3
ARTICLE IN PRESS C. Eyermann et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2017) xxx–xxx
reported in the international literature [2], mostly arising from the piriform sinus, as in the case reported here. Clinically, the main symptoms are related to compression of adjacent anatomical structures: the most characteristic feature is progressively worsening dysphagia with weight loss that may be present for months before the diagnosis. Episodes of expulsion of the tumour from the mouth, and even asphyxiation due to upper airway obstruction, have sometimes been described [7,8]. The unusual feature of the case reported here is the large size of the liposarcoma, which was 15 cm long, while the mean length reported in the literature is 7.60 cm [2]. Hypopharyngeal liposarcoma is usually polypoid and pedunculated, which can be suggestive of a diagnosis of benign oesophageal polyp on the imaging assessment. On computed tomography and MRI, liposarcoma presents as a fat density mass with regular contours. The fat signal is more intense with the increasing degree of differentiation of the tumour [2,5]. On imaging, well-differentiated liposarcoma of the hypopharynx can be easily confused with other benign tumours, especially giant oesophageal polyp, corresponding to various histological diagnoses (lipoma, fibroma, fibrolipoma, myxolipoma, leiomyoma, fibrosarcoma or leiomyosarcoma). These various oesophageal and hypopharyngeal adipose tumours can only be distinguished by histological examination. The standard treatment of well-differentiated liposarcoma is surgical resection [2]. Lymph node dissection is not recommended, as well-differentiated liposarcomas do not metastasize [1,9]. Wide resection is rarely possible in the neck without inducing severe functional sequelae. Radiotherapy may be indicated in the case of incomplete resection or inoperable tumours [1,4,5]. There are two surgical approaches to the treatment of hypopharyngeal liposarcoma: open neck incision (lateral pharyngotomy) or endoscopy. Endoscopic laser or scalpel resection allows a reduction of postoperative morbidity (decreased length of hospital stay, rapid resumption of feeding, no need for tracheotomy), but only allows limited resection with surgical margins that are difficult to evaluate. Lateral pharyngotomy allows wide resection with good control of surgical margins, but with a potentially more complicated postoperative course (risk of fistulas, need for drainage, later resumption of feeding) [10]. The main risk associated with liposarcoma is local recurrence, especially in the case of incomplete resection [9], independently of the technique used [2,10]. However, well-differentiated liposarcomas are slowly growing, low-grade malignant tumours with a 5-year survival rate of 100% despite recurrences [1]. Loss of differentiation of the liposarcoma [2,5] should be suspected in the case of repeated recurrences.
3
Long-term follow-up of these patients by clinical, endoscopic or even radiological surveillance for at least 5 years is essential [2]. 4. Conclusion Liposarcoma of the hypopharynx is a rare entity that must be considered in a context of benign tumour of the oesophagus, presenting in the form of progressively worsening dysphagia. A preoperative histological diagnosis allows the largest possible primary resection, by endoscopic or open surgery. Clinical, endoscopic and radiological surveillance is essential due to the high potential for local recurrence. Disclosure of interest The authors declare that they have no competing interest. Appendix 1. Supplementary data Supplementary data (Video) associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/ j.anorl.2017.07.001. References [1] Golledge J, Fisher C, Rhys-Evans PH. Head and neck liposarcoma. Cancer 1995;76(6):1051–8. [2] Corvino A, Riva G, Sensini M, Garzaro M, Pecorari G. Liposarcomas of the hypopharynx: a systematic review of the literature. J Health Soc Sci 2016;1(1):57–66. [3] Frey-Schlottmann ML, Stiens R. Liposarkom im Hypopharynxbereich. Arch Otorhinolaryngol 1979;223(2–4):419–22. [4] Gritli S, Khamassi K, Lachkhem A, et al. Head and neck liposarcomas: a 32 years experience. Auris Nasus Larynx 2010;37(3):347–51. [5] Nouri H, Hassani R, Aderdour L, Raji A. Liposarcome bien différencié de l’hypopharynx. Ann Fr Otorhinolaryngol Pathol Cervicofac 2011;128(3):162–4. [6] World Health Organization, International Agency for Research on Cancer. WHO classification of tumours of soft tissue and bone, 4th ed. Lyon: IARC Press; 2006 [Volume 5]. [7] Bergmark R, Jung D, Vakharia K, Gray ST, Lin DT, Rosenberg AE. Pathology quiz case 1. Liposarcoma of the hypopharynx. Arch Otolaryngol Head Neck Surg 2012;138(3):313–5. [8] Takiguchi G, Nakamura T, Otowa Y, et al. Successful resection of giant esophageal liposarcoma by endoscopic submucosal dissection combined with surgical retrieval: a case report and literature review. Surg Case Rep 2016;2(1):90. [9] McCulloch TM, Makielski KH, McNutt MA. Head and neck liposarcoma. A histopathologic reevaluation of reported cases. Arch Otolaryngol Head Neck Surg 1992;118(10):1045–9. [10] Luna-Ortiz K, Campos-Ramos E, Carmona-Luna T, Mohar-Betancourt A, FerrariCarballo T. Laser resection of liposarcoma of the hypopharynx. Med Oral Patol Oral Cirugia Bucal 2009;14(5):E252–6.
Please cite this article in press as: Eyermann C, et al. Well-differentiated, pedunculated liposarcoma of the hypopharynx. European Annals of Otorhinolaryngology, Head and Neck diseases (2017), http://dx.doi.org/10.1016/j.anorl.2017.07.001