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Dedifferentiated liposarcoma in the maxillary gingiva: A clinical report and review of the literature
G Model JOMSMP-646; No. of Pages 4
ARTICLE IN PRESS Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2017) xxx–xxx
Contents lists available at ScienceDirect
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology journal homepage: www.elsevier.com/locate/jomsmp
Case report
Dedifferentiated liposarcoma in the maxillary gingiva: A clinical report and review of the literature Akifumi Enomoto ∗ , Kazuhide Matsunaga, Takeshi Shimoide, Takao Mukai, Takayuki Uchihashi, Suguru Hamada Department of Oral and Maxillofacial Surgery, Kindai University, Faculty of Medicine, 377-2, Onohigashi, Osaka-Sayama 589-8511, Japan, Japan
a r t i c l e
i n f o
Article history: Received 13 January 2017 Received in revised form 1 May 2017 Accepted 14 June 2017 Available online xxx Keyword: Dedifferentiated liposarcoma Oral cavity Surgery
a b s t r a c t Liposarcoma in the oral cavity is extremely rare, and dedifferentiated liposarcoma has seldom been reported in the oral region. No clear consensus has been established regarding the management of oral dedifferentiated liposarcoma. The case of a 28-year-old Japanese woman who presented with dedifferentiated liposarcoma in the maxillary gingiva is reported. She complained of swelling of the right maxillary gingiva, and swelling was evident in the region. No lymph nodes were palpable in either side of the neck. Incisional biopsy of the tumor region was performed, revealing dedifferentiated liposarcoma. The patient was treated with maxillectomy, and she has shown no clinical or radiological evidence of local recurrence or distant metastases as of 30 months after the end of treatment. This case provides a basis for the future appropriate management of oral cancer in patients with dedifferentiated liposarcoma. © 2017 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.夽
1. Introduction
2. Case report
Most liposarcomas arise in the extremities and retroperitoneum. Diagnosis and management planning for the staging of patients with liposarcoma of these soft tissue areas has increasingly been reported. However, liposarcomas affecting the oral region are very unusual, accounting for approximately 0.3% of all liposarcomas. These mainly arise on the buccal mucosa or the floor of the mouth, and very few arise in the gingiva. Regarding the pathological features, the atypical lipomatous tumor/well-differentiated (ALT/WD) liposarcoma is identified as the most common subtype, followed by myxoid variants in the oral region. Dedifferentiated liposarcoma is a rare subtype, occurring in about 10% of cases, and it can be defined as juxtaposition of a non-lipogenic sarcoma of variable histological grade and ALT/WD liposarcoma. The issues associated with liposarcoma in the oral region have not been widely discussed, and no clear consensus has been established regarding the management of oral liposarcoma. To address the lack of information on oral liposarcoma, further reports are required. Thus, a case of liposarcoma in the maxillary gingiva is presented along with a review of literature.
A 28-year-old Japanese woman was referred to our clinic in May 2014 complaining of swelling of the right maxillary gingiva.
∗ Corresponding author. E-mail address: [email protected] (A. Enomoto).
Fig. 1. Intraoral findings. Intraoral photographs at initial presentation. Small but considerable swelling in the right maxillary gingiva is evident. The arrowhead showed a swelling lesion in the right maxillary gingiva.
http://dx.doi.org/10.1016/j.ajoms.2017.06.007 2212-5558/© 2017 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.夽
Please cite this article in press as: Enomoto A, et al. Dedifferentiated liposarcoma in the maxillary gingiva: A clinical report and review of the literature. J Oral Maxillofac Surg Med Pathol (2017), http://dx.doi.org/10.1016/j.ajoms.2017.06.007
G Model
ARTICLE IN PRESS
JOMSMP-646; No. of Pages 4
A. Enomoto et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2017) xxx–xxx
2
Fig. 2. CT and MRI assessment. Contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) were performed. CT shows a clear enhanced lesion in the right maxillary gingiva. The arrowhead showed a clear, enhanced lesion of the right maxillary gingiva (A). STIR MR images on initial presentation show a prominent hyperintense lesion in the right maxillary gingiva. The arrowhead showed a clear hyperintensity lesion of the right maxillary gingiva on STIR imaging (B).
A painless small swelling in the right maxillary gingiva was evident (Fig. 1), and no lymph nodes were palpable in either side of the neck. The patient had no other associated disease. Contrastenhanced computed tomography (CT) and magnetic resonance imaging (MRI) were performed. CT evaluation of the right maxillary gingiva showed a clear, enhanced lesion (Fig. 2). On MRI, hyperintensity on short T1 inversion recovery (STIR) images and contrast-enhanced T1-weighted images was observed in the right maxillary gingiva (Fig. 2). Biopsy of the maxillary gingival lesion was performed, revealing dedifferentiated liposarcoma (FNCLCC grading system: G3). Finally, a diagnosis of liposarcoma of the right maxillary gingiva with no lymph node metastasis was established. The tumor was staged as T1aN0M0, stage IIA according to the Union for International Cancer Control (UICC) staging system. After sufficient consultation with an interdisciplinary team and obtaining informed consent from the patient, surgery was selected as the treatment strategy. A right side maxillectomy was performed under general anesthesia. The final diagnosis was dedifferentiated liposarcoma, the same as the initial diagnosis. On pathological examination, the tumor
has two distinct areas represented by a well-differentiated liposarcoma and a dedifferentiated area. Dedifferentiated tumor had non-lipogenic area composed of spindle or fibroblastic cells. These coexistence of a well-differentiated liposarcoma and a nonlipogenic area indicated dedifferentiated liposarcoma (Fig. 3). Immunohistochemical studies showed positivity for S-100, MDM2, and CDK4 and negativity for desmin, ␣SMA, CD34, h-caldesmon, and myogenin. CT, MRI, and PET-CT assessments have been obtained to ensure adequate follow-up, and recurrence and lymph or distant metastases have not been seen 30 months after treatment. 3. Discussion This case report represents the first description of dedifferentiated liposarcoma involving the maxillary gingiva. Liposarcoma is one of the most common soft tissue sarcomas in adults, and it accounts for approximately 20% of all soft tissue malignancies. Diagnosis and management planning for liposarcoma of the soft tissues in the extremities and retroperitoneum
Table 1 Review of the literature: 9 cases of oral dedifferentiated liposarcoma. Authors
Age/Sex
Location/Size(cm)
Treatment
Follow-up information
Diamond et al. Fanburg-Smith et al. Fanburg-Smith et al. Nascimento et al. Werneck da Cunha et al. Gustavo de la Roza et al. Angiero F et al. Henricks et al. Present case
57/M 39/M 56/M 83/M 42/F 61/M 41/F NA 28/F
Cheek/8.0 Tongue/6.0 Cheek/5.0 Tongue/2.5 Cheek/6.0 Cheek/5.0 Maxillary gingiva/1.0 Cheek/NA Maxirally gingiva/2.0
Surg + RT Surg Surg Surg Surg Surg Surg Surg Surg
no REC for 12 months no REC for 72 months 6 RECs in 26 years Lost F/U no REC for 12 months one REC and Lost F/U no Rec for 12 months NA no REC for 24 months
Surg: surgery. RT: radiation therapy. REC: recurrence. F/U: follow-up. NA: not available.
Please cite this article in press as: Enomoto A, et al. Dedifferentiated liposarcoma in the maxillary gingiva: A clinical report and review of the literature. J Oral Maxillofac Surg Med Pathol (2017), http://dx.doi.org/10.1016/j.ajoms.2017.06.007
G Model JOMSMP-646; No. of Pages 4
ARTICLE IN PRESS A. Enomoto et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2017) xxx–xxx
Fig. 3. Pathological findings. The tumor has two different areas represented by a well-differentiated liposarcoma and a dedifferentiated area (upper). Lipogenic area is identical to original tumor (middle). The high-grade dedifferentiated area showed a spindle proliferation consistent with fibrosarcoma (bottom). (H-E x 40).
has been well established. According to the 2002 WHO classification, histological diagnosis of liposarcoma is classified as ALT/WD liposarcoma, myxoid liposarcoma, pleomorphic liposarcoma, differentiated liposarcoma, and mixed-type liposarcoma. Evans reported that the overall survival rate of dedifferentiated liposarcoma was significantly poorer than that of myxoid and ALT/WD liposarcoma because of local recurrence and metastasis [1]. Soft tissue dedifferentiated liposarcomas recur locally in 41%,
3
metastasize in 17%, and cause death from disease in 28% of cases [2]. Liposarcoma of the oral region is unusual, and dedifferentiated liposarcoma is a rare subtype, occurring in about 3% of cases. There are only a few reports suggesting suitable treatment options for oral dedifferentiated liposarcoma. No clear consensus has been established regarding the management of oral dedifferentiated liposarcoma. To the best of our knowledge, only 8 cases of dedifferentiated liposarcoma have been reported in the English language literature [2–8] (Table 1). Local recurrence is a critical factor for survival [9,10]. Cheng et al. reported evaluations of clinical cases of primary liposarcoma in the oral and maxillofacial region, which included 4 dedifferentiated liposarcomas [11]. The recurrence rate of oral dedifferentiated liposarcoma in this report was 25.0%, indicating poorer results than of other subtypes of liposarcoma (18.1%). Among 9 cases of oral dedifferentiated liposarcoma, including the present patient, in the table, the recurrence rate was 22.2% (2/9). On the other hand, Fanburg-Smith reported that oral dedifferentiated liposarcoma does not necessarily have a poor outcome, compared to other subtypes of liposarcoma on clinical evaluation [6]. Indeed, the number of reports of oral dedifferentiated liposarcoma is still very few, and more clinical experience with oral dedifferentiated liposarcoma is needed for better understanding. Regarding treatment, surgical excision with an extensive wide margin is suggested, and the importance of complete excision is generally emphasized. Therefore, extensive resection with a wide margin of the maxillary sinus was performed in the present case. Surgery is the critical therapy that achieves excellent local control. As for non-surgical treatment, there have been several reports about radiation therapy for liposarcoma. In the first case in the table, surgery and postoperative radiation were performed for oral dedifferentiated liposarcoma [5]. This patient was alive 12 months after treatment. However, for the rest of the cases in the table, the treatment was only surgery. This may be because poorly differentiated liposarcoma has low sensitivity to radiation therapy, while myxoid liposarcoma has a predictable response to radiotherapy [12–14]. Furthermore, recent studies suggested that small tumors (less than or equal to 5 cm) are less frequently associated with local recurrence, indicating that radiation therapy may not be necessary for these patients [15–17]. Additionally, there is another report that surgery with radiation therapy does not show improve the 5-year survival rate compared to surgery alone [11]. Radiation treatment modalities generally still have unclear and non-definitive evidence for oral liposarcoma [11,18]. There are several reports about chemotherapy for liposarcoma. Chemotherapy is currently standard therapy for Ewing’s sarcoma or rhabdomyosarcoma. As for liposarcoma, the effect of chemotherapy on survival remains controversial, and it has not been standardized. Eilber et al. reported the impact of ifosfamide-based chemotherapy on large (>10 cm), high-grade, extremity liposarcoma [19]. However, there are almost no reports of chemotherapy for oral liposarcoma. For larynx liposarcoma, as a head and neck region, a combination of chemotherapy, radiation, and surgery was performed. The chemotherapy menu was cyclophosphamide, doxorubicin, dimethyl triazeno imidazole carboxymide (DTIC), and vincristine sulfate. Despite this treatment, the patient died of uncontrolled disease [20]. In the present case, the postoperative margin was tumornegative after extensive surgery, and neither radiation nor chemotherapy was performed. The prognosis or the overall survival rate of liposarcoma has been reported to be related to histological type, and a high survival rate generally cannot be expected with dedifferentiated liposarcoma. The treatment strategy for oral dedifferentiated liposarcoma is not definitive and is poorly known in many respects. A multi-
Please cite this article in press as: Enomoto A, et al. Dedifferentiated liposarcoma in the maxillary gingiva: A clinical report and review of the literature. J Oral Maxillofac Surg Med Pathol (2017), http://dx.doi.org/10.1016/j.ajoms.2017.06.007
G Model JOMSMP-646; No. of Pages 4
ARTICLE IN PRESS A. Enomoto et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2017) xxx–xxx
4
disciplinary approach is thus required, and further experience is needed. Conflict of interest None. Ethical approval N/A. Acknowledgment None. References [1] Evans HL. Liposarcoma a study of 55 cases with a reassessment of its classification. Am J Surg Pathol 1979;3:507–23. [2] Henricks WH, Chu YC, Goldblum JR, Weiss SW. Dedifferentiated liposarcoma: a clinicopathological analysis of 155 cases with a proposal for an expanded definition of dedifferentiation. Am J Surg Pathol 1997;21:271–81. [3] da Cunha IW, Kowalski LP, Soares FA. Dedifferentiated liposarcoma of the oral cavity with angiosarcomatous dedifferentiation. Virchows Arch 2005;446:456–9. [4] de la Roza G, Baredes S, Aisner SC. Dedifferentiated liposarcoma of the cheek. Ann Diagn Pathol 2004;8:352–7. [5] Diamond C, Prince ME, Covert AA, Morris SF. Dedifferentiated liposarcoma of the cheek: case report and literature review. J Otolaryngol 2002;31: 125–8. [6] Fanburg-Smith JC, Furlong MA, Childers EL. Liposarcoma of the oral and salivary gland region: a clinicopathologic study of 18 cases with emphasis on specific sites, morphologic subtypes, and clinical outcome. Mod Pathol 2002;15:1020–31.
[7] Nascimento AF, McMenamin ME, Fletcher CD. Liposarcomas/atypical lipomatous tumors of the oral cavity: a clinicopathologic study of 23 cases. Ann Diagn Pathol 2002;6:83–93. [8] Angiero F, Sidoni A, Stefani M. Liposarcoma of the oral cavity–case reports of the pleomorphic and the dedifferentiated variants and a review of the literature. Anticancer Res 2006;26:4857–67. [9] Ueda T, Yoshikawa H, Mori S, Araki N, Myoui A, Kuratsu S, et al. Influence of local recurrence on the prognosis of soft-tissue sarcomas. J Bone Joint Surg Br 1997;79:553–7. [10] Stojadinovic A, Leung DH, Allen P, Lewis JJ, Jaques DP, Brennan MF. Primary adult soft tissue sarcoma: time-dependent influence of prognostic variables. J Clin Oncol 2002;20:4344–452. [11] Cheng J, Wang Y, Cheng A, Wang L, Tian Z, Yu H, et al. Primary liposarcoma in oral and maxillofacial region. J Craniofac Surg 2011;22:1765–71. [12] Friedman M, Egan JW. Irradiation of liposarcoma. Acta Radiol 1960;54:225–39. [13] Roberge D, Skamene T, Nahal A, Turcotte RE, Powell T, Freeman C. Radiological and pathological response following pre-operative radiotherapy for soft-tissue sarcoma. Radiother Oncol 2010;97:404–7. [14] Wortman JR, Tirumani SH, Tirumani H, Shinagare AB, Jagannathan JP, Hornick JL, et al. Neoadjuvant radiation in primary extremity liposarcoma: correlation of MRI features with histopathology. Eur Radiol 2015. [15] Coindre JM, Terrier P, Bui NB, Bonichon F, Collin F, Le Doussal V, et al. Prognostic factors in adult patients with locally controlled soft tissue sarcoma. A study of 546 patients from the French Federation of Cancer Centers Sarcoma Group. J Clin Oncol 1996;14:869–77. [16] Ramanathan RC, A’Hern R, Fisher C, Thomas JM. Modified staging system for extremity soft tissue sarcomas. Ann Surg Oncol 1999;6:57–69. [17] Pisters PW, Leung DH, Woodruff J, Shi W, Brennan MF. Analysis of prognostic factors in 1,041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol 1996;14:1679–89. [18] McCulloch TM, Makielski KH, McNutt MA. Head and neck liposarcoma: a histopathologic reevaluation of reported cases. Arch Otolaryngol Head Neck Surg 1992;118:1045–9. [19] Eilber FC, Eilber FR, Eckardt J, Rosen G, Riedel E, Maki RG, et al. The impact of chemotherapy on the survival of patients with high-grade primary extremity liposarcoma. Ann Surg 2004;240:686–95, discussion 95–7. [20] Tobey DN, Wheelis RF, Yarington Jr CT. Electron microscopy in the diagnosis of liposarcoma and fibrosarcoma of the larynx. Ann Otol Rhinol Laryngol 1979;88:867–71.
Please cite this article in press as: Enomoto A, et al. Dedifferentiated liposarcoma in the maxillary gingiva: A clinical report and review of the literature. J Oral Maxillofac Surg Med Pathol (2017), http://dx.doi.org/10.1016/j.ajoms.2017.06.007
ARTICLE IN PRESS Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2017) xxx–xxx
Contents lists available at ScienceDirect
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology journal homepage: www.elsevier.com/locate/jomsmp
Case report
Dedifferentiated liposarcoma in the maxillary gingiva: A clinical report and review of the literature Akifumi Enomoto ∗ , Kazuhide Matsunaga, Takeshi Shimoide, Takao Mukai, Takayuki Uchihashi, Suguru Hamada Department of Oral and Maxillofacial Surgery, Kindai University, Faculty of Medicine, 377-2, Onohigashi, Osaka-Sayama 589-8511, Japan, Japan
a r t i c l e
i n f o
Article history: Received 13 January 2017 Received in revised form 1 May 2017 Accepted 14 June 2017 Available online xxx Keyword: Dedifferentiated liposarcoma Oral cavity Surgery
a b s t r a c t Liposarcoma in the oral cavity is extremely rare, and dedifferentiated liposarcoma has seldom been reported in the oral region. No clear consensus has been established regarding the management of oral dedifferentiated liposarcoma. The case of a 28-year-old Japanese woman who presented with dedifferentiated liposarcoma in the maxillary gingiva is reported. She complained of swelling of the right maxillary gingiva, and swelling was evident in the region. No lymph nodes were palpable in either side of the neck. Incisional biopsy of the tumor region was performed, revealing dedifferentiated liposarcoma. The patient was treated with maxillectomy, and she has shown no clinical or radiological evidence of local recurrence or distant metastases as of 30 months after the end of treatment. This case provides a basis for the future appropriate management of oral cancer in patients with dedifferentiated liposarcoma. © 2017 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.夽
1. Introduction
2. Case report
Most liposarcomas arise in the extremities and retroperitoneum. Diagnosis and management planning for the staging of patients with liposarcoma of these soft tissue areas has increasingly been reported. However, liposarcomas affecting the oral region are very unusual, accounting for approximately 0.3% of all liposarcomas. These mainly arise on the buccal mucosa or the floor of the mouth, and very few arise in the gingiva. Regarding the pathological features, the atypical lipomatous tumor/well-differentiated (ALT/WD) liposarcoma is identified as the most common subtype, followed by myxoid variants in the oral region. Dedifferentiated liposarcoma is a rare subtype, occurring in about 10% of cases, and it can be defined as juxtaposition of a non-lipogenic sarcoma of variable histological grade and ALT/WD liposarcoma. The issues associated with liposarcoma in the oral region have not been widely discussed, and no clear consensus has been established regarding the management of oral liposarcoma. To address the lack of information on oral liposarcoma, further reports are required. Thus, a case of liposarcoma in the maxillary gingiva is presented along with a review of literature.
A 28-year-old Japanese woman was referred to our clinic in May 2014 complaining of swelling of the right maxillary gingiva.
∗ Corresponding author. E-mail address: [email protected] (A. Enomoto).
Fig. 1. Intraoral findings. Intraoral photographs at initial presentation. Small but considerable swelling in the right maxillary gingiva is evident. The arrowhead showed a swelling lesion in the right maxillary gingiva.
http://dx.doi.org/10.1016/j.ajoms.2017.06.007 2212-5558/© 2017 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.夽
Please cite this article in press as: Enomoto A, et al. Dedifferentiated liposarcoma in the maxillary gingiva: A clinical report and review of the literature. J Oral Maxillofac Surg Med Pathol (2017), http://dx.doi.org/10.1016/j.ajoms.2017.06.007
G Model
ARTICLE IN PRESS
JOMSMP-646; No. of Pages 4
A. Enomoto et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2017) xxx–xxx
2
Fig. 2. CT and MRI assessment. Contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) were performed. CT shows a clear enhanced lesion in the right maxillary gingiva. The arrowhead showed a clear, enhanced lesion of the right maxillary gingiva (A). STIR MR images on initial presentation show a prominent hyperintense lesion in the right maxillary gingiva. The arrowhead showed a clear hyperintensity lesion of the right maxillary gingiva on STIR imaging (B).
A painless small swelling in the right maxillary gingiva was evident (Fig. 1), and no lymph nodes were palpable in either side of the neck. The patient had no other associated disease. Contrastenhanced computed tomography (CT) and magnetic resonance imaging (MRI) were performed. CT evaluation of the right maxillary gingiva showed a clear, enhanced lesion (Fig. 2). On MRI, hyperintensity on short T1 inversion recovery (STIR) images and contrast-enhanced T1-weighted images was observed in the right maxillary gingiva (Fig. 2). Biopsy of the maxillary gingival lesion was performed, revealing dedifferentiated liposarcoma (FNCLCC grading system: G3). Finally, a diagnosis of liposarcoma of the right maxillary gingiva with no lymph node metastasis was established. The tumor was staged as T1aN0M0, stage IIA according to the Union for International Cancer Control (UICC) staging system. After sufficient consultation with an interdisciplinary team and obtaining informed consent from the patient, surgery was selected as the treatment strategy. A right side maxillectomy was performed under general anesthesia. The final diagnosis was dedifferentiated liposarcoma, the same as the initial diagnosis. On pathological examination, the tumor
has two distinct areas represented by a well-differentiated liposarcoma and a dedifferentiated area. Dedifferentiated tumor had non-lipogenic area composed of spindle or fibroblastic cells. These coexistence of a well-differentiated liposarcoma and a nonlipogenic area indicated dedifferentiated liposarcoma (Fig. 3). Immunohistochemical studies showed positivity for S-100, MDM2, and CDK4 and negativity for desmin, ␣SMA, CD34, h-caldesmon, and myogenin. CT, MRI, and PET-CT assessments have been obtained to ensure adequate follow-up, and recurrence and lymph or distant metastases have not been seen 30 months after treatment. 3. Discussion This case report represents the first description of dedifferentiated liposarcoma involving the maxillary gingiva. Liposarcoma is one of the most common soft tissue sarcomas in adults, and it accounts for approximately 20% of all soft tissue malignancies. Diagnosis and management planning for liposarcoma of the soft tissues in the extremities and retroperitoneum
Table 1 Review of the literature: 9 cases of oral dedifferentiated liposarcoma. Authors
Age/Sex
Location/Size(cm)
Treatment
Follow-up information
Diamond et al. Fanburg-Smith et al. Fanburg-Smith et al. Nascimento et al. Werneck da Cunha et al. Gustavo de la Roza et al. Angiero F et al. Henricks et al. Present case
57/M 39/M 56/M 83/M 42/F 61/M 41/F NA 28/F
Cheek/8.0 Tongue/6.0 Cheek/5.0 Tongue/2.5 Cheek/6.0 Cheek/5.0 Maxillary gingiva/1.0 Cheek/NA Maxirally gingiva/2.0
Surg + RT Surg Surg Surg Surg Surg Surg Surg Surg
no REC for 12 months no REC for 72 months 6 RECs in 26 years Lost F/U no REC for 12 months one REC and Lost F/U no Rec for 12 months NA no REC for 24 months
Surg: surgery. RT: radiation therapy. REC: recurrence. F/U: follow-up. NA: not available.
Please cite this article in press as: Enomoto A, et al. Dedifferentiated liposarcoma in the maxillary gingiva: A clinical report and review of the literature. J Oral Maxillofac Surg Med Pathol (2017), http://dx.doi.org/10.1016/j.ajoms.2017.06.007
G Model JOMSMP-646; No. of Pages 4
ARTICLE IN PRESS A. Enomoto et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2017) xxx–xxx
Fig. 3. Pathological findings. The tumor has two different areas represented by a well-differentiated liposarcoma and a dedifferentiated area (upper). Lipogenic area is identical to original tumor (middle). The high-grade dedifferentiated area showed a spindle proliferation consistent with fibrosarcoma (bottom). (H-E x 40).
has been well established. According to the 2002 WHO classification, histological diagnosis of liposarcoma is classified as ALT/WD liposarcoma, myxoid liposarcoma, pleomorphic liposarcoma, differentiated liposarcoma, and mixed-type liposarcoma. Evans reported that the overall survival rate of dedifferentiated liposarcoma was significantly poorer than that of myxoid and ALT/WD liposarcoma because of local recurrence and metastasis [1]. Soft tissue dedifferentiated liposarcomas recur locally in 41%,
3
metastasize in 17%, and cause death from disease in 28% of cases [2]. Liposarcoma of the oral region is unusual, and dedifferentiated liposarcoma is a rare subtype, occurring in about 3% of cases. There are only a few reports suggesting suitable treatment options for oral dedifferentiated liposarcoma. No clear consensus has been established regarding the management of oral dedifferentiated liposarcoma. To the best of our knowledge, only 8 cases of dedifferentiated liposarcoma have been reported in the English language literature [2–8] (Table 1). Local recurrence is a critical factor for survival [9,10]. Cheng et al. reported evaluations of clinical cases of primary liposarcoma in the oral and maxillofacial region, which included 4 dedifferentiated liposarcomas [11]. The recurrence rate of oral dedifferentiated liposarcoma in this report was 25.0%, indicating poorer results than of other subtypes of liposarcoma (18.1%). Among 9 cases of oral dedifferentiated liposarcoma, including the present patient, in the table, the recurrence rate was 22.2% (2/9). On the other hand, Fanburg-Smith reported that oral dedifferentiated liposarcoma does not necessarily have a poor outcome, compared to other subtypes of liposarcoma on clinical evaluation [6]. Indeed, the number of reports of oral dedifferentiated liposarcoma is still very few, and more clinical experience with oral dedifferentiated liposarcoma is needed for better understanding. Regarding treatment, surgical excision with an extensive wide margin is suggested, and the importance of complete excision is generally emphasized. Therefore, extensive resection with a wide margin of the maxillary sinus was performed in the present case. Surgery is the critical therapy that achieves excellent local control. As for non-surgical treatment, there have been several reports about radiation therapy for liposarcoma. In the first case in the table, surgery and postoperative radiation were performed for oral dedifferentiated liposarcoma [5]. This patient was alive 12 months after treatment. However, for the rest of the cases in the table, the treatment was only surgery. This may be because poorly differentiated liposarcoma has low sensitivity to radiation therapy, while myxoid liposarcoma has a predictable response to radiotherapy [12–14]. Furthermore, recent studies suggested that small tumors (less than or equal to 5 cm) are less frequently associated with local recurrence, indicating that radiation therapy may not be necessary for these patients [15–17]. Additionally, there is another report that surgery with radiation therapy does not show improve the 5-year survival rate compared to surgery alone [11]. Radiation treatment modalities generally still have unclear and non-definitive evidence for oral liposarcoma [11,18]. There are several reports about chemotherapy for liposarcoma. Chemotherapy is currently standard therapy for Ewing’s sarcoma or rhabdomyosarcoma. As for liposarcoma, the effect of chemotherapy on survival remains controversial, and it has not been standardized. Eilber et al. reported the impact of ifosfamide-based chemotherapy on large (>10 cm), high-grade, extremity liposarcoma [19]. However, there are almost no reports of chemotherapy for oral liposarcoma. For larynx liposarcoma, as a head and neck region, a combination of chemotherapy, radiation, and surgery was performed. The chemotherapy menu was cyclophosphamide, doxorubicin, dimethyl triazeno imidazole carboxymide (DTIC), and vincristine sulfate. Despite this treatment, the patient died of uncontrolled disease [20]. In the present case, the postoperative margin was tumornegative after extensive surgery, and neither radiation nor chemotherapy was performed. The prognosis or the overall survival rate of liposarcoma has been reported to be related to histological type, and a high survival rate generally cannot be expected with dedifferentiated liposarcoma. The treatment strategy for oral dedifferentiated liposarcoma is not definitive and is poorly known in many respects. A multi-
Please cite this article in press as: Enomoto A, et al. Dedifferentiated liposarcoma in the maxillary gingiva: A clinical report and review of the literature. J Oral Maxillofac Surg Med Pathol (2017), http://dx.doi.org/10.1016/j.ajoms.2017.06.007
G Model JOMSMP-646; No. of Pages 4
ARTICLE IN PRESS A. Enomoto et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2017) xxx–xxx
4
disciplinary approach is thus required, and further experience is needed. Conflict of interest None. Ethical approval N/A. Acknowledgment None. References [1] Evans HL. Liposarcoma a study of 55 cases with a reassessment of its classification. Am J Surg Pathol 1979;3:507–23. [2] Henricks WH, Chu YC, Goldblum JR, Weiss SW. Dedifferentiated liposarcoma: a clinicopathological analysis of 155 cases with a proposal for an expanded definition of dedifferentiation. Am J Surg Pathol 1997;21:271–81. [3] da Cunha IW, Kowalski LP, Soares FA. Dedifferentiated liposarcoma of the oral cavity with angiosarcomatous dedifferentiation. Virchows Arch 2005;446:456–9. [4] de la Roza G, Baredes S, Aisner SC. Dedifferentiated liposarcoma of the cheek. Ann Diagn Pathol 2004;8:352–7. [5] Diamond C, Prince ME, Covert AA, Morris SF. Dedifferentiated liposarcoma of the cheek: case report and literature review. J Otolaryngol 2002;31: 125–8. [6] Fanburg-Smith JC, Furlong MA, Childers EL. Liposarcoma of the oral and salivary gland region: a clinicopathologic study of 18 cases with emphasis on specific sites, morphologic subtypes, and clinical outcome. Mod Pathol 2002;15:1020–31.
[7] Nascimento AF, McMenamin ME, Fletcher CD. Liposarcomas/atypical lipomatous tumors of the oral cavity: a clinicopathologic study of 23 cases. Ann Diagn Pathol 2002;6:83–93. [8] Angiero F, Sidoni A, Stefani M. Liposarcoma of the oral cavity–case reports of the pleomorphic and the dedifferentiated variants and a review of the literature. Anticancer Res 2006;26:4857–67. [9] Ueda T, Yoshikawa H, Mori S, Araki N, Myoui A, Kuratsu S, et al. Influence of local recurrence on the prognosis of soft-tissue sarcomas. J Bone Joint Surg Br 1997;79:553–7. [10] Stojadinovic A, Leung DH, Allen P, Lewis JJ, Jaques DP, Brennan MF. Primary adult soft tissue sarcoma: time-dependent influence of prognostic variables. J Clin Oncol 2002;20:4344–452. [11] Cheng J, Wang Y, Cheng A, Wang L, Tian Z, Yu H, et al. Primary liposarcoma in oral and maxillofacial region. J Craniofac Surg 2011;22:1765–71. [12] Friedman M, Egan JW. Irradiation of liposarcoma. Acta Radiol 1960;54:225–39. [13] Roberge D, Skamene T, Nahal A, Turcotte RE, Powell T, Freeman C. Radiological and pathological response following pre-operative radiotherapy for soft-tissue sarcoma. Radiother Oncol 2010;97:404–7. [14] Wortman JR, Tirumani SH, Tirumani H, Shinagare AB, Jagannathan JP, Hornick JL, et al. Neoadjuvant radiation in primary extremity liposarcoma: correlation of MRI features with histopathology. Eur Radiol 2015. [15] Coindre JM, Terrier P, Bui NB, Bonichon F, Collin F, Le Doussal V, et al. Prognostic factors in adult patients with locally controlled soft tissue sarcoma. A study of 546 patients from the French Federation of Cancer Centers Sarcoma Group. J Clin Oncol 1996;14:869–77. [16] Ramanathan RC, A’Hern R, Fisher C, Thomas JM. Modified staging system for extremity soft tissue sarcomas. Ann Surg Oncol 1999;6:57–69. [17] Pisters PW, Leung DH, Woodruff J, Shi W, Brennan MF. Analysis of prognostic factors in 1,041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol 1996;14:1679–89. [18] McCulloch TM, Makielski KH, McNutt MA. Head and neck liposarcoma: a histopathologic reevaluation of reported cases. Arch Otolaryngol Head Neck Surg 1992;118:1045–9. [19] Eilber FC, Eilber FR, Eckardt J, Rosen G, Riedel E, Maki RG, et al. The impact of chemotherapy on the survival of patients with high-grade primary extremity liposarcoma. Ann Surg 2004;240:686–95, discussion 95–7. [20] Tobey DN, Wheelis RF, Yarington Jr CT. Electron microscopy in the diagnosis of liposarcoma and fibrosarcoma of the larynx. Ann Otol Rhinol Laryngol 1979;88:867–71.
Please cite this article in press as: Enomoto A, et al. Dedifferentiated liposarcoma in the maxillary gingiva: A clinical report and review of the literature. J Oral Maxillofac Surg Med Pathol (2017), http://dx.doi.org/10.1016/j.ajoms.2017.06.007