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Large liposarcoma developing in the ischiorectal fossa: Report of a rare case
CASE REPORT – OPEN ACCESS International Journal of Surgery Case Reports 4 (2013) 51–53
Contents lists available at SciVerse ScienceDirect
International Journal of Surgery Case Reports journal homepage: www.elsevier.com/locate/ijscr
Large liposarcoma developing in the ischiorectal fossa: Report of a rare case Ryo Seishima a,∗ , Yoshiyuki Ishii a , Hirotoshi Hasegawa a , Takashi Endo a , Hiroki Ochiai a , Koji Okabayashi a , Makio Mukai b , Yuko Kitagawa a a b
Department of Surgery, Keio University School of Medicine, Japan Division of Diagnostic Pathology, Keio University School of Medicine, Japan
a r t i c l e
i n f o
Article history: Received 15 August 2012 Received in revised form 24 September 2012 Accepted 25 September 2012 Available online 11 October 2012 Keywords: Liposarcoma Ischiorectal fossa Soft tissue tumor
a b s t r a c t INTRODUCTION: We herein report an extremely rare case of a liposarcoma developing in the ischiorectal fossa that was completely resected using a transsacral approach. PRESENTATION OF CASE: A 74-year-old man was admitted to our institute because of a large perianal tumor. A pelvic magnetic resonance imaging (MRI) revealed an 8 cm × 7 cm × 5 cm sized encapsulated heterogeneous tumor on the right side of the ischiorectal fossa. Transsacral excision was performed under a diagnosis of liposarcoma. The tumor was partially fixed to the external sphincter and puborectalis. Therefore, the muscles were partially resected to ensure sufficient margins. Histopathological examination revealed a well differentiated liposarcoma and negative surgical margins. DISCUSSION: The basic treatment strategy of liposarcoma is surgical resection, whereas the effects of chemotherapy or radiotherapy remain limited. Although no postoperative adjuvant therapy has been performed in our case, no recurrences have occurred for 1 year after surgery. CONCLUSION: Although the curative resection of large tumors located in the ischiorectal fossa is usually difficult, the complete resection of liposarcoma is the main potentially curative treatment. A transsacral approach is useful for a complete resection. © 2012 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.
1. INTRODUCTION Liposarcoma is one of the most common types of soft tissue sarcoma. The basic treatment strategy is surgical resection, whereas the effects of chemotherapy or radiotherapy remain limited. We herein report an extremely rare case of a liposarcoma developing in the ischiorectal fossa that was completely resected using a transsacral approach. 2. PRESENTATION OF CASE A 74-year-old man had noticed a perianal lump with a 5-year history that had recently increased in size, with no symptoms affecting bowel habits. On a physical examination, an elastic soft mass located on the right side of the perianus with a protuberance (Fig. 1) pushing the anal canal and lower rectum, without any pain or tenderness, was noted. Magnetic resonance imaging (MRI) and computed tomographic (CT) scans were performed for a differential diagnosis and to assess the extent of tumor involvement. The pelvic MRI scan showed a well-defined tumor (8 cm × 7 cm × 5 cm) with a heterogeneously contrasted appearance in the right ischiorectal
∗ Corresponding author at: Department of Surgery, Keio University School of Medicine, 35 Shinano-machi, Shinjuku-ku, Tokyo 160-8582, Japan. Tel.: +81 3 3353 1211; fax: +81 3 3355 4707. E-mail address: [email protected] (R. Seishima).
fossa below the levator plane (Fig. 2a and b). The tumor was adjacent to the anal canal and lower rectum, but no clear invasion of the adjacent tissues, such as the external sphincter or puborectalis, was seen. A chest to pelvic CT scan showed no other local or distant lesions. The first choice for the differential diagnosis was a liposarcoma, which was considered to be surgically resectable with or without the excision of the adjacent tissues. The patient was placed jack knife position on the operating table under general anesthesia and a transsacral approach was selected for the resection of the tumor. We made an incision 2 cm distant from the right edge of lower sacrum and coccyx (Fig. 1). We transected the muscle fibers of the gluterus maximus on the lateral side around the tumor. On the medial side, fibers of the external anal sphincter were visualized. Superiorly, the surface of the lower half of the sacrum was exposed down to the coccyx. We did not resect the coccyx. The lateral and upper margins of the tumor were completely divided from the adjacent tissues: however, medial margin was unclear and fixed to the external sphincter and puborectalis. To ensure a sufficient distance from the tumor margin, the external sphincter and puborectalis muscles were partially excised and the resulting defects were repaired with an appropriate anal tonus. The patient has not experienced any postoperative morbidities or difficulties with his bowel habits and was discharged 7 days after the surgery. A pathological examination revealed the complete resection of a well-differentiated liposarcoma, which had lipocytes with dense atypical nuclei (Fig. 3).
2210-2612/$ – see front matter © 2012 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijscr.2012.09.011
CASE REPORT – OPEN ACCESS 52
R. Seishima et al. / International Journal of Surgery Case Reports 4 (2013) 51–53
No recurrences have occurred within 1 year after surgery (Fig. 2c and d), and no postoperative therapy has been performed.
3. DISCUSSION
Fig. 1. Aspect of the large perianal tumor. The mass located in the right side of perianus with protuberance. The incision line is described by a black line.
Among more than 50 histological types of soft tissue sarcoma, the most common subtype is liposarcoma, accounting for 24% of sarcomas in extremities and 45% of sarcomas in the retroperitoneum.1 The present case was an extremely rare liposarcoma that developed in the ischiorectal fossa; to the best of our knowledge, there are only few reports of such liposarcomas have been made.2 Liposarcoma is pathologically classified into 5 types (well-differentiated, myxoid, pleomorphic, round cell, and mixed). The histologic subtype (as well as the tumor location) is a predictor of patient outcome. Pleomorphic type, round cell type, and mixed type are high-grade liposarcomas, which are aggressive
Fig. 2. MRI imagings of the tumor and after surgery. (a) Axial T1 weighted image of MRI showed a well-defined tumor with a heterogeneously contrasted content. (b) Sagital T2 weighted image of MRI showed that the tumor was adjacent to the anal canal and lower rectum, but not clearly invasive to the adjacent tissues such as external sphincter and puborectalis. (c) Axial T1 weighted and (d) sagital T2 weighted image of MRI, showed no clear evidence of recurrence after surgery.
Fig. 3. Histopathological findings of the tumor. (a) Hyperplasia of variable-sized lipocytes with fibrosis are seen (hematoxylin and eosin staining, ×10). (b) Dense atypical nuclei are seen in various lipocytes (hematoxylin and eosin staining, ×40).
CASE REPORT – OPEN ACCESS R. Seishima et al. / International Journal of Surgery Case Reports 4 (2013) 51–53
tumors with metastatic potential; meanwhile, well-differentiated and myxoid type liposarcomas are low-grade liposarcomas that follow a more indolent clinical course.3 The current therapy for liposarcoma follows the guidelines established for the treatment of soft tissue sarcomas and heavily relies on the achievement of complete surgical extirpation.4 Complete resection is strongly associated with a favorable outcome. Therefore, an aggressive approach, such as en bloc resection with adjacent organs, is generally selected depending on the conditions of individual patients. Although a 1–2 cm distance from the tumor margin is widely accepted to be adequate, no studies have suggested the superiority of larger margins in negative cases. At the minimum, a macroscopically negative margin is essential for a good prognosis after surgical treatment. Another treatment available for soft tissue sarcoma is chemotherapy, generally using anthracyclines (doxorubicin and epirubicin) and ifosfamide. However, no clear evidence of the positive effects of postoperative adjuvant therapy on the relapse-free and overall survival rates is available.5 The European Organization for Research and Treatment of Cancer (EORTC) Soft Tissue and Bone Sarcoma Group retrospectively evaluated the effects of doxorubicin versus ifosfamide-based regimens for patients with advanced soft tissue sarcoma. The results of this study indicated that ifosfamide-based therapies may be less beneficial in liposarcoma patients compared with doxorubicin monotherapy.6 A pivotal meta-analysis from the Sarcoma Meta-Analysis Collaboration (SMAC) published in 1997 showed that postoperative doxorubicin adjuvant chemotherapy improved local control and relapse free survival rates, but not the overall survival rate, compared with surgery alone.5 Although radiation therapy has been reported to enhance the local control rate, compared with surgery alone, in some circumstances, this modality did not lead to an improvement in the overall survival rate.7,8 Accordingly, radiation therapy is especially applicable for intermediate or high-grade soft tissue sarcomas to minimize the extent of local surgical resection required. The advantages of pre- or post-operative radiation therapy remain unclear for local control.9 However, preoperative radiation therapy is associated with a high incidence of wound complications, while postoperative radiation therapy is associated with an increased rate of fibrosis or edema. Therefore, radiation therapy must be performed on an individual patient basis. Taking these matters into consideration, we decided to carefully observe this patient, who had a low-grade histological type of well-differentiated liposarcoma and negative surgical margins, without performing any postoperative adjuvant therapies.
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4. CONCLUSION The main potentially curative treatment of liposarcoma is the complete resection. A transsacral approach is useful for a complete resection for large tumors located in the ischiorectal fossa. CONFLICT OF INTEREST STATEMENT None declared. FUNDING None. ETHICAL APPROVAL Written informed consent was obtained from the patient for publication of this case report and accompanying images. No identification details have been used. AUTHOR CONTRIBUTIONS Authors contributed equally. References 1. Crago AM, Singer S. Clinical and molecular approaches to well differentiated and dedifferentiated liposarcoma. Current Opinion in Oncology 2011;23:373–8. 2. Long Pretz P, Detry R, Kestens PJ, Haot J. Liposarcoma of the ischiorectal fossa, an unusual tumoral site. Acta Chirurgica Belgica 1988;88:151–4. 3. Dadal KM, Kattan MW, Antonescu CR, Brennan MF, Singer S. Subtype specific prognostic nomogram for patients with primary liposarcoma of the retroperitoneum, extremity, or trunk. Annals of Surgery 2006;244:381–91. 4. Hoffman A, Lazar AJ, Pollock RE, Lev D. New frontiers in the treatment of liposarcoma, a therapeutically resistant malignant cohort. Drug Resistance Updates 2011;14:52–66. 5. Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Sarcoma Meta-analysis Collaboration. Lancet 1997;350:1647–1654. 6. Sleijfer S, Quali M, van Glabbeke M, Kraup-Hansen A, Rodenhuis S, Le Cesne A, et al. Prognostic and predictive factors for outcome to first-line ifosfamidecontaining chemotherapy for adult patients with advanced soft tissue sarcomas: an exploratory, retrospective analysis on large series from the European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group (EORTC-STBSG). European Journal of Cancer 2010;46:72–83. 7. Pisters PW, Harrison LB, Leung DH, Woodruff JM, Casper ES, Brennan MF. Longterm results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. Journal of Clinical Oncology 1996;14:859–68. 8. 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. Journal of Clinical Oncology 1998;16:197–203. 9. O’Sullivan B, Davis AM, Turcotte R, Bell R, Catton C, Chabot P, et al. Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomised trial. Lancet 2002;359:2235–41.
Open Access This article is published Open Access at sciencedirect.com. It is distributed under the IJSCR Supplemental terms and conditions, which permits unrestricted non commercial use, distribution, and reproduction in any medium, provided the original authors and source are credited.
Contents lists available at SciVerse ScienceDirect
International Journal of Surgery Case Reports journal homepage: www.elsevier.com/locate/ijscr
Large liposarcoma developing in the ischiorectal fossa: Report of a rare case Ryo Seishima a,∗ , Yoshiyuki Ishii a , Hirotoshi Hasegawa a , Takashi Endo a , Hiroki Ochiai a , Koji Okabayashi a , Makio Mukai b , Yuko Kitagawa a a b
Department of Surgery, Keio University School of Medicine, Japan Division of Diagnostic Pathology, Keio University School of Medicine, Japan
a r t i c l e
i n f o
Article history: Received 15 August 2012 Received in revised form 24 September 2012 Accepted 25 September 2012 Available online 11 October 2012 Keywords: Liposarcoma Ischiorectal fossa Soft tissue tumor
a b s t r a c t INTRODUCTION: We herein report an extremely rare case of a liposarcoma developing in the ischiorectal fossa that was completely resected using a transsacral approach. PRESENTATION OF CASE: A 74-year-old man was admitted to our institute because of a large perianal tumor. A pelvic magnetic resonance imaging (MRI) revealed an 8 cm × 7 cm × 5 cm sized encapsulated heterogeneous tumor on the right side of the ischiorectal fossa. Transsacral excision was performed under a diagnosis of liposarcoma. The tumor was partially fixed to the external sphincter and puborectalis. Therefore, the muscles were partially resected to ensure sufficient margins. Histopathological examination revealed a well differentiated liposarcoma and negative surgical margins. DISCUSSION: The basic treatment strategy of liposarcoma is surgical resection, whereas the effects of chemotherapy or radiotherapy remain limited. Although no postoperative adjuvant therapy has been performed in our case, no recurrences have occurred for 1 year after surgery. CONCLUSION: Although the curative resection of large tumors located in the ischiorectal fossa is usually difficult, the complete resection of liposarcoma is the main potentially curative treatment. A transsacral approach is useful for a complete resection. © 2012 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.
1. INTRODUCTION Liposarcoma is one of the most common types of soft tissue sarcoma. The basic treatment strategy is surgical resection, whereas the effects of chemotherapy or radiotherapy remain limited. We herein report an extremely rare case of a liposarcoma developing in the ischiorectal fossa that was completely resected using a transsacral approach. 2. PRESENTATION OF CASE A 74-year-old man had noticed a perianal lump with a 5-year history that had recently increased in size, with no symptoms affecting bowel habits. On a physical examination, an elastic soft mass located on the right side of the perianus with a protuberance (Fig. 1) pushing the anal canal and lower rectum, without any pain or tenderness, was noted. Magnetic resonance imaging (MRI) and computed tomographic (CT) scans were performed for a differential diagnosis and to assess the extent of tumor involvement. The pelvic MRI scan showed a well-defined tumor (8 cm × 7 cm × 5 cm) with a heterogeneously contrasted appearance in the right ischiorectal
∗ Corresponding author at: Department of Surgery, Keio University School of Medicine, 35 Shinano-machi, Shinjuku-ku, Tokyo 160-8582, Japan. Tel.: +81 3 3353 1211; fax: +81 3 3355 4707. E-mail address: [email protected] (R. Seishima).
fossa below the levator plane (Fig. 2a and b). The tumor was adjacent to the anal canal and lower rectum, but no clear invasion of the adjacent tissues, such as the external sphincter or puborectalis, was seen. A chest to pelvic CT scan showed no other local or distant lesions. The first choice for the differential diagnosis was a liposarcoma, which was considered to be surgically resectable with or without the excision of the adjacent tissues. The patient was placed jack knife position on the operating table under general anesthesia and a transsacral approach was selected for the resection of the tumor. We made an incision 2 cm distant from the right edge of lower sacrum and coccyx (Fig. 1). We transected the muscle fibers of the gluterus maximus on the lateral side around the tumor. On the medial side, fibers of the external anal sphincter were visualized. Superiorly, the surface of the lower half of the sacrum was exposed down to the coccyx. We did not resect the coccyx. The lateral and upper margins of the tumor were completely divided from the adjacent tissues: however, medial margin was unclear and fixed to the external sphincter and puborectalis. To ensure a sufficient distance from the tumor margin, the external sphincter and puborectalis muscles were partially excised and the resulting defects were repaired with an appropriate anal tonus. The patient has not experienced any postoperative morbidities or difficulties with his bowel habits and was discharged 7 days after the surgery. A pathological examination revealed the complete resection of a well-differentiated liposarcoma, which had lipocytes with dense atypical nuclei (Fig. 3).
2210-2612/$ – see front matter © 2012 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijscr.2012.09.011
CASE REPORT – OPEN ACCESS 52
R. Seishima et al. / International Journal of Surgery Case Reports 4 (2013) 51–53
No recurrences have occurred within 1 year after surgery (Fig. 2c and d), and no postoperative therapy has been performed.
3. DISCUSSION
Fig. 1. Aspect of the large perianal tumor. The mass located in the right side of perianus with protuberance. The incision line is described by a black line.
Among more than 50 histological types of soft tissue sarcoma, the most common subtype is liposarcoma, accounting for 24% of sarcomas in extremities and 45% of sarcomas in the retroperitoneum.1 The present case was an extremely rare liposarcoma that developed in the ischiorectal fossa; to the best of our knowledge, there are only few reports of such liposarcomas have been made.2 Liposarcoma is pathologically classified into 5 types (well-differentiated, myxoid, pleomorphic, round cell, and mixed). The histologic subtype (as well as the tumor location) is a predictor of patient outcome. Pleomorphic type, round cell type, and mixed type are high-grade liposarcomas, which are aggressive
Fig. 2. MRI imagings of the tumor and after surgery. (a) Axial T1 weighted image of MRI showed a well-defined tumor with a heterogeneously contrasted content. (b) Sagital T2 weighted image of MRI showed that the tumor was adjacent to the anal canal and lower rectum, but not clearly invasive to the adjacent tissues such as external sphincter and puborectalis. (c) Axial T1 weighted and (d) sagital T2 weighted image of MRI, showed no clear evidence of recurrence after surgery.
Fig. 3. Histopathological findings of the tumor. (a) Hyperplasia of variable-sized lipocytes with fibrosis are seen (hematoxylin and eosin staining, ×10). (b) Dense atypical nuclei are seen in various lipocytes (hematoxylin and eosin staining, ×40).
CASE REPORT – OPEN ACCESS R. Seishima et al. / International Journal of Surgery Case Reports 4 (2013) 51–53
tumors with metastatic potential; meanwhile, well-differentiated and myxoid type liposarcomas are low-grade liposarcomas that follow a more indolent clinical course.3 The current therapy for liposarcoma follows the guidelines established for the treatment of soft tissue sarcomas and heavily relies on the achievement of complete surgical extirpation.4 Complete resection is strongly associated with a favorable outcome. Therefore, an aggressive approach, such as en bloc resection with adjacent organs, is generally selected depending on the conditions of individual patients. Although a 1–2 cm distance from the tumor margin is widely accepted to be adequate, no studies have suggested the superiority of larger margins in negative cases. At the minimum, a macroscopically negative margin is essential for a good prognosis after surgical treatment. Another treatment available for soft tissue sarcoma is chemotherapy, generally using anthracyclines (doxorubicin and epirubicin) and ifosfamide. However, no clear evidence of the positive effects of postoperative adjuvant therapy on the relapse-free and overall survival rates is available.5 The European Organization for Research and Treatment of Cancer (EORTC) Soft Tissue and Bone Sarcoma Group retrospectively evaluated the effects of doxorubicin versus ifosfamide-based regimens for patients with advanced soft tissue sarcoma. The results of this study indicated that ifosfamide-based therapies may be less beneficial in liposarcoma patients compared with doxorubicin monotherapy.6 A pivotal meta-analysis from the Sarcoma Meta-Analysis Collaboration (SMAC) published in 1997 showed that postoperative doxorubicin adjuvant chemotherapy improved local control and relapse free survival rates, but not the overall survival rate, compared with surgery alone.5 Although radiation therapy has been reported to enhance the local control rate, compared with surgery alone, in some circumstances, this modality did not lead to an improvement in the overall survival rate.7,8 Accordingly, radiation therapy is especially applicable for intermediate or high-grade soft tissue sarcomas to minimize the extent of local surgical resection required. The advantages of pre- or post-operative radiation therapy remain unclear for local control.9 However, preoperative radiation therapy is associated with a high incidence of wound complications, while postoperative radiation therapy is associated with an increased rate of fibrosis or edema. Therefore, radiation therapy must be performed on an individual patient basis. Taking these matters into consideration, we decided to carefully observe this patient, who had a low-grade histological type of well-differentiated liposarcoma and negative surgical margins, without performing any postoperative adjuvant therapies.
53
4. CONCLUSION The main potentially curative treatment of liposarcoma is the complete resection. A transsacral approach is useful for a complete resection for large tumors located in the ischiorectal fossa. CONFLICT OF INTEREST STATEMENT None declared. FUNDING None. ETHICAL APPROVAL Written informed consent was obtained from the patient for publication of this case report and accompanying images. No identification details have been used. AUTHOR CONTRIBUTIONS Authors contributed equally. References 1. Crago AM, Singer S. Clinical and molecular approaches to well differentiated and dedifferentiated liposarcoma. Current Opinion in Oncology 2011;23:373–8. 2. Long Pretz P, Detry R, Kestens PJ, Haot J. Liposarcoma of the ischiorectal fossa, an unusual tumoral site. Acta Chirurgica Belgica 1988;88:151–4. 3. Dadal KM, Kattan MW, Antonescu CR, Brennan MF, Singer S. Subtype specific prognostic nomogram for patients with primary liposarcoma of the retroperitoneum, extremity, or trunk. Annals of Surgery 2006;244:381–91. 4. Hoffman A, Lazar AJ, Pollock RE, Lev D. New frontiers in the treatment of liposarcoma, a therapeutically resistant malignant cohort. Drug Resistance Updates 2011;14:52–66. 5. Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Sarcoma Meta-analysis Collaboration. Lancet 1997;350:1647–1654. 6. Sleijfer S, Quali M, van Glabbeke M, Kraup-Hansen A, Rodenhuis S, Le Cesne A, et al. Prognostic and predictive factors for outcome to first-line ifosfamidecontaining chemotherapy for adult patients with advanced soft tissue sarcomas: an exploratory, retrospective analysis on large series from the European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group (EORTC-STBSG). European Journal of Cancer 2010;46:72–83. 7. Pisters PW, Harrison LB, Leung DH, Woodruff JM, Casper ES, Brennan MF. Longterm results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. Journal of Clinical Oncology 1996;14:859–68. 8. 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. Journal of Clinical Oncology 1998;16:197–203. 9. O’Sullivan B, Davis AM, Turcotte R, Bell R, Catton C, Chabot P, et al. Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomised trial. Lancet 2002;359:2235–41.
Open Access This article is published Open Access at sciencedirect.com. It is distributed under the IJSCR Supplemental terms and conditions, which permits unrestricted non commercial use, distribution, and reproduction in any medium, provided the original authors and source are credited.