J Gastrointest Canc (2014) 45 (Suppl 1):S248–S251 DOI 10.1007/s12029-014-9647-2
CASE REPORT
Peduncular Liposarcoma of the Colon: a Case Report and Literature Review Yusuke Kito & Tetsuya Fujii & Takeyoshi Nishiyama & Chiemi Saigo & Tatuo Okumoto & Nobuji Yokoyama & Tamotsu Takeuchi
Published online: 13 September 2014 # Springer Science+Business Media New York 2014
Introduction Liposarcoma is one of the most common types of soft tissue tumor in adults. Most liposarcomas occur in the extremities or retroperitoneum, whereas primary liposarcoma of the alimentary tract is rare. Especially, colonic liposarcoma remains limitedly reported in eleven cases [1–11] (summarized in Table 1). Because of its low incidence rate, the pathobiological properties of primary colonic liposarcoma remain largely unknown. In this sentence, accumulation of cases is desired. Here, we describe a case of colonic liposarcoma, which exhibited a unique peduncular growth pattern in the colonic lumen. The tumor was largely composed of dedifferentiated liposarcoma cells, with an intense and diffuse cyclindependent kinase 4 (CDK4) immunoreactivity. Notably, a well-differentiated liposarcoma component was also observed in the submucosal region of the tumor. To our knowledge, this is the first case of dedifferentiated peduncular colonic liposarcoma.
Case Presentation An 84-year-old woman who presented with abdominal pain due to intussusception was admitted to Himeji St. Maria
Y. Kito : C. Saigo : T. Takeuchi (*) Department of Pathology and Translational Research, Gifu University Graduate School of Medicine, Yanagido 1-1, Gifu 501-1194, Japan e-mail: [email protected] T. Fujii : T. Nishiyama : T. Okumoto : N. Yokoyama Department of Surgery, Himeji St. Maria Hospital, Nitoyo, Himeji 670-0801, Japan
Hospital. A colonoscopy was performed, showing an obstructive intraluminal mass (Fig. 1), and the patient underwent a right hemicolectomy. Macroscopic examination of the resected ascending colon revealed a dumbbell-shaped polypoid mass, which occupied the colonic lumen. This polypoid tumor measured 10×3×3 cm with a narrow stalk approximately 1 cm in diameter and was covered by mucosa with a focal ulceration (Fig. 2). The cut surface demonstrated a multilobulated circumscribed grayish polypoid mass (Fig. 2, Inset). Histopathological examination revealed that the mass was largely composed of a sarcomatous spindle cell tumor harboring giant cells, which often exhibited bizarre nuclei. These sarcomatous tumor cells cohesively occupied the submucosa and focally invaded the muscularis propria and subserosal tissues. Numerous mitotic figures were observed. Notably, tumor areas composed of irregular-shaped adipose tissues with multivacuolar lipoblasts were also found. The representative microscopic morphological features are shown in Fig. 3. There was no evidence of metastasis in regional resected lymph nodes. Subsequently, we performed immunohistochemical staining as previously reported [12]. Notably, CDK4 and S-100 immunoreactivities were found in proliferating spindle tumor cells and multivacuolar lipoblasts, respectively (Fig. 3c, f, respectively). Immunohistochemical staining was negative for CD117, CD34, and HMB45. It is well established that diffuse, intense CDK4 immunoreactivity in sarcomatous tumor is useful for the diagnosis of dedifferentiated liposarcoma [13]. Taking the results of the immunohistochemical analysis into consideration, we concluded that this peduncular tumor was a dedifferentiated liposarcoma with focal peripheral welldifferentiated liposarcoma. After surgery, the patient was discharged without any complications.
J Gastrointest Canc (2014) 45 (Suppl 1):S248–S251
S249
Table 1 Reported characteristics of cases of colonic liposarcoma
1 2 3 4 5 6 7 8 9 10 11 12
Wood and Morgenstern Parks et al. Magro et al. Chen Gutsu et al. Shahidzadeh et al. Chaudhary et al. Jarboui et al. D’Annibale et al. Choi et al. Türkoğlu et al. Present case
Age
Sex
Location
Histological subtype
62 54 65 52 46 56 66 69 79 41 71 84
F F F F M F F M F M F F
Ileocecal valve Ascending colon Cecum Descending colon Ascending colon Hepatic flexure Descending colon Splenic flexure Hepatic flexure Ascending colon Transverse colon Ascending colon
Myxoid Pleomorphic Well-differentiated Well-differentiated Myxoid Well-differentiated Well-differentiated Dedifferentiated Pleomorphic Myxoid Dedifferentiated Dedifferentiated
Bold denotes a case of peduncular liposarcoma, which is characterized by a macroscopic appearance of an intraluminal polypoid tumor with a slender stalk
Discussion Until now, only eleven cases of primary liposarcoma of the colon had been found in the English literature [1–11]. However, unsurprisingly, several of these colonic liposarcomas are found on the peritoneal side, sometimes forming a peritoneal mass with invasion to adjacent tissues, i.e., the stomach, omentum, or mesentery. It is likely that these colonic liposarcomas may arise in adipose tissues in the mesocolon and subsequently invade the colonic wall. In fact, Sato et al. recently described a retroperitoneal liposarcoma, which infiltrated to form a multinodular solid mass in the lumen [14]. The pathobiological properties of these liposarcomas may be similar to those of retroperitoneal liposarcomas because of their robust tumor infiltration. By contrast, Parks et al. first reported a peduncular, intraluminal liposarcoma of the colon [2]. Unlike in the present case, this liposarcoma was pleomorphic, with lymph node
Fig. 1 Colonoscopic intraluminal view. Note the obturation due to the intraluminal mass
metastasis. However, the macroscopic appearance (a peduncular polypoid submucosal tumor) of the present case is very similar to that reported by Parks et al. Subsequently, Chen reported a well-differentiated liposarcoma, which formed an intraluminal polypoid mass and an ill-defined nodule in the adjacent mesocolon [4]. Shahidzadeh et al. described a colonic peduncular well-differentiated liposarcoma polyp, which was excised by endoscopic polypectomy [6]. Chaudhary et al. also reported a peduncular, intraluminal liposarcoma, which caused colo-colic intussusception as in the present case [7]. In summary, the present case is the fifth of peduncular liposarcoma of the colon reported in the English literature and the first of dedifferentiated peduncular liposarcoma. D’Annibale et al. described a colonic endoluminal liposarcoma with a dissemination of the tumor at the stomach, omentum, mesentery, and mesocolon [8]. This liposarcoma was histopathologically diagnosed as pleomorphic liposarcoma, which was mainly located in the submucosal layer without a narrow stalk, thus not applicable to peduncular liposarcoma. Türkoğlu et al. reported dedifferentiated liposarcoma of the transverse colon [11], which was located in the bowel wall and invaded greater curvature of the stomach, without the feature of peduncular liposarcoma. Notably, except in the case presented by Parks et al., the three previously reported peduncular liposarcomas were welldifferentiated liposarcomas in the submucosa. These patients’ ages were from 52 to 66 years, whereas the present patient was 84 years old. Weiss and Rao explain that dedifferentiation of liposarcoma is not a site-specific phenomenon but is more likely time-dependent [15]. They reported that the median time to dedifferentiation were 8 and 14 years for welldifferentiated liposarcoma of the retroperitoneum and groin, respectively. We speculate that dedifferentiation may also occur in the well-differentiated liposarcoma of the colon, as
S250 Fig. 2 Macroscopic features of the colonic tumor. Peduncular polypoid intraluminal tumor (white arrow) with a slender stalk (asterisk) is noted at ascending colon. Histopathological examination revealed that spindle tumor cells invaded the adjacent adipose tissues (black arrow). The cut section of the tumor revealed gray solid nodules (Inset, asterisk also indicates stalk)
Fig. 3 Histopathological and immunohistochemical findings of the tumor. a, b, and c: Dedifferentiated liposarcoma cells are major components of the tumor. a The tumor is mainly located in the submucosal layer. b Note the spindle-shaped tumor cells with storiform-like growth pattern. c Strong CDK4 immunoreactivity is found in tumor cells. d, e, and f: A well-differentiated liposarcoma component is also found in the tumor. d Irregular variable size adipose tissue is found in the submucosa. Note the multinucleated lipoblasts e with floret-like features and f with S-100 immunoreactivity
J Gastrointest Canc (2014) 45 (Suppl 1):S248–S251
J Gastrointest Canc (2014) 45 (Suppl 1):S248–S251
proposed by Weiss and Rao. The difference in patient age, 52 to 66 years old for well-differentiated liposarcoma and 84 years for the dedifferentiated liposarcoma in the present case, might reflect the periods of dedifferentiation in colonic submucosal liposarcoma. The prognosis for colonic liposarcoma, especially peduncular liposarcoma, is difficult to predict due to the limited number of cases reported. It may be affected by a variety of factors, including size, dissemination, as well as histopathological type. In conclusion, we demonstrated a rare liposarcoma, which exhibited a unique intraluminal projecting polypoid mass with a narrow stalk, or peduncular liposarcoma of the colon. Further case reports may be helpful in understanding the pathobiological properties of colonic liposarcoma and moreover contribute to the general understanding of liposarcoma.
References 1. Wood DL, Morgenstern L. Liposarcoma of the ileocecal valve: a case report. Mt Sinai J Med. 1989;56:62–4. 2. Parks RW, Mullan FJ, Kamel HM, Walsh MY, McKelvey ST. Liposarcoma of the colon. Ulster Med J. 1994;63:111–3. 3. Magro G, Gurrera A, Di Cataldo A, Licata A. Vasquez: well differentiated lipoma like liposarcoma of the caecum. Histopathology. 2000;36:378–80. 4. Chen KT. Liposarcoma of the colon: a case report. Int J Surg Pathol. 2004;12:281–5. 5. Gutsu E, Ghidirim G, Gagauz I, Mishin I, Iakovleva I. Liposarcoma of the colon: a case report and review of literature. J Gastrointest Surg. 2006;10:652–6.
S251 6. Shahidzadeh R, Ponce CR, Lee JR, Chamberlain SM. Liposarcoma in a colonic polyp: case report and review of the literature. Dig Dis Sci. 2007;52:3377–80. 7. Chaudhary A, Arora R, Sharma A, Aggarwal S, Safaya R, Sharma S. Primary colonic liposarcoma causing colo-colic intusussception: a case report and review of literature. J Gastrointest Cancer. 2007;38: 160–3. 8. D’Annibale M, Cosimelli M, Covello R, Stasi E. Liposarcoma of the colon presenting as an endoluminal mass. World J Surg Oncol. 2009;7:78. 9. Jarboui S, Moussi A, Jarraya H, Ben Mna K, Abdesselem MM, Kourda A, et al. Primary dedifferentiated liposarcoma of the colon: a case report. Gastroenterol Clin Biol. 2009;33(10–11): 1016–8. 10. Choi YY, Kim YJ, Jin SY. Primary liposarcoma of the ascending colon: a rare case of mixed type presenting as hemoperitoneum combined with other type of retroperitoneal liposarcoma. BMC Cancer. 2010;10:239. 11. Türkoğlu MA, Elpek GÖ, Doğru V, Calış H, Uçar A, Arıcı C. An unusual case of primary colonic dedifferentiated liposarcoma. Int J Surg Case Rep. 2014;5:8–11. 12. Takeuchi T, Misaki A, Liang SB, Tachibana A, Hayashi N, Sonobe H, et al. Expression of T-cadherin (CDH13, H-Cadherin) in human brain and its characteristics as a negative growth regulator of epidermal growth factor in neuroblastoma cells. J Neurochem. 2000;74: 1489–97. 13. Thway K, Flora R, Shah C, Olmos D, Fisher C. Diagnostic utility of p16, CDK4, and MDM2 as an immunohistochemical panel in distinguishing well-differentiated and dedifferentiated liposarcomas from other adipocytic tumors. Am J Surg Pathol. 2012;36:462–9. 14. Sato Y, Yamamoto S, Fujita S. Retroperitoneal liposarcoma with colonic involvement: a case report. Jpn J Clin Oncol. 2014;44:374–8. 15. Weiss SW, Rao VK. Well-differentiated liposarcoma (atypical lipoma) of deep soft tissue of the extremities, retroperitoneum, and miscellaneous sites. A follow-up study of 92 cases with analysis of the incidence of “dedifferentiation”. Am J Surg Pathol. 1992;11:1051–8.
CASE REPORT
Peduncular Liposarcoma of the Colon: a Case Report and Literature Review Yusuke Kito & Tetsuya Fujii & Takeyoshi Nishiyama & Chiemi Saigo & Tatuo Okumoto & Nobuji Yokoyama & Tamotsu Takeuchi
Published online: 13 September 2014 # Springer Science+Business Media New York 2014
Introduction Liposarcoma is one of the most common types of soft tissue tumor in adults. Most liposarcomas occur in the extremities or retroperitoneum, whereas primary liposarcoma of the alimentary tract is rare. Especially, colonic liposarcoma remains limitedly reported in eleven cases [1–11] (summarized in Table 1). Because of its low incidence rate, the pathobiological properties of primary colonic liposarcoma remain largely unknown. In this sentence, accumulation of cases is desired. Here, we describe a case of colonic liposarcoma, which exhibited a unique peduncular growth pattern in the colonic lumen. The tumor was largely composed of dedifferentiated liposarcoma cells, with an intense and diffuse cyclindependent kinase 4 (CDK4) immunoreactivity. Notably, a well-differentiated liposarcoma component was also observed in the submucosal region of the tumor. To our knowledge, this is the first case of dedifferentiated peduncular colonic liposarcoma.
Case Presentation An 84-year-old woman who presented with abdominal pain due to intussusception was admitted to Himeji St. Maria
Y. Kito : C. Saigo : T. Takeuchi (*) Department of Pathology and Translational Research, Gifu University Graduate School of Medicine, Yanagido 1-1, Gifu 501-1194, Japan e-mail: [email protected] T. Fujii : T. Nishiyama : T. Okumoto : N. Yokoyama Department of Surgery, Himeji St. Maria Hospital, Nitoyo, Himeji 670-0801, Japan
Hospital. A colonoscopy was performed, showing an obstructive intraluminal mass (Fig. 1), and the patient underwent a right hemicolectomy. Macroscopic examination of the resected ascending colon revealed a dumbbell-shaped polypoid mass, which occupied the colonic lumen. This polypoid tumor measured 10×3×3 cm with a narrow stalk approximately 1 cm in diameter and was covered by mucosa with a focal ulceration (Fig. 2). The cut surface demonstrated a multilobulated circumscribed grayish polypoid mass (Fig. 2, Inset). Histopathological examination revealed that the mass was largely composed of a sarcomatous spindle cell tumor harboring giant cells, which often exhibited bizarre nuclei. These sarcomatous tumor cells cohesively occupied the submucosa and focally invaded the muscularis propria and subserosal tissues. Numerous mitotic figures were observed. Notably, tumor areas composed of irregular-shaped adipose tissues with multivacuolar lipoblasts were also found. The representative microscopic morphological features are shown in Fig. 3. There was no evidence of metastasis in regional resected lymph nodes. Subsequently, we performed immunohistochemical staining as previously reported [12]. Notably, CDK4 and S-100 immunoreactivities were found in proliferating spindle tumor cells and multivacuolar lipoblasts, respectively (Fig. 3c, f, respectively). Immunohistochemical staining was negative for CD117, CD34, and HMB45. It is well established that diffuse, intense CDK4 immunoreactivity in sarcomatous tumor is useful for the diagnosis of dedifferentiated liposarcoma [13]. Taking the results of the immunohistochemical analysis into consideration, we concluded that this peduncular tumor was a dedifferentiated liposarcoma with focal peripheral welldifferentiated liposarcoma. After surgery, the patient was discharged without any complications.
J Gastrointest Canc (2014) 45 (Suppl 1):S248–S251
S249
Table 1 Reported characteristics of cases of colonic liposarcoma
1 2 3 4 5 6 7 8 9 10 11 12
Wood and Morgenstern Parks et al. Magro et al. Chen Gutsu et al. Shahidzadeh et al. Chaudhary et al. Jarboui et al. D’Annibale et al. Choi et al. Türkoğlu et al. Present case
Age
Sex
Location
Histological subtype
62 54 65 52 46 56 66 69 79 41 71 84
F F F F M F F M F M F F
Ileocecal valve Ascending colon Cecum Descending colon Ascending colon Hepatic flexure Descending colon Splenic flexure Hepatic flexure Ascending colon Transverse colon Ascending colon
Myxoid Pleomorphic Well-differentiated Well-differentiated Myxoid Well-differentiated Well-differentiated Dedifferentiated Pleomorphic Myxoid Dedifferentiated Dedifferentiated
Bold denotes a case of peduncular liposarcoma, which is characterized by a macroscopic appearance of an intraluminal polypoid tumor with a slender stalk
Discussion Until now, only eleven cases of primary liposarcoma of the colon had been found in the English literature [1–11]. However, unsurprisingly, several of these colonic liposarcomas are found on the peritoneal side, sometimes forming a peritoneal mass with invasion to adjacent tissues, i.e., the stomach, omentum, or mesentery. It is likely that these colonic liposarcomas may arise in adipose tissues in the mesocolon and subsequently invade the colonic wall. In fact, Sato et al. recently described a retroperitoneal liposarcoma, which infiltrated to form a multinodular solid mass in the lumen [14]. The pathobiological properties of these liposarcomas may be similar to those of retroperitoneal liposarcomas because of their robust tumor infiltration. By contrast, Parks et al. first reported a peduncular, intraluminal liposarcoma of the colon [2]. Unlike in the present case, this liposarcoma was pleomorphic, with lymph node
Fig. 1 Colonoscopic intraluminal view. Note the obturation due to the intraluminal mass
metastasis. However, the macroscopic appearance (a peduncular polypoid submucosal tumor) of the present case is very similar to that reported by Parks et al. Subsequently, Chen reported a well-differentiated liposarcoma, which formed an intraluminal polypoid mass and an ill-defined nodule in the adjacent mesocolon [4]. Shahidzadeh et al. described a colonic peduncular well-differentiated liposarcoma polyp, which was excised by endoscopic polypectomy [6]. Chaudhary et al. also reported a peduncular, intraluminal liposarcoma, which caused colo-colic intussusception as in the present case [7]. In summary, the present case is the fifth of peduncular liposarcoma of the colon reported in the English literature and the first of dedifferentiated peduncular liposarcoma. D’Annibale et al. described a colonic endoluminal liposarcoma with a dissemination of the tumor at the stomach, omentum, mesentery, and mesocolon [8]. This liposarcoma was histopathologically diagnosed as pleomorphic liposarcoma, which was mainly located in the submucosal layer without a narrow stalk, thus not applicable to peduncular liposarcoma. Türkoğlu et al. reported dedifferentiated liposarcoma of the transverse colon [11], which was located in the bowel wall and invaded greater curvature of the stomach, without the feature of peduncular liposarcoma. Notably, except in the case presented by Parks et al., the three previously reported peduncular liposarcomas were welldifferentiated liposarcomas in the submucosa. These patients’ ages were from 52 to 66 years, whereas the present patient was 84 years old. Weiss and Rao explain that dedifferentiation of liposarcoma is not a site-specific phenomenon but is more likely time-dependent [15]. They reported that the median time to dedifferentiation were 8 and 14 years for welldifferentiated liposarcoma of the retroperitoneum and groin, respectively. We speculate that dedifferentiation may also occur in the well-differentiated liposarcoma of the colon, as
S250 Fig. 2 Macroscopic features of the colonic tumor. Peduncular polypoid intraluminal tumor (white arrow) with a slender stalk (asterisk) is noted at ascending colon. Histopathological examination revealed that spindle tumor cells invaded the adjacent adipose tissues (black arrow). The cut section of the tumor revealed gray solid nodules (Inset, asterisk also indicates stalk)
Fig. 3 Histopathological and immunohistochemical findings of the tumor. a, b, and c: Dedifferentiated liposarcoma cells are major components of the tumor. a The tumor is mainly located in the submucosal layer. b Note the spindle-shaped tumor cells with storiform-like growth pattern. c Strong CDK4 immunoreactivity is found in tumor cells. d, e, and f: A well-differentiated liposarcoma component is also found in the tumor. d Irregular variable size adipose tissue is found in the submucosa. Note the multinucleated lipoblasts e with floret-like features and f with S-100 immunoreactivity
J Gastrointest Canc (2014) 45 (Suppl 1):S248–S251
J Gastrointest Canc (2014) 45 (Suppl 1):S248–S251
proposed by Weiss and Rao. The difference in patient age, 52 to 66 years old for well-differentiated liposarcoma and 84 years for the dedifferentiated liposarcoma in the present case, might reflect the periods of dedifferentiation in colonic submucosal liposarcoma. The prognosis for colonic liposarcoma, especially peduncular liposarcoma, is difficult to predict due to the limited number of cases reported. It may be affected by a variety of factors, including size, dissemination, as well as histopathological type. In conclusion, we demonstrated a rare liposarcoma, which exhibited a unique intraluminal projecting polypoid mass with a narrow stalk, or peduncular liposarcoma of the colon. Further case reports may be helpful in understanding the pathobiological properties of colonic liposarcoma and moreover contribute to the general understanding of liposarcoma.
References 1. Wood DL, Morgenstern L. Liposarcoma of the ileocecal valve: a case report. Mt Sinai J Med. 1989;56:62–4. 2. Parks RW, Mullan FJ, Kamel HM, Walsh MY, McKelvey ST. Liposarcoma of the colon. Ulster Med J. 1994;63:111–3. 3. Magro G, Gurrera A, Di Cataldo A, Licata A. Vasquez: well differentiated lipoma like liposarcoma of the caecum. Histopathology. 2000;36:378–80. 4. Chen KT. Liposarcoma of the colon: a case report. Int J Surg Pathol. 2004;12:281–5. 5. Gutsu E, Ghidirim G, Gagauz I, Mishin I, Iakovleva I. Liposarcoma of the colon: a case report and review of literature. J Gastrointest Surg. 2006;10:652–6.
S251 6. Shahidzadeh R, Ponce CR, Lee JR, Chamberlain SM. Liposarcoma in a colonic polyp: case report and review of the literature. Dig Dis Sci. 2007;52:3377–80. 7. Chaudhary A, Arora R, Sharma A, Aggarwal S, Safaya R, Sharma S. Primary colonic liposarcoma causing colo-colic intusussception: a case report and review of literature. J Gastrointest Cancer. 2007;38: 160–3. 8. D’Annibale M, Cosimelli M, Covello R, Stasi E. Liposarcoma of the colon presenting as an endoluminal mass. World J Surg Oncol. 2009;7:78. 9. Jarboui S, Moussi A, Jarraya H, Ben Mna K, Abdesselem MM, Kourda A, et al. Primary dedifferentiated liposarcoma of the colon: a case report. Gastroenterol Clin Biol. 2009;33(10–11): 1016–8. 10. Choi YY, Kim YJ, Jin SY. Primary liposarcoma of the ascending colon: a rare case of mixed type presenting as hemoperitoneum combined with other type of retroperitoneal liposarcoma. BMC Cancer. 2010;10:239. 11. Türkoğlu MA, Elpek GÖ, Doğru V, Calış H, Uçar A, Arıcı C. An unusual case of primary colonic dedifferentiated liposarcoma. Int J Surg Case Rep. 2014;5:8–11. 12. Takeuchi T, Misaki A, Liang SB, Tachibana A, Hayashi N, Sonobe H, et al. Expression of T-cadherin (CDH13, H-Cadherin) in human brain and its characteristics as a negative growth regulator of epidermal growth factor in neuroblastoma cells. J Neurochem. 2000;74: 1489–97. 13. Thway K, Flora R, Shah C, Olmos D, Fisher C. Diagnostic utility of p16, CDK4, and MDM2 as an immunohistochemical panel in distinguishing well-differentiated and dedifferentiated liposarcomas from other adipocytic tumors. Am J Surg Pathol. 2012;36:462–9. 14. Sato Y, Yamamoto S, Fujita S. Retroperitoneal liposarcoma with colonic involvement: a case report. Jpn J Clin Oncol. 2014;44:374–8. 15. Weiss SW, Rao VK. Well-differentiated liposarcoma (atypical lipoma) of deep soft tissue of the extremities, retroperitoneum, and miscellaneous sites. A follow-up study of 92 cases with analysis of the incidence of “dedifferentiation”. Am J Surg Pathol. 1992;11:1051–8.
