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ARTICLE IN PRESS
JVS-431; No. of Pages 3
Journal of Visceral Surgery (2014) xxx, xxx—xxx
Available online at
ScienceDirect www.sciencedirect.com
SURGICAL IMAGES
Primary rectal linitis: A rare clinical condition C. Sabbagh a,b, E. Chapuis-Roux a,b, D. Chatelain b,c, J.-M. Regimbeau a,b,∗ a
Service de chirurgie digestive et oncologique, CHU d’Amiens, place Victor-Pauchet, 80054 Amiens cedex 1, France b Université de Picardie-Jules-Verne, chemin du Thil, 80000 Amiens, France c Service d’anatomo-pathologie, CHU d’Amiens, place Victor-Pauchet, 80054 Amiens cedex 1, France
KEYWORDS Rectal linitis; Oncological management
Summary Rectal linitis is a rare tumor with a poor prognosis that may be seen as a primary tumor or in association with gastric linitis plastica. In this article we describe the morphological appearance on CT and magnetic resonance imaging, which is typical. We also discuss the oncologic management, which is not well defined, including the potential value of neo-adjuvant treatment. © 2014 Elsevier Masson SAS. All rights reserved.
Clinical case presentation A 62-year old patient presented with a circumferential tumor of the low rectum that was impassable for the colonoscope. Biopsies showed a signet-cell adenocarcinoma. It was classified as an mrT3N0 lesion based on magnetic resonance imaging (MRI) with a virtually zero circumferential margin (Fig. 1A and B). Thoraco-abdominal-pelvic CT scan found no evidence of metastatic lesions. Upper endoscopy and echo-endoscopy showed no evidence of gastric primary tumor. Neoadjuvant radiochemotherapy (RCT) combining 50.4 grays and xeloda was proposed. MRI re-evaluation after RCT showed no evidence of tumor regression (Fig. 1C and D). At surgery, the patient was found to have diffuse peritoneal carcinomatosis with a Peritoneal Cancer Index (PCI) Score of 35. Proctectomy was performed because of the patient’s severe rectal symptoms. Performance of mesorectal dissection was difficult because of the circumferential mesorectal infiltration by the tumor that completely prevented traction and counter-traction maneuvers typically performed during total mesorectal excision (Fig. 2A). Pathology examination classified the lesion as an ypT4N1M1 with zero circumferential margin (Fig. 2B and C). Adjuvant chemotherapy with FOLFOX and Erbitux was proposed in a multidisciplinary conference.
∗
Corresponding author. Service de chirurgie digestive et oncologique, CHU d’Amiens, place Victor-Pauchet, 80054 Amiens cedex 1, France. E-mail address: [email protected] (J.-M. Regimbeau).
http://dx.doi.org/10.1016/j.jviscsurg.2014.09.005 1878-7886/© 2014 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Sabbagh C, et al. Primary rectal linitis: A rare clinical condition. Journal of Visceral Surgery (2014), http://dx.doi.org/10.1016/j.jviscsurg.2014.09.005
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ARTICLE IN PRESS C. Sabbagh et al.
Figure 1. A. Pre-treatment MRI: transverse cut in T2 phase before neo-adjuvant RCT treatment showing thickening of the rectal wall (*) with a diffuse circumferential infiltration of the mesorectum (◦ ). B. Pre-treatment MRI: sagittal cut in T2 phase, showing thickening of the rectal wall (*). C. Post-treatment MRI: transverse cut in T2 phase showing a lack of tumoral response to neo-adjuvant RCT therapy (*). D. Post-treatment MRI: sagittal cut in T2 phase showing unchanged thickening of the rectal wall consistent with a lack of response to neo-adjuvant RCT therapy.
Figure 2. A. Operative view of the resected specimen showing the tubular aspect of the mesorectum. B. Macroscopic appearance of a transverse section of the operative specimen showing tumor invasion through the full thickness of the rectal wall and the mesorectum extending to the circumferential resection margins (blue ink). C. The pathologist’s macroscopic view of the operative specimen showing tumor infiltration of the rectal wall. D. CT appearance (axial cut) of a colorectal linitis showing a typical rigid appearance with segmental thickening of a portion of the rectal tube.
Please cite this article in press as: Sabbagh C, et al. Primary rectal linitis: A rare clinical condition. Journal of Visceral Surgery (2014), http://dx.doi.org/10.1016/j.jviscsurg.2014.09.005
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ARTICLE IN PRESS
Primary rectal linitis: A rare clinical condition Primary colorectal linitis represents less than 0.1% of all colorectal tumors [1]. This tumor has a very poor prognosis and is most often metastatic at the time of diagnosis. Diagnosis may often be difficult since transanal biopsies may be too superficial showing normal mucosa that overlies a submucosal tumor involvement [1]. The CT appearance is characterized by the presence of a rigid thickened tubular lumen; the rectal area should be systematically evaluated before surgery for gastric linitis (Fig. 2D). Rectal MRI found an aspect of circumferential mesorectal thickening in T1 sequences and concentric annular narrowing of the rectal wall with submucosal infiltration on T2 sequences [2]. Primary gastric linitis should always be sought in order to avoid missing the diagnosis [3]. Peri-operative management is not well defined and we offered this patient the choice of either immediate surgical resection, analogous to treatment of primary signet-cell carcinoma of the stomach [4], or a course of neoadjuvant RCT [1]. We opted for the latter choice because of the lack of circumferential margin. In conclusion, rectal linitis is a rare form of rectal cancer of the rectum with very poor prognosis; its management is not codified.
3
Disclosure of interest The authors declare that they have no conflicts of interest concerning this article.
References [1] Hyngstrom JR, Hu CY, Xing Y, et al. Clinicopathology and outcomes for mucinous and signet ring colorectal adenocarcinoma: analysis from the National Cancer Data Base. Ann Surg Oncol 2012;19:2814—21. [2] Rudralingam V, Dobson MJ, Pitt M, Stewart DJ, Hearn A, Susnerwala S. MR imaging of linitis plastica of the rectum. AJR Am J Roentgenol 2003;181:428—30. [3] Katsinelos P, Papaziogas B, Chatzimavroudis G, et al. Secondary rectal linitis plastica as first manifestation of urinary bladder carcinoma. Ann Gastroenterol 2012;25:173—5. [4] Messager M, Lefevre JH, Pichot-Delahaye V, Souadka A, Piessen G, Mariette C. The impact of perioperative chemotherapy on survival in patients with gastric signet ring cell adenocarcinoma: a multicenter comparative study. Ann Surg 2011;254:684—93 [discussion 693].
Please cite this article in press as: Sabbagh C, et al. Primary rectal linitis: A rare clinical condition. Journal of Visceral Surgery (2014), http://dx.doi.org/10.1016/j.jviscsurg.2014.09.005
ARTICLE IN PRESS
JVS-431; No. of Pages 3
Journal of Visceral Surgery (2014) xxx, xxx—xxx
Available online at
ScienceDirect www.sciencedirect.com
SURGICAL IMAGES
Primary rectal linitis: A rare clinical condition C. Sabbagh a,b, E. Chapuis-Roux a,b, D. Chatelain b,c, J.-M. Regimbeau a,b,∗ a
Service de chirurgie digestive et oncologique, CHU d’Amiens, place Victor-Pauchet, 80054 Amiens cedex 1, France b Université de Picardie-Jules-Verne, chemin du Thil, 80000 Amiens, France c Service d’anatomo-pathologie, CHU d’Amiens, place Victor-Pauchet, 80054 Amiens cedex 1, France
KEYWORDS Rectal linitis; Oncological management
Summary Rectal linitis is a rare tumor with a poor prognosis that may be seen as a primary tumor or in association with gastric linitis plastica. In this article we describe the morphological appearance on CT and magnetic resonance imaging, which is typical. We also discuss the oncologic management, which is not well defined, including the potential value of neo-adjuvant treatment. © 2014 Elsevier Masson SAS. All rights reserved.
Clinical case presentation A 62-year old patient presented with a circumferential tumor of the low rectum that was impassable for the colonoscope. Biopsies showed a signet-cell adenocarcinoma. It was classified as an mrT3N0 lesion based on magnetic resonance imaging (MRI) with a virtually zero circumferential margin (Fig. 1A and B). Thoraco-abdominal-pelvic CT scan found no evidence of metastatic lesions. Upper endoscopy and echo-endoscopy showed no evidence of gastric primary tumor. Neoadjuvant radiochemotherapy (RCT) combining 50.4 grays and xeloda was proposed. MRI re-evaluation after RCT showed no evidence of tumor regression (Fig. 1C and D). At surgery, the patient was found to have diffuse peritoneal carcinomatosis with a Peritoneal Cancer Index (PCI) Score of 35. Proctectomy was performed because of the patient’s severe rectal symptoms. Performance of mesorectal dissection was difficult because of the circumferential mesorectal infiltration by the tumor that completely prevented traction and counter-traction maneuvers typically performed during total mesorectal excision (Fig. 2A). Pathology examination classified the lesion as an ypT4N1M1 with zero circumferential margin (Fig. 2B and C). Adjuvant chemotherapy with FOLFOX and Erbitux was proposed in a multidisciplinary conference.
∗
Corresponding author. Service de chirurgie digestive et oncologique, CHU d’Amiens, place Victor-Pauchet, 80054 Amiens cedex 1, France. E-mail address: [email protected] (J.-M. Regimbeau).
http://dx.doi.org/10.1016/j.jviscsurg.2014.09.005 1878-7886/© 2014 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Sabbagh C, et al. Primary rectal linitis: A rare clinical condition. Journal of Visceral Surgery (2014), http://dx.doi.org/10.1016/j.jviscsurg.2014.09.005
+Model JVS-431; No. of Pages 3
2
ARTICLE IN PRESS C. Sabbagh et al.
Figure 1. A. Pre-treatment MRI: transverse cut in T2 phase before neo-adjuvant RCT treatment showing thickening of the rectal wall (*) with a diffuse circumferential infiltration of the mesorectum (◦ ). B. Pre-treatment MRI: sagittal cut in T2 phase, showing thickening of the rectal wall (*). C. Post-treatment MRI: transverse cut in T2 phase showing a lack of tumoral response to neo-adjuvant RCT therapy (*). D. Post-treatment MRI: sagittal cut in T2 phase showing unchanged thickening of the rectal wall consistent with a lack of response to neo-adjuvant RCT therapy.
Figure 2. A. Operative view of the resected specimen showing the tubular aspect of the mesorectum. B. Macroscopic appearance of a transverse section of the operative specimen showing tumor invasion through the full thickness of the rectal wall and the mesorectum extending to the circumferential resection margins (blue ink). C. The pathologist’s macroscopic view of the operative specimen showing tumor infiltration of the rectal wall. D. CT appearance (axial cut) of a colorectal linitis showing a typical rigid appearance with segmental thickening of a portion of the rectal tube.
Please cite this article in press as: Sabbagh C, et al. Primary rectal linitis: A rare clinical condition. Journal of Visceral Surgery (2014), http://dx.doi.org/10.1016/j.jviscsurg.2014.09.005
+Model JVS-431; No. of Pages 3
ARTICLE IN PRESS
Primary rectal linitis: A rare clinical condition Primary colorectal linitis represents less than 0.1% of all colorectal tumors [1]. This tumor has a very poor prognosis and is most often metastatic at the time of diagnosis. Diagnosis may often be difficult since transanal biopsies may be too superficial showing normal mucosa that overlies a submucosal tumor involvement [1]. The CT appearance is characterized by the presence of a rigid thickened tubular lumen; the rectal area should be systematically evaluated before surgery for gastric linitis (Fig. 2D). Rectal MRI found an aspect of circumferential mesorectal thickening in T1 sequences and concentric annular narrowing of the rectal wall with submucosal infiltration on T2 sequences [2]. Primary gastric linitis should always be sought in order to avoid missing the diagnosis [3]. Peri-operative management is not well defined and we offered this patient the choice of either immediate surgical resection, analogous to treatment of primary signet-cell carcinoma of the stomach [4], or a course of neoadjuvant RCT [1]. We opted for the latter choice because of the lack of circumferential margin. In conclusion, rectal linitis is a rare form of rectal cancer of the rectum with very poor prognosis; its management is not codified.
3
Disclosure of interest The authors declare that they have no conflicts of interest concerning this article.
References [1] Hyngstrom JR, Hu CY, Xing Y, et al. Clinicopathology and outcomes for mucinous and signet ring colorectal adenocarcinoma: analysis from the National Cancer Data Base. Ann Surg Oncol 2012;19:2814—21. [2] Rudralingam V, Dobson MJ, Pitt M, Stewart DJ, Hearn A, Susnerwala S. MR imaging of linitis plastica of the rectum. AJR Am J Roentgenol 2003;181:428—30. [3] Katsinelos P, Papaziogas B, Chatzimavroudis G, et al. Secondary rectal linitis plastica as first manifestation of urinary bladder carcinoma. Ann Gastroenterol 2012;25:173—5. [4] Messager M, Lefevre JH, Pichot-Delahaye V, Souadka A, Piessen G, Mariette C. The impact of perioperative chemotherapy on survival in patients with gastric signet ring cell adenocarcinoma: a multicenter comparative study. Ann Surg 2011;254:684—93 [discussion 693].
Please cite this article in press as: Sabbagh C, et al. Primary rectal linitis: A rare clinical condition. Journal of Visceral Surgery (2014), http://dx.doi.org/10.1016/j.jviscsurg.2014.09.005
