Int J Colorectal Dis DOI 10.1007/s00384-014-2034-4
LETTER TO THE EDITOR
Primary signet ring cell carcinoma of rectum: a rare entity with unusual presentation Jitin Yadav & Sanjay Kumar Yadav & Kumar Gaurav & Aftab Ahmed & Kattamanchi Adithya Reddy & Om Prakash
Accepted: 9 October 2014 # Springer-Verlag Berlin Heidelberg 2014
Introduction Laufman and Saphir first described primary signet ring cell carcinoma of the colon and rectum in 1951 [1]. Primary signet ring cell carcinoma of colon and rectum is a distinctive malignant disease and rarer than conventional adenocarcinoma, with a reported incidence ranging from 0.01 to 2.6 % [2]. Signet cell carcinoma is a poorly differentiated type of adenocarcinoma which behaves more aggressively and is more common in younger patients (especially under 40) compared to other types of colorectal cancers. Herein, we use a typical patient along with relevant literature to illustrate the multimodality approach to signet ring cell cancer rectum (SRCC) and discuss important factors associated with the treatment and prognosis.
narrowing of lumen and perirectal strandings without any evidence of distant metastases. Patient underwent abdominoperineal resection and end colostomy. Postoperative period was uneventful and patient was discharged on the sixth postoperative day. The final pathology from the resection specimen demonstrated freely scattered signet ring cells infiltrating into the muscularis propria, margins were negative. Lymph node metastasis was present. Chemotherapy was started with 5-FU 450 mg/ m2 and luecovorin 100 mg/m2 every 3 weeks for 6 cycles. After 6 months of completion of chemotherapy, patient had no evidence of recurrence or metastasis on computed tomography. He is in regular follow-up and doing well.
Discussion Case discussion We report a 19-year-old male patient who presented with a 4-month history of intermittent bleeding per rectum, tenesmus, and mass per rectum. On digital rectal examination, a firm to hard palpable mass from 3 to 11 o’clock was noted approximately 1 cm proximal to the anal verge. Rigid proctoscopy demonstrated two circumferential masses with bleeding. Multiple biopsies were taken, and pathologic results were consistent with signet ring cell carcinoma. Computed tomography (CT) suggested diffuse concentric thickening of rectum with J. Yadav : S. K. Yadav (*) : K. Gaurav : A. Ahmed : K. A. Reddy : O. Prakash Department of General Surgery, Rajendra Institute of Medical Sciences, Hostel No. 03, Room No. 50, Rims, Ranchi, Jharkhand 834009, India e-mail: [email protected]
Primary SRCC of the rectum is a very rare tumor, which shows a significant number of tumor cells with intracellular mucin accumulation which pushes the nucleus to the periphery giving a signet ring appearance. Regardless of the tissue origin, SRCCs frequently metastasize to the regional lymph nodes, peritoneal surfaces, ovaries, and lungs. In pure SRCC of the rectum, the lesion is more aggressive than adenocarcinoma, presents at an early age and carries poor prognosis. One of the characteristic features of colorectal SRCC is the late manifestation of symptoms, and many of them are diagnosed at advanced stages [3]. Colorectal SRCC usually presents with rectal bleeding, small bowel obstruction, and abdominal pain. Diagnosis of colorectal SRCC does not differ from that of the conventional adenocarcinomas. In addition to the routine blood tests and radiological assessments, endoscopic biopsy is the common diagnostic technique for detecting the tumor in colorectum [3]. The 5-year
Int J Colorectal Dis
survival rate in larger series of colorectal SRCC range from 0–12 %. Disease recurrence is more frequent in colorectal SRCC compared to mucinous adenocarcinomas. Due to its relative rarity, considerable dispute exists regarding the optimal multimodality approach for SRCC rectum. While it is clear that surgical resection is favored, the extent of surgery has been called into question as up to 99 % of patients with colorectal SRCC present with advance tumors, either because of distant metastases or aggressive locoregional disease (stage III/ IV) [4]. Eighty-four percent of patients present with local lymphatic spread [4]. This aggressive local disease led to the suggestion that an abdominoperineal resection (APR) be the treatment of choice in order to address local lymph nodes. Due to the rarity of this disease process, no prospective, randomized trials can definitively elucidate the ideal surgical approach. Given the morbidity of APR and higher recurrence rate (36.8 %) [4] with any surgical approach, there has been persistent concerns regarding an aggressive surgical approach with no clearly demonstrated survival advantage. Combination chemotherapy of Levamisole and 5-FU and FOLFOX regimen which includes fluorouracil, calcium folinate, oxaliplatin, and bevacizumab (FOLFOX + bevacizumab) has been tried with only minimal survival benefit. Patients with SRCC have significantly worse 5-year overall survival (29.5 %) [4]. Because there was no distant metastasis, we did APR with end colostomy and patient received 6 cycles of chemotherapy. Our case is rare, because despite late presentation, patient is doing well after 1 year of surgery and is having no sign of recurrence.
Conclusion Rectosigmoid SRCC is a rare malignancy that seems to have a poor prognosis, with distant metastases and locoregional infiltration being a common and fatal development. While neither surgery nor chemotherapy has had a clear impact on survival, adjuvant chemotherapy does appear to provide disease control and slightly improved survival. Due to lack of feasibility of conducting a randomized controlled trial, it is difficult to establish proper multimodality approach. Despite this, our experience leads us to recommend a complete R0 resection with adjuvant chemotherapy for patients with anorectal SRCC.
Conflict of interest Authors declare no conflict of interest.
References 1. Laufman H, Safir O (1951) Primary linitus plastica type of carcinoma of the colon. AMA Arch Surg 62:79–91 2. Anthony T, George R, Rodriguez-Bigas M, Petrelli NJ (1996) Primary signet-ring cell carcinoma of the colon and rectum. Ann Surg Oncol 3: 344–348 3. Fu KI, Sano Y, Kato S, Saito H, Ochiai A, Fujimori T, Saito Y, Matsuda T, Fujii T, Yoshida S (2006) Primary signet-ring cell carcinoma of the colon at early stage: a case report and a review of the literature. World J Gastroenterol 12:3446–3449 4. Lin H-H, Lin C-C, Lan Y-T, Wang H-S, Yang S-H, Jiang J-K, Chen W-S, Lin T-C, Lin J-K, Chang S-C (2012) Clinicopathological differences between mucinous adenocarcinoma and signet-ring cell carcinoma in the colorectum. J Soc Colon Rectal Surg (Taiwan) 23:151–159
LETTER TO THE EDITOR
Primary signet ring cell carcinoma of rectum: a rare entity with unusual presentation Jitin Yadav & Sanjay Kumar Yadav & Kumar Gaurav & Aftab Ahmed & Kattamanchi Adithya Reddy & Om Prakash
Accepted: 9 October 2014 # Springer-Verlag Berlin Heidelberg 2014
Introduction Laufman and Saphir first described primary signet ring cell carcinoma of the colon and rectum in 1951 [1]. Primary signet ring cell carcinoma of colon and rectum is a distinctive malignant disease and rarer than conventional adenocarcinoma, with a reported incidence ranging from 0.01 to 2.6 % [2]. Signet cell carcinoma is a poorly differentiated type of adenocarcinoma which behaves more aggressively and is more common in younger patients (especially under 40) compared to other types of colorectal cancers. Herein, we use a typical patient along with relevant literature to illustrate the multimodality approach to signet ring cell cancer rectum (SRCC) and discuss important factors associated with the treatment and prognosis.
narrowing of lumen and perirectal strandings without any evidence of distant metastases. Patient underwent abdominoperineal resection and end colostomy. Postoperative period was uneventful and patient was discharged on the sixth postoperative day. The final pathology from the resection specimen demonstrated freely scattered signet ring cells infiltrating into the muscularis propria, margins were negative. Lymph node metastasis was present. Chemotherapy was started with 5-FU 450 mg/ m2 and luecovorin 100 mg/m2 every 3 weeks for 6 cycles. After 6 months of completion of chemotherapy, patient had no evidence of recurrence or metastasis on computed tomography. He is in regular follow-up and doing well.
Discussion Case discussion We report a 19-year-old male patient who presented with a 4-month history of intermittent bleeding per rectum, tenesmus, and mass per rectum. On digital rectal examination, a firm to hard palpable mass from 3 to 11 o’clock was noted approximately 1 cm proximal to the anal verge. Rigid proctoscopy demonstrated two circumferential masses with bleeding. Multiple biopsies were taken, and pathologic results were consistent with signet ring cell carcinoma. Computed tomography (CT) suggested diffuse concentric thickening of rectum with J. Yadav : S. K. Yadav (*) : K. Gaurav : A. Ahmed : K. A. Reddy : O. Prakash Department of General Surgery, Rajendra Institute of Medical Sciences, Hostel No. 03, Room No. 50, Rims, Ranchi, Jharkhand 834009, India e-mail: [email protected]
Primary SRCC of the rectum is a very rare tumor, which shows a significant number of tumor cells with intracellular mucin accumulation which pushes the nucleus to the periphery giving a signet ring appearance. Regardless of the tissue origin, SRCCs frequently metastasize to the regional lymph nodes, peritoneal surfaces, ovaries, and lungs. In pure SRCC of the rectum, the lesion is more aggressive than adenocarcinoma, presents at an early age and carries poor prognosis. One of the characteristic features of colorectal SRCC is the late manifestation of symptoms, and many of them are diagnosed at advanced stages [3]. Colorectal SRCC usually presents with rectal bleeding, small bowel obstruction, and abdominal pain. Diagnosis of colorectal SRCC does not differ from that of the conventional adenocarcinomas. In addition to the routine blood tests and radiological assessments, endoscopic biopsy is the common diagnostic technique for detecting the tumor in colorectum [3]. The 5-year
Int J Colorectal Dis
survival rate in larger series of colorectal SRCC range from 0–12 %. Disease recurrence is more frequent in colorectal SRCC compared to mucinous adenocarcinomas. Due to its relative rarity, considerable dispute exists regarding the optimal multimodality approach for SRCC rectum. While it is clear that surgical resection is favored, the extent of surgery has been called into question as up to 99 % of patients with colorectal SRCC present with advance tumors, either because of distant metastases or aggressive locoregional disease (stage III/ IV) [4]. Eighty-four percent of patients present with local lymphatic spread [4]. This aggressive local disease led to the suggestion that an abdominoperineal resection (APR) be the treatment of choice in order to address local lymph nodes. Due to the rarity of this disease process, no prospective, randomized trials can definitively elucidate the ideal surgical approach. Given the morbidity of APR and higher recurrence rate (36.8 %) [4] with any surgical approach, there has been persistent concerns regarding an aggressive surgical approach with no clearly demonstrated survival advantage. Combination chemotherapy of Levamisole and 5-FU and FOLFOX regimen which includes fluorouracil, calcium folinate, oxaliplatin, and bevacizumab (FOLFOX + bevacizumab) has been tried with only minimal survival benefit. Patients with SRCC have significantly worse 5-year overall survival (29.5 %) [4]. Because there was no distant metastasis, we did APR with end colostomy and patient received 6 cycles of chemotherapy. Our case is rare, because despite late presentation, patient is doing well after 1 year of surgery and is having no sign of recurrence.
Conclusion Rectosigmoid SRCC is a rare malignancy that seems to have a poor prognosis, with distant metastases and locoregional infiltration being a common and fatal development. While neither surgery nor chemotherapy has had a clear impact on survival, adjuvant chemotherapy does appear to provide disease control and slightly improved survival. Due to lack of feasibility of conducting a randomized controlled trial, it is difficult to establish proper multimodality approach. Despite this, our experience leads us to recommend a complete R0 resection with adjuvant chemotherapy for patients with anorectal SRCC.
Conflict of interest Authors declare no conflict of interest.
References 1. Laufman H, Safir O (1951) Primary linitus plastica type of carcinoma of the colon. AMA Arch Surg 62:79–91 2. Anthony T, George R, Rodriguez-Bigas M, Petrelli NJ (1996) Primary signet-ring cell carcinoma of the colon and rectum. Ann Surg Oncol 3: 344–348 3. Fu KI, Sano Y, Kato S, Saito H, Ochiai A, Fujimori T, Saito Y, Matsuda T, Fujii T, Yoshida S (2006) Primary signet-ring cell carcinoma of the colon at early stage: a case report and a review of the literature. World J Gastroenterol 12:3446–3449 4. Lin H-H, Lin C-C, Lan Y-T, Wang H-S, Yang S-H, Jiang J-K, Chen W-S, Lin T-C, Lin J-K, Chang S-C (2012) Clinicopathological differences between mucinous adenocarcinoma and signet-ring cell carcinoma in the colorectum. J Soc Colon Rectal Surg (Taiwan) 23:151–159
