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 ma- lignant 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 adeno- carcinoma 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. 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 exam- ination, 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 circum- ferential masses with bleeding. Multiple biopsies were taken, and pathologic results were consistent with signet ring cell carcinoma. Computed tomography (CT) sug- gested diffuse concentric thickening of rectum with narrowing of lumen and perirectal strandings without any evidence of distant metastases. Patient underwent abdominoperineal resection and end colostomy. Postop- erative period was uneventful and patient was discharged on the sixth postoperative day. The final pathology from the resection specimen demonstrated freely scattered sig- net ring cells infiltrating into the muscularis propria, margins were negative. Lymph node metastasis was present. Chemotherapy was started with 5-FU 450 mg/ m 2 and luecovorin 100 mg/m 2 every 3 weeks for 6 cy- cles. 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 Primary SRCC of the rectum is a very rare tumor, which shows a significant number of tumor cells with intracel- lular 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, endo- scopic biopsy is the common diagnostic technique for detecting the tumor in colorectum [ 3]. The 5-year 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: sky1508@gmail.com Int J Colorectal Dis DOI 10.1007/s00384-014-2034-4