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Background: Signet-ring cell adenocarcinoma of the colon is well-recognized in adult patients who are extremely rare and not well-documented in children. Our study aims to raise awareness about this rare disease and its long-term outcomes., Methods: We retrospectively evaluated patients with signet-ring cell colon adenocarcinoma., Results: Six patients, three boys and three girls, with a mean age of 14.83 (range, 13-17 years), presented with signs of intesti-nal obstruction and were diagnosed with signet-ring cell colon adenocarcinoma. All patients had air-fluid levels on abdominal X-ray. Abdominal ultrasonography of all patients revealed subileus. Abdominal computed tomography was performed in five patients, and pre-operative colonoscopy was conducted in two patients before the emergency intervention. All of the patients underwent emergent exploratory laparotomy with the preliminary diagnosis of acute abdomen. In two patients, debulking surgery followed by a stoma was performed. The remaining four patients were treated with anastomosis following intestinal resection. All girls had metastases on the ovary. One of the patients died due to the burden of multiple metastases in the early period, and three died in the sixth post-operative year. We have been following the remaining two patients since then., Conclusion: Although signet-ring cell carcinomas (SRCCs) are rare, they should be considered in the differential diagnosis of acute abdomen and intestinal obstruction in pediatric patients. Despite early diagnosis and treatment, SRCC has a poor prognosis in the pediatric population.

Background: The impact of adjuvant chemotherapy (AT) on resected colon adenocarcinoma based on histologic subtype is poorly defined and extrapolated from patients with advanced disease. We evaluated the receipt and effect of AT on overall survival stratified by histologic subtype-mucinous, non-mucinous, and signet ring adenocarcinomas., Methods: A retrospective cohort study from 2004 to 2015 was conducted using the National Cancer Database. Patients with colon adenocarcinoma who underwent curative resection with pathologic stage III were included. Appendiceal and rectal tumors were excluded. The predictor variable was histologic subtype, and outcome variables were overall survival and receipt of AT., Results: Absolute survival was increased for mucinous, non-mucinous, and signet ring tumors with receipt of AT (88.1, 108.9, and 38.1 months, respectively). In multivariable analysis, there was no difference in overall survival for mucinous patients relative to non-mucinous patients. In subgroup analysis, a modest survival advantage for non-mucinous patients relative to the mucinous patients was observed (HR, 0.92; 95% CI, 0.89-0.95). In multivariable modeling, non-mucinous and signet ring adenocarcinoma had decreased odds of receipt of AT relative to mucinous adenocarcinoma patients., Conclusions: Histologic subtype is an important prognostic factor for overall survival for stage III colon adenocarcinoma. Although the magnitude of the benefit of AT may vary in stage III curatively resected patients, it has a substantial survival benefit across all histologic subtypes. Based on these observations, there is no indication that patients with stage III mucinous adenocarcinoma of the colon should not receive AT. All patients with resected stage III colon cancer should be referred for AT regardless of histologic subtype.

Competing Interests: None

A 35-year-old man was referred to our hospital for chronic abdominal pain and diarrhea. Computed tomography showed wall thickening, poor contrast enhancement and calcification of the ascending colon, which were consistent with phlebosclerotic colitis. Malignant character was not detected from a biopsy specimen. Operatively, we observed a scirrhous mass of the ascending colon invading surrounding tissue, which was diagnosed as signet ring cell carcinoma based on analysis of an intraoperative frozen section. Right hemicolectomy with regional lymph node dissection was performed. This case was extremely similar to phlebosclerotic colitis in clinical findings; surgical resection was required for correct diagnosis., Competing Interests: No potential conflict of interest relevant to this article was reported.

Cutaneous vasculitis may behave as a paraneoplastic syndrome. Paraneoplastic vasculitis as a phenomenon of colonic adenocarcinoma has been described in the literature. To the best of my knowledge, this is the first case report of urticarial vasculitis being an initial presentation of signet-ring cell carcinoma of the colon. I here report a case of 27-year-old woman who had rash, arthritis, and episodic bleeding per rectum. The rash was biopsy-proven to be leukocytoclastic vasculitis. There was only partial response to high-dose steroid, and rectal bleeding continued. Colonoscopy revealed a stenosing growth of sigmoid colon, which proved to be signet ring cell carcinoma of the colon. It is important to remain vigilant with regard to the association between leukocytoclastic vasculitis and malignancies.

Signet ring cell colorectal carcinoma is a rare aggressive tumor. The incidence of this desease is very low in young patients and accounts up to 1% of all surgical patients with colorectal cancer. The infrequency of the disease among young patients makes the diagnosis more difficult and the prognosis less favourable. Biopsy results are crucial for verification of signet ring cell carcinoma. Treatment is similar as in other types of colorectal cancer. We report a case of signet ring cell colon cancer in a 22 years old female patient.The aim of this paper is to point out this rare case of infrequent colorectal cancer in a young female patient.

A 81-year-old woman confined to full bed rest due to the effects of a stroke 7 years prior, was brought to our hospital with acute cholecystitis in late April 2013. After PTGBD, her condition improved and she was discharged in early June. However, she was urgently hospitalized at the beginning of August with melena and a fever. A detailed examination revealed aspiration pneumonia, which was resolved with a course of antibiotics. A colonoscopy, conducted to find the cause of the melena, revealed a circumferential type 2 tumor in the ascending colon, and a fiberscope was unable to pass through the stenosis. A biopsy confirmed a diagnosis of signet-ring cell carcinoma. Using abdominal computed tomography, thickening of the ascending colon wall, suggesting infiltration to the periphery, was visible. The adjacent lymph nodes were enlarged, but there were no clear signs of liver metastasis. Cancerous peritonitis was suspected due to the presence of ascitic fluid. Considering the overall condition of the patient, surgery was not performed, and colonic stent was not placed due to the proximity of the stenosis to the ileocecal valve. The patient's family chose best supportive care. The patient's condition worsened and she died 3 months after leaving the hospital.

Primary signet ring cell carcinoma of the colorectum detected at an early stage is very rare; most cases are detected at an advanced stage. Therefore, its prognosis is poorer than that of ordinary colorectal cancer. A 56-year-old Korean man was seen at this hospital for management of signet ring cell carcinoma of the colon. Colonoscopic examination revealed a IIa-like, ill-defined and flatly elevated 9-mm residual tumor in the cecum. Endoscopic mucosal resection was preformed. Pathological examination of the resected specimen revealed signet ring cell carcinoma that had invaded the lamina propria without venous or perineural invasion. Abdominal computed tomography (CT) and positron CT showed no evidence of primary lesions or distant metastasis. An additional laparoscopic right-hemicolectomy was performed; no residual tumor or lymph node metastasis was found. We report a case of primary signet ring cell carcinoma of the colon detected at an early stage and provide a review of the literature.

We report a case of microcystic/reticular schwannoma of the proximal sigmoid colon in a 61-year-old man. A 12-mm polyp was detected while the patient was undergoing screening for colorectal neoplasm. This rare variant of schwannoma was initially described in 2008 and shows a predilection for the visceral organs, predominantly the gastrointestinal tract. We also review 11 other reported cases of microcystic/reticular schwannomas in the gastrointestinal tract. Unlike conventional gastrointestinal schwannomas, which are more common in the stomach, this variant appears to be more common in the large intestine. Histologic examination of this polyp showed predominant lipoblast-like vacuolated cells within a myxoid stroma with focal spindle cell areas. Features suggestive of malignancy, like nuclear pleomorphism, mitosis, or necrosis, were absent. Immunohistochemistry for S100 protein showed strong nuclear and cytoplasmic positivity, whereas cytokeratin and CD117 stains were negative. It is important to entertain microcystic/reticular schwannoma in the differential diagnosis of a signet ring cell adenocarcinoma or a myxoid gastrointestinal stromal tumor, particularly on small biopsy specimens.

The presence of synchronous multiple primary carcinomas, although recognized, remains very uncommon. Their etiopathogeny is unclear. The wide spread of imaging techniques currently facilitates diagnosis of simultaneous tumors. We present the rare case of a signet ring cell colon carcinoma coexisting with a transitional cell carcinoma (TCC) of the ureter, which debuted with symptoms of intestinal obstruction in a 75-year-old male patient, with no prior relevant history. Computed tomography showed signs of a tumor mass on the transverse colon, as well as left hydronephrosis with a tumor in the lower third of the ureter, which prompted for immediate emergency surgery. After the appropriate surgical maneuvers, tissue collected from both tumors underwent usual histological preparation and Hematoxylin-Eosin staining, as well as multiple immunostaining with a complex panel of markers. The patient had a favorable postoperative course and during a six-month follow-up, we did not detect any signs of illness.

Background: Signet ring carcinoma is an exceedingly rare and aggressive variant of primary bladder carcinoma. The cytomorphologic features of this rare entity in urinary specimens have not been well characterized., Methods: Twenty-seven cases of signet ring cell carcinoma of the urinary bladder were identified from the pathology archives of The Johns Hopkins Hospital (1989-2011). Of these, 6 cases with prior urinary cytology specimens were studied., Results: There were 5 males and 1 female, with a mean age of 62 years. The presenting complaints included hematuria with or without symptoms of bladder irritation. Histopathologically, there were 5 cases of primary bladder carcinoma and 1 case of metastatic colonic signet ring cell carcinoma. The salient cytomorphologic features included scattered malignant epithelial cells, displaying distinct cell borders, abundant cytoplasm with a single large, discrete mucin vacuole, and eccentric irregular nuclei with prominent nucleoli. Primary adenocarcinoma additionally revealed a few intact malignant glandular epithelial fragments. Metastatic colonic signet ring cell carcinoma displayed predominantly singly dispersed malignant cells with eccentrically placed, oval nuclei with occasional small nucleoli and a moderate amount of vacuolated cytoplasm., Conclusion: Signet ring cell carcinomas are rarely encountered in urinary cytology. The differential diagnosis includes distended histiocytes or degenerated urothelial cells, primary signet ring cell carcinoma, and metastatic adenocarcinoma., (Copyright © 2012 S. Karger AG, Basel.)

An 86 year old male with a four-day history of nonspecific gastrointestinal symptoms was found on colonoscopy to have evidence of sigmoid colon obstruction and possible perforation. Emergent operative exploration revealed diffuse peritonitis, sigmoid perforation, adjacent dense adhesions, and a foreign body protruding through the perforated area. Pathologic examination showed the foreign body to be a sliver of bone consistent with chicken bone and the sigmoid subacute perforation to be associated distally with a circumferential ulcerated obstructing mass, microscopically seen to be transmurally infiltrating adenocarcinoma, signet-ring cell type. There was extensive acute and organizing peritonitis, 100% Escherichia coli was cultured from peritoneal fluid, and the patient died two days postoperatively with sepsis and hypotension. This appears to be the fifth reported case of colonic perforation resulting from foreign body perforation due to previously undiagnosed adenocarcinoma. The four previously reported cases were all deeply invasive adenocarcinoma of sigmoid colon, and the foreign bodies included three chicken/poultry bones and a metallic staple. These five cases are highly unusual examples of a potentially lethal malignant neoplasm being clinically revealed by a usually (but not always) innocuous event, the ingestion of a small foreign body.

Background: Krukenburg's tumour, a metastatic cancer to the ovary can pose difficulties in early diagnosis. The paucity of reports from our environment coupled with the difficulties we encountered makes it imperative that we report this case., Methods: We present a 32-year-old lady who presented with mucoid and bloody diarrhoea associated with menstrual irregularity, weight loss and lower abdominal pains 2 years after surgical treatment for gastric cancer. Literature on Krukenburg's tumour was also reviewed., Results: While serum level of CEA was found to be elevated and colonoscopy showed an ulcerated mass obliterating the lumen of the sigmoid colon, abdominal CT scan revealed bilateral adnexal masses. The histology of the colonic lesion showed signet ring carcinoma while the enlarged ovaries turned out to have evidence of metastatic adenocarcinoma., Conclusion: Early diagnosis of KT can be difficult. Periodic surveillance is, therefore, essential especially in those who have had a gastrointestinal malignancy.

A 67-year-old man, who had undergone surgery to resect multiple gastric cancers 4 years ago, visited our hospital for surveillance colonoscopy. Colonoscopy revealed a discolored, 7-mm in diameter, flat-elevated lesion with central depression in the transverse colon near the splenic flexure. Although the findings of endoscopy and barium enema were suggestive of submucosal invasion, the patient chose to undergo endoscopic mucosal resection. Pathological examination of the resected specimen revealed signet-ring cell carcinoma and a positive surgical margin. A second operation was performed, and no residual tumor or metastasis to lymph nodes was found in the resected specimens. Primary colorectal cancers composed of signet-ring cell carcinoma detected and treated at an early stage are extremely rare. We present a case and review the literature.

Primary signet-ring cell carcinoma of the colon is a rare entity with a dismal prognosis, mainly due to a delay in diagnosis. Here, we present a case of a 30-year-old Filipino woman who presented with symptoms mimicking inflammatory bowel disease. A barium enema and colonoscopy demonstrated a stricture in the rectosigmoid region. A biopsy revealed granulomatous changes indicative of inflammatory bowel disease. Despite initial improvement of her symptoms on total parenteral nutrition and steroids, the patient relapsed several weeks later with recurrent left lower quadrant pain. A subsequent biopsy revealed poorly differentiated signet-ring cell carcinoma of the colon. She was treated surgically with a left hemi-colectomy and primary repair. A high degree of suspicion is necessary to correctly diagnose these, often young, patients with primary signet-ring cell carcinoma early and have a positive impact on survival. The literature on primary signet-ring cell carcinoma is reviewed.

We describe a case of adenomatous polyp of the colon that harbored small foci of signet ring cell carcinoma. The patient was a 64-year-old woman with end-stage renal disease and sepsis who underwent colonoscopy to evaluate the possibility of pseudomembranous colitis. A polyp was found incidentally in the right colon and a biopsy was performed. Histologic examination of the polyp revealed typical features of tubular adenoma without evidence of high-grade dysplasia. However, 2 small foci of signet ring cell carcinoma were identified that infiltrated the lamina propria. In contrast to adenomatous epithelium, the signet ring cells were immunohistochemically positive for cytokeratin 7 and negative for cytokeratin 20, suggesting a metastasis rather than a primary tumor. Multiple random biopsies from the right and left colon, as well as the ileum, exhibited no histologic evidence of malignancy. Subsequently, signet ring cell carcinoma with similar morphology and identical immunophenotype was detected in biopsies from the endometrium, an unusual location for primary signet ring cell carcinoma. Preliminary workup excluded the breast as a possible primary site, but further investigation was not possible because of the patient's death with no autopsy granted. To the best of our knowledge, this is the first reported case of metastatic signet ring cell carcinoma to an adenomatous polyp of the colon. This case illustrates the necessity of submitting all polyps entirely and the importance of examining them carefully.

A 72-year-old woman was admitted with a complaint of a sensation of abdominal fullness. Cytologic examination of ascites revealed many poorly differentiated adenocarcinoma cells. Barium enema study and colonoscopy revealed IIa+IIc-type carcinoma of the descending colon. Endoscopic mucosal resection was performed to determine the histological type and the depth of invasion. The resected tumor was 7 x 6 mm in size, and an amorphous pit pattern was observed in the depressed area by stereomicroscopy. Poorly differentiated adenocarcinoma with signet-ring cells had diffusely infiltrated into the deeper part of the submucosal layer. Immunohistochemical findings showed this tumor to have mucin derived from gastric foveolar epithelium, suggesting that the signet-ring cell carcinoma of the colon showed gastric differentiation. Primary signet-ring cell carcinoma of the colon and rectum is a rare form of adenocarcinoma of the large intestine and shows more malignant biological behavior than ordinary colorectal carcinoma. Early diagnosis and curative operation are important.

We describe three cases of benign signet-ring cell aggregates in the colon associated with pseudomembranous colitis, adenomatous polyp of the colon and ulcerated mucosa of the gallbladder excised for gallstones. In all cases, we found loose, benign signet-ring cell aggregates overlying the ulcerated mucosa surface, simulating signet ring-cell carcinoma. The most important sign of the benign signet-ring cell aggregates is that they are always confined to the surface of the mucosa of the intestine or gallbladder mucosa or crypts of the intestinal epithelium. In no case did we see an invasion of these cells into the lamina propria of the mucosa. In all cases, the benign signet-ring cell aggregates were immunohistochemically positive with antibodies to cytokeratins. The occurrence of benign signet-ring cell aggregates is a rare and very misleading diagnostic pitfall which must be differentiated from signet-ring cell carcinoma of the colon and gallbladder.