Early-stage primary signet ring cell carcinoma of the colon

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 progno-sis 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 co-lon. 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 re-vealed 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 me-tastasis. An additional laparoscopic right-hemicolectomy was performed; no residual tumor or lymph node me-tastasis 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.     

Endoscopic features of early-stage signet-ring-cell carcinoma of the stomach

AIM: To identify the features of early signet ring cell gastric carcinoma using magnification endoscopy with narrow band imaging (NBI).METHODS: A retrospective review was conducted of 12 cases of early signet ring cell gastric carcinoma who underwent treatment in a single institution between January 2009 and April 2013. All patients had magnification endoscopy with NBI and indigo carmine contrast to closely examine the mucosal architecture, including the microvasculature and arrangement of gastric pits. Histologic examination of the final endoscopic submucosal dissection or gastrectomy specimen was performed and compared with the endoscopic findings to identify patterns specific to signet ring cell carcinoma.RESULTS: Twelve patients with early signet ring cell gastric carcinoma were identified; 75% were male, and average age was 61 years. Most of the lesions were stage T1a (83%), while the remainder were T1b (17%). The mean lesion size was 1.4 cm². On standard endoscopy, all 12 patients had a pale, flat lesion without any evidence of mucosal abnormality such as ulceration, elevation, or depression. On magnification endoscopy with NBI, all of the patients had irregularities in the glands and microvasculature consistent with early gastric cancer. In addition, all 12 patients exhibited the “stretch sign”, an elongation or expansion of the architectural structure. Histologic examination of the resected specimens demonstrated an expanded and edematous mucosal layer infiltrated with tumor cells.CONCLUSION: The “stretch sign” appears to be specific for signet ring cell carcinoma and may aid in the early diagnosis and treatment of this aggressive pathology.     

Primary hepatic signet ring cell neuroendocrine tumor: a case report with literature review

Primary hepatic signet ring cell neuroendocrine tumor is extremely rare and is characterized by distinct intracytoplasmic hyaline vacuoles that are mucin negative and cytokeratin positive. The unique histological features may cause difficulty in diagnosis and delay patient care. Here the authors report a 49-year-old man with an incidental finding of a 2.7 cm liver mass in the absence of chronic liver disease. The resected tumor was grossly unencapsulated but well demarcated with friable tissue texture. Microscopically, the entire tumor consisted of sheets of monotonous cells separated by delicate microvasculature. The tumor cells had granular chromatin, inconspicuous nucleoli, and eosinophilic cytoplasm. Many of the tumor cells had eccentric, pale intracytoplasmic vacuoles resembling signet ring cells in adenocarcinoma. Immunohistochemical studies showed that the tumor cells were positive for neuroendocrine markers and that the intracytoplasmic vacuoles were negative for mucin but strongly positive for cytokeratins. Careful systemic search including OctreoScan scintigraphy (Mallinckrodt Medical, Inc., St. Louis, MO) and capsule endoscopy failed to reveal any other tumors. A diagnosis of primary hepatic signet ring cell neuroendocrine tumor was established. Ten months after surgery, the patient is well without any other detectable tumor on radiology. Serological neuroendocrine markers are also within normal limits.     

Primary rectal signet ring cell carcinoma with peritoneal dissemination and gastric secondaries

Disseminated signet ring cell carcinomas frequently arise from the stomach. However, primaries in the colon and rectum have also been reported. We present a 68 year old lady who presented with a change in her bowel habit. Colonoscopy showed a stenosing rectal tumour at 7 cm to 8 cm from the anal verge. Multiple scattered ulcers were also noted along the entire length of the colon. Biopsy of the lesions revealed signet ring cell adenocarcinoma. Gastroscopy showed multiple nodules with ulceration over several areas of the stomach which were similar in appearance to the colonic lesions. However, no primary tumour of the stomach was seen. Biopsy of the gastric lesions also showed signet ring cell adenocarcinoma. Computed tomography scan of the abdomen and pelvis revealed circumferential tumour at the rectosigmoid junction with possible invasion into the left ischiorectal fossa. The overall picture was that of a primary rectal signet ring cell carcinoma with peritoneal dissemination. The patient was referred for palliative chemotherapy in view of the disseminated disease. In the present report, we discuss this interesting pathological entity and review the role of various histolological techniques in helping to identify the primary tumor.     

Signet ring cell carcinoma of the extrahepatic bile duct

Most tumors affecting the extrahepatic bile duct are adenocarcinomas; the other histologic types occur only rarely. We herein report the extremely rare case of signet ring cell carcinoma (SRCC) originating from the extrahepatic bile duct. A 55-year-old man was hospitalized for jaundice and pruritus. Computed tomography and positron emission tomography suggested the presence of distal extrahepatic bile-duct cancer. He underwent a pylorus preserving pancreaticoduodenectomy. A histologic study confirmed a signet ring cell neoplasm of the distal common bile duct. Because the upper resection margin was invaded by the tumor, he received postoperative concurrent chemoradiotherapy and four cycles of chemotherapy. The patient has survived with no evidence of recurrence for 2 years. This is the second case of primary SRCC of the distal extrahepatic bile duct reported in the literature; further reports of cases are warranted to determine the nature of SRCC in the extrahepatic bile duct.     

Small gastrointestinal stromal tumor concomitant with early gastric cancer： A case report

The term gastrointestinal stromal tumors (GISTs)is defined diagnostically as the main group of mesenchymal tumors with spindle or epithelioid cells arising from the wall of the gastrointestinal tract with immunohistochemical reactivity for CD117 antibody.Previous studies revealed that cells in GISTs express a growth factor receptor with tyrosine kinase activity (termed c-kit), which is the product of the c-kit protooncogene. The most specific and practical diagnostic criteria for GISTs are: immunohistochemically determined c-kit (CD117) expression; mitotic score; and tumor size.A small GIST concomitant with early gastric cancer is rarely encountered clinically. Herein we have reported a case of a 1.1-cm GIST detected by esophagogastroduo denoscopy concomitant with a Ⅱc type of early gastric cancer (signet ring cell type). It was detected during a routine physical health examination. To our knowledge,this is the first report of a small GIST concomitant with a signet ring cell type of early gastric cancer.     

Two cases of histopathologically advanced (stage IV) early gastric cancer]

Various minimally invasive surgical techniques in some cases of early gastric cancer are becoming common practice. However, there are rare cases of advanced cancer with distant metastasis although the invasion of the gastric wall is limited to the mucosa and/or submucosa (defined as early gastric cancer according to UICC-TNM classification). We report two cases of early gastric cancer with distant metastasis (stage IV). Both tumors were defined as early cancer because they were confined to the submucosa. One was a type IIa early cancer, histologically classifiable as a signet ring cell carcinoma (according to the Japanese Classification of Gastric Carcinoma and UICC-TNM classification); the other was a surface spreading type IIb + IIc, classifiable as a signet ring cell carcinoma, too. Stage IV factors were ovarian metastasis (Krukenberg tumor) in the former and N3 in the latter case.     

Signet ring cell carcinoma of the lower bile duct with rapid growth: report of a case

Signet ring cell carcinoma occurring in the biliary tract is extremely rare. We herein report the case of a 78-year-old Japanese woman demonstrating signet ring cell carcinoma of the lower bile duct with a rapid growth. Computed tomography of the pancreas head pointed out a circular thickness in the lower bile ductal wall and stenosis of the common bile duct. Cholangiography revealed tapering stenosis at the lower bile duct. Biopsy specimens taken from these lesions and scratched specimens taken from stenotic portion of the lower bile duct were analyzed and demonstrated signet ring cell carcinoma. To the best of our knowledge, this is the first reported case of primary signet ring cell carcinoma of the lower bile duct reported in the English literature. Based on our experience, signet ring cell carcinoma of the lower bile duct is considered to demonstrate both transmural dispersion and an aggressive nature.     

Metastatic signet ring gastric adenocarcinoma presenting with microangiopathic hemolytic anemia.

Metastatic adenocarcinoma presenting as microangiopathic hemolytic anemia (MAHA) and leukoerythroblastic blood picture is rare. We report three patients who presented with MAHA as the initial symptom of metastatic signet ring cell gastric adenocarcinoma. One patient had past history of gastric ulcer. In all these patients the initial diagnosis was based on peripheral blood smear followed by bone marrow biopsy; upper GI endoscopy showed presence of gastric ulcers with focally scattered neo-plastic signet ring cells on histopathology. All patients died within a week of diagnosis.     

Correlation of plasma CEA and CEA tissue staining in poorly differentiated colorectal cancer

A review of patients operated on for colorectal cancer disclosed poorly differentiated histologies in those whose preoperative and postoperative carcinoembryonic antigen (CEA) levels were not elevated, even in the presence of metastatic disease. CEA was, therefore, of little prognostic value or predictive of disease recurrence in these patients. The amount of CEA in tumor tissue of 17 patients with poorly differentiated colorectal cancer was estimated with the immunoperoxidase staining technique and was correlated with histology and plasma CEA levels obtained during various stages of disease. Five tumors did not stain negatively for CEA and all had predominantly poorly differentiated histologies. In all of these patients metastatic disease developed but not elevated plasma CEA levels. In contrast 12 tumors stained positively for CEA and were found to contain either differentiated areas or signet ring cells. Serial plasma CEA levels correctly monitored the postoperative courses of all 12 patients. Six of these had a relapse and all were detected by serial increases in plasma CEA. The remaining six were disease free >48 months after resection and had normal plasma CEA levels.Among poorly differentiated tumors, those that contain gland-forming areas or signet ring cells can be assumed to produce CEA, and plasma CEA levels can be used effectively for monitoring. On the other hand, undifferentiated tumors which do not stain for CEA identify those patients whose plasma CEA levels do not provide a useful monitor.     

Adenocarcinoma of the colon with neuroendocrine features and secretory diarrhea

We report the case of a 63-yr-old man who had severe secretory diarrhea associated with colonic adenocarcinoma, with a prominent signet ring cell component and numerous endocrine cells as demonstrated by positive chromogranin-A staining. Improvement in the secretory diarrhea by the somatostatin analog Sandostatin suggested that the diarrhea was related to a functional neuroendocrine tumor within the colonic tumor, the first case to be reported in the literature.     

肺转移性印戒细胞癌1例并文献复习

目的研究胃印戒细胞癌肺转移的临床特点。方法介绍1例对肺转移性印戒细胞癌的病例,并进行文献复习。结果以慢性咳嗽患者影像学上支气管周围斑片状阴影及多发纵隔淋巴结肿大并伴有外周血嗜酸粒细胞的增多,应考虑消化道肿瘤的可能。结论恶性度极高的黏液细胞型胃癌,可以跳跃转移到肺及纵膈。如果慢性咳嗽的患者,外周血嗜酸粒细胞的增多时,应尽早行肿瘤标记物及内窥镜的检查,以提高肿瘤的早期诊断率。     

Minute signet ring cell carcinoma occurring in gastric hyperplastic polyp

We describe a 45-year-old woman with minute signet ring cell carcinoma occurring in a gastric hyperplastic polyp. A biopsy specimen obtained from the gastric hyperplastic polyp revealed signet ring cell carcinoma. Endoscopic mucosal resection （EMR） was performed to confirm the diagnosis. Histological examination of the EMR specimen revealed focal signet ring cell carcinoma in the hyperplastic polyp. There are few cases of gastric hyperplastic polyp associated with signet ring cell carcinoma.     

Primary signet ring cell carcinoma of the uterine cervix: A case report

Rationale:Primary signet ring cell carcinoma of the uterine cervix is extremely rare and the clinical characteristics and prognosis are not well known and there are no specific guidelines for treatment.Patient concerns:A 43-year-old woman was referred to our hospital for abnormal uterine bleeding lasting 1 month.Diagnoses:Histological examination revealed a signet ring cell carcinoma of the uterine cervix. After evaluation of extragenital origin, the patient was diagnosed International Federation of Gynecology and Obstetrics stage IIIC1 primary signet ring cell carcinoma or the uterine cervix.Intervention:The patient was prescribed concomitant chemo-radiation followed by intracavitary brachytherapy.Outcomes:She showed no evidence of disease after treatment but, it recurred after 7 months of last treatment.Lessons:Different approaches to diagnosis and treatment of this rare disease are needed and molecular pathological studies related to the onset of the disease are required.     

早期胃癌淋巴结转移规律及内镜切除指征的探讨

目的探讨不同组织学类型早期胃癌的淋巴结转移情况及内镜下治疗的可行性。方法回顾性分析524例行胃癌根治术治疗并经病理确诊的早期胃癌患者的病例资料,比较不同组织学类型早期胃癌的临床病理特征并对其与淋巴结转移的相关性进行单因素及多因素分析。结果印戒细胞癌与分化型腺癌、低分化腺癌相比,在肿瘤大小（P值分别为0．048和0．023）和浸润深度（P值均为0．000）方面差异均有统计学意义,其淋巴结转移率（9．7％,11／113）明显低于低分化型腺癌（22．2％,20／90）,差异有统计学意义（P=0．018）,但与分化型腺癌（13．t％,42／321）比较差异无统计学意义（P=0．406）。单因素分析显示肿瘤大小（P=0．007）、浸润深度（P=0．000）、组织学类型（P=0．030）、淋巴管肿瘤浸润（P=0．000）和有无溃疡（P=0．002）与淋巴结转移显著相关;多因素分析结果显示浸润深度（P=0．007）、肿瘤大小（P=0．010）、组织学分型（P=0．000）和淋巴管肿瘤浸润（P=0．000）为淋巴结转移的独立危险因素。联合上述4个独立危险因素分析显示肿瘤直径小于2cm且无淋巴管肿瘤浸润的印戒细胞型黏膜内癌未见淋巴结转移。结论印戒细胞型早期胃癌的临床病理特征与分化型和低分化型早期腺癌存在差异,直径小于2cm且无淋巴管肿瘤浸润的印戒细胞型黏膜内癌患者可行内镜切除术。     

Microangiopathic hemolytic anemia (MAHA) as paraneoplastic syndrome in metastasized signet ring cell carcinomas: case reports and review of the literature

We report on two spontaneous cases of microangiopathic hemolytic anemia (MAHA) as first manifestation due to metastasized signet ring carcinoma, one of gastric and one of unknown origin. The patients presented with an acute onset of Coombs negative hemolytic anemia and fragmentocytes in the peripheral blood smear which are typical for MAHA. These case reports present MAHA as a rare paraneoplastic syndrome in patients with metastasized signet ring carcinoma. Parallel to symptomatic treatment we started chemotherapy treatment (ELF and PLF regimen, respectively). In both cases we were able to control the MAHA and cancer progression.     

Lymphangitic spread from appendiceal adenocarcinoma to ileocecal valve,mimicking Crohn’s disease

Due to the anatomical peculiarity of the appendix,diagnosis of tumors arising from this area can be challenging by clinicoradiologic means.We report a case of a rare primary appendiceal signet ring carcinoma with an uncommon presentation.An 86-year-old woman was admitted toour hospital with subacute epigastric pain.Computed tomography demonstrated bowel wall thickening with fat stranding in the ileocecal region.The leading diagnostic consideration was inflammatory bowel disease.Upon colonoscopy,a swollen,distorted ileocecal valve was identified.The remaining colon was otherwise unremarkable.Extensive biopsy sampling of the ileocecal region and colon was performed.A lymphangitic signet ring carcinoma within the ileocecal region was diagnosed on biopsy;there was no dysplasia or carcinoma of the remaining biopsies.By cytomorphology and immunoprofile,a lymphangitic signet ring carcinoma of appendiceal origin was the primary consideration,further confirmed upon subsequent laparotomy.This case represents an unusual pattern of appendiceal tumor spread with localized,lymphangitic involvement,creating a milieu which closely simulates Crohn’s disease on imaging modalities.     

Gastric cancer with metastasis to the gingiva

The present case report describes a gastric cancer which showed unusual metastasis in the oral region. A 56-year-old male patient underwent total gastrectomy and splenectomy due to advanced gastric cancer in the upper third of the stomach. Fifteen months later, he presented with anorexia and gingival swelling of durations of approximately 3 and 1 month, respectively. The gastric tumor was histologically a signet ring cell and a poorly differentiated cancer with a moderate degree of vascular invasion. Biopsy specimens from the gingival tumor revealed a signet ring cell cancer. Other metastatic sites were the brain, limb bones and abdominal lymph nodes. A bone scintigram revealed an abnormal uptake in the limb bones, while it did not exhibit any abnormality in the oral region. Correlation between the histology of the gingival tumor with that of the gastric cancer, as well as the absence of a gingival tumor at the time of prior gastrectomy, led to a diagnosis that the gingival tumor was a metastasis from gastric cancer. Gastric cancer metastasizing to the oral region, either the osseus or the oral soft tissue, is very rare. Although it cannot be proved without an autopsy, negative findings in the mandible by bone scanning in the present case suggest that direct gingival metastasis can be considered, rather than mandibular metastasis involving the gingiva. Hematogenous spread could be a mechanism of metastasis for this unusual tumor.     

Primary signet ring cell carcinoma of the appendix： A rare case report and our 18-year experience

Primary adenocarcinoma of the appendix is a rare malignancy that constitutes 〈 0.5% of all gastrointestinal neoplasms. Moreover, primary signet ring cell carc noma of the appendix is an exceedingly rare entity. We have encountered 15 cases of primary appendiceal cancer among 3389 patients who underwent appen- dectomy over the past 18 years. In the present report, we describe a rare case of primary signet ring cell carcinoma of the appendix with ovarian metastases and unresectable peritoneal dissemination occurring in a 67-year-old female patient. She underwent ap- pendectomy and bilateral salpingo-oophorectomy with a laparoscopy procedure. She then received palliative systemic chemotherapy with 12 cycles of oxaliplatin, 5-flurorouracil, and leucovorin （FOIFOX-4）. The patient currently is well without progression of disease 12 mo after beginning chemotherapy.     

Signet-ring cell carcinoma arising from a fundic gland polyp in the stomach

Fundic gland polyps (FGPs) are currently the most common type of gastric polyps and are usually benign. However, although rare, gastric adenocarcinoma of FGP has been recently proposed as a new variant of gastric adenocarcinoma. Here we report the first case of a 49-year-old woman with focal signet ring cell carcinoma that arose from an FGP of the stomach. The tumor was completely excised by endoscopic snare polypectomy. FGPs should therefore be evaluated for malignant changes although they occur rarely, if the FGP has an erosive or irregular surface.