Primary Signet Ring Cell Carcinoma Research Articles

The Impact of Epidemiological Factors and Treatment Interventions on Survival in Patients With Signet Ring Cell Carcinoma of the Pancreas.

Primary pancreatic signet ring cell carcinoma (SRCC) is a rare histologic variant of pancreatic carcinoma. A population-based analysis of pancreatic SRCC was performed to determine the predictive effects of epidemiological factors and treatment interventions on overall survival (OS) and disease-specific survival (DSS). The Surveillance, Epidemiology, and End Results registry was searched for pancreatic SRCC cases diagnosed between January 1, 1973 and December 31, 2013. Statistical analysis was performed using the Fisher exact test, χ(2) analysis, Kaplan-Meier method, log-rank test, and Cox proportional hazards regression. The mean age among 497 patients was 66.6 years (SD, 11.9). Most patients were white (82.7%) and male (54.5%). The 1-, 2-, and 5-year OS rates were 17%, 9%, and 4%, respectively, while the corresponding 1-, 2-, and 5-year rates for DSS were 18%, 10%, and 5%, respectively. On univariable analysis; age, site, grade, stage, and treatment were predictive of OS and DSS (P<0.05). On multivariable analysis; radiation improved OS and DSS (adjusted hazard ratio [aHR], 0.592 and 0.589, respectively), pancreatectomy improved OS and DSS (aHR, 0.360 and 0.355, respectively), and combination therapy improved OS and DSS (aHR, 0.295 and 0.286, respectively). Age, site, and stage were also independent predictors of OS and DSS. Subgroup analysis demonstrated treatment to be an independent predictor of OS and DSS in localized/regional disease, in distant disease, and in patients diagnosed between 2000 and 2013. Age, site, stage, and treatment independently predict OS and DSS in pancreatic SRCC.

Palliative Radiation Therapy for metastatic Primary Signet Ring Cell Carcinoma of the Lung: A SEER Analysis

Palliative Radiation Therapy for metastatic Primary Signet Ring Cell Carcinoma of the Lung: A SEER Analysis

Stercoral Rectal Ulceration or a Missed Diagnosis of Rectal Carcinoma: Primary Signet Ring Cell Carcinoma of the Rectum Presenting as Fecal Incontinence in an Elderly Female

Primary signet ring cell carcinoma of the rectum is a rare entity accounting for approximately 1% of all colorectal carcinomas. It arises more frequently in the stomach and is associated with younger patient population and advanced stage on presentation. Constipation is the main factor for development of stercoral ulcer which originates as a result of pressure necrosis of the colorectal mucosa. We describe an atypical case of an elderly female with primary signet ring cell carcinoma of the rectum presenting with fecal incontinence. A 68-year old woman presented with worsening fecal incontinence and hematochezia. Rectal exam revealed a firm tissue in the lateral anal canal. The endoscopic evaluation showed a friable partially necrotic ulcerated lesion at the anorectal junction extending at least 5 cm proximally. The histologic examination of the lesion was diagnostic for a primary signet ring cell carcinoma of the rectum (positive for CK 7 and CK 20 and negative for CDX-2). Computed tomography scan of the abdomen/pelvis revealed rectal wall thickening and MRI of the pelvis demonstrated a low rectal tumor with perianal involvement, clinical T3N2 with an enlarged right external iliac lymph node. Patient received chemo-radiation and later underwent laparotomy with abdominal perineal resection and an end colostomy was performed. Patient had good clinical progress and was discharged to a skilled nursing facility. Patient had a colonoscopy done three years prior for similar symptoms and was noted to have stercoral ulcerations in the rectum but rectal biopsies at that time were unremarkable. This case highlights the following important points. Ulcers particularly stercoral ulcers may herald a concealed malignant disease, hence surveillance colonoscopy/sigmoidoscopy should be considered. Also, the diagnosis of primary rectal signet ring cell carcinoma should be investigated in the elderly presenting with the complaint of fecal incontinence.1670_A Figure 1. Colonoscopy revealed a friable, necrotic and ulcerated rectal lesion.1670_B Figure 2. CT scan demonstrated rectal wall thickening.

Primary ovarian signet ring cell carcinoma: A rare case report.

Signet ring cell carcinoma (SRCC) of the ovary is most commonly metastatic from a primary lesion. Primary ovarian SRCC is rare, and the distinction between primary and metastatic SRCC of the ovary may be difficult. We herein present a case of primary SRCC of the ovary in a 54-year-old woman presenting with a right ovarian mass sized 20.5×16.5×11.5 cm. Total abdominal hysterectomy with bilateral salpingo-oophorectomy, partial omentectomy and incidental appendectomy were performed. Upon histological examination, mucinous carcinoma composed predominantly of signet ring cells was observed in the right ovary. The results of immunohistochemical examination included diffuse positivity for cytokeratin (CK)7 and CK20, but the tumor was negative for estrogen receptor, progesterone receptor, caudal type homeobox 2 and Wilms' tumor gene 1. A preoperative computed tomography (CT) scan of the abdomen and a postoperative positron emission tomography-CT scan did not reveal any suspicious extraovarian lesions. Based on the histological and clinicoradiological examinations, this case was diagnosed as a primary ovarian SRCC.

126 A Case of Primary Signet Ring Cell Carcinoma of the Pancreas

126 A Case of Primary Signet Ring Cell Carcinoma of the Pancreas

Primary Signet Ring Cell Carcinoma of Rectum Diagnosed by Boring Biopsy in Combination with Endoscopic Mucosal Resection

A 46-year-old man with severe back pain visited our hospital. Magnetic resonance imaging revealed extensive bone metastasis and rectal wall thickness. Colonoscopy revealed circumferential stenosis with edematous mucosa, suggesting colon cancer. However, histological findings of biopsy specimens revealed inflammatory cells but no malignant cells. The patient underwent endoscopic ultrasound, which demonstrated edematous wall thickness without destruction of the normal layer structure. After unsuccessful detection of neoplastic cells by boring biopsies, we performed endoscopic mucosal resection followed by boring biopsies that finally revealed signet ring cell carcinoma. Herein, we present a case and provide a review of the literature.

Dermatomyositis – Initial Manifestation of Advanced Stage Primary Signet Ring Cell Ovarian Carcinoma

Dermatomyositis is a type of inflammatory myopathy characterized by inflammatory and degenerative changes of the muscles and skin which are almost associated with malignancies. Herein, we describe a case of paraneoplastic dermatomyositis due to primary ovarian signet ring cell carcinoma (POSRC) which is an extremely rare neoplasm of the ovary. We believe this to be the first case of paraneoplastic dermatomyositis as a consequence of POSRC.

Primary Signet Ring Cell Carcinoma of the Gallbladder Mimicking Calculous Cholecystitis

Primary Signet Ring Cell Carcinoma of the Gallbladder Mimicking Calculous Cholecystitis

Clinicopathological Characteristics and Survival Outcomes of Primary Signet Ring Cell Carcinoma of the Prostate: A SEER-Based Study

Clinicopathological Characteristics and Survival Outcomes of Primary Signet Ring Cell Carcinoma of the Prostate: A SEER-Based Study

An Unusual Presentation of Gastric Signet Ring Cell Carcinoma: Lower Back Pain as the Primary Symptom

An Unusual Presentation of Gastric Signet Ring Cell Carcinoma: Lower Back Pain as the Primary Symptom A 50 year old Hispanic woman was admitted with a 6 hour history of severe, shooting mid/lower back pain radiating to the upper left abdomen, sudden in onset, 10/10 intensity. She denied nausea, vomiting, and diarrhea. She had an episode of burning epigastric pain 2 months ago that resolved spontaneously, as well as another episode of back pain. She also reported a recent 15 pound weight loss. She had an H. pylori infection in the past which was treated. Her sister died of breast cancer. She drank alcohol occasionally but denied any drugs or cigarette use. On physical exam, she had normal vital signs but had tenderness in the left upper abdomen and lower paraspinal region. Laboratory studies were unremarkable except for an elevated ALP and CEA. CT scan of the abdomen and pelvis with contrast revealed thickening of the stomach wall and multiple enlarged lymph nodes. CT of the chest without contrast was remarkable for lytic lesions in the sternum and in the T1-L1 spine. An esophagogastroduodenoscopy was done, showing a circumferential, fungating and firm ulcerated mass with friable surfaces in the gastric body. Endoscopic ultrasound showed circumferential mucosal and submucosal thickening with hypoechoic islands in the gastric body, eroding into the propria and serosa with several enlarged lymph nodes. Primary gastric signet ring cell carcinoma (SRCC) was diagnosed on biopsy and was staged as T3N3M1. She declined to have a bone biopsy to confirm metastatic disease. She was not a surgical candidate and was eventually discharged on a course of chemotherapy. This is a unique case of SRCC due to the presenting symptom of back pain. Gastric carcinomas, especially SRCC, typically do not metastasize to the bone. The common sites of metastasis are the lymph nodes (35.4%), peritoneum (30.3%), liver (24.3%), and lung (10.4%). This case highlights the importance of a thorough clinical workup, using a combination of history, physical, labs and imaging. Without this, an uncommon presentation of a serious disease would likely have been missed.FigureFigureFigure

Comparison of CT manifestations of primary colorectal mucinous adenocarcinoma and signet ring cell carcinoma

To compare the difference in CT manifestations between primary colorectal mucinous adenocarcinoma and signet ring cell carcinoma in order to improve radiologic diagnosis. Clinicopathological data and CT findings of 109 patients with colorectal mucinous adenocarcinoma and 46 patients with primary colorectal signet-ring cell carcinoma confirmed by surgery and pathology from March 2008 to February 2015 in the Tumor Hospital of Yunnan Province were retrospectively collected. Differences in age, gender, tumor location, length and thickness of the involved intestinal wall, thickening pattern of the intestinal wall, lesion density, calcification, contrast-enhanced form, peri-intestinal invasion, occurrence of intestinal obstruction and metastasis of other organs were compared between the two groups. Among 109 patients with colorectal mucinous adenocarcinoma, 68 were men and 41 were women with a mean age of (56.8±15.4) years. Among 46 patients with primary colorectal signet-ring cell carcinoma, 26 were men and 20 were women with a mean age of (42.9±15.6) years. Compared with mucinous adenocarcinoma group, signet-ring cell carcinoma group showed more concentric bowel-wall thickening[93.5%(43/46) vs. 81.6%(89/109), χ2=9.19, P=0.030], higher lesion density [(42.0±3.0) Hu vs. (28.5±1.5) Hu, t=37.30, P=0.000], more marked enhancement [54.3%(25/46) vs. 12.8%(14/109), χ2=35.21, P=0.000], less vast-low-density region in enhanced CT imaging[2.2%(1/46) vs. 45.0%(49/109), χ2=73.31, P=0.000] and more severe peri-intestinal invasion [41.3% (19/46) vs. 17.4%(19/109), χ2=10.25, P=0.006]. Calcification was found in 18.3%(20/109) of mucinous adenocarcinoma cases, but was not found in signet-ring cell carcinoma cases (χ2=9.69, P=0.002). Target ring sign in contrast-enhanced scan was observed in 15.2%(7/46) of signet-ring cell carcinoma cases, while in none of mucinous adenocarcinoma cases (χ2=17.37, P=0.000). There were no statistically significant differences in lesion location, length and thickness of the involved intestinal wall, occurrence of intestinal obstruction, lymph node metastasis, liver or peritoneum metastasis between two groups(all P>0.05). Signet-ring cell carcinoma is often found in younger patients, whose CT manifestation is characterized by the target ring sign in contrast-enhanced scan, while primary colorectal mucinous adenocarcinoma is often in older patients, whose CT manifestation is characterized by calcification in unenhanced scan and low density region in enhanced CT show.

A New Case of Primary Signet Ring Cell Carcinoma of the Uterine Cervix: A Case Report and Review of the Literature

Primary signet-ring cell carcinoma of cervix is extremely rare in the literature. Usually Signet-Ring Cell Carcinomas (SRCCs) of the cervix are metastatic from a primary gastric, colonic, ovarian, or breast carcinoma. A 48-year-old woman was referred to our Department due to persistent abnormal vaginal bleeding during the last two months. Gynecologic examination revealed cervical tumor. Biopsy revealed a signet ring cell type of mucinous adenocarcinoma. Extensive systemic examination reveals liver metastases biopsies confirmed. The patient was treated with palliative chemotherapy. The prognosis of primary signet ring cell adenocarcinoma of the uterine cervix is still unclear because of the rare incidence of cases. In this report we reviewed the literature to identify the clinical, pathological and immunohistochemical features of this rare malignancy.

A potential pitfall in evaluating HER2 immunohistochemistry for gastric signet ring cell carcinomas

Signet ring cell carcinoma (SRC) more commonly presents as metastatic disease and renders patients to be considered for chemotherapy. While treatment options are limited overall, trastuzumab has been shown to be effective for HER2 positive SRC cases. The current algorithm for HER2 evaluation heavily relies on positive membrane-specific staining by immunohistochemistry (IHC), but several anecdotal reports have suggested that SRCs may be susceptible to misinterpretation due to non-specific staining in the marginated cytoplasm with/without nucleus. Results of two FDA-approved IHC methods of HER2 evaluation, Pathway and HercepTest, along with silver in situ hybridisation were interpreted retrospectively and compared in 155 primary SRC cases. IHC results were discrepant between the two assays in SRC even at the strongest IHC scores. Discordance indeed occurred due to dark, non-specific staining obscured by the unique SRC morphology. True HER2 positive SRC was identified in three cases (3/155, 1.9%). In this study, we demonstrate the importance of recognising potential discrepancy in interpreting HER2 status depending on the assay used. Understanding this possible pitfall may prevent unnecessary trastuzumab in SRC patients.

Reacquisition of E-cadherin expression in metastatic deposits of signet-ring cell carcinoma of the upper gastrointestinal system: a potential anchor for metastatic deposition

AimsTo examine the expression of E-cadherin in paired primary and metastatic signet-ring cell carcinomas (SRCC) of various organ systems in order to explore the potential role of the molecule in...

Primary signet ring cell carcinoma of the prostate treated by radical cystoprostatectomy and chemoradiotherapy.

Primary signet ring cell carcinoma (SRCC) of the prostate is very rare. Although SRCC is primarily found in the stomach and colon, it can also be found in the pancreas, breast, thyroid, bladder, and prostate. We recently diagnosed and treated a case of primary SRCC of the prostate. A 56-year-old Korean man was referred to our institution for evaluation of a one-month history of hematuria and recently identified bladder mass. Transurethral resection of the bladder tumour was performed and histological and immunohistochemical evaluation revealed a diagnosis of SRCC with tumour invading into the outer half of the deep muscularis propria. After three weeks, the patient had radical cystoprostatectomy with ileal conduit. Tumour involved both prostate and bladder, but the centre of the tumour was located in the prostate. Duodenoscopy and colon fibroscopy both indicated no evidence of tumour origin in the gastrointestinal (GI) tract. Overall, this tumour was regarded as primary SRCC of the prostate. Concurrent chemoradiotherapy (CCRT) using leucovorin and fluorouracil was initiated two months later. The patient eventually developed bone and liver metastases and died of hepatopathy.

Primary tiny pure signet-ring cell carcinoma of vermiform appendix presenting as bilateral huge Krukenberg tumors

Primary signet-ring cell carcinoma (SRCC) of vermiform appendix is extremely rare; only three cases have been reported in the English literature. A 52-year-old woman presented with abdominal mass. Physical examination revealed bilateral huge tumors in her ovarian sites. Blood tests demonstrated anemia and elevated tumor markers (CEA, 40 ng/ml; CA125, 580 ng/ml; CA72.4, 1771 U/ml; STN-AG, 19,000 U/ml; sialyl LE, 75 U/ml). Serum AFP, CA19-9, and CA15-3 were within normal limits. Upper and lower gastrointestinal endoscopy revealed no remarkable pathology. CT and MRI showed huge bilateral ovarian solid tumors without clinically apparent other tumors. The clinical diagnosis was primary bilateral ovarian tumors and bilateral oophorectomy was performed. During operation, several quick frozen sections were performed and both ovarian tumors (left: 18 cm, right: 13 cm) were found to be Krukenberg tumors. Accordingly, gynecologists did comprehensive abdominal examination to find out the primary site. They found a small tumor (3 cm × 1 cm) in the distal part of vermiform appendix, frozen sections of which revealed an SRCC. Cytologic evaluation of associated ascites at the operation was positive for carcinoma cells. Then the diagnosis of primary SRCC of appendix with both ovarian metastases (Krukenberg tumors) with peritoneal dissemination was given. Subsequent formalin fixation pathological examination gave the diagnosis. Immunohistochemically, the signet ring cells were positive for cytokeratin (CK) AE1/3, CK CAM5.2, CK8, CK18, CK19, CK20, EMA, CEA, CA19-9, p53, Ki-67 in 50% of tumor cells, CDX2, and MUC2. They were negative for CK34E1, CK5/6, CK7, CK14, p63, vimentin, TTF-1, MUC1, MUC5AC, MUC6, NSE, synaptophysin, chromogranin, and CD56. She was now treated with chemotherapy 3 months after the operation.

Primary Signet-ring Cell Carcinoma of the Uterine Corpus: A Case Report and Review of the Literature

Pure signet-ring cell carcinomas of the uterine corpus usually raise the suspicion for a metastatic tumor from other primary sites, e.g., breast or the gastrointestinal tract. And if primary in the uterus, it is more commonly of uterine cervical origin with secondary involvement of the corpus. In general, pure mucinous carcinoma of the endometrium is rare and pure signet-ring cell carcinoma, a form of mucinous carcinoma, is even rarer. Only sparse case reports of primary mucinous adenocarcinoma of the uterine corpus with signet-ring cell features have been described in literature. We report here a case of pure mucinous signet-ring cell carcinoma of the uterine corpus in a 63 year-old morbidly obese woman.

Primary signet ring cell carcinoma of the colon and rectum

Colorectal primary signet ring cell carcinoma (SRCC) is a rare entity accounting for nearly 1% of all colorectal carcinomas. It is an independent prognostic factor associated with less favorable outcome. This aggressiveness is mainly due to the intrinsic biology of these tumors. Here is an overview of the literature related to clinicopathological features, molecular biology, and management of SRCC of the colon and the rectum.

Malignant sweat gland tumours: an update

Cutaneous adnexal tumours can be a diagnostic challenge for the pathologist. This is particularly true in the case of tumours with sweat gland differentiation, due to a large number of rare entities, a multiplicity of names to designate the same neoplasms and consequent lack of consensus regarding their classification and nomenclature. In the traditional view, sweat gland tumours were divided into eccrine and apocrine. However, this has been challenged in recent years, and in fact many of these tumours may have both eccrine and apocrine variants. Some display more complex features and defy classification, due to the presence of other lines of differentiation, namely follicular and/or sebaceous (in the case of apocrine tumours, due to the close embryological relationship between apocrine glands, hair follicles and sebaceous glands). The present paper reviews and updates the basic concepts regarding the following malignant sweat gland tumours: apocrine carcinoma, porocarcinoma, hidradenocarcinoma, spiradenocarcinoma, cylindrocarcinoma, microcystic adnexal carcinoma and related entities, squamoid eccrine ductal carcinoma, digital papillary adenocarcinoma, primary cutaneous mucinous carcinoma, endocrine mucin-producing sweat gland carcinoma and primary cutaneous signet ring cell carcinoma. Particular emphasis is put in recent findings that may have implications in the diagnosis and management of these tumours.

Metastatic signet-ring cell carcinoma of the urinary bladder: A novel management approach to a rare tumour.

Primary signet-ring cell carcinoma (SRCC) of the urinary bladder, a variant of adenocarcinoma, is exceedingly rare and as a result no gold standard exists for its management. We report a case of primary SRCC of the bladder with recurrent metastases; we utilized an innovative diagnostic approach and the patient exhibited a treatment response to palliative FOLFOX-6 chemotherapy.