Primary Signet Ring Cell Adenocarcinoma Research Articles

Retraction: Primary signet ring cell adenocarcinoma arising in exstrophy of the urinary bladder (epispadias exstrophy complex)

Retraction: Primary signet ring cell adenocarcinoma arising in exstrophy of the urinary bladder (epispadias exstrophy complex)

A160 A CASE OF BREAST CANCER METASTASIS MASQUERADING AS SIGNET RING CELL ADENOCARCINOMA OF THE STOMACH

Abstract Background Gastric cancer has a poor prognosis, particularly at advanced stages. Endoscopic submucosal dissection (ESD) can be used to treat selected early-stage gastric cancers. This case study explores the challenge of diagnosing and managing patients when metastatic lesions mimic primary gastric cancer, as seen in this case of breast cancer metastasis resembling signet ring cell adenocarcinoma of the stomach. Aims The aim of this case report is to describe an unusual case of breast cancer metastasis to the stomach identified unexpectedly on random gastric biopsies, initially diagnosed as a primary gastric signet ring cell adenocarcinoma. Methods Clinical and pathological records of the described patient were reviewed retrospectively. Furthermore, a review of the relevant literature was also performed. Results A 68 year-old-woman underwent gastroscopy for nausea and vomiting. Her past medical history was remarkable for bilateral mastectomies ten years ago for invasive lobular carcinoma, with metastatic bone disease diagnosed by imaging five years prior. She had been well controlled on letrozole taken intermittently due to side effects. At endoscopy, while the stomach appeared grossly normal, random biopsies done for Helicobacter pylori identified a single focus of signet ring cell adenocarcinoma. Subsequent chromoendoscopy identified an area of subtle mucosal abnormality for which the patient declined surgery and was instead managed by ESD which surprisingly yielded no dysplasia. A follow up gastroscopy with random biopsies also failed to identify any areas of dysplasia, but random biopsies on a subsequent gastroscopy again identified a minute focus of adenocarcinoma with signet ring cell morphology. Given the unusual presentation of apparent signet ring cell adenocarcinoma in two distinct areas in the stomach without any visible endoscopic abnormality an in-depth pathological analysis was conducted. By immunohistochemistry the tumor cells stained positive for Estrogen Receptor, GATA3, and CK7 and negative for antibodies targeting CK20 and CDX2, which supported an interpretation of minute multifocal breast cancer metastases to the stomach from hematogenous spread. Conclusions In this case, repeated endoscopic investigations and immunohistochemical staining revealed minute multifocal breast cancer metastases to the stomach. Conventional tumor staging methods often cannot identify microscopic metastases, potentially impacting disease progression and patient care. A high level of suspicion for metastatic breast cancer and a detailed pathological analysis allowed for the correct diagnosis in this patient. Funding Agencies None

Primary signet ring cell adenocarcinoma arising in exstrophy of the urinary bladder (epispadias-exstrophy complex).

Bladder exstrophy is a rare congenital anomaly in which the urinary bladder develops outside the body. It may be part of the bladder epispadias-exstrophy-cloacal complex, which is a serious embryological defect where a spectrum of abnormalities involving the urinary tract, genital tract, musculoskeletal system, and intestinal tract is seen. Primary signet ring cell adenocarcinoma (PSRCA) arising in exstrophy of the urinary bladder is extremely rare with only a few cases reported in the literature. A 32-year-old man with congenital, untreated bladder exstrophy presented with a large fungating mass on the lower abdominal wall. Physical examination revealed a 15 × 12 cm exophytic mass with a penile epispadias. Ultrasonography showed a replacement of the urinary bladder by a mass of size 9 × 8 × 7 cm with a defect in the abdominal wall. On Contrast-enhanced computed tomography (CECT), a mass protruding from a large, inferior abdominal wall defect of 5.3 cm was seen. Widened pubic symphysis with divarication of recti and an absent urinary bladder, consistent with bladder exstrophy, was also noted. The biopsy showed a signet ring cell adenocarcinoma. A radical cystectomy with ileal conduit and left inguinal lymph node dissection was done. Histopathology examination revealed a PSRCA with metastasis to the left inguinal lymph nodes. Immunohistochemistry (IHC) showed strong positivity with CDX2 and CK20 while CK7 was negative. PSRCA shows an aggressive behavior, as was seen in our case, which had metastatic inguinal lymph nodes at the time of presentation. Metastatic signet ring cell adenocarcinomas from other sites need to be ruled out. Bladder exstrophy can develop high-grade malignancies at any stage of life. Hence, surgical treatment early in life with lifelong follow-up is recommended.

Lymphangitic carcinomatosis as the initial manifestation of primary signet-ring cell adenocarcinoma of the lung: A case report

Signet-ring cell carcinomas are an aggressive, poorly differentiated, and highly invasive adenocarcinoma carrying a poor prognosis. Most of these tumors originate in gastrointestinal organs; however, primary lung signet-ring cell adenocarcinomas can rarely occur. Tumoral lymphatic infiltration is a complication of these tumors and can cause phenomena such as lymphangitic carcinomatosis, characterized by a nodular thickening of the pleura, pleural effusions, and mediastinal lymphadenopathies. We report a case of a 63-year-old ex-smoker with a 2-week clinical course of dyspnea and pleuritic chest pain in which a nodular thickening of the pleura and pleural effusion were documented and led to the diagnosis of a primary signet-ring cell adenocarcinoma of the lung with lymphangitic carcinomatosis. This complication has never been described in the context of a primary lung tumor of this subtype. Both entities carry a high mortality and have no therapeutical options. This report adds to the information available about them.

Primary signet ring cell adenocarcinoma as a rare cause of colonic stenosis in a young patient with Crohn’s disease – the case report

Summary: Signet ring cell carcinoma (SRCC) is a rare type of highly malignant adenocarcinoma that produces mucin and predominantly affects the stomach, ovaries, and the small intestine. Primary localisation in the colon is rare, and the prognosis of this type of adenocarcinoma is very poor. We present a case report of a 44-year-old woman who suffered from Crohn‘s disease beginning in the year 2000. During the last three years of her treatment, she was treated using bio­logical therapy with ustekinumab. The treatment led to clinical, laboratory, and endoscopic remission. In February 2021, she had widely metastatic primary signet ring cell carcinoma of the colon, which mimicked the IBD activity in the case of her colon obstruction. Because of the extensive infiltration of her liver and peritoneum at the time of the dia­gnosis, in addition to the symptoms of the passage disorders she was experiencing, she proceeded with an end ileostomy and palliative treatment was started. The patient‘s prognosis was unfavourable, and she died within 5 months of the dia­gnosis in hospice care. Key words: primary signet ring cell carcinoma – colon cancer – Crohn‘s disease – colonic stenosis

Large Pleural Effusion Secondary to Primary Signet-Ring Cell Adenocarcinoma of the Lung

Primary signet-ring cell adenocarcinomas (SRCA) that occur outside the gastrointestinal tract are rare with few cases reported in the literature. They carry a poor prognosis and can be a challenge to diagnose. In this case report, we present the case of a 69-year-old female who presented to our hospital with a large left-sided pleural effusion. The findings seen on histopathology and immunohistochemistry (IHC) staining of the pleural fluid were consistent with primary signet-ring cell adenocarcinoma of the lung, and further investigations excluded a secondary source of malignancy.To the best of our knowledge, this is the first reported case of pleural effusion without an underlying lung mass diagnosed as primary signet-ring cell adenocarcinoma of the lung. Given how rare primary signet-ring cell adenocarcinoma of the lung is, it is important for physicians to carry out a comprehensive diagnostic workup to differentiate between primary and metastatic signet-ring cell adenocarcinoma as this will determine prognosis and treatment.

Primary Signet-ring Cell Adenocarcinoma of the Urinary Bladder That Invades the Pelvic Wall Treated with Partial Cystectomy and Adjuvant Chemotherapy: A Case Report and Review of the Literature

Primary Signet-ring Cell Adenocarcinoma of the Urinary Bladder That Invades the Pelvic Wall Treated with Partial Cystectomy and Adjuvant Chemotherapy: A Case Report and Review of the Literature

An application of ERBB2 receptor inhibitors in a rare case of S310F somatic ERBB2 mutation of primary signet-ring cell adenocarcinoma of vagina: A case report and review literature of S310F somatic ERBB2 mutation in breast and gynecologic cancers

•The extremely uncommon subtype of epithelial vaginal cancer.•Comprehensive genomic sequencing of the rare subtype vaginal cancer.•Incorporated of the actionable targeted drugs in the treatment of uncommon cancer based on the comprehensive genomic profile.•A promising response of uncommon disease after the actionable targeted therapy.

2677 A Rare Case of Primary Signet Ring Cell Bladder Adenocarcinoma With Metastases to the Stomach

INTRODUCTION: Primary signet ring cell adenocarcinoma (SRCA) of the urinary bladder is a rare malignancy, accounting for about 0.24% of all bladder malignancies. Additionally, metastases to the stomach regardless of primary malignancy is also rare, with rates estimated to be between 0.2-0.7%. Here we are reporting a case of primary SRCA of the bladder with eventual metastases to the stomach. CASE DESCRIPTION/METHODS: A 65-year-old male with rheumatoid arthritis on chronic immunosuppression and history of T4N0M0 primary SRCA of the bladder status-post radical cystectomy was admitted with persistent nausea, vomiting and reduced oral intake leading to acute kidney injury. Symptoms were refractory to conservative measures so CT Abdomen was obtained which revealed distal esophageal wall thickening. Due to concern for infectious esophagitis given his chronic immune suppression, esophagogastroduodenoscopy (EGD) was performed. EGD revealed multiple nodules (5-10 mm) with central umbilication scattered within the gastric fundus and body. The lesions were biopsied and stains were consistent with signet ring cell adenocarcinoma positive for CX7, CX20, CDX-2; Beta-catenin was not performed. Bladder specimen from prior cystectomy was also positive for CX7, CX20, CDX-2, and predominately membranous staining for Beta-catenin. PET scan obtained after EGD was significant only for retroperitoneal lymphadenopathy thought to represent metastases. Previous workup at the time of initial bladder adenocarcinoma diagnosis including colonoscopy and imaging were all negative for additional foci of malignancy confirming primary bladder adenocarcinoma. Hence, it was concluded that the gastric lesions represented metastasis to the stomach from primary SRCA of urinary bladder. Due to underlying comorbidities and poor baseline functionality, patient elected to proceed with hospice care. DISCUSSION: Primary urinary bladder SRCA is a rare type of malignancy that typically presents at an advanced stage. Standard therapy of local urinary bladder SRCA is radical cystectomy. Despite advances in identifying immunohistochemical markers which aid in determination of primary bladder vs. colorectal origin, rates of metastases after surgical intervention are uncertain which limits the discussion regarding the role of adjuvant therapy. Our case highlights the need for further investigation to evaluate the risk-benefit ratio of adjuvant chemotherapy after radical cystectomy in primary bladder SRCA in minimizing development of future distant metastases.

A CASE THAT TS-1+CDDP THERAPY WAS EFFECTIVE FOR BLADDER PRIMARY SIGNET RING CELL ADENOCARCINOMA

Since we experienced a case that S-1+Cisplatin (CDDP) therapy was effective for bladder primary signet ring cell adenocarcinoma of pT4N0M0, we report here the time course of its pathophysiology and the treatment of that patient, and discuss the justification of the chemotherapy for the T4 case with the comparison of the previous cases. The present case is a 66 years old man. Because he was aware of the urinary frequency and the sense of incongruity at abdominal region for approximately three months, he visited our hospital for the consultation of his symptoms in July, 2015. The anterior wall showed a torose lesion by cystoscopy from the left sidewall, and we found the histopathology signet ring cell adenocarcinoma after transurethral resection of the bladder tumor. As a result of thorough investigation of thoracic abdominal CT, whole body PET-CT and endoscopy of the upper gastrointestinal tract, we diagnosed it as bladder primary signet ring cell adenocarcinoma with cT3N0M0. Consequently, we tried total cystectomy in August, 2015, however both bladder and pelvic wall were adhered strongly each other; we gave up the total cystectomy and changed the strategy to the urinary diversion by bilateral ureterocutaneous fistula. A biopsy of the adhesion site of the right pelvic wall showed the invasion of the signet ring cell adenocarcinoma, which enabled us to diagnose as pT4bN0M0. Therefore, we performed the chemotherapy with the S-1+CDDP for 12 courses, 16 months from August, 2015 after the surgery. After the initiation of the chemotherapy, the tumor marker was tended to decrease for eight months, but turned to increase thereafter. Because there was no evidence for neither distant nor lymph node metastases of tumor revealed by CT and the MRI, we diagnosed it as Stable Disease at least by CT and MRI examinations for 16 months. The major side-effects were not found during the strengthened chemotherapy without the recurrence until January, 2017. Since this case is very rare, we report the time courses and treatment strategy of a patient with bladder primary signet ring cell adenocarcinoma.

A Case with Primary Signet Ring Cell Adenocarcinoma of the Prostate and Review of the Literature

A Case with Primary Signet Ring Cell Adenocarcinoma of the Prostate and Review of the Literature

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 Rare Case of Signet-ring Cell Carcinoma of Cecum with Endocrine Differentiation and Isolated Neuroendocrine Tumor 1 of Duodenum Presenting as Peritoneal Carcinomatosis

Introduction: Colorectal cancer is the third leading cause of cancer related death in United States. Signet ring cell adenocarcinoma (SRCC) of colon is a very rare entity, accounting for less than 1% of cases. To date only 27 cases have been reported. We present an interesting case of signet cell carcinoma of cecum presenting as peritoneal carcinomatosis. Case: A 44-year-old male presented with diffuse left lower quadrant abdominal pain 2-3 years without weight loss, fever, chills and or night sweats. Initial CT abdomen and pelvis showed mural thickening of distal ileum and cecum, moderate ascites with infiltration of omentum, consistent with peritoneal carcinomatosis. Ascitic fluid cytology was negative for malignancy but showed reactive mesothelial cells. Colonoscopy revealed an exophytic mass in caecum (Figure 1) with biopsy positive for invasive adenocarcinoma, poorly differentiated with signet ring cells and endocrine differentiation (Figure 2). Upper endoscopy showed duodenal bulb polyp with pathology consistent with neuroendocrine type 1 tumor which appeared as an isolated finding. Patient had a repeat CT abdomen and pelvis for staging which showed further extensive lymphadenopathy involving the mediastinum, hilum and retroperitoneum. PET scan and peritoneal biopsy were requested by surgery and oncology after hospital discharge for further staging and management.Figure 1Figure 2Discussion: SRCC usually presents at an advanced stage with node positive disease and peritoneal dissemination, which results in poor prognosis. Pathologically the signet ring malignant cells are seen floating in abundant extracellular mucin pools. A unique feature of SRCC is its fibrotic appearance and preference for peritoneal metastasis over the liver. It is associated with a higher grade tumor burden associated with young age and distinct molecular patterns like microsatellite instability(MSI). In view of poor prognosis an adjuvant 5-FU-based therapy should be initiated to prevent recurrence of SRCC. The overall 5-year survival is 0-12%. There are no reports of SRCC with NET 1 in literature to date and this was likely an isolated rare finding. Conclusion: Primary SRCC is considered a distinct entity based on the clinical and pathological features. In this case report, we have shown a rare case of primary SRCC of the cecum with endocrine differentiation and neuroendocrine type 1 tumor of duodenum.

Primary signet-ring cell adenocarcinoma of the head and neck: a case study and brief review

We present a patient who had an aggressive primary signet-ring cell adenocarcinoma in the oral cavity that spread rapidly and led to his death. Most reports describe an indolent clinical course, but further reports are needed to better evaluate the particular clinical characteristics and course of this uncommon and biologically variable condition.

Primary signet-ring cell adenocarcinoma of the head and neck

Primary signet-ring cell adenocarcinoma of the head and neck

Primary signet-ring cell carcinoma of the lung treated with crizotinib: A case report.

Primary signet-ring cell adenocarcinoma (SRCA) of the lung is an extremely rare subtype of lung adenocarcinoma with a poor prognosis. The presence of an SRC component is considered to be a prominent clinicopathological characteristic of EML4-ALK-positive non-small cell lung cancer (NSCLC). Crizotinib, an anaplastic lymphoma kinase inhibitor, has been approved for the treatment of EML4-ALK NSCLC by previous studies, but its effect on SRCA, an extremely rare subtype of lung adenocarcinoma, has yet to be elucidated. Therefore, the present study aimed to evaluate the clinical response of SRCA to crizotinib, and examine the potential use of crizotinib as a treatment for the carcinoma. A 43-year-old male was admitted to the Qingdao Municipal Hospital (Qingdao, China) with dyspnea. Chest computed tomography (CT) revealed a mass in the middle lobe of the right lung. Transbronchial lung biopsies revealed the presence of SRCA (70%) mixed with poorly-differentiated adenocarcinoma (30%). Immunohistochemically, the SRCA cells were positive for cytokeratin (CK)7 and thyroid transcription factor-1, and negative for CK20. An inversion of the EML4-ALK gene was detected by fluorescence in situ hybridization and crizotinib was injected by nasogastric tube. The patient was highly responsive to crizotinib. The symptoms of dyspnea were relieved and the volumes of pericardial and pleural effusion were gradually reduced. A CT scan revealed lung tumor regression. The overall response was a partial response. Therefore, crizotinib exists an attractive therapeutic option for patients with SRCA. However, in the present study, acquired drug resistance to crizotinib developed after only one month of treatment. It would consequently be valuable to investigate the mechanisms underlying acquired crizotinib resistance in future studies.

Clinicopathological features and survival outcomes of primary signet ring cell and mucinous adenocarcinoma of colon: retrospective analysis of VACCR database.

Signet ring cell carcinoma (SRCC) accounts for less than 1% of all colon cancers. We examined the clinicopathological features and prognosis of signet ring cell and mucinous adenocarcinoma (MCC) of colon. A total of 206 patients diagnosed with SRCC from 1995 to 2009 were identified from the VA Central Cancer Registry (VACCR) database. Age, race, histology, grade, lymph node status, stage and type of treatment received data were collected. Out of 206 patients, 173 (84%) were white, 31 (15%) were black, and 2 patients were listed as unknown. Median age of diagnosis was 67 years as compared to 70 years for both mucinous cell (MCC) and non-mucinous adenocarcinoma (NMCC) of colon. Pathological T-stages were as follows: T1 =3%, T2 =5%, T3 =34%, T4 =26%, and unknown 32%. Of the total, 22.3% were located in cecum, 7.7% in appendix, 21.8% in ascending colon, 7.7% in hepatic flexure of colon, 11% in transverse colon, 2.9% in splenic flexure 4.4% in descending colon, and 15.5% in sigmoid colon. 46.5% were lymph node positive, 21% were lymph node negative, and 32.5% were unknown. SRCC were in general poorly differentiated tumors (57%), small proportion of patients included were well-differentiated tumors with focal signet ring cell pathology (10%) and in 33% grade was unknown. Among stage 3 patients, 34% patients received only surgery while 64% received surgery with adjuvant chemotherapy and 2% received chemotherapy alone. The stage specific 5-year survivals for SRCC, MCC and NMCC were--Stage I: 100%, 61%, and 41% respectively (P<0.0001); Stage II: 42%, 58% and 32% respectively (P<0.0001); Stage III: 19%, 41% and 47% respectively (P=0.0002); Stage IV: 1.5%, 7% and 31% respectively (P<0.0001). Median survival of SRCC compared to NMCC was 18.6 vs. 46 months (P<0.0001) and mucinous cell adenocarcinoma versus NMCC was 47.8 and 46 months (P=0.63) respectively. SRCC of colon has poor survival rates compared to other histological subtypes. SRCC presents at an earlier age, has higher tumor grade and advanced stage at diagnosis when compared to mucinous and NMCC of colon. Due to rarity of this disease further prospective multi-institute studies are required for in-depth understanding of this disease.

Multidisciplinary management of primary signet-ring cell adenocarcinoma of the urinary bladder and the specific role of adjuvant radiotherapy

Primary signet-ring cell (SRC) of the bladder is an aggressive, extremely uncommon subtype of bladder adenocarcinoma, comprising of <1 % of all primary bladder neoplasms [1]. A report based on analysis of the Surveillance, Epidemiology, and End Results (SEER) Program database found only 103 patients with SRC of the bladder diagnosed between 2001 and 2004, compared to 14,648 patients diagnosed with urothelial carcinoma [2]. The prognosis for patients with SRC is poor, attributed to presentation at advanced stages following asymptomatic progression [3], reported inefficacy of multimodality therapy [4], and possibly a more aggressive underlying biological phenotype [2]. Diagnosis of bladder SRC can be challenging, both as it requires an extensive work-up to rule out more common metastatic adenocarcinomas [5], and comprehensive studies detailing the immunohistochemical profile of these tumors are understandably lacking [6]. Current treatment for this malignancy is often based on experiences gained from single institutional case series. Based on the aggressive natural history of bladder SRCs, treatment often features aggressive surgery in conjunction with chemoand radiotherapy, although standardized recommendations for the use of adjuvant therapy are lacking. In this article, we present a case of locoregionally advanced SRC of the bladder, successfully managed with multimodality therapy. Literature regarding the diagnosis/work-up and management of bladder SRC is reviewed, with a focus on the specific role of adjuvant treatment.

Signet Ring Cell of the Non-Neoplastic Endometrium: A Case Report and Literature Review

The observation of signet ring cells in endometrial biopsy results raises the possibility of the existence of a malignant primary or metastatic adenocarcinoma. We describe the case of a middle aged woman whose endometrial biopsy and curettage specimens contained signet ring cells indicating stromal decidual changes. Positive cytokeratin and CD10 immunohistochemistry staining results were revealed in the signet ring cells, features favoring a diagnosis of endometrial stromal cells showing decidual changes rather than a primary or metastatic endometrial neoplasm. Upon immunohistochemistry evaluation, they presented as vacuolated, decidualized endometrial stromal cells. Awareness of this morphologic change in the endometrial stroma can prevent overdiagnosis and overtreatment as primary or metastatic signet ring cell adenocarcinoma. Recognized by this morphologic feature, signet ring cells, or their close mimics, should not always be considered as a high grade or poorly differentiated adenocarcinoma. doi: http://dx.doi.org/10.4021/jcgo42e

Primary pure signet-ring cell adenocarcinoma of the urinary bladder: a report of three cases with an immunohistochemical study

Although several case series of primary signet-ring cell adenocarcinoma (SRCA) of the urinary bladder (UB) have been published, primary pure SRCA of the UB composed exclusively of signet-ring cells is rare. In addition, there have been no comprehensive studies of immunohistochemical studies of primary SRCA of the UB. This report shows immunohistochemical profiles of 3 cases of primary SRCA of UB. The age of patients was 37, 43, and 84years. The male-to-female ratio was 2:1. One specimen was cystectomy, and other two specimens were transurethral bladder tumorectomy. Two patients died of bladder tumor 12 and 16months after the diagnosis, and one patient is now alive 6months after the diagnosis. Histologically, all the three tumors were composed exclusively of SRCA cells. Histochemically, all tumors contained neutral and acidic mucins. Immunohistochemically, consistent findings were positive cytokeratin (CK) AE1/3, CK CAM5.2, CK8, CK18, CK19, CK20, p53, Ki-67 (30, 50, 70%), MUC1, and MUC2. CK34BE12, CK5/6, CK14, EMA, p63, vimentin, and MUC6 were consistently negative. CK7 was positive in 1/3, CA19-9 1/3, and MUC5AC 2/3 cases. These data may provide basic knowledge of SRCA of the UB. In conclusion, the author presents three cases of primary pure SRCA with an emphasis of immunohistochemical findings.