The updated WHO classification of digestive system tumours-gastric adenocarcinoma and dysplasia.

The fifth edition of the World Health Organization (WHO) classification of digestive system tumours was published in 2019. The classification of invasive carcinoma is basically the same as in the fourth edition, but the description of each histological type has been updated, and some rare subtypes such as micropapillary carcinoma, gastric adenocarcinoma of the fundic gland type and undifferentiated carcinoma have been added and explained. Although this classification did not provide specific numerical criteria for the diagnosis of signet-ring cell carcinoma in poorly cohesive carcinoma, an additional study defined signet-ring cell carcinoma as having more than 90% signet-ring cells. The molecular classification of gastric cancer (Epstein-Barr virus-positive type, microsatellite instability type, genomically stable type, chromosomally unstable type) was additionally introduced. Many pages in the present classification have been devoted to precancerous lesions, and this article focuses on foveolar-type adenoma/dysplasia.

Primary signet-ring cell carcinoma of the urinary bladder.

Primary signet-ring cell carcinoma of the urinary bladder is a rare tumor. The overall incidence is approximately 0.12-0.6% of all urinary bladder malignancies. The majority of the patients present in an advanced stage with a uniformly grim prognosis. As signet-ring cell carcinomas are more common in the gastrointestinal tract, a possibility of metastasis needs to be considered. Here we report, a 42-year-old patient who presented with hematuria and was diagnosed with a urinary bladder tumor. The patient was managed with partial cystectomy and pelvic lymph node dissection. The histopathological examination confirmed primary signet-ring cell carcinoma of the urinary bladder.

One case report of primary mucinous adenocarcinoma combined with signet-ring cell carcinoma of female urethra / 中华泌尿外科杂志

Primary female urethral adenocarcinoma is rare. This paper reports a case of primary urethral mucinous adenocarcinoma complicated with signet ring cell carcinoma. The patient underwent urethral tumor resection in another hospital. Postoperative examination indicated that the tumor remained, and the tumor was completely removed after urethral tumor resection. After 11 months of follow-up, there was no tumor residue or recurrence.

Primary signet-ring cell carcinoma of the urinary bladder

Primary signet-ring cell carcinoma of the urinary bladder is a rare tumor. The overall incidence is approximately 0.12-0.6% of all urinary bladder malignancies. The majority of the patients present in an advanced stage with a uniformly grim prognosis. As signet-ring cell carcinomas are more common in the gastrointestinal tract, a possibility of metastasis needs to be considered. Here we report, a 42-year-old patient who presented with hematuria and was diagnosed with a urinary bladder tumor. The patient was managed with partial cystectomy and pelvic lymph node dissection. The histopathological examination confirmed primary signet-ring cell carcinoma of the urinary bladder.

Ileal Signet Ring Cell Carcinoma Masked by Crohn Disease.

Background: Signet ring cell carcinoma (SRCC) is a rare, highly malignant adenocarcinoma that generally involves the stomach; ileal involvement is uncommon. Crohn disease (CD) is associated with long-standing inflammation that may predispose to small intestine adenocarcinoma. Case Report: A 67-year-old male with ileal CD since age 23 years, maintained in remission by mesalamine, presented with mild intermittent attacks of abdominal cramping, an increase in bowel movements from 3 to 5 daily, and bloating for 3 months. Computed tomography enterography with contrast enhancement demonstrated 2 segments of ileal wall thickening. Colonoscopy performed 7 years prior was unremarkable. The patient received oral prednisone with mild symptomatic improvement; he declined biologics. Ileocolonoscopy 1 month later revealed a nontraversable terminal ileal stricture 15 cm from the ileocecal valve. Biopsy demonstrated signet ring cells infiltrating the lamina propria. The patient underwent laparoscopic ileocecectomy and ileocolic anastomosis. Histopathology of a 2.5-cm ileal mass showed poorly differentiated adenocarcinoma with mucin production and signet ring cell features. One metastatic mesenteric lymph node was identified. Adjuvant chemotherapy was initiated. Conclusion: This case of metastatic ileal SRCC occurred in the setting of long-standing, clinically controlled CD. Although the absolute risk of small-bowel adenocarcinoma in CD is low, active surveillance for small-bowel adenocarcinoma in patients with longstanding CD may be prudent, given the overlapping symptomology of SRCC and CD, the aggressiveness of SRCC, and the association of SRCC with subclinical inflammation.

Signet ring cells in carcinomatous lymphangitis due to gastric adenocarcinoma

Abstract Cutaneous metastases are rare. They usually present as nodules or tumors. Diagnosis is based on histopathological examination and prognosis is unfavorable. This report describes the case of a female patient, 72 years old, with surgically treated gastric antrum adenocarcinoma. Pathology showed poorly differentiated adenocarcinoma with signet ring cells. It evolved with bone involvement, lymph node enlargement in the inguinal region, and skin infiltration in the lower limbs, abdomen, and root of the upper limbs. Skin biopsy demonstrated signet ring carcinoma embolizing the dermal and hypodermic vessels and invasion of adipose tissue, confirming carcinomatous lymphangitis. Carcinomatous lymphangitis is the cutaneous and subcutaneous lymphatic invasion by tumor cells. Cutaneous metastasis is relatively uncommon and presents mainly as cutaneous or subcutaneous nodules, and more rarely as inflammatory lesions. The present case reports carcinomatous lymphangitis associated with gastric cancer.

Signet ring cells in carcinomatous lymphangitis due to gastric adenocarcinoma.

Cutaneous metastases are rare. They usually present as nodules or tumors. Diagnosis is based on histopathological examination and prognosis is unfavorable. This report describes the case of a female patient, 72 years old, with surgically treated gastric antrum adenocarcinoma. Pathology showed poorly differentiated adenocarcinoma with signet ring cells. It evolved with bone involvement, lymph node enlargement in the inguinal region, and skin infiltration in the lower limbs, abdomen, and root of the upper limbs. Skin biopsy demonstrated signet ring carcinoma embolizing the dermal and hypodermic vessels and invasion of adipose tissue, confirming carcinomatous lymphangitis. Carcinomatous lymphangitis is the cutaneous and subcutaneous lymphatic invasion by tumor cells. Cutaneous metastasis is relatively uncommon and presents mainly as cutaneous or subcutaneous nodules, and more rarely as inflammatory lesions. The present case reports carcinomatous lymphangitis associated with gastric cancer.

Breast metastasis of gastric signet ring cell carcinoma mimicking breast lymphangitic carcinomatosis / Metástase mamária de carcinoma gástrico em anel de sinete simulando linfangite carcinomatosa da mama

Introduction: The incidence of breast metastasis from gastric adenocarcinoma is extremely low. Since 1908, 44 cases have been reported in the literature, of which 30 are signet ring cell type. Case report: A 49-year-old patient being investigated for digestive bleeding was found to have left axillary lymphadenopathy, associated with breast asymmetry, associated with breast asymmetry, edema and thickening of the skin. Breast ultrasonography showed a heterogeneous lesion in the left breast. Core biopsy histology was compatible with Lauren diffuse gastric adenocarcinoma with signet ring cells. There was positive immunohistochemical staining for CK7, CK20 and CDX2 and negative for RE, RP and ERB2. Our findings were compatible with gastric adenocarcinoma (lymphatic embolism), favoring the possibility of a secondary neoplasm. At the time of diagnosis, the patient already had radiological signs of multiple metastases. Discussion: Breast metastases of gastric carcinoma differ from primary breast cancer in histopathological features. The clinical manifestations of gastric cancer metastasis vary, but it is known that there is a greater tendency for inflammatory disorders compared to primary tumors. In the metastatic process, breast involvement may be the first event or occur in a context of multiple metastases. Most patients have a one-year survival after diagnosis. There is no gain in survival with breast surgery, but it can alleviate the symptoms in some cases. Conclusion: Gastric cancer with breast metastasis is a rare condition associated with poor prognosis. The diagnosis is based on clinical history, histological findings and immunohistochemical markers, differing from primary tumors of the breast, to provide patients with adequate treatment.

Introdução: A incidência de metástase mamária de adenocarcinoma gástrico é extremamente baixa. De 1908 até o momento, 44 casos foram relatados na literatura, dos quais 30 são do tipo em anel de sinete. Relato do caso: Paciente de 49 anos em propedêutica de sangramento digestivo alto. Apresentava linfadenomegalia axilar esquerda, associada a assimetria mamária, edema e espessamento de pele. Ultrassonografia mamária evidenciou lesão heterogênea em mama esquerda. Histologia de core biopsy da área compatível com adenocarcinoma gástrico tipo difuso de Lauren, com células em anel de sinete. Imuno-histoquímica positiva para pancitoqueratinas CK7, CK20, CDX2 e negativa para RE, RP e ERB2. Achados compatíveis com adenocarcinoma gástrico (embolia linfática), favorecendo a possibilidade de neoplasia secundária. Ao momento do diagnóstico, a paciente já apresentava sinais radiológicos de múltiplas metástases. Discussão: As metástases mamárias do carcinoma gástrico diferem do câncer de mama primário nas características histopatológicas. As manifestações clínicas das metástases de câncer gástrico são variadas, mas é sabido que há tendência maior de alterações inflamatórias que nos tumores primários. No processo metastático, o envolvimento mamário pode ser o primeiro evento ou ocorrer em um contexto polimetastático. A maioria dos pacientes tem sobrevida inferior a um ano. Não há ganho de sobrevida com a cirurgia de mama, mas ela pode aliviar os sintomas em alguns casos. Conclusão: O câncer gástrico com metástase na mama é uma condição rara associada a mau prognóstico. O diagnóstico é baseado em história clínica, achados histológicos e marcadores imuno-histoquímicos, o que diferencia a metástase de um tumor primário da mama, a fim de oferecer aos pacientes o tratamento adequado.

Human Epidermal Growth Factor Receptor 2-positive Mucinous Carcinoma with Signet Ring Cell Differentiation, Which Showed Complete Response after Neoadjuvant Chemotherapy.

Mucinous carcinoma (MC) is a rare subtype of breast cancer, which is composed of tumor cells floating in the abundant extracellular mucin. This form of cancer is usually estrogen receptor/progesterone receptor positive and human epidermal growth factor receptor 2 (HER2) negative. Here, we present a case of HER2-positive MC with an unusual signet ring cell differentiation. It is very rare that a breast tumor consists entirely of signet ring cells. The tumor showed pathologic complete response (pCR) after neoadjuvant chemotherapy with trastuzumab and pertuzumab. pCR of HER2-positive MC has rarely been described in literature. It is important to consider the biological heterogeneity of MCs for effective management.

Human Epidermal Growth Factor Receptor 2-positive Mucinous Carcinoma with Signet Ring Cell Differentiation, Which Showed Complete Response after Neoadjuvant Chemotherapy / 한국유방암학회지

Mucinous carcinoma (MC) is a rare subtype of breast cancer, which is composed of tumor cells floating in the abundant extracellular mucin. This form of cancer is usually estrogen receptor/progesterone receptor positive and human epidermal growth factor receptor 2 (HER2) negative. Here, we present a case of HER2-positive MC with an unusual signet ring cell differentiation. It is very rare that a breast tumor consists entirely of signet ring cells. The tumor showed pathologic complete response (pCR) after neoadjuvant chemotherapy with trastuzumab and pertuzumab. pCR of HER2-positive MC has rarely been described in literature. It is important to consider the biological heterogeneity of MCs for effective management.

18F-FDG PET/CT Imaging of Pulmonary Mucinous Cystadenocarcinoma with Signet Ring Cells.

A 63-year-old male with a recently diagnosed right lung lesion was referred for staging. F-FDG PET/CT scan revealed a hypodense, cystic-like mass in the right upper lung lobe, which demonstrated low, diffuse 18F-FDG uptake, likely due to the presence of mucus, as well as intensely hypermetabolic right hilar and right paratracheal lymph nodes. Transbronchial biopsy revealed a primary pulmonary mucinous cystadenocarcinoma with the presence of signet ring cell carcinoma, a co-existence of two rare variants of lung adenocarcinoma. This case report demonstrates the metabolic phenotype along with the radiographic characteristics of this rare tumor and its metastases.

Carcinoma vesicular de células en anillo de sello: diseminación poco frecuente: reporte de un caso con tomografía computada por emisión de positrones con fluor 18-deoxiglucosa / Bone metastases of a gallbladder carcinoma detected by positron emission tomography/computed tomography: case report

Signet ring gallbladder carcinoma is a rare aggressive variant of mucinous adenocarcinoma with poor prognosis. Positron emission tomography/computed tomography (PET/CT) with Fluor18 deoxyglucose (F18-FDG) is a useful tool in the staging of gallbladder cancer. We report a 68 years old man with a surgically resected acute cholecystitis, whose biopsy was positive for signet ring cell gallbladder carcinoma. During surgery, locoregional lymph nodes, liver or peritoneal involvement were not detected. A PET/CT was performed for staging, finding multiple hypermetabolic lytic bone lesions. Percutaneous biopsy of a pelvis bone lesion, confirmed a metastasis of the tumor. In this case, the staging with PET/CT allowed the diagnosis of unsuspected bone metastases and was a useful tool for deciding the best site of biopsy for histologic confirmation.

Signet-ring cell carcinoma of the appendix: a case report with an emphasis on sonographic findings.

In this report, we present a rare case of primary signet-ring cell carcinoma of the appendix in a 51-year-old woman with right lower quadrant pain. Since non-specific concentric appendiceal wall thickening was found in a radiologic evaluation, it was misdiagnosed as non-tumorous appendicitis. An in-depth examination of the correlation between sonographic and histopathologic findings demonstrated that a single markedly thickened hypoechoic layer was well correlated with the diffuse infiltration of tumor cells in both the submucosal and muscle layers. If this sonographic finding is observed in certain clinical settings, such as potential ovarian and peritoneal metastasis, submucosal infiltrative tumors, including signet-ring cell carcinoma, should be considered in the differential diagnosis.

The Rare and Challenging Presentation of Gastric Cancer during Pregnancy: A Report of Three Cases.

Pregnancy-associated gastric cancer is extremely rare. In many cases, it is diagnosed at an advanced stage because the symptoms during pregnancy are generally overlooked. We report three cases of gastric cancer during pregnancy with various outcomes. The first case included a patient with stage IV gastric cancer who received palliative chemotherapy. This patient had a preterm birth and died 7 months after diagnosis. The second case received neoadjuvant chemotherapy during pregnancy and a total gastrectomy was performed after delivery. She then received adjuvant chemoradiotherapy. This patient developed pulmonary metastasis and died of recurrence 41 months after surgery. In the third case, a distal subtotal gastrectomy was performed at week 14 of pregnancy, with no complications. The patient received adjuvant chemoradiotherapy. She is currently without recurrence 14 months after surgery. In patients with pregnancy-associated gastric cancer, treatment decisions are predominantly influenced by clinical stage and gestational age at diagnosis.

Rare Case of Anal Canal Signet Ring Cell Carcinoma Associated with Perianal and Vulvar Pagetoid Spread.

A 61-year-old woman was referred to surgery for incidentally found colonic polyps during a health examination. Physical examination revealed widespread eczematous skin lesion without pruritus in the perianal and vulvar area. Abdominopelvic computed tomography showed an approximately 4-cm-sized, soft tissue lesion in the right perianal area. Inguinal lymph node dissection and Mils' operation extended to perianal and perivulvar skin was performed. Histologically, the anal canal lesion was composed of mucin-containing signet ring cells, which were similar to those found in Pagetoid skin lesions. It was diagnosed as an anal canal signet ring cell carcinoma (SRCC) with perianal and vulvar Pagetoid spread and bilateral inguinal lymph node metastasis. Anal canal SRCC is rare, and the current case is the third reported case in the English literature. Seven additional cases were retrieved from the world literature. Here, we describe this rare case of anal canal SRCC with perianal Pagetoid spread and provide a literature review.

Signet-ring cell carcinoma of the appendix: a case report with an emphasis on sonographic findings

In this report, we present a rare case of primary signet-ring cell carcinoma of the appendix in a 51-year-old woman with right lower quadrant pain. Since non-specific concentric appendiceal wall thickening was found in a radiologic evaluation, it was misdiagnosed as non-tumorous appendicitis. An in-depth examination of the correlation between sonographic and histopathologic findings demonstrated that a single markedly thickened hypoechoic layer was well correlated with the diffuse infiltration of tumor cells in both the submucosal and muscle layers. If this sonographic finding is observed in certain clinical settings, such as potential ovarian and peritoneal metastasis, submucosal infiltrative tumors, including signet-ring cell carcinoma, should be considered in the differential diagnosis.

The Rare and Challenging Presentation of Gastric Cancer during Pregnancy: A Report of Three Cases

Pregnancy-associated gastric cancer is extremely rare. In many cases, it is diagnosed at an advanced stage because the symptoms during pregnancy are generally overlooked. We report three cases of gastric cancer during pregnancy with various outcomes. The first case included a patient with stage IV gastric cancer who received palliative chemotherapy. This patient had a preterm birth and died 7 months after diagnosis. The second case received neoadjuvant chemotherapy during pregnancy and a total gastrectomy was performed after delivery. She then received adjuvant chemoradiotherapy. This patient developed pulmonary metastasis and died of recurrence 41 months after surgery. In the third case, a distal subtotal gastrectomy was performed at week 14 of pregnancy, with no complications. The patient received adjuvant chemoradiotherapy. She is currently without recurrence 14 months after surgery. In patients with pregnancy-associated gastric cancer, treatment decisions are predominantly influenced by clinical stage and gestational age at diagnosis.

Rare Case of Anal Canal Signet Ring Cell Carcinoma Associated with Perianal and Vulvar Pagetoid Spread

A 61-year-old woman was referred to surgery for incidentally found colonic polyps during a health examination. Physical examination revealed widespread eczematous skin lesion without pruritus in the perianal and vulvar area. Abdominopelvic computed tomography showed an approximately 4-cm-sized, soft tissue lesion in the right perianal area. Inguinal lymph node dissection and Mils' operation extended to perianal and perivulvar skin was performed. Histologically, the anal canal lesion was composed of mucin-containing signet ring cells, which were similar to those found in Pagetoid skin lesions. It was diagnosed as an anal canal signet ring cell carcinoma (SRCC) with perianal and vulvar Pagetoid spread and bilateral inguinal lymph node metastasis. Anal canal SRCC is rare, and the current case is the third reported case in the English literature. Seven additional cases were retrieved from the world literature. Here, we describe this rare case of anal canal SRCC with perianal Pagetoid spread and provide a literature review.

Clinical Features and Prognosis of Resectable Primary Colorectal Signet-Ring Cell Carcinoma.

BACKGROUND/AIMS: We attempted to investigate the prognosis of signet-ring cell carcinoma (SRC) patients who underwent curative surgery by comparing them with age-, sex-, and stage-matched non-mucinous adenocarcinoma (NMAC) patients. METHODS: Between January 2003 and December 2011, 19 patients with primary SRC of the colorectum underwent curative surgery. Four SRC patients under the age of 40 were excluded, and the clinicopathological data of 15 patients (7 men; median age, 56 years) were reviewed and compared with the data of 75 NMAC patients matched by age, sex, and pathologic stage. RESULTS: The median follow-up duration was 30.1 months for the SRC group and 43.7 months for the NMAC group (P=0.141). Involvement of the left side of the colon (73.3% vs. 26.7%, P=0.003) and infiltrative lesions such as Borrmann types 3 and 4 (85.7% vs. 24.0%, P=0.001) were more common in the SRC group than in the NMAC group. The five-year overall survival rate was significantly lower for patients with SRC than for those with NMAC (46.0% vs. 88.7%, hazard ratio, 6.99; 95% confidence interval, 2.33-20.95, P=0.001). CONCLUSIONS: Patients with even resectable primary colorectal SRC had a poorer prognosis than age-, sex-, and stage-matched colorectal NMAC patients.

Growth Patterns of Signet Ring Cell Carcinoma of the Stomach for Endoscopic Resection.

BACKGROUND/AIMS: It is difficult to precisely detect the lateral margin during endoscopic submucosal dissection (ESD) for signet ring cell carcinoma (SRC) because SRC often expands to lateral direction through the lamina propria. Thus, the aim of this study was to classify the intramucosal spreading patterns of SRC and to analyze the patients' clinicopathological findings according to the spreading patterns. METHODS: The intramucosal spreading patterns of SRC were classified as expansive or infiltrative types. A total of 100 surgical and 42 ESD specimens were reviewed. RESULTS: In the surgical specimens, the proportions of expansive and infiltrative types were 44% and 56%, respectively. The infiltrative type was more commonly associated with old age, atrophy, and intestinal metaplasia in surrounding mucosa and the absence of Helicobacter pylori compared with the expansive type. In ESD specimens, the proportions of expansive and infiltrative types were each 50%. When lateral margin-positive lesions were compared with -negative lesions, larger size, residual lesion, and the lack of a neutrophil infiltration were more significantly associated with lateral margin-positive lesions. All cases with residual tumors in lateral margin-positive lesions were classified as the infiltrative type. CONCLUSIONS: SRC surrounded with atrophy and/or intestinal metaplasia often spreads subepithelially in the margin. This finding may suggest that a larger safety margin is necessary in this type during ESD.