Adenocarcinoma primario de pulmón con células en anillo de sello y reordenamiento de ALK. Reporte de dos casos / Primary adenocarcinoma of the lung with signet-ring cells and ALK rearrangement. Two case reports.

El adenocarcinoma primario de pulmón con células en anillo de sello representa una variante rara y muy agresiva de cáncer de pulmón. El carcinoma de células en anillo de sello es un tipo particular de adenocarcinoma secretor de mucina, que se deposita intracitoplasmáticamente y desplaza el núcleo hacia la periferia de la célula. El origen pulmonar de este subtipo tumoral es raro y el hallazgo de reordenamiento de ALK es una asociación común. Presentamos dos casos de adenocarcinomas mucinosos primarios de pulmón con células en anillo de sello y reordenamiento de ALK. Todos los pacientes con cáncer de pulmón deben contar con estudios histopatológicos, inmunohistoquímicos y de biología molecular con los que se puedan obtener las principales características del tumor, que permitan ofrecer la mejor opción terapéutica para el paciente.

Primary adenocarcinoma of the lung with signet-ring cells represents a rare and highly aggressive variant of lung cancer. Signet­ring cell carcinoma is a particular type of mucin­secreting adenocarcinoma, which is deposited intracytoplasmatically and displaces the nucleus towards the periphery of the cell. The pulmonary origin of this tumor subtype is rare, and the finding of ALK rearrangement is a common association. We present two cases of primary mucinous adenocarcinomas of the lung with signet-ring cells and ALK rearrangement. All patients with lung cancer must have histopathological, immunohistochemical, and molecular biology studies with which the main features of the tumor can be obtained, which allow the clinician to offer the best possible treatment for the patient.

Immunohistochemical analysis of PD-L1 and tumor-infiltrating immune cells expression in the tumor microenvironment of primary signet ring cell carcinoma of the prostate / 亚洲男科学杂志(英文版)

Primary signet ring cell carcinoma (SRCC) of the prostate is a rare neoplasm. However, its potential tumorigenic mechanism, clinicopathological features, and prognostic outcome have not been systematically described. To determine the pathogenic mechanism, we detected distributions of programmed cell death-ligand 1 (PD-L1), programmed death 1 (PD-1), and cellular components in the tumor microenvironment, including tumor-infiltrating lymphocytes (CD4 and CD8), tumor-associated macrophages (TAMs; CD163 and CD68), and tumor-associated fibroblasts (vimentin and alpha-smooth muscle actin [α-SMA]), in tumor tissues from four patients with primary prostatic SRCC compared with corresponding adjacent tissues and tumor tissues from 30 patients with prostate adenocarcinoma (PCa) by immunohistochemical staining. We found higher expression of PD-L1, CD163, and CD68 in primary SRCC specimens than that in both corresponding adjacent nontumor specimens and PCa specimens with different Gleason scores, indicating that TAMs may participate in the malignant biological behavior of primary SRCC of the prostate. For further analysis, we searched electronic journal databases and Surveillance, Epidemiology, and End Results (SEER) to identify 200 eligible patients including our four cases. According to Kaplan-Meier survival curve analysis, patients <68 years old, with radical prostatectomy (RP), Gleason score of 7-8, and lower clinical stage had longer overall survival (OS). Moreover, Cox multivariate analysis indicated that race (hazard ratio [HR] = 1.422), surgical approach (HR = 1.654), and Gleason score (HR = 2.162) were independent prognostic factors for OS. Therefore, primary SRCC of the prostate represents a distinct and aggressive subtype of prostate cancer associated with a higher distribution of PD-L1 and TAMs, which warrants further clinical investigation.

Linfoma con morfología en anillo de sello: patología con distintivos diagnósticos diferenciales / Lymphoma with signet ring morphology: pathology with distinctive differential diagnoses

Introducción: el linfoma con células en anillo de sello es una entidad poco frecuente y simuladora de neoplasias epiteliales, sarcomas y condiciones reactivas de histiocitos. Representa una variante morfológica de distintos linfomas no Hodgkin, por lo que su diagnóstico puede representar un desafío y debe ser considerado al realizar estudios complementarios. Objetivo: mostrar un caso con una morfología muy poco frecuente y recalcar la importancia de conocer esta entidad para no cometer errores en su diagnóstico. Caso clínico: se documenta el caso de un hombre de 67 años que desarrolló crecimientos ganglionares en axila derecha, cuello, ingle derecha, y región submandibular, con aparente afección en pulmones y bazo, que fue diagnosticado como linfoma B difuso de células grandes con morfología en anillo de sello, originado en el centro germinal. Se realizó estudio de microscopía electrónica de transmisión para una mejor caracterización de la morfología. Desafortunadamente el paciente no regresó a consulta de seguimiento, por lo que no inició tratamiento, falleció a los 6 meses posteriores al diagnóstico. Conclusiones: el linfoma con fenotipo en anillo de sello es poco frecuente, y puede presentarse en cualquier tipo de linfomas no Hodgkin; sin embargo, esta morfología es más comúnmente asociada a carcinomas y, en menor frecuencia, a sarcomas, melanomas o histiocitos reactivos, por lo que el considerar esta entidad junto con el uso adecuado de estudios complementarios es de gran importancia para su adecuado diagnóstico.

Background: Signet-ring cell lymphoma is a rare entity that simulates epithelial neoplasms, sarcomas and reactive histiocytes conditions. It represents a morphological variant of non-Hodgkin's lymphomas, its diagnosis can represent a challenge, therefore it should be considered in complementary studies. Objective: The aim of this work is to show a case with a very rare morphology and to emphasize the importance of awareness this entity and avoid mistakes in its diagnosis. Clinical case: We present a case of a 67-year-old man, who developed lymph node growths in the right armpit, neck, right groin, and submandibular region, with apparent involvement of the lungs and spleen; was diagnosed as diffuse large B cell lymphoma with signet-ring morphology, originated in the germinal center. Transmission electron microscopy study was carried out for a more precise characterization of the morphology. Unfortunately, the patient did not return for a follow-up consultation, so he did not start treatment and died 6 months after diagnosis. Conclusions: Lymphoma with the signet-ring phenotype is rare, and can occur in any type of non-Hodgkin lymphoma; however, this morphology is more commonly associated with carcinomas and, less frequently, with sarcomas, melanomas or reactive histiocytes conditions, therefore should be considered this entity together with the appropriate use of complementary studies for proper diagnosis.

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.

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.

Mieloptisis y cáncer gástrico: Una asociación infrecuente / Myelophthisis and gastric cancer. An unusual association

Se denomina mieloptisis a la infiltración de la médula ósea por células no hematopoyéticas. En pacientes con cáncer gástrico esta invasión es extremadamente infrecuente y la supervivencia suele ser menor a tres meses. Presentamos el caso de un hombre de 35 años con compromiso de la médula ósea secundario a un carcinoma gástrico difuso de células en anillo de sello.

The infiltration of the bone marrow y non-hematopoietic cells is called myelophthisis. In patients with gastric cancer, this invasion is extremely infrequent and the survival is usually less than three months. We present the case of a 35-year-old man with bone marrow involvement secondary to diffuse gastric carcinoma of signet ring cells.

Clinicopathological Characteristics of Asymptomatic Young Patients with Gastric Cancer Detected during a Health Checkup / 대한소화기병학회잡지

BACKGROUND/AIMS: The Korean National Cancer Screening Program recommends biennial gastric cancer screening for patients aged ≥40 years. This study compared the characteristics of asymptomatic young gastric cancer patients aged <40 years, whose cancer was detected during a health checkup (screening group), with those whose disease was detected because of symptoms (diagnostic group). METHODS: Data were collected retrospectively from 84 subjects who underwent a gastroduodenoscopy before the age of 40 years and who were diagnosed with gastric cancer from January 2006 to February 2017 in three tertiary centers in Korea. The clinicopathological characteristics, including age, sex, stage, location, pathology, and survival, were compared according to the purpose of endoscopy (screening group, n=23 vs. diagnostic group, n=61). RESULTS: The median age of the screening group was higher than that of the diagnostic group (37 vs. 35 years, p=0.027), as was the proportion of early gastric cancer cases (78.3% vs. 29.5%, p<0.01), curative endoscopic treatment or operation rate (95.7% vs. 52.5%, p<0.01), and the overall survival (p<0.01). Poorly differentiated or signet ring cell carcinoma was less common in the screening group than in the diagnostic group (56.5% vs. 83.6%, p=0.006). The sex ratio, smoking status, family history of gastric cancer, Helicobacter pylori infection status, and tumor location were similar in the two groups. CONCLUSIONS: Screening gastroduodenoscopy may enable the early detection of gastric cancer and prolong survival in patients <40 years of age.

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.

Clinicopathological Characteristics of Asymptomatic Young Patients with Gastric Cancer Detected during a Health Checkup / 대한소화기학회지

BACKGROUND/AIMS: The Korean National Cancer Screening Program recommends biennial gastric cancer screening for patients aged ≥40 years. This study compared the characteristics of asymptomatic young gastric cancer patients aged <40 years, whose cancer was detected during a health checkup (screening group), with those whose disease was detected because of symptoms (diagnostic group).METHODS: Data were collected retrospectively from 84 subjects who underwent a gastroduodenoscopy before the age of 40 years and who were diagnosed with gastric cancer from January 2006 to February 2017 in three tertiary centers in Korea. The clinicopathological characteristics, including age, sex, stage, location, pathology, and survival, were compared according to the purpose of endoscopy (screening group, n=23 vs. diagnostic group, n=61).RESULTS: The median age of the screening group was higher than that of the diagnostic group (37 vs. 35 years, p=0.027), as was the proportion of early gastric cancer cases (78.3% vs. 29.5%, p<0.01), curative endoscopic treatment or operation rate (95.7% vs. 52.5%, p<0.01), and the overall survival (p<0.01). Poorly differentiated or signet ring cell carcinoma was less common in the screening group than in the diagnostic group (56.5% vs. 83.6%, p=0.006). The sex ratio, smoking status, family history of gastric cancer, Helicobacter pylori infection status, and tumor location were similar in the two groups.CONCLUSIONS: Screening gastroduodenoscopy may enable the early detection of gastric cancer and prolong survival in patients <40 years of age.

Postoperative Radiotherapy Improves Survival in Gastric Signet-Ring Cell Carcinoma: a SEER Database Analysis

PURPOSE: To identify the potential therapeutic role of postoperative radiotherapy (RT) in patients with locally advanced (stage II and stage III) gastric signet ring cell carcinoma (SRC).MATERIALS AND METHODS: Patients with locally advanced gastric SRC from the Surveillance, Epidemiology, and End Results program database between 2004 and 2012 were included in our study. Univariate and multivariate Cox proportional models were performed, and survival curves were generated to evaluate the prognostic effect of postoperative RT and surgery alone on SRC patients. Propensity score matching (PSM) was used to avoid selection bias among the study cohorts.RESULTS: We found that patients with postoperative RT had better probability of survival compared with those who did not receive RT (overall survival [OS], P<0.001; cancer-specific survival [CSS], P<0.001). After PSM, analysis of both overall and CSS showed that patients who underwent postoperative RT had better prognosis than those receiving surgery alone in the matched cohort (OS, P=0.00079; CSS, P=0.0036). Multivariate Cox proportional model indicated that postoperative RT had better effect on prognosis compared with surgery alone with respect to both overall (hazard ratio [HR], 0.716; 95% confidence interval [95% CI], 0.590–0.87; P=0.001) and CSS (HR, 0.713; 95% CI, 0.570–0.890; P=0.003).CONCLUSIONS: Postoperative RT had better prognosis compared with surgery alone for both overall and CSS for patients with locally advanced gastric SRC.

An elderly man with gastric cancer, Saint's triad and Heyde's syndrome / Un anciano con cáncer gástrico, tríada de Saint y síndrome de Heyde

This case study describes a 71-year-old man with signet-ring cell gastric adenocarcinoma and malignant sigmoidal polyp; and typical features of Saint's triad and Heyde syndrome. He had digestive bleeding, two types of hernia, diverticulosis, arterial hypertension, malignant polyp, and antecedent of smoking, lung tuberculosis, and surgical correction of aortic valve stenosis. There is a hypothetical inverse relationship between herniosis and development of malignancy; however, the patient herein described presented gastric and sigmoidal cancers. Gastrointestinal malignancies are sometimes associated with paraneoplastic entities, isolated or manifested as syndromes, but neither Saint's triad or Heyde syndrome have been included. This patient persisted clinically stable during the preoperative period, but suddenly died; Trousseau's syndrome would be the most probable mechanism of sudden death in this setting. Case reports can stimulate further studies to get additional knowledge about unusual entities.

Este estudio de caso describe un hombre de 71 años de edad, con adenocarcinoma gástrico con células en anillo de sello y un pólipo maligno sigmoideo; y características típicas de la tríada de Saint y del síndrome de Heyde. Tuvo una hemorragia digestiva, dos tipos de hernias, divertículos, hipertensión arterial, y pólipo maligno; con antecedente de tabaquismo, tuberculosis pulmonar, y corrección quirúrgica de estenosis de la válvula aórtica. Hay una hipotética relación inversa entre hernioses y el desarrollo de malignidades; sin embargo, el paciente que se describe en el presente documento presentó cánceres gástrico y sigmoideo. Neoplasias gastrointestinales se asocian a veces con entidades para neoplásicas aisladas o manifiestan síndromes, pero ni la tríada de Saint ni el síndrome de Heyde se ha incluido. Este paciente persistió clínicamente estable durante el período preoperatorio, pero de repente murió; síndrome de Trousseau sería el mecanismo más probable de muerte súbita en esta situación. Los informes de casos pueden estimular más estudios para obtener un conocimiento adicional sobre esas entidades inusuales.

La presencia de células en anillo de sello en la biopsia endoscópica no es un buen predictor para el diagnóstico de carcinoma gástrico de células en anillo de sello / The presence of signet-ring cell in endoscopic biopsy is not a good predictor for the diagnosis of signet-ring cell carcinoma of the stomach

Resumen Introducción El carcinoma gástrico de células en anillo de sello (CGCAS) es un tipo histopatológico, que tiene menor respuesta a la quimioterapia (QT) y un peor pronóstico en los pacientes con cáncer gástrico (CG) avanzado. Se desconoce los valores diagnósticos de la presencia de células en anillo de sello (CAS) en la biopsia endoscópica, para el diagnóstico de CGCAS. Objetivo Determinar los valores diagnósticos de la presencia de CAS en la biopsia endoscópica para el diagnóstico de CGCAS en la biopsia de la pieza operatoria. Material y Método Estudio retrospectivo de pruebas diagnósticas. Se incluyeron los pacientes con CG operados en forma consecutiva entre 1996-2016. Se calculó los valores diagnósticos de la presencia de CAS en la biopsia endoscópica para el diagnóstico de CGCAS en la biopsia definitiva. Se utilizaron intervalos de confianza (IC) del 95%. Resultados Se incluyeron 851 pacientes. Un 16,3% tuvieron CAS en la biopsia endoscópica y la prevalencia de CGCAS fue de 16,4%. Los valores diagnósticos de la presencia de CAS de la biopsia endoscópica para el diagnóstico de CGCAS fueron: Valor predictivo positivo (VPP) de 56,1% (IC 95%, 47,8-64,1%); Valor predictivo negativo (VPN) de 91,3% (IC 95%, 89-93,1%); sensibilidad de 55,7% (IC 95%, 47,4-63,7%); especificidad de 91,4% (IC 95%, 89,1%-93,3%); Likelihood ratio (LR) positivo de 6,5 (IC 95%, 4,9-8,6); LR negativo de 0,48 (IC 95%, 0,4-0,6); probabilidad post-test positivo fue de 56,1% (IC 95%, 47,8-64,1%) y probabilidad post-test negativo fue de 8,7% (IC 95%, 6,9-11%). Conclusiones La presencia de CAS en la biopsia endoscópica es insuficiente para el diagnóstico de un CGCAS. La ausencia de CAS en la biopsia endoscópica tiene un alto valor predictivo negativo.

Introduction Signet-ring cell carcinoma (SRCC) of the stomach is a histopathological type that has less response to chemotherapy and worse prognosis in patients with advanced gastric cancer, than other types of gastric carcinomas. Diagnostic value of the presence of signet-ring cells (SRC) in the endoscopic biopsy for the diagnosis of SRCC of the stomach, are unknown. Objectives To calculate the diagnostic values of the presence of SRC in endoscopic biopsy for the diagnosis of SRCC of the stomach in a definitive surgical specimen biopsy. Materials and Methods Retrospective diagnostic test study to determine the value of the presence of SRC in the endoscopic biopsy for the diagnosis of SRCC of the stomach in the surgical specimen biopsy. Inclusion criteria: Patients who underwent gastric surgery between 1996-2016. We calculated positive and negative predictive values (PPV and NPV), sensitivity, specificity, and positive and negative likelihood ratio (LR+ and LR−) of the presence of SRC in the endoscopic biopsy that predicts the diagnosis of SRCC of the stomach in the definitive biopsy. Confidence intervals (CI) of 95% were defined. Results The diagnostic values of the presence of SRC in endoscopic biopsy to diagnose SRCC of the stomach in the surgical specimen biopsy were: PPV of 56.1% (95% CI, 47.8-64.1%), NPV of 91.3% (95% CI, 89-93.1%), sensitivity of 55.7% (95% CI, 47.4-63.7%), specificity of 91.4% (95% CI, 89.1-93.3%), LR+ of 6.5 (95% CI, 4.9-8.6) and LR- of 0.48 (95% CI, 0.4-0.6), a positive post-test probability of 56.1% (95% CI, 47.8-64.1%), and a negative post-test probability of 8.7% (95% CI, 6.9-11%). Conclusions The presence of SRC in the endoscopic biopsy is not sufficient to diagnose SRCC of the stomach. The absence of SRC in the endoscopic biopsy has a high negative predictive value.

Outcomes of Endoscopic Submucosal Dissection for Early Gastric Cancer with Undifferentiated-Type Histology: A Clinical Simulation Using a Non-Selected Surgical Cohort

BACKGROUND/AIMS: Outcomes of endoscopic submucosal dissection (ESD) for undifferentiated-type early gastric cancer (EGC) need to be further evaluated. We aimed to simulate the outcomes of ESD for undifferentiated-type EGC from a surgical database. METHODS: Among 802 patients who underwent gastrectomy with endoscopic biopsy for poorly differentiated adenocarcinoma (PD-type) or signet ring cell carcinoma (SRC-type), ESD candidates meeting the expanded indication (n=280) were selected by reviewing the endoscopic images. According to the surgical pathologic results, the outcomes of the ESD simulation were evaluated. RESULTS: Among the candidates, 104 (37.1%) were PD-type and 176 (62.9%) were SRC-type. The curative resection (CR) rate was 42.1%. Among the patients with CR, three patients (2.5%) showed lymph node metastasis (LNM). Three EGCs with CR and LNM were mucosal cancers ≥1.0 cm in size. The CR rate was higher in the SRC-type than in the PD-type (48.3% vs 31.7%, respectively, p=0.007). In the SRC-type, the CR rate was increased, with a smaller size criterion for the ESD indication, but was similar between the 1.0 cm and 0.6 cm criteria (63.3% and 63.6%, respectively), whereas the CR rate was below 50% in all of the different tumor size criteria (2.0 to 0.6 cm) in the PD-type. CONCLUSIONS: In undifferentiated-type EGC, ESD should be considered in selected patients with tumor sizes < 1 cm and SRC histology.

Bicytopenia and leukoerythroblastosis: a rare initial presentation of signet ring cell gastric adenocarcinoma

Gastric adenocarcinoma is a common neoplasia and is responsible for up to 30% of the overall deaths due to cancer. Advanced disease is mostly characterized by peritoneum, liver, and lung involvement. The spread of the disease to the bone is rare, and bone marrow dissemination is even rarer. In this setting, leukoerythroblastosis may be the initial manifestation of the disease. The authors report the case of a 64-year-old Caucasian man who sought medical care complaining of back pain, weakness, and weight loss. The physical examination revealed pallor, and the laboratory work-up depicted severe anemia and thrombocytopenia; the peripheral blood smear was consistent with leukoerythroblastosis. The ongoing investigation through a bone marrow biopsy showed massive involvement of the bone marrow by a signet ring cell adenocarcinoma. During hospitalization, the patient presented melena, and an upper digestive endoscopy depicted an ulcerated and infiltrative lesion in the cardia, upon which the histological examination revealed a signet ring cell adenocarcinoma. This case highlights the bone marrow invasion represented by bicytopenia and leukoerythroblastosis as the initial manifestation of this histological type of gastric cancer. Although treatment attempts were made with chemotherapy and radiotherapy, the patient died early on, showing the aggressive behavior of this form of tumoral presentation.

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.

Adenocarcinoma gástrico difuso en un niño de 10 años: reporte de un caso / Diffuse type of gastric adenocarcinoma in 10 years old boy: report of a case

El objetivo del presente estudio fue presentar un caso extremadamente raro de adenocarcinoma gástrico difuso con células en anillo de sello en un niño de 10 años. Se presenta un niño de 10 años, con un tiempo de enfermedad de 12 meses con sintomatología de disfagia a sólidos, luego a líquidos, pérdida de peso, anorexia, astenia, mareos, vómitos y dolor en el hipocondrio izquierdo. Presenta desnutrición crónica, marcada palidez y máculas "café con leche" en tronco y extremidades. Los exámenes de laboratorio evidenciaron 7,5 gr de hemoglobina, albúmina 2,62 gr, thevenon en heces positivo. Una ecografía abdominal mostró masas periaórticas y lesiones difusas a nivel hepático. A la endoscopía se observó lesión elevada esófago-gástrica que obstruye la luz. A nivel subcardial tumoración de 3 cm de bordes irregulares, superficie erosionada y sangrante al roce. Se realizó biopsia múltiple. Murió un año después por metástasis generalizada. Nació de parto normal, de madre primigesta, con 3 500 gr de peso. Antecedentes familiares: tía abuela materna falleció de cáncer gástrico. La biopsia mostró un adenocarcinoma difuso con presencia de células en anillo de sello, PAS positivo y a la inmunohistoquímica fue positiva a la CK 8.

We report a rare case of diffuse type of gastric cancer with signet ring cells in 10 years old boy who was admitted with a 12 months history with weight loss, dysphagia to solids first and to liquids later, anorexia, fatigue, dizziness, vomiting and later, with pain in the left upper quadrant. On examination, he appeared pale, malnourished, with café-au-lait spots over the trunk and extremities. Laboratory tests showed; Hb 7.5 g, albumin 2.62 g, Thevenon positive on stools. Abdominal ultrasound examination showed periaortic masses and diffuse space occupying lesions in the liver. Endoscopic examination of the stomach showed multiple elevated tumor lesions. One located at 3 cm on the subcardial region presented irregular borders, partially eroded, that bleed easily when rubbing its surface. Multiple biopsy samples were taken. They showed a diffuse gastric signet cell type carcinoma of the stomach. Immunohistochemistry was positive to CK 8. The patient died a year later with wide spread metastasis. The boy was born through a normal delivery after a normal pregnancy to a primipara mother. His family history recorded a grandmother aunt dying of gastric cancer.

Geographic distribution of the incidence of colorectal cancer in Iran: a population-based study / 한국역학회지

OBJECTIVES: Colorectal cancer (CRC) is the third most common cancer and the fourth most common cause of cancer death in the world. The aim of this study was to investigate the provincial distribution of the incidence of CRC across Iran. METHODS: This epidemiologic study used data from the National Cancer Registry of Iran and the Center for Disease Control and Prevention of the Ministry of Health and Medical Education of Iran. The average annual age-standardized rate (ASR) for the incidence of CRC was calculated for each province. RESULTS: We found that adenocarcinoma (not otherwise specified) was the most common histological subtype of CRC in males and females, accounting for 81.91 and 81.95% of CRC cases, respectively. Signet ring cell carcinoma was the least prevalent subtype of CRC in males and females and accounted for 1.5 and 0.94% of CRC cases, respectively. In patients aged 45 years or older, there was a steady upward trend in the incidence of CRC, and the highest ASR of CRC incidence among both males and females was in the age group of 80-84 years, with an ASR of 144.69 per 100,000 person-years for males and 119.18 per 100,000 person-years for females. The highest incidence rates of CRC in Iran were found in the central, northern, and western provinces. Provinces in the southeast of Iran had the lowest incidence rates of CRC. CONCLUSIONS: Wide geographical variation was found in the incidence of CRC across the 31 provinces of Iran. These variations must be considered for prevention and control programs for CRC, as well as for resource allocation purposes.

Clinicopathologic characteristics of teenage sporadic colorectal cancer

PURPOSE: Colorectal cancer (CRC) is generally considered a disease of old age. Most CRCs are diagnosed at age 50 and over. CRC rarely occurs in teenagers, and the clinical features and prognosis of CRC are not clear in this population. The aim of this study was to uncover the clinicopathologic characteristics of teenage sporadic CRC.METHODS: Of the 21,042 patients who underwent operation for primary CRC at Asan Medical Center between July 1989 and December 2014, 19 cases (0.09%) without a familial history of CRC before 20 years of age at diagnosis were enrolled in this study. The clinicopathologic features of the teenage sporadic CRC patients were retrospectively reviewed.RESULTS: Of the 19 patients, 16 patients (84.2%) were male. The most common primary site was the left colon (descending colon & sigmoid colon) in nine patients. With respect to histologic type, adenocarcinoma represented 57.8% of cases, mucinous adenocarcinoma, 31.5%, and signet ring cell carcinoma, 10.5%. Six (31.5%) patients showed peritoneal seeding at presentation. In survival analysis, the 5-year overall survival rate of the patients who underwent curative surgery was 71.3%.CONCLUSION: Teenage sporadic CRC is a very rare disease and the proportion of patients with a poor histologic subtype is high, but early detection and radical treatment can lead to favorable survival rates.

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

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.