Gastric Cancer in BRCA1 Germline Mutation Carriers: Results of Endoscopic Screening and Molecular Analysis of Tumor Tissues.

INTRODUCTION: There is some evidence suggesting a link between BRCA1/2 germline mutations and increased risk of gastric cancer. METHODS: Endoscopic screening for stomach malignancies was performed in 120 BRCA1 mutation carriers in order to evaluate the probability of detecting the tumor disease. RESULTS: No instances of gastric cancer were revealed at the first visit. The analysis of atrophic changes performed by OLGA (Operative Link for Gastritis Assessment) criteria revealed that OLGA stages I-IV alterations were observed in 26 of 41 (63%) subjects aged >50 years as compared to 29 of 79 (37%) in younger subjects (p = 0.007, χ2 test). One BRCA1 mutation carrier developed gastric cancer 4 years after the first visit for endoscopic examination. We performed next-generation sequencing analysis for this tumor and additional 4 archival gastric cancers obtained from BRCA1/2 mutation carriers. Somatic loss of the remaining BRCA1/2 allele was observed in 3 out of 5 tumors analyzed; all of these carcinomas, but none of the malignancies with the retained BRCA1/2 copy, showed chromosomal instability. CONCLUSION: Taken together, these data justify further studies on the relationships between the BRCA1/2 and gastric cancer.

Primary gastric lymphoma: A report of 16 pediatric cases treated at a single institute and review of the literature.

Gastrointestinal tract is the most common extranodal site for childhood non-Hodgkin lymphomas (NHLs). However, primary gastric lymphoma (PGL) is very rare. We report our experience with PGL. Between 1972 and 2019, patients with PGL among 1696 NHL cases were evaluated retrospectively. Patient characteristics, treatments, and survival rates were recorded. We also reviewed the cases reported in literature. There were 16 PGL (11 males, five females) cases with a median age of 10 years. Most frequent complaints, similarly to the literature, were pain and vomiting. Hematemesis/melena and anemia were present in 20% of patients. Most common tumor location was antrum. Histopathological subtypes were Burkitt and non-Burkitt B-cell lymphoma in 43.75% and marginal zone lymphoma (MZL) in 6.25% of cases while mucosa-associated lymphoid tissue (MALT) and low-grade lymphomas constitute 15.3% of cases reported in the literature. In our series, Helicobacter pylori (H. pylori) was analyzed in only the case with MZL and found to be positive. However, H. pylori positivity was reported in 75% of the cases in the literature. H. pylori eradication, chemotherapy, and radiotherapy were applied in one, 14, and five patients. Subtotal gastrectomy with gastroduodenostomy/jejunostomy was performed in three patients. Gastrojejunostomy was done without tumor resection in two patients. Nine patients lived without disease for a median of 59 (12-252) months. Five-year EFS and OS were 69.6% and 64.3%, respectively. PGL constitutes 0.94% of our NHL cases. Interestingly, most of the cases in the literature were from Turkey. While adult PGL is mostly MALT lymphoma, most pediatric cases had high-grade histopathology. Although surgery and radiotherapy were applied earlier, chemotherapy alone is sufficient.

Rapidly grown congenital fetal immature gastric teratoma causing severe neonatal respiratory distress.

A pregnant woman in the mid-third trimester developed complications with enlarged fetal abdomen and polyhydramnios. Prenatal ultrasound visualized dilated bowel, intraperitoneal calcifications, ascites, hydroceles and polyhydramnios, giving the impression of meconium peritonitis. The fetal abdomen continued to increased in size, and maternal dyspnea due to polyhydramnios was aggravated. She underwent a cesarean section at 36 + 1 weeks' gestation. The delivery was followed by severe neonatal respiratory distress due to the huge mass in the abdomen. The tumor was successfully removed by emergency surgery and diagnosed as immature gastric teratoma. No other associated anomaly was found. The infant made a good progress after the operation.

[Hereditary non-polyposis colorectal cancer associated with hereditary gastric cancer. A case report.]. / Cáncer colorrectal hereditario no polipósico asociado a cáncer gástrico hereditario. Informe de un caso.

The information regarding the association of gastric cancer and type 2 Lynch syndrome is limited. Previous studies have reported that both entities may be present in the same individual in less than 5% of the cases. The most frequent form of hereditary colorectal cancer is the Lynch syndrome or Hereditary Non-polyposic Colorectal Cancer, which is associated with germ-line mutation mostly of two genes, MLH1 and MSH2, which account for almost 90 percent of all identified mutations. The hereditary diffuse gastric cancer syndrome is caused by a germ-line mutation in the E-cadherin (CDH1) gene; only about 50 families with this syndrome have been reported. We present a case report of a patient who was diagnosed with both syndromes.

Recurrent yolk sac tumor following resection of a neonatal immature gastric teratoma.

Gastric teratomas are very rare and usually benign. Only a few cases of gastric teratomas with malignant components have been reported. This report describes recurrence of a yolk sac tumor following resection of a neonatal immature gastric teratoma. Gastric teratoma recurring as a malignant lesion has not been previously reported. Recurrence of immature gastric teratomas should be considered, and a periodic follow-up check with alpha-fetoprotein level should be mandatory.

Congenital gastric teratoma with gastric perforation mimicking meconium peritonitis.

A case of congenital immature gastric teratoma along with spontaneous gastric perforation in a 1-day-old boy is presented. The clinical features are similar to those of meconium peritonitis. Coincidentally, the child had an arachnoid cyst at 25 months of age. To the best of the authors knowledge, this case of congenital immature gastric teratoma associated with gastric perforation mimicking meconium peritonitis, is the first description in the English-language literature.

[Gastric teratoma]. / Das Magenteratom.

Gastric teratomas are rare and mostly benign. They frequently become evident as a palpable abdominal mass. A malignant, aggressive solid tumor must be excluded. We present a 6-month-old male infant with a history of bleeding of the upper gastrointestinal tract. After in toto excision the histopathology was that of a benign gastric teratoma. Our method of reading the diagnosis and the subsequent therapy are described.

Malignant gastric teratoma: case report.

Gastric teratomas are rare and usually benign. A 4 month old boy presented with an abdominal mass and computed tomographic and ultrasound examination demonstrated a large multi-loculated tumour which was totally excised. The pathological diagnosis was of a malignant gastric teratoma and 12-month follow-up was uneventful.

Congenital double pylorus with accessory pyloric channel communicating with an intraluminal duplication cyst of the duodenum.

Congenital double pylorus is a rare anomaly in which two pyloric openings connect the antrum of the stomach to the duodenal bulb. We report an unusual case of congenital double pylorus in which one of the channels led to an intraluminal cystic duplication of the duodenal bulb. An embryologic relation between congenital double pylorus and antroduodenal duplications is suggested.

Heterotopic pancreas as a rare cause of gastrointestinal hemorrhage in the newborn: report of a case.

We report herein a female infant in whom a heterotopic pancreas in the stomach caused gastrointestinal hemorrhage during the newborn period. Endoscopy was essential for ruling out other causes of hemorrhage and to follow the patient until the time of elective surgery at 6 months of age. Heterotopic pancreas should be borne in mind as a rare cause of gastrointestinal hemorrhage in the newborn when an unexplained gastrointestinal hemorrhage continues, and the lesion was discussed in relation to our experience of upper gastrointestinal endoscopies and a review of the literature.

Gastric teratoma: a case report.

Gastric teratoma is a very rare, usually benign, tumour of childhood. In industrialized countries, the diagnosis is facilitated by advanced technology when the infant is still in utero or soon after birth, and surgery is performed quite early in life, long before symptoms appear. This tumour was seen in our hospital in a 4-month-old boy. A mass had been noted at birth, but the infant remained symptomless. He was brought to hospital only on account of its rapid growth. The teratoma was successfully excised and the child was not seen again after discharge from hospital. We presume he remains well.

Malignant gastric teratoma in an infant.

Malignancy in gastric teratomas is extremely rare. Such teratomas are widely accepted and treated as benign lesions. A malignant gastric teratoma treated by total excision is presented. According to our knowledge, this is the second case of gastric teratoma having a malignant histology.

[Teratoma of the stomach in a 4-day-old newborn infant]. / Teratom des Magens bei einem 4-Tage-alten Neugeborenen.

Teratoma of the stomach is a very rare tumor. Nearly all previously reported patients were males. We report a 4 day-old male infant, who presented with a palpable abdominal mass. A preliminary diagnosis of teratoma was made by abdominal x-ray, sonography and MR imaging. Surgery was performed and diagnosis of teratoma of the stomach was confirmed by histological examination of the tumor. Because teratoma of the stomach is always a benign neoplasm surgery is curative and no further therapy is needed.