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Annals of the Royal College of Surgeons... Nov 2016Although sporadic lipomas are not uncommon in the upper gastrointestinal tract, diffuse gastroduodenal lipomatosis is a rare clinical entity. Medical literature reveals...
Although sporadic lipomas are not uncommon in the upper gastrointestinal tract, diffuse gastroduodenal lipomatosis is a rare clinical entity. Medical literature reveals a limited number of such cases presenting with upper gastrointestinal obstruction or bleeding. We present the management experience of a 43-year-old woman who presented with intussusception causing high small-bowel obstruction secondary to jejunal lipomatosis. Computed tomography showed diffuse fatty thickening of the gastric wall in addition to multiple lipomas in stomach, duodenum and in the jejunum with jejunal intussusception. As complete resection of the affected segment was not possible, a side-to-side jejunal bypass was made. The patient remains well on review after 18 months.
Topics: Adult; Duodenal Neoplasms; Female; Humans; Intussusception; Lipoma; Stomach Diseases; Stomach Neoplasms; Tomography, X-Ray Computed
PubMed: 27551904
DOI: 10.1308/rcsann.2016.0243 -
The American Journal of Case Reports Jan 2021BACKGROUND Neurofibromatosis type 1 (NF1) is a multi-tumor syndrome in which affected patients develop malignancies that are rare in the overall population, such as... (Review)
Review
Pancreatic Gastrinoma, Gastrointestinal Stromal Tumor (GIST), Pheochromocytoma, and Hürthle Cell Neoplasm in a Patient with Neurofibromatosis Type 1: A Case Report and Literature Review.
BACKGROUND Neurofibromatosis type 1 (NF1) is a multi-tumor syndrome in which affected patients develop malignancies that are rare in the overall population, such as tumors of neural or endocrine origin. CASE REPORT A 67-year-old woman with a clinical diagnosis of NF1 presented with abdominal pain and pneumoperitoneum. She underwent small-bowel resections for a perforated jejunal lesion and a second lesion in the ileum; pathology showed a neurofibroma at the site of the perforation and a 1-cm low-grade GIST, respectively. Additional staging with cross-sectional imaging identified a 3.7-cm pancreatic head mass and a 1.7-cm left adrenal mass; biochemical studies revealed elevated serum gastrin and urinary free metanephrines and catecholamines consistent with pheochromocytoma. Initial surgical management was a left posterior retroperitoneoscopic adrenalectomy. Postoperatively, gallium-68-DOTATOC PET/CT showed uptake in the pancreatic head and a 28-mm left thyroid nodule. Months later, she had an open pancreaticoduodenectomy. Pathology showed pheochromocytoma and a low-grade (G1) gastrinoma involving 2/8 peripancreatic lymph nodes (pT3pN1M0), respectively. Fine-needle aspiration biopsy of the thyroid nodule showed features consistent with a Hürthle cell neoplasm. Genetic testing identified a pathogenic mutation in NF1 and no mutations in BRCA1/2, CDC72, MEN1, or PALB2. The patient continues surveillance, with no evidence of recurrent disease. CONCLUSIONS We report the fifth case of gastrinoma associated with NF1 and the first to arise from the pancreas. This case of a pancreatic neuroendocrine tumor was associated with multiple additional neoplasms. Neuroendocrine tumors found in NF1 should raise suspicion of other malignancies.
Topics: Adenoma, Oxyphilic; Aged; Endocrine Gland Neoplasms; Female; Gastrinoma; Gastrointestinal Neoplasms; Gastrointestinal Stromal Tumors; Humans; Neurofibromatosis 1; Pheochromocytoma
PubMed: 33452231
DOI: 10.12659/AJCR.927761 -
Pleura and Peritoneum Sep 2017Cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) has changed treatment for selected patients with peritoneal metastases (PM) arising...
BACKGROUND
Cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) has changed treatment for selected patients with peritoneal metastases (PM) arising from appendiceal, colorectal, epithelial ovarian, primary peritoneal and gastric cancers. However, the results of CRS with HIPEC remain unclear in PM from other tumor histologies.
METHODS
We report a series of 10 patients who underwent CRS and HIPEC between 2006 and 2015, for PM arising from uncommon tumor origins.
RESULTS
Ten patients with PM from uncommon tumor origins underwent CRS and HIPEC. Median age was 46.5 years. Two patients had ovarian Sertoli-Leydig cell tumors (SLCT) and two had small bowel adenocarcinomas. The other histologies included: ovarian transitional cell carcinoma, ovarian granulosa cell tumor, endometroid adenocarcinoma, endocervical adenocarcinoma, synovial sarcoma, and ovarian leiomyosarcoma. Median peritoneal cancer index was 9 (2-18) and complete cytoreduction was achieved for all patients. Median follow-up was 14 months (2-100), and median time to recurrence from CRS and HIPEC was 16.0 months by Kaplan-Meier estimate. Four patients remain alive and disease-free, five are alive with disease, and one had died with disease. Median survival was not reached.
CONCLUSIONS
Eight of ten patients with peritoneal metastases in the above rare indications survived 10 months or more after CRS and HIPEC. These encouraging results are a rationale for prospective clinical trials in these tumor histologies.
PubMed: 30911642
DOI: 10.1515/pp-2017-0017 -
Medicine Sep 2022A jejunal gastrointestinal stromal tumor (GIST) is a rare neoplasm of the gastrointestinal (GI) tract. Massive bleeding due to a jejunal GIST is a diagnostic and...
BACKGROUND
A jejunal gastrointestinal stromal tumor (GIST) is a rare neoplasm of the gastrointestinal (GI) tract. Massive bleeding due to a jejunal GIST is a diagnostic and therapeutic challenge. It may be a life-threatening GIST complication that requires urgent intervention. Acute GI bleeding, which requires urgent surgical intervention, is a very rare clinical manifestation of GIST. A jejunal GIST with massive hemorrhage with coronavirus disease 2019 in a male patient in older age with many comorbidities has been not reported in the worldwide literature.
METHODS
In this case report, we present an 80-year-old man who was admitted to surgery due to abdominal pain, melena, and hematochezia for several hours. An upper endoscopy and colonoscopy were inconclusive. A multidetector contrast-enhanced computed tomography (CT) of the abdominal and pelvic cavity showed concentric irregular thickening in the distal jejunum.The histopathological finding showed a GIST measuring 6 cm with a mitotic index 2/50 high power fields. The patient's hemodynamic condition deteriorated despite initial conservative treatment including a blood transfusion. Therefore, patient underwent the emergency surgery 24 hours after admission: partial jejunal resection with the tumor followed by primary end-to-end anastomosis.
RESULTS
The mass was removed completely. There were no surgical complications in the postoperative course. On the first postoperative day, a severe acute respiratory syndrome coronavirus 2 polymerase chain reaction test was performed due to a persistent dry cough, which yielded a positive result. After 14 days, the patient died due to pneumonia and circulatory failure.
CONCLUSIONS
This case indicates that jejunal GIST can present as massive lower gastrointestinal bleeding and urgent surgery can successfully stop bleeding and save the patient's life. The CT scan was the most effective investigation to find the source of GI bleeding in this case. Therefore, we suggest performing CT in patients with acute massive lower gastrointestinal bleeding when the source of bleeding is not visible on endoscopy, and urgent surgical jejunal resection to stop life-threatening bleeding caused by a jejunal GIST.
Topics: Aged, 80 and over; COVID-19; Endoscopy, Gastrointestinal; Gastrointestinal Hemorrhage; Gastrointestinal Stromal Tumors; Humans; Jejunum; Male
PubMed: 36107510
DOI: 10.1097/MD.0000000000030098 -
The American Journal of Case Reports Mar 2022BACKGROUND Esophageal invasion in hypopharyngeal cancer is an uncommon lesion and has a poor prognosis. Total pharyngo-laryngo-esophagectomy is the most effective...
BACKGROUND Esophageal invasion in hypopharyngeal cancer is an uncommon lesion and has a poor prognosis. Total pharyngo-laryngo-esophagectomy is the most effective treatment option. Reconstruction of the gastrointestinal tract in the same period of surgery is required. There are many different options, such as pedicle flap, gastric pull-up, pedicled transverse colon flaps, and free flap. In cases where only cervical esophagectomy is required, with benefits for patients, jejunal free flap is the first choice to reconstruct the esophagus. However, the extensive surgical resection requires interdisciplinary collaboration among surgical specialities, which is not always available. CASE REPORT A 52-year-old male patient came to us with a chief concern of dysphagia, which first appeared 3 months ago. Rigid fiberoptic and direct laryngoscopes indirectly observed the tumor at the posterior wall; it had passed the esophagus opening and infiltrated to the cervical esophagus, with the rightmost part spread into the right pyriform sinus. Histopathology studies of the tumor showed a squamous cell carcinoma. The patient underwent total pharyngo-laryngo-cervico-esophagectomy, reconstructed with a jejunal free flap, and adjuvant radiation therapy after surgery. At follow-up 1 year after surgery, the patient was significantly recovery with no signs of tumor recurrence, no difficulties of oral intake, and his speech was restored by electrolarynx. CONCLUSIONS We reported the case of a patient with hypopharyngeal cancer who underwent total pharyngo-laryngo-cervico-esophagectomy and esophagus reconstruction using a jejunal free flap, performed for the first time in Vietnam. As expected, with good oncological and functional results, the degree of invasiveness and sequelae of the surgery can be well tolerated.
Topics: Developing Countries; Esophageal Neoplasms; Esophagectomy; Free Tissue Flaps; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Vietnam
PubMed: 35342189
DOI: 10.12659/AJCR.934974 -
BMJ Case Reports Dec 2018Peutz-Jeghers syndrome (PJS) is an autosomal dominant cancer-predisposing condition characterised by intestinal hamartomatous polyps and distinct melanin depositions in...
Peutz-Jeghers syndrome (PJS) is an autosomal dominant cancer-predisposing condition characterised by intestinal hamartomatous polyps and distinct melanin depositions in skin and mucosa. Small intestinal cancer in patients with PJS usually presents by the third decade. A 7-year-old-PJS boy presented with recurrent episodes of colicky abdominal pain and melena requiring repeated blood transfusions. Abdominal CT scan revealed multiple jejunal polyps with jejunoileal intussusception. On exploration, the intussuscepted bowel was resected along with its mesentery and anastomosed. Simultaneously, multiple enterotomies with resection of palpable polyps were performed. The resected bowel showed well-differentiated stage 2A adenocarcinoma with clear resected margins. Postoperatively, the complaints were relieved. On follow-up, he was asymptomatic and is now on yearly cancer surveillance. This is probably the youngest reported case of small bowel cancer in PJS.
Topics: Adenocarcinoma; Child; Humans; Intestinal Polyps; Intestine, Small; Intussusception; Jejunal Neoplasms; Male; Melena; Pedigree; Peutz-Jeghers Syndrome
PubMed: 30567229
DOI: 10.1136/bcr-2018-225076 -
Neuroendocrinology 2021Small intestinal neuroendocrine tumors (SI-NETs) are difficult to diagnose in the early stage of disease. Current blood biomarkers such as chromogranin A (CgA) and...
BACKGROUND
Small intestinal neuroendocrine tumors (SI-NETs) are difficult to diagnose in the early stage of disease. Current blood biomarkers such as chromogranin A (CgA) and 5-hydroxyindolacetic acid have low sensitivity (SEN) and specificity (SPE). This is a first preplanned interim analysis (Nordic non-interventional, prospective, exploratory, EXPLAIN study [NCT02630654]). Its objective is to investigate if a plasma protein multi-biomarker strategy can improve diagnostic accuracy (ACC) in SI-NETs.
METHODS
At the time of diagnosis, before any disease-specific treatment was initiated, blood was collected from patients with advanced SI-NETs and 92 putative cancer-related plasma proteins from 135 patients were analyzed and compared with the results of age- and sex-matched controls (n = 143), using multiplex proximity extension assay and machine learning techniques.
RESULTS
Using a random forest model including 12 top ranked plasma proteins in patients with SI-NETs, the multi-biomarker strategy showed SEN and SPE of 89 and 91%, respectively, with negative predictive value (NPV) and positive predictive value (PPV) of 90 and 91%, respectively, to identify patients with regional or metastatic disease with an area under the receiver operator characteristic curve (AUROC) of 99%. In 30 patients with normal CgA concentrations, the model provided a diagnostic SPE of 98%, SEN of 56%, and NPV 90%, PPV of 90%, and AUROC 97%, regardless of proton pump inhibitor intake.
CONCLUSION
This interim analysis demonstrates that a multi-biomarker/machine learning strategy improves diagnostic ACC of patients with SI-NET at the time of diagnosis, especially in patients with normal CgA levels. The results indicate that this multi-biomarker strategy can be useful for early detection of SI-NETs at presentation and conceivably detect recurrence after radical primary resection.
Topics: Biomarkers; Duodenal Neoplasms; Humans; Ileal Neoplasms; Jejunal Neoplasms; Machine Learning; Neuroendocrine Tumors
PubMed: 32721955
DOI: 10.1159/000510483 -
Journal of Gastroenterology May 2024The clinicopathological features and prognosis of primary small bowel adenocarcinoma (PSBA), excluding duodenal cancer, remain undetermined due to its rarity in Japan.
BACKGROUND
The clinicopathological features and prognosis of primary small bowel adenocarcinoma (PSBA), excluding duodenal cancer, remain undetermined due to its rarity in Japan.
METHODS
We analyzed 354 patients with 358 PSBAs, between January 2008 and December 2017, at 44 institutions affiliated with the Japanese Society for Cancer of the Colon and Rectum.
RESULTS
The median age was 67 years (218 males, 61.6%). The average tumor size was 49.9 (7-100) mm. PSBA sites consisted of jejunum (66.2%) and ileum (30.4%). A total of 219 patients (61.9%) underwent diagnostic small bowel endoscopy, including single-balloon endoscopy, double-balloon endoscopy, and capsule endoscopy before treatment. Nineteen patients (5.4%) had Lynch syndrome, and 272 patients (76.8%) had symptoms at the initial diagnosis. The rates for stages 0, I, II, III, and IV were 5.4%, 2.5%, 27.1%, 26.0%, and 35.6%, respectively. The 5-year overall survival rates at each stage were 92.3%, 60.0%, 75.9%, 61.4%, and 25.5%, respectively, and the 5-year disease-specific survival (DSS) rates were 100%, 75.0%, 84.1%, 59.3%, and 25.6%, respectively. Patients with the PSBA located in the jejunum, with symptoms at the initial diagnosis or advanced clinical stage had a worse prognosis. However, multivariate analysis using Cox-hazard model revealed that clinical stage was the only significant predictor of DSS for patients with PSBA.
CONCLUSIONS
Of the patients with PSBA, 76.8% had symptoms at the initial diagnosis, which were often detected at an advanced stage. Detection during the early stages of PSBA is important to ensure a good prognosis.
Topics: Aged; Humans; Male; Adenocarcinoma; Capsule Endoscopy; Duodenal Neoplasms; Ileal Neoplasms; Intestinal Neoplasms; Japan; Jejunal Neoplasms; Prognosis
PubMed: 38411920
DOI: 10.1007/s00535-024-02081-3 -
PloS One 2017While enteric bacteria have been shown to play a critical role in other forms of intestinal damage, their role in mediating the response to the chemotherapeutic drug...
BACKGROUND & AIMS
While enteric bacteria have been shown to play a critical role in other forms of intestinal damage, their role in mediating the response to the chemotherapeutic drug Doxorubicin (Doxo) is unclear. In this study, we used a mouse model of intestinal bacterial depletion to evaluate the role enteric bacteria play in mediating Doxo-induced small intestinal damage and, more specifically, in mediating chemokine expression and leukocyte infiltration following Doxo treatment. An understanding of this pathway may allow for development of intervention strategies to reduce chemotherapy-induced small intestinal damage.
METHODS
Mice were treated with (Abx) or without (NoAbx) oral antibiotics in drinking water for four weeks and then with Doxo. Jejunal tissues were collected at various time points following Doxo treatment and stained and analyzed for apoptosis, crypt damage and restitution, and macrophage and neutrophil number. In addition, RNA expression of inflammatory markers (TNFα, IL1-β, IL-10) and cytokines (CCL2, CC7, KC) was assessed by qRT-PCR.
RESULTS
In NoAbx mice Doxo-induced damage was associated with rapid induction of apoptosis in jejunal crypt epithelium and an increase weight loss and crypt loss. In addition, we observed an increase in immune-modulating chemokines CCL2, CCL7 and KC and infiltration of macrophages and neutrophils. In contrast, while still positive for induction of apoptosis following Doxo treatment, Abx mice showed neither the overall weight loss nor crypt loss seen in NoAbx mice nor the increased chemokine expression and leukocyte infiltration.
CONCLUSION
Enteric bacteria play a critical role in Doxo-induced small intestinal damage and are associated with an increase in immune-modulating chemokines and cells. Manipulation of enteric bacteria or the damage pathway may allow for prevention or treatment of chemotherapy-induced small intestinal damage.
Topics: Animals; Anti-Bacterial Agents; Apoptosis; Doxorubicin; Drug-Related Side Effects and Adverse Reactions; Enterobacteriaceae; Gene Expression Regulation, Neoplastic; Humans; Intestine, Small; Macrophages; Mice; Neoplasm Proteins; Neoplasms; Neutrophils
PubMed: 28257503
DOI: 10.1371/journal.pone.0173429 -
BMC Cancer Aug 2021The efficacy and safety of bevacizumab-containing chemotherapy for patients with metastatic duodenal and jejunal adenocarcinoma (mDJA) are unclear. The present study...
BACKGROUND
The efficacy and safety of bevacizumab-containing chemotherapy for patients with metastatic duodenal and jejunal adenocarcinoma (mDJA) are unclear. The present study aimed to evaluate the efficacy of bevacizumab and to explore immunohistochemical markers that can predict the efficacy of bevacizumab for patients with mDJA.
METHODS
This multicentre study included patients with histologically confirmed small bowel adenocarcinoma who received palliative chemotherapy from 2008 to 2017 at 15 hospitals. Immunostaining was performed for vascular endothelial growth factor-A (VEGF-A), TP53, Ki67, β-catenin, CD10, MUC2, MUC5AC, MUC6, and mismatch repair proteins.
RESULTS
A total of 74 patients were enrolled, including 65 patients with mDJA and 9 with metastatic ileal adenocarcinoma. Patients with mDJA who received platinum-based chemotherapy with bevacizumab as first-line treatment tended to have a longer progression-free survival and overall survival than those treated without bevacizumab (P = 0.075 and 0.077, respectively). Multivariate analysis extracted high VEGF-A expression as a factor prolonging progression-free survival (hazard ratio: 0.52, 95% confidence interval: 0.30-0.91). In mDJA patients with high VEGF-A expression, those who received platinum-based chemotherapy with bevacizumab as a first-line treatment had significantly longer progression-free survival and tended to have longer overall survival than those treated without bevacizumab (P = 0.025 and P = 0.056, respectively), whereas no differences were observed in mDJA patients with low VEGF-A expression.
CONCLUSION
Immunohistochemical expression of VEGF-A is a potentially useful biomarker for predicting the efficacy of bevacizumab-containing chemotherapy for patients with mDJA.
Topics: Adenocarcinoma; Aged; Antineoplastic Combined Chemotherapy Protocols; Bevacizumab; Biomarkers, Tumor; Capecitabine; Duodenal Neoplasms; Female; Follow-Up Studies; Humans; Immunohistochemistry; Jejunal Neoplasms; Leucovorin; Male; Organoplatinum Compounds; Prognosis; Retrospective Studies; Survival Rate; Vascular Endothelial Growth Factor A
PubMed: 34465291
DOI: 10.1186/s12885-021-08724-5