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Medicine Jan 2023Type 1 neurofibromatosis (NF1) is one of the most prevalent genetic conditions. NF1 is characterized by cutaneous plexiform neurofibromas and café au lait skin... (Review)
Review
Synchronous cutaneous malignant peripheral nerve sheath tumor and jejunal gastrointestinal stromal tumor and submucosal angiomyolipoma in type 1 neurofibromatosis: A case report and literature review.
RATIONALE
Type 1 neurofibromatosis (NF1) is one of the most prevalent genetic conditions. NF1 is characterized by cutaneous plexiform neurofibromas and café au lait skin pigmentation, and is inherited in an autosomal dominant trait with mutation in the neurofibromin 1 gene on chromosome 17. Neurofibromin is involved in Ras proto-oncogene regulation. Accordingly, NF1 may lead to malignancies, with a lifetime cancer risk of 60%. Malignant peripheral nerve sheath tumor (MPNST) is the leading cause of mortality due to NF1. The relevance of gastrointestinal stromal tumor (GIST) in NF1 is increasingly being reported in the literature and NF1-associated GIST has been identified to have an alternative molecular pathogenesis.
PATIENT CONCERNS
A 62-years-old female had a 7 × 5 cm growing back mass in the background of various sized cutaneous neurofibromas with café au lait spots. Computed tomography performed in the workup revealed a 4.1 cm enhancing mass near the ileal mesentery.
DIAGNOSES
NF1 affected by cutaneous MPNST of the back, and synchronous GIST and submucosal angiomyolipoma (AML) of the jejunum.
INTERVENTIONS
The patient underwent laparoscopic jejunal mass excision, and excision and flap coverage for the back mass owing to the suspicion of multiple MPNSTs. However, the abdominal masses were diagnosed as GIST and AML following confirmation of the immunohistochemical profiles. Accordingly, the patient was administered adjuvant radiotherapy to the MPNST after surgery.
OUTCOMES
Symptomatic improvements were achieved, and no subsequent relapses were observed.
LESSONS
Although MPNST and GIST are not rare neoplasm in NF1, only 2 case reports have been published on the synchronous occurrence of these tumors. Moreover, no case report has been published on AML in NF1, except 1 renal AML in segmental neurofibromatosis. Identifying the clinical and pathologic significances of the NF1 is important to achieve improved diagnostic accuracy.
Topics: Female; Humans; Middle Aged; Neurofibromatosis 1; Neurofibrosarcoma; Gastrointestinal Stromal Tumors; Jejunum; Angiomyolipoma; Neurofibromin 1; Neurofibroma; Skin Neoplasms; Hamartoma; Leukemia, Myeloid, Acute
PubMed: 36701730
DOI: 10.1097/MD.0000000000032696 -
International Journal of Surgery Case... Jan 2022Choriocarcinoma is form of malignant neoplasm that arise from trophoblastic cells that occurs mostly in ovaries and testes and it can metastasis to lungs, liver or to...
INTRODUCTION
Choriocarcinoma is form of malignant neoplasm that arise from trophoblastic cells that occurs mostly in ovaries and testes and it can metastasis to lungs, liver or to gastrointestinal tract.
PRESENTATION OF CASE
This is 37 years old female presented as a case of lower gi bleeding and was diagnosed to have metastatic jejunal and ilium choriocarcinoma of unknown primary and underwent ileocecal resection first then followed up with small bowel resection around 60 cm from duodenojejunal junction with primary anastomosis. The patient was found to have liver and brain metastasis and received chemotherapy with full response.
DISCUSSION
Choriocarcinoma is the most aggressive form of gestational trophoblastic disease that metastasizes through the lymphatic and hematogenous routes, and when its metastasis to the small bowel it's considered the worst prognosis with high mortality rate. Treatment consists of surgery and combined chemotherapy which is what our patient responded with.
CONCLUSION
Metastatic choriocarcinoma with unknown primary can be treated surgically with chemotherapy.
PubMed: 34952309
DOI: 10.1016/j.ijscr.2021.106636 -
Surgical Endoscopy May 2022Anastomotic leakage (AL) after gastrectomy in gastric cancer patients is associated with high mortality rates. Various endoscopic procedures are available to manage this...
BACKGROUND
Anastomotic leakage (AL) after gastrectomy in gastric cancer patients is associated with high mortality rates. Various endoscopic procedures are available to manage this postoperative complication. The aim of study was to evaluate the outcome of two endoscopic modalities, clippings and stents, for the treatment of AL.
PATIENTS AND METHODS
There were 4916 gastric cancer patients who underwent gastrectomy between December 2007 and January 2016 at the National Cancer Center, Korea. A total of 115 patients (2.3%) developed AL. Of these, 85 patients (1.7%) received endoscopic therapy for AL and were included in this retrospective study. The endpoints were the complete leakage closure rates and risk factors associated with failure of endoscopic therapy.
RESULTS
Of the 85 patients, 62 received endoscopic clippings (with or without detachable snares), and 23 received a stent insertion. Overall, the complete leakage closure rate was 80%, and no significant difference was found between the clipping and stent groups (79.0% vs. 82.6%, respectively; P = 0.89). The complete leakage closure rate was significantly lower in the duodenal and jejunal stump sites (60%) than esophageal sites (86.1%) and gastric sites (94.1%; P = 0.026). The multivariate analysis showed that stump leakage sites (adjusted odds ratio [aOR], 4.51; P = 0.031) and the presence of intra-abdominal abscess (aOR, 4.92; P = -0.025) were associated with unsuccessful leakage closures.
CONCLUSIONS
Endoscopic therapy using clippings or stents is an effective method for the postoperative management of AL in gastric cancer patients. This therapy can be considered a primary treatment option due to its demonstrated efficacy, safety, and minimally invasive nature.
Topics: Anastomotic Leak; Endoscopy; Gastrectomy; Humans; Retrospective Studies; Stents; Stomach Neoplasms; Treatment Outcome
PubMed: 34254185
DOI: 10.1007/s00464-021-08582-z -
Asian Journal of Surgery Jan 2021The incidence of proximal gastric cancer has been increasing continuously. This status has prevailed despite the application of laparoscopic proximal gastrectomy as a... (Review)
Review
The incidence of proximal gastric cancer has been increasing continuously. This status has prevailed despite the application of laparoscopic proximal gastrectomy as a surgical treatment for early proximal gastric cancer. The widespread adoption and standardization of this surgical procedure as the primary treatment for the abovementioned cancer has been hampered by the lack of consensus on the optimal reconstruction method after proximal gastrectomy. In addition, the oncological safety of proximal gastrectomy for advanced gastric disease remains unclear. We reviewed the English-language literature to clarify the current status of laparoscopic proximal gastrectomy in proximal gastric cancer. Japanese gastric cancer guidelines have suggested three types of reconstructions for proximal gastrectomy, namely, esophagogastrostomy, double-tract reconstruction, and jejunal interposition. Optimal reconstruction methods remain to be determined because of the lack of adequately performed and well-designed randomized controlled trials. The technical complexity and challenging implementation of reconstruction procedures have resulted in several complications with anastomoses. Multicenter randomized controlled trials are necessary to evaluate the various reconstruction methods and the oncological safety of laparoscopic proximal gastrectomy for advanced gastric disease.
Topics: Gastrectomy; Humans; Laparoscopy; Plastic Surgery Procedures; Recovery of Function; Safety; Stomach Neoplasms; Treatment Outcome
PubMed: 32981822
DOI: 10.1016/j.asjsur.2020.09.006 -
Pancreas Oct 2021Diabetes mellitus (DM) is associated with an increased risk of gastroenteropancreatic neuroendocrine tumors (GEP-NETs), but the association between DM and GEP-NET...
OBJECTIVES
Diabetes mellitus (DM) is associated with an increased risk of gastroenteropancreatic neuroendocrine tumors (GEP-NETs), but the association between DM and GEP-NET survival is unknown. We evaluated disease characteristics and survival in individuals with DM and GEP-NETs.
METHODS
Using the Surveillance, Epidemiology, and End Results registry linked to Medicare (SEER-Medicare) claims database, we examined sociodemographics, GEP-NET characteristics, and treatment in patients with and without DM before GEP-NET diagnosis. We compared survival using univariate and multivariate analyses.
RESULTS
We identified 1858 individuals with GEP-NETs: 478 (25.7%) with DM and 1380 (74.3%) without. Significant differences in race (P = 0.002) were found between the DM and non-DM groups. Compared with individuals without DM, those with DM had more gastric (9.7% vs 14.9%), duodenal (6.5% vs 10.0%), and pancreatic (17.0% vs 21.8%), and less jejunal/ileal (18.1% vs 12.8%) NETs (P < 0.0001). Patients with DM had earlier stages (stage I, 37.0%; stage IV, 30.8%) than those without (stage I, 30.6%; stage IV, 36.4%; P = 0.0012). We found no difference in survival (multivariate hazard ratio, 0.97; 95% confidence interval, 0.76-1.23) between groups.
CONCLUSIONS
Among patients with and without DM before GEP-NET diagnosis, we found differences in tumor location and stage, but not survival.
Topics: Aged; Aged, 80 and over; Comorbidity; Diabetes Mellitus; Female; Humans; Intestinal Neoplasms; Kaplan-Meier Estimate; Male; Medicare; Multivariate Analysis; Neuroendocrine Tumors; Pancreatic Neoplasms; Prognosis; Registries; SEER Program; Stomach Neoplasms; United States
PubMed: 34860814
DOI: 10.1097/MPA.0000000000001911 -
BMC Surgery Oct 2014Free jejunal interposition is a useful technique for reconstruction of the cervical esophagus. However, the distal anastomosis between the graft and the remaining... (Review)
Review
BACKGROUND
Free jejunal interposition is a useful technique for reconstruction of the cervical esophagus. However, the distal anastomosis between the graft and the remaining thoracic esophagus or a gastric conduit can be technically challenging when located very low in the thoracic aperture. We here describe a modified technique for retrograde stapling of a jejunal graft to a failed gastric conduit using a circular stapler on a delivery system.
CASE PRESENTATION
A 56 year-old patient had been referred for esophageal squamous cell carcinoma at 20 cm from the incisors. On day 8 after thoracoabdominal esophagectomy with gastric pull-up, an anastomotic leakage was diagnosed. A proximal-release stent was successfully placed by gastroscopy and the patient was discharged. Two weeks later, an esophagotracheal fistula occurred proximal to the esophageal stent. Cervical esophagostomy was performed with cranial closure of the gastric conduit, which was left in situ within the right hemithorax. Three months later, reconstruction was performed using a free jejunal interposition. The anvil of a circular stapler (Orvil®, Covidien) was placed transabdominally through an endoscopic rendez-vous procedure into the gastric conduit. A free jejunal graft was retrogradely stapled to the proximal end of the conduit. Microvascular anastomoses were performed subsequently. The proximal anastomosis of the conduit was completed manually after reperfusion.
CONCLUSIONS
This modified technique allows stapling of a jejunal interposition graft located deep in the thoracic aperture and is therefore a useful method that may help to avoid reconstruction by colonic pull-up and thoracotomy.
Topics: Anastomosis, Surgical; Carcinoma, Squamous Cell; Esophageal Neoplasms; Esophageal Squamous Cell Carcinoma; Esophagectomy; Esophagus; Humans; Jejunum; Male; Middle Aged; Surgical Stapling
PubMed: 25319372
DOI: 10.1186/1471-2482-14-78 -
Acta Clinica Croatica Dec 2018- There are several options for hypopharyngeal reconstruction depending on defect size. Reconstructive options include primary closure, local flaps, regional axial flaps...
- There are several options for hypopharyngeal reconstruction depending on defect size. Reconstructive options include primary closure, local flaps, regional axial flaps or regional intestinal flaps, and free flap transfer with skin or intestinal free flaps. The preferred method of reconstruction should minimize early postoperative complications that prolong hospital stay and/or become life threatening, ensure early restoration of function and decrease donor site morbidity. The purpose of this study was to evaluate functional outcomes of different flap reconstruction methods in type II hypopharyngeal defects. In this non-randomized retrospective cohort study, data on 31 (27 male and four female) patients were collected over a 10-year period of single institution type II hypopharyngeal defect reconstructions. The following measures of functional outcome were extracted from patient medical histories: postoperative complications (flap failure, fistula formation, donor site related complications), hospital stay in days and swallowing function after 14 days, 1 month and 6 months. There were nine patients in the radial forearm free flap (RFFF) reconstruction group, seven in the jejunum reconstruction group, and 15 in the gastric tube reconstruction group. In the RFFF group, three patients experienced flap failure; in the jejunal transfer group, no donor site morbidity was observed; whereas three patients from the gastric tube reconstruction group had minor abdominal skin wound dehiscence. Out of the 3 different reconstructive methods, RFFF was most likely to fail. The mean duration of hospital stay was 22.6 days, being shortest in the RFFF group. There were no significant differences in early postoperative swallowing function among the groups. The choice of flap used for hypopharynx reconstruction should be driven by donor site factors and functional outcomes. When assessing type II hypopharyngeal defect reconstruction results, the findings of this study suggest that free jejunal flaps and gastric tubes offer superior functional results in comparison with RFFFs.
Topics: Adult; Aged; Cohort Studies; Female; Free Tissue Flaps; Humans; Hypopharyngeal Neoplasms; Hypopharynx; Jejunum; Male; Middle Aged; Retrospective Studies
PubMed: 31168205
DOI: 10.20471/acc.2018.57.04.10 -
Digestive Surgery 2023A systematic review and meta-analysis of the literature was carried out to determine the clinical and oncological outcome of patients who had enucleation of solitary... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
A systematic review and meta-analysis of the literature was carried out to determine the clinical and oncological outcome of patients who had enucleation of solitary pancreatic metastases from renal cell carcinoma.
METHODS
Operative mortality, postoperative complications, observed survival, and disease-free survival were analyzed. The clinical outcomes of patients who had enucleation were compared to those of 947 patients collected from the literature who had standard or atypical pancreatic resection for the same disease using propensity score matching.
RESULTS
There was no postoperative mortality in the 56 patients who had enucleation of pancreatic metastases from renal cell carcinoma. In 51 patients, postoperative complications could be analyzed. Ten patients (10/51 = 19.6%) had postoperative complications. Three patients (3/51 = 5.9%) had major complications (Clavien-Dindo III or more). Five-year observed survival rates and disease-free survival for patients with enucleation were 92% and 79%, respectively. These results compared favorably with those obtained in patients who had standard resection and other forms of atypical resection (also using propensity score matching). Patients who had partial pancreatic resection (atypical or not) with pancreatic-jejunal anastomosis had increased rates of postoperative complications and local recurrences.
CONCLUSIONS
Enucleation of pancreatic metastases offers a valid solution in selected patients.
Topics: Humans; Carcinoma, Renal Cell; Pancreatic Neoplasms; Pancreas; Pancreatectomy; Postoperative Complications; Kidney Neoplasms; Retrospective Studies; Treatment Outcome
PubMed: 36809760
DOI: 10.1159/000528823 -
Annals of Hepato-biliary-pancreatic... May 2022Pancreaticoduodenectomy is the most common procedure for the management of duodenal pathologies. However, it is associated with substantial morbidity and a low risk of...
BACKGROUNDS/AIMS
Pancreaticoduodenectomy is the most common procedure for the management of duodenal pathologies. However, it is associated with substantial morbidity and a low risk of mortality. Pancreas-preserving limited duodenal resection (PPLDR) can be performed under specific scenarios. We share our experience with PPLDR and its outcome.
METHODS
We retrospectively analyzed a prospectively maintained database of patients undergoing limited duodenal resection in the form of wedge (sleeve) resection or segmental resection of one or more duodenal segments from March 2016 to March 2021 at a tertiary care center in North India.
RESULTS
During the study period, 10 patients (including 9 males) underwent PPLDR. Five of these 10 patients showed primary duodenal or proximal jejunal pathology, while the remaining five had duodenal pathology involving an adjacent organ tumor. Four patients underwent wedge (sleeve) resection, while the remaining six underwent segmental duodenal resection of one or more duodenal segments. Mean hospital stay was 6 days (range, 3-11 days) without 30-day mortality. Morbidity occurred in 4 patients (Grade I-II, n = 3; Grade III, n = 1). All patients were alive and disease-free at the time of last follow-up. The mean follow-up duration was 23 months (range, 2-48 months).
CONCLUSIONS
PPLDR is a safe and effective alternative for pancreaticoduodenectomy when selected carefully for specific tumor types and location.
PubMed: 35168204
DOI: 10.14701/ahbps.21-124 -
International Journal of Surgery Case... May 2023A small bowel gastrointestinal stromal tumor (GIST) is a rare neoplasm of the gastrointestinal tract. The manifestation of bleeding is a diagnostic challenge and could...
INTRODUCTION
A small bowel gastrointestinal stromal tumor (GIST) is a rare neoplasm of the gastrointestinal tract. The manifestation of bleeding is a diagnostic challenge and could present as a life-threatening situation that needs urgent intervention.
PRESENTATION OF CASE
64-year-old woman consulted for episodes of melena and anemia. The upper and lower endoscopies were not diagnostic. Capsule endoscopy (CE) revealed a probable jejunal hemangioma, however double-balloon enteroscopy and magnetic resonance imaging (MRI) did not show any intestinal nodule but MRI show a pelvic mass apparently related to the uterus confirmed by a gynecologist. Even so, the patient returned with melena, and a contrast-enhanced computed tomography (CT) scan again identified a pelvic mass, highlighting that its vascularization drained into the superior mesenteric territory and seemed to invade the jejunum, with active bleeding, suspicious for jejunal GIST. A laparotomy was performed to remove the jejunal mass. Histopathology and immunohistochemical studies confirmed the diagnosis.
DISCUSSION
Bleeding is a common symptom in small bowel GISTs but its diagnoses could be difficult because its location. In most cases, gastroscopy and colonoscopy are not useful and CE or imaging studies are necessary to find the cause of bleeding. Moreover, it has recently proved that bleeding is a prognostic risk factor because it is related to tumor rupture and tumor invasion of blood vessels.
CONCLUSION
In this case, bleeding caused by small bowel GIST was misdiagnosed in endoscopic procedures and the clinical management was delayed. CT angiography was the most effective investigation to detect the source of bleeding.
PubMed: 37137174
DOI: 10.1016/j.ijscr.2023.108257