Dermatofibrosarcoma protuberans (DFSP) is an aggressive tumour with multiple local recurrences and rare metastatic potential. Fibrosarcomatous transformation occurs in a few cases of DFSP which makes them more aggressive in terms of recurrence and metastasis. Here we report the case of a woman in her late 30s who presented with massive lower gastrointestinal (GI) bleeding with a history of multiple surgeries for DFSP on her anterior abdominal wall. The bleeding source was identified to be a mass lesion in the jejunum, which was excised. The patient recovered well and the histopathology revealed fibrosarcoma of the jejunum. Follow-up investigations showed multiple lung nodules, ascites and abdominal lymph nodes suggesting progressive disease. She is currently receiving chemotherapy and progressing well 3 months postoperatively. Patients with fibrosarcomatous changes within DFSP must be followed up closely as it is associated with increased metastatic potential.

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A case describes a 49-year-old male patient who underwent emergency exploratory laparotomy for small intestinal perforation. Peritonitis was present due to perforation of the jejunal tumor. Resection of the jejunal tumor with perforation was performed followed by end-to-end anastomosis of the jejunum. The resected jejunal tumor was identified in the histopathological examination as metastatic from a clear cell variant of squamous cell/large cell carcinoma of the lung. It was associated with metastatic lesions in the brain. Metastasis from the lung carcinoma in the jejunum is a very rare condition predisposing to small intestinal perforation which is also associated with brain metastasis.

BACKGROUND Schwannomas are tumors that arise from Schwann cells that surround and support nerve cells. Most common sites for presentations are head, neck, and extremities. Schwannomas of gastrointestinal tract are rare, slow-growing tumors, usually benign, arising from gastrointestinal tract\'s neural plexus. They are histologically distinguishable from conventional schwannomas that arise in soft tissue or the central nervous system. Preoperative diagnosis of gastrointestinal schwannoma is challenging, requiring immunohistological confirmation of the nature of the tumor. Here, we report a case of 57-year-old woman with an incidental finding of an asymptomatic submucosal jejunal schwannoma. CASE REPORT A 57-year-old woman with a medical history of hematological disorder underwent a contrast abdominal computed tomography as part of medical follow-up. The imaging revealed the presence of a jejunal mass. The patient underwent laparoscopic surgical resection of the lesion, followed by side-to-side jejuno-jejunal anastomosis with 4-cm clear surgical margins. The final pathologic study revealed the presence of jejunal schwannoma, as tested positive for S-100 protein. The patient was discharged home on the fourth postoperative day, having an uneventful recovery. CONCLUSIONS Jejunal schwannoma are usually benign and asymptomatic, and they are often discovered incidentally during diagnostic tests for other conditions; therefore, it should be included in the differential diagnosis of gastrointestinal tumors. Surgical treatment appears to be necessary to achieve a definitive diagnosis through a biopsy of the tumor tissue. Benign jejunal schwannomas have a good prognosis.

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The clinicopathological features and prognosis of primary small bowel adenocarcinoma (PSBA), excluding duodenal cancer, remain undetermined due to its rarity in Japan.
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.
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.
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.

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BACKGROUND: Small intestinal adenocarcinomas (SIAs) are rare. Hence, randomized controlled trials are lacking and understanding of the disease features is limited. This nationwide cohort investigates incidence, treatment and prognosis of SIA patients, to improve disease outcome.
METHODS: Data of 2697 SIA patients diagnosed from January 1999 through December 2019 were retrieved from the Netherlands Cancer Registry and Pathology Archive. Incidence was calculated using the revised European Standardized Rate. The influence of patient and tumor characteristics on overall survival (OS) was studied using survival analyses.
RESULTS: The age-standardized incidence rate almost doubled from 0.58 to 1.06 per 100,000 person-years, exclusively caused by an increase in duodenal adenocarcinomas. OS did not improve over time. Independent factors for a better OS were a younger age, jejunal tumors, Lynch syndrome and systemic therapy. Only 13.8% of resected patients was treated with adjuvant chemotherapy, which improved OS compared to surgery alone in stage III disease (HR 0.47 (0.35-0.61)), but not in the limited group of deficient mismatch repair (MMR) patients (n = 53, HR 0.93 (0.25-3.47)). In the first-line setting, CAPOX was associated with improved OS compared to FOLFOX (HR 0.51 (0.36-0.72)). For oligometastatic patients, a metastasectomy significantly improved OS (HR 0.54 (0.36-0.80)).
CONCLUSIONS: The incidence of SIAs almost doubled in the past 20 years, with no improvement in OS. This retrospective non-randomized study suggests the use of adjuvant chemotherapy for stage III disease and first-line CAPOX for metastatic patients. For selected oligometastatic patients, a metastasectomy may be considered. MMR-status testing could aid in clinical decision-making.

UNASSIGNED: Locally advanced jejunal stromal tumors stand as a captivating and relatively rare entity, garnering attention for several reasons. Their inaccessible location by conventional endoscopy poses a diagnostic challenge. Further, treatment decisions necessitate a multidisciplinary approach, compounded by the absence of high-level evidence studies.
METHODS: A 54-year-old patient was admitted to our surgical department with abdominal pain and chronic anemia. Abdominal CT imaging confirmed the presence of a non-metastatic sizable jejunal tumor. The patient underwent laparotomy, revealing a locally advanced jejunal tumor contracting the ileum and the ascending colon. A monobloc oncological resection was performed, followed by the restoration of digestive continuity. Anatomopathological analysis delineated a locally advanced Stromal Tumor with a high risk of recurrence. The patient underwent a course of tyrosine kinase inhibitors for 3 years, with no reported recurrence during the subsequent 3-year follow-up.
CONCLUSIONS: Locally advanced jejunal stromal tumors are rare. Most patients present with unspecific symptoms. Diagnosis remains challenging due to their intricate anatomical location. Decisions regarding management must be deliberated within a multidisciplinary framework, tailored to each patient\'s unique characteristics. While combined therapeutic modalities have demonstrated efficacy in recent studies, prudence is advised given the heightened incidence of both short and long-term complications.
CONCLUSIONS: In the absence of randomized controlled trials, the management of locally advanced jejunal stromal tumors underscores the imperative of multidisciplinary collaboration in treatment deliberations. A wide, sometimes mutilating excision is only permissible if it is complete.