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Background: Leiomyosarcoma (LMS) originating from the adrenal gland is exceedingly rare, constituting a minute fraction of soft tissue sarcomas. Due to its rarity, with less than 50 documented cases in English medical literature, the diagnosis and management of adrenal LMS remain challenging. The aim of this study was to perform a review of the literature, in order to evaluate the prognosis of these rare cancers and report our specific case. Methods: A systematic review of the literature was conducted using PubMed, Web of Science, Google Scholar, and Scopus databases, up to December 2020. The search utilized MeSH terms such as "Adrenal Gland Neoplasms," "Leiomyosarcoma," "Adrenalectomy," and "Smooth Muscle Tumor." The inclusion criteria focused on studies reporting patients with a histopathological diagnosis of adrenal leiomyosarcoma. The PRISMA guidelines were followed to ensure a comprehensive analysis. Results: Out of 63 identified studies, 43 met the inclusion criteria and were reviewed. These studies highlighted the rarity and aggressive behavior of adrenal leiomyosarcoma. Surgical excision remains the cornerstone of treatment, often complemented by adjuvant therapies. The reviewed case involved a 52-year-old woman who underwent a right laparoscopic adrenalectomy for a 9 × 7 × 6 cm grade 3 leiomyosarcoma. Despite subsequent adjuvant chemotherapy, hepatic metastases were detected, illustrating the aggressive nature of the disease. The literature underscores the importance of histopathological analysis and long-term surveillance for managing disease progression. Conclusions: Optimal management of adrenal leiomyosarcoma requires a multidisciplinary approach and meticulous follow-up. The rarity of the disease poses challenges for standardizing treatment, but surgical excision and tailored adjuvant therapies show promise. Further research is essential to refine treatment strategies and improve prognosis for this rare malignancy. [ABSTRACT FROM AUTHOR]

Background: Pancreatic neuroendocrine tumors (pNETs) represent a rare subset of pancreatic cancer. Functional tumors cause hormonal changes and clinical syndromes, while non-functional ones are often diagnosed late. Surgical management needs multidisciplinary planning, involving enucleation, distal pancreatectomy with or without spleen preservation, central pancreatectomy, pancreaticoduodenectomy or total pancreatectomy. Minimally invasive approaches have increased in the last decade compared to the open technique. The aim of this study was to analyze the current diagnostic and surgical trends for pNETs, to identify better interventions and their outcomes. Methods: The study adhered to the PRISMA guidelines, conducting a systematic review of the literature from May 2008 to March 2022 across multiple databases. Several combinations of keywords were used ("NET", "pancreatic", "surgery", "laparoscopic", "minimally invasive", "robotic", "enucleation", "parenchyma sparing") and relevant article references were manually checked. The manuscript quality was evaluated. Results: The study screened 3867 manuscripts and twelve studies were selected, primarily from Italy, the United States, and China. A total of 7767 surgically treated patients were collected from 160 included centers. The mean age was 56.3 y.o. Enucleation (EN) and distal pancreatectomy (DP) were the most commonly performed surgeries and represented 43.4% and 38.6% of the total interventions, respectively. Pancreatic fistulae, postoperative bleeding, re-operation, and follow-up were recorded and analyzed. Conclusions: Enucleation shows better postoperative outcomes and lower mortality rates compared to pancreaticoduodenectomy (PD) or distal pancreatectomy (DP), despite the similar risks of postoperative pancreatic fistulae (POPF). DP is preferred over enucleation for the pancreas body–tail, while laparoscopic enucleation is better for head pNETs. [ABSTRACT FROM AUTHOR]