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Pancreatic Neoplasms: HELP
Articles by Francesco Tonelli
Based on 9 articles published since 2008
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Between 2008 and 2019, Francesco Tonelli wrote the following 9 articles about Pancreatic Neoplasms.
 
+ Citations + Abstracts
1 Editorial How to follow up and when to operate asymptomatic pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1? 2014

Tonelli, Francesco. ·Department of Surgery and Translational Medicine, University of Florence, Firenze, Italy. ·J Clin Gastroenterol · Pubmed #24714102.

ABSTRACT: -- No abstract --

2 Review Biliary tree gastrinomas in multiple endocrine neoplasia type 1 syndrome. 2013

Tonelli, Francesco / Giudici, Francesco / Nesi, Gabriella / Batignani, Giacomo / Brandi, Maria Luisa. ·Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi, Surgical Unit, Department of Surgery and Translational Medicine, University of Florence, 50014 Florence, Italy. ·World J Gastroenterol · Pubmed #24363522.

ABSTRACT: AIM: To describe our patients affected with ectopic biliary tree gastrinoma and review the literature on this topic. METHODS: Between January 1992 and June 2012, 28 patients affected by duodenopancreatic endocrine tumors in multiple endocrine neoplasia type 1 (MEN1) syndrome underwent surgery at our institution. This retrospective review article analyzes our experience regarding seventeen of these patients subjected to duodenopancreatic surgery for Zollinger-Ellison syndrome (ZES). Surgical treatment consisted of duodenopancreatectomy (DP) or total pancreatectomy (TP). Regional lymphadenectomy was always performed. Any hepatic tumoral lesions found were removed during surgery. In MEN1 patients, removal of duodenal lesions can sometimes lead to persistence or recurrence of hypergastrinemia. One possible explanation for this unfavorable outcome could be unrecognized ectopic localization of gastrin-secreting tumors. This study described three cases among the seventeen patients who were found to have an ectopic gastrinoma located in the biliary tree. RESULTS: Seventeen MEN1 patients affected with ZES were analyzed. The mean age was 40 years. Fifteen patients underwent DP and two TP. On histopathological examination, duodeno pancreatic endocrine tumors were found in all 17 patients. Eighty-one gastrinomas were detected in the first three portions of the duodenum. Only one gastrinoma was found in the pancreas. The mean number of gastrinomas per patient was 5 (range 1-16). Malignancy was established in 12 patients (70.5%) after lymph node, liver and omental metastases were found. Three patients exhibited biliary tree gastrinomas as well as duodenal gastrinoma(s). In two cases, the ectopic gastrinoma was removed at the same time as pancreatic surgery, while in the third case, the biliary tree gastrinoma was resected one year after DP because of recurrence of ZES. CONCLUSION: These findings suggest the importance of checking for the presence of ectopic gastrinomas in the biliary tree in MEN1 patients undergoing ZES surgery.

3 Review Pancreatic endocrine tumors in multiple endocrine neoplasia type 1 syndrome: review of literature. 2011

Tonelli, Francesco / Giudici, Francesco / Fratini, Geri / Brandi, Maria Luisa. ·Department of Clinical Physiopathology, Surgical Unit, University of Florence Medical School, Viale G B Morgagni 85, Florence, Italy. f.tonelli@dfc.unifi.it ·Endocr Pract · Pubmed #21550956.

ABSTRACT: OBJECTIVE: To analyze the surgical approach to multiple endocrine neoplasia type 1 (MEN 1)-related pancreatic endocrine tumors (PETs). METHODS: We reviewed selected publications and our personal experience with MEN 1-associated PETs to delineate their general characteristics, current practice and controversies, preoperative imaging and intraoperative assessment, and appropriate therapeutic strategies including radical surgical procedures. RESULTS: The penetrance of PETs in the setting of MEN 1 is similar to that of parathyroid tumors, even though hyperparathyroidism is usually the first manifestation of MEN 1 syndrome. In contrast with the sporadic counterparts, MEN 1-related PETs are characterized by an early onset, multiplicity of lesions, variable expression of the tumors, and propensity for malignant degeneration. Both the histologic type and the size of these tumors correlate with malignant potential. CONCLUSION: The rationale for surgical considerations for these tumors is to curtail the malignant progression of the disease and to cure or aid in management of the associated biochemical syndromes. A surgical procedure is often the treatment of choice for PETs in patients with MEN 1. Monitoring of pancreatic peptides and use of diagnostic imaging allow an early pancreatic resection, in conjunction with prevention of metastatic PETs and improvement of long-term survival. Hepatic metastatic lesions can be successfully treated by surgical resection.

4 Review Multiple endocrine neoplasms. 2008

Falchetti, Alberto / Marini, Francesca / Luzi, Ettore / Tonelli, Francesco / Brandi, Maria Luisa. ·Surgery Unit, Department of Clinical Physiopathology, University of Florence, 6-50139 Florence, Italy. ·Best Pract Res Clin Rheumatol · Pubmed #18328987.

ABSTRACT: Multiple endocrine neoplasia type 1 (MEN1) and type 2 (MEN2) are rare autosomal-dominant disorders characterized by primary tumours in at least two different endocrine tissues. Both syndromes present as sporadic (a single case with two of the characteristic endocrine tumours) or familial form (an MEN case plus at least one first-degree relative showing one of the characteristic endocrine tumours). MEN1 is characterized by the occurrence of parathyroid, gastro-entero-pancreatic and anterior pituitary tumours, but it can include various combinations of more than 20 endocrine and non-endocrine tumours. Generally, tumours in MEN1 are benign, although gastrinomas and foregut carcinoids may exhibit a malignant course. MEN2 is characterized by medullary thyroid carcinoma (MTC), uni- or bi-lateral pheochromocytoma, and other tumours of different endocrine tissues. If not diagnosed precociously, MTC can be fatal. MEN1 develops after tissue inactivation of both MEN1 gene copies. Activating mutations of c-RET proto-oncogene causes MEN2.

5 Article Natural History of MEN1 GEP-NET: Single-Center Experience After a Long Follow-Up. 2017

Giudici, Francesco / Cavalli, Tiziana / Giusti, Francesca / Gronchi, Giorgio / Batignani, Giacomo / Tonelli, Francesco / Brandi, Maria Luisa. ·Department of Surgery and Translational Medicine, University of Florence, Largo Brambilla 3, 50139, Florence, Italy. francesco.giudici1@gmail.com. · Department of Surgery and Translational Medicine, University of Florence, Largo Brambilla 3, 50139, Florence, Italy. ·World J Surg · Pubmed #28429092.

ABSTRACT: BACKGROUND: The multiple endocrine neoplasia type 1 syndrome (MEN1) natural history is poorly evaluated, and few single-institution experiences about hereditary gastroenteropancreatic neuroendocrine tumors (GEP-NET) are reported. Our purpose is to analyze the role of GEP-NET in MEN1-related death, as well as the behavior of these lesions during follow-up. METHODS: The study population consists of 77 patients diagnosed with MEN1 GEP-NET, regularly followed up since 1990. Extensive clinical data were prospectively recorded. Statistical analysis was performed both on the whole population of 77 patients and on two subgroups including patients who, during the long lasting study period, underwent GEP-NET surgery (50 pts) and who did not (27 pts), respectively. RESULTS: Twenty-five males (32.5%) and 52 females (67.5%) were enrolled. Sixty-four patients had MEN1 family history (83.1%), and genetic mutation was detected in 67 cases (87%). The mean age at GEP-NET diagnosis was 41.4 years (SD = 13.6); 16 patients (20.8%) had GEP-NET diagnosed before age 30 and 12 cases (15.6%) before 1996. The mean interval time between MEN1 diagnosis and GEP-NET detection was 5.7 years (range -11/37; SD = 8.1 years). Overall, the mean follow-up time from MEN1 diagnosis was 15.8 years (SD = 9.7 years) and from GEP-NET diagnosis was 9.6 years (SD = 6.9 years). Gastrinoma was the most frequent functioning GEP-NET and pancreatoduodenectomy the most adopted surgery. GEP-NET progression affected 12 patients within the non-surgical group, while 18 subjects developed progression after surgery. CONCLUSIONS: Our single-center data provide information on epidemiologic, clinical and pathological features of GEP-NET in MEN1 making possible to clarify their natural history.

6 Article Operation for insulinomas in multiple endocrine neoplasia type 1: When pancreatoduodenectomy is appropriate. 2017

Tonelli, Francesco / Giudici, Francesco / Nesi, Gabriella / Batignani, Giacomo / Brandi, Maria Luisa. ·Department of Surgery and Translational Medicine, University of Florence, Florence, Italy. Electronic address: francesco.tonelli@unifi.it. · Department of Surgery and Translational Medicine, University of Florence, Florence, Italy. ·Surgery · Pubmed #27863775.

ABSTRACT: BACKGROUND: Distal pancreatectomy is the most frequent operation for insulinomas complicating multiple endocrine neoplasia type 1 insulinoma, although there are conditions for which a different operative approach might be preferable. In this article, we report the operative experience of a referral center for multiple endocrine neoplasia type 1 insulinoma. METHODS: Twelve patients underwent operations between 1992 and 2015: 8 underwent a distal pancreatic resection, and 4 underwent a pancreatoduodenectomy. Enucleation of other macroadenomas present in the remnant pancreas was performed in 9 out of these 12 patients. RESULTS: Operative complications (2 pancreatic fistulas and 2 cases of pancreatitis) occurred in 4 of the 8 distal pancreatic resections. In 1 patient, reoperation was required to resolve the complications of the first operation. At pathologic analysis, multiple insulinomas were found in 5 patients, lymph-nodal metastasis positive for insulin in 2 patients, multiple nonfunctioning pancreatic tumors in all patients, glucagonoma in 4 patients, and gastrinoma in the duodenum or lymph nodes in 4 patients. All the patients were treated successfully for the hypoglycemic/hyperinsulinemic syndrome with no clinical recurrence at a mean follow-up of 85 months (range 4-242 months). Recurrent nonfunctioning pancreatic tumor macroadenomas in the remnant pancreas occurred in only 1 of the 12 patients, and no progression of the gastrinomas was observed. None of the patients developed diabetes mellitus. CONCLUSION: Resection of the most severely affected part of the pancreas, whether left or right, associated with enucleation of concomitant macroadenomas in the preserved pancreas is recommended for the treatment of hypoglycemic/hyperinsulinemic syndrome and to prevent malignant progression of nonfunctioning pancreatic tumors in patients with multiple endocrine neoplasia type 1. If the head of the pancreas is the most affected site and the Zollinger-Ellison syndrome is concomitant, then pancreatoduodenectomy should be preferred over distal pancreatectomy.

7 Article Ventricular fibrillation resulting from electrolyte imbalance reveals vipoma in MEN1 syndrome. 2016

Cavalli, Tiziana / Giudici, Francesco / Santi, Raffaella / Nesi, Gabriella / Brandi, Maria Luisa / Tonelli, Francesco. ·Department of Surgery and Translational Medicine, University of Florence, Largo Brambilla 3, 50139, Florence, Italy. tiziana.cavalli@gmail.com. · Department of Surgery and Translational Medicine, University of Florence, Largo Brambilla 3, 50139, Florence, Italy. ·Fam Cancer · Pubmed #27071757.

ABSTRACT: Sporadic VIPoma is an exceedingly rare tumor with an annual incidence of 1:10 million people worldwide, yet it is described in approximately 5 % of MEN1 patients. The majority of VIPomas are malignant and radical surgery is the best therapeutic option. A 58-year-old man presented with cardiocirculatory arrest due to ventricular fibrillation. The patient had a 3-month history of epigastric pain with diarrhea. After reanimation, laboratory data revealed severe hypokalemia and hypercalcemia. Further investigations showed hyperparathyroidism, left adrenal adenoma and pituitary microprolactinoma and genetic diagnosis of MEN1 syndrome was made. Abdominal computed tomography revealed a 45 × 30 mm mass of the pancreatic head and two hepatic lesions, which proved to be neuroendocrine after 68 Ga PET and needle biopsy. Vasoactive intestinal peptide (VIP) serum level had increased. Subsequently the patient underwent pylorus-preserving pancreaticoduodenectomy and hepatic resection. Intraoperative VIP returned to normal values. Histopathology confirmed a pancreatic VIPoma metastatic to the liver. The postoperative course was unremarkable and the patient is well with no evidence of disease at a 48 months follow-up. Even in case of anusual presentation, when two or more main clinical findings of MEN1 related tumors are present, unrespectively to the presence of MEN1 mutation, MEN1 syndrome should be suspected. Surgery in MEN1 pancreatic neuroendocrine tumors is indicated both to treat symptoms and to avoid oncological progression even in advanced cases.

8 Article Surgical management of insulinomas in multiple endocrine neoplasia type 1. 2012

Giudici, Francesco / Nesi, Gabriella / Brandi, Maria Luisa / Tonelli, Francesco. ·Department of Clinical Physiopathology, Surgical Unit, Medical School, University of Florence, Florence, Italy. ·Pancreas · Pubmed #22228047.

ABSTRACT: OBJECTIVE: This study aimed to evaluate the accuracy of preoperative and intraoperative diagnostic tools and the surgical strategy to obtain cure in multiple endocrine neoplasia type 1 (MEN-1) patients affected with insulinoma. METHODS: Eight MEN-1 patients (1992-2009) were operated on for hypoglycemic crisis. Preoperative tumor localization was carried out. Ultrasound and modification of the insulin/glucose (I/G) ratio were applied intraoperatively. Pancreatic lesions larger than 0.5 cm were removed by resection of the most affected pancreatic region and by enucleation of nodules in least affected regions. RESULTS: Two pancreatoduodenectomies and 6 distal pancreatectomies were performed; enucleation of nodules was necessary in 6 patients. There was no postoperative mortality. At the histopathologic analysis, a mean of 6 macrotumors and of 15.5 microlesions were found. Intraoperative ultrasound proved a sensitivity of 87.5% for detecting pancreatic insulinoma. Decrease in the I/G ratio after resection predicted postoperative outcome in all patients. At a mean follow-up of 81.5 months, all patients were normoglycemic with no evidence of disease recurrence. CONCLUSIONS: Multiple endocrine neoplasia type 1 insulinomas should be considered surgically curable. Pancreatic resection seems preferable to a less radical surgical approach in ensuring higher cure rates. Intraoperative ultrasound and I/G ratio are of value in the assessment of surgical decision and in the evaluation of the surgical cure.

9 Article In vitro effects of oestrogens, antioestrogens and SERMs on pancreatic solid pseudopapillary neoplasm-derived primary cell culture. 2010

Tognarini, Isabella / Tonelli, Francesco / Nesi, Gabriella / Martineti, Valentina / Galli, Gianna / Gozzini, Alessia / Colli, Emanuela / Zonefrati, Roberto / Paglierani, Milena / Marini, Francesca / Sorace, Sabina / Cavalli, Tiziana / Cavalli, Loredana / Tanini, Annalisa / Brandi, Maria Luisa. ·Department of Internal Medicine, University of Florence Medical School, Florence, Italy. ·Cell Oncol · Pubmed #20364070.

ABSTRACT: BACKGROUND: Solid-pseudopapillary neoplasms of the pancreas (SPNs) are uncommon tumours usually frequent in young women. Although the pathogenesis of SPNs is uncertain a potential influence of the sex hormone milieu on the biology of these tumours has been suggested. The controversial expression of oestrogen receptors (ERs) in SPNs, provide a rationale for studying the effects of oestrogenic molecules on SPN development. METHODS: The expression of a large series of hormonal ligands and receptors was evaluated in tissue specimens and in a primary cell culture (SPNC), obtained from a SPN in young female patient. The effects of 17beta-oestradiol (17betaE2), ICI 182,780 and tamoxifen (Tam) on cell replication and growth were examined. RESULTS: We have established SPNC primary line. Immunocytochemical analysis was positive for vimentin, cyclin D1 and beta-catenin and negative for cytokeratin, CD10 and neuroendocrine markers, in line with the immunostaining features of the tumoral tissue. Expression of ERalpha, ERbeta and progesterone mRNAs was demonstrated in SPNC and tumor tissue. A proliferative and antiproliferative action of 17betaE2 and Tam respectively were proved in SPNC. CONCLUSION: In conclusion, we provide the first direct evidence that oestrogenic molecules can influence proliferation of SPNC, offering future strategies in the control of this neoplasia via selective ER modulators.