Systemic Chemotherapy for Advanced Rare Pancreatic Histotype Tumors

Brunetti, Oronzo; Aprile, Giuseppe; Marchetti, Paolo; Vasile, Enrico; Casadei-Gardini, Andrea; Scartozzi, Mario; Barni, Sandro; Delfanti, Sara; Vita, F. De; Costanzo, Francesco Di; Milella, Michèle; Cella, Chiara Alessandra; Berardi, Rossana; Cataldo, Ivana; Scarpa, Aldo; Basile, Debora; Mazzuca, Federica; Graziano, Giusi; Argentiero, Antonella; Santini, Daniele; Reni, Michele; Cascinu, Stefano; Silvestris, Nicola

doi:10.1097/mpa.0000000000001063

Cited by 34 publications

(31 citation statements)

References 91 publications

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“…10,11,18) around the body and tail of the pancreas were dissected)this concurs with the previous findings of Suzuki et al 22 The role of neo-adjuvant or adjuvant chemotherapy, and radiotherapy, in the treatment of SPT is limited, 23,24 but Brunetti et al find an interesting result that a patient diagnosed to SPTP with malignant transformation treated with adjuvant chemotherapy achieved a longduration. 25 In the present study, we found that pancreatic fistulas were the predominant complication (n=21 patients); grade A fistulas were found in 19 patients, while grade B fistulas were found in two patients; we did not identify any grade C fistulas. Postoperative bleeding was the most dangerous complication and one of the patients who underwent vascular resections suffered hemorrhage post operation and was cured by conservative treatment such as drainage and hemostatic.…”

Section: Discussioncontrasting

confidence: 45%

<p>Diagnosis and Surgical Treatment and Pathological Findings of Solid Pseudopapillary Tumor of the Pancreas: A Single-Institution Experience</p>

Guo¹,

Wang²,

Xie³

et al. 2020

CMAR

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Purpose: To investigate the diagnosis, surgical treatment, and pathology of solid pseudopapillary tumors of the pancreas in our institution. Patients and Methods: We retrospectively analyzed the demographic details, clinical features, imaging findings, and pathological findings of 87 patients with a confirmed diagnosis of solid pseudopapillary tumors of the pancreas (SPTP) and underwent surgery in Tongji Hospital of Tongji Medical College, over a period of 8 years from 2011 to 2018. Results: Our study involved a total of 87 patients (16 males and 71 females) with a mean age of 31.3±13.1 years (range: 10-61 years). The main compliant was abdominal pain or discomfort (n=49) and the median tumor size was 58.6±31.7 mm (range: 16-156mm). Tumors were located in the head (27 patients, 31%), the neck (13 patients, 15%), and the body and tail (47 patients, 54%). There were no significant differences between the patients in terms of sex, age, or tumor location. Partial pancreatectomy was performed in 79 patients, enucleation in six patients, and total pancreatectomy in two patients. R0 resection was achieved in 86 patients. The postoperative morbidity was 36.8%, and the main complication was pancreatic fistula. Pathological examination and immunohistochemical markers were used to provide a final diagnosis. The main follow-up period was 46 months (range: 13-97 months). At the end of the follow-up period, 86 patients were alive and had not experienced recurrence; one patient was lost to follow-up. Conclusion: The accurate diagnosis of SPTP is vital. Our data showed that surgical resection is safe and associated with low morbidity and mortality rates. Pathological findings can play an important role in diagnosis and long-term survival.

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Section: Discussioncontrasting

confidence: 45%

<p>Diagnosis and Surgical Treatment and Pathological Findings of Solid Pseudopapillary Tumor of the Pancreas: A Single-Institution Experience</p>

Guo¹,

Wang²,

Xie³

et al. 2020

CMAR

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“…Acinar cell carcinoma (ACC) is an extremely rare pancreatic tumor comprising 1%‐2% of pancreatic tumors with 50% of tumors presenting with metastases at the time of diagnosis and those patients with advanced disease (Stage III and IV) having very poor prognosis . Cross‐sectional imaging modalities such as computed tomography and magnetic resonance imaging that are enhanced with contrast agents are often utilized to define the extent of primary lesions and possible metastatic disease .…”

Section: Discussionmentioning

confidence: 99%

Lipase hypersecretion syndrome: A rare cutaneous manifestation of advanced pancreatic acinar cell carcinoma

Nizam

Shah

Rajack

et al. 2020

Clinical Case Reports

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“…Small cohort studies and case reports list a number of regimens with an overall response rate of 23% and a median PFS of 5.6 mo (Al-Hader et al 2017;Yoo et al 2017). Chemotherapeutic substances include gemcitabine, capecitabine, 5-fluoruracil, irinotecan, cisplatin/oxaliplatin, and taxanes (Lowery et al 2011;Al-Hader et al 2017;Yoo et al 2017;Brunetti et al 2018) and also targeted therapies such as erlotinib or panitumumab in KRAS wild-type tumors (Morales et al 2013;Kruger et al 2016). Regarding predictors of treatment response, defects in DNA repair genes such as BRCA1, BRCA2, and PALB2 have been linked to prolonged survival following platinum-based regimens in some patients (Al-Hader et al 2017;Yoo et al 2017).…”

Section: Discussionmentioning

confidence: 99%

Successful BRAF/MEK inhibition in a patient with BRAF^V600E-mutated extrapancreatic acinar cell carcinoma

Busch

Kreutzfeldt

Agaimy

et al. 2020

Cold Spring Harb Mol Case Stud

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Pancreatic acinar cell carcinoma (PAC) is a rare disease with a poor prognosis. Treatment options for metastatic PAC are limited and often follow chemotherapeutic regimens for pancreatic ductal adenocarcinoma. Although recurrent genomic alterations, such as BRAF fusions and defects in genes involved in homologous recombination DNA repair, have been described in PAC, data on the clinical efficacy of molecularly guided, targeted treatment are scarce. Here we describe the case of a 27-yr-old patient with BRAF V600E-mutated PAC who was successfully treated with a combination of BRAF and MEK inhibitors. The patient presented to our clinic with abdominal pain and weight loss. Imaging showed extensive retroperitoneal disease as well as mediastinal lymphadenopathy. Because of elevated α-fetoprotein (AFP) levels and inconclusive histologic findings, a germ cell tumor was suspected; however, PEI chemotherapy was unsuccessful. A repeat biopsy yielded the diagnosis of PAC and treatment with FOLFIRINOX was initiated. Comprehensive molecular profiling within the MASTER (Molecularly Aided Stratification for Tumor Eradication Research) precision oncology program revealed a somatic BRAF V600E mutation and a germline PALB2 stop-gain mutation. Therapy was therefore switched to BRAF/MEK inhibition, resulting in almost complete remission and disease control for 12 mo and a remarkable improvement in the patient's general condition. These results indicate that BRAF alterations are a valid therapeutic target in PAC that should be routinely assessed in this patient population.

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Systemic Chemotherapy for Advanced Rare Pancreatic Histotype Tumors

Abstract: Because of the lack of clinical trials in patients affected by these rare pancreatic histotypes, only their molecular classification would help clinicians in future therapeutic choice.

Cited by 34 publications

References 91 publications

<p>Diagnosis and Surgical Treatment and Pathological Findings of Solid Pseudopapillary Tumor of the Pancreas: A Single-Institution Experience</p>

<p>Diagnosis and Surgical Treatment and Pathological Findings of Solid Pseudopapillary Tumor of the Pancreas: A Single-Institution Experience</p>

Lipase hypersecretion syndrome: A rare cutaneous manifestation of advanced pancreatic acinar cell carcinoma

Successful BRAF/MEK inhibition in a patient with BRAF^V600E-mutated extrapancreatic acinar cell carcinoma

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Systemic Chemotherapy for Advanced Rare Pancreatic Histotype Tumors

Abstract: Because of the lack of clinical trials in patients affected by these rare pancreatic histotypes, only their molecular classification would help clinicians in future therapeutic choice.

Cited by 34 publications

References 91 publications

<p>Diagnosis and Surgical Treatment and Pathological Findings of Solid Pseudopapillary Tumor of the Pancreas: A Single-Institution Experience</p>

<p>Diagnosis and Surgical Treatment and Pathological Findings of Solid Pseudopapillary Tumor of the Pancreas: A Single-Institution Experience</p>

Lipase hypersecretion syndrome: A rare cutaneous manifestation of advanced pancreatic acinar cell carcinoma

Successful BRAF/MEK inhibition in a patient with BRAFV600E-mutated extrapancreatic acinar cell carcinoma

Contact Info

Product

Resources

About

Successful BRAF/MEK inhibition in a patient with BRAF^V600E-mutated extrapancreatic acinar cell carcinoma