Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) are a heterogeneous subgroup of rare neoplasms that represent about a third of all poorly differentiated neuroendocrine carcinomas (PDNEC). MiNEN combine a neuroendocrine component, usually a PDNEC, and a non-neuroendocrine component, generally an adenocarcinoma, both accounting for at least 30% of the neoplasm. MiNEN are classified as high-, intermediate-, or low-grade malignancies depending on the metastatic potential of the tumour components. High-grade malignant component should be considered even if it represents <30% of the tumour. The prognosis of MiNEN is generally intermediate between those of the two “pure” components composing it. The aim of this comprehensive review of the literature is to suggest a standardized management of MiNEN. An increasing body of evidence suggests that PDNEC components share molecular abnormalities with their adenocarcinoma counterparts, but also display additional alterations. This advocates for a common origin, and that the presence of a PDNEC component in an adenocarcinoma could indicate a turning point in carcinogenesis.

Objectives Acinar cell carcinoma is a rare tumor of the pancreas. Our current series aimed to assess the clinical and morphological features of pancreatic acinar cell carcinoma and to evaluate the treatment strategies and prognosis. Methods This retrospective study was conducted in 3 French referral centers. Clinical data were obtained from medical records, and data about survival were then calculated and compared using statistical analysis. Results Forty-four patients were included (men, 81.8%; median age, 65.5 years; range, 21–85). Tumors were localized, locally advanced, or metastatic in 48.8%, 14.0%, and 37.2% of cases, respectively. Twenty-nine patients (65.9%) underwent a curative-intent resection (R0, 79.2%). First-line chemotherapy in metastatic patients was heterogeneous but mainly consisted in 5-fluorouracil-based or gemcitabine plus oxaliplatin combinations. Median disease-free survival was 12 months (range, 0–82 months). Median overall survival was 55.5 months; it was 40 months in patients with metastatic tumor compared with 106.5 months (P = 0.1058) in those with a nonmetastatic one. Age older than 60 years and a proliferation index greater than 30% were poor prognostic factors. Conclusions In this large series of patients with pancreatic acinar cell carcinoma, the rate of R0 resection and the prognosis of patients appeared to be much better than that of classic ductal adenocarcinomas.

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PurposePrimary anal actinomycosis of cryptoglandular origin, a rare cause of anal suppurative disease, requires specific management to be cured. The aims of this retrospective study were to describe clinical, morphological, and microbiological features of this entity and to evaluate management practices for new cases observed since 2001.MethodsThis was a retrospective case series conducted at the Diaconesses-Croix Saint-Simon Hospital in Paris.ResultsFrom January 2001 to July 2016, 7 patients, 6 males and 1 female (median, 49 years), presenting with an actinomycotic abscess with a cryptoglandular anal fistula were included for study. The main symptom was an acute painful ischioanal abscess. One patient exhibited macroscopic small yellow granules (“sulfur granules”), another “watery pus” and a third subcutaneous gluteal septic metastasis. All patients were overweight (body mass index ≥ 25 kg/m2). Histological study of surgically excised tissue established the diagnosis. All the patients were managed with a combination of classical surgical treatment and prolonged antibiotic therapy. No recurrence was observed during follow-up, the median follow-up being 3 years.ConclusionActinomycosis should be suspected particularly when sulfur granules are present in the pus, patients have undergone multiple surgeries or suppuration has an unusual aspect. Careful histological examination and appropriate cultures of pus are needed to achieve complete eradication of this rare, but easily curable, disease.