A Case of Neuroendocrine Neoplasm in the Accessory Papilla of the Duodenum

AbstractPrimary ovarian neuroendocrine neoplasms (Ov-NENs) are infrequent and mainly represented by well-differentiated forms (neuroendocrine tumors — NETs — or carcinoids). Poorly differentiated neuroendocrine carcinomas (Ov-NECs) are exceedingly rare and only few cases have been reported in the literature. A subset of Ov-NECs are admixed with non-neuroendocrine carcinomas, as it occurs in other female genital organs, as well (mostly endometrium and uterine cervix), and may be assimilated to mixed neuroendocrine/non-neuroendocrine neoplasms (MiNENs) described in digestive and extra-digestive sites. Here, we present a case of large cell Ov-NEC admixed with an endometrioid carcinoma of the ovary, arising in the context of ovarian endometriosis, associated with a uterine endometrial atypical hyperplasia (EAH). We performed targeted next-generation sequencing analysis, along with a comprehensive immunohistochemical study and FISH analysis for TP53 locus, separately on the four morphologically distinct lesions (Ov-NEC, endometrioid carcinoma, endometriosis, and EAH). The results of our study identified molecular alterations of cancer-related genes (PIK3CA, CTNNB1, TP53, RB1, ARID1A, and p16), which were present with an increasing gradient from preneoplastic lesions to malignant proliferations, both neuroendocrine and non-neuroendocrine components. In conclusion, our findings underscored that the two neoplastic components of this Ov-MiNEN share a substantially identical molecular profile and they progress from a preexisting ovarian endometriotic lesion, in a patient with a coexisting preneoplastic proliferation of the endometrium, genotypically and phenotypically related to the ovarian neoplasm. Moreover, this study supports the inclusion of MiNEN in the spectrum ovarian and, possibly, of all gynecological NENs, among which they are currently not classified.

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CD56‐ and CD117‐positive neuroendocrine neoplasm presenting with leukemic phase and bone marrow infiltration mimicking acute leukemia

International Journal of Laboratory Hematology ◽

10.1111/ijlh.13578 ◽

2021 ◽

Author(s):

Carlos Bravo‐Perez ◽

Esmeralda García‐Torralba ◽

María J. Lopez‐Poveda ◽

Francisco J. Ortuño

Keyword(s):

Bone Marrow ◽

Acute Leukemia ◽

Bone Marrow Infiltration ◽

Neuroendocrine Neoplasm ◽

Leukemic Phase

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Five‐year outcomes in patients treated with surgery and radiotherapy for primary neuroendocrine neoplasm of the breast

The Breast Journal ◽

10.1111/tbj.14264 ◽

2021 ◽

Author(s):

Pierre Loap ◽

Fatima Laki ◽

Philippe Beuzeboc ◽

Alain Fourquet ◽

Youlia M Kirova

Keyword(s):

Neuroendocrine Neoplasm

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Double-Balloon Enteroscopy in Detecting Small-Bowel Neuroendocrine Neoplasms: A Single-Center Prospective Study

Digestion ◽

10.1159/000511850 ◽

2020 ◽

pp. 1-9

Author(s):

Roberta Elisa Rossi ◽

Luca Elli ◽

Federica Branchi ◽

Dario Conte ◽

Sara Massironi

Keyword(s):

Small Bowel ◽

Neuroendocrine Neoplasm ◽

Unknown Primary ◽

Neuroendocrine Neoplasms ◽

Double Balloon Enteroscopy ◽

Balloon Enteroscopy ◽

Double Balloon ◽

Diagnostic Role ◽

Positive Results

Background and Aim: Small-bowel neuroendocrine neoplasm (sbNEN) diagnosis has improved with double-balloon enteroscopy (DBE). DBE efficacy in the detection of sbNENs is unknown. We aimed to report the experience at a single referral center for NENs. Methods: All consecutive patients with a suspected sbNEN selected for diagnostic DBE were enrolled. Results: Between 2011 and 2016, 25 patients were referred for a suspected sbNEN. In 15/25 patients, a primary NEN was detected outside the small bowel; in 4, NEN was excluded. After extensive workup, 6 patients (4 males, median age 50 years) underwent DBE (3 anterograde, 2 retrograde, and 1 both; median time: 60 min; median insertion 200 cm). DBE was positive in 3 patients: one had an ileal 2-cm NEN G1, one had an ileal 1.3-cm NEN G1, and one had an ileal 1-cm NEN G2, all surgically removed. Of the 3 other patients, one had a metastatic NEN of unknown primary, the other two had small intestinal NENs, both surgically removed (1.6-cm G1 and G2 NEN). DBE showed a sensitivity of 60% and, in absence of false-positive results, a specificity of 100%. Accuracy resulted 67%. No complications were observed. Conclusions: In line with data from the literature, the present series showed that DBE is a safe and effective procedure in the diagnosis of sbNENs. Further studies are needed to better clarify the diagnostic role of DBE in the neuroendocrine tumor setting and its relationship with other techniques.

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A First Case Report of Orbital Extra-Adrenal Paraganglioma in Cat

Veterinary Sciences ◽

10.3390/vetsci8050086 ◽

2021 ◽

Vol 8 (5) ◽

pp. 86

Author(s):

Leonardo Leonardi ◽

Raluca Ioana Rizac ◽

Ilaria Pettinari ◽

Luca Mechelli ◽

Carlo De Feo

Keyword(s):

Parasympathetic Nervous System ◽

Neuron Specific Enolase ◽

Final Diagnosis ◽

Neuroendocrine Cells ◽

Neuroendocrine Neoplasm ◽

Glial Fibrillary Acid Protein ◽

Veterinary Clinic ◽

First Case ◽

The Right ◽

Extra Adrenal Paraganglioma

Paraganglioma is a rare neuroendocrine neoplasm originating from paraganglia and consisting of neuroendocrine cells of the sympathetic and parasympathetic nervous system. Extra-adrenal paraganglioma occurs with a low incidence in both humans and animals. This report presents the first case of paraganglioma in a cat with orbital primary location. An 18-year-old spayed female European domestic shorthair cat of 3.60 kg body weight was evaluated in a private veterinary clinic in Perugia, Italy, for a pronounced exophthalmos of the right eye. The cat underwent surgery for the enucleation of the right eye and of the mass. The biopsy samples of the removed tissue were fixed in 10% buffered neutral formalin for histological and immunohistochemical evaluations. Therefore, specific markers were used for immunohistochemical investigations, such as anti-neuron specific enolase (NSE), anti-synaptophysin, anti-glial fibrillary acid protein, anti-cytokeratin and anti-chromogranin. The results of these investigations allowed establishing the final diagnosis of ocular extra-adrenal paraganglioma of the cat.

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The Value of Alkaline Phosphatase-to-Albumin Ratio in Detecting Synchronous Metastases and Predicting Postoperative Relapses among Patients with Well-Differentiated Pancreatic Neuroendocrine Neoplasms

Journal of Oncology ◽

10.1155/2020/8927531 ◽

2020 ◽

Vol 2020 ◽

pp. 1-8 ◽

Cited By ~ 1

Author(s):

Wentao Zhou ◽

Yuan Fang ◽

Xu Han ◽

Tiantao Kuang ◽

Xuefeng Xu ◽

...

Keyword(s):

Alkaline Phosphatase ◽

Distant Metastasis ◽

Vascular Invasion ◽

Biological Behavior ◽

Neuroendocrine Neoplasm ◽

Albumin Ratio ◽

Pancreatic Neuroendocrine Neoplasm ◽

Synchronous Metastases ◽

Well Differentiated ◽

Synchronous Distant Metastasis

Backgrounds. Pancreatic neuroendocrine neoplasm (pNEN) is a highly heterogeneous entity, presenting widely varied biological behavior as well as long-term prognosis. Reliable biomarkers are urgently needed to make risk stratifications for pNEN patients, which could be beneficial to the development of individualized therapeutic strategy in the clinical practice. Here, we aimed to evaluate the predictive and prognostic roles of serum alkaline phosphatase-to-albumin ratio (APAR) in well-differentiated pNEN patients. Methods. We retrospectively analyzed the pathologically confirmed grade 1/2 pNEN patients, who were originally treated in our hospital from February 2008 to April 2018. Univariate and multivariate analyses were performed to assess the value of APAR in detecting synchronous metastases and predicting relapses following curative resections. Results. A total of 170 eligible cases were included into analysis. Logistic univariate analysis indicated APAR (P=0.002) was significantly associated with synchronous distant metastasis among well-differentiated pNEN patients, which was further demonstrated to be an independent risk factor by multivariate analysis (odds ratio 8.127, 95% confidence interval (CI) 2.105–31.372, P=0.002). For the prognostic value, APAR (P=0.007) was statistically associated with recurrence-free survival (RFS) in nonmetastatic resected pNEN patients, but it was not an independent predictor. Further subgroup analysis showed that APAR was independently related to RFS in patients with no nerve (hazard ratio (HR) 7.685, 95% CI 1.433–41.209, P=0.017) or vascular invasion (HR 4.789, 95% CI 1.241–18.473, P=0.023), respectively. Conclusion. APAR may work as a convenient pretreatment marker to detect synchronous distant metastasis for well-differentiated pNEN patients and predict recurrences for curatively resected cases without nerve or vascular invasion. However, these findings should be further verified in prospectively well-designed studies.

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