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Journal of Kidney Cancer and VHL 2022In this article, we present a Von Hippel-Lindau (VHL) patient with synchoronus bilateral testicular metastasis from renal cell carcinoma (RCC). A 50 year-old man, a...
In this article, we present a Von Hippel-Lindau (VHL) patient with synchoronus bilateral testicular metastasis from renal cell carcinoma (RCC). A 50 year-old man, a known case of VHL syndrome was referred with palpable masses in both the testes. His medical history demonstrated that he had undergone the brain surgery for cerebellar hemangioblastoma. He had undergone simultaneous Whipple's pancreatectomy and left radical nephrectomy becuase of well-differentiated neuroendocrine tumors in head and body of the pancreas and a 6-cm clear cell-type grade-3 RCC in the left kidney. Scrotal sonography demonstrated vascular and heteroechogen masses measuring 19×14 mm in lower pole of the right testicle, 19×16 mm in upper pole of the right testicle, and 23×16.5 mm in upper pole of the left testicle. After having patient's consent, bilateral orchiectomy was performed by inguinal incision. Histopathologic examination and immunohistochemistry staining revealed metastasis from RCC. The most common neoplasm of reproductive system in VHL patients is epididymal papillary cystadenoma. Owing to it's benign nature, the management is conservative with routine physical examination and ultrasonography. Our patient indicated that every scrotal mass in patients with VHL is not to be considered as epididymal papillary cystadenoma.
PubMed: 36118791
DOI: 10.15586/jkcvhl.v9i2.211 -
Pancreatology : Official Journal of the... Nov 2022Although emerging data evidences that EUS-guided needle-based confocal laser endomicroscopy (nCLE) accurately diagnoses pancreatic cystic lesions (PCLs), there are a...
BACKGROUND
Although emerging data evidences that EUS-guided needle-based confocal laser endomicroscopy (nCLE) accurately diagnoses pancreatic cystic lesions (PCLs), there are a lack of interobserver agreement (IOA) studies utilizing reference histopathological diagnosis and for specific PCL subtypes. Hence, we sought to assess the IOA, intra-observer reliability (IOR), and diagnostic performance of EUS-nCLE using a large cohort of patients with histopathological diagnosis amongst a broad panel of international observers.
METHODS
EUS-nCLE videos (n = 76) of subjects with PCLs [intraductal papillary mucinous neoplasm (IPMN), mucinous cystic neoplasm (MCN), serous cystadenoma (SCA), pseudocyst, and cystic-neuroendocrine tumors/solid pseudopapillary neoplasm (cystic-NET/SPN)], simulating clinical prevalence rates were obtained from 3 prospective studies. An international panel of 13 endosonographers with nCLE experience, blinded to all PCL data, evaluated the video library twice with a two-week washout for PCL differentiation (mucinous vs. non-mucinous) and subtype diagnosis.
RESULTS
The IOA (κ = 0.82, 95% CI 0.77-0.87) and IOR (κ = 0.82, 95% CI 0.78-0.85) were "almost perfect" to differentiate mucinous vs. non-mucinous PCLs. For PCL subtype, IOA was highest for SCA (almost perfect; κ = 0.85), followed by IPMN (substantial, κ = 0.72), and cystic-NET/SPN (substantial, κ = 0.73). The IOA was moderate for MCN (κ = 0.47), and pseudocyst (κ = 0.57). Compared to histopathology, observers differentiated mucinous vs. non-mucinous PCLs with high accuracy (94.8%, 95% CI 93.3-96.1). For detecting specific PCLs subtypes, EUS-nCLE was highly accurate in diagnosing non-mucinous cysts (SCA: 98%; cystic-NET/SPN: 96%; pseudocyst: 96%) and slightly less accurate for mucinous lesions (IPMN: 86%; MCN: 84%).
CONCLUSION
Diagnosis of PCLs by EUS-nCLE guided virtual biopsy is very accurate and reliable for the most prevalent pancreatic cysts in clinical practice.
Topics: Humans; Endoscopic Ultrasound-Guided Fine Needle Aspiration; Prospective Studies; Reproducibility of Results; Pancreatic Intraductal Neoplasms; Microscopy, Confocal; Pancreatic Cyst; Cystadenoma, Serous; Pancreatic Neoplasms; Neuroendocrine Tumors
PubMed: 36089484
DOI: 10.1016/j.pan.2022.08.012 -
Radiographics : a Review Publication of... 2022Borderline epithelial ovarian tumors are a distinct pathologic entity characterized by increased epithelial proliferation and nuclear atypia, but without frank stromal...
Borderline epithelial ovarian tumors are a distinct pathologic entity characterized by increased epithelial proliferation and nuclear atypia, but without frank stromal invasion. Borderline tumor (BT) is now considered to represent an intermediate phase in the stepwise progression from benign to malignant ovarian epithelial tumor. Since BTs commonly manifest at early stages in women of reproductive age and are associated with a good prognosis, making the correct diagnosis is important in determining whether a patient is a candidate for fertility-sparing surgery. There are six histologic BT subtypes (serous, mucinous, seromucinous, endometrioid, clear cell, and Brenner), and each has different MRI features, reflecting their unique histologic architectures. Radiologists should be aware of the MRI features that can suggest BTs. These features include a hyperintense papillary architecture with hypointense internal branching, which can be observed with serous and seromucinous BTs on T2-weighted images; aggregates of microcysts that have hypointensity on T2-weighted images and reticular enhancement on contrast-enhanced T2-weighted images, which can be seen with mucinous BTs; and moderately high signal intensity on diffusion-weighted images along with relatively high apparent diffusion coefficient values, which can be observed regardless of the histologic subtype. Nevertheless, because the imaging features of BTs overlap with those of many benign lesions (eg, cystadenoma and cystadenofibroma, decidualized endometriosis, and polypoid endometriosis) and malignant tumors (ovarian cancers and metastases), histologic confirmation is required for the final diagnosis. Special emphasis is placed on the MRI features of BTs, pathologic correlation, and the challenges related to diagnosis. RSNA, 2022.
Topics: Female; Humans; Endometriosis; Ovarian Neoplasms; Carcinoma; Magnetic Resonance Imaging; Diffusion Magnetic Resonance Imaging
PubMed: 36083804
DOI: 10.1148/rg.220068 -
Ear, Nose, & Throat Journal Sep 2022
PubMed: 36074080
DOI: 10.1177/01455613221124731 -
Indian Journal of Gastroenterology :... Aug 2022Pancreatic cystic lesions (PCLs) are being diagnosed with increased frequency and have varying neoplastic potential. We conducted this multimodal, prospective study to...
BACKGROUND AND AIM
Pancreatic cystic lesions (PCLs) are being diagnosed with increased frequency and have varying neoplastic potential. We conducted this multimodal, prospective study to evaluate the role of tumor cytology and molecular markers to differentiate PCL subtypes.
METHODS
Consecutive undiagnosed patients with PCLs (n = 100, mean age: 50.37 years; 41% males) were prospectively studied. Cyst fluid carcinoembryonic antigen (CEA), CA19.9, CA125, CA72.4, and vascular endothelial growth factor-alpha (VEGF-α) levels were measured by quantitative enzyme-linked immunosorbent assay (ELISA) method. Mutational analysis of the KRAS gene (exon 2, Codon 12 and 13) and GNAS gene (Exon 8, Codon 201) were performed by Sanger's sequencing.
RESULTS
The mean cyst size was 4.32 ± 2.4 cm. Fluid cytology revealed definitive diagnosis in 21 (22.3%) patients. All malignant PCLs could be identified on cytology whereas 10/14 (71%) non-malignant mucinous PCLs could also be identified on cytology based on mucin staining. Among the tested tumor markers, cyst fluid CEA had the best diagnostic performance for differentiation between mucinous and non-mucinous PCLs (AUC 0.933 [95% CI 0.86-0.91]). At a cyst fluid CEA cutoff level of 45.0 ng/mL, the sensitivity, specificity, positive predictive value, and negative predictive value for differentiation between mucinous and non-mucinous cysts were 88.5%, 96.8%, 92.0%, and 95.3%, respectively (p < 0.05). KRAS and GNAS mutation had no significant diagnostic benefit in comparison to fluid cytology and CEA levels.
CONCLUSIONS
Fluid CEA at a lower cutoff of 45 ng/mL is the most accurate marker to differentiate between mucinous and non-mucinous PCL. The KRAS and GNAS mutational analysis does not improve upon the diagnostic performance of fluid cytology and tumor markers.
Topics: Biomarkers, Tumor; Carcinoembryonic Antigen; Cyst Fluid; Diagnosis, Differential; Female; Humans; Male; Middle Aged; Pancreatic Cyst; Pancreatic Neoplasms; Prospective Studies; Proto-Oncogene Proteins p21(ras); Vascular Endothelial Growth Factor A
PubMed: 36057043
DOI: 10.1007/s12664-022-01269-w -
Surgical Pathology Clinics Sep 2022The development of cross-sectional imaging techniques has enhanced the detection of pancreatic cystic lesions (PCLs). PCLs are found in approximately 2% of the general... (Review)
Review
The development of cross-sectional imaging techniques has enhanced the detection of pancreatic cystic lesions (PCLs). PCLs are found in approximately 2% of the general population, often as incidentally detected lesions on computed tomography or MRI during the evaluation of other medical conditions. Broadly, PCLs are classified as mucinous or nonmucinous. Mucinous PCLs include mucinous cystic neoplasms and intraductal papillary mucinous neoplasms. Nonmucinous PCLs include pseudocysts, serous cystadenomas, solid pseudopapillary neoplasms, and cystic pancreatic neuroendocrine tumors, as well as cystic acinar cell carcinoma, cystic degeneration of pancreatic ductal adenocarcinoma, lymphoepithelial cyst, and others.
Topics: Carcinoma, Pancreatic Ductal; Cystadenoma, Serous; Humans; Pancreatic Cyst; Pancreatic Ducts; Pancreatic Neoplasms; Pancreatic Pseudocyst
PubMed: 36049828
DOI: 10.1016/j.path.2022.05.009 -
Surgical Endoscopy Jan 2023Risk factors for postoperative pancreatic fistula (POPF) following pancreatic enucleation by the open approach (OpenEN) are well known. However, ENs are more frequently...
BACKGROUND
Risk factors for postoperative pancreatic fistula (POPF) following pancreatic enucleation by the open approach (OpenEN) are well known. However, ENs are more frequently performed laparoscopically (LapEN). The aim of this study was to analyze the risk factors of POPF following LapEN.
METHODS AND PATIENTS
All patients in our prospective database who underwent LapEN were evaluated. We report the demographics, surgical, early and long-term outcomes. Numerous variables were analyzed to identify the risk factors of POPF.
RESULTS
From 2008 to 2020, 650 laparoscopic pancreatic resections were performed including 64 EN (10%). The median age was 51 years old (17-79), median BMI was 24 (19-48), and 44 patients were women (69%). The main presentation was an incidental diagnosis (n = 40; 62%), pain (n= 10;16%), and hypoglycemia (n = 8;12%). The main indications were neuroendocrine tumors (40; 63%), mucinous cystadenomas (15; 23%), intraductal papillary mucinous neoplasie (3; 5%), and other benign cysts (6; 9%). Lesions were located on the distal pancreas (43; 67%), head (n = 17; 27%), and neck (4; 6%). The median size was 20 mm (9-110); 30 mm (20-110) for mucinous cystadenoma and 18 mm (8-33) for NET. The median operative time was 90 mn (30-330), median blood loss was 20 ml (0-800) ml, and there were no transfusions and one conversion. There were no mortalities and overall morbidity (n = 22; 34%) included grades B and C POPF (10;16%) and post-pancreatectomy hemorrhage (4; 6%). The median hospital stay was 7 days (3-42). There were no invaded lymph nodes and all cystic lesions were nonmalignant. After a mean follow-up of 24 months, there was no recurrence. The risk factors for grades B/C POPF were mucinous cystadenoma and proximity to the Wirsung duct < 3 mm.
CONCLUSION
In this series, the outcome of LapEN was excellent with no mortality and a low rate of morbidity. However, the risk of POPF is increased with cystic lesions and those close to the Wirsung duct.
Topics: Humans; Female; Middle Aged; Male; Pancreatic Fistula; Pancreatic Neoplasms; Cystadenoma, Mucinous; Treatment Outcome; Pancreatic Ducts; Pancreatectomy; Laparoscopy; Postoperative Complications; Retrospective Studies
PubMed: 36002687
DOI: 10.1007/s00464-022-09527-w -
Zeitschrift Fur Gastroenterologie Jun 2023Cystic pancreatic neoplasms (CPN) are frequently diagnosed due to better diagnostic techniques and patients becoming older. However, diagnostic accuracy of endoscopic...
Cystic pancreatic neoplasms in a tertiary gastroenterologic referral center: Evaluation of the diagnostic accuracy of endoscopic ultrasound, progression rate and malignancy rate in a large unicentric cohort.
INTRODUCTION
Cystic pancreatic neoplasms (CPN) are frequently diagnosed due to better diagnostic techniques and patients becoming older. However, diagnostic accuracy of endoscopic ultrasound (EUS) and value of follow-up are still unclear.
MATERIAL AND METHODS
The aim of our retrospective study was to investigate the frequency of different cystic pancreatic neoplasms (intraductal papillary mucinous neoplasm [IPMN], serous and mucinous cystadenoma, solid pseudopapillary neoplasia), diagnostic accuracy, size progression, and rate of malignancy using EUS in a tertiary reference center in Germany. Between January 1, 2012 and December 31, 2018, 455 patients were diagnosed with cystic pancreatic lesions (798 EUS examinations).
RESULTS
Endoscopic ultrasound diagnosed 223 patients with cystic pancreatic neoplasms, including 138 (61.9%) patients with branch duct IPMN, 16 (7.2%) with main duct IPMN, and five (2.2%) with mixed-type IPMN. In the largest subgroup of branch duct IPMN, cysts were size progressive in 20 patients (38.5%). Fine needle aspiration (FNA) was performed in 21 patients, and confirmed the suspected diagnosis in 12/21 patients. 28 surgical resections were performed, in 7/28 patients (25%), high-grade dysplasia or cancer was diagnosed. Endoscopic ultrasound diagnosis of serous and mucinous cystic pancreatic neoplasms was correct in 68.4%.
CONCLUSIONS
Endoscopic ultrasound differential diagnosis of CPNs is challenging. Even in a tertiary expert center, differentiation of serous and mucinous cystic neoplasia is not guaranteed. Relevant size progression of CPN, however, is rare, as is the rate of malignancy. The data of this study suggest that morphologic criteria to assess pancreatic cysts alone are not sufficient to allow a clear diagnosis. Hence, for the improved assessment of pancreatic cysts, EUS should be combined with additional tests and techniques such as MRT/MRCP, contrast-enhanced EUS, and/or FNA/fine needle biopsy including fluid analysis. The combination and correlation of imaging studies with EUS findings is mandatory.
Topics: Humans; Pancreatic Cyst; Retrospective Studies; Pancreatic Intraductal Neoplasms; Endoscopic Ultrasound-Guided Fine Needle Aspiration; Pancreatic Neoplasms; Endosonography; Referral and Consultation
PubMed: 35878606
DOI: 10.1055/a-1852-5644 -
The American Journal of Surgical... Sep 2022The literature on liver cysts is highly conflicting, mostly owing to definitional variations. Two hundred and fifty-eight ≥1 cm cysts evaluated pathologically using...
The literature on liver cysts is highly conflicting, mostly owing to definitional variations. Two hundred and fifty-eight ≥1 cm cysts evaluated pathologically using updated criteria were classifiable as: I. Ductal plate malformation related (63%); that is, cystic bile duct hamartoma or not otherwise specified-type benign biliary cyst (35 with polycystic liver disease). These were female predominant (F/M=2.4), large (10 cm), often multifocal with degenerative/inflammatory changes and frequently misclassified as "hepatobiliary cystadenoma." II. Neoplastic (13%); 27 (10.5%) had ovarian-type stroma (OTS) and qualified as mucinous cystic neoplasm (MCN) per World Health Organization (WHO). These were female, solitary, mean age 52, mean size 11 cm, and 2 were associated with carcinoma (1 in situ and 1 microinvasive). There were 3 intraductal papillary neoplasms, 1 intraductal oncocytic papillary neoplasm, 1 cystic cholangiocarcinoma, and 2 cystic metastasis. III. Infectious/inflammatory (12%). These included 23 hydatid cysts (including 2 Echinococcus alveolaris both misdiagnosed preoperatively as cancer), nonspecific inflammatory cysts (abscesses, inflammatory cysts: 3.4%). IV. Congenital (7%). Mostly small (<3 cm); choledochal cyst (5%), foregut cyst (2%). V. Miscellaneous (4%). In conclusion, hepatic cysts occur predominantly in women (3/1), are mostly (90%) non-neoplastic, and seldom (<2%) malignant. Cystic bile duct hamartomas and their relative not otherwise specified-type benign biliary cysts are frequently multifocal and often misdiagnosed as "cystadenoma/carcinoma." Defined by OTS, MCNs (the true "hepatobiliary cystadenoma/carcinoma") are solitary, constitute only 10.5% of hepatic cysts, and have a significantly different profile than the impression in the literature in that essentially all are perimenopausal females, and rarely associated with carcinoma (7%). Since MCNs can only be diagnosed by demonstration of OTS through complete microscopic examination, it is advisable to avoid the term "cystadenoma/cystadenocarcinoma" solely based on radiologic examination, and the following simplified terminology would be preferable in preoperative evaluation to avoid conflicts with the final pathologic diagnosis: (1) noncomplex (favor benign), (2) complex (in 3 subsets, as favor benign, cannot rule out malignancy, or favor malignancy), (3) malignant features.
Topics: Bile Duct Neoplasms; Bile Ducts, Intrahepatic; Choledochal Cyst; Cystadenocarcinoma; Cystadenoma; Cysts; Diagnosis, Differential; Female; Humans; Liver Diseases; Male; Middle Aged; Pancreatic Neoplasms
PubMed: 35778790
DOI: 10.1097/PAS.0000000000001930 -
Diagnostic Cytopathology Jan 2023Molecular diagnostics has impacted the diagnosis, prediction of prognosis, and selection of targeted therapy for many tumor types. While pulmonary adenocarcinomas and...
BACKGROUND
Molecular diagnostics has impacted the diagnosis, prediction of prognosis, and selection of targeted therapy for many tumor types. While pulmonary adenocarcinomas and melanomas are among the neoplasms most associated with molecular diagnostics and targeted therapy, malignancies of the pancreaticobiliary system have also been impacted by precision medicine.
METHODS
We undertook an electronic search using PubMed and Embase to review the published literature to determine what forms of molecular testing, mutations and oncogenetic pathways are associated with neoplasms of the pancreaticobiliary system. Keywords utilized were pancreas, bile duct, mutations, ERCP, FNA, KRAS, SMAD4, TP53, next-generation sequencing, serous cystadenoma, pancreatic ductal adenocarcinoma, intraductal papillary mucinous neoplasm, cystic mucinous neoplasm, solid pseudo-papillary neoplasm.
RESULTS
A search between 1999 and 2022 yielded 6874 manuscripts. Screening of these yielded 302 more focused manuscripts of which 55 were used for the study. Ductal adenocarcinoma of the pancreas is associated with a progression of mutations beginning wit KRAS mutations and ending with a set of mutations in the TP53, SMAD4, and DPC4 genes. Similar mutations are found in neoplastic mucinous cysts. Specific mutations characterize serous cystadenomas, solid, and pseudo papillary neoplasms and adenocarcinomas of the bile ducts.
CONCLUSIONS
Mutational analysis of cytologic specimens obtained by fine-needle aspiration, and duct brushings and washings are helpful in the diagnosis of pancreaticobiliary neoplasms and may supply prognostic information.
Topics: Humans; Pancreatic Neoplasms; Biliary Tract Neoplasms
PubMed: 35751521
DOI: 10.1002/dc.25005