Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome is a rare congenital disorder characterized by the absence or underdevelopment of the uterus and upper part of the vagina in females with a normal 46, XX karyotype. It affects approximately 1 in 4500–5000 female live births and ranks as the second-most common cause of primary amenorrhea. This case report describes a 28-year-old nulligravid woman who presented with primary amenorrhea, difficulties during sexual intercourse manifesting as pain and resistance, and an incidental finding of a right ovarian new growth. Physical examination revealed normal secondary sexual characteristics and a blind-ending vagina measuring 5 cm in depth. Transvaginal ultrasound confirmed the presence of a transverse vaginal septum with hematocolpos, an infantile uterus with endometrium and cervix, a right ovarian new growth, and a normal left ovary. Both kidneys appeared normal, and hormonal assays were within normal limits. Karyotype analysis confirmed a genotype of 46, XX, indicating a normal chromosomal complement for a female without any detectable structural or numerical chromosomal abnormalities, consistent with typical female development. She subsequently underwent ultrasound-guided excision of the transverse vaginal septum combined with laparoscopic oophorocystectomy. Intraoperatively, findings included a normal left ovary, a right ovarian new growth, absence of fallopian tubes, and an infantile uterus. Histological analysis confirmed a serous cystadenoma in the right ovary. Karyotype analysis confirmed a genotype of 46, XX. The index case was diagnosed with MRKH type II (atypical), characterized by the absence of fallopian tubes and a right ovarian new growth without associated renal, skeletal, or cardiac anomalies.

Human ; Female ; Adult: 25-44 Yrs Old ; Cystadenoma, Serous

No abstract available.
Bile ; Cystadenoma, Serous ; Dilatation ; Pancreas ; Pancreatic Ducts

No abstract available.
Bile ; Cystadenoma, Serous ; Dilatation ; Pancreas ; Pancreatic Ducts

The most common hypervascular neoplasm of the pancreas is neuroendocrine tumor (NET). Microcystic serous cystadenomas, certain metastases, and accessory spleens can also show hypervascularity and can mimic pancreatic NET. It is important to discriminate hypervascular pancreatic lesions because of different treatment option and prognosis. Although computed tomography (CT) is the most common imaging modality for initial identification of pancreatic tumor, CT alone cannot correctly interpret hypervascular pancreatic lesions. Therefore, when a hypervascular tumor in the pancreas is detected on CT, magnetic resonance imaging should be considered. In this essay, I describe imaging features those are helpful for differential diagnosis of NET from other hypervascular lesions in pancreas.
Cystadenoma, Serous ; Diagnosis, Differential* ; Magnetic Resonance Imaging ; Multidetector Computed Tomography ; Neoplasm Metastasis ; Neuroendocrine Tumors* ; Pancreas* ; Pancreatic Neoplasms ; Prognosis ; Spleen

Pancreatic malignancy is the third leading cause of cancer related death in the United States with limited viable screening options. By the end of this decade, cancers are poised to become the leading cause of death with pancreatic cancer projected to be the second leading cause of cancer related mortality. Pancreatic cystic lesions (PCLs) are found in approximately 5%–14% of patients due to the increased utilization of cross-sectional imaging, with approximately 8%–10% of pancreatic cancers originating as PCLs. Current screening guidelines have shown discrepancies between morphologic characteristics of PCLs and identifying advanced pancreatic disease. Molecular analysis has emerged as a novel technology to aid in adequate diagnosis and management decisions of PCLs. Mucinous cysts including intraductal papillary mucinous neoplasms (IPMNs) or mucinous cystic neoplasms have similar oncogenic mutations including KRAS, TP53, SMAD4, PIK3CA, PTEN, or CKDN2A, while GNAS and RNF43 mutations are specific only to IPMNs. Serous cystadenomas have been associated with a loss of tumor suppressor gene VHL, while solid-psuedopapillary neoplasms have an oncogenic mutation CTNNB1. A specific molecular marker to diagnose existing high-grade dysplasia or impending malignant transformation is yet to be identified. Moving forward it is important to advance technology in isolating and identifying high-risk molecular markers from cyst fluid while considering their increased utilization in the evaluation of PCLs.
Biomarkers, Tumor ; Cause of Death ; Cyst Fluid ; Cystadenoma, Serous ; Diagnosis ; Genes, Tumor Suppressor ; Humans ; Loss of Heterozygosity ; Mass Screening ; Mortality ; Mucins ; Neoplasms, Cystic, Mucinous, and Serous ; Pancreatic Cyst* ; Pancreatic Diseases ; Pancreatic Neoplasms ; United States

OBJECTIVETo evaluate the value of intraoperative fine needle aspiration cytology (IFNAC) examination in the diagnosis of pancreatic lesions.

METHODSThe clinicopathological data of 491 patients with pancreatic lesions treated in our hospital from May 1998 to June 2013 were retrospectively analyzed. Their clinical features, IFNAC findings, pathological results after IFNAC examination and related complications were summarized. The factors affecting the aspiration biopsy accuracy were analyzed using logistic regression and multi factor analysis.

RESULTS491 patients with pancreatic lesions were examined by IFNAC. Among them, cancer cells were found in 434 cases (positive), and were not found in 57 cases (negative). Among the 310 cases who underwent surgical operation, postoperative pathology confirmed 209 cases of pancreatic ductal adenocarcinoma, 8 cases of pancreatic cystadenocarcinoma, 23 cases of solid pseudopapillary tumor of the pancreas, 15 cases of pancreatic neuroendocrine tumor, 14 cases of intraductal papillary mucinous tumor, 2 cases of primary pancreatic gastrointestinal stromal tumor, 17 cases of pancreatic serous cystadenoma, and 22 cases of chronic mass-forming type pancreatitis. The IFNAC test showed a sensitivity of 97.9% (425/434), and specificity of 89.5% (51/57). The IFNAC examination-related complications were pancreatic leakage in a total of 12 patients which were cured after treatment. No bleeding complication was observed. Logistic multivariate analysis showed that tumor size, cystic degeneration, lymph node metastasis and associated chronic pancreatitis are independent factors affecting the IFNAC examination of pancreatic carcinoma.

CONCLUSIONSIFNAC examination has a high sensitivity and specificity, and with a good safety in clinical use. IFNAC can be used as a powerful tool for the diagnosis of pancreatic cancer, with a high clinical value in use. In the cytology-negative cases, cytology alone can not rule out the diagnosis of pancreatic cancer. Through repeated sampling and combined with intraoperative frozen section pathology can improve the diagnostic accuracy.

Biopsy, Fine-Needle ; Biopsy, Needle ; Carcinoma, Pancreatic Ductal ; diagnosis ; pathology ; Cystadenoma, Serous ; diagnosis ; pathology ; Frozen Sections ; Humans ; Pancreas ; pathology ; Pancreatic Neoplasms ; diagnosis ; pathology ; Retrospective Studies ; Sensitivity and Specificity

Rapid advancements, access to and use of imaging techniques have increased the frequency of identification of pancreatic cystic neoplasms in clinical practice. However, a diagnostic dilemma among pancreatic cystic neoplasms remains. Solid variant serous cystadenoma is extremely rare and difficult to accurately diagnose preoperatively, as they are commonly mistaken for malignant solid tumors of other types. Here, we present a case of a solid variant serous cystadenoma preoperatively misdiagnosed as a neuroendocrine tumor of the pancreas with a review of the relevant literature.
Cystadenoma, Serous* ; Neuroendocrine Tumors* ; Pancreas* ; Pancreatic Cyst

No abstract available.
Cystadenoma, Serous* ; Female ; Ovary*

Pancreatic cystic lesions include retention cysts (congenital cysts), pseudocysts, and cystic neoplasms. Pancreatic cystic neoplasms have recently been diagnosed more commonly, possibly due to advances in imaging and widespread screening programs. Cystic neoplasms of the pancreas account for 10-20% of pancreatic tumors. Mucinous cystic neoplasms (MCN) and intraductal papillary mucinous neoplasms are regarded as premalignant lesions, whereas serous cystadenoma is not. In the clinical setting of acute pancreatitis, pancreatic cystic lesions are usually diagnosed as pseudocysts. However, cystic neoplasms of the pancreas should be considered in the differential diagnosis of pancreatic cysts, even in patients with a history of pancreatitis. In the Korean literature, MCN combined with acute pancreatitis has rarely been reported. Here, we report a case of MCN presenting with acute pancreatitis in a 22-year-old female, which was initially misdiagnosed as pancreatic pseudocyst.
Cystadenoma, Serous ; Diagnosis, Differential ; Female ; Humans ; Mass Screening ; Mucins* ; Pancreas* ; Pancreatic Cyst ; Pancreatic Neoplasms ; Pancreatic Pseudocyst ; Pancreatitis* ; Young Adult

No abstract available.
Cystadenoma, Serous* ; Diagnosis* ; Endoscopic Ultrasound-Guided Fine Needle Aspiration*