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 pseudocysts are the most common cystic lesions of the pancreas and may complicate acute pancreatitis, chronic pancreatitis, or pancreatic trauma. While the majority of acute pseudocysts resolve spontaneously, few may require drainage. On the other hand, pancreatic cystic tumors, which usually require extirpation, may disguise as pseudocysts. Hence, the distinction between the two entities is crucial for a successful outcome. We conducted this study to highlight the fundamental differences between pancreatic pseudocysts and cystic tumors so that relevant management plans can be devised. We reviewed the data of patients with pancreatic cystic lesions that underwent intervention between June 2007 and December 2010 in our hospital. We identified 9 patients (5 males and 4 females) with a median age of 40 years (range, 30-70 years). Five patients had pseudocysts, 2 had cystic tumors, and 2 had diseases of undetermined pathology. Pancreatic pseudocysts were treated by pseudocystogastrostomy in 2 cases and percutaneous drainage in 3 cases. One case recurred after percutaneous drainage and required pseudocystogastrostomy. The true pancreatic cysts were serous cystadenoma, which was treated by distal pancreatectomy, and mucinous cystadenocarcinoma, which was initially treated by drainage, like a pseudocyst, and then by distal pancreatectomy when its true nature was revealed. We conclude that every effort should be exerted to distinguish between pancreatic pseudocysts and cystic tumors of the pancreas to avoid the serious misjudgement of draining rather than extirpating a pancreatic cystic tumor. Additionally, percutaneous drainage of a pancreatic pseudocyst is a useful adjunct that may substitute for surgical drainage.
Adult ; Aged ; Cystadenocarcinoma, Mucinous ; diagnostic imaging ; pathology ; surgery ; Cystadenoma, Serous ; diagnostic imaging ; pathology ; surgery ; Diagnostic Errors ; Drainage ; Female ; Humans ; Male ; Middle Aged ; Pancreatectomy ; Pancreatic Cyst ; diagnostic imaging ; pathology ; surgery ; Pancreatic Neoplasms ; diagnostic imaging ; pathology ; surgery ; Pancreatic Pseudocyst ; diagnostic imaging ; pathology ; surgery ; Retrospective Studies ; Tomography, X-Ray Computed

OBJECTIVE: To describe the computed tomography (CT) features of neuroendocrine tumors (NETs) and solid pseudopapillary tumors (SPTs) with unilocular cyst-like appearance, and to compare them with those of unilocular cystic tumors of the pancreas. MATERIALS AND METHODS: This retrospective study was approved by our Institutional Review Board, and informed consent was waived. We included 112 pancreatic tumors with unilocular cyst-like appearance on CT (16 solid tumors [nine NETs and seven SPTs] and 96 cystic tumors [45 serous cystadenomas, 30 mucinous cystic neoplasms, and 21 branch-duct intraductal papillary mucinous neoplasms]). Two radiologists reviewed the CT images in consensus to determine tumor location, long diameter, morphological features, wall thicknesses, ratio of wall thickness to tumor size, wall enhancement patterns, intratumoral contents, and accompanying findings. Fisher's exact test was used to analyze the results. RESULTS: All 16 solid tumors had perceptible walls (mean thickness, 2.7 mm; mean ratio of wall thickness to tumor size, 7.7%) with variable enhancement. Four NETs and seven SPTs had hemorrhage, calcifications, and/or mural nodules. Six CT findings were specific for solid tumors with unilocular cyst-like appearance: a thick (> 2 mm) wall, uneven thickness of the wall, high ratio of wall thickness to tumor size, hyper- or hypo-attenuation of the wall in the arterial and portal phase, and heterogeneous internal contents. When three or more of the above criteria were used, 100% specificity and 87.5-92% accuracy were obtained for solid tumors with unilocular cyst-like appearance. CONCLUSION: A combination of CT features was useful for distinguishing solid tumors with unilocular cyst-like appearance from unilocular cystic tumors of the pancreas.
Adenocarcinoma, Mucinous/diagnosis/*radiography ; Adult ; Aged ; Carcinoma, Papillary/diagnosis/*radiography ; Cystadenoma, Serous ; Diagnosis, Differential ; Female ; Humans ; Male ; Middle Aged ; Neuroendocrine Tumors/diagnosis/*radiography ; Pancreatic Neoplasms/diagnosis/*radiography ; Retrospective Studies ; Sensitivity and Specificity ; Tomography, X-Ray Computed