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.

Objective: To study the clinicopathologic and genetic features of papillary cystic low-grade mucoepidermoid carcinoma (LG-MEC) and cystadenoma. Methods: A retrospective review was performed on salivary gland tumor patients with papillary cystic architecture who presented to department of oral pathology, Peking University School and Hospital of Stomatology between January 2010 and June 2022. Among this cohort, there were 17 males and 17 females with a range age of 23-82 years [(55.6±14.6) years]. Diagnosis was confirmed by histological, immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH) analysis. Finally, 15 papillary cystic LG-MEC and 19 cystadenoma patients were included in the present study. All patients were followed clinically and radiologically, and the duration of follow-up ranged from 1 to 141 months. Results: All neoplasms showed papillary proliferation with multilocular or giant cystic tumors. Papillary cystic LG-MEC was characterized by epidermoid cells, intermediate cell and mucous cells with multiple lining-layers. Papillary cystic LG-MEC had mild cellular atypia and a pushing infiltration. Cystadenoma was characterized by cuboidal, columnar and ciliated pseudostratified columnar lining epithelium. Squamous metaplasia, mucinous metaplasia and acidophilic degeneration could also be observed focally in cystadenoma. For IHC staining, papillary cystic LG-MEC showed diffusely and strongly positive for mucin 4 (MUC4) (15/15) and mucin 5 Subtype AC (MUC5AC) (4/15) in the epidermoid cells, intermediate cell and mucous cells. The epidermoid cells and intermediate cells were diffusely positive for p40 and p63. The Ki-67 index was about 10%-15% in LG-MEC. As a contrast, p40 (17/19) and p63 (14/15) were only detected in the basal cells of cystadenoma. Cystadenoma showed focal MUC5AC (4/19)expression and MUC4 (19/19)diffuse expression. In addition, the Ki-67 index was 5%-10% in cystadenoma. The MAML2 gene translocation was detected in 11 LG-MEC patients, but none in cystadenoma. Conclusions: The differential diagnosis points between papillary cystic LG-MEC and cystadenoma included the specific epidermoid cells, intermediate cells and mucus cells in LG-MEC, cell atypia, the pushing-infiltration pattern, diffuse expression of p40 and p63 in the lining epithelium, and a MAML2 gene rearrangement. The molecular test of MAML2 should be recommended to reduce missed LG-MEC diagnoses.
Male ; Female ; Humans ; Carcinoma, Mucoepidermoid/pathology* ; In Situ Hybridization, Fluorescence ; Ki-67 Antigen/genetics* ; Biomarkers, Tumor/analysis* ; Salivary Gland Neoplasms/diagnosis* ; Transcription Factors/metabolism* ; Cystadenoma ; Metaplasia

OBJECTIVE: To investigate the effect of inverse ratio ventilation (IRV) combined with positive end-expiratory pressure (PEEP) in infants undergoing thoracoscopic surgery with single lung ventilation (OLV) for lung cystadenomas. METHODS: A total of 66 infants undergoing thoracoscopic surgery with OLV for lung cystadenomas in our hospital from February, 2018 to February, 2019 were randomized into conventional ventilation groups (group N, =33) and inverse ventilation group (group R, =33). Hemodynamics and respiratory parameters of the infants were recorded and arterial blood gas analysis was performed at 15 min after two lung ventilation (TLV) (T), OLV30 min (T), OLV60 min (T), and 15 min after recovery of TLV (T). Bronchoalveolar lavage fluid was collected before and after surgery to detect the expression level of advanced glycation end product receptor (RAGE). RESULTS: Sixty-three infants were finally included in this study. At T and T, Cdyn, PaO and OI in group R were significantly higher ( < 0.05) and Ppeak, PaCO and PA-aO were significantly lower than those in group N ( < 0.05). There was no significant difference in HR or MAP between the two groups at T and T ( > 0.05). The level of RAGE significantly increased after the surgery in both groups ( < 0.05), and was significantly lower in R group than in N group ( < 0.05). CONCLUSIONS In infants undergoing thoracoscopic surgery with OLV for pulmonary cystadenoma, appropriate IRV combined with PEEP does not affect hemodynamic stability and can increases pulmonary compliance, reduce the peak pressure, and improve oxygenation to provide pulmonary protection.
Cystadenoma ; surgery ; Humans ; Infant ; Lung ; surgery ; One-Lung Ventilation ; Positive-Pressure Respiration ; Thoracoscopy ; Treatment Outcome

OBJECTIVE: To investigate the effect of inverse ratio ventilation (IRV) combined with positive end-expiratory pressure (PEEP) in infants undergoing thoracoscopic surgery with single lung ventilation (OLV) for lung cystadenomas. METHODS: A total of 66 infants undergoing thoracoscopic surgery with OLV for lung cystadenomas in our hospital from February, 2018 to February, 2019 were randomized into conventional ventilation groups (group N, =33) and inverse ventilation group (group R, =33). Hemodynamics and respiratory parameters of the infants were recorded and arterial blood gas analysis was performed at 15 min after two lung ventilation (TLV) (T), OLV30 min (T), OLV60 min (T), and 15 min after recovery of TLV (T). Bronchoalveolar lavage fluid was collected before and after surgery to detect the expression level of advanced glycation end product receptor (RAGE). RESULTS: Sixty-three infants were finally included in this study. At T and T, Cdyn, PaO and OI in group R were significantly higher ( < 0.05) and Ppeak, PaCO and PA-aO were significantly lower than those in group N ( < 0.05). There was no significant difference in HR or MAP between the two groups at T and T ( > 0.05). The level of RAGE significantly increased after the surgery in both groups ( < 0.05), and was significantly lower in R group than in N group ( < 0.05). CONCLUSIONS In infants undergoing thoracoscopic surgery with OLV for pulmonary cystadenoma, appropriate IRV combined with PEEP does not affect hemodynamic stability and can increases pulmonary compliance, reduce the peak pressure, and improve oxygenation to provide pulmonary protection.
Cystadenoma ; therapy ; Humans ; Infant ; Lung ; One-Lung Ventilation ; Positive-Pressure Respiration ; Thoracoscopy

In recent years, there have been more and more reports about cystadenoma. Cystadenoma can occur in many parts of the body, and cystadenoma in different parts may show different clinical symptoms, however, some patients with cystadenoma have no symptoms. The vast majority of cystadenomas are benign lesions, but a small number of cystadenomas can be malignant. For example, a small number of ovarian cystadenomas and pancreatic cystadenomas may be malignant. This study reported a patient with small intestinal cystadenoma diagnosed by pathology. The patient's physical examination revealed a lesion in the left upper abdomen. He had only abdominal distension and no other discomfort. His laboratory examination results were basically normal, i.e. blood routine, urine routine, stool routine, liver function, kidney function, myocardial enzyme, tumor marker, etc. The patient underwent sectional small intestine resection and the pathological sample was analyzed. The histological findings of the resected intestinal sample were consistent with cystadenoma. Computed tomography scan of the abdomen was performed 4 months after the surgery. No recurrence of the tumor was found. The patient recovered in good condition. By consulting the literature, I found very few reports of small intestinal cystadenoma before, it was very rare. This article described the clinical manifestation, diagnosis and differential diagnosis, treatment and prognosis of a case of small intestinal cystadenoma, it suggested that cystadenoma can occur in the small intestine, other than the ovary, pancreas, liver, lung, thyroid, prostate, seminal vesicle, skin, etc. The cystadenoma in small intestine is easy to be mistaken with other tumors, such as small intestine stromal tumor, small intestine adenocarcinoma, small intestine lipoma, small intestine hemangiomas, etc., and it is difficult to fully confirm through imaging examinations, such as computed tomography and magnetic resonance imaging. Laparotomy and histopathological examination are necessary before definitive diagnosis. This disease can be treated by small bowel resection at the affected region and good prognosis can be achieved.
Cystadenoma ; Humans ; Intestinal Neoplasms ; Intestine, Small ; Male ; Neoplasm Recurrence, Local ; Pancreatic Neoplasms ; Prostate

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

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

Apocrine hidrocystoma, also called apocrine cystadenoma, is a benign cystic tumor-like lesion that arises from the proliferation of apocrine glands. Clinically, it usually occurs singly as a unilocular or multilocular, dome-shaped translucent cyst. Histologically, it appears as unilocular or multilocular cysts composed of an inner layer of single or double layer of secretory columnar epithelium with decapitation secretion lying above an outer myoepithelial cell layer. Apocrine hidrocystomas mostly occur within the head and neck region and involvement of genitalia is extremely rare. This paper emphasizes the importance of considering the differential diagnosis of a genital cystic lesion. Herein, we report a case of apocrine hidrocystoma occurring in the penis and compare the clinicopathological characteristics of apocrine hidrocystoma in genitalia with the previous cases.
Apocrine Glands ; Cystadenoma ; Decapitation ; Deception ; Diagnosis, Differential ; Epithelium ; Genitalia ; Head ; Hidrocystoma ; Male ; Neck ; Penis

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

Cystadenoma ; diagnosis ; Genital Neoplasms, Male ; diagnosis ; Humans ; Male ; Middle Aged ; Seminal Vesicles ; pathology