Background The umbilical cord forms the connection between the fetus and the placenta at the feto-maternal interface and normally comprises two umbilical arteries and one umbilical vein. In some cases, only a single umbilical artery (SUA) is present. This study was conducted to evaluate associations between SUA and other congenital malformations discovered in perinatal autopsies and to ascertain the existence of preferential associations between SUA and certain anomalies.
Methods We evaluated records of all fetuses sent for autopsy to the Department of Pathology during the 10-year period from 2013 through 2022 (n = 1,277). The data were obtained from the hospital’s pathology laboratory records. The congenital anomalies were grouped by organ or system for analysis and included cardiovascular, urinary tract, nervous system, gastrointestinal tract, musculoskeletal, and lung anomalies.
Results A SUA was present in 8.61% of the autopsies. The gestational age of the affected fetuses ranged between 13 to 40 weeks. An SUA presented as an isolated single anomaly in 44 cases (3.4%). Of the 110 SUA cases, 60% had other congenital anomalies. There was a significant association between birth defects and SUAs (p < .001). Strong associations between SUA and urinary tract, lung, and musculoskeletal anomalies were observed.
Conclusions A SUA is usually seen in association with other congenital malformations rather than as an isolated defect. Therefore, examination for associated anomalies when an SUA is detected either antenatally or postnatally is imperative. The findings of this study should be helpful in counseling expectant mothers and their families in cases of SUA.
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Acute atherosis is unique vascular changes of the placenta associated with poor placentation. It is characterized by subendothelial lipid-filled foam cells, fibrinoid necrosis of the arterial wall, perivascular lymphocytic infiltration, and it is histologically similar to early-stage atherosclerosis. Acute atherosis is rare in normal pregnancies, but is frequently observed in non- transformed spiral arteries in abnormal pregnancies, such as preeclampsia, small for gestational age (SGA), fetal death, spontaneous preterm labor and preterm premature rupture of membranes. In preeclampsia, spiral arteries fail to develop physiologic transformation and retain thick walls and a narrow lumen. Failure of physiologic transformation of spiral arteries is believed to be the main cause of uteroplacental ischemia, which can lead to the production of anti-angiogenic factors and induce endothelial dysfunction and eventually predispose the pregnancy to preeclampsia. Acute atherosis is more frequently observed in the spiral arteries of the decidua of the placenta (parietalis or basalis) than in the decidual or myometrial segments of the placental bed. The presence and deeper location of acute atherosis is associated with poorer pregnancy outcomes, more severe disease, earlier onset of preeclampsia, and a greater frequency of SGA neonates in patients with preeclampsia. Moreover, the idea that the presence of acute atherosis in the placenta may increase the risk of future cardiovascular disease in women with a history of preeclampsia is of growing concern. Therefore, placental examination is crucial for retrospective investigation of pregnancy complications and outcomes, and accurate placental pathology based on universal diagnostic criteria in patients with abnormal pregnancies is essential for clinicopathologic correlation.
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Primary tumors of the great vessels are rare. Most encountered cases are sarcomas which most commonly develop in the aorta, pulmonary artery, and inferior vena cava. We experienced an intimal sarcoma arising in the left common iliac artery in a 68-year-old male, who suffered from claudication in his left lower extremity for a year and was diagnosed as arteriosclerosis obliterans, clinically. Bypass surgery was performed on the obstructive lesion. Grossly, the vascular lumen was filled with dark hemorrhagic materials. Microscopically, the lesion showed proliferation of anaplastic spindle cells with a marked nuclear atypia, arranged haphazardly. There were numerous mitotic figures.
Foci of cholesterol clefts were also found in the intima.
Immunohistochemically, the tumor cells were positive for vimentin, smooth muscle actin, and cytokeratin in certain areas. Stains for CD34, desmin, myosin heavy chain, caldesmon, and S-100 protein were negative. A pathologic diagnosis was made as intimal sarcoma with myofibroblastic differentiation.
BACKGROUND Pulmonary vessel sarcomas are rare, and their pathogenesis is still unclear. METHODS We focus on the pathologic findings of fourteen pulmonary artery and/or vein sarcomas along with clinical prognosis. RESULTS Nine patients were male and five were female, and they ranged in age from 26 to 72 years (mean, 47 years).
There were ten cases of pulmonary artery sarcoma, three cases of pulmonary artery and vein sarcoma, and one case of pure pulmonary vein sarcoma. Ten out of the fourteen cases were associated with pulmonary thromboembolism.
Microscopically, all the tumors showed an undifferentiated sarcomatous portion. There were leiomyosarcoma portions in 8 cases, malignant fibrous histiocytomatous portions in 7 cases, angiosarcomatous differentiation in 3 cases, and osteosarcomatous portion in 1 case. All but two patients died during the follow up period (range, 1 to 78 months).
The mean survival time of the patients who died was 14 months and the longest survival time was 78 months after surgical resection. CONCLUSIONS The current study is one of the largest single institutional reviews of pulmonary artery and/or vein sarcoma. Regardless of the histological components and macroscopic growth patterns, these rare tumors have a grave prognosis.
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This 64-year-old man presented with an 18-month history of cramping pain of sudden onset in the left calf and claudication. Angiographic findings showed a 6 cm length of luminal obliteration of the popliteal artery. Segmentally resected popliteal artery showed two longitudinally directed cystic masses measuring 3.5x1.5 cm and 2.5x1.5 cm in the adventitia. Microscopic examination revealed cystic space in the arterial adventitia compressing arterial lumen. There were a number of foamy histiocytes collected along the cystic lumen.
Multiple involvement of arterial fibromuscular dysplasia is unusal and such a case is quite limited in the literature.
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