Claudication due to adventitial cystic degeneration: A differential diagnosis of peripheral artery disease

Background Cystic adventitial disease (CAD) is an important and rare non-atherosclerotic cause of intermittent claudication and critical limb ischemia. Since the first case of CAD involving the external iliac artery was described by Atkins and Key in 1947, approximately 300 additional cases have been reported. Objectives The aim of this article is to report a rare vascular disorder, predominantly seen in young healthy men with minimal cardiovascular risk factors. Methods We report a rare case of cystic adventitial disease of a young policeman. To confirm the diagnosis, an ultrasonography and a conventional angiography were performed. The therapeutic approach was surgical first. Results The procedure was successful without any complication, and the patient was discharged to home 4 days after procedure. Conclusion While CAD is rare, the diagnosis should be suspected in a young patient who presents with arterial insufficiency and no risk factors for atherosclerosis. Catheter angiography is the investigation of choice in the absence of multislice CT and good MRA. It seems that the treatment that assures the best long-term results is reconstructive arterial bypass surgery.

Bladder paraganglioma: CT and MR imaging characteristics in 16 patients

Background Bladder paraganglioma (BPG) is a rare extra-adrenal pheochromocytoma with variable symptoms and easy to be misdiagnosed and mishandled. The aim of the study was to document the imaging features of BPG using computed tomography (CT) and magnetic resonance imaging (MRI). Patients and methods We retrospectively enrolled consecutive patients with pathology-proven BPG, who underwent CT or MRI examinations before surgery between October 2009 and October 2017. The clinical characteristics, CT, and MRI features of the patients were described and analysed. Results A total of 16 patients with 16 bladder tumours (median age 51 years, 9 females) were included. Among them, 13 patients underwent CT examinations and eight patients underwent MRI examinations preoperatively. Tumour diameters ranged from 1.6−5.4 cm. Most of the tumours grew into the bladder cavity (n = 11) with oval shapes (n = 10) and well-defined margins (n = 14). Intratumour cystic degeneration or necrosis (n = 2) was observed. Two lesions showed peripheral tissue invasion, suggesting malignant BPGs. All 13 lesions imaged with CT exhibited slight hypoattenuation and moderate to marked enhancement. Compared to the gluteus maximus, all lesions showed slight h yperintensity in T2-weighted images, hyperintensity on diffusion-weighted images (DWI), hypointensity on apparent diffusion coefficient maps, hyperintensity on T1-weighted images and a “fast in and slow out” enhanced pattern on contrast-enhanced MRI images. Conclusions BPGs are mostly oval-shaped, broadly-based and hypervascular bladder tumours with hypoattenuation on non-contrast CT, T2 hyperintensity, slight T1 hyperintensity compared to the muscle, marked restricted diffusion on DWI. Peripheral tissue invasion can suggest malignancy of the BPGs. All of these features contribute to preoperative decision-making.

Effect of the prenatal action of fulvestrant on the ovaries of the offspring of laboratory mice

Relevance. Fulvestrant is used for the treatment of breast cancer in combination with other drugs. The aim of the study was to determine the effect of the prenatal action of fulvestrant on the ovaries of the offspring of laboratory mice. Materials and Methods. The experimental animals were divided into 4 groups: intact, control and 2 experimental, 5 animals in each group. Injections were administered to females after fertilization at the gestational stage E 11.5 once intramuscularly. In the control group (n=5), sterile castor oil was administered at a dose of 0.8 mcg/kg. In the first experimental group (n=5), an antiestrogen was introduced in the form of an oil solution of fulvestrant 0.08 ml 0.0005% at a dose of 20 mcg/kg. In the second group (n=5), an antiestrogen was introduced in the form of an oil solution of fulvestrant 0.4 ml 0.0005% at a dose of 100 mcg/kg. Results and Discussion . The study revealed that in the ovaries when the drug was administered at a dose of 20 mcg/kg (F-20), the number of primordial follicles was reduced. Accordingly, the number of follicles of subsequent generations decreased. With the introduction of the drug fulvestrant 100 mcg/kg (F-100) on the section of the ovary, sclerosis of the stromal component is observed, accompanied by a rearrangement of the vascular network with signs of atresia and cystic degeneration of the follicular epithelium in the secondary and tertiary follicles, formed cysts are observed in the ovarian parenchyma. Conclusion. The prenatal effect of the drug fulvestrant on the maternal body during pregnancy leads to persistent structural changes in the ovaries of the offspring, manifested in the late stages of ontogenesis, which, in turn, can lead to violations of reproductive function. The depth and scale of these changes are dose-dependent.

Imaging Features and Pathological Analysis of 43 Parotid Basal Cell Adenomas

Purpose. To investigate the correlation between sonographic and computed tomography and pathological features of basal cell adenomas (BCAs) of the parotid gland. Methods. This retrospective study included 41 patients (43 tumors) with BCAs. The tumors were divided into three types based on their location in the parotid gland and their imaging features. The features of the tumors were analyzed. Results. Imaging manifestations and corresponding pathological results of most BCAs of the parotid glands resembled those of benign parotid gland tumors. Malignant transformation occurred in membranous BCAs and in those with extensive cribriform structures. Type-II and type-III tumors accounted for 82.93% of the total proportion. Thirteen tumors showed cystic degeneration with 30.23%, among which type-III tumors could easily develop cystic degeneration. These cystic areas might correspond to cystic degeneration or focal necrosis. Cystic change was not dependent on the tumor size. The pathological features of the tumors were correlated to their imaging manifestations. Conclusion. Most BCAs of the parotid glands have imaging manifestations similar to those of benign parotid gland tumors. BCAs with extensive cribriform structures and of the membranous type can show malignant transformation and should be treated with caution in clinical practice.

Solid Pseudopapillary Neoplasm (SPN) of the Pancreas: A 7-year Study in A Tertiary Level Teaching Hospital in Bangladesh

Solid pseudopapillary neoplasm (SPN) of the pancreas, also referred to as Frantz's tumour, is a rare tumour and represents 1-3% of all pancreatic tumours, which typically affects young women without significant clinical symptoms. This cross-sectional study was done in the Department of Pathology, Bangabandhu Sheikh Mujib Medical Un iversity (BSMMU) between January 2009 and December 2015. A total of nineteen cases of solid pseudopapillary neoplasm (SPN) of pancreas were detected in patients and included in this study. In all the patients the diagnosis was confirmed by histopathology after the surgery. Tumours were nodular, cystic or solid-cystic, often encapsulated. Some of them were received as irregular fragmented pieces, as intact removal was not possible. Cut surfaces were partly grey-white and partly tan-brown with cystic degeneration. Areas of necrosis and hemorrhage were present in all the specimens.The age range was of 14-45 years (mean age 26.9±3.7 years) (Table-I). Among the tumours, 18 were found in female patients, while only 1 was from male patient. In all the cases, preoperative imaging contributed to diagnosis of an abdominal mass in the patients. 11 of these were reported as pancreatic mass, while 3 as pancreatic cystic mass, 2 as retroperitoneal mass, 2 as mesenteric mass and only 1 as adrenal mass. Tumour-size ranged from 6 to 19 cm (mean size 9.3±1.5 cm). 7 tumours were solid and cystic in nature, while 8 were solid and 4 were only cystic. Complete capsule was found in 17 specimens. Calcifications were found in only 5 specimens. Surgical resection was found generally curative. In our study, absence of metastasis suggests that these tumours hardly show aggressive clinical behaviour. However, follow-up is important to observe potential local recurrence and metastasis. CBMJ 2020 July: Vol. 09 No. 02 P: 34-38

Leukoencephalopathy with vanishing white matter caused by EIF2B5 gene mutations: a case report

Leukoencephalopathy with vanishing white matter (VWM disease) is a progressive neurodegenerative disease with a specific magnetic resonance pattern characterized by diffuse lesions to the white matter and cystic degeneration. In this article, we report a case VWM disease in a boy with white matter lesions, in whom early onset and neurological symptoms suggested infantile form of the disease. The diagnosis was confirmed by the detection of biallelic mutations c.1688G>A (p.Arg563Gln) and c.1309G>A (p.Val437Met) in the EIF2B5 gene. The c.1309G>A mutation (p.Val437Met) was detected for the first time; it caused the development of severe disease.

Co-analysis of pancreatic cyst fluid carcinoembryonic antigen and glucose with novel cut-off levels better distinguishes between mucinous and non-mucinous neoplastic pancreatic cystic lesions

Background Pancreatic cyst fluid analysis plays an important role in distinguishing between mucinous and non-mucinous cyst lesions. We aimed to compare the diagnostic performances of cyst fluid carcinoembryonic antigen (CEA), CA 19-9, and glucose in differentiating mucinous from non-mucinous neoplastic pancreatic cystic lesions (PCLs) and determine the best cut-off levels. Methods Patients’ data were evaluated retrospectively. 102 patients’ PCLs were grouped as non-neoplastic ( n = 25), non-mucinous neoplastic ( n = 20), mucinous neoplastic ( n = 47) and pancreatic adenocarcinomas with cystic degeneration ( n = 10); and CEA, CA 19-9, and glucose levels were compared. Receiver-operating characteristic analysis was performed, and the ideal cut-off values were determined. Results Cyst fluid CEA and CA 19-9, levels were significantly higher ( P < 0.001, P < 0.001, respectively) and glucose levels were significantly lower ( P = 0.001) in mucinous than in non-mucinous neoplastic PCLs. Area under curve with 95% confidence interval of CEA, glucose and CEA and glucose test combination was 0.939 (95% CI = 0.885–0.993, P = 0.001), 0.809 (95% CI = 0.695–0.924, P < 0.001) and 0.937 (95% CI = 0.879–0.995), respectively. CEA cut-offs to rule-in and rule-out mucinous neoplastic were 135.1 ng/mL (sensitivity = 62%, specificity = 94.7%) and 6.12 ng/mL (sensitivity = 94.1%, specificity = 80.4%), respectively. Glucose cut-off of 2.8 mmol/L was chosen both to rule-in and rule-out mucinous neoplastic PCLs (sensitivity = 78%, specificity = 80%). Co-analysis of CEA and glucose to distinguish mucinous from non-mucinous neoplastic PCLs had sensitivity = 87.8%, specificity = 93.3%, and diagnostic accuracy = 89.3%. Conclusions We concluded that co-analysis of cyst fluid CEA (cut-off = 135.1 ng/mL) and glucose (cut-off = 2.8 mmol/L) at novel cut-offs had the best testing performance to rule-in mucinous neoplastic PCLs. To rule-out mucinous PCLs co-analysis of CEA (cut-off = 6.12 ng/mL) and glucose (cut-off = 2.8 mmol/L) added value to prediction.