The pentalogy of Cantrell, a rare syndrome, consists of the defects in the anterior diaphragm, diaphragmatic pericardium, lower sternum and supraumbilical abdominal wall, along with congenital cardiac abnormalities. Till date, only few patients with full spectrum of this syndrome have been reported with only 2 cases showing associated exencephaly and spinal dysraphism. We report extremely rare association of complete pentalogy of Cantrell syndrome with exencephaly and spinal dysraphism on antenatal sonogram and autopsy in a 18 weeks fetus of a 19 year-old primi gravida female.
Introduction:Transrectal rectal ultrasound (TRUS)-guided systematic biopsy is the gold standard for diagnosis of prostate cancer. However, systematic biopsy has high false-negative rate and often misses anteriorly located tumors. Magnetic resonance imaging (MRI)-TRUS fusion biopsy can potentially improve cancer detection by better visualization and targeting of cancer focus. We evaluated the role of fusion biopsy in detection of prostate cancer and the association of prostate imaging reporting and data system (PI-RADS) score for predicting cancer risk and its aggression.Methods:Ninety-six consecutive men with suspected prostate cancer underwent MRI-TRUS fusion-targeted biopsy of suspicious lesions and standard 12 core biopsy from May 2014 to July 2015 in our institution. All patients underwent 3.0 T multiparametric MRI before biopsy. mp-MRI included T2W, DWI, DCE and MRS sequences to identify lesions suspicious for prostate cancer. Suspected lesions were scored according to PI-RADS scoring system. Comparison of cancer detection between standard 12 core biopsy and MRI-TRUS fusion biopsy was done. Detection of prostate cancer was primary end point of this study.Results:Mean age was 64.4 years and median prostate-specific antigen was 8.6 ng/ml. Prostate cancer was detected in 57 patients (59.3%). Of these 57 patients, 8 patients (14%) were detected by standard 12 core biopsy only, 7 patients (12.3%) with MRI-TRUS fusion biopsy only, and 42 patients (73.7%) by both techniques. Of the 7 patients, detected with MRI-TRUS fusion biopsy alone, 6 patients (85.7%) had Gleason ≥7 disease. Prostate cancer was detected on either standard 12 core biopsy or MRI-TRUS fusion biopsy in 0%, 42.8%, 74%, and 89.3% patients of suspicious lesions of highest PI-RADS score 2, 3, 4, and 5, respectively.Conclusions:MRI-TRUS fusion prostate biopsy improves cancer detection rate when combined with standard 12 cores biopsy and detects more intermediate or high-grade prostate cancer (Gleason ≥7). With increasing PI-RADS score, there is an increase chance of detection of cancer as well as its aggressiveness.
Ga-prostate-specific membrane antigen (PSMA) ligand PET/CT is commonly believed to be prostate specific as the name itself suggests. However, PSMA-expressing nonprostatic cancers as well as benign lesions are continuously being added to the list. We reported PSMA-expressing Schmorl's node in L1 vertebra of a 74-year-old man with prostate cancer. Prostate-specific membrane antigen expression in the vertebral lesion raised a metastatic suspicion. However, on analysis of previous CT scan done 2 years before, the same lesion was observed with no significant interval change, confirming its benign nature. With more use of PSMA ligand PET/CT, more limitations and false positivities will be explored.
Inflammatory myofibroblastic tumor (IMT) of lung is a rare tumor, accounting for ~0.7% of all lung tumors with varied clinical and radiological presentations. The origin of this tumor is unknown but some studies suggest that it might be a true neoplasm as some mutations on chromosome 2p23 of anaplastic lymphoma kinase (ALK) have been found to be related to this tumor. The morphology of IMT is quite vague and the histopathological diagnosis is predominantly given on excision specimens; in fact, only 6.3% of cases are diagnosed based on analysis of biopsy specimens alone. We illustrate a case of IMT diagnosed in a young male on core biopsy, where the case presented with a large tumor in the lung with metastases to multiple sites that was hence unresectable. Post 3 months of treatment with Crizotinib, there was significant reduction in the tumor size. Another interesting finding was that the ALK immunostain, which helped immensely in the diagnosis, was appreciated better on the Ventana platform rather than on the Dako platform.
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