Sudden death due to biventricular non-compaction cardiomyopathy in a 14-year-old.

A 14-year-old African-American female with a long-standing medically and ablation-treated history of tachycardia with associated seizures died suddenly. Upon autopsy, evidence of gross non-compaction involving the left ventricle, as well as possible subtle non-compaction of the right ventricle was discovered. Microscopically, there was focal myocyte hypertrophy as well as myxoid connective tissue and subendocardial fibroelastosis in the areas affected by the non-compaction. Arrhythmia, precipitated by the underlying cardiomyopathy, led to this young girl's death. Non-compaction cardiomyopathy is a rare cardiac disorder which commonly goes undiagnosed until post-mortem, although diagnosis through echocardiogram, CT, or MRI is possible and there is criterion for diagnosis with each of these.

Progression of Cystadenoma to Mucinous Borderline Ovarian Tumor in Young Females: Case Series and Literature Review.

STUDY OBJECTIVE: To study the progression of benign ovarian lesions to mucinous borderline ovarian tumors (mBOTs); analyze the clinicopathologic features, diagnosis, and management of mBOTs in pediatric and adolescent girls; and provide a review of the literature on mBOTs in this population. DESIGN: Retrospective chart review of female adolescents younger than 18 years diagnosed with mBOTs between July 2017 and February 2021. SETTING: Yale New Haven Hospital, New Haven, Connecticut; and Yale New Haven Health Bridgeport Hospital, Bridgeport, Connecticut. PARTICIPANTS: Three female patients diagnosed with mBOTs between ages 12 and 17 years. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Clinical presentation, preoperative characteristics, surgical technique, histology, tumor stage, treatment, progression, outcome, and rate of recurrence. RESULTS: Three adolescent patients were identified to have mBOTs. All three patients presented with a chief complaint of abdominal pain. One of the 3 patients was premenarchal at presentation. Two of the 3 patients were initially diagnosed with a mucinous cystadenoma and had recurrences of an ovarian cyst in the same ovary within 5 and 17 months, respectively. Pathology of the recurrent cyst was consistent with mBOT. Two of the 3 patients initially underwent cystectomy, and all ultimately had a unilateral salpingo-oophorectomy. Subsequent surveillance over 2 to 4 years found no evidence of disease recurrence. CONCLUSION: mBOTs are rare in the pediatric and adolescent population and could arise from benign ovarian tumors.

Intracystic Papillary Neoplasm of Gallbladder Mimicking Metastatic Malignancy on PET/CT.

ABSTRACT: Intracystic papillary neoplasm of the gallbladder is a rare preinvasive neoplastic lesion with similar characteristics as intraductal papillary mucinous neoplasm and other papillary neoplasms of pancreaticobiliary system. We report a case of 48-year-old woman with a history of recurrent right flank chondrosarcoma and gallbladder lesion on MRI and PET/CT interpreted as indeterminate for metastatic disease. Subsequent cholecystectomy showed intracystic papillary neoplasm. With gallbladder lesions being rare on PET/CT, this case illustrates the importance of considering both primary and secondary tumors in the differential diagnosis.

Inverse p16 and p63 expression in small cell carcinoma and high-grade urothelial cell carcinoma of the urinary bladder.

Small cell carcinoma (SmCC) of the urinary bladder is a rare, highly aggressive neoplasm. The diagnosis is usually made on morphologic grounds, with the help of immunohistochemistry to document neuroendocrine differentiation. However, neuroendocrine markers generally have low sensitivity, ranging between 30-70%. Recent studies have reported p16 over-expression in SmCC of the lung, suggesting that p16 immunohistochemistry may be useful in the diagnosis of bladder SmCC. This is the first study to analyze the usefulness of p16 in the distinction of small cell and high grade urothelial cell carcinoma (HG-UCC). Fourteen cases of SmCCs and sixteen cases of HG-UCC of the bladder were stained with p16, p63, cytokeratin 20 (CK20), cytokeratin 7 (CK7), chromogranin (Chr), synaptophysin (Syn), and CD56. P16 expression was significantly higher in SmCCs (92.8%) when compared to HG-UCCs (43.7%). P63 and CK20, on the other hand, were positive in the majority of HG-UCCs (81.3% and 50%, respectively), while only 14.3% of SmCCs showed focal immunoreactivity with CK20. The sensitivity of the traditional neuroendocrine markers was low, ranging between 28.6% (Chr) and 71.4% (CD56) in SmCCs. P16 positivity in the absence of p63 and CK20 is highly characteristic of SmCC, while p63 and CK20 positivity with or without p16 expression is typical of HG-UCC.

Skolem and pessimism about proof in mathematics.

Attitudes towards formalization and proof have gone through large swings during the last 150 years. We sketch the development from Frege's first formalization, to the debates over intuitionism and other schools, through Hilbert's program and the decisive blow of the Gödel Incompleteness Theorem. A critical role is played by the Skolem-Lowenheim Theorem, which showed that no first-order axiom system can characterize a unique infinite model. Skolem himself regarded this as a body blow to the belief that mathematics can be reliably founded only on formal axiomatic systems. In a remarkably prescient paper, he even sketches the possibility of interesting new models for set theory itself, something later realized by the method of forcing. This is in contrast to Hilbert's belief that mathematics could resolve all its questions. We discuss the role of new axioms for set theory, questions in set theory itself, and their relevance for number theory. We then look in detail at what the methods of the predicate calculus, i.e. mathematical reasoning, really entail. The conclusion is that there is no reasonable basis for Hilbert's assumption. The vast majority of questions even in elementary number theory, of reasonable complexity, are beyond the reach of any such reasoning. Of course this cannot be proved and we present only plausibility arguments. The great success of mathematics comes from considering 'natural problems', those which are related to previous work and offer a good chance of being solved. The great glories of human reasoning, beginning with the Greek discovery of geometry, are in no way diminished by this pessimistic view. We end by wishing good health to present-day mathematics and the mathematics of many centuries to come.