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Rhabdoid meningiomas (RM) shows heterogeneous histological findings, and a wide variety of chromosomal copy number alterations (CNA) are associated with an unpredictable course of the disease. In this study, we analyzed a series of 305 RM samples from patients previously reported in the literature and 33 samples from 23 patients studied in our laboratory. Monosomy 22-involving the minimal but most common recurrent region loss of the 22q11.23 chromosomal region was the most observed chromosomal alteration, followed by losses of chromosomes 14, 1, 6, and 19, polysomies of chromosomes 17, 1q, and 20, and gains of 13q14.2, 10p13, and 21q21.2 chromosomal regions. Based on their CNA profile, RM could be classified into two genetic subgroups with distinct clinicopathologic features characterized by the presence of (1) chromosomal losses only and (2) combined losses and gains of several chromosomes. The latter displays a higher frequency of WHO grade 3 tumors and poorer clinical outcomes.

Congenital cytomegalovirus (CMV) infection can cause severe neurological sequelae or even fetal death. We present a 17-year-old pregnant woman with fetal CMV infection, leading to voluntary termination of pregnancy. Fetopsy demonstrated a brainstem hemorrhage and focal polymicrogyria. CMV inclusions were observed in the lung, liver, thyroid, pancreas, kidneys, adrenal, placenta, and central nervous system. Intracranial hemorrhage is a rare finding in the context of congenital CMV infection, with isolated brainstem hemorrhage being an exceptional form of presentation. Polymicrogyria appears to be a more frequent finding, although its actual incidence is unknown. Future studies are needed to determine the causal association., (© 2022 The Authors. Congenital Anomalies published by John Wiley & Sons Australia, Ltd on behalf of Japanese Teratology Society.)

Background: Heparan sulfate proteoglycans (HSPGs) promote amyloid-β peptide and tau fibrillization in Alzheimer's disease (AD) and provide resistance against proteolytic breakdown. Heparanase (HPSE) is the only enzyme that cleaves heparan sulfate (HS). Heparanase 2 (HPSE2) lacks HS-degrading activity, although it is able to interact with HS with high affinity., Objective: To analyze HPSE and HPSE2 expressions at different stages of AD., Methods: RT-PCR was used to analyze transcription levels of both heparanases at different stages of AD, and immunohistochemistry was performed to localize each one in different parts of the brain., Results: Both proteins appeared overexpressed at different stages of AD. Immunohistochemistry indicated that the presence of the heparanases was related to AD pathology, with intracellular deposits found in degenerated neurons. At the extracellular level, HPSE was observed only in neuritic plaques with a fragmented core, while HPSE2 appeared in those with compact cores as well., Conclusion: Given the involvement of HSPGs in AD pathology, there would seem to be a relationship between the regulation of heparanase expression, the features of the disease, and a possible therapeutic alternative.

Neurodegenerative diseases that course with dementia represent a public health problem that is growing in importance, mostly due to the aging of the population. Imaging techniques provide essential diagnostic and prognostic information. Some classifications of these diseases have recently been modified through the incorporation of histological, immunohistochemical, and genetic criteria. This article reviews the different radiological and pathological aspects of the most common diseases that course with dementia. We studied brains from our community's tissue bank with postmortem MRI and posterior histological examination and illustrate this exposition with images from the radiopathologic correlation.

Objective: Leiomyosarcoma of the prostate is an uncommon neoplasm with a poor prognosis. We review the three cases of leiomyosarcoma of the prostate observed in our hospital in the last twenty years and studied their clinical follow-up,, Methods: We have found three cases and we have studied their clinical follow-up, immunohistochemical profile and ultrastructural features., Results: In all cases tumor cells were positive for vimentin and also for either desmin or actin. Two cases were considered grade III sarcomas, with an aggressive course even with treatment, they died 5 and 24 months later, respectively. The third case, was considered grade II and is still alive, 60 months after diagnosis, without evidence of disease., Conclusions: Leiomyosarcoma of the prostate is an uncommon neoplasm that accounts for less than 0.1% of prostate tumors. We found no prognostic factors for predicting prolonged survival although complete resection and low mitotic activity may be predictive.

Objectives: Clinical and pathological study of eight cases of localized amyloidosis of the seminal vesicles. Review of the literature., Methods: Immunohistochemical and histochemical study in surgical specimens., Results: Two of the eight cases studied for amyloidosis in seminal vesicles, were obtained from radical prostatectomy specimens; patients had prior androgen deprivation therapy for two months. Four cases were obtained from radical cistoprostatectomy specimens because of urothelial carcinoma and the last two cases were diagnosed by transrectal prostatic needle biopsy which including a portion of seminal vesicle. Amyloidosis of seminal the vesicle is permanganate-sensitive; A-Amyloid, laminin, amyloid P protein and collagen IV negative., Conclusion: Localized amyloidosis ot the seminal vesicle is not an unusual finding. The incidence increases with age. The histochemical and immunohistochemical features are different from other amyloid deposits.

Objective: To report two cases of papillary cystadenoma of the epididymis., Methods: Clinical and pathological study of 2 male patients 48 and 26-year-old respectively, presenting with a solid, painful mass in the head of the epididymis. The nodules were removed., Results: Pathological analysis showed two papillary cystadenomas of the epididymis., Conclusions: Papillary cystadenoma of the epididymis is an epithelial tumor of unclear origin. It is associated with von Hippel-Lindau disease and infertility. They are uncommon and benign.

Objective: To report a case of primitive neuroectodermal tumor (PNET) of the kidney and review the literature and the 25 cases previously reported as PNET., Methods: A 39-year-old man who consulted for nephric colic is described. Ultrasound evaluation disclosed a mass arising from the left kidney. The clinical, radiological and pathologic features, treatment and differential diagnosis of small cell tumors are discussed, as well as the important role of immunohistochemical techniques (positive staining with O13 or 12E7 antibodies) and cytogenetic analysis [a characteristic chromosomal translocation t(11;22) (q24;q12) or variant translocation, such as t(21;22) (q22;q12), may be detected by fluorescence in situ hybridization (FISH) or polymerase chain reaction-reverse transcriptase (PCR-RT)]., Results: Survival of our patient was 20 months. Only three of the 25 previously reported cases had a longer survival: 60, 48 and 24 months. Mean survival was 10 months. 95.24% of the cases were positive for NSE. Immunostaining (CD99) was performed in 16 patients and was found to be positive in all cases. Cytogenetic and molecular analyses were performed in 11 cases; PCR-RT was negative in two, as well as in the case described herein., Conclusions: PNET is a highly aggressive neoplasm that tends to recur locally and to metastasize. Despite the poor response to standard therapy combining surgical resection, postoperative irradiation and chemotherapy, the results might change due to current research on genetic therapy based on creating antisense oligonucleotides against the EWS-FLI 1 fusion gene.

We report a case with synchronous presentation of inverted papilloma (I.P) and transitional cell carcinoma of the right renal pelvis (pT2G1) associated to urothelial carcinoma on left lateral wall of the bladder (T1G2). Urothelial inverted papilloma is an uncommon, generally benign tumor that account for 2.2% of all urothelial tumors. Although the preferred location is the bladder (90%), above all in trigone and bladder neck, also can be located at the UUT "upper urinary tract" (7-8%) and urethra (3%). A close follow-up is recommended after conservative therapy, mainly endoscopic procedure, due to likelihood of recurrence and synchronous or metachronous association with transitional cell carcinoma.

Objective: To review the treatment strategies for adrenal melanoma and to emphasize the role of curative surgical resection and adjuvant treatment in selected patients with melanoma metastatic to the adrenal gland versus chemotherapy alone in the treatment of patients with advanced malignant melanoma., Methods: A case of adrenal gland metastasis of a cutaneous melanoma (Clark IV, Breslow 5 mm.) treated by excision one year before that was referred to the Urology Department for Wünderlich syndrome is presented., Results: The analyzed series of programmed adrenalectomy for adrenal metastases from melanoma describe survivals of 26 (3), 36 (9), 59 (3) and 72 (5) months. In our case the patient died at home one month later due to stroke, although concomitant brain metastasis is suspected. Autopsy was not performed., Conclusions: In the differential diagnosis of an incidentaloma, metastatic disease is likely in a patient with a history of malignant disease. The frequency of malignant melanoma among metastatic adrenal disease varies between 1% and 8.6%; the majority are asymptomatic and incidental findings. We believe that in selected patients with advanced malignant melanoma, with no major coexisting morbidity factors who have isolated melanoma metastatic to the adrenal gland or with limited extra-adrenal sites of disease, curative surgical resection and adjuvant treatment may improve their survival. It must be emphasized that all patients should be followed after surgical resection of the primary tumor because it will facilitate staging of the disease and avoid emergency situations of ruptured friable metastases that make complete resection difficult.