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We report a 38-year-old female with a history of primary pelvic PEComa and subsequent metastatic PEComa to the mediastinum who presented with recurrent mediastinal metastasis in April 2021. At initial presentation in 2010, the patient reported a three-month history of abdominal pain, hot flashes, night sweats, and a one-day history of bloating. Surgery revealed an 18-cm multilobulated pelvic mass involving the posterior uterus, bladder, and bilateral adnexa. Histology showed spindled and epithelioid tumor cells immunopositive for HMB-45, desmin, TFE3, SMA, caldesmon, Melan-A and calretinin. Cytokeratin, S-100, inhibin, myogenin, and OCT3/4 immunostains were negative. In 2013 the patient re-presented with chest pain. Imaging confirmed a mediastinal mass involving the pericardium with histology resembling the previous pelvic mass. Following initial resection and mTOR therapy, the mediastinal mass recurred once in 2015 with similar histologic findings. This case provides the first description of a malignant pelvic PEComa metastasizing to the mediastinum, and demonstrates the challenges associated with diagnosing metastatic PEComa. Though malignant PEComa with metastasis to the mediastinum is rare, it is important to recognize the natural process of this tumor to ensure adequate follow-up and patient care.

Combined hepatocellular-cholangiocarcinoma (cHCC-CCA) is a rare primary liver carcinoma displaying both hepatocytic and cholangiocytic differentiation within the same tumor. Relative to classic hepatocellular carcinoma (HCC), cHCC-CCA has more aggressive behavior and a poorer prognosis. Though recent advances have improved our understanding of the biology underlying cHCC-CCAs, they remain diagnostically challenging for pathologists because of their morphologic and phenotypic diversity. Accurate diagnosis of cHCC-CCA is important for patient management and prognostication. Herein, we review recent updates on cHCC-CCA, focusing on tumor classification, pathology, and diagnostic approach.

* Context.--Grade Group assessed using Gleason combined score and tumor extent is a main determinant for risk stratification and therapeutic planning of prostate cancer. Objective.--To develop a 3-dimensional magnetic resonance imaging (MRI) model regarding Grade Group and tumor extent in collaboration with uroradiologists and uropathologists for optimal treatment planning for prostate cancer. Design.--We studied the data from 83 patients with prostate cancer who underwent multiparametric MRI and subsequent MRI--transrectal ultrasound fusion biopsy and radical prostatectomy. A 3-dimensional MRI model was constructed by integrating topographic information of MRI-based segmented lesions, biopsy paths, and histopathologic information of biopsy specimens. The multiparametric MRI--integrated Grade Group and laterality were assessed by using the 3-dimensional MRI model and compared with the radical prostatectomy specimen. Results.--The MRI-defined index tumor was concordant with radical prostatectomy in 94.7% (72 of 76) of cases. The multiparametric MRI--integrated Grade Group revealed the highest agreement (weighted j, 0.545) and a significantly higher concordance rate (57.9%) than the targeted (47.8%, P=.008) and systematic (39.4%, P=.01) biopsies. The multiparametric MRI--integrated Grade Group showed significantly less downgrading rates than the combined biopsy (P = .001), without significant differences in upgrading rate (P = .06). The 3-dimensional multiparametric MRI model estimated tumor laterality in 66.2% (55 of 83) of cases, and contralateral clinically significant cancer was missed in 9.6% (8 of 83) of cases. The tumor length measured by multiparametric MRI best correlated with radical prostatectomy as compared with the biopsy-defined length. Conclusions.--The 3-dimensional model incorporating MRI and MRI--transrectal ultrasound fusion biopsy information easily recognized the spatial distribution of MRIvisible and MRI-nonvisible cancer and provided better Grade Group correlation with radical prostatectomy specimens but still requires validation. [ABSTRACT FROM AUTHOR]