Anatomic pathology selected abstracts

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Liver injury after SARS-CoV-2 vaccination: clinical features, treatment response, and outcome

A few cases of autoimmune hepatitis–like liver injury following SARS-CoV-2 vaccination have been reported. The authors assembled a large case series to evaluate clinical features, treatment response, and outcomes of liver injury in patients following such vaccination. They collected data from patients in 18 countries. The R-value was used to assess the type of liver injury. The study population was categorized according to features of immune-mediated hepatitis (positive autoantibodies and elevated immunoglobulin G levels) and use of corticosteroid therapy for liver injury. The authors identified 87 patients (63 percent of whom were female), who were a median age of 48 years (range, 18–79 years) at presentation. Liver injury was diagnosed a median of 15 days (range, 3–65 days) after vaccination. Fifty-one (59 percent) cases of liver injury were attributed to the Pfizer-BioNTech (BNT162b2) vaccine, 20 (23 percent) to the Oxford-AstraZeneca (ChAdOX1 nCoV-19) vaccine, and 16 (18 percent) to the Moderna (mRNA-1273) vaccine. Liver injury was predominantly hepatocellular (84 percent), and 57 percent of patients showed features of immune-mediated hepatitis. Corticosteroids were more often given to patients who had grade 3–4 liver injury than to those who had grade 1–2 liver injury (88.9 versus 43.5 percent; P=.001). They were also more often given to patients who had immune-mediated hepatitis than to those who did not (71.1 versus 38.2 percent; P=.003). Liver injury was resolved in all patients, except one (1.1 percent) man who developed liver failure and underwent liver transplantation. Steroid therapy was withdrawn during the observation period for 12 (26 percent) patients after complete biochemical resolution. None of the patients relapsed during follow-up. The authors concluded that SARS-CoV-2 vaccination can be associated with liver injury. Corticosteroid therapy may benefit those with immune-mediated features or severe hepatitis. Overall outcome was generally favorable, but vaccine-associated liver injury led to fulminant liver failure in one patient.

Efe C, Kulkarni AV, Terziroli Beretta-Piccoli B, et al. Liver injury after SARS-CoV-2 vaccination: Features of immune-mediated hepatitis, role of corticosteroid therapy and outcome. Hepatology. 2022;76(6):1576–1586.

Correspondence: Dr. Cumali Efe at scumaliefe@gmail.com

Comparison of malignant rhabdoid tumors of the vulva to epithelioid sarcomas

It has been suggested that most, if not all, extrarenal rhabdoid tumors of the vulva represent proximal-type epithelioid sarcomas. To better understand rhabdoid tumors of the vulva, the authors studied the clinicopathologic, IHC, and molecular features of eight such tumors and 13 extragenital epithelioid sarcomas. IHC analysis for cytokeratin AE1/AE3, epithelial membrane antigen, S100, CD34, ERG, smooth muscle actin, desmin, and SMARCB1 (INI1) was performed. An ultrastructural study of one vulvar rhabdoid tumor was conducted. Next-generation sequencing of the SMARCB1 gene was performed in all cases. The eight vulvar tumors occurred in adult women (mean age, 49 years). The tumors were poorly differentiated neoplasms with a rhabdoid morphology. The ultrastructural study showed large amounts of intermediate filaments (10 nm). All cases had loss of expression of INI1 and were negative for CD34 and ERG. One case showed two SMARCB1 mutations: c.592C>T in exon 5 and c.782delG in exon 6. Follow-up revealed that four patients died of disease, one was alive with disease, and three were alive without evidence of disease. Epithelioid sarcomas occurred in young adults (mean age, 41 years), most of whom were men. Seven tumors arose in the distal extremities, and the other six tumors had a proximal location. The tumors showed the characteristic granulomatous arrangement of neoplastic cells. The recurrent tumors were more proximal and often showed a rhabdoid morphology. All cases had loss of expression of INI1. CD34 and ERG were expressed by eight (62 percent) and five (38 percent) tumors, respectively. No SMARCB1 mutations were encountered. Follow-up revealed that five patients died of disease, one was alive with disease, and seven were alive without evidence of disease. The authors concluded that, based on the difference in their morphology and biological behavior, rhabdoid tumors of the vulva and epithelioid sarcomas are different diseases with distinct clinicopathologic features. Undifferentiated vulvar tumors with rhabdoid morphology should be classified as malignant rhabdoid tumors rather than proximal-type epithelioid sarcomas.

Espinosa I, D’Angelo E, De Brot L, et al. Malignant rhabdoid tumors of the vulva versus epithelioid sarcomas: a clinicopathologic, immunohistochemical, and molecular genetics study. Hum Pathol. 2023;135. https://doi.org/10.1016/j.humpath.2023.02.006

Correspondence: Dr. Jaime Prat at jpratdl@gmail.com