Miyagawa‐Hayashino, Aya

Abstract Context.—Sclerosing hemangioma is an unusual pulmonary tumor. Previously, 4 patients with pulmonary sclerosing hemangioma and lymph node metastases have been described in the literature. Objective.—To report 4 additional cases of metastatic sclerosing hemangioma. Design.—Retrospective review of the authors' consultation files and review of histologic sections of pulmonary tumors and lymph node metastases. Results.—Four cases of a morphologically benign pulmonary sclerosing hemangioma with regional lymph node metastases (including hilar, peribronchial, and interlobar metastases) were identified. The patients (3 female, 1 male) had a mean age of 39 years (range, 10–56 years). The tumors ranged in size (greatest dimension) from 1.5 to 4.7 cm (mean, 3.1 cm). The pulmonary tumors were typical circumscribed sclerosing hemangiomas without mitotic activity, angiolymphatic invasion, or necrosis. One tumor had focal cytologic atypia. The metastases were identified in hilar lymph nodes that were removed at operation for the lung nodule. One patient received adjuvant chemotherapy for adenocarcinoma. All of the patients are alive. No recurrences or residual disease has been detected at a mean follow-up of 4.7 years (range, 2.3–10 years). Conclusions.—On the basis of case data from the 4 patients described here and the 4 patients described previously, metastases to regional lymph nodes from pulmonary sclerosing hemangioma may occur but are rare and do not appear to affect prognosis.

Abstract Background Progressive familial intrahepatic cholestasis (PFIC) results in liver cirrhosis during the disease course, although the etiology includes unknown mechanisms. Some PFIC patients require liver transplantation (LT). Methods In this study, 11 patients with PFIC type 1 (PFIC1) and 3 patients with PFIC type 2 (PFIC2) who underwent living‐donor LT (LDLT) were evaluated. Results Digestive symptoms after LDLT were confirmed in 10 PFIC1 recipients (90.9%); 8 PFIC1 recipients showed steatosis after LDLT (72.7%), which began during the early postoperative period (71.5 ± 55.1 days). Seven of the eight steatosis‐positive PFIC1 recipients (87.5%) showed a steatosis degree of ≥80%, which was complicated with steatohepatitis and resulted in fibrosis. Cirrhotic findings persisted in six PFIC1 recipients even after LDLT (54.5%), and three PFIC1 recipients finally died. The survival rates of the PFIC1 recipients at 5, 10, and 15 years were 90.9%, 72.7%, and 54.5%, respectively. In contrast, the PFIC2 recipients showed good courses and outcomes without any steatosis after LDLT. Conclusions The clinical courses and outcomes after LDLT are still not sufficient in PFIC1 recipients owing to steatosis/steatohepatitis and subsequent fibrosis, in contrast to PFIC2 recipients. PFIC2 is good indication for LDLT. PFIC1 patients require LT during the disease course; therefore, we suggest that the therapeutic strategies for PFIC1 patients, including the timing of LDLT, under the donor limitation should be reconsidered. The establishment of more advanced treatments for PFIC1 patients is required to improve the long‐term prognosis of these patients.