A unique case of isolated thoracic spinal Rosai-Dorfman disease related to IgG4.

Rosai-Dorfman disease (RDD) is a rare benign histiocytosis usually characterized by massive cervical lymphadenopathy and systemic manifestations. Extranodal, especially spinal involvement, is extremely rare. Our case was deemed worthy of presentation because it was the first reported isolated case of spinal RDD related to IgG4 and mimicked meningioma clinically and radiologically. A case with an intradural extramedullary mass causing neurological compression findings in the thoracic spinal region and radiologically mimicking meningioma is presented. In the histomorphological examination of the resection material, polymorphonuclear leukocytes in the dura, histiocytes showing emperipolesis, an increase in collagenized fibrous connective tissue, and intense lymphoplasmacytic cell infiltration accompanied by obliterative phlebitis were observed. Immunohistochemically, the histiocytic cells were found to be S-100 protein, CD68, and CD163 positive and CD1a and langerin negative, and more than half of the plasma cells were immunoglobulin-G4 (IgG4) positive. Although rare, RDD or IgG4-related meningeal disease should be considered in the differential diagnosis of dural-based spinal masses that radiologically suggest meningioma. The pathologist should be aware that these two histopathological entities may coexist. To our knowledge, this is the first case of "isolated spinal RDD related to IgG4" reported in the literature.

Clinicopathologic and Prognostic Significance of CD47 Expression and Tumor-associated Macrophages in Endometrial Carcinoma.

Tumor-associated macrophages (TAMs) influence cancer progression. CD47 is an antiphagocytic molecule aiding tumor resistance against host immune surveillance. The relationship between CD47 expression and TAM-related microenvironment in endometrial carcinoma (EC) is poorly understood. The expression and prognostic significance of CD47 and CD163-labeled TAMs in 165 EC cases was assessed with CD47 and CD163 immunohistochemical studies. CD47 expression was found in 156/165 (94.6%) cases. CD47 expression was significantly higher in nonendometrioid carcinomas. CD47 overexpression was associated with histologic grade. High epithelial and stromal TAMs counts were also associated with high tumoral CD47 expression. High epithelial, stromal, and margin TAMs counts were associated with higher histologic grade and lymphovascular invasion. Epithelial TAMs counts were higher in patients with nonendometrioid carcinomas (P=0.0001) and cases with recurrence (P=0.018). High stromal TAMs counts were associated with deeper myometrial invasion (P=0.017) and the presence of distant metastasis (P=0.024). The counts of margin TAMs was significantly correlated with the depth of myometrial invasion, lymphovascular invasion, FIGO stage, lymph node metastases, distant metastasis, and recurrence (P=0.0001, 0.0001, 0.004, 0.005, 0.014, and 0.04, respectively). CD47 expression was not associated with overall survival (OS) and progression-free survival. However, high epithelial and stromal TAM counts were associated with shorter OS. Besides, high epithelial and margin TAM counts were associated with shorter progression-free survival. Furthermore, increased stromal and margin TAM counts were determined to be an independent prognostic marker of reduced OS. TAM count is, therefore, a significant prognostic factor in EC and the CD47 assessment has potential benefit for future clinical use.

Pretransplant Renal Arterial Vasculopathy of Donor Predicts Poor Renal Allograft Survival.

OBJECTIVES: Transplant vasculopathy is a significant predictor of poor outcome. We investigated whether age or pretransplant renal arterial vasculopathy of grafted kidneys affected allograft survival. MATERIALS AND METHODS: This study included 148 recipients and their donors. All donors underwent pretransplant renal arterial biopsy, with renal artery vascular score determined for each artery. Chronic rejection and graft loss were noted for all patients. RESULTS: Variable grades of pretransplant renal arterial lesions were noted in 103 donors (69.6%). A positive correlation was found between donor age and renal artery score (r = 0.650, P < .001), and chronic rejection and graft loss were found to increase with increasing score (P < .001). Recipient and donor age was significantly associated with graft loss and chronic rejection. With either younger or older donors, recipients had similar and best results regarding chronic rejection and graft loss if donors had renal artery scores of 0 or 1, but worse effects if donors had scores of 2 or 3. Five-year allograft survival rates for scores of 0, 1, 2, and 3 were 91%, 68%, 46%, and 33%. Univariate analyses showed that acute rejection episode (relative risk: 2.729, 95% confidence interval, 1.496-4.977; P = .001), older (? 50 y) donor age (relative risk: 1.970, 95% confidence interval, 1.038-3.736; P = .04), and donor renal artery score (relative risk: 2.466, 95% confidence interval, 1.382-4.401; P = .002) were associated with decreased allograft survival. Multivariate Cox analysis showed that only acute rejection episode (relative risk: 3.585, 95% confidence interval, 1.781-7.217; P < .001) and renal artery score (relative risk: 2.642; 95% confidence interval, 1.355-5.150; P = .004) were independent predictors of allograft survival. CONCLUSIONS: Pretransplant vasculopathy in donor renal artery implies a poor prognosis for renal allograft survival and is independent of other risk factors. Pretransplant renal artery biopsy is recommended for both deceased and living donors, and therapeutic interventions to modify transplant vasculopathy progression should start early posttransplant in recipients with affected renal arteries.

Traumatic Neuroma Causing Biliary Stricture After Orthotopic Liver Transplant, Treated With Hepaticojejunostomy: A Case Report.

Traumatic neuromas of the biliary tract have occasionally been reported to cause strictures at the cystic duct stump as a late complication of cholecystectomy with common bile duct exploration. The incidence of symptomatic traumatic biliary neuroma appears to be low after orthotopic liver transplant, as only 25 patients have been described previously in the English-language literature. Traumatic (amputation) neuroma is a reactive proliferation of pericholangial nerve fibers induced by injury, but it is not a true neoplasm. The diagnosis of traumatic neuroma is possible only by histopathologic examination; the diagnostic finding is a mass of hyperplastic nerve bundles. We report a patient with a traumatic neuroma causing an early biliary stricture with intrahepatic extension after an orthotopic liver transplant. The lesion failed to respond to repeated endoscopic stenting and eventually required hepaticojejunostomy. A biopsy of the liver graft, performed in the 13th month after transplant, showed chronic ductopenic rejection.

Histologic Changes in Bone Marrow Biopsies From Liver Transplant Patients.

OBJECTIVES: Liver transplant may complicated by various hematologic conditions, resulting in indication for bone marrow biopsy. Immunosuppressive therapies, specific infections, and secondary neoplasms affect bone marrow. In the present study, we evaluated the histologic spectrum of bone marrow findings in liver allograft recipients. MATERIALS AND METHODS: Of 338 patients who received liver transplants and were followed at the Baskent University, Faculty of Medicine, 44 patients underwent bone marrow biopsy. The medical and pathologic information about these patients were evaluated, including age at liver transplant, age at bone marrow biopsy, sex, primary disease, bone marrow histology, and indication for bone marrow biopsy. RESULTS: Of 44 patients who required bone marrow sampling, 30 were male (68.2%), and 14 were female (31.8%). Fifteen patients (34.1%) were in pediatric age group at the time of transplant. The most common cause of liver insufficiency leading to liver transplant was viral hepatitis in 11 patients (25%), followed by cryptogenic cirrhosis in 10 patients (22.8%). The source of the graft liver was a living donor in 40 patients (90.9%). The average age at transplant was 28.8 years, and the mean age at bone marrow sampling was 29.9 years. Nineteen patients (43.2%) required bone marrow sampling within the first year after transplant. The most common histologic findings were hypocellular, and normocellular bone marrow, observed in 18 patients (40.9%) each. Six patients (13.6%) had bone marrow biopsies for staging of posttransplant lymphoproliferative disorder. Only 1 patient of the 6 with this disease (16.7%) had malignant infiltration of the bone marrow, which was a case of Burkitt lymphoma developed as posttransplant lymphoproliferative disorder, and this was the only malignant infiltration in this patient group (2.3%). Neither specific infections nor granulomatous inflammation was detected. CONCLUSIONS: Bone marrow morphology has a major role in the follow-up of liver transplant patients, who may present with peripheral blood cytopenias. The present study represents the first systematic evaluation of bone marrow findings in liver allograft recipients.