Lymphoid interstitial pneumonitis (LIP), a frequent pulmonary complication in human immune deficiency virus (HIV)-infected pediatric patients, is characterized histologically by marked infiltration of lymphoid cells. Several theories have been suggested that LIP may be caused by Epstein-Barr virus (EBV). To identify the reservoir of EBV and pathogenesis of lymphoid infiltrates in HIV subtype E infected pediatric LIP, we examined the distribution and expression of EBV in the inflammatory cell recruitment in surgical lung biopsy-proven LIP from 9 vertically HIV subtype E-infected pediatric patients. The dominant microscopic feature of LIP demonstrated widespread widening of alveolar septum by mononuclear inflammatory cell infiltrate mainly composed of mature lymphocytes and plasma cells surrounding airways and expanding to the lung interstitium. EBV-encoded RNA (EBER) in situ hybridization, performed from paraffin-embedded lung tissues, revealed positive intranuclear signals in all 9 LIP cases. Interestingly, combined immunohistochemical and in situ hybridization analyses in 6 out of 9 LIP cases revealed 30% to 50% of the Langerhans and related dendritic cells were infected with EBV, whereas <30% of the T and B cells were infected with EBV. These results suggested that a chronic antigenic stimulus of EBV played important roles in the pathogenesis of LIP in these patients. This supports the notion that Langerhans cells (LCs) are more readily infected with EBV, indicating that LCs are reservoirs for EBV in lungs of HIV subtype E-infected pediatric LIP. And possibly LCs may play an important role in the recruitment of inflammatory cell infiltrates, especially T cells into these tissues. In addition, HIV may provide a milieu or microenvironment for the evolution of LIP, which represent an immunologic response to EBV infection. Interactions between LCs and related dendritic cells together with T cells are important for effective HIV and EBV replications.
Background Programmed death-ligand 1 (PD-L1) expression has now been implicated in gastric cancer (GC). However, data on PD-L1 expression in GC patients receiving standard-of-care are limited. Therefore, this study was conducted to determine the impact of clinicopathological characteristics on PD-L1 expression and its association with overall survival in Thai patients with GC. Methods We retrospectively collected clinical data on 268 patients with GC from Chiang Mai University Hospital between January 2018 to December 2021. PD-L1 expression was assayed by immunohistochemistry staining using the Dako 22C3 pharmDx. PD-L1 positivity was defined as the combined positive score (CPS) ≥ 1. Results The rate of PD-L1 positivity in Thai patients with GC was 22%. PD-L1 expression had no statistical correlation with gender, tumor location, diameter, pTNM stage, Lauren classification, or lymphatic and vascular invasion. However, PD-L1 positivity was significantly higher in patients younger than 55 than those older than 55 (32.6% vs. 16.5%, p = 0.035). In addition, PD-L1 positivity was observed more frequently in GC with metastases than without (28.6% vs. 20.8%, p = 0.694). Moreover, patients with PD-L1 positive had a significantly shorter median overall survival than those with PD-L1 negative (32.7 vs. 41.6 months, p = 0.042). Conclusions PD-L1 expression is evident in one-fourth of Thai patients with GC. The expression of PD-L1 in GC patients has been associated with young age, short survival, and metastases, although unrelated to the tumor stage. Therefore, testing GC tumors for PD-L1 expression is recommended to provide a therapeutic advantage in Thai patients.
Caroli syndrome is a developmental disorder caused by complete or partial arrest of ductal plate remodeling, leading to dilated bile ducts along with fibrosis surrounding the portal tracts. It is most commonly associated with autosomal recessive polycystic kidney (ARPKD). We report a unique case of Caroli syndrome, diagnosed prenatally at 24 weeks of gestation in a 29-year-old Thai woman. Ultrasound findings revealed the association of a fetal giant choledochal cyst with ARPKD. Autopsy findings showed ductal plate malformation, typical of Caroli syndrome, associated with giant choledocal cyst and ARPKD.
Objective:Rare disease Background:Primary adrenal epithelioid angiosarcoma (PAEA) is a very uncommon primary adrenal gland tumor that usually occurs around the age of 60 years and is more common among males. Owing to its rarity and histopathological features, PAEA could be misdiagnosed as adrenal cortical adenoma, adrenal cortical carcinoma, or other metastatic cancers, such as metastatic malignant melanoma and epithelioid hemangioendothelioma. Case Report:A 59-year-old male patient presented to our hospital with a complaint of abdominal bloating that started 2 months prior. His vital signs and the results of his physical and neurological examinations were unremarkable. A computed tomography scan showed a lobulated mass arising from the hepatic limb of the right adrenal gland but no evidence of metastasis to the chest or abdomen. The patient underwent right adrenalectomy, and the macroscopic pathological findings from a right adrenalectomy specimen revealed atypical tumor cells with an epithelioid appearance in the background of an adrenal cortical adenoma. Immunohistochemical staining was performed to confirm the diagnosis. The final diagnosis was epithelioid angiosarcoma involving the right adrenal gland with a background adrenal cortical adenoma. The patient had no postoperative complications, pain in the surgical wound, or fever. Therefore, he was discharged with a schedule for followup appointments. Conclusions:PAEA may be misinterpreted as adrenal cortical carcinoma, metastatic carcinoma, or malignant melanoma radiologically and histologically. Immunohistochemical stains are essential for diagnosing PAEA. Surgery and strict monitoring are the main treatments. In addition, early diagnosis is essential for patient recovery.
The concomitant occurrence of diabetes mellitus and cardiomyopathy secondary to occult malignant pheochromocytoma has rarely been reported. This case report describes the case of a 48-year-old female with a previous history of diabetes mellitus, hypertension, and cardiomyopathy who presented with fatigue and significant weight loss. Neither typical symptoms of pheochromocytoma nor metastatic symptoms were presented. Pheochromocytoma with extension to the liver was incidentally found from computed tomography of the abdomen and laboratory investigations during the work-up to identify the cause for the weight loss. Right adrenalectomy and a right hepatectomy were performed. Malignant pheochromocytoma was diagnosed based on pathology. All of her underlying conditions including diabetes mellitus, hypertension, and cardiomyopathy, were improved following the complete resection of the tumor. This case emphasizes the importance of early suspicion and diagnosis of malignant pheochromocytoma in individuals with atypical presentation of a chromaffin-secreting tumor.
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