Late antibody-mediated rejection in a kidney transplant recipient: COVID 19 induced?

BACKGROUND: Antibody-mediated rejection (AMR) was described in kidney transplant patients after viral infections, such as the cytomegalovirus. Very few cases were recently reported after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, probably in the context of lowering of immunosuppressive therapy. To date, no direct immunological link was proved to explain a connection between the coronavirus disease 19 (COVID-19) infection and antibody-mediated rejection (AMR) if it exists. CASE PRESENTATION: Here we try to find this association by presenting the case of a low immunological risk patient who presented, six years post-transplant, with c4d negative antibody mediated rejection due to an anti-HLA-C17 de novo donor specific antibody (DSA) after contracting the coronavirus disease 19. The HLA-Cw17 activated the antibody-dependent cell-mediated cytotoxicity via the KIR2DS1 positive NK cells. DISCUSSION AND CONCLUSIONS: This case report may prove a direct role for COVID-19 infection in AMRs in the kidney transplant recipients, leading us to closely monitor kidney transplant recipients, especially if they have "at-risk" donor antigens.

Long-term extracranial metastatic relapse of an intraventricular solitary fibrous tumor: a case report.

Solitary fibrous tumors (SFTs), an uncommon subtype of soft tissue sarcomas mesenchymal, are frequently diagnosed in the intra-thoracic region. Distant metastasis is detected in 10-40% of SFT patients with a predilection site to the lungs; nevertheless, these can occur rarely in the liver, brain, and bones. This entity was also reported in the abdomen, trunk, head, and neck, extremities but also, in the central nervous system (CNS). In the latter form of SFTs, the meninges were mostly affected while only a few cases were reported in the intraventricular and intraparenchymal region with distinct clinical and behavioral characteristics. In this article, we report the rare case of a relapsing intraventricular SFT with secondary extra-cranial metastases to the bones and soft tissues after 11 years of initial diagnosis, with focus on the available data in the literature on CNS SFTs.

Chemotherapy in the management of periosteal osteosarcoma: A narrative review.

Periosteal osteosarcoma (PO), an intermediate-grade chondroblastic osteosarcoma (OST) arising from the surface of the bones, is a rare histological subtype among primary bone sarcomas, most commonly diagnosed in young patients. It is characterized by distinct specific radiological and pathological features. The current management strategy is based on several case reports and series, without any solid international recommendations. Most sarcoma experts agree on the crucial role of an optimal complete surgical approach. However, with the paucity of available reports, the role of systemic treatment and its timing remains debatable. With this paper, we will review the available data on the actual impact of chemotherapy in PO patients with emphasis on the radiological, pathological, and therapeutic characteristics of this rare entity.

An Unusual Course of a 2,8-Dihydroxyadeninuria Crystalline Nephropathy Secondary to Adenine Phosphoribosyltransferase Deficiency.

Adenine phosphoribosyltransferase (APRT) deficiency is a rare disorder caused by an autosomal recessive genetic disease leading to the deposition of 2,8-dihydroxyadenine (2,8-DHA) in the kidney. The disease remains under-recognized, oftentimes diagnosed in late stages of renal insufficiency or a failed kidney allograft with biopsy-proven disease recurrence. Here, we present the case of a 59-year-old middle eastern male patient diagnosed with 2,8-DHA nephropathy after a very unusual presentation, and we show how the initiation of an appropriate therapy slowed down his evolution toward kidney replacement therapies. His disease was found to be secondary to a specific APRT gene variant c.188G>A p (Gly63Asp) also described in 4 other patients, all from middle eastern origins.

Pathogenic ATM Variant-Harbouring Well-Differentiated Aggressive Type 1 Gastric Neuroendocrine Tumour with High-grade Features (G3 NET): a New Addition to the Clinical and Pathological Spectrum of Gastric Neuroendocrine Neoplasms.

Gastric type 1 neuroendocrine tumours are considered to have low rates of proliferation and a good prognosis. We report here a patient with an aggressive well-differentiated high-grade gastric neuroendocrine tumour (gastric grade 3 NET), in a context of autoimmune gastritis. Consistent with grade 3 disease, the tumour had a Ki-67 proliferation index of 30%. Targeted next-generation sequencing identified variants of four genes, including a pathogenic ATM variant underlying the differentiation and metastatic potential of the tumour. Liver metastasis was diagnosed during follow-up, and the patient died after 6 years, due to disease progression.

[Acrometastasis revealing a pulmonary adenocarcinoma: Report of a case with unusual histopathological findings]. / Acrométastase révélatrice d'un adénocarcinome pulmonaire : à propos d'un cas de présentation histopathologique inhabituelle.

Acrometastasis are rare and can be exceptionally indicative of an occult carcinoma. The prognosis is generally poor. The radiological and immunohistochemical findings can be of great value to determine the primary and to guide treatment. We report a case of a 56-years-old man with acrometastasis at the fourth finger of the left hand revealing a pulmonary adenocarcinoma. Histopathological analysis showed a cribriform adenocarcinoma with an unusual cytoplasmic co-expression of TTF1 and Hepar-1 upon immunohistochemical analysis. There was no nuclear TTF1 immunostaining. Imaging explorations showed a 6-cm mass of the left superior pulmonary lobe. The patient received immunochemotherapy. Upon follow-up, there was evidence of disease progression on chest computed tomography scan.

New insights in gut-liver axis in wild-type murine imiquimod-induced lupus.

BACKGROUND: Intestinal and hepatic manifestations of lupus seem to be underestimated in comparison to other major organ lesions. Although recent data point to gut-liver axis involvement in lupus, gut permeability dysfunction and liver inflammation need to be more investigated. OBJECTIVE: This study aims to assess fecal calprotectin, intestinal tight junction proteins and liver inflammation pathway in wild-type murine imiquimod- induced lupus. METHODS: C57BL/6 mice were topically treated on their right ears with 1.25 mg of 5% imiquimod cream, three times per week for six weeks. Fecal calprotectin was collected at day 0, 22 and 45. Renal, liver and intestinal pathology, as well as inflammatory markers, intestinal tight junction proteins, and E. coli protein in liver were assessed at sacrifice. RESULTS: At six weeks, lupus nephritis was confirmed on histopathology and NGAL and KIM-1 expression. Calprotectin rise started at day 22 and persists at day 45. Protein expression of Claudine, ZO-1 and occludin was significantly decreased. E. coli protein was significantly increased in liver with necro-inflammation and increased TLR4, TLR7, and pNFκB/NFκB liver expression. CONCLUSION: This study is the first to demonstrate early fecal calprotectin increase and liver activation of TLR4- NFκB pathway in wild-type murine imiquimod-induced lupus.

Targeting CDK4 (cyclin-dependent kinase) amplification in liposarcoma: A comprehensive review.

Well-differentiated/dedifferentiated (WD/DD) liposarcomas, the most common form of liposarcomas, constitute up to 20 % of all soft tissue sarcomas. Several oncogenes are thought to be involved in liposarcoma pathogenesis, mainly MDM2, CDK4 and HMGA2. While MDM2 inhibitors are now tested in clinical trials, a second actionable and promising target appears to be cyclin-dependent kinase 4 (CDK4), which is amplified in up to 90 % of well differentiated or dedifferentiated (WD/DD) liposarcoma. With the paucity of available therapeutic options, the inhibition of CDK4 represent a potential therapeutic option. In this paper, we review the role of CDK4/6 inhibitors in targeting the commonly identified CDK4 amplification in WD/DD liposarcoma, with an emphasis on the published and currently ongoing trials.

TRK inhibition in soft tissue sarcomas: A comprehensive review.

Soft-tissue sarcomas (STS) are a group of rare mesenchymal tumors that constitutes ∼1% of all solid tumors. It remains a rare tumor which lacks effective treatment options. Precision oncology may be of interest in this regard by identifying potential targets for emerging novel therapies. Neurotrophic receptor tyrosine kinase (NTRK) fusions are rare oncogenic driver mutations found in a broad range of common and rare tumor subtypes including STS. The recent approvals of NTRK inhibitors (larotrectinib and entrectinib) represent new therapeutic options in the drug armamentarium especially valuable in advanced STS given the paucity of treatment options and the generally poor prognosis of these tumors. We review the methods used to detect NTRK fusions in STS with focus on incidence, diagnosis and management of these rare and intriguing oncogenic targets.

A comprehensive review on the diagnosis and management of intimal sarcoma of the pulmonary artery.

Only a few hundred cases of intimal sarcomas of pulmonary artery (ISPA) were reported on the literature. Diagnosis of this rare entity is a challenging dilemma with the need for a high expertise in the radiological and pathological identification of ISPA. Treatment strategies rely initially on an early aggressive surgery aiming for complete surgical resection with clear margins while no clear recommendations guiding the choice for additional drug therapy or radiotherapy exist. In this article, we perform an extensive review of the literature on ISPA with details on the clinical presentation, diagnosis and management strategies. An additional goal of this paper is to make practicing oncologists aware of this rare entity with clear idea on the initial management.

Clinicopathological and Molecular Study of Peritoneal Carcinomatosis Associated with Non-Small Cell Lung Carcinoma.

To retrospectively characterize the molecular features of Non-Small Cell Lung Carcinomas (NSCLC) with peritoneal carcinomatosis (PC), clinicopathological data of 12 patients diagnosed with NSCLC and PC between 2007 and 2016 were collected. Immunohistochemistry and Next Generation Sequencing (NGS) were performed on cases with available material. PC was the initial presentation of NSCLC in 17% of the cases. Overall, patients with PC displayed a poor median survival of 12 weeks. Histology was adenocarcinoma in 11 cases. 37.5% of cases showed PD-L1 immunostaining positivity (50% cut-off). ALK and ROS1 immunostainings were negative. Using NGS, we identified 17 molecular alterations in 9 genes (TP53, KRAS, STK11, BRAF, EGFR, DDR2, ERBB4, SMAD4, CTNNB1) in 88.9% of adenocarcinomas. To the best of our knowledge, 5 of these variants are not referenced in the literature. In conclusion, PC might be the initial presentation of NSCLC. Molecular profiling of our cases did not find any effective targetable alteration, except from high PD-L1 expression.

[Acinic cell carcinoma: an unsuspected malignancy of the nasal cavity]. / Une présentation inhabituelle d'un carcinome à cellules acineuses.

Acinic cell carcinoma (ACC) is a low grade malignant tumor of the salivary glands. Primary ACC affects most frequently the parotid gland and can rarely arise in the minor salivary glands of the oral cavity, pharynx and larynx. It is extremely rare in the nasal cavity; to our knowledge only 18 cases of primary ACC of the nasal cavity are reported in the English-written literature. Herein we report a case of acinic cell carcinoma of the nasal cavity, describe the clinical, radiological and microscopic features of this uncommon presentation and finally provide a discussion in the light of relevant literature.

[Cardiac intimal sarcoma: A case report of a rare tumor with peculiar histopathological findings]. / Le sarcome intimal cardiaque : tumeur rare illustrée par un cas de présentation histopathologique inhabituelle.

Primary cardiac sarcomas are rare tumors with poor prognosis. Intimal sarcoma, a mesenchymal malignant tumor described mainly in the great vessels, may rarely involve the heart. Herein we describe the case of a 70-years-old female who was found to have a left atrial mass during an investigation of a new onset dyspnea. The patient underwent surgery and the resected mass was found to be an intimal sarcoma. The objectives of this report were to describe a case of this rare disease entity and to discuss its pathological and molecular findings based on relevant literature.

Peritoneal Pulse Granulomas With Spiral Bodies Mimicking Peritoneal Carcinomatosis: A Case Report.

Pulse (hyaline ring) granuloma, a rare entity first described in lung and oral cavity, has been reported under various names before the identification of hyaline rings as fragments of pulses (the edible seeds of legumes). Similar lesions were thereafter described in extra-oral localizations, mainly the gastrointestinal tract, or localizations having potential communication with the gastrointestinal tract. Recently, 2 reports described "spiral bodies" surrounded by foreign body-type multinucleated giant cell reaction in pulse granulomas, corresponding to remnant plant vascular structures (helical xylem elements). In this article, we report a case of a 70-year-old male patient presenting to our hospital for an incisional hernia repair. He had a history of antrectomy 2 years previously for perforated duodenal ulcer complicated with fecal peritonitis. During the hernia repair procedure, multiple peritoneal whitish nodules and one subserosal appendiceal nodule were found. Appendectomy and biopsy of a peritoneal nodule were performed. Microscopic examination showed nodular lesions located in the subserosa to be pulse granulomas. Also surrounded by histiocytes, spiraled thin and rigid foreign bodies were identified. In this article, we report a case of pulse granuloma with spiral bodies complicating perforated duodenal ulcer and mimicking a peritoneal carcinomatosis. We also provide a discussion on the origin of spiral bodies in light of relevant literature.

Xanthomatous Posttraumatic Fibro-Osseous Lesion of the Rib: A Rare and Underrecognized Entity. Case Report and Literature Review.

Posttraumatic fibro-osseous lesion (PTFOL) is a rare lesion that typically affects the ribs and is probably a posttraumatic reactive process. Because PTFOL is often misdiagnosed as fibrous dysplasia, osteoid osteoma, benign fibrous histiocytoma or rib metastases, chest wall resection, leading to a significant morbidity, is the main treatment modality. We report the case of a 30-year-old male patient with no history of previous trauma presenting with chest pain. Computed tomography scan showed an eighth left rib well-defined ovoid and hypodense lesion with circumferential sclerotic margin and no cortical breakthrough. Posterolateral thoracotomy was performed and a histological diagnosis of xanthomatous posttraumatic fibro-osseous lesion of the rib was made. PTFOL is a benign lesion that should be recognized to avoid unnecessary surgical treatment and complications. We provide a summary of clinical, histopathological, and radiological aspects of PTFOL and discuss differential diagnoses.

Atrial angioleiomyoma with myopericytoma-like features: a case report.

A 66-year-old female patient was referred to our hospital for resection of a right atrial mass. Four months earlier, she had suffered an acute cerebrovascular accident due to occlusion of the sylvian segment of the right middle cerebral artery from atheromatous tight stenosis in the right internal carotid artery. Later, investigations with transthoracic and transesophageal echocardiography revealed a 3.4-cm right atrial mass that was resected surgically. Microscopic evaluation revealed a well-circumscribed nodular tumor, located within the interatrial septum, and corresponding to an angioleiomyoma (ALM). This tumor differs histologically from atrial myxoma. ALM is a ubiquitous benign tumor but has never been reported to occur in the atrium. ALM can mimic cardiac myxoma and should be considered in the differential diagnosis of atrial tumors.