Journal of Kidney Cancer and VHL <p>Journal of Kidney Cancer and VHL (ISSN: 2203-5826) is indexed in <strong>PubMed</strong>,&nbsp;Emerging Sources Citation Index&nbsp;of <strong>Web of Science</strong>, and the Directory of Open Access Journals (<strong>DOAJ</strong>). Journal is dedicated for the dissemination of research findings in kidney cancer and VHL.</p> en-US (​Dr. Ulka Vaishampayan) (Scott Bryant, Managing Editor) Fri, 03 Sep 2021 18:49:06 +0000 OJS 60 Optical Coherence Tomography Angiography of Early Stage 1a Retinal Hemangioblastoma in Von-Hippel-Lindau <p>Von-Hippel-Lindau (VHL) syndrome is characterized by focal vasoproliferative tumors of retinal capillaries called retinal capillary hemangioblastomas (RCH). These tumors are initially small and can be easily missed if not looked for carefully. As they grow, these tumors are more demanding to treat and hence the importance of detecting them early and treating them. Herein, we describe and review the optical coherence tomography angiography (OCTA) of the early-stage lesion, which suggested the involvement of superficial and a deeper retinal capillary plexus. In addition, to helping us detect these lesions earlier, OCTA may also help to understand the in vivo changes occurring at an earlier phase.</p> Ananya Goswami, Abhidnya Surve, Pradeep Venkatesh Copyright (c) 2021 Ananya Goswami, Abhidnya Surve, Pradeep Venkatesh Thu, 23 Sep 2021 00:00:00 +0000 Renal Metastases from ACC of the Nasal Cavity <p>Adenoid cystic carcinoma (ACC) is a rare malignant cancer that arises from secretory glands. Slow growth, perineural invasion, and late recurrences are the main characteristics of ACC. Only few cases of kidney metastases from ACC have been reported in the literature. We report here the case of a 66-year-old female patient who presented with bilateral renal metastases from ACC of the nasal cavity, detected 14 years after treatment of primary tumor and 6 years after metastasectomy of lung metastases. Histological examination confirmed diagnosis and the patient was treated with systemic chemotherapy. Radiological evaluation showed stability of the disease. However, a progression with occurrence of metastases in other sites (lung and bones) has been observed after 7 months. She is still receiving second-line chemotherapy. To the best of our knowledge, this is the second case of kidney metastases from ACC of the nasal cavity.</p> Jihene Feki, Maissa Lajnef, Manel Mallouli, Kheireddine Ben mahfoudh, Tahia Boudawara, Afef Khanfir Copyright (c) 2021 Jihene Feki, Maissa Lajnef, Manel Mellouli, Kheireddine Ben mahfoudh, Tahia Boudawara, Afef Khanfir Thu, 23 Sep 2021 00:00:00 +0000 Primary Renal Synovial Sarcoma Presenting as Haemorrhagic Shock <p>Primary synovial sarcoma (PSS) of the kidney is considered the rarest type of all renal sarcomas with specific chromosomal translocation t (X; 18) (p11.2; q11.2). We report the case of a 65-year-old man with no medical conditions who presented to the emergency department with sudden severe right flank pain associated with haemodynamic instability and haemorrhagic shock. Computed tomography (CT) of the abdomen and pelvis revealed a right renal mass. A right open radical nephrectomy was performed. Histopathology revealed a monophasic synovial sarcoma. The patient received six cycles of docetaxel and gemcitabine as adjuvant chemotherapy. No sign of recurrence was seen on a follow-up CT urogram. This rare tumour often presents atypically, and clear guidelines regarding appropriate treatment are lacking. Our case showed that treatment with docetaxel/gemcitabine after an open radical nephrectomy is promising.</p> Ibrahim Alzahrani, Nasser Albqami, Abdullah Alkhayal, Norah AlOudah, Ali Alyami Copyright (c) 2021 Ibrahim Alzahrani, Nasser Albqami, Abdullah Alkhayal, Norah AlOudah, Ali Alyami Fri, 03 Sep 2021 00:00:00 +0000 Histological Tracking into the Third Dimension <p>Using a novel three-dimensional (3D) approach, we tracked histological changes to elucidate the earliest stages of renal clear cell neoplasia in normal kidney tissue of patients with von Hippel-Lindau (VHL) disease. Tissue blocks of interest were procured, serially sectioned, and 3D reconstruction of the entirety of pathologic events was performed. The results reveal an abundance of foci with aberrant clear cell proliferation that initially develop along the tubular lining, but have the potential to aggregate within individual tubules. This stage is followed by the extension of clear cell aggregates beyond the tubular basement membrane, which allows for the recruitment of angiogenesis derived from interstitial vasculature. The results suggest that the most frequent pathologic event in VHL kidneys is the presence of isolated or aggregated clear cells within the tubular epithelium, potentially developing further into a protracted process of neoplasia. The abundance of independent pathologic events in VHL kidneys confirms developmental mechanisms to precede tumor initiation. To our knowledge, this is the first report demonstrating that tracking of histologic changes in the 3rd dimension enables the confirmation of the sequence of events from the earliest pathologic change in the VHL kidney to the neoplastic stage. This approach is not only useful for visualization and quantification of pathologic changes but also for targeted sampling allowing selective analysis of the earliest stages of clear cell carcinogenesis.</p> Mayyan Mubarak, Nayef Al Gharaibeh, Samuel Sommaruga , Jie Li, Alexander Vortmeyer Copyright (c) 2021 Dr. Mubarak, Professor Al Gharaibeh, Dr. Sommaruga, Dr. Li, Professor Vortmeyer Fri, 10 Sep 2021 00:00:00 +0000