Journal of Kidney Cancer and VHL最新文献

Renal angiomyolipoma (rAML) occurs rarely sporadically but is commonly encountered in patients with tuberous sclerosis complex and lymphangioleiomyomatosis. rAML is a rare entity, not seen regularly in daily practice; however, is commonly encountered and diagnosed by clinicians who approach and treat kidney masses. Basic knowledge of this entity is necessary to recognize that despite being benign, these tumors can rarely cause deadly complications such as hemorrhage or severe renal dysfunction or may have malignant components associated with them.

Primary renal chondrosarcomas are rare tumors that are high-grade in nature and, unfortunately, have poorly understood pathogenesis and extremely low prognosis. The coexistence of a discrete malignancy in the urinary bladder is even rarer, with the occurrence of distinct papillary urothelial carcinoma in the urinary bladder in this case. The clinical presentation is nonspecific, and the primary radiological investigations have a limited scope in providing specific diagnosis of this entity. The final diagnosis is possible on thorough histopathological examination of the resected specimen, which requires extensive sampling and meticulous reporting. As of now, the only way to achieve a better prognosis is by early diagnosis. It is necessary to keep the possibility of occurrence of sarcomas at rare sites in the differential diagnoses. The cytogenetic and molecular abnormalities associated with this entity need to be elucidated to achieve a more satisfactory outcome concerning the overall management of the patient.

Renal oncocytomas are asymptomatic, benign tumors often encountered incidentally on various imaging modalities. Renal oncocytomas comprise 5-7% of primary renal neoplasms and are derived from cells of the distal renal tubule. We present a case report of renal oncocytoma in a 22-year-old male having right-sided flank pain and symptomatic gross hematuria with a giant urinary bladder clot retention. The tumor was excised, and the patient underwent laparoscopic partial nephrectomy. Typical features of renal oncocytoma were observed upon histopathological examination of the resected specimen. The patient was catheterized, and bladder irrigation with clot retraction was performed.

Von Hippel-Lindau syndrome (VHL) is an autosomal dominant disease caused by a genetic aberration of the tumor suppressor gene VHL and characterized by multi-organ tumors. The most common neoplasm is retinal or cerebral hemangioblastoma, although spinal hemangioblastomas, Renal Clear Cell Carcinoma (RCCC), pheochromocytomas (Pheo), paragangliomas, Pancreatic Neuroendocrine Tumors (PNETs), cystadenomas of the epididymis, and tumors of the lymphatic sac can also be found. Neurological complications from retinal or CNS hemangioblastoma and metastases of RCCC are the most common causes of death. There is a strong association between pheochromocytoma and VHL syndrome, and pheochromocytoma is often a classic manifestation of the syndrome. RCCCs are often incidental and identified during other tests. Between 35 and 70% of patients with VHL have pancreatic cysts. These can manifest as simple cysts, serous cysto-adenomas, or PNETs with a risk of malignant degeneration or metastasis of no more than 8%. The objective of this retrospective study is to analyze abdominal manifestations of VHL from a surgical point of view.

After nephron-sparing surgery (NSS), postsurgical fatty tumor could be mistakenly reported as angiomyolipoma during radiologic imaging of some patients. In the present paper, we studied the postsurgical fatty tumor detected after NSS but not covered before in the literature. In addition, we also evaluated whether the postsurgical fatty tumor was related to the surgical technique employed. Patients admitted to the urology department of our university hospital from 2014 to 2019 and operated with open NSS were evaluated retrospectively. We detected those 156 patients were operated with NSS. Nine patients with angiomyolipoma as primary pathology and four patients with surgical border positivity were excluded from the study. The patients were divided into two groups based on the repair of tumor extraction region. In Group 1, fatty tissue was used for repair, and Group 2 is the primary repair group. In all, 143 patients (Group 1 = 79, and Group 2 = 64) were included in the study. No demographic and radiologic differences, such as number of patients, age, gender, positioning of tumor, mass localization, tumor diameter, and RENAL nephrometry scoring system, were detected between the two groups. Postsurgical fatty tumors were detected in 28 patients in Group 1 and in two patients in Group 2 (P < 0.001). In patients with negative surgical margins after partial nephrectomy, lesions that were radiologically detected mimicking as angiomyolipoma were defined as "postsurgical fatty tumor." This mass containing adipose tissue only neither depicted vascularization and enhancement nor increase in size for at least 1 year. We assumed that these lesions must be followed as benign lesions not requiring additional treatment.