Anaplastic Lymphoma Kinase (ALK) Translocation Re-arrangement Renal Cell Carcinomas: A Review and Update of the Literature

Abstract

Less than 50 cases of anaplastic lymphoma kinase (ALK) re-arrangement-associated renal cell carcinoma (ALK-RCC) been reported in the literature. ALK-RCC does affect children who have sickle cell trait and also adults with no evidence of sickle cell trait or sickle cell disease. ALK-RCC may be diagnosed incidentally or in individuals who have typical symptoms that simulate those or more common renal tumours.. Diagnosis of ALK-RCC does depend upon histopathology examination, immunohistochemistry studies as well as electron microscopy studies of nephrectomy / biopsy specimens of the kidney lesion that that show: typical pathology examination features of ALK-RCC as described within the text. Immunohistochemistry studies in ALK-RCC tend to show positive staining for: ALK, PAX8, INI1 (Intact), AE1/AE3, CAM5.2, CK7, EMA, LMWCK, P53, +Vimentin, CD10 (this tends to be variable), AMACR (this tends to be variable), and TFE3 (within the majority of the tumour cells). Majority of ALK-RCCs have been localized tumours at the time of initial diagnosis, but some cases of locally advanced tumours or tumours associated with metastases have been reported. There is no consensus opinion on the treatment of ALK-RCC but nephrectomy has been undertaken in most cases with good short term and medium term; nevertheless metastases and death have been reported. Response to utilization of to ALK inhibitor alectinib, as well as ALK Translocation Renal Cell Carcinoma does respond to Crizotinib. Improvements in the outcome of ALK-RCC could possibly be achieved through routine screening of sickle cell trait individuals, routine pre-operative per-cutaneous radiology imaging guided biopsies of all renal lesions, small localized tumours could be treated by (a) partial nephrectomy, or (b) radical nephrectomy, (c) cryotherapy, (d) radiofrequency ablation, (e ) irreversible electroporation plus / minus adjuvant therapy (radiotherapy, chemotherapy or ALK inhibitor plus or minus immunotherapy.