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.
Highlights
Anaplastic lymphoma kinase (ALK) is stated to be a membraneassociated receptor kinase which belongs to insulin superfamily [1]
It has been iterated that the identification of anaplastic lymphoma kinase (ALK)-renal cell carcinomas (RCCs) is important or pertinent in that ALK inhibitors have been demonstrated to be effective with regard to treatment of ALK re-arrangement renal cell carcinoma
ALK Translocation-Associated Renal Cell Carcinoma is an uncommon carcinoma that tends to be sporadically reported in children with sickle cell trait, and adults who do not have sickle cell trait
Summary
Anaplastic lymphoma kinase (ALK) is stated to be a membraneassociated receptor kinase which belongs to insulin superfamily [1]. It has been iterated that the recognition of ALK alterations within neoplasms is important in view of the potential benefit of ALK inhibitors Despite this knowledge, it had been noted that screening for ALK re-arrangement within RCC has not been routinely undertaken in view of costeffectiveness problems. [3] It has been documented that out of the aforementioned genes, VCL-ALK Renal cell carcinoma had been described in children who have sickle cell trait as well as that TPM3 had been primarily reported as a partner in ALK-RCC [3]. Considering that less than 30 cases of ALK Translocation-associated renal cell carcinomas have been reported so far, it would not be surprising if majority of clinicians globally are not aware of the diagnostic features, clinical behaviour, management as well as outcome of the malignancy because most people globally would certainly have not encountered the tumour before. The ensuing review and update of the literature on ALK Translocation-associated Renal Cell Carcinoma is divided into two parts: (A) Overview, and (B) Miscellaneous narrations and discussions from some case reports, case series, and studies related to ALK TranslocationAssociated Renal Cell Carcinoma
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