Is There a Higher Incidence of Sporadic Renal Angiomyolipoma in Childhood Cancer Survivors?

Abstract

Background Cancer treatment can cause various long-term side effects, including those that impact ultrasound findings. During follow-up of childhood cancer survivors (CCSs), we often detected sporadic renal angiomyolipomas without histological confirmation (SAMLs), which is why we initiated this study. We compared the occurrence of SAML in CCSs to the previously reported data from a non-cancer population and correlated SAML with cancer treatment-related factors. Methods The cohort included 1098 CCSs (median age at cancer diagnosis (dg) 4.3 years) who had ultrasound follow-up (2014–2019). Of the CCSs, 525 (48%) were female, 132 (12%) had subsequent neoplasms (SNs), and 110 (10%) had genetic syndromes. CCSs were treated for lymphomas 269 (24%) and solid tumors 829 (76%). None of the CCSs had tuberous sclerosis complex (TSC). Results SAML developed in 48 (4.4%) CCSs; of these, 20 (42%) had SNs. The coincidence of SAMLs and SNs was found in CCSs with a follow-up period exceeding 20 years. The median age at SAML dg was 27.9 years (interquartile range (IQR) 22.3–34.1), and the median time to SAML dg was 22.6 years (IQR 17.4–27.6). Twenty-one (44%) CCSs developed multiple or bilateral SAMLs lesions; of these, six (12%) were in the radiotherapy field. SAML occurrence correlated with radiotherapy of the retroperitoneum (1.65-fold higher with 95% CI 0.90–3.02). The correlations with other cancer treatment factors and with female sex were less clear. Conclusion This study revealed the occurrence of SAMLs in CCSs to be 10 times higher than that in non-cancer studies. The current characteristics of CCSs with SAMLs: younger age, and more bilateral or multiple lesions are more similar to TSC associated angiomyolipoma. Moreover, we observed a coincidence of SAMLs with SNs. Our results support the hypothesis that SAML development in CCSs is not simply a late effect of therapy, and indicates other factors are involved in SAML development.

Possible Mechanisms of Subsequent Neoplasia Development in Childhood Cancer Survivors: A Review

“…The participation of genetic factors in the formation of SMN may also be evidenced by the frequent coincidence of malignant and benign SNs. In our group of 170 survivors with SMNs (4.2% of all survivors), 34 had both malignant and benign SN (0.8% of all survivors) [7] and we found coincidence of sporadic renal angiomyolipomas with SNs [55]. Moreover, incidence of sporadic renal angiomyolipomas in CCSs was approximately ten times higher than in the general population [55] The above-described results of clinical studies demonstrate the role of genetic background in the development of SMNs.…”

“…In our group of 170 survivors with SMNs (4.2% of all survivors), 34 had both malignant and benign SN (0.8% of all survivors) [7] and we found coincidence of sporadic renal angiomyolipomas with SNs [55]. Moreover, incidence of sporadic renal angiomyolipomas in CCSs was approximately ten times higher than in the general population [55] The above-described results of clinical studies demonstrate the role of genetic background in the development of SMNs. So far, we cannot detect all genetic risk factors for the development of SMNs, except for hereditary cancer syndromes, which occur rarely.…”

Premature aging in childhood cancer survivors (Review)

“…These patients are three times more likely to develop chronic diseases (e.g., subsequent neoplasms, cardiovascular diseases and endocrinopathies) compared with a control group of their siblings (8,9). In our recent study on CCSs (10), ultrasound sporadic renal angiomyolipomas were detected much earlier in CCSs (median age at diagnosis, 27.9 years) compared with that reported in the general population (50-60 years).…”

Spectrum of Presentations and Management Strategies in Renal Angiomyolipoma

“…In general, the prevalence is higher in women and the tumors tend to occur at a young age, usually in the fourth or fifth decade ( 9 – 11 ). Young-onset rAMLs have however been reported in patients with TS and childhood cancer survivors ( 12 ). The natural history of rAML is controversial and varies between studies.…”