Tuberous sclerosis complex (TSC) is a multisystem genetic disorder characterised by widespread hamartomas in organs such as the skin, brain, heart, lung, liver and kidney. Although associations of TSC with hamartomas, angiomyolipomas and fibromas have been reported, there has been no report of its association with malignant melanoma. Herein, we describe a 31-year-old man with malignant melanoma associated with TSC. The patient had a history of epilepsia, multiple hypomelanotic macules, periungual fibromas and multiple hepatic lesions. Malignant melanoma was diagnosed by hepatic biopsy. To the best of our knowledge, this is the first report of malignant melanoma coexisting with TSC in the literature. We also present and discuss the imaging findings, prognosis, underlying mechanisms and practical approaches in relation to the disease.
Adult ; Biopsy, Needle ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Magnetic Resonance Imaging ; methods ; Male ; Melanoma ; complications ; diagnosis ; therapy ; Multimodal Imaging ; methods ; Positron-Emission Tomography ; methods ; Rare Diseases ; Risk Assessment ; Skin Neoplasms ; complications ; diagnosis ; therapy ; Tomography, X-Ray Computed ; methods ; Tuberous Sclerosis ; complications ; diagnosis

Objective: : Postoperative data on Cushing’s disease (CD) are equivocal in the literature. These discrepancies may be attributed to different series with different criteria for remission and variable follow-up durations. Additional data from experienced centers may address these discrepancies. In this study, we present the results obtained from 96 endoscopic transsphenoidal surgeries (ETSSs) for CD conducted in a well-experienced center. Methods: : Pre- and postoperative data of 96 ETSS in 87 patients with CD were included. All cases were handled by the same neurosurgical team between 2014 and 2022. We obtained data on remission status 3−6 months postoperatively (medium-term) and during the latest follow-up (long-term). Additionally, magnetic resonance imaging (MRI) and pathology results were obtained for each case. Results: : The mean follow-up duration was 39.5±3.2 months. Medium and long-term remission rates were 77% and 82%, respectively. When only first-time operations were considered, the medium- and long-term remission rates were 78% and 82%, respectively. The recurrence rate in this series was 2.5%. Patients who showed remission between 3−6 months had higher longterm remission rates than did those without initial remission. Tumors >2 cm and extended tumor invasion of the cavernous sinus (Knosp 4) were associated with lower postoperative remission rates. Conclusion : Adenoma size and the presence/absence of cavernous sinus invasion on preopera-tive MRI may predict long-term postoperative remission. A tumor size of 2 cm may be a supporting criterion for predicting remission in Knosp 4 tumors. Further studies with larger patient populations are necessary to support this finding.