Abstract

David Esteban Moreno¹, Graciela García Álvarez², Daniel Rivas Sánchez³, Daniela Gonsalves Pieretti⁴, Sofía Suarez Casanova⁴, Isabel Castro Rizos⁴, Ramón Molina Cruz⁴, Luis Alberto Glaría Enríquez⁴, Escarlata López Ramírez⁵

¹GenesisCare Toledo, Radiation Oncology , Toledo, Spain; ²GenesisCare Madrid, San Francisco de Asis, Radiation Oncology, Madrid, Spain; ³GenesisCare Malaga, Radiation Oncology, Malaga, Spain; ⁴GenesisCare Toledo, Radiation Oncology, Toledo, Spain; ⁵GenesisCare Madrid, Arturo Soria, Radiation Oncology, Madrid, Spain

Medulloblastoma is observed in 5-10% of patients with Gorling Sindrome (GS) and it usually occurs in the first two years of life. Radiotherapy administered for medulloblastoma in GS induces basal cell carcinomas (BCC) in the area of radiation, so radiotherapy has traditionally been contraindicated due to reports of BCC induction in GS.

We describe our experience with a patient with GS treated with radiotherapy. We have also performed a comprehensive literature review about the use of radiotherapy in BCC associated to GS. 

Our case is a 73-year-old woman diagnosed with GS at the age of 33. She had multiple BCCs on her scalp, face, head and back. Previous therapies included multiple surgeries (Mohs last surgery on complex flap scalp), topical treatments and radiation therapy to the back at the age of 40. The patient presented 8 new lesions on the scalp and she refused surgery. She was treated in october of 2019 with 55 Gy in 25 fractions with volumetric arcotherapy (VMAT) with 6MV photons over all lesions and a sequential boost with 6MeV electrons, 10Gy in 5 fractions in two lesions greater than 3cm in diameter at diagnosis.

We describe the flow of information through the different phases of the literature review in Image 1.

After 10 months of follow up, our patient had fully regression of the lesions (Image 2). After 17 months follow up, the patient presents vertigos pending study by otorhinolaryngology without secondary malignancy tumors.

In the literature review 5 studies show how the radiotherapy in pediatric age cause BCC, meningiomas and mediastinal lymphoma in the area of radiation. One case report shows an adult patient without secondary malignancy at 57 months follow-up and made a comprehensive literature review of radiotherapy outcomes in adult patients with GS. Another case series evaluated the use of radiotherapy in 3 adult patients with 17 BCCs treated and complete remission without carcinogenic effects was reached in all treated lesions, with a mean follow-up of 30.35 months. 

There is evidence that shows how radiotherapy can induce tumors in pediatric patients, so it is recommended to avoid the use of radiotherapy in these patient population. In the limited literature available, there is no evidence that radiotherapy induces tumors in adult patients with GS.

The recommend treatment for BCCs in GS patients is surgical excision, but CO2 laser, electrocauterization, cryotherapy and photodynamic therapy may be used. In addition, imiquimod cream 5%, topical 5-fluorouracil and vismodegib in locally-advanced and metastatic forms are other treatment options. However, in selected adult patients with BCC not candidates for other treatments, radiotherapy could also provide a favorable outcome.