A ʺCosa Nostra- like fellowʺ with a Problem?

We present a 68-year-old man with a lesion dating from about 5 years, located near the right eye. Within the anamnesis made an impression that the person behaves incredulously, strangely and does not want to give detailed data during the anamnesis in terms of his profession, place of residence, as well as previous hospitalizations. We were also impressed by his unwillingness to remove his strange glasses. There were also some scarce anamnestic data for repeated sunburns as a teenager and a document for cytological investigation with data indicative for Basal cell carcinoma. The patient was hospitalized for clinical clarification of the diagnosis and determination of the most appropriate therapeutic and diagnostic approach.


Case report
We present a 68-year-old man with a lesion dating from about 5 years, located near the right eye. Within the anamnesis made an impression that the person behaves incredulously, strangely and does not want to give detailed data during the anamnesis in terms of his profession, place of residence, as well as previous hospitalizations. We were also impressed by his unwillingness to remove his strange glasses. There were also some scarce anamnestic data for repeated sunburns as a teenager and a document for cytological investigation with data indicative for Basal cell carcinoma. The patient was hospitalized for clinical clarification of the diagnosis and determination of the most appropriate therapeutic and diagnostic approach.
Within the dermatological examination we observed the presence of a tumor-like formation located in the right periorbital area, just below the lateral corner of the lower eyelid and extending downwards towards the mandibular bone (Figures 1a,  1b, 1c, 1d), composed of many confluent grains with the color of the skin, with the presence of small blood vessels on its surface and a centrally located necrosis. Paraclinical parameters were without pathological changes. From the performed ultrasound of lymph nodes in the neck, head, axillary and inguinal -without data for metastasis, the performed radiographic examination of the lungs, abdominal ultrasound and CT head showed no pathological changes. From the collected anamnestic and available clinical data, we concluded that the finding was suspicious for High risky BCC. Possible Surgical treatment was denied by the patient and systemic therapy with Vismodegib has been also discussed.
Basal Cell Carcinoma (BCC) is one of the most common malignant cancers of the skin [1]. These tumors can easily be treated in their early stages of development by surgical removal [1]. The first-line treatment is the surgical eradication [1]. However, the larger the tumor is, the more extensive treatment is needed. Especially some locations (eyelids, nose, lips) pose a challenge to the surgeon, completely removing the tumor with the correct boundaries and at the same time maintain functionality and a satisfactory aesthetic appearance. In addition, large and neglected tumors, especially those located in the facial area, require complex reconstructive procedures after their removal [1]. The other therapeutic modalities include radiation therapy, phototherapy, and topical medications [2].
The first line of treatment is surgical removal, as over time the eye may leak and nearby bones may infiltrate, and due to the proximity of the angular veins and sinus cavernous, sinus cavernous thrombosis and / or meningoencephalitis may occur.
A small proportion of patients with periocular BCC develop a more extensive disease called locally advanced BCC, characterized by massive involvement of the eyelids or orbit [3]. Surgical resection of BCC is usually preferred, although there is no effective treatment for locally advanced or metastatic disease, as in the presented patient, due to the specific location of the tumor, radiation therapy is not possible, as well as surgical removal due to high risk of eyeball los and vision, respectively. The proposed possible treatment with Vismogedib has been also not accepted by the patient.
Vismodegib is indicated for the treatment of adults with symptomatic metastatic BCC or adults with locally advanced basal cell carcinoma who do not meet the requirements for surgery or radiotherapy approved based on a one-arm study (ERIVANCE) [4]. Vismodegib offers a new therapeutic alternative for extended BCC presentations. Molecular and genetic studies have shown that abnormal signaling by the Hedgehog pathway leads to uncontrolled basal cell proliferation [5].

Authors' contributions
We all provided clinical care for the patient, wrote and approved the manuscript.

Informed consent for publication
Written informed consent for publication was obtained from the patient.

Declaration of interest
We declare no competing interests.