Background: Brooke–Spiegler Syndrome is a rare genetic autosomal dominant disorder with variable penetrance. Its main feature consists of the development of multiple adnexal tumors that originate from the follicular-sebaceous-apocrine unit, most commonly: cylindromas, trichoepitheliomas and spiradenomas. Case presentation: We present four cases of Brooke–Spiegler Syndrome found in our clinic, as well as their clinicopathological traits and the surgical techniques used in their management. The familial history of three of the presented cases supports the genetic component of the disease. Cylindromas, spiradenomas and trichoepitheliomas coexisted in one of the cases presented. The therapeutic options used were electrocautery, CO2 laser, as well as tumor debulking followed by closure with metal staples. Discussion: The treatment remains a challenge and must be individualized based on the type, location and number of the lesions. Conservative methods such as CO2 laser and tumor debulking accompanied by closure with metal staples remain a viable option taking into account the large number of lesions. As patients usually develop multiple neoplasms throughout their lifetime, repeated procedures may be needed. Conclusion: Considering the few numbers of Brooke–Spiegler syndrome cases in the current literature, the authors report these patients in order to increase awareness and to help establish the most appropriate approach in managing the disease.

Angiosarcomas are rare, aggressive tumours with a propensity for the head and neck region. Surgery is the mainstay of treatment of localised angiosarcoma, but in unresectable or metastatic disease, the treatment is much more challenging. Traditionally, chemotherapy (Paclitaxel in particular) has been the treatment of choice. Evidence from case reports and small trials shows that PD1 inhibitors have activity against angiosarcomas, despite the lack of a large clinical trial data demonstrating definitive efficacy. The present case exemplifies the susceptibility of this malignancy to anti‐PD1 agents. We report a 77‐year‐old male patient with an advanced, multifocal angiosarcoma of the frontotemporal and malar region. The patient was pretreated using the association of Propranolol and Endoxan and then Paclitaxel. Because of the lack of efficacy, the patient was started on Nivolumab as the third line of therapy. After five cycles of immunotherapy, an excellent response was achieved, with complete remission of the cutaneous lesions as well as a partial remission of the hepatic nodules. The duration of response is currently 14 months, the patient being still alive at the present time. This case suggests that immune checkpoint inhibitors may represent a viable therapeutic option that needs further exploration for the treatment of locally advanced, unresectable or metastatic cases of cutaneous angiosarcoma.