UNASSIGNED: Spiradenocylindroma is a benign tumor of skin adnexal origin with overlapping features of two distinct neoplasms: spiradenoma and cylindroma. This cutaneous tumor typically presents on the head and neck and extracutaneous presentations are uncommon. The presentation described below involves a spiradenocylindroma within a mature ovarian teratoma is very rare.
UNASSIGNED: The aim of this article is to portray the diagnostic process of this unusual spiradenocylindroma presentation.
UNASSIGNED: A 65 year-old female with a left adnexal mass underwent ultrasonography and magnetic resonance imaging (MRI) which showed a left ovarian multiseptated lesion, with mural calcifications and projections into the mass. Excisional surgery was performed and histopathological examination revealed a spiradenocylindroma.
UNASSIGNED: Spiradenocylindroma is rare, hard to identify, and often misdiagnosed. Our study described the process of diagnosis and depicts the rare presentation of this lesion arising within a mature teratoma.

Aim of the report: Brooke-Spiegler syndrome (BSS) is a rare autosomal dominant disease characterized by the growth of cylindromas, spiradenomas, trichoepitheliomas, or their combination. These neoplasms usually begin in the second decade and progressively increase in number and size over the years. Diagnosis necessitates consideration of family history, clinical examination, histological findings, and genetic analysis. The aim of this paper is to explore the clinical overlap between Brooke-Spiegler syndrome (BSS) and neurofibromatosis type 1 (NF1). We aim to highlight the challenges associated with their differential diagnosis and emphasize the lack of standardized diagnostic criteria and treatment approaches. Case presentation: Hereby, we introduce the case of a 28-year-old male referred for suspicion of neurofibromatosis type 1 (NF1) who initially declined the recommended surgical excision for a scalp mass. After four years, he returned with larger masses of the scalp, and underwent excision of multiple masses, revealing cylindromas, spiradenomas, and spiradenocylindromas. Family history reported similar tumors in his father, who was also diagnosed with NF1 for the presence of multiple subcutaneous lesions on the scalp. Clinical overlap led to a genetic consultation, but testing for CYLD mutations yielded no significant variations. Despite this, the strong family history and consistent findings led to a revised diagnosis of Brooke-Spiegler syndrome, correcting the initial misdiagnosis of NF1 syndrome. Conclusions: Thanks to the evolving landscape of BSS research over the past two decades, its molecular underpinnings, clinical presentation, and histopathological features are now clearer. However, a thorough family history assessment is mandatory when BSS is suspected. It is our belief that a multidisciplinary approach and cooperation between specialists are essential when dealing with BSS. By sharing this case, we hope to underscore the importance of considering BSS as a differential diagnosis, especially in cases with atypical presentations or overlapping features with other syndromes like NF1.

UNASSIGNED: Spiradenocylindroma is an extremely rare entity composed by two distinct neoplasms in one lesion: spiradenoma and cylindroma. It may arose solitary or multiple, sporadic or familial and often affect the scalp. Surgical removal is curative and histopathological examination is mandatory for diagnosis.
UNASSIGNED: The aim of this article is to define the clinical features of spiradenocylindroma and its importance in the differential diagnoses of head and neck tumors.
UNASSIGNED: A 58 years-old female with a preauricolar painless, tender nodule presented to our attention. The patient under-went ultrasonography and MRI, which showed a non-specific cystic lesion. Surgery was performed and histopathological examination revealed a spiradenocylindroma. A 3-years disease-free follow-up was achieved.
UNASSIGNED: Spiradenocylindroma is often misdiagnosed and, in our study, we highlight its role in the differential diagnoses of head and neck masses.