Trichoblastic carcinoma arising in trichoepithelioma in Brooke-Spiegler syndrome.

Brooke-Spiegler syndrome (BSS) is a rare autosomal dominant disease characterized by the development of multiple cutaneous adnexal neoplasms namely cylindroma, trichoepithelioma and spiradenoma. The neoplasms associated with this syndrome are generally benign, but rarely they may undergo malignant transformation. A 63-year-old male presented with an ulcerated nodular lesion over glabella and multiple asymptomatic nodular lesions over face, scalp, chest and limbs. His father, grandfather and paternal cousins had history of similar lesions. Histopathological examination revealed trichoblastic carcinoma arising from trichoepithelioma over glabella and cylindroma on the chest. With these findings we arrived at a diagnosis of BSS with malignant transformation of trichoepithelioma. Trichoblastic carcinoma arising in trichoepithelioma in a patient with BSS is extremely rare with only a single case reported in literature.

Sinonasal Teratocarcinosarcoma- A Rare Tumour not so Rarely Misdiagnosed.

Sinonasal teratocarcinosarcoma (STCS) is a rare, morphologically heterogeneous and highly aggressive tumour of ambiguous origin. It is characterized by the presence of benign and malignant epithelial, mesenchymal and neuroectodermal components. Because of their rarity and heterogeneity, these lesions are often misdiagnosed, leading to management difficulties. Adequate sampling with a high index of suspicion is needed to diagnose this rare tumour. We reported here a 48-year old man with right nasoethmoidal mass eroding the cribriform plate with intracranial extension. An initial incisional biopsy was performed and a diagnosis of craniopharyngioma was made. Subtotal endoscopic excision of the mass revealed features of STCS. Immunohistochemistry confirmed the same. The patient was subsequently treated with radiotherapy. The histogenesis, histopathological features, immunohistochemistry findings, clinical features and treatment were discussed here. Till date, there are less than 100 cases reported in English literature.