Syringocystadenocarcinoma papilliferum (SCACP), a rare skin adnexal carcinoma with apocrine differentiation is the malignant counterpart of syringocystadenoma papilliferum (SCAAP). It usually occurs in the head and neck region of elderly individuals. We describe a 46 year old south Indian female with a lesion in the scalp. Morphologically the tumour had the characteristic features of SCAAP along with frank invasion into deep dermis and malignant cytologic features. Immunohistochemically, the tumour cells stained strongly with cytokeratin (CK) 7, carcinoembryonic antigen (CEA), gross cystic disease fluid protein-15 (GCDFP-15), We made a diagnosis of SCACP in the patient, and a wide excision with skin grafting was performed on the patient.
Langerhans cell (LC) histiocytosis (LCH) is a rare disease predominantly affecting children and young adults, with an annual incidence between 4 and 5.4 per million individuals. Involvement of the thyroid by LCH is very rare, even in a multifocal disease. It can easily be confused on clinical grounds with other more common entities such as undifferentiated carcinoma, lymphoma, lymphocytic thyroiditis, chronic granulomatous thyroiditis, and cystic degeneration of multinodular goiter. Histology remains the most sensitive diagnostic modality. Immunohistochemical studies are extremely helpful in confirming the histologic impression because LCs are positive for S-100 protein and CD1a, and both markers can be used to assess formalin-fixed, paraffin-embedded tissue sections. Electron microscopy is also helpful by allowing identification of the pathognomonic Birbeck granules but not essential for diagnosis. Single-organ involvement in LCH is associated with excellent survival close to 100%. We report a rare case of isolated thyroid gland involvement by LCH.
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