Abstract Apocrine sweat gland carcinoma is a rare cutaneous adnexal neoplasm that may occur in the scalp, eyelid, and ear canal. More often, it presents as an asymptomatic, slow-growing, solid or cystic mass with variable color. The clinical appearance may be confused with basal cell carcinoma and the pathologic appearance with metastatic breast carcinoma. Supportive morphologic and immunohistochemical findings, together with careful clinicopathologic correlation, aid in diagnosis. The mainstay of treatment is wide local excision and neck dissection for node positive disease. The role of elective neck dissection and adjuvant radiotherapy remain controversial. Chemotherapy has been used for palliation in disseminated disease. This case report offers an algorithm that can aid the clinician in differential diagnosis of this often elusive neoplasm.
Keywords: apocrine carcinoma; adnexal neoplasm; scalp carcinoma; immunohistochemistry

Abstract Merkel cell carcinoma (MCC) is a rare malignant tumor of the skin with tendency to rapid local progression and frequent spread to regional lymph nodes. In this paper we retrospectively describe the atypical presentation of 5 cases of Merkel cell carcinoma observed in our surgical department in the last ten years. Four patients had buttock localization whilst one patient had primary nodal presentation. Since integration of Merkel cell polyomavirus (MCPyV) DNA into the tumor genome is frequently recorded in this type of cancer, we analyzed formalin-fixed paraffin embedded MCC tissue samples from our five patients for the presence of MCPyV DNA by means of polymerase chain reaction (PCR). MCPyV DNA was present in all five carcinomas. All patients were treated with wide surgical excision of the tumor and sentinel node biopsy. One patient had stage I disease, three patients had stage II disease, and one patient had stage III disease. Adjuvant radiotherapy was administered in all cases for local control. Chemotherapy was administered to the patient with primary nodal presentation and in stage III disease. Median time of follow-up was 84 months. None of the patients relapsed. Despite the low number of patients examined, our experience suggests that surgery is a necessary step whereas implementation of adjuvant therapy, radiotherapy and chemotherapy depends on individual risk assessment. Treatment outcome was very good, probably due to early detection of MCC.
Keywords: Merkel cell carcinoma; polyomavirus; DNA detection; chemotherapy

Abstract Cystic adrenal neoplasms are uncommon and may be incidentally discovered during radiologic procedure not directly performed for the evaluation of adrenal disease. Cystic pheochromocytomas are uncommon neoplasms that originate from the adrenal medulla, clinically differing from the more frequent solid pheochromocytomas. Here, we report a case of 72 year-old woman affected by asymptomatic pseudocystic pheochromocytoma (70x50mm), incidentally discovered, with a false negative ¹²³I-monoiodobenzylguanidine (¹²³I-MIBG) scintigraphy. Histopathological examination led to diagnosis of pseudocystic pheochromocytoma with typical architecture. The screening for germline mutation of succinate dehydrogenase complex subunits (SDHB, SDHC), Von Hipple-Lindau syndrome (VHL) and multiple endocrine neoplasia type 2 (RET) genes were negative, finding only some polymorphisms for RET and SDHB. We emphasize the relevance of an accurate pre-operative diagnosis, especially in rare diseases, and the requirement of experience and multidisciplinary management in these rare pathologies.
Keywords: adrenal cyst; pseudocystic pheochromocytoma; adrenal incidentaloma; false-negative ¹²³I-monoiodobenzylguanidine (MIBG) scintigraphy