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Syringocystadenoma papilliferum (SCAP), apocrine gland cyst (AGC, also called apocrine hidrocystoma or apocrine cystadenoma), and tubular papillary adenoma (TPA) with apocrine differentiation are defined as proliferations of apocrine epithelium with myoepithelial cells. At Sapporo Dermatopathology Institute, we retrieved 308 benign neoplastic lesions diagnosed as SCAP, AGC, or TPA and combinations of these entities. Among the 308 lesions, 202 (66%) exhibited features of only one type, of which 144 (47%) were AGC, 39 (13%) were TPA, and 19 (6%) were SCAP. The other 106 lesions (34%) had features of 2 or more types, including 56 lesions that were AGC + TPA (18%), 2 that were AGC + SCAP (1%), 34 that were TPA + SCAP (11%), and 14 that were AGC + TPA + SCAP (5%). The most frequent site of these lesions was the face (56%), followed by the scalp (13%). Lesions with the features of AGC were more frequently found on the face, especially the periocular region, than at other sites. TPA lesions were more frequent on the face and scalp than at other sites, whereas SCAP lesions were preferentially found on the face, scalp, and trunk. We also retrieved clinicopathological data and other information. We propose a unifying concept for AGC, TPA, and SCAP. Approximately one-third of these lesions are composite entities with the features of 2 or 3 different tumors, and we propose calling such tumors tubulopapillary cystic adenoma with apocrine differentiation.

Background: Malignant sweat gland adnexal tumors are rare with an incidence of 0.001%. Of these, clear cell hidradenocarcinoma is an extremely uncommon subtype that accounts for 6% of malignant eccrine sweat gland tumors. They occur commonly in the head, neck, and extremities. Although they have a propensity for local recurrence, intracranial extension with brain invasion is extremely rare., Case Description: We report a 76-year-old man with a large, recurring, ulcerated, fungating scalp swelling of 14 years who presented with focal seizures and drowsiness. Neuroimaging revealed a massive tumor arising from the scalp to invade the left parietal lobe and extending to the right side with occlusion of the superior sagittal sinus. The overlying parietal bone was lytic with a "moth-eaten" appearance. He underwent wide excision of the scalp lesion, near-total cerebral tumor decompression followed by titanium mesh cranioplasty, rotation flap reconstruction of the scalp, and adjuvant radiotherapy to the skull vault. Histopathology revealed clear cell hidradenocarcinoma. Whole-body positron emission tomography scan did not reveal any other lesion. At 24 months' follow-up, he remains recurrence free., Conclusion: We report a rare indolent case of clear cell hidradenocarcinoma invading the brain, which was managed with near-total decompression and adjuvant radiotherapy. Intracranial extension in such aggressive tumors poses challenges in management, and regular neuroimaging surveillance is advised., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Eccrine spiradenoma (ES) is a benign adnexal tumor predominantly located in the head and neck regions. Multiple neoplasms located on the scalp have been reported but never with a zosteriform configuration on the first trigeminal area., Case Report: We describe an original case report of a 75-year-old Caucasian man presenting multiple subcutaneous blue and purple nodules disseminated on the first left trigeminal dermatome. All the nodules appeared gradually on a one-year period. Biopsy revealed a nodular adnexal tumor in the dermis without malignant eccrine spiradenoma (MES) transformation. The surgical procedure was performed in a manner to protect the galea aponeurotica in the upper half on the first left trigeminal area. The frontalis muscle was raised with the surgical specimen in the lower half of the first trigeminal area. A split-thickness skin graft was applied on the surgical defect. Histological examination revealed multilobular well-defined tumors located in the dermis., Conclusion: The presence of multiple subcutaneous nodules in a trigeminal pattern should suggest a multiple localized zosteriform ES. The diagnosis is focused on clinical findings and the treatment is based on a large surgical excision. The histological examination is essential for not to fail a MES transformation., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

A 62-year-old man presented with a 2-year history of a 2-cm cystic mass involving his occiput. There had been recent enlargement, and the clinical impression was that of a pilar cyst. Histopathological sections showed a partially dermal solid and cystic proliferation. The tumor contained areas of glandular differentiation with cuboidal to columnar cells lining luminal and cystic spaces. A concurrent spindle cell proliferation was seen interspersed between glands and also formed broad, cellular sheets of cells. The stroma was sclerotic and without chondroid or myxoid elements. Immunohistochemistry showed that the spindled cells expressed S100 protein, cytokeratin and smooth muscle myosin. The immunohistochemical profile and the relationship with ductal elements supported myoepithelial differentiation. The proliferation warranted the diagnosis of myoepithelioma arising from a hidradenoma, which to our knowledge has not been previously described. In addition to discussing this case, we provide a brief review of epithelial-myoepithelial neoplasms encountered in the skin., (Copyright © 2011 John Wiley & Sons A/S.)

Eccrine porocarcinoma may arise from eccrine poroma or it may occur de novo. We present a rapidly growing occipital malignant eccrine poroma on the scalp following to a radiotherapy for cervical carcinoma. A wide excision with skin grafting resulted no recurrence during a 1-year followup. Although malignant transformation is well known for other skin cancers this was not reported for eccrine poroma.

The eccrine poroma is a rare benign sudoral tumour, which creates a fleshy formation. The diagnosis is made according to the anatomopathological study. The usual site is the sol, but in rare cases, other sites have also been reported. The authors report three cases of eccrine poroma localised in the scalp with literature review.

We describe a man with a pigmented poroma on the scalp mimicking a pigmented melanocytic nevus. Histopathological examination showed that melanocytes were mostly distributed at the periphery of the tumor masses. It is suggested that melanocytes in the tumor masses may have migrated from the adjacent epidermis.

Background: Nodular hidradenocarcinoma was first reported as clear-cell eccrine carcinoma by Keasby and Hadley in 1954 (Cancer 1954;7:934-52) and rare malignant tumor. Several synonyms and related terms for nodular hidradenocarcinoma have appeared in the literature., Objective: They have potential for uncontrollable local recurrence, tend to metastasize, and often cause death. Most cases have been reported in the pathology literature with limited clinical information., Methods: We report a 27-year-old woman with nodular hidradenocarcinoma on the scalp., Result: The management of rare cases is not well defined. In our case, she was only treated with a wide local excision, and no recurrence was observed 2 years after excision., Conclusion: Most authors have concluded that early wide surgical excision of the tumor is the treatment of choice. The efficiency of adjuvant therapy generally has not established.

Porocarcinoma is a very rare malignant tumor arising from the duct of eccrine sweat glands. Its prognosis is variable. We report on a patient who developed lymph node and multiple distant metastases, and who died of this malignancy only 6 months after the initial diagnosis.

Eccrine porocarcinoma is a rare malignant tumor of the true sweat gland. It commonly presents in the lower extremities with lymphatic metastasis. The authors describe the clinical presentation, radiographic evidence, operative discoveries, and pathological findings in a patient with an eccrine porocarcinoma involving the soft tissue of the occiput, which had eroded through the cranium. A review of the literature failed to reveal any other such case. The discussion includes the epidemiology, pathogenesis, treatment, and outcome of eccrine porocarcinomas. The six reported cases of scalp eccrine tumors are reviewed.