Syringocystadenocarcinoma papilliferum: a rare tumor with a favorable prognosis.

Syringocystadenocarcinoma papilliferum (SCACP) is a rare adenexal carcinoma with only 21 cases reported in the literature. Most patients describe a long-standing mass with recent change, supporting the idea that SCACP arises from malignant transformation of pre-existing syringocystadenoma papilliferum (SCAP). Syringocystadenocarcinoma papilliferum is generally treated with wide surgical excision of the lesion and patients do exceeding well and require no systemic therapy.

An unusual case of congenital linear eccrine spiradenoma.

Eccrine spiradenoma (ES) is a benign uncommon tumor of skin adnexa with a characteristic clinical and histopathological presentation. Typically, it presents as a painful, slow growing and solitary nodule on the head or upper trunk in adult patients. We report a child with linear ES which presented with asymptomatic papulonodular lesions in a blaschkoid distribution on the face. Cases reported in the literature of multiple spiradenomas are very rare and multiple linear lesions are even rarer. To date, 21 cases of linear/multiple ES have been reported. Of these, eight were in children or adolescents. We report an additional case of this rare clinical presentation and review the literature.

Congenital syringocystadenoma papilliferum.

We present a boy with a congenital, ulcerated nodule on the scalp. At birth, the lesion was considered to be the result of a traumatic injury, but a biopsy at the age of 6 months pointed to a diagnosis of syringocystadenoma papilliferum. We draw attention to the difficulty of identifying head lesions in young children from clinical signs alone.

Congenital blaschkoid eccrine spiradenoma on the face.

Eccrine spiradenoma is an uncommon tumour of the sweat gland(s) and generally occurs as a solitary small painful nodule. Very rarely, it may present as multiple tumours arranged in a linear/blaschkoid pattern. An 8-year-old girl had many papulonodular lesions, arranged in a linear pattern on the left half of the face, involving the forehead and lateral side of the nose. The lesions were along Blaschko's lines. Lesions were reported to be present at birth; no family member had similar lesion(s), either in solitary or multiple form. Histopathologic examination of a biopsy specimen revealed characteristic features of eccrine spiradenoma. The case is reported because of the rare occurrence of this presentation.

Cutaneous and ocular manifestations of Down syndrome.

A patient with Down syndrome and extensive elastosis perforans serpiginosa is presented. There is an increased incidence of elastosis perforans serpiginosa, alopecia areata, syringomas, and Norwegian scabies in patients with this syndrome. Ocular findings include slanting of the palpebral fissures, Brushfield's spots, nystagmus, and epicanthal folds. Serious ocular complications that may impair vision include keratoconus, congenital cataracts, and retinal detachment.

Fine needle aspiration cytology of malignant spiradenoma arising in congenital eccrine spiradenoma.

The fine needle aspiration (FNA) cytology of malignant eccrine spiradenoma arising in a large benign congenital eccrine spiradenoma is described. The malignant tumor was characterized by cohesive, pleomorphic cells with malignant nuclear features and numerous mitoses. FNA of the adjacent benign eccrine spiradenoma revealed prominent basement membrane deposition with an irregular tubular and nesting growth pattern and bland basaloid cells. To our knowledge, the FNA of malignant eccrine spiradenoma has not been previously described. In this case, FNA cytology was influential in directing patient management.

Congenital spiradenoma.

A case of an adenomatous sweat gland hamartoma present since birth is described. The histological and ultrastructural findings were similar to those of spiradenoma, and carcinoembryonic antigen, usually present in cutaneous sweat gland tumors, was also demonstrated. Sweat gland nevi are rare, and a spiradenomatous variant has not been previously documented.

Congenital papillated apocrine cystadenoma: a mixed form of hidrocystoma, hidradenoma papilliferum, and syringocystadenoma papilliferum.

Two small congenital tumors on the left thigh of an 8-year-old boy were excised. Their histologic picture was peculiar in that they presented features in common with apocrine hidrocystoma, syringocystadenoma papilliferum, and hidradenoma papilliferum.