Syringoid eccrine carcinoma: a clinicopathological and immunohistochemical study of four cases.

BACKGROUND: Syringoid eccrine carcinoma (SEC) is a rare malignant adnexal tumour with variable presentations. AIM: To examine the clinicopathological and immunohistochemical features of SEC. METHODS: Four cases were reviewed by three dermatopathologists and the immunohistochemical profile was examined using antibodies against CK5/6, CK7, CK14, CK20, LMWK, HMWK, EMA, mCEA, p63, ER, PR, AR, S-100 and Ber-EP4. RESULTS: The cases occurred in two men and two women, ranging in age from 61 to 87 years (mean 68.5). Two of the lesions were from the face and two from the trunk. All four lesions were composed of an atypical infiltrative mass with syringoma-like tadpole morphology with ductular differentiation and prominent desmoplasia. Three cases demonstrated perineural invasion and two had positive lymph node metastases. Immunostaining was variable. Immunohistochemistry positivity was as follows: three out of four cases were positive for CK5/6, CK7 (2/4), CK14 (1/3), CK20 (0/2), HMWK (0/2), LMWK (1/2), EMA (3/4), mCEA (4/4), p63 (2/3), ER (2/3), PR (1/2), AR (0/3), S-100 (0/3) and Ber-EP4 (2/2). CONCLUSION: SEC can present on the trunk and are not limited to the head and neck region. In addition to syringoma-like tadpole structures and glandular differentiation, these tumours can also exhibit squamoid and cribriform growth patterns. Immunostaining in SEC is variable and this variability is believed to stem from this tumour's ability to differentiate along multiple routes, including sweat secretory and/or ductal differentiation.

Adnexal skin carcinomas of the face.

Skin adnexal neoplasms are rare tumors, and knowledge on their exact histological development and pathogenesis remains fragmented. Their diagnosis and management are frequently troublesome, especially for malignant tumors. We present a retrospective case series of malignant appendageal tumors of the face. Records of patients with histologically confirmed adnexal skin carcinoma managed at a single department during the period 1995 to 2004 were reviewed. Patient biodata, surgical management, and main outcome measures such as locoregional recurrence, distant metastasis, and disease-free and overall survival were recorded.Eleven patients were identified from record analysis. Lesions were mainly located at the eyelids and the nose. All patients were managed with wide local excision and restoration with local flaps. Two patients showed regional metastasis and underwent radical neck dissection. Nine of 11 patients were alive and free of local and/or regional disease on final follow-up. Two patients died of causes unrelated to the disease. Clinical and histological features, tumor biological behavior, diagnostic difficulties, and recommended management are discussed.In conclusion, adnexal skin tumors should be considered in the differential diagnosis of skin tumors, particularly in view of their more sinister prognosis compared with other nonmelanoma skin tumors. They are acknowledged to demand a more aggressive plan of surgical treatment.

Sweat gland carcinoma with lung metastases.

Sweat gland carcinoma is a rare skin tumor. The tumor has propensity to spread to lymph nodes and distant metastases has been reported. Their exact incidence in the Indian setting is not known. Aspects related to treatment are also not clearly defined. Though surgery forms the initial treatment approach, adjuvant treatment has not been properly explored. We report here a case of sweat gland carcinoma with bilateral lung metastases.

High-grade trichoblastic carcinoma arising in trichoblastoma: a rare adnexal neoplasm often showing metastatic spread.

It has been debated whether malignant transformation of trichoblastoma occurs. The concept was recently forwarded that basal cell carcinoma is as a malignant neoplasm of follicular germinative cells and should be named trichoblastic carcinoma to show its relationship to trichoblastoma. Almost all basal cell carcinomas are low-grade malignant neoplasms and develop metastases only very rarely, and if so, only after very long duration and untreated growth. Only rare basal cell carcinomas arise in trichoblastomas. Up to now there have only been two reports of high-grade trichoblastic carcinoma arising in trichoblastoma, showing systemic metastatic spread and death. We add two further cases of trichoblastic carcinoma with anaplastic nuclei, arising in trichoblastoma. One of the tumors arose in a small nodular trichoblastoma on the right forearm of an 84-year-old male patient. The other one was a trichoblastic carcinoma at the base of a trichoepithelioma on the right thigh of an 87-year-old woman with Brooke-Spiegler syndrome. Our cases emphasize that high-grade trichoblastic carcinoma develops via malignant transformation of trichoblastoma, and is very rare.

Sentinel lymphonodectomy in nonmelanoma skin malignancies.

BACKGROUND: Whereas the value of sentinel lymphonodectomy (SLNE) in malignant melanoma is established, experience with SLNE in nonmelanoma skin cancers is limited. OBJECTIVES: The feasibility of SLNE in nonmelanoma skin tumours is evaluated. METHODS: Thirty-seven patients with high-risk nonmelanoma skin tumours underwent SLNE: 11 squamous cell carcinomas (SCCs), seven Merkel cell carcinomas (MCCs), five cutaneous lymphomas, eight adnexal carcinomas and six other skin cancers, all clinical stage N0. RESULTS: In nine patients (four MCCs, two SCCs, three lymphomas) the sentinel lymph nodes (SLNs) showed histological evidence of microinvolvement. In five of these nine patients, radical lymph node dissection (RLND) was performed, revealing further micrometastases in three patients (two SCCs, one MCC). No patient with negative SLN showed tumour dissemination during the follow-up over a mean of 2.5 years (range 2 months to 4.5 years, median 2.4 years). CONCLUSIONS: Our data provide evidence that SLNE is a minimally invasive and highly sensitive staging tool in selected patients with high-risk nonmelanoma skin cancers.

Recurrent polymorphous sweat gland carcinoma of the skin.

Polymorphous sweat gland carcinoma is an unusual, recently described variant of low-grade malignant adnexal neoplasm of the skin characterized by a prolonged clinical course and predilection for the extremities. We describe a case of recurrent polymorphous sweat gland carcinoma in a 56-year-old man who presented with multiple large skin nodules distributed along the flexor surface of his left arm. The lesions were treated by surgical excision; multiple local recurrences, as well as the development of new lesions, were observed over a period of 5 years. No distant metastases have been observed so far. The clinical differential diagnosis and management of these unusual lesions are discussed.

Metastatic microcystic adnexal carcinoma in an immunocompromised patient.

BACKGROUND: Microcystic adnexal carcinoma is an uncommon, locally aggressive cutaneous neoplasm. To date, there are only two reports of histologically proven lymph node involvement with this tumor. We describe a case of a patient with microcystic adnexal carcinoma who developed multiple local metastasis in transit with histologically proven lymph node involvement and was diagnosed with chronic lymphocytic leukemia. OBJECTIVE: To describe the details of our case and to review what is currently known about this tumor. METHODS: Mohs micrographic surgery was utilized for tumor removal. RESULTS: This patient developed multiple tumors of the scalp over the period of a 1 year which were histologically proven to be microcystic adnexal carcinoma. All tumors were noncontiguous and presented on the scalp. During the histologic analysis of the last tumor removed by Mohs micrographic surgery a lymph node was resected which revealed infiltrative microcystic adnexal carcinoma. CONCLUSIONS: We present the case of an immunocompromised patient treated for microcystic adnexal carcinoma with Mohs micrographic surgery who proceeded to develop local metastasis in transit.