Sunitinib efficacy in the treatment of metastatic skin adnexal carcinomas: report of two patients with hidradenocarcinoma and trichoblastic carcinoma.

BACKGROUND: Adnexal carcinomas are rare and diverse cutaneous tumours. They are locally aggressive and have the potential for distant metastasis. Metastatic adnexal carcinomas are very resistant to conventional chemotherapies. Sunitinib, an oral tyrosine kinase inhibitor, is reportedly effective for the treatment of various solid cancers. Its use in adnexal carcinomas has never been reported. OBSERVATIONS: The first patient had metastatic clear cell hidradenocarcinoma and was stabilized over 8 months with sunitinib, before she relapsed. The second patient had a metastatic malignant hair follicle tumour (trichoblastic carcinoma) and achieved a partial remission with sunitinib, and disease stabilized after 10 months. Dynamic contrast-enhanced ultrasound (DCE-US) performed to evaluate tumour vascularization during treatment depicted a dramatic and early decrease in the tumour blood volume. CONCLUSIONS: Sunitinib was effective in controlling the disease in our two patients. DCE-US using linear raw data may have an early predictive value for tumour response to sunitinib. Further studies involving larger cohorts of patients are warranted in order to confirm the efficacy of sunitinib in these rare tumours.

Porocarcinoma: a rare sweat gland malignancy.

Eccrine Porocarcinoma (ECP) is a malignant tumour arising from the intraepithelial ductal parts of the sweat gland. It has also been described as malignant hidroacanthoma simplex, sweat gland carcinoma, malignant intra-epidermal eccrine poroma, eccrine poroepithelioma, dysplastic poroma, malignant syringo acanthoma and porocarcinoma. Treatment with wide local excision but metastatic lesions can be treated with chemotherapy. Here, we present a case report of 52 years old male who presented with a fungating growth on left pre-auricular region that came out to be a case of ECP on histopathological examination.

Treatment of advanced malignant eccrine poroma with locoregional chemotherapy.

Malignant eccrine poroma is a rare disease with approximately 200 cases reported in the literature. Regional cutaneous and systemic metastases are rarely observed and their management has been generally unsuccessful. We report on a case in which topical 5-fluorouracil application and intra-arterial chemotherapy with docetaxel resulted in a histologically confirmed complete response of multiple regional skin metastases for more than 2 years. Despite intravenous administration of docetaxel, slow progression of systemic disease was observed.

Antitumour activity of paclitaxel and interferon-alpha in a case of metastatic eccrine porocarcinoma.

BACKGROUND: Eccrine porocarcinoma (EP) is a rare malignant tumour arising in the acrosyringium, with about 50% of the cases developing local recurrence or metastatic disease. No standard therapy protocols for metastatic disease exist. In the past, only short remissions were achieved by applying combinations of cytotoxic agents, which were associated with severe side-effects. AIM OF THE STUDY: In the case reported here, the aim was to find a protocol with fewer side-effects for a patient who was not willing to undergo extensive polychemotherapy. SUBJECT: A 67-year-old male patient with local recurrence and regional lymph node metastases after resection of EP was treated with a combination of interferon-alpha (IFN-alpha) 9 million units s.c. three times per week and paclitaxel 100 mg/m(2) weekly i.v., which shows a side-effect profile similar to taxotere and is used in the treatment of a variety of neoplasms such as advanced squamous cell carcinoma. MAIN OUTCOME: This less aggressive treatment was tolerated well and the patient responded with minor remission and long-term stable disease.

Metastatic eccrine porocarcinoma: response to docetaxel (Taxotere) chemotherapy.

BACKGROUND: Eccrine porocarcinoma is an uncommon neoplasm of the intra-epidermal sweat gland duct. PATIENTS AND METHODS: A case of eccrine porocarcinoma in a female renal transplant patient aged 45 years is described with a review of pertinent literature. RESULTS: The primary tumour was highly pleomorphic. In places large and small cells merged and focally the former component infiltrated the epidermis in a manner akin to Paget's disease of the breast. The majority of the tumour was high grade; using the modified Bloom and Richardson grading system, usually applied to mammary ductal carcinomas, the tumour graded as 3. Metastatic disease developed nine months following primary surgical treatment. The metastatic eccrine porocarcinoma was resistant to epirubicin but responded to docetaxel chemotherapy. CONCLUSIONS: There are no data to support the use of adjuvant therapy in the management of eccrine porocarcinoma. The use of the modified Bloom and Richardson grading system may define cases at high risk of relapse in which adjuvant therapy might be considered. Metastatic eccrine porocarcinoma has proven resistant to many chemotherapeutic agents. We report the first use of docetaxel in the management of this disease. The treatment was well tolerated and resulted in marked symptomatic and radiological responses. Treatment with docetaxel should be considered in future cases of this rare tumour.

[A case of porocarcinoma arising on the penile shaft].

BACKGROUND: Porocarcinoma is a rare eccrine sweat gland tumor, and a varied treatment is not yet determined for metastatic disease because of the chemo-resistant and radio-resistant nature of this tumor. CASE: This report describes a case of porocarcinoma arising on the penile shaft of 83-year-old man with extensive lymph node metastases. He was treated with emasculation and bilateral ilio-inguinal lymph node dissection. Histological examination of the resected tumor showed a homogeneous round cells with duct-like formation in some part and Pagetoid infiltration was also noted in the dermis. Immunohistochemical examination demonstrated a positive CEA and negative S-100 staining and this result was consistent with the pathological diagnosis of porocarcinoma. One course of chemotherapy which consisted of methotrexate, cisplatin, adriamycin, and bleomycin was given to the patient following surgery for the treatment of residual lymph nodes in the paraaortic area. Abdominal CT scan revealed partial response, about -94% shrinkage, after one course of chemotherapy. The duration of the response lasted four 4 months. He died of pneumonia caused by MRSA after 4 months and the autopsy revealed multiple liver metastases and a massive infiltration of tumor cells in the bone marrow of lumbar vertebra. CONCLUSIONS: There is no definite treatment modality available for metastatic porocarcinoma. Because of the patient's age, only one course of chemotherapy was given, however, a fairly good response against this rare tumor suggested that this new regimen might be effective against porocarcinoma.