Eccrine porocarcinoma.

Eccrine porocarcinoma (EPC) is a rare cutaneous neoplasm that grows slowly over a long period of time but often experiences an accelerated growth phase. This malignant tumor may arise denovo or evolve from a pre-existing benign eccrine poroma. Histologic evaluation demonstrates anaplastic cells involving the epidermis and infiltrating the dermis. Metastasis to regional lymph nodes distally occurs in a significant number of cases. Treatment modalities have included standard excision, Mohs micrographic surgery (MMS), chemotherapy, and radiation therapy. Recently sentinel lymph node biopsy has begun to be investigated as a staging tool. The literature on EPC is reviewed with attention paid to which of the various therapeutic options offers a clear advantage over the others. MMS affords the greatest likelihood of clear margins and cure in absence of regional and distant metastases.

Eccrine porocarcinoma, tricholemmal carcinoma and multiple squamous cell carcinomas in a single patient.

A 75-year old woman without remarkable medical history presented with a large eccrine porocarcinoma of the trabelucar type located on her leg. This tumor was associated with two invasive squamous cell carcinomas of the legs, two Bowen's diseases of her thigh and arm and multiple actinic keratoses of the face. Despite major surgery, local recurrence of the porocarcinoma was observed 6 months later. During the 3-year follow-up after a second surgical intervention, this lesion did not reccur, but the patient developed a rapidly enlarging ulcerated tumor of the forehead which proved to be tricholemmal carcinoma. Although no carcinogenetic factor or familial history of multiple neoplasms could be evidenced, the occurrence of multiple tumors of various histogenesis over a 1 year period of time is unlikely to be fortuitous. Such an association of rare adnexal neoplasms has not yet been described.

Malignant eccrine hidradenoma of neck causing acute heart failure.

Tumours of eccrine sweat glands are uncommon, with complex classification and different terms used even for the same tumour. Therefore, for practical purposes, it may be sufficient to differentiate between benign and malignant. Malignant eccrine hidradenoma has a predilection for head and neck, with high incidence of recurrence following surgical excision and also regional and distant metastases. We describe a case, which presented as a medical emergency with symptoms of severe anaemia and acute heart failure secondary to intermittent bleeding from a huge ulcerative neck lesion, which was subsequently diagnosed as eccrine hidradenocarcinoma. The tumour was successfully treated with complete surgical excision and reconstruction followed by radiotherapy. Two years postoperatively, the patient is very well with no sign of recurrence.

Malignant eccrine acrospiroma with metastasis to the parotid.

Malignant eccrine acrospiromas are rare. Clinically, they resemble other cutaneous lesions. A high index of suspicion must be maintained in cases of histologically benign eccrine acrospiromas for three reasons: (1) malignant transformation can occur, (2) the presence of both benign and malignant tissue can lead to a false-negative diagnosis if only the benign component is obtained in the biopsy specimen, and (3) benign-appearing tumors can recur locally or metastasize. The primary treatment is wide local excision with or without lymph node dissection. The efficacy of adjuvant chemotherapy and radiation therapy requires further investigation. We describe a case of malignant eccrine acrospiroma in an 80-year-old man, and we review the literature on this tumor, with emphasis on the differential diagnosis.

Malignant eccrine spiradenoma: a meta-analysis of reported cases.

BACKGROUND: Malignant eccrine spiradenoma is an aggressive sweat gland tumor with poorly understood behavior and no currently accepted therapeutic regimen. METHODS: An individual patient data meta-analysis with Kaplan­Meier survival curves was performed on 72 reported cases of malignant eccrine spiradenoma. RESULTS: In 35 patients with no distant metastasis, local resection resulted in 100% disease-free survival. Of 7 patients with lymph node but no distant metastasis treated with surgical resection and lymph node dissection, 6 patients remained disease-free at final follow-up evaluation. For the 24 cases with confirmed distant metastatic disease, patient survival did not significantly differ between local resection and surgery with adjuvant chemoradiotherapy (P = .8763). CONCLUSIONS: Heightened awareness is recommended among surgeons likely to treat this entity. An aggressive surgical approach is supported in the absence of metastasis. When lymph nodes are not clinically involved, sentinel node may have a role followed by lymph node dissection in patients with a positive node.

Unusual skin tumors: Merkel cell carcinoma, eccrine carcinoma, glomus tumors, and dermatofibrosarcoma protuberans.

This article discusses the epidemiology, etiology, presentation, pathology, evaluation and staging, and treatment of unusual skin tumors, such as Merkel cell carcinoma, eccrine carcinoma, glomus tumors, and dermatofibrosarcoma protuberans.

Metastatic eccrine porocarcinoma: a 5.6-year follow-up study of a patient treated with a combined therapeutic protocol.

BACKGROUND: Metastatic eccrine porocarcinoma (EP) is an uncommon, malignant, and potentially lethal neoplasm that arises from the eccrine sweat glands. OBJECTIVE: To present the 5.6-year follow-up study of a male patient with metastatic EP, highlighting the widespread cutaneous involvement and the response to the treatment carried out. METHODS: We describe the evolution of the tumor and the combined therapy carried out and review the treatments employed in previously reported cases, comparing them with ours. RESULTS: The patient developed multiple cutaneous and regional lymph node metastases 15 months after surgical excision of the primary tumor. He was treated with prophylactic lymphadenectomy, radiotherapy, and oral isotretinoin, subsequently substituted by tegafur. We have not found evidence of distant metastases after a 5.6-year follow-up. CONCLUSIONS: The optimum treatment for metastatic EP is not standardized, and the results obtained to date were generally poor. In this context, we consider it of interest to highlight the response of our patient to the therapeutic regime employed, which may be advantageous in future cases of this rare tumor.

Vulvar eccrine porocarcinoma.

A rare case of a slowly growing vulvar tumor in a 75-year-old woman is presented. This vulvar neoplasm was an eccrine porocarcinoma with left inguinal nodal metastases. This is believed to be only the second reported case of a vulvar eccrine porocarcinoma. Treatment included a left hemivulvectomy, bilateral inguinal-femoral lymphadenectomy, and postoperative radiation therapy. A review of the literature regarding this unusual malignancy is included.