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Cancers Oct 2022Eccrine porocarcinoma, also known as porocarcinoma (PC) and malignant eccrine poroma, is very rare and is known to arise from the cutaneous intraepidermal ducts of the... (Review)
Review
Eccrine porocarcinoma, also known as porocarcinoma (PC) and malignant eccrine poroma, is very rare and is known to arise from the cutaneous intraepidermal ducts of the sweat glands. Its etiology is not well understood; however, some studies suggest that PC tumors originate from benign eccrine poroma. Recently, several gene alterations have been reported in PC that can reveal mechanisms of the oncogenic process. Since the clinical and histopathological findings of PC are variable, PC is difficult to diagnose precisely, especially when the histology resembles that of cutaneous squamous cell carcinoma or poroma. Immunohistochemical staining with carcinoembryonic antigen and epithelial membrane antigen may help to distinguish PC from other tumors. The standard treatment for local PC is wide local excision. The prognosis of patients with metastatic PC is poor, with mortality rates of approximately 60-70%. The efficacy of radiation and chemotherapy for metastatic PC is limited; however, immunotherapy with pembrolizumab, a programmed cell death protein 1 inhibitor, could be a promising treatment. This review focuses on the history, pathogenesis, pathological features, diagnosis, and treatment of eccrine porocarcinoma.
PubMed: 36358649
DOI: 10.3390/cancers14215232 -
Diagnostics (Basel, Switzerland) Apr 2023Eccrine porocarcinoma (EPC) constitutes a rare malignant adnexal tumor, which accounts for about 0.005-0.01% of all cutaneous malignancies. It may develop de novo or... (Review)
Review
Eccrine porocarcinoma (EPC) constitutes a rare malignant adnexal tumor, which accounts for about 0.005-0.01% of all cutaneous malignancies. It may develop de novo or arise from an eccrine poroma, after a latency period of years or even decades. Accumulating data suggest that specific oncogenic drivers and signaling pathways may be implicated in its tumorigenesis, while recent data have demonstrated a high overall mutation rate attributed to UV exposure. Diagnosis may be challenging and should rely on the combination of clinical, dermoscopical, histopathological and immunohistochemical findings. The literature is controversial regarding tumor behavior and prognosis and, therefore, there is no consensus on its surgical management, utility of lymph-node biopsy and further adjuvant or systemic treatment. However, recent advances in tumorigenesis of EPC may aid in the development of novel treatment strategies, which could improve survival of advanced or metastatic disease, such as immunotherapy. This review presents an update of the epidemiology, pathogenesis and clinical presentation of EPC and summarizes current data on diagnostic evaluation and management of this rare cutaneous malignancy.
PubMed: 37189532
DOI: 10.3390/diagnostics13081431 -
Diagnostics (Basel, Switzerland) Aug 2023Eccrine poroma (EP) is a relatively rare benign adnexal neoplasm that usually affects elderly patients. Its pathogenesis is still under investigation, but recent gene... (Review)
Review
Eccrine poroma (EP) is a relatively rare benign adnexal neoplasm that usually affects elderly patients. Its pathogenesis is still under investigation, but recent gene studies have revealed gene fusions as key incidences resulting in oncogenetic pathways. It often presents as a solitary, firm papule, mostly asymptomatic, located on the soles or palms. Due to its clinical and dermoscopic variability, it is characterized as the great imitator. We performed a literature review, aiming to summarize current data on the pathogenetic mechanisms, new dermoscopic features, and novel diagnostic tools that may aid in early diagnosis and proper management of this rare adnexal tumor. Furthermore, we reviewed the possible pathogenetic associations between EP and its malignant counterpart, namely eccrine porocarcinoma. This systematic approach may aid in understanding the pathogenetic mechanisms and how to use novel histopathologic markers and imaging methods to overcome the diagnostic dilemma of this rare tumor.
PubMed: 37627947
DOI: 10.3390/diagnostics13162689 -
BMJ Case Reports Mar 2022Eccrine porocarcinoma is a rare skin adnexal malignant neoplasm that may arise from a pre-existing benign eccrine poroma or without a predisposing factor. It is a highly...
Eccrine porocarcinoma is a rare skin adnexal malignant neoplasm that may arise from a pre-existing benign eccrine poroma or without a predisposing factor. It is a highly invasive neoplasm and has a strong metastatic potential. The most frequently affected organs are the lymph nodes and rarely solid organs such as the liver, lungs and breast. We report a case of a woman with a history of surgically treated eccrine porocarcinoma that a year later presented with multiple lesions in both breasts and axillary lymphadenopathies. After a detailed imaging investigation, the diagnosis of metastatic lesions from porocarcinoma was made. To our knowledge, until the moment, only one case of breast metastasis of eccrine porocarcinoma has been reported in the literature.
Topics: Eccrine Porocarcinoma; Female; Humans; Melanoma; Poroma; Skin Neoplasms; Sweat Gland Neoplasms
PubMed: 35256370
DOI: 10.1136/bcr-2021-247900 -
Case Reports in Oncology 2020Eccrine porocarcinoma (EPC) is an infrequent cutaneous neoplasm, and was described in 1963 by Pinkus and Mehregan. It is a rare type of skin tumor (0.005-0.01% of all... (Review)
Review
Eccrine porocarcinoma (EPC) is an infrequent cutaneous neoplasm, and was described in 1963 by Pinkus and Mehregan. It is a rare type of skin tumor (0.005-0.01% of all skin tumors). Less than 300 cases have been described in the entire world medical literature. To our knowledge, no case of intergluteal cleft EPC has been reported in the literature in English and Spanish to date, so this would be the first reported case of such pathology. Metastatic EPC is less frequent, since only <10% of metastatic type have been reported and the rest as localized disease. The primary treatment of choice is surgical wide local excision of the tumor with histological confirmation of tumor-free margins. Prognosis is difficult to determine because of the rarity of EPC and the variations in natural history. There are no data to support the use of adjuvant chemotherapy or radiotherapy, and there are currently no agreed criteria to define patients at high risk of relapse. We present a 67-year-old man with intergluteal cleft eccrine tumor by biopsy. Metastasis to left inguinal region and lung was reported by contrasted abdominal and chest computed tomography. He started chemotherapy based on etoposide, vincristine, carboplatin. A review of pertinent literature is provided.
PubMed: 33442371
DOI: 10.1159/000510311 -
Archives of Craniofacial Surgery Feb 2019Eccrine porocarcinoma is a rare malignant tumor arising from the intraepidermal ductal portion of the eccrine sweat gland. It develops either spontaneously or from a...
Eccrine porocarcinoma is a rare malignant tumor arising from the intraepidermal ductal portion of the eccrine sweat gland. It develops either spontaneously or from a long standing benign eccrine poroma. This entity usually affects older people and is commonly located on the lower extremities, the trunk, and the head. We report a case of eccrine porocarcinoma on the left cheek in an 85-year-old male. In our case, the tumor was treated with wide excision and postoperative adjuvant radiation therapy. The patient recovered well without local recurrence and distant metastasis during the 14-month follow-up period. Wide excision and postoperative adjuvant radiation therapy can be considered as a safe and effective treatment option in treating patients with eccrine porocarcinoma.
PubMed: 30739434
DOI: 10.7181/acfs.2018.02180 -
Annals of Dermatology Oct 2022
PubMed: 36198637
DOI: 10.5021/ad.21.009 -
Cureus Feb 2020Eccrine porocarcinoma is a rare malignant tumor that develops in the eccrine glands, appearing as a primary tumor, or by malignant transformation of an eccrine poroma....
Eccrine porocarcinoma is a rare malignant tumor that develops in the eccrine glands, appearing as a primary tumor, or by malignant transformation of an eccrine poroma. It is a carcinoma with high metastatic and recurrent potential; it has the same incidence in both sexes, and mainly affects the elderly. Its diagnosis, rather than clinical, is histological, and due to the rarity of the disease, it is a pathological challenge. There are no standardized treatment guidelines for porocarcinoma, but surgical resection with tumor-free margins is considered the basis of treatment, in addition to sentinel node biopsy under risk factors and individualization of each patient. For the metastatic form, chemotherapy and radiotherapy are the treatment of choice. Herein, we present the case of a man with eccrine porocarcinoma with extensive zosteriform skin metastasis and lymph node involvement, treated with chemotherapy and concomitant radiotherapy.
PubMed: 32181102
DOI: 10.7759/cureus.6873