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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 -
Dermatologic Therapy 2008Eccrine 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... (Review)
Review
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.
Topics: Acrospiroma; Antineoplastic Agents; Humans; Lymphatic Metastasis; Mohs Surgery; Neoplasm Invasiveness; Neoplasm Staging; Sentinel Lymph Node Biopsy; Sweat Gland Neoplasms
PubMed: 19076620
DOI: 10.1111/j.1529-8019.2008.00243.x -
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 -
Dermatologica 1991We describe a 78-year-old woman with eccrine porocarcinoma presenting a zosteriform appearance on the lateral upper part of the buttock. Erosion and bleeding were... (Review)
Review
We describe a 78-year-old woman with eccrine porocarcinoma presenting a zosteriform appearance on the lateral upper part of the buttock. Erosion and bleeding were observed in the center of a 50 X 80 mm, erythematous indurated plaque with an elevated border. Posterior to the plaque, there was another erosive plaque, 50 X 20 mm in diameter, with peripheral induration overlaid by two reddish papules. Based on the previous reports of 21 patients with eccrine porocarcinoma in Japan, we discuss the relationship between the clinical pattern and lymph node metastasis. The pedunculated eccrine porocarcinoma has less tendency to metastasize to the regional lymph nodes than the nonpedunculated form.
Topics: Adenoma, Sweat Gland; Adult; Aged; Aged, 80 and over; Buttocks; Eccrine Glands; Female; Humans; Male; Middle Aged; Sweat Gland Neoplasms
PubMed: 1653154
DOI: 10.1159/000247804 -
Head & Neck Sep 2020The aim of the study is to analyze potential prognostic factors and to evaluate therapy strategies regarding clinical outcome in patients with eccrine porocarcinoma... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
The aim of the study is to analyze potential prognostic factors and to evaluate therapy strategies regarding clinical outcome in patients with eccrine porocarcinoma (EPC) of the head and neck.
METHODS
One hundred and sixteen EPC cases from ninety studies and four authors' EPC cases were included in the meta-analysis.
RESULTS
At an average follow up of 20.48 months, the 3-year overall survival and regional recurrence rate were 70.3% and 19.0%, respectively. Patients without surgical treatment had a significantly worse 3-year overall survival. Mohs microscopic surgery led to significantly less occurrence of regional recurrences compared to wide excision. An ulcerating lesion, high mitotic activity, and lymphovascular invasion were significant prognostic factors.
CONCLUSION
Surgical resection is the cornerstone in the therapy of EPC and represents the therapeutic modality that offers the best chance of disease-free survival. Due to the high probability of recurrence, close follow-ups are strongly recommended.
Topics: Eccrine Porocarcinoma; Head; Humans; Mohs Surgery; Neoplasm Recurrence, Local; Sweat Gland Neoplasms
PubMed: 32314845
DOI: 10.1002/hed.26178 -
Cutis Aug 2013Eccrine porocarcinoma (EP) is a rare malignant tumor arising from the intraepidermal sweat duct. The lesions have a varied clinical appearance and usually are... (Review)
Review
Eccrine porocarcinoma (EP) is a rare malignant tumor arising from the intraepidermal sweat duct. The lesions have a varied clinical appearance and usually are asymptomatic. In reported cases, metastasis is rare but can be devastating when it occurs. We describe an unusual case of EP in a patient who presented with metastatic disease. New-onset exquisite pain prompted his evaluation. This case and review of metastatic disease is presented to raise awareness of this tumor, its varied clinical presentations, differential diagnosis, and management.
Topics: Aged, 80 and over; Diagnosis, Differential; Eccrine Porocarcinoma; Follow-Up Studies; Humans; Male; Neoplasm Metastasis; Pain; Sweat Gland Neoplasms
PubMed: 24087778
DOI: No ID Found -
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 -
Giornale Italiano Di Dermatologia E... Aug 2020Eccrine porocarcinoma is a rare skin cancer that originates from the acrosyringium of eccrine sweat glands. From the clinical point of view the differential diagnosis... (Review)
Review
Eccrine porocarcinoma is a rare skin cancer that originates from the acrosyringium of eccrine sweat glands. From the clinical point of view the differential diagnosis with other skin cancers such as basal cell carcinoma and squamous cell carcinoma it is often impossible, only the histopathologic features can lead to the definitive diagnosis. Eccrine porocarcinoma can arise from a previous poroma or de novo, it may recur after surgical excision and cause lymph node and visceral metastasis. There are no international guidelines for treatment or follow-up of patients. The aim of this work was to present a rare case of eccrine porocarcinoma of the scalp successfully treated in our clinic and to extrapolate from the international literature the main clinical and histopathological features of eccrine porocarcinoma and the various experiences regarding the types of treatment.
Topics: Aged, 80 and over; Eccrine Porocarcinoma; Humans; Male; Scalp; Sweat Gland Neoplasms
PubMed: 33050682
DOI: 10.23736/S0392-0488.17.05182-3 -
Dermatologic Surgery : Official... Oct 2018Eccrine porocarcinoma (EPC) is a rare cutaneous neoplasm with high potential for morbidity and mortality. Due to its rarity, there is a paucity of data profiling... (Review)
Review
BACKGROUND
Eccrine porocarcinoma (EPC) is a rare cutaneous neoplasm with high potential for morbidity and mortality. Due to its rarity, there is a paucity of data profiling diagnosis, work-up, and management.
OBJECTIVE
To consolidate reported information on demographics, diagnostics, clinical behavior, treatment modalities, and patient outcomes in EPC to provide a reference tool to optimize diagnosis and management.
METHODS
A comprehensive PubMed search was performed from 1963 to November 2017 using PRISMA guidelines. This yielded 155 articles detailing 206 cases of porocarcinoma.
RESULTS
Eccrine porocarcinoma most often presents in elderly patients on the head and neck or lower limbs. Metastatic disease at presentation is not uncommon (22%). Primary tumor location is significantly correlated with presence of metastasis (p = .038). The most common treatment is excision followed by Mohs micrographic surgery (MMS), although the outcomes after MMS were superior to those after surgical excision.
CONCLUSION
This systematic review of individual patient data reveals that all patients should have a histological diagnosis with imaging considered for high-risk cases. Primary tumor location should also be considered in diagnostic and therapeutic decision-making. Although wide local excision (WLE) is currently the first-line treatment, MMS is becoming increasingly used, with evidence indicating improved outcomes as compared to those seen with WLE.
Topics: Aged; Eccrine Porocarcinoma; Female; Humans; Male; Middle Aged; Sweat Gland Neoplasms
PubMed: 29894433
DOI: 10.1097/DSS.0000000000001566 -
Cutis Jun 2023
Topics: Humans; Eccrine Porocarcinoma; Sweat Gland Neoplasms; Male; Aged; Biopsy; Female; Adult
PubMed: 37487125
DOI: 10.12788/cutis.0810