Porocarcinoma Research Articles

Eccrine porocarcinoma of the face, an aggressive and unusual cutaneous tumor

El porocarcinoma ecrino (PE) es un cáncer de las glándulas sudoríparas de la piel, con una presentación clínica diversa que incluye placas, nódulos ulcerados y lesiones polipoides. Los síntomas pueden involucrar picazón, dolor e inflamación, y signos de degeneración maligna como úlceras, sangrado, crecimiento rápido o dolor. La histopatología muestra la proliferación de células atípicas con rasgos malignos, incluyendo pleomorfismo e hipercromasia nuclear. Puede metastatizar a órganos distantes, siendo el hueso, vejiga, retroperitoneo y otros sitios comunes. La supervivencia depende del tamaño del tumor, con peores pronósticos para tumores mayores de 4 cm. El tratamiento principal es la extirpación quirúrgica, especialmente en estadios tempranos, con vaciamiento ganglionar en casos de compromiso. La cirugía micrográfica de Mohs se utiliza para casos localizados. La radioterapia y quimioterapia pueden ser opciones en metástasis o recurrencia. El seguimiento a largo plazo es crucial, y se necesita investigación continua para mejorar las estrategias de tratamiento.

Navigating Head and Neck Porocarcinoma: Systematic Review with Special Emphasis on Surgical Safety Margins.

Eccrine porocarcinoma, sharing many features with other skin tumours, is diagnostically challenging. A conventional biopsy might be misleading and surgical excision becomes a primary diagnostic tool and a treatment method. However, the data on surgical safety margins are not consistent. We present a systematic review analysing the surgical margins of porocarcinoma in the head and neck area, which was conducted across the PubMed, Cochrane, and Web of Science databases including studies published from inception to November of 2023. In this systematic review, the PRISMA-ScR checklist was used, and a Cohen's Kappa coefficient of 0.92 was applied, indicating very good agreement between reviewers. Out of 529 identified articles, 18 studies yielding 20 cases in total were selected for a thorough analysis. Nine (45%) cases were observed in the facial regions, eight (40%) on the scalp, and three (5%) on the neck. The primary treatment of choice was wide local excision with safety margins ranging from 3 to 22 mm (mean: 10.1). It demonstrated that surgical margins do not differ by age or anatomic regions, with the main point of reference being the tumour size. As observed, the bigger the tumour, the wider the safety margins were. However, the limited disclosure of surgical safety margins in analysed case reports impeded our ability to define the minimum safety margins. Further investigation and a consensus on recommended safety margins are required.

MALIGNANT SWEAT GLAND TUMOURS: A CASE SERIES

Objectives: Malignant sweat gland tumor is a very rare skin cancer with variable presentations. The objectives of this study are to examine the clinicopathological and immunohistochemical features of malignant sweat gland tumors and to discuss the treatment and follow-up for postsurgical recurrence rates. Methods: Six patients diagnosed with sweat gland carcinoma at the National Hospital of Dermatology and Venereology between 2018 and 2023 were carefully reviewed. Results: The cases occurred in four men and two women, ranging in age from 31 to 78 years (mean 59.3). Four of the lesions were from the scalp, one from the nose, and one from the right arm. There were three cases of Eccrine Porocarcinoma, two cases of Hidradenocarcinoma, and one case of Syringoid Eccrine Carcinoma. All of them were treated with surgery, five cases with wide local excision, and one case with Mohs micrographic surgery. Only one case had a recurrence. Conclusion: Malignant sweat gland tumors demonstrate significant variability in clinicopathology and immunohistopathology. Surgery is the mainstay of treatment. Received 28 June 2023Revised 26 September 2023Accepted 29 November 2023

Pazopanib elicits remarkable response in metastatic porocarcinoma: a functional precision medicine approach.

Metastatic porocarcinomas (PCs) are vanishingly rare, highly aggressive skin adnexal tumors with mortality rates exceeding 70%. Their rarity has precluded the understanding of their disease pathogenesis, let alone the conduct of clinical trials to evaluate treatment strategies. There are no effective agents for unresectable PCs. Here, we successfully demonstrate how functional precision medicine was implemented in the clinic for a metastatic PC with no known systemic treatment options. Comprehensive genomic profiling of the tumor specimen did not yield any actionable genomic aberrations. However, ex vivo drug testing predicted pazopanib efficacy, and indeed, administration of pazopanib elicited remarkable clinicoradiological response. Pazopanib and its class of drugs should be evaluated for efficacy in other cases of PC, and the rationale for efficacy should be determined when PC tumor models become available. A functional precision medicine approach could be useful to derive effective treatment options for rare cancers.

Evolving Pink Papule in a Young Female

Eccrine porocarcinoma (EPC) is a rare cutaneous adnexal tumor derived from sweat glands. Typically, EPC presents as a nonspecific erythematous papule or nodule most commonly on the head, neck, or lower extremities of females in the sixth or seventh decade of life. This tumor may arise from an existing benign eccrine poroma or occur de novo. Herein, we report a case of a growing erythematous nodule with overlying telangiectasia on the lower extremity of a woman in her twenties. Biopsy revealed a cyst-like lesion beneath the epidermis comprised of clear fluid material. Both solid and cystic components were present alongside pleomorphic tumor cell aggregates characterized by mitotic figures, hyperchromasia, and necrosis. The combination of clinical presentation and the presence of ductal structures on histopathologic analysis led to a diagnosis of EPC. This case reinforces the critical importance of histopathologic analysis of a nonspecific lesion that has evolved over time and may resemble other forms of cutaneous malignancy. 

44190 Differences in demographic variables and survival in eccrine porocarcinoma patients undergoing Mohs micrographic surgery on a national scale

44190 Differences in demographic variables and survival in eccrine porocarcinoma patients undergoing Mohs micrographic surgery on a national scale

Eccrine Poroma: Pathogenesis, New Diagnostic Tools and Association with Porocarcinoma-A 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.

Eccrine Porocarcinoma: Clinical and Histopathological Study of 14 Patients with Special Emphasis on Sentinel Lymph Node Biopsy.

Eccrine porocarcinoma is a rare skin adnexal tumour that affects elderly people. Most eccrine porocarcinomas are stage I or II according to the American Joint Committee on Cancer. The prognosis is good in early stages, but worsens when advanced. Since information on the use of sentinel lymph node biopsy in these patients is scarce, this study examined the records of all patients with eccrine porocarcinoma treated at Helsinki University Hospital during a 17-year period and focused on sentinel lymph node biopsy patients. The study identified 14 patients (9 male, 5 female). There were 2 metastases to the lymph nodes and 2 recurrences at initial referral to our institution. All primary tumours had wide local excision and 6 patients also had sentinel lymph node biopsy, of whom none had positive lymph nodes. There were no new metastases or recurrences during follow-up. Three patients died of causes other than eccrine porocarcinoma. When comparing the wide local excision only and wide local excision with sentinel lymph node biopsy groups, no parameters reached statistical significance. The decision process of the multidisciplinary tumour board meeting on whether to perform sentinel lymph node biopsy was not clear, perhaps due to the limited knowledge of eccrine porocarcinoma. Further studies and international collaboration are warranted.

Porocarcinoma of the Left Foot: A Case Review.

Eccrine porocarcinoma is a rare malignant tumor of the eccrine sweat gland. This malignancy occurs most commonly in the lower extremities. It tends to occur in patients aged 60 to 80 years, affecting men and women equally. We present the case of a 62-year-old man with a lesion on the left foot. The diagnosis of the initial biopsy was squamous cell carcinoma. Six months later, the lesion reoccurred, and a second biopsy confirmed it to be eccrine porocarcinoma.

Eccrine Porocarcinoma of the Vulva With Sarcomatoid Transformation, a New Human Papillomavirus-associated Malignancy? Case Report and Review of the Literature.

Eccrine porocarcinoma is a rare cutaneous neoplasm, and rarer still in the anogenital region. In the vulva, the most common carcinoma by far is squamous cell carcinoma; however, eccrine porocarcinoma can arise at this site. As the distinction between porocarcinoma and squamous cell carcinoma has important prognostic implications at other cutaneous sites, it stands to reason that it may have these same implications in the vulva. We present a case of an eccrine porocarcinoma in the vulva of a 70-year-old woman that, in addition, showed sarcomatoid transformation. This tumor harbored human papillomavirus-18 DNA and mRNA, raising the question of the role of the oncogenic virus in sweat gland neoplasms of the vulva.

Wide Local Excision for Eccrine Porocarcinoma of the Forearm.

Eccrine porocarcinoma is a rare and aggressive cutaneous malignancy that develops in the seventh and eight decades of life. We present a 76-year-old male with eccrine porocarcinoma developing from a long standing previously benign lesion who underwent successful treatment with wide local excision. It can also develop de novo, presenting most commonly as a mass or nodule. Tissue biopsy with histopathology is required to confirm the diagnosis. Wide local excision is recommended for local disease. Radiation and chemotherapy can be used as adjuncts in advanced and metastatic disease. Given its rarity, there are no guidelines to direct therapy for locally advance or metastatic disease and for follow-up. Further studies are needed to better understand and guide management of this entity.

Eccrine Porocarcinoma: A Review of the Literature

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.

Epidemiology of porocarcinoma in England 2013-2018: apopulation-based registry study.

Porocarcinoma (PC) is a cutaneous malignancy that differentiates towards (possibly arises from) the sweat ducts and glands. Lack of histological diagnostic markers makes clinical and pathological diagnosis complex. The limited data available suggest the incidence is increasing; however, this remains to be established in national epidemiological studies. To report the incidence, treatment and survival of patients with PC in England from 1 January 2013 to 31 December 2018 using national cancer registry data. PC diagnoses in England during 2013-2018 were identified from the National Disease Registration Service using morphology and behaviour codes. These were registered from routinely collected pathology reports and cancer outcomes and services datasets. The 2013 European age standardized incidence rates (EASRs), Kaplan-Meier all-cause survival and log-rank test were calculated. In total, 738 tumours (396 in males and 342 in females) were diagnosed. The median age at diagnosis was 82 years old (interquartile range 74-88). The most frequently affected site were lower limbs (35.4%), followed by the face (16%). The majority of the cohort received surgical excision (73.0%). The Kaplan-Meier all-cause survival was 45.4% at 5 years, which was lower than in previous studies. The EASR for the whole population was 0.25 [95% confidence interval (CI) 0.23-0.27] per 100 000 person-years (PY)]. PC incidence rates in the East of England (EASR of 0.54, 95% CI 0.47-0.63 per 100 000 PY) were three times higher than the South West (EASR of 0.14, 95% CI 0.10-0.19 per 100 000 PY) where the regional rates were the lowest. This study shows that there is large variation in the EASRs of PC across England. This may reflect differences in how PC is diagnosed and registered in different regions in England. These data support national assessment of the management of PC, which will inform future studies and guideline development.

Eccrine Porocarcinoma of Dorsum of Nose: A Rare Case Report.

Eccrine porocarcinoma (EPC) is an uncommon malignant tumor derived from the eccrine sweat glands. Because of its various pathological features, it is often confused with other malignant cutaneous tumors. We present a case of 78-year- old female having ulcerative lesion over external nasal pyramid. Biopsy suggested of squamous cell carcinoma. Tumor was excised and reconstruction was done using paramedian forehead flap. Post operative Histopathological Examination (HPE) suggested of eccrine porocarcinoma.

Immunohistochemical and clinicopathological study regarding nardilysin on extramammary Paget's disease.

It has been reported that nardilysin (NRDC), a metalloendopeptidase which regulates various growth factors and cytokines, is associated with malignancies in a conflicting manner, in which it promoted gastric, hepatocellular, and colorectal cancers and suppressed pancreatic ductal adenocarcinoma. However, it has not been investigated how NRDC is associated with cutaneous malignancies for now. Immunohistochemical staining has revealed that NRDC expression is observed in all extramammary Paget's disease (EMPD) cases. Notably, other cutaneous malignancies including basal cell carcinoma, squamous cell carcinoma, and eccrine porocarcinoma, did not show increased NRDC expression in immunohistochemistry. EMPD typically presents several types of lesions including nodules, and positive staining of NRDC on EMPD was observed regardless of the type of lesions. Examination using samples taken from nodular lesions showed that some cases showed heterogenous NRDC expression within each lesion. We also found that NRDC staining was weaker in the marginal parts of EMPD lesion than in the central parts in several cases, and tumor cells tend to be distributed beyond the macroscopic skin lesions in these cases. It was speculated that decreased NRDC expression in the marginal zones of the skin lesions may be associated with the ability of tumor cells to produce the cutaneous manifestation of EMPD. This study suggests that NRDC may be associated with EMPD like other malignancies reported previously.

Distinct regulations driving YAP1 expression loss in poroma, porocarcinoma and RB1-deficient skin carcinoma.

Recently, YAP1 fusion genes have been demonstrated in eccrine poroma and porocarcinoma, and the diagnostic use of YAP1 immunohistochemistry has been highlighted in this setting. In other organs,loss of YAP1 expression can reflect YAP1 rearrangement or transcriptional repression, notably through RB1 inactivation. In this context, our objective was to re-evaluate the performance of YAP1 immunohistochemistry for the diagnosis of poroma and porocarcinoma. The expression of the C-terminal part of the YAP1 protein was evaluated by immunohistochemistry in 543 cutaneous epithelial tumours, including 27 poromas, 14 porocarcinomas and 502 other cutaneous tumours. Tumours that showed a lack of expression of YAP1 were further investigated for Rb by immunohistochemistry and for fusion transcripts by real-time PCR (YAP1::MAML2 and YAP1::NUTM1). The absence of YAP1 expression was observed in 24 cases of poroma (89%), 10 porocarcinoma (72%), 162 Merkel cell carcinoma (98%), 14 squamous cell carcinoma (SCC) (15%), one trichoblastoma and one sebaceoma. Fusions of YAP1 were detected in only 16 cases of poroma (n=66%), 10 porocarcinoma (71%) all lacking YAP1 expression, and in one sebaceoma. The loss of Rb expression was detected in all cases except one of YAP1-deficient SCC (n=14), such tumours showing significant morphological overlap with porocarcinoma. In-vitro experiments in HaCat cells showed that RB1 knockdown resulted in repression of YAP1 protein expression. In addition to gene fusion, we report that transcriptional repression of YAP1 can be observed in skin tumours with RB1 inactivation, including MCC and a subset of SCC.

Porocarcinoma as a rare case of malignant skin tumor

Background: Porocarcinoma is a rare and malignant tumor of the eccrine sweat glands of the intraepidermal ductal portion that accounts for approximately 0.005% to 0.01% of skin tumors. It was first described in 1963 by Pinkus and Mehregan and was called epidermotropic ecrino carcinoma. Porocarcinoma usually occurs in the lower extremities 44%, trunk 24% and head 18%, in older people (average age 65 years), with no gender preference. Because eccrine porocarcinoma is a rare entity, it can be misdiagnosed as squamous cell carcinoma, basal cell carcinoma, pyogenic granuloma, amelanotic melanoma, Bowen's disease, fibroid, common wart, or metastatic adenocarcinoma. It has a variable clinical appearance which delays diagnosis. Multinodularity, ulceration, size and rapid growth are risk factors.

18F Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Findings in a Case of Metastatic Eccrine Porocarcinoma - An Extremely Rare Malignant Adnexal Tumor.

Porocarcinoma is a rare malignant neoplasm of eccrine sweat glands representing 0.005 to 0.1% of all cutaneous tumors. As eccrine porocarcinoma carries a high risk of recurrence and metastases, early diagnosis and management are crucial to lower mortality rate. We present the case of porocarcinoma in a 69-years-old woman who underwent 18F-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) for staging the disease. PET/CT showed metabolically active multiple cutaneous lesions and also picked up lymph nodal and distant metastases to lungs and breast accurately. PET/CT is useful for accurate staging of the disease and for treatment planning.

A peculiar case of eccrine porocarcinoma of the pinna

A peculiar case of eccrine porocarcinoma of the pinna

Epidemiology and Survival of Eccrine Porocarcinoma by Sex in the United States: A Surveillance, Epidemiology, and End Results Database Analysis.

Epidemiology and Survival of Eccrine Porocarcinoma by Sex in the United States: A Surveillance, Epidemiology, and End Results Database Analysis.