Malignant Adnexal Tumors Research Articles

Predicting Malignancy in Adnexal Tumors With FAPI PET/CT and FDG PET/CT: A Case Report on a Borderline Ovarian Tumor.

A 54-year-old woman presented with a 55 × 64-mm tumor in the ovary with high [18F]FDG uptake on [18F]FDG PET/CT highly suggestive of ovarian cancer. Prior to surgery, the patient underwent [68Ga]-Ga- fibroblast activation protein inhibitor (FAPI)-46 PET/CT, which revealed low [68Ga]-Ga-FAPI-46 tumor uptake. Histopathology revealed a borderline ovarian tumor, which has low malignant potential and a 10-year survival rate greater than 93%. High [18F]FDG uptake is considered a fair predictor of malignancy in adnexal tumors. The present case demonstrates the potential superiority of [68Ga]-Ga-FAPI-46 PET/CT over [18F]-FDG PET/CT in differentiating malignant adnexal tumors from borderline ovarian tumors.

Enhanced Disease-Specific Survival Among Individuals With Malignant Adnexal Tumors of the Skin Treated With Mohs Surgery: A National Database Study.

Malignant adnexal tumors of the skin are a rare group of tumors that can be locally aggressive and require surgical excision with strict margin control to achieve clearance. Given the rarity of these tumors, there is a limited understanding within the medical community regarding optimal treatment approaches. To examine surgical management trends and outcomes for patients diagnosed with cutaneous adnexal tumors from 2000 to 2020. The Surveillance, Epidemiology, and End Results database was queried to identify biopsy-proven cases of cutaneous adnexal tumors between 2000 and 2020. Clinical and disease characteristics were examined, and disease-specific survivals were compared between surgical approaches using Kaplan-Meier curves and Cox proportional hazards models. Mohs surgery demonstrated a significant increase in utilization from 2000 to 2020 (+447.1%) and improvements in disease-specific survival (mean 231.7 months; p < .001) compared with no surgery; wide local excision exhibited no improved disease-specific survival (227.7 months; p = .070). Multivariable Cox regression further highlighted that only Mohs surgery exhibited a reduced disease-specific mortality risk compared with no surgery (adjusted HR 0.49; p = .011). Given the enhanced disease-specific survival coupled with tissue preservation strategies, Mohs surgery emerges as a promising surgical approach for the treatment of malignant adnexal tumors of the skin.

S1‐Leitlinie Talgdrüsenkarzinom

Sebaceous gland carcinomas are rare malignant cutaneous adnexal tumors with sebocytic differentiation. The typical predilection area is the head and neck region, where sebaceous gland carcinomas are the most common malignant adnexal tumors of the skin. According to their localization a distinction is made between periocular and extraocular sebaceous gland carcinomas. Muir-Torre syndrome (MTS) should always be ruled out if it is suspected. In terms of prognosis, sebaceous gland carcinomas are potentially aggressive tumors with a clear tendency to recur and metastasize. Only small extraocular sebaceous gland carcinomas that have been completely resected have a very good prognosis. Sebaceous gland carcinomas most frequently metastasize lymphogenously to regional or distant lymph nodes; organ metastasis occurs less frequently. Periocular sebaceous gland carcinomas have a higher metastasis rate (up to 15%) than extraocular sebaceous gland carcinomas (up to 2%). Complete micrographically controlled surgery (MCS) of the primary tumor is the therapy of first choice, regardless of periocular or extraocular localization. Adjuvant or therapeutic radiotherapy may be considered. There is currently no established standard therapy for advanced, inoperable or metastatic sebaceous gland carcinomas. Local procedures and system therapies such as chemotherapy or immunotherapy can be considered. The procedure should be determined individually in an interdisciplinary tumor board. Close follow-up care is recommended for these potentially aggressive carcinomas.

S1-Guideline Sebaceous Carcinoma.

Sebaceous gland carcinomas are rare malignant cutaneous adnexal tumors with sebocytic differentiation. The typical predilection area is the head and neck region, where sebaceous gland carcinomas are the most common malignant adnexal tumors of the skin. According to their localization a distinction is made between periocular and extraocular sebaceous gland carcinomas. Muir-Torre syndrome (MTS) should always be ruled out if it is suspected. In terms of prognosis, sebaceous gland carcinomas are potentially aggressive tumors with a clear tendency to recur and metastasize. Only small extraocular sebaceous gland carcinomas that have been completely resected have a very good prognosis. Sebaceous gland carcinomas most frequently metastasize lymphogenously to regional or distant lymph nodes; organ metastasis occurs less frequently. Periocular sebaceous gland carcinomas have a higher metastasis rate (up to 15%) than extraocular sebaceous gland carcinomas (up to 2%). Complete micrographically controlled surgery (MCS) of the primary tumor is the therapy of first choice, regardless of periocular or extraocular localization. Adjuvant or therapeutic radiotherapy may be considered. There is currently no established standard therapy for advanced, inoperable, or metastatic sebaceous gland carcinomas. Local procedures and systemic therapies such as chemotherapy or immunotherapy can be considered. The procedure should be determined individually by an interdisciplinary tumor board. Close follow-up care is recommended for these potentially aggressive carcinomas.

Extraocular Sebaceous Carcinoma in the Lower Back: Rare Presentation of a Great Masquerader

Abstract Malignant adnexal tumors of the skin are rare. Sebaceous carcinoma (SC) is an aggressive malignant tumor of the sebaceous glands. It is traditionally classified into periocular and extraocular types. We present a rare case of extraocular SC of a 38-year-old female who presented with nontender swelling over the right flank for 1 year. Clinically, it was diagnosed with a sebaceous cyst. The tumor was excised and sent for histopathological examination. Sections showed sheets and lobules of tumor cells separated by fibrovascular stroma. Individual tumor cells were atypical basaloid to clear cells with prominent pleomorphism. Mitosis was brisk with areas of necrosis. On immunohistochemistry, the tumor cells were focally positive for epithelial membrane antigen and negative for S100 and PAX8 with a high Ki-67 index of 80%. Her postoperative period and follow-up for 1 year have been uneventful. This case is being reported for its rarity.

Sebaceous carcinoma in a 54-year-old Black African man after cancer chemotherapy: a case report

BackgroundSebaceous carcinoma is a very rare malignant skin adnexal tumor that is occasionally aggressive. We have not seen a case of sebaceous carcinoma in our center in the last 10 years. It is extremely rare in Black Africans.Case presentationWe described the case of a 55-year-old man African man who presented to our ophthalmologist with complaints of growth on the right upper eyelid for 8 months. He had surgery and chemotherapy for rectal carcinoma 6 years prior to presentation and received his last dose of chemotherapy 5 years before seeing our ophthalmologist. There was a history of spontaneous unprovoked bleeding from the lesion. He subsequently underwent surgical excision under general anesthesia. Histology of the mass showed an effaced architecture due to proliferating malignant epithelial cells disposed as trabecules, solid nests, and tongues. The microscopic features of widespread multivacuolated cytoplasm of the neoplastic cells led us to conclude that the tumor was a sebaceous carcinoma. The patient is alive and well.ConclusionSebaceous carcinoma is a rare malignant skin adnexal tumor in Black Africans. It can present as an eyelid mass with spontaneous bleeding. It can follow cancer chemotherapy either because of its association with other tumors in Muir–Torre syndrome or because of mutagenic effects of chemotherapeutic agents.

Malignant Adnexal Carcinoma of the Face: A Case Report

Malignant adnexal tumors (MAT) form a group of rare, typically low-grade- malignancy carcinomas with follicular, sebaceous, apocrine, or eccrine differentiation or a combination of the first 3 subtypes, the biopsy is required to establish the differentiation subtype and the definitive diagnosis. our case is a 67 years old female presented to our department of maxillofacial surgery of Ibn Sina hospital in Rabat with a left frontal skin lesion that had begun evolving 6 months ago. An excisional biopsy was performed afterwards using an elliptical excision with 1 cm of margins laterally and including the periost deeply, and then the sking defect was sutured in 3 layers. Malignant pilosebaceous tumors are more rare, especially those of the hair follicle. Sebaceous gland carcinoma commonly arises in the periorbital area. Surgical resection is the most commonly implemented treatment modality in MATs.

O-RADS MRI scoring system has the potential to reduce the frequency of avoidable adnexal surgery

ObjectiveTo assess the potential impact of the O-RADS MRI score on the decision-making process for the management of adnexal masses. MethodsEURAD database (prospective, European observational, multicenter study) was queried to identify asymptomatic women without history of infertility included between March 1st and March 31st 2018, with available surgical pathology or clinical findings at 2-year clinical follow-up. Blinded to final diagnosis, we stratified patients into five categories according to the O-RADS MRI score (absent i.e. non adnexal, benign, probably benign, indeterminate, probably malignant). Prospective management was compared to theoretical management according to the score established as following: those with presumed benign masses (scored O–RADS MRI 2 or 3) (follow-up recommended) and those with presumed malignant masses (scored O–RADS MRI 4 or 5) (surgery recommended). ResultsThe accuracy of the score for assessing the origin of the mass was of 97.2 % (564/580, CI95% 0.96–0.98) and was of 92.0 % (484/526) for categorizing lesions with a negative predictive value of 98.1 % (415/423, CI95% 0.96–0.99). Theoretical management using the score would have spared surgery in 229 patients (87.1 %, 229/263) with benign lesions and malignancy would have been missed in 6 borderline and 2 invasive cases. In patients with a presumed benign mass using O-RADS MRI score, recommending surgery for lesions >= 100 mm would miss only 4/77 (4.8 %) malignant adnexal tumors instead of 8 (50 % decrease). ConclusionThe use of O-RADS MRI scoring system could drastically reduce the number of asymptomatic patients undergoing avoidable surgery.

Diagnostic approach to adnexal lesions in young females in their second and third decades

BackgroundTo review the imaging options and features of different ovarian lesions in young females. We also aimed to put a simple approach to reach the appropriate diagnosis.Patients and methodsA retrospective study of 120 patients were included in their second and third decades. All cases had examined by ultrasound then other preferred imaging modalities were used either CT or MRI to reach the appropriate diagnosis. Further, serological and pathological assessments were done in some cases.ResultsOvarian pathologies were 89.1% and extra-ovarian adnexal lesions were 10.8%. Non-tumoral ovarian pathologies were the upper hand in both decades, followed by benign tumors in the first decade; however, the overall malignancy was common in the second decade. The ultrasound provided substantial proof of the diagnosis in 34.1% without further need for advanced imaging with a sensitivity and specificity of 100% besides it helped in the discrimination between benignity and malignancy of the ovarian tumors with calculated overall sensitivity and specificity of 93.7 and 88%, respectively. MRI and DWI have raised the sensitivity and specificity up to 95.4% and 96.3% respectively in the setting of ovarian tumor's nature discrimination.ConclusionsThe majority of adnexal lesions were benign in this age group. It was not always possible to distinguish between benign and malignant adnexal tumors using ultrasound only, so adding CT and /or MRI for more characterization of some lesions raises the diagnostic performance of the diagnosis to achieve proper management for saving fertility and crucial when ovarian cancer discovered to help adjuvant therapy and enhance patient outcomes.

Case of Malignant Proliferating Trichilemmal Tumour in Gluteal Region

Malignant Proliferating Trichilemmal Tumour (MPTT) is a rare, malignant cutaneous adnexal tumour that originates from the outer root sheath cells of the isthmus of hair follicles. This article aims to report a rare case of a high-grade malignant trichilemmal tumour arising in the context of a proliferating Trichilemmal Cyst (TC) in a 65-year-old female who presented with a painless swelling in the left gluteal region that had been present for two months. There have been only about 100 cases reported so far. Both the clinical features and the imaging findings were suggestive of an ostensibly benign lesion. As a result, the cyst was excised; however, further histopathological evaluation revealed an unexpected case of rare MPTT. Consequently, a timely second revision surgery was performed to ensure tumour-free margins. This case illustrates that the diagnosis of MPTT is highly challenging. Without prior knowledge and a high index of suspicion, the diagnosis of MPTT could easily be missed. MPTT is often misdiagnosed as a variant of Squamous Cell Carcinoma (SCC). The subtle nuances of the unique histopathological pattern of MPTT can help differentiate it from SCC.

Разработка нового нанобиотехнологического метода и его применение для дифференциальной диагностики опухолей придаточного аппарата глаза

Aim: to develop a new nanobiotechnology method for the differential diagnosis of ocular adnexal tumors. Patients and Methods: 192 eyelid or conjunctival tumor samples from 192 patients were examined. Controls were healthy tissue samples from the same eye. To register and obtain significant differences in the generation of biogenic silver nanoparticles in situ, the key parameters of the reaction of the reduction of silver cations were selected and optimized. To generate de novo biogenic silver nanoparticles, tissue samples were placed in a sterile solution of a cation source in aseptic conditions. Nanoparticles were generated in situ using a sterile solution of Tollens' reagent (Ag(HN3)2NO3). Results: the optimal parameters of a new nanobiotechnology method were established to conduct the reduction reaction to generate biogenic silver nanoparticles. The required concentration of Tollens' reagent (source of cations) was 63 μg/ml. The wavelength used to observe the de novo generation of biogenic silver nanoparticles (λ) was 410 nm. Obtaining the most significant changes in the optical density of the reaction mixture with biogenic nanoparticles required 10 to 20 min, although the first changes were documented starting from the 5th minute of the reduction reaction. Benign and malignant tumors are characterized by different metabolic activities, i.e., benign eyelid and conjunctival tumors reduce cations more slowly than malignant tumors. The parameters of reducibility of benign ocular adnexal tumors did not significantly differ depending on tumor location and type. Among the malignant conjunctival tumors, melanoma is characterized by the highest reducibility p&lt;0.05). Among the malignant eyelid tumors, no significant differences were found for different tumor types. Conclusions: this novel nanobiotechnology method provides a differential diagnosis of benign and malignant ocular adnexal tumors. The practical relevance of this method lies in differentiating benign and malignant tumors to schedule management strategies and surgical interventions. KEYWORDS: tumor, eyelids, conjunctiva, silver nanoparticles, biogenic synthesis, express diagnostics, ocular oncology. FOR CITATION: Saakyan S.V., Skladnev D.A., Alekseeva A.P., Beznos O.V., Sorokin V.V. Development of a new nanobiotechnology method for the differential diagnosis of ocular adnexal tumors. Russian Journal of Clinical Ophthalmology. 2024;24(1):25–29 (in Russ.). DOI: 10.32364/2311-7729-2024-24-1-5.

A clinico-histopathological study of skin appendageal tumours in a tertiary health care centre in western Odisha– A case series

Skin appendageal tumours (SATs) are those neoplasms that arise from pilosebaceous, apocrine, or eccrine sweat glands. These are a diverse group of tumours with both benign and malignant counterparts. They can be single, multiple, or have a syndromic association with internal malignancies. Benign adnexal tumours are more common, while malignant adnexal tumours are rare, usually locally aggressive, and have the potential for nodal involvement and distant metastasis with a poor clinical outcome. Therefore, proper diagnosis of SATs is important for therapeutic and prognostic reasons.This study aims to determine the clinico-histopathological correlation in cases of SATs in our hospital. It is a case series conducted over a one-and-a-half year period from January 2021 to July 2022. All clinically suspected cases of SATs were examined, biopsied, and subjected to histopathological examination. Histopathologically confirmed cases of SATs were finally analysed. Among twenty-four thousand two hundred twenty-four new patients attending OPD, 30 suspected cases of ATs underwent histopathological examination. Histopathology was confirmatory in only 12 cases (40%). 10 cases were benign, and two were malignant. Out of 10 benign cases, hidradenoma was noted in 3 (25%), trichoepithelioma in 2 (16%), proliferating pilar tumour, apocrine hidrocystoma, steatocystoma multiplex, hidradenoma papilliferum, and pilomatrixoma were seen in one each. Females (75%) outnumbered males (25%) in our study population.: SATs are infrequent and are not frequently observed in the field of surgical pathology. Histopathology is the gold standard for diagnosis.

Subjective assessment and IOTA ADNEX model in evaluation of adnexal masses in patients with history of breast cancer.

To evaluate the performance of subjective assessment and the Assessment of Different NEoplasias in the adneXa (ADNEX) model in discriminating between benign and malignant adnexal tumors and between metastatic and primary adnexal tumors in patients with a personal history of breast cancer. This was a retrospective single-center study including patients with a history of breast cancer who underwent surgery for an adnexal mass between 2013 and 2020. All patients had been examined with transvaginal or transrectal ultrasound using a standardized examination technique and all ultrasound reports had been stored and were retrieved for the purposes of this study. The specific diagnosis suggested by the original ultrasound examiner in the retrieved report was analyzed. For each mass, the ADNEX model risks were calculated prospectively and the highest relative risk was used to categorize each into one of five categories (benign, borderline, primary Stage I, primary Stages II-IV or metastatic ovarian cancer) for analysis of the ADNEX model in predicting the specific tumor type. The performance of subjective assessment and the ADNEX model in discriminating between benign and malignant adnexal tumors and between primary and metastatic adnexal tumors was evaluated, using final histology as the reference standard. Included in the study were 202 women with a history of breast cancer who underwent surgery for an adnexal mass. At histology, 93/202 (46.0%) masses were benign, 76/202 (37.6%) were primary malignancies (four borderline and 72 invasive tumors) and 33/202 (16.3%) were metastases. The original ultrasound examiner classified correctly 79/93 (84.9%) benign adnexal masses, 72/76 (94.7%) primary adnexal malignancies and 30/33 (90.9%) metastatic tumors. Subjective ultrasound evaluation had a sensitivity of 93.6%, specificity of 84.9% and accuracy of 89.6%, while the ADNEX model had higher sensitivity (98.2%) but lower specificity (78.5%), with similar accuracy (89.1%), in discriminating between benign and malignant ovarian masses. Subjective evaluation had a sensitivity of 51.5%, specificity of 88.8% and accuracy of 82.7% in distinguishing metastatic and primary tumors (including benign, borderline and invasive tumors), and the ADNEX model had a sensitivity of 63.6%, specificity of 84.6% and similar accuracy (81.2%). The performance of subjective assessment and the ADNEX model in discriminating between benign and malignant adnexal masses in this series of patients with history of breast cancer was relatively similar. Both subjective assessment and the ADNEX model demonstrated good accuracy and specificity in discriminating between metastatic and primary tumors, but the sensitivity was low. © 2023 International Society of Ultrasound in Obstetrics and Gynecology.

42330 Predictors of Receipt of Sentinel Lymph Node Biopsy in Patients with Malignant Cutaneous Adnexal Tumors

42330 Predictors of Receipt of Sentinel Lymph Node Biopsy in Patients with Malignant Cutaneous Adnexal Tumors

O-RADS combined with contrast-enhanced ultrasound in risk stratification of adnexal masses

BackgroundOvarian-Adnexal Reporting and Data System (O-RADS) for ultrasound is a lexicon and risk stratification system that includes all risk categories and relevant management recommendation. It has high sensitivity in diagnosing malignant adnexal tumors, but the specificity is lower.ObjectiveTo explore the value of O-RADS combined with contrast-enhanced ultrasound (CEUS) in risk stratification of adnexal masses.MethodsA retrospective study was performed on 85 patients with 100 adnexal masses that preoperatively underwent conventional ultrasound as well as CEUS examination and obtained the postoperative pathological results. The masses were classified into O-RADS2, 3, 4, and 5 by conventional ultrasound. After contrast enhancement, the classification of O-RADS was adjusted according to CEUS imaging features. The O-RADS 2 and 3 lesions with suspected malignant features like irregular blood vessels or internal inhomogeneous hyperenhancement were upgraded to O-RADS 4, and the O-RADS 4 lesions with the above features were upgraded to O-RADS 5. The O-RADS 4 lesions with suspicious benign angiographic features like a regular vessel, interior hypoenhancement or non-enhancement were downgraded to O-RADS 3; the O-RADS 5 lesions with rim ring-enhancement and interior non-enhancement were downgraded to O-RADS 3. The sensitivity, specificity, accuracy, PPV, NPV, and AUC of the two methods were compared, taking pathological results as the gold standard.ResultsThe sensitivity, specificity, accuracy, PPV, NPV, and AUC of O-RADS and O-RADS combined with CEUS in the diagnosis of malignant adnexal tumors were 96.6%, 66.2%, 75.0%, 53.8%, 97.9%, 0.910 and 96.6%, 91.5%, 93.0%, 82.4%, 98.5%, 0.962, respectively. The specificity, accuracy, PPV, and AUC of O-RADS combined with CEUS were considerably higher than those of O-RADS (P < 0.01). Furthermore, both methods had excellent sensitivity and NPV but there were no significant differences between them(P > 0.05).ConclusionCombination of O-RADS and CEUS can significantly improve the specificity and PPV in diagnosing malignant adnexal tumors. It seems promising in the clinical application of risk stratification of adnexal masses.

A rapidly-growing erythematous nodule on the right upper neck

A rapidly-growing erythematous nodule on the right upper neck

Pregnant Women With Malignant Ovarian Tumors: A Case Series

Ovarian cancer management during pregnancy is a topic of limited research due to low occurrence rates of malignant adnexal tumors. To shed further light on this issue, we present a case series of 22 pregnant ovarian cancer patients referred to an academic hospital's gynecology oncology department over six years. Data on each patient's demographic and clinical background were collected using a registry software recording surgical tumor staging, disease-free survival (DFS), and overall survival (OS). According to the data analysis reports, subtype epithelial tumor and germ cell pathology were equally 45.4%. However, sex-cord tumors were observed in a smaller percentage of cases (9.1%). Serous adenocarcinoma was the most common subtype among those with epithelial tumors (60%). Meanwhile, 72.7% of these pregnant women had a palpable mass in physical examination. In addition, adnexal mass was detected in 95.4% of ultrasonography. Due to the young age of the patients, fertility-preserving surgery was performed on 63.6% of patients, and chemotherapy was prescripted on 59% of patients. Over a six-year follow-up period, there was a recurrence rate of 22.7%, while DFS and OS were reported as 56% and 82%, respectively. In conclusion, treatment of ovarian malignancies during pregnancy requires an experienced multidisciplinary approach. However, more extensive studies with larger samples are needed to gain more insight into the treatment of ovarian cancer during pregnancy.

A Case Report on Recurrent Trichilemmal Carcinoma of Scalp- Role of Clinico-Radiological Correlation

Purpose: To evaluate diagnosis of Trichilemmal carcinoma scalp with clinico-radiological and pathological correlation. Introduction: Trichilemmal carcinoma is a rare slow growing malignant adnexal tumour that originates from the external hair sheath of hair follicle accounting for &lt;0.005% of adnexal carcinoma 1 . It mimics squamous cell carcinoma, basal cell carcinoma, sebaceous carcinoma. Rarity, indolent clinical course inspite of local aggressiveness and a good survival rate post excision further undermine the importance of this unique entity. Herewith, we are reporting a case of recurrent trichilemmal carcinoma of scalp presented to the outpatient department of Radiation Oncology at our institute

Safety and efficacy of the anti-PD1 immunotherapy with nivolumab in trichoblastic carcinomas.

Trichoblastic carcinoma is a rare malignant cutaneous adnexal tumor with a risk of local invasion and distant metastasis. As of today, there is no consensus for the treatment of locally advanced or metastatic trichoblastic carcinoma. "AcSé Nivolumab" is a multi-center Phase II basket clinical trial (NCT03012581) evaluating the safety and efficacy of nivolumab in several cohorts of rare, advanced cancers. Here we report the results of nivolumab in patients with trichoblastic carcinoma. Of the eleven patients enrolled in the study, five patients had been previously treated by sonic hedgehog inhibitors. The primary endpoint 12-week objective response rate was 9.1% (N = 1/11) with 1 partial response. Six patients who progressed under previous lines of treatment showed stable disease at 12weeks, reflecting a good control of the disease with nivolumab. Furthermore, 54.5% of the patients (N = 6/11) had their disease under control at 6months. The 1-year overall survival was 80%, and the median progression-free survival was 8.4months (95%CI, 5.7 to NA). With 2 responders (2 complete responses), the best response rate to nivolumab at any time was 18.2% (95%CI, 2.3-51.8%). No new safety signals were identified, and adverse events observed herein were previously described and well known with nivolumab monotherapy. These results are promising, suggesting that nivolumab might be an option for patients with advanced trichoblastic carcinomas. Further studies on larger cohorts are necessary to confirm these results and define the role of nivolumab in the treatment of trichoblastic carcinomas.

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.