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Actas Dermo-sifiliograficas May 2017Eccrine porocarcinoma is a rare, malignant cutaneous adnexal tumor that arises from the ducts of sweat glands. Found mainly in patients of advanced age, this tumor has...
Eccrine porocarcinoma is a rare, malignant cutaneous adnexal tumor that arises from the ducts of sweat glands. Found mainly in patients of advanced age, this tumor has diverse clinical presentations. Histology confirms the diagnosis, detects features relevant to prognosis, and guides treatment. Growth is slow, but the prognosis is poor if the tumor metastasizes to lymph nodes or visceral organs. We report 7 cases of eccrine porocarcinoma, describing patient characteristics, the clinical and histopathologic features of the tumors, and treatments used. Our observations were similar to those of other published case series. Given the lack of therapeutic algorithms or protocols for this carcinoma, we propose a decision-making schema based on our review of the literature and our experience with this case series. The algorithm centers on sentinel lymph node biopsy and histologic features.
Topics: Adult; Aged; Aged, 80 and over; Clinical Decision-Making; Disease Management; Eccrine Porocarcinoma; Female; Humans; Lymph Node Excision; Lymphatic Metastasis; Male; Middle Aged; Prognosis; Radiotherapy, Adjuvant; Sentinel Lymph Node Biopsy; Sweat Gland Neoplasms
PubMed: 28110827
DOI: 10.1016/j.ad.2016.04.024 -
The American Journal of Case Reports Nov 2020BACKGROUND Eccrine porocarcinoma (EPC) was first described in 1963 as an epidermotropic eccrine carcinoma. Fifty years later, its etiology remains poorly understood. The...
BACKGROUND Eccrine porocarcinoma (EPC) was first described in 1963 as an epidermotropic eccrine carcinoma. Fifty years later, its etiology remains poorly understood. The infrequent nature of this disease merits further inquiry into its etiology, presentation, and standards of management. Furthermore, the propensity for metastasis, which may be as high as 31% on presentation, increases the importance of investigating this rare disease. CASE REPORT The patient was a 63-year-old mechanic who presented with the lesion as a chronic wound following a chemical exposure. The lesion involved the ulnar aspect of his right palm and had concern for extension to the underlying tendons. He underwent a wide excision extending from the wrist to the proximal interphalangeal joint, preserving the ulnar neurovascular bundle. The hand was reconstructed with an anterolateral thigh fascia perforator flap and a skin graft. He had an excellent functional and cosmetic recovery. Unfortunately, he developed metastases to the lymph nodes, necessitating an axillary lymphadenectomy followed by adjuvant chemoradiation using concurrent cisplatin and docetaxel with radiation for 6 weeks. Follow-up at 18 months found no recurrence. CONCLUSIONS Cases of EPC presenting in the fingers have been managed with amputation of the involved phalanges; however, in addition to obtaining complete excision with negative margins, surgeons who deal with tumors of the hand must also consider the goals of limb preservation, functional preservation, and functional reconstruction. Options for reconstruction following excision include primary closure, dermal regeneration templates, skin grafts, flaps, and free-tissue transfer, depending on what tissue types are needed.
Topics: Eccrine Porocarcinoma; Free Tissue Flaps; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Plastic Surgery Procedures; Sweat Gland Neoplasms; Treatment Outcome
PubMed: 33242313
DOI: 10.12659/AJCR.925231 -
Cancers Mar 2024Eccrine porocarcinoma, sharing many features with other skin tumours, is diagnostically challenging. A conventional biopsy might be misleading and surgical excision... (Review)
Review
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.
PubMed: 38610942
DOI: 10.3390/cancers16071264 -
BMC Surgery Oct 2019Eccrine porocarcinoma is an extremely rare skin adnexal malignant neoplasia with highly invasive and metastatic potential. We report an additional case of eccrine... (Review)
Review
BACKGROUND
Eccrine porocarcinoma is an extremely rare skin adnexal malignant neoplasia with highly invasive and metastatic potential. We report an additional case of eccrine porocarcinoma with intracranial metastases. This case is characterized by a complete record of the progress of eccrine porocarcinoma, its immunohistochemistry after three operations showed a progressive increase in the level of Ki-67 index.
CASE PRESENTATION
We herein report a case of a 37-year-old-male with eccrine carcinoma occurring on the left posterior occipital scalp which invaded the skull and dura, presenting with progressive headache. This patient has performed three surgeries in total. During the last hospitalization, he underwent an extended surgical resection, lymphadenectomy, myocutaneous flap transplantation and vascular anastomosis in our institution. After surgery, he was treating with radiotherapy at 200 Gray in 12 fractions. But one year after the operation, he developed chest tightness, imaging examination and biopsy puncture revealed pulmonary metastasis.
CONCLUSION
Intracranial metastasis of eccrine porocarcinoma is a late event with poor prognosis. This case emphases on that progressively increased level of Ki-67 index may predict more chance to occur the intracranial metastasis of scalp eccrine porocarcinoma, long-term follow-up and appropriately dense follow-up interval is necessary.
Topics: Adult; Eccrine Porocarcinoma; Humans; Immunohistochemistry; Ki-67 Antigen; Lymph Node Excision; Male; Skin Neoplasms; Sweat Gland Neoplasms
PubMed: 31601219
DOI: 10.1186/s12893-019-0595-4 -
World Journal of Surgical Oncology Mar 2011Eccrine porocarcinoma (EPC) is a rare type of skin cancer arising from the intraepidermal portion of eccrine sweat glands or acrosyringium, representing 0.005-0.01% of... (Review)
Review
Eccrine porocarcinoma (EPC) is a rare type of skin cancer arising from the intraepidermal portion of eccrine sweat glands or acrosyringium, representing 0.005-0.01% of all cutaneous tumors. About 20% of EPC will recur and about 20% will metastasize to regional lymph nodes. There is a mortality rate of 67% in patients with lymph node metastases. Although rare, the occurrence of distant metastases has been reported.We report a case of patient with EPC of the left arm, with axillary nodal involvement and subsequent local relapse, treated by complete lymph node dissection and electrochemotherapy (ECT).EPC is an unusual tumor to diagnose. Neither chemotherapy nor radiation therapy has been proven to be of clinical benefit in treating metastatic disease. Although in the current case the short follow-up period is a limitation, we consider in the management of EPC a therapeutic approach involving surgery and ECT, because of its aggressive potential for loregional metastatic spread.
Topics: Adult; Eccrine Porocarcinoma; Humans; Lymphatic Metastasis; Male; Neoplasm Recurrence, Local; Sweat Gland Neoplasms
PubMed: 21410982
DOI: 10.1186/1477-7819-9-32 -
Cureus Nov 2021Eccrine porocarcinoma (EPC) is a rare malignancy of the sweat glands. Currently, there is no standard algorithm for its presentations, diagnosis, and management....
Eccrine porocarcinoma (EPC) is a rare malignancy of the sweat glands. Currently, there is no standard algorithm for its presentations, diagnosis, and management. However, immunotherapy is an emerging option that may be crucial to the treatment of EPC. This report presents a case of a 79-year-old male who had a skin biopsy of an anterior scalp lesion, which revealed EPC. The patient underwent Mohs micrographic surgery to excise the tumor followed by two additional Mohs surgeries for recurrence and adjuvant radiotherapy. A follow-up positron emission tomography (PET) scan revealed yet another recurrence at the scalp as well as metastases to the left parotid gland and left submandibular lymph node. The patient was started on immunotherapy with pembrolizumab, a programmed cell death protein 1 (PD-1)/programmed death-ligand 1 (PD-L1) inhibitor, and later achieved remission. This report demonstrates the effective management of EPC using immunotherapy with pembrolizumab.
PubMed: 34987895
DOI: 10.7759/cureus.20004 -
NPJ Precision Oncology 2018Eccrine porocarcinomas (EPs) are rare malignant tumours of the intraepidermic sweat gland duct and most often arise from benign eccrine poromas. Some recurrent somatic...
Eccrine porocarcinomas (EPs) are rare malignant tumours of the intraepidermic sweat gland duct and most often arise from benign eccrine poromas. Some recurrent somatic genomic events have been identified in these malignancies, but very little is known about the complexity of their molecular pathophysiology. We describe the whole genome and whole transcriptome genomic profiling of a metastatic EP in a 66-year-old male patient with a previous history of localized porocarcinoma of the scalp. Whole genome and whole transcriptome genomic profiling was performed on the metastatic EP. Whole genome sequencing was performed on blood-derived DNA in order to allow a comparison between germline and somatic events. We found somatic copy losses of several tumour suppressor genes including , and , and . We identified a somatic hemizygous pathogenic splice site variant. De novo transcriptome assembly revealed abnormal splicing of p14 and p16. Elevated expression of oncogenes and was noted and no somatic mutations were found in these genes. Wnt pathway somatic alterations were also observed. In conclusion, our results suggest that the molecular pathophysiology of malignant EP features high complexity and subtle interactions of multiple key genes. Cell cycle dysregulation and loss of function was found to be a new potential driver in EP tumourigenesis. Moreover, the combination of somatic copy number variants and abnormal gene expression perhaps partly related to epigenetic mechanisms, all likely contribute to the development of this rare malignancy in our patient.
PubMed: 29872726
DOI: 10.1038/s41698-018-0050-5 -
Dermatology Practical & Conceptual May 2022
PubMed: 35646434
DOI: 10.5826/dpc.1202a79 -
Indian Journal of Dermatology 2019Eccrine porocarcinoma (EPC) is a rare malignant neoplasm which originates from the intraepidermal portion of eccrine sweat glands or acrosyringium. Here, we report a...
Eccrine porocarcinoma (EPC) is a rare malignant neoplasm which originates from the intraepidermal portion of eccrine sweat glands or acrosyringium. Here, we report a unique case of cutaneous metastases of EPC presenting with an erosive plaque on the left thumb and multiple nodules on left forearm. The histopathological examination of the wrist lesion revealed islands of basaloid tumor cells with eosinophilic cytoplasm, downward infiltrating growths, ductal differentiation, and intracytoplasmic lumen formations and focally connected to the epidermis. Acrosyringeal differentiation was confirmed by positive immunohistochemical staining using antibodies to carcinoembryonic antigen (CEA) and epithelial membrane antigen (EMA) in some of porocarcinoma cells. Lymphatic embolization was shown by immunostaining of podoplanin. To the best of our knowledge, this is the first reported EPC case with a primary lesion on the left thumb and zosteriform growth pattern of lymphangitic metastases on left forearm. Our case highlights the diagnostic challenges and reveals lymphangitic metastasis mode of EPC.
PubMed: 31543539
DOI: 10.4103/ijd.IJD_440_18 -
Anais Brasileiros de Dermatologia 2022Eccrine poroma is the term that includes benign neoplasms of the terminal duct of the eccrine sweat glands, which may clinically and dermoscopically resemble other...
Eccrine poroma is the term that includes benign neoplasms of the terminal duct of the eccrine sweat glands, which may clinically and dermoscopically resemble other melanoma and non-melanoma skin tumors. They are often located on the extremities (especially palms and soles), presenting as normochromic or erythematous papules and nodules, measuring up to 2 cm. Pigmented variants are uncommon, accounting for less than 20% of cases. This report describes a 37-year-old man who developed a large pigmented eccrine poroma on his right shoulder, causing diagnostic difficulty. Histopathological examination revealed a nodular neoplasm consisting of small, monomorphic, cuboidal cells, with ample, eosinophilic cytoplasm and well-defined borders, in addition to conspicuous intercellular bridges, with melanin deposits diffusely distributed inside them. The absence of cytological atypia, cellular pleomorphism, increased mitotic activity, and necrosis foci corroborated the diagnostic exclusion of porocarcinoma, which can develop from eccrine poroma.
Topics: Adult; Diagnosis, Differential; Eccrine Porocarcinoma; Humans; Male; Poroma; Skin Neoplasms; Sweat Gland Neoplasms
PubMed: 35811192
DOI: 10.1016/j.abd.2021.10.006