Porocarcinoma: Clinical and Histological Features, Immunohistochemistry and Outcomes: A Systematic Review.

Porocarcinoma (PC) is a rare adnexal tumor, mainly found in the elderly. The tumor arises from the acrosyringium of eccrine sweat glands. The risk of lymph node and distant metastasis is high. Differential diagnosis with squamous cell carcinoma is difficult, although NUT expression and YAP1 fusion products can be very useful for diagnosis. Currently, wide local excision is the main surgical treatment, although Mohs micrographic surgery is promising. To date, there is no consensus regarding the role of sentinel lymph node biopsy and consequential lymph node dissection. No guidelines exist for radiotherapy, which is mostly performed based on tumor characteristics and excision margins. Only a few studies report systemic treatment for advanced PC, although therapy with pembrolizumab and EGFR inhibitors show promise. In this review, we discuss epidemiology, clinical features, histopathological features, immunohistochemistry and fusion products, surgical management and survival outcomes according to stage, surgical management, radiotherapy and systemic therapy.

Eccrine porocarcinoma: case report and review of the literature.

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.

Eccrine Porocarcinoma: New Insights and a Systematic Review of the Literature.

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.

Eccrine Porocarcinoma: Patient Characteristics, Clinical and Histopathologic Features, and Treatment in 7 Cases. / Porocarcinoma ecrino: aspectos demográficos, clínicos, histopatológicos y terapéuticos en una serie de 7 pacientes.

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.

Long-term survival after multidisciplinary management of a metastatic sarcomatoid porocarcinoma with repeated exeresis, radiotherapy, chemotherapy and cetuximab: case report and review of literature.

Eccrine porocarcinoma is a rare and aggressive skin neoplasm; only two cases of sarcomatoid differentiation have been reported. Whereas surgery is effective as first line treatment, optimal management of recurrent or metastatic porocarcinoma is not defined and needs multidisciplinary approach. Here we described the first reported case of metastatic sarcomatoid porocarcinoma. Our patient experienced multiple recurrences, mainly loco-regional, and was treated with a multidisciplinary treatment, involving surgery, radiotherapy, chemotherapy and target therapy, leading to a more than 4 years survival, from the first recurrence. We conclude that multidisciplinary approach in metastatic porocarcinoma must involve surgeon, radiotherapist and medical oncologist. The combination of local and systemic treatments can delay recurrence and prolong survival also in very aggressive cases.

Eccrine porocarcinoma of the vulva: a case report and review of the literature.

BACKGROUND: Malignant tumors arising from the vulva account for only 0.6 % of all cancers in female patients. The predominant histologic type, representing about 90 % of these malignancies, is squamous cell carcinoma. Eccrine porocarcinoma is a rare malignant tumor arising from sweat glands. The incidence of eccrine porocarcinoma is estimated at 0.005-0.01 % of all cutaneous tumors. To the best of our knowledge, only seven previous cases of vulvar eccrine porocarcinoma have been reported in the English-language literature. We present the case of a patient with eccrine porocarcinoma of the vulva, and we summarize the clinical features of this disease using seven previously reported cases. CASE PRESENTATION: A 54-year-old Japanese woman visited a local hospital complaining of fever and left vulvar pain for 2 months. An initial examination revealed a 1 × 1 cm, firm, ulcerative mass in the inner aspect of the left labium minorum. With a preoperative diagnosis of vulvar squamous cell carcinoma, we performed a radical local excision followed by bilateral inguinal lymphadenectomy. Histological examination showed eccrine porocarcinoma, stage IB (T1bN0M0). Radiation therapy with weekly cisplatin administration was then given as adjuvant therapy. One month after treatment was completed, computed tomography revealed multiple metastases in the bilateral lungs and in the sacral bone. The patient received three courses of chemotherapy (paclitaxel and carboplatin) and underwent palliative radiation therapy to the sacrum. She died of her disease 12 months after surgery. CONCLUSIONS: We report the case of a patient with eccrine porocarcinoma of the vulva and summarize the clinical features and the treatment options of eccrine porocarcinoma from a few retrospective case reports. Although eccrine porocarcinoma is a rare disease, clinicians and pathologists should be aware of its clinical and histological features and its biological behavior.

[A Case of Breast Metastasis of Eccrine Porocarcinoma].

An 80's woman was diagnosed with eccrine porocarcinoma of the head in 2010.T he tumor was removed surgically but relapsed in the cervical and axillary lymph nodes 2 years later.The patient underwent surgery, and received systemic chemotherapy and radiation.Chest CT after treatment revealed an irregular mass and thickened skin in the left breast.Core needle biopsy specimens were used to diagnose metastasis of eccrine porocarcinoma.A wide excision with a 1 cm margin was performed under local anesthesia.After surgery, supraclavicular lymph node recurrence was detected.The patient received palliative care because there was no effective treatment available.Eccrine porocarcinoma is a rare malignant tumor of the intraepidermal sweat duct.Breast metastasis from malignant disease is also rare.To our knowledge, breast metastasis of eccrine porocarcinoma has not been reported.

Nonmelanoma skin cancer.

Nonmelanoma skin cancer (NMSC) is the most common form of malignancy in humans. The incidence of NMSC continues to increase despite increased awareness and sun-protective measures. If neglected or mismanaged, NMSC can cause significant morbidity and even death. The most common forms of NMSC on the head and neck include basal cell carcinoma, squamous cell carcinoma, sebaceous carcinoma, eccrine porocarcinoma, Merkel cell carcinoma, atypical fibroxanthoma, and microcystic adnexal carcinoma. Surgery is the mainstay of treatment (standard excision, Mohs micrographic surgery, curettage); however, other modalities exist, including radiation, topical immunomodulators, photodynamic therapy, and new systemic medications.

[Neglected skin tumors. Three cases]. / Tumeurs "historiques": tumeurs négligées. Trois observations.

BACKGROUND: Giant skin tumours are defined as greater than 10 cm in size and are frequently a consequence of neglect. We report three cases of giant skin tumours and discuss possible factors associated with delayed diagnosis and treatment. OBSERVATIONS: Two men and one woman, aged 52, 62 and 78 years, presented with giant skin tumours. One was mentally retarded, one had a borderline personality and another was presenting a major depressive disorder. Pain, smell, bleeding and/or loss of function forced the patients to seek medical care. Tumour sizes were 11 to 30 cm and were present for between six months and 20 years. All patients had hidden their lesion from their doctor and families. In one case, a sebaceous carcinoma of the right shoulder required amputation of the right upper limb. In a second, palliative surgery and medical care was given for a metastatic porocarcinoma. The last patient received neoadjuvant chemotherapy for an undifferentiated carcinoma. DISCUSSION: Neglected skin tumours continue to be encountered even in 2011. Consultation was delayed due to fear of the diagnosis and/or lack of corresponding physicians, resulting in worsening of the prognosis. A larger scale study could help us to identify the factors associated with neglected giant tumours and to improve their management.