Malignant Eccrine Poroma Research Articles

Diagnosis and Management of Porocarcinoma.

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.

A SURGEON'S SURPRISE TO VERRUCOUS GROWTH OVER CHEEK - CASE REPORT ON MALIGNANT ECCRINE POROMA

Malignant eccrine poroma is a rare malignancy of eccrine glands , whose diagnosis is an index of high suspicion and challenging to a clinician. A case of 67 year old female with swelling over left cheek since 20 years with no radiation exposure and no comorbidities , was investigated and wide local excision with 5mm margins from the tumor was performed , and specimen sent for biopsy - which revealed tumor cells arranged in lobules with peripheral palisading , ductal and squamous differentiation - suggestive of Porocarcinoma.Wide surgical excision is the mainstay of treatment, resulting in cure rates of 70–80% when the margins are clear. Some studies also reported good outcomes with Mohs micrographic surgery, and this technique could also be considered 1. Other treatments such as chemotherapy and radiotherapy have also been reported in the literature without clear standard guidelines.

Extracalvarial Porocarcinoma- A Rare Case Report

Eccrine Porocarcinoma (EPC) is a very rare malignant neoplasm of skin. It usually emerges from the ductal parts of sweat glands. It is seen mostly between the age group of 60 to 80 years with a female preponderance. Several cases of EPC have been reported in medical literature till date. Though, it rarely presents with distant metastasis, still evidences of metastasis to organs like lung, retroperitoneum, breast, urinary bladder and ovaries have been reported. Conventionally, it arises de novo, and may develop on sites of long standing eccrine poroma. Several unusual sites like trunk, head, face, eyelids, upper limbs, and scrotum have been reported; though lower extremities are the most common site of origin. Locoregional recurrence and metastasis have also been reported. Histopathological documentation of mitoses, lymphovascular invasion and measurement of tumour depth predicts adverse prognosis in EPC. Earlier, eccrine adenocarcinoma or malignant eccrine poroma terminology were used. As far as clinical presentation is concerned, the EPC is usually symptomless to begin with, which further ulcerates and may become aching and irritable. From a clinicopathological perspective the importance of the tumour lies in its early correct diagnosis, awareness of the close differentials, adequate surgery and most importantly clearance of surgical margins and adjuvant radiotherapy are mandatory decisions to prevent the high local recurrence. The basis of management involves wide local excision with clear margin. The following case, a 42-year-old male presented with an ulcerated mass on the scalp, which on further evaluation was diagnosed as an EPC of the scalp after histopathologic confirmation. The patient was treated with local wide excision with clear margin and was referred for radiotherapy.

YAP1-MAML2-Rearranged Poroid Squamous Cell Carcinoma (Squamoid Porocarcinoma)\xa0Presenting as a Primary Parotid Gland Tumor

Porocarcinoma (synonym: malignant eccrine poroma) is a rare aggressive carcinoma type with terminal sweat gland duct differentiation. The squamous variant of porocarcinoma is even less frequent and might be indistinguishable from conventional squamous cell carcinoma (SCC). We herein describe the first case of a carcinoma presenting as a primary parotid gland malignancy in a 24-year-old male without any other primary tumor. Total parotidectomy and neck dissection were performed followed by adjuvant chemoradiation. The patient remained alive and well 10 months after diagnosis. Histology showed keratinizing SCC infiltrating extensively the parotid gland with subtle poroid cell features. Oncogenic HPV infection was excluded by DNA-based testing. NGS analysis using the TruSight RNA fusion panel (Illumina) revealed a novel YAP1-MAML2 gene fusion. This gene fusion was reported recently in a subset of cutaneous porocarcinoma and poroma. This case of poroid SCC (or squamoid porocarcinoma) adds to the differential diagnosis of SCC presenting as parotid gland tumor and highlights the value of molecular testing in cases with unusual presentation.

A Useful and Effective Option for the Repair of Facial Defects: Vy Advancement Flaps and Modifications

Objective: V-Y advancement flaps are pliable local flaps that can be used in nearly every part of the body with relatively minimal technical difficulty. These flaps provide the best color and quality match, especially in the facial region. With effective planning, donor site morbidity can be acceptable, even on the face.Methods: The results of 38 patients who underwent 53 V-Y advancement flap facial reconstructions were analyzed retrospectively. The defects were evaluated according to their anatomical region, etiology, postoperative complications, and final aesthetic results.Results: V-Y advancement flaps were used for defects that were either not amenable to primary repair or had a high risk of cosmetic distortion. The defects were located in the cheek (9 patients), nasolabial fold (7 patients), nose (6 patients), forehead (5 patients), preauricular area (3 patients), infraorbital region (3 patients), lower lip (1 patients), gingivobuccal sulcus (2 patients), and medial canthal region (2 patients). In 32 patients, the primary pathology was malignant tumor (22 basal cell carcinomas, 9 epidermoid carcinomas, 1 malignant eccrine poroma). Etiologies of the other patients’ defects were benign tumors (n=2), traumatic wounds (n=2), and contractures (n=2).Conclusions: Despite the widespread use of V-Y flaps in surgical practice, the technique continues to demonstrate additional advantages, particularly in facial reconstruction. They are not only applicable for medium and small defects, but can be used for all defects with sufficient surrounding tissue. They enable various modifications and the elevation of multiple flaps.

Results of Surgical Treatment of Patients with Malignant Eccrine Poroma.

Objectives:Malignant eccrine poroma is a rare cutaneous malignancy. This study was a review of a series of patients with malignant eccrine poroma who underwent surgical treatment conducted in order to evaluate the management techniques and outcomes of treatment modalities.Methods:All cases of surgically excised malignant eccrine poroma performed in a single clinic between 2012 and 2018 were included in the study. The details of patient age, gender, anatomical location of the tumor, histopathological features, and treatment modalities were analyzed.Results:The average tumor size was 2.53 cm (range: 0.3-7 cm). The average tumor thickness was 3.06 mm (range: 2.5-4 mm). The mean clean tumor margin after the first excision was 1.28 mm and the mean tumor margin after the second excision was 8.83 mm. No recurrence or distant metastasis was detected in any of the patients during the follow-up period.Conclusion:Unlike frequent skin cancers, rare skin cancers, like malignant eccrine poroma, don’t have definite treatment algorithms constituted from randomized trials. The findings of patient series are very useful to guide physicians in these cases.

Eccrineporocarcinoma of thigh : A rare adnexal tumour

We report a rare case of eccrine porocarcinoma (EPC) in a 65 year old male patient. EPC arises from the intraepidermal portion of the eccrine sweat glands (acrosyringium). Previously the tumour was known as eccrine adenocarcinoma or malignant eccrine poroma with incidence predominantly in extreme of old age. The tumour arises most commonly on hands and feet. Rare sites like scalp, face, penis and abdomen are also reported in the literature. The distinction of porocarcinoma and basal cell carcinoma can be a diagnostic dilemma on a superficial biopsy. The histological features of high mitotic rate, tumour depth and lymphovascular invasion are bad prognostic markers. Immunohistochemistry for CK15, CK7, epithelial membrane antigen (EMA) and estrogen confirmed the diagnosis of EPC. This tumour is preferably treated with Moh’s surgery though few cases of metastasis are reported. DOI:10.21276/APALM.1713

Malignant Eccrine Poroma of the Vulva: An Intriguing Case of a Rare Tumor at an Unusual Site.

Malignant eccrine poroma is a rare malignancy of the eccrine sweat glands, occurring most frequently on the lower extremities. It affects both sexes equally usually in the 6th to 7th decade of life. Metastasis to regional lymph nodes may occur in 20% that may be fatal in 60% cases. Its aggressive nature, rarity of occurrence, and unusual presentations make it very important to be evaluated properly by the clinician. We hereby report a case of a 75-year-old female presenting with two exophytic tumors over her vulva with local extension. On histopathological examination, it was diagnosed as malignant eccrine poroma. On magnetic resonance imaging of the pelvic region, metastatic extension in regional lymph nodes was found. She was treated by radical vulvectomy with bilateral inguinal and femoral lymph node dissection followed by radiotherapy.

Mohs micrographic surgery for eccrine porocarcinoma: an update and review of the literature.

Eccrine porocarcinoma (EPC) is a rare malignancy of the eccrine sweat glands that is locally aggressive with a high propensity to metastasize. Most cases have been treated by wide local excision (WLE) with 20% local recurrence rate. There have been 20 cases of EPC treated with Mohs micrographic surgery (MMS) in the literature. To review the literature regarding the management of this aggressive tumor using MMS. A comprehensive literature review was conducted by searching the PubMed database using the keywords Mohs, porocarcinoma, malignant eccrine poroma, and eccrine neoplasms. Most of the reported cases of EPC were treated by WLE, and only 20 cases were treated with MMS. Of the 20 EPC cases treated with MMS, there was only 1 nodal recurrence and no local recurrence. Other reported treatment modalities include radiation and excision with frozen sections. The authors report the second case of EPC on the temple, and the 21st case successfully treated with MMS. Eccrine porocarcinoma is a rare neoplasm with potentially aggressive clinical behavior. In cases where tissue conservation is important, MMS should be considered.

Diagnostic dilemma whether Malignant hidroacanthoma or seborrheic keratosis

Eccrine porocarcinoma is a rare cutaneous neoplasm that mainly affects elderly people and grows slowly over a long period but often experiences an accelerated growth phase. Malignant eccrine poroma is a rare skin adnexal tumor arising from the eccrine sweat glands. It arises from different sites in the body extremities, palm and trunk. Porocarcinoma is a rare condition, and we are presenting a case of porocarcinoma of pinna.

English

Malignant eccrine poroma is a rare skin adnexal tumor arising from the eccrine sweat glands. It arises from different sites in the body lower extremities, palmand trunk. Porocarcinoma from scalp is rare and we are presenting a case of porocarcinoma arising from the scalp post radiotherapy for the hypo-pharyngeal tumor. The tumor was presented with multiple secondaries in brain and lung.

English

Malignant eccrine poroma is a malignant tumor arising from skin adnexal structure the eccrine gland. It can be a localized tumor or sometimes it can be associated with multiple cutaneous metastases and visceral metastases.

Successful Reconstruction After Resection of Malignant Eccrine Poroma Using Retroauricular Artery Perforator-Based Island Flap

Malignant eccrine poroma (MEP), or porocarcinoma, is a rare malignant tumor arising from the intraepidermal eccrine duct. It has propensity to arise on the lower limbs (44%), trunk (24%), or head and neck region (24%) but rarely occurs on the scalp. It is very difficult to find proper technique for the first time. In this paper, we introduce rare MEP developing in a longstanding eccrine poroma on the scalp (postauricular area) and successful reconstruction method using perforator-based island flap. A 52-year-old man who had an abrupt growth of a mass on the temporal area presented to us. It was a 3.7 × 2.1-cm mass with fungated margin, but it does not involve the perivertebral muscle and fat tissues in computed tomography scan. The next we executed was general operation for wide excision and scalp reconstruction of a large postauricular defect using retroauricular artery perforator-based island flap. We harvested 7 × 5 cm sized flap and donor site closed primarily without any skin graft. We found that the flap had mild congestion after the suture and mild fullness because of swelling. Retroauricular artery perforator-based island flap can survive over the bone, protect the infection, and bear the radiological treatment. Aesthetically, the patient was very satisfied with the result: similar skin texture, color, thickness, and pliability. There was only a linear scar on the lower margin of the mandible.

Subungal malignant eccrine porocarcinoma: An unusual presentation

We describe the case of a 58-year-old man who presented with a lesion consistent with paronychia on his right middle finger. Biopsies were suspicious for squamous cell carcinoma, and the patient was taken to the operating room for definitive excision. Frozen section revealed microscopic extension into the distal phalanx. The dorsal half of the phalanx was excised, which then had a negative margin. Histologic examination was consistent with a malignant eccrine poroma (porocarcinoma), an uncommon malignancy which is rarely found on the hands. Subungal and periungal porocarcinomas have historically been treated with distal finger amputations, but our patient fortunately has had no local recurrence or lymphatic involvement to date.

Eccrine Porocarcinoma of Scalp: An Uncommon Tumor at an Unusual Site

Eccrine porocarcinoma (EPC) is a very rare malignant sweat gland tumor and those arising in the scalp, are still rarer. Very few cases are reported in literature [1]. Malignant eccrine poroma was first described in 1963 by Pinkus and Mehregan using the term epidermotropic eccrine carcinoma. It is regarded as the rare malignant counterpart of eccrine poroma, a benign tumor of the intraepidermal eccrine sweat gland duct [2]. Porocarcinoma is commonly found in older age group and usually located in the lower extremities (more than 50%), rarely may occur on scalp, face, ear, upper extremities, trunk and abdomen [1, 3]. In the scalp, it is commonly seen in occipital region [4, 5]. The tumor presents as nodular, infiltrative, ulcerated or polypoid growth [6]. Surgical resection is the treatment of choice. There is 17% incidence of local recurrence and an 11% incidence of distant metastasis [3]. We report a case of EPC of scalp arising from a pigmented eccrine poroma in a 35 yr old.

Malignant eccrine poroma (eccrine porocarcinoma): report of a clinical case

Malignant eccrine poroma is a rare malignancy of the eccrine gland usually presenting as a long-standing growth on the skin of the involved areas. It may arise in a pre-existing eccrine poroma. A rare case presenting as a large and painless verrucous crusted swelling over the infra-orbital region of left cheek in a 72-year-old man is described. Gross and histological examination is presented.

Eccrine Porocarcinoma of the Vulva

A case of a 48-year-old woman with a 5 cm left vulvar mass that grew over a six month period is presented. Histopathologic examination of the excised mass was diagnostic of malignant eccrine poroma (eccrine porocarcinoma) with in-situ component. Due to the rarity of this tumor (only five vulvar cases previously reported) and its heterogeneous non-specific clinical and histologic features, the tumor often presents as a diagnostic challenge to clinicians and pathologists. The tumor may be mistaken for a metastasis in cases where the tumor cells are poorly differentiated or for a squamous cell carcinoma in cases with extensive squamous differentiation. There is a need for correct diagnosis of this rare entity - so that appropriate therapy can be instituted in a timely manner. The pertinent literature is reviewed highlighting the diagnostic histomorphologic features and the immunohistochemical profile for making the correct diagnosis of eccrine porocarcinoma.

Expression of cytokeratin subtypes in intraepidermal malignancies: a guide for differentiation

Among intraepidermal malignancies of epithelial origin, Bowen's disease, bowenoid actinic keratosis (BAK), intraepidermal malignant eccrine poroma (MEP), and Paget's disease may pose diagnostic difficulties. Histologic features and immunohistochemical profiles of 24 cases of Bowen's disease, 21 cases of BAK, 18 cases of intraepidermal MEP, and 11 cases of Paget's disease were analyzed. Using multivariate logistic regression test, multinuclear giant cells and solar degeneration were found to be the only histologic parameters of diagnostic help. On the other hand, a widespread positive reaction for CK 5/8, CK 7, CK 19, and negative reaction for CK 10, was a helpful feature in the differentiation of Paget's disease from Bowen's disease and BAK. The widespread and strong expression of CK 10 was seen in almost all cases of Bowen's disease in contrast to BAK. The widespread expression of CK 5/8 and CK 7, and negative reaction for CK 10, was in favor of Paget's disease, compared to intraepidermal MEP. On the other hand, widespread expression of CK 19 was a common finding in intraepidermal MEP, in contrast to Bowen's disease. An immunohistochemical panel may provide significant hints on the differentiation of common intraepidermal malignancies, especially in problematic cases.

Malignant eccrine poroma presenting with pulmonary and liver metastases

Malignant eccrine poroma presenting with pulmonary and liver metastases

Das maligne ekkrine Porom

Malignant eccrine poroma is a very rare tumour of the sweat glands with high malignancy and presenting with a polymorph clinical and histological picture. We describe the case of a 99-year-old patient with a malignant poroma on the buttock. Despite the large size of the tumour, no metastasis was found with standard examination techniques. Radical excision and defect closure with a Limberg flap was performed. The healing course was uneventful and without complications. The malignant poroma is a tumour of high malignancy which can easily be misdiagnosed because of its different forms of presentation. Radical surgical therapy is the only known effective treatment.