Case Of Eccrine Porocarcinoma Research Articles

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

Case Report and Review of Eccrine Porocarcinoma in Skin of Color

Skin cancers are often misdiagnosed or diagnosed late in skin of color (Fitzpatrick types 4-6) resulting in increased morbidity and mortality. Rare skin cancers such as Eccrine Porocarcinoma (EPC), which accounts for 0.005% to 0.01% of all epidermal skin neoplasms, are even less likely to be accurately diagnosed in skin of color. Eccrine Porocarcinoma is often misdiagnosed as Squamous cell carcinoma (SCC), Basal Cell Carcinoma (BCC), melanoma, and seborrheic keratosis. The paucity of case reports of EPC in skin of color adds to the challenge of achieving accurate, timely diagnosis and treatment in this patient population. The purpose of this research is to review case reports of EPC documented in skin of color, highlighting salient clinical and histopathological characteristics of EPC, and to describe an additional case of EPC in skin of color that was initially misdiagnosed as seborrheic keratosis. Our search combined “eccrine porocarcinoma” and one of the following terms: “ethnic skin”, “skin of color”, “black”, “dark skin”, “African American”, “Indian”, “Native American”, “Asian”, “Hispanic”, “Indigenous Peoples”, “Middle Eastern” —as these tend to correspond with Fitzpatrick IV – VI types. Google scholar, PubMed, and Ovid MedLine Databases were used to search for articles. Case reports ranging from 1994 to present day were included in analysis.

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.

Engrailed Homeobox 1 and Cytokeratin 19 Are Independent Diagnostic Markers of Eccrine Porocarcinoma and Distinguish It From Squamous Cell Carcinoma.

The diagnostic utility of En1 in the histopathologic differentiation of eccrine porocarcinoma (EPC) from invasive squamous cell carcinoma (SCC) was investigated. Expression of En1 and CK19 in 16 cases of EPC was immunohistochemically examined and compared with that in 32 cases of SCC. In all 16 EPCs, En1 was expressed in 3% to 100% of tumor cells. In 20 of the 32 SCCs, En1 was expressed in 3% to 90% of tumor cells. A total of 13 of the 16 EPCs and five of the 32 SCCs were judged as En1 positive, with a cutoff value of 25%. In addition, 11 of the 16 EPCs and four of the 32 SCCs were CK19 positive. The frequencies of En1- and CK19-positive cases were significantly higher in EPCs than in SCCs. In a logistic regression analysis for predicting EPC, En1 and CK19 were independent markers. When expression patterns of En1 and CK19 were combined, none of the 32 SCCs was both positive. In contrast, 15 of the 16 EPCs were positive for either En1 or CK19. A combination of En1 and CK19 expression can improve the accuracy of histologic diagnosis of EPC.

A case of eccrine porocarcinoma characterized by a progressive increase in the level of Ki-67 index: case report and review of literature

BackgroundEccrine 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 presentationWe 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.ConclusionIntracranial 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.

A Case of Eccrine Porocarcinoma, Located in the Breast: The Pitfalls Reviewed

Evidence on eccrine porocarcinoma (EPC) was revised in response to the case of a 74-year old patient, presenting with an EPC located in the breast and initially treated with local excision only. She was referred to our center after loco-regional recurrence, but re-excision with lymphadenectomy and adjuvant radiotherapy to a dose of 66 Gy failed to obtain loco-regional control. EPC is a rare disease, occurring in 0.005-0.01% of all skin cancers. The probability of misdiagnosis is high, especially in case of an unusual primary site of tumour location. Clinical and histological diagnosis encompasses a large list of differential diagnoses. Treatment primarily consists of surgery, with lymphadenectomy in case of unfavorable characteristics. No evidence exists for adjuvant systemic or loco-regional treatment.

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

Treatment of eccrine porocarcinoma with Mohs micrographic surgery: a cases series and literature review.

Eccrine porocarcinoma (EPC) is a rare malignant tumor of the eccrine sweat gland. It is a potentially fatal neoplasm that is locally aggressive and commonly recurs. Wide surgical excision has traditionally been the treatment of choice and is curative in approximately 70-80% of cases. The disease is metastatic to lymph nodes and distant sites in 20% and 10% of cases, respectively. Metastatic EPC has not shown any great response to adjuvant chemotherapy or radiation. The purpose of this study was to evaluate the efficacy of Mohs micrographic surgery (MMS) for EPC as an alternative to wide local excision. Five patients diagnosed with EPS between 2011 and 2014 at the University of Louisville and treated with MMS were studied. Recurrence-free periods subsequent to the treatment of EPC with MMS were measured. The five patients with EPC treated by MMS remained recurrence-free for a mean of 11 months (range: 2-26 months). Mohs micrographic surgery is a highly effective treatment for EPC. Given the high rate of recurrence, propensity for lymph node metastases, and the often ineffective options for treating advanced disease, MMS should be considered in the treatment of all cases of EPC.

Eccrine Porocarcinoma Treated by Mohs Micrographic Surgery: Over 6-Year Follow-up of 12 Cases and Literature Review.

Eccrine porocarcinoma (EPC) has a poor prognosis after standard wide local excision (WLE), with 20% local recurrence, 20% regional and 12% distant metastatic rates. Mohs micrographic surgery (MMS) has been used as a promising treatment. To review the use of MMS for EPC and assess treatment outcomes. This was a retrospective case series of 12 EPC patients treated by MMS between 1984 and 2013 in the institution. Furthermore, a literature review revealed an additional 17 cases of EPC managed by MMS. Of 29 cases of EPC treated by MMS, outcome was established in 27 cases. The patients had a significantly longer mean follow-up period of 6 years (range, 4-206 months), as compared with 19 months (range, 2-48 months) in reported cases. Two patients had regional lymph node metastasis after MMS. The regional metastatic rates to lymph nodes were 7% (2/27). There was no local recurrence, distant metastasis, or disease-specific death in the 27 cases studied. To the best of the authors' knowledge, this is the single largest case series of EPCs treated by MMS and the authors' data demonstrated that MMS may be superior to the standard WLE.

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.

多発性リンパ節転移と脈管浸潤を伴った悪性エクリン汗孔腫の1例

多発性リンパ節転移と脈管浸潤を伴った悪性エクリン汗孔腫の1例

治療に難渋したエクリン汗孔癌の1例

治療に難渋したエクリン汗孔癌の1例

CYFRA上昇を示した進行期エクリン汗孔癌の2例

症例1：54歳，男性。初診の1年前より右臀部に紅色腫瘤が出現した。初診時，右鼠径リンパ節の著明な腫大もみられ，PET－CTでは肺転移，多発性のリンパ節転移を認めた。エクリン汗孔癌と診断し，原発巣を切除，右鼠径リンパ節郭清を姑息的に施行した。化学療法，放射線療法を行ったが，肺転移による呼吸不全により死亡した。症例2：47歳，男性。初診の数年前より左下腿に紅色腫瘤が出現し，エクリン汗孔癌と診断した。原発巣を切除し，左鼠径，骨盤リンパ節郭清を施行，術後化学療法，放射線療法を行ったが，肺転移による呼吸不全により死亡した。2例ともに病勢の進行とともにCYFRAの著明な上昇がみられた。免疫組織染色において腫瘍細胞に一致して，CK19が陽性であったことからCYFRAがエクリン汗孔癌の病勢を反映していた可能性が示唆された。

胸腔内播種を認めた皮膚エクリン汗孔癌の 1 例

背景 : エクリン汗孔癌はまれな皮膚悪性腫瘍であり, われわれが調べえたかぎりでは胸腔内播種を伴った症例の報告はない. 今回われわれは, 胸水中に腫瘍細胞の出現を認めたエクリン汗孔癌を経験したので, その細胞像について報告する.症例 : 80 歳, 女性. 6 ヵ月前に下腹部皮膚のエクリン汗孔癌を切除された. 3 ヵ月前より発熱と呼吸苦および胸水貯留が出現したため胸水細胞診が施行された. 胸水中の異型細胞は炎症細胞を背景として形態学的に明らかに中皮細胞や肺腺癌細胞と異なる大型の腫瘍細胞の集塊を認め, 確定診断のためにセルブロックを作製して免疫染色を施行した. 臨床経過および皮膚原発腫瘍との免疫染色結果を比較検討し, エクリン汗孔癌の胸腔播種と診断した. 胸水中の癌細胞の一部は核が偏在性で, Cytokeratin 7 (CK7) および CK19 が陽性の細胞質内封入体様構造物を認めた.結論 : きわめてまれな皮膚エクリン汗孔癌の胸腔内播種を経験した. 胸水中の癌細胞には特徴的な封入体様構造物を認めた. 確定診断にはセルブロックによる免疫染色が有用であった.

Break a Sweat in the Diagnosis and Management of a Flesh‐Colored Nodule

Eccrine porocarcinoma (EPC), which is a tumor of the sweat ducts, most commonly affects older adults, with an average age of 68, but may occur at any age.1 It may arise originally in malignant form2 but most often develops from a long-standing benign eccrine poroma as a form of degenerative progression.3 It is not uncommon for the lesion to be misdiagnosed. Because the tumor has the capacity to metastasize, with consequent poor prognosis, it is imperative for accurate diagnosis and management. A 63-year-old man presented with a 4-month history of a flesh-colored nodule over the left upper cutaneous lip that had been enlarging gradually and was asymptomatic. There had been no preexisting lesion at the site before onset. His past medical history included hypertension for which he was taking antihypertensives. Clinical examination revealed a flesh-colored 15- × 10-mm nodule over the left upper cutaneous lip extending to the vermillion. There was telangiectasia over the surface. There was no pearly margin (Figure 1). A punch biopsy was performed that revealed a dermal-based tumor with nests of tumor cells with ductal structures showing numerous mitotic figures at low magnification. Polyclonal carcinoembryonic antigen (CEA) highlighted ducts, confirming their eccrine differentiation. A diagnosis of EPC was made, and the lesion was excised in one stage with two blocks using Mohs micrographic surgery (MMS) and the defect closed with mucosal advancement of the lip. EPC has a propensity to arise on the lower limbs (44%), trunk (24%), or head and neck (24%).4-6 One problem with EPC is delay in definitive treatment because of the tumor's indolent behavior or misleading clinical diagnosis.7 The tumor is typically formed of cohesive basaloid epithelial cells and may be misdiagnosed as basal cell carcinoma. Foci of squamous differentiation that can resemble well-differentiated squamous cell carcinoma are often observed within EPC. The presence of ductal structures and use of immunostains help in making the histological diagnosis. Regional lymph node metastases are found in approximately 20% of patients, and distant metastases arise in approximately 10% of patients. There is an approximately 67% mortality in individuals with lymph node metastases.8 Its metastatic potential and poor outlook in the event of metastasis dictates the need for early identification and complete excision as the best chance of a cure. EPC has been treated using a variety of methods, including electrosurgery, simple and wide excision, MMS, and radiation. Curative rates of 70% to 80% have been reported for excision, with recurrence rates as high as 20% being reported.1, 5 Recurrences had been reported to occur 4 months to 12 years after treatment.9 One study7 reported that three of eight individuals had recurrences after wide excision (≥10-mm margins). Thus simple or wide excision is not the optimal treatment for EPC. MMS appears most promising. Another study10 reported a series of five cases of primary EPC treated using MMS without recurrence after 2 to 4 years of follow-up. The current patient was treated using MMS. Tumor extirpation was achieved in one stage with two blocks. One year after treatment, no recurrence was noted. primary and metastatic EPC have shown poor response to radiation therapy. Chemotherapy also has a poor response and is generally reserved for treatment of documented metastatic EPC. EPC is challenging for surgeons and medical and radiation oncologists. Because of its rarity and lack of consensus and guidelines for its management, EPC is a challenge to treat. MMS, which allows for full evaluation of the tumor margins, may be the ideal treatment for this rare tumor. Because tumor recurrence had been reported 12 years after treatment, monitoring is essential after treatment. Conflict of Interest: The editor in chief has reviewed the conflict of interest checklist provided by the authors and has determined that the authors have no financial or any other kind of personal conflicts with this paper. Author Contributions: Both authors were involved in the analysis and interpretation of data and preparation of manuscript. Sponsor's Role: None.

A Case of Eccrine Porocarcinoma of the Auricle

A Case of Eccrine Porocarcinoma of the Auricle

Factors in the surgical management of primary eccrine porocarcinoma: prognostic histological factors can guide the surgical procedure

Primary eccrine porocarcinoma is a rare cutaneous neoplasm and there is no consensus concerning its surgical management. To conduct a retrospective analysis of all the cases of eccrine porocarcinoma seen in our hospital in order to identify prognostic factors. Clinical data were retrieved from the patients' files. Each histological sample was reviewed by the pathologist and classified into three eccrine porocarcinoma subtypes: 'infiltrative', 'pushing' and 'pagetoid' eccrine porocarcinoma, according to Robson's criteria. Statistical methods were used to estimate the recurrence risk. Twenty-four patients were included. The surgical margins realized were ≥ 2 cm or < 2 cm in six and 13 patients, respectively, and slow Mohs procedure was performed in two patients. Ten, seven and two patients presented with 'infiltrative', 'pushing' or 'pagetoid' eccrine porocarcinoma, respectively. Eight patients (of 23) experienced recurrences (35%). The recurrence risk was linked to the histological subtype: two out of two pagetoid eccrine porocarcinoma, four out of 10 'infiltrative' and none of seven 'pushing' eccrine porocarcinoma recurred (P=0·026). The recurrence risk was not associated with epidermal growth factor receptor status, nor the margin realized. The recurrence risk of 'pushing' eccrine porocarcinoma is significantly lower than for 'infiltrative' eccrine porocarcinoma. Based on these considerations, a decisional algorithm might be proposed: first step, excisional biopsy of the primary eccrine porocarcinoma and identification of the histological subtype; second step, infiltrative or pagetoid porocarcinoma should be excised with an additional modified micrographic Mohs procedure, while no additional surgery is needed for 'pushing' eccrine porocarcinoma if the first excisional biopsy is complete. Although demonstrated in a limited case series, the histological subtype might guide the surgical management of primary eccrine porocarcinoma.

Eccrine Porocarcinoma of the External Ear

Eccrine porocarcinoma (EP) is believed to arise from the intraepithelial portion of the eccrine sweat glands. The incidence rate of EP is reported to be 18 per 450,000 biopsies. Most of the diagnosed cases involve the lower extremities; a case of EP involving the ear is rare. We describe the clinical, radiologic, and histopathologic features of EP in an 11-year-old boy.

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.

Porocarcinoma écrino: relato de quatro casos e revisão da literatura

Eccrine porocarcinoma is an extremely rare skin tumor derived from the acrosyringium. Four cases of eccrine porocarcinoma are described, two males and two females, aged from 49 to 64 years. Tumors grew in an interval of one to 20 years. Lesions ranged from 1.2 x 2 cm to 4 x 5 cm and were on limbs or face. Two patients had basal cell carcinoma in a site different from the eccrine porocarcinoma. Two biopsies were described as eccrine poroma. Malignancy was observed in all cases after wide excision. Therefore, eccrine porocarcinoma should always be considered after a biopsy compatible with eccrine poroma.