Liver, lung and bones are the distant sites of metastasis with fatal results. It is often misdiagnosed as seborrhoeic keratosis, pyogenic granuloma, viral wart verruca or squamous cell carcinoma. It starts as a rapidlygrowing bump on the head or abdomen, and may cause tenderness, redness, or an open wound. Squamoid eccrine ductal carcinoma is rare, with as few as cases reported in the literature. Mucinous eccrine adenocarcinoma may occur in the periocular region.
But the docs did not know from where, breast, ovary or lung took more margins and scaned her from head to toe. Eccrine mucinous carcinoma genetic and rare diseases. Mar 27, 2012 march 27, 2012 orlando, florida a large study of patients with eccrine carcinoma has revealed the importance of histologic subtype and pathologic grade to 5year survival after surgical. Abstractsquamoid eccrine ductal carcinoma sedc is an exceedingly rare tumor that shows both. Eccrine differentiation in basal cell carcinoma core. Skin adnexal neoplasm suny downstate medical center. Squamoid eccrine ductal carcinoma is an eccrine carcinoma subtype, and only twelve cases have been reported until now.
Rare malignant tumor arising from the intraepidermal portion of the eccrine sweat gland duct epithelium. Skin adnexal neoplasms of the vulva are uncommon, and malignant adnexal neoplasms of the vulva are rare. So it was a fluke that my husband discovered he has eccrine cancer and he is not feeling sick in any way at this moment in time. The designation of sedc should likely encompass both the divergent biphasic neoplasms that have been previously reported in the literature, but also include a well differentiation eccrine carcinoma in which there is an intrinsic squamous component within a tumor exhibiting classic features of eccrine ductal carcinoma.
Histologically the following four distinct variants were identified. It can arise denovo or from preexisting eccrine poroma in 18% of cases. Squamoid eccrine ductal carcinoma semantic scholar. A clinicaldermoscopic study of seven cases article pdf available in acta dermato venereologica 892. Syringoid eccrine carcinoma of external auditory canal.
May 30, 2019 risk factors for eccrine carcinoma include family history, immunosuppression, and ultraviolet radiation uvr. Signet ring cell carcinoma of the eccrine sweat gland in the. A rare case of eccrine carcinoma pdf free download. Excision biopsy showed a squamous cell carcinoma arising from an. There is a mortality rate of 67% in patients with lymph node metastases. Malignant adnexal neoplasms modern pathology nature. Mucinous eccrine carcinoma of the eyelid dermatology. Eccrine poroma is an uncommon appendage skin neoplasm originating from the terminal distal portion of the sweat glands annexed to the skin. Carcinomas of the eccrine sweat gland represent a rare group of tumors with potential for. It is frequently found on the lower extremities, and it equally affects both sexes in the sixth and seventh decade. Get a printable copy pdf file of the complete article 589k, or click on a page image below to browse page by page. Pdf squamoid eccrine ductal carcinoma is an eccrine carcinoma subtype, and only twelve cases have been reported until now. Even though it rarely metastasizes, 20% of patients with a porocarcinoma will present with.
Read eccrine syringofibroadenoma surrounding a squamous cell carcinoma. A 76yearold caucasian man presented to our hospital with a left temporal mass. Sweat gland carcinoma is very rare with a reported incidence of less than 0. Apocrine eccrine carcinomas and breast carcinomas are analogous tumors often with similar histology. It was originally described as a basal cell tumor with eccrine differentiation in 1969 1. Sweat gland carcinoma an overview sciencedirect topics. Eccrine carcinoma is the most common subtype of adnexal. Abstractmalignant eccrine spiradenomas are exceedingly rare tumours.
About 20% of epc will recur and about 20% will metastasize to regional lymph nodes. If you have problems viewing pdf files, download the latest version of adobe reader. Pdf eccrine porocarcinoma is a rare carcinoma arising from the intraepidermal duct of eccrine sweat glands and constitute about 0. There is controversy regarding whether mucinous eccrine carcinoma arrises in a sweat gland or apocrine gland. However, studies on genetic alterations and egfr and her2 status of apocrine eccrine carcinomas are few in number.
Cutaneous apocrine gland carcinoma, a subtype of sweat gland carcinoma, is a very rare malignant neoplasm arising in areas of high apocrine. It is interesting that in this study we detected mutations in pik3ca and tp53 in a subset of apocrine eccrine carcinomas, a therapeuticallyrelevant finding since both of these genes are known to be frequently mutated in breast carcinomas. Chemotherapeutic drugs that selectively target cancerpromoting pathways may complement conventional therapeutic approaches. We report a case of this rare entity presenting in the abdomen, which is the. It must be distinguished on clinical grounds from metastatic mucinproducing adenocarcinoma colloid carcinoma particularly of the breast and colon, which may have an identical histological appearance. Eccrine carcinoma syringoid carcinoma eccrine carcinoma syringoid carcinoma, ductal eccrine carcinoma is a rare tumor which usually presents as a slowly growing infiltrating plaque on the scalp, 1458 or as a plaque or nodule on the extremities or trunk. Involvement of the extremities is uncommon, with only a few cases. Eccrine carcinoma is an extremely rare malignancy of the skin with few well documented cases reported in the literature. Pathology of eccrine porocarcinoma dr sampurna roy md.
The lesion may be locally invasive and recur after incomplete excision, although it rarely metastasizes. Full text full text is available as a scanned copy of the original print version. This article is from journal of clinical medicine research, volume 1. Photodynamic therapy as palliative therapy for invasive. Apocrine eccrine carcinomas are rare and associated with poor prognosis. Nov 14, 2011 malignant eccrine spiradenoma is a type of tumor that develops from a sweat gland in the skin. Scalp is the most common site of occurrence and it usually spreads to lymph nodes. Sec differs from basal cell carcinoma in its cytology.
March 27, 2012 orlando, florida a large study of patients with eccrine carcinoma has revealed the importance of histologic subtype and pathologic grade to 5year survival after surgical. It is a rare tumor and its histopathological diagnosis is difficult. It was first described, in 1963, by pinkus and mehregan as epidermotropic eccrine carcinoma and probably represents the commonest form of sweat gland carcinoma. Petct imaging of squamoid eccrine ductal carcinoma. Syringoid eccrine carcinoma understanding eccrine sweat.
Signet ring cell carcinoma, papillary syringadenocarcinoma also includes adenoid cystic carcinoma, apocrine carcinoma, malignant acrospiroma, malignant chondroid syringoma malignant mixed tumor, malignant dermal cylindroma, malignant eccrine poroma most common, malignant myoepithelioma, malignant syringoma syringoid eccrine carcinoma. There is a growing tendency to regard this tumor as a variant of microcystic adnexal carcinoma. Eccrine porocarcinoma usually are found in adults older than 60 and appear as a single red elevated solid mass nodule or a thickened or elevated lesion plaque. Infiltrating syringomatous adenoma of the nipple is a rare, benign, infiltrating neoplasm of eccrine origin located in the dermis of the areola and nipple region first described by rosen in 1983. Given its rather poor response to adjuvant therapy, wide excision of the lesion with tumorfree margins may offer a reasonable chance for longterm control of this neoplasm. Links to pubmed are also available for selected references. Signet ring cell carcinoma of the eccrine sweat gland in.
A case report usha ku burra md, avninder singh md, sunita saxena md dcp dermatology online journal 11 2. Eccrine sweat gland carcinomas arise most often in the skin of the head and neck or the extremities and usually manifest as slowgrowing, painless papules or nodules. A clinicopathologic study was made of 35 patients with primary eccrine adenocarcinoma diagnosed in the past 20 years from among 450,000 consecutive skin biopsy specimens. Hidrocystomas are common, benign adnexal neoplasms most frequently found on the eyelids, canthi, or periocular areas. Mucinous eccrine carcinoma usually remains localized with a tendency to recur but not to metastasize. Herein, we report a case of multiple hidrocystomas distributed over less common facial areas.
The differentiation between apocrine and eccrine metastatic sweat gland carcinoma is often difficult. Sweat gland adenocarcinoma is a rare malignancy with high metastatic potential seen more commonly in later years of life. Syringoid eccrine carcinoma, journal of the european. Editor,the signet ring cell carcinoma of the eccrine sweat gland is a very rare tumour of the eyelid. Difficulties in making a diagnosis with histological and immunohistochemical examinations are reported. Pdf eccrine porocarcinoma associated with an eccrine poroma.
We represent a rare case report of clear cell eccrine carcinoma with comedonecrosis in the neck region which is an. Malignant eccrine spiradenoma is a type of tumor that develops from a sweat gland in the skin. If the inline pdf is not rendering correctly, you can download the pdf file here. Infiltrating syringomatous eccrine adenoma of the nipple. Feb 04, 20 eccrine carcinoma is an extremely rare malignancy of the skin with few well documented cases reported in the literature. They affect the eccrine sweat glands, which are the major sweat glands of the body. Eccrine porocarcinoma is a rare cutaneous malignancy that arises from the intraepidermal ductal portion of the eccrine sweat gland acrosyringium. Eccrine porocarcinoma epc is a rare type of skin cancer arising from the intraepidermal portion of eccrine sweat glands or acrosyringium, representing 0.
Since the initial description of this tumor in 1952 1, 2, there have been several individual case reports described. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Merkel cell carcinoma, eccrine carcinoma, glomus tumors, and dermatofibrosarcoma protuberans. May 05, 2016 the designation of sedc should likely encompass both the divergent biphasic neoplasms that have been previously reported in the literature, but also include a well differentiation eccrine carcinoma in which there is an intrinsic squamous component within a tumor exhibiting classic features of eccrine ductal carcinoma. Pigmented eccrine poroma mimicking a pigmented basal cell. We present the case of a 91yearold woman with a lesion involving the left index finger confirmed to be squamoid eccrine ductal carcinoma by dermatopathologic evaluation. Eccrine porocarcinomas are typically slowgrowing tumours. Mar 01, 2001 a rare case of a syringoid eccrine carcinoma in a 52. Anyway it was the genetic history that gave the doctors the impetus to do more intense pathology tests. Get a printable copy pdf file of the complete article. Nonsquamous malignancy of the vulva should be considered. Porocarcinoma or eccrine porocarcinoma is a rare type of malignant eccrine sweat gland tumor, more frequently encountered in the lower extremities feet and legs. A case combining features of eccrine poroma and pagets dermatosis. It is very locally invasive, destructive, and is often recur rent.
Syringoid eccrine carcinoma sec is a very rare tumor. Recently, two series of 7 and 30 cases, respectively, were. A case of mac was reported in the neck of a man who received radiation therapy to the site years earlier. Eccrine carcinoma is a quite rare malignant tumor that typically arises from a normal sweat gland and that features a rather high recurrence rate subsequent to simple excision. Jun 01, 2007 anyway it was the genetic history that gave the doctors the impetus to do more intense pathology tests. Case report of a rare tumor occasionally detected in the vulva.
Lack of metastasis is the rule, but local recurrences can occur when the. This tumour shares some histological features with breast carcinoma, the metastasis of which represents the most important. Primary mucinous eccrine carcinoma of the skin a rare clinical tumor with many differential diagnoses. Cutaneous eccrine porocarcinomas are uncommon malignant tumors of the sweat gland. Nuclei are relatively uniform with notably prominent nucleoli hematoxylineosin. Direct intracranial extension of malignant eccrine. Syringoid eccrine carcinoma sec is an extremely rare malignant adnexal neoplasm derived from eccrine sweat glands, of. Regarding their product, skin glands are classified into glands secreting sebum sebaceous glands and sweat sweat glands.
Almost half of patients are misdiagnosed as squamous cell carcinoma by the incisional biopsy. A tumor showed up on her back i thought it was a cyst the tumor was shown to be metastatic. We describe a case of eccrine porocarcinoma with metastasis to the parotid gland with special emphasis on the role of surgical resection and adjuvant radiation therapy. A rare case of a syringoid eccrine carcinoma in a 52. Treatment of eccrine porocarcinoma with metastasis to the. Syringoid eccrine carcinoma, journal of the european academy. This benign affection commonly appears as a skin colored papules or nodules localized on the extremities.
Your hardworking eccrine sweat glands may be the site of something more sinister especially if a syringoid eccrine carcinoma is beginning to multiply within them. Sections of eccrine carcinoma show a tumour composed of basaloid cells infiltrating the dermis figure 1. Skin nonmelanocytic tumor papillary eccrine adenoma. May arise from preexisting benign eccrine poroma, with increase in size, tenderness, and sometimes odor 300 cases reported in the literature. During the first hospitalization, syringoma was diagnosed, both clinically and histologically, keeping in mind the possibility of its malignant alteration. Downloaded for anonymous user na at the university of hong kong from. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Neoplasm of eccrine sweat gland with potential for destructive local tissue infiltration and metastasis. Article information, pdf download for syringoid eccrine carcinoma of the. Survival after eccrine carcinoma resection clarified. Pigmented eccrine poroma mimicking a pigmented basal cell carcinoma author. For language access assistance, contact the ncats public information officer. Squamoid eccrine ductal carcinoma the dermatologist.
Three major types of skin glands are recognized according to their product, the excretory function, and the location, where the excretory ducts release their products diseases of these glands are listed in table 61. A clinicopathologic study was made of 35 patients with primary eccrine adenocarcinoma diagnosed in the past 20 years from among 450,000 consecutive skin biops. Eccrine syringofibroadenoma surrounding a squamous cell. Excision biopsy showed a squamous cell carcinoma arising from an eccrine. Radiation oncologyporocarcinoma wikibooks, open books. Skin adnexal neoplasm volodymyr labinskyy, md suny downstate. Eccrine carcinoma is a rare skin condition characterized by a plaque or nodule on the scalp, trunk, or extremities 669 it originates from the eccrine sweat glands of the skin, accounting for less than 0. Pdf clear cell eccrine carcinoma with comedonecrosis in neck. Although rare, staging of ec with 18ffdg petct imaging of the entire body is indicated. Malignant eccrine spiradenoma genetic and rare diseases. It can take many years before a diagnosis is made because eccrine porocarcinoma can mimic other skin conditions.
Eccrine hidrocystomas presenting as multiple papules on. In our case, we present a 46 yearold female with a recurring exophytic tumor on the right lower extremity, without local extension. Mucinous eccrine carcinoma of the eyelid dermatology jama. It arises from the intraepidermal portion of eccrine sweat glands or acrosyringium. Multiple eccrine hidrocytomas of the face request pdf. This article is from korean journal of pathology, volume 46. Eccrine porocarcinoma is a rare type of skin cancer that may occur by chance or may develop from a benign noncancerous skin tumor. Besides surgical resection, little is known about the role of. Eccrine carcinoma clinical presentation and pathology.
But we are very concerned about what to expect in the future. Eccrine and apocrine neoplasms present a bewildering array of morphologies, which often defy precise classification. Eccrine carcinoma an overview sciencedirect topics. Eccrine carcinoma is a rare tumour that typically presents as a slow growing plaques or nodules on the scalp but can arise at other sites. A case report, journal of cutaneous pathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Mar 16, 2011 eccrine porocarcinoma epc is a rare type of skin cancer arising from the intraepidermal portion of eccrine sweat glands or acrosyringium, being a primary tumor or, even more common, a malignant transformation of an eccrine poroma ep, representing 0.
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