Endocrine Mucin-producing Sweat Gland Carcinoma Research Articles

Renal Cell Carcinoma Antigen Expression in Primary Cutaneous Endocrine Mucinous Carcinomas: A Case Series of 14 Patients and Review of the Literature

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) and primary cutaneous mucinous carcinoma (PCMC) are both uncommon low-grade cutaneous adnexal tumors with predilection for the eyelids of elderly women. Their clinical appearance is nonspecific, typically presenting as a slowly growing poorly circumscribed papule, nodule, plaque, or swelling. Histological features of EMPSGC include a lobulated dermal neoplasm with bland cytology and an invasive mucinous component in up to half of the cases. PCMC exhibits tumor nests suspended in abundant pools of mucin with focal strands or nests of tumor cells infiltrating the dermis. Because of their rarity and banal cytological features, both entities pose a risk for misdiagnosis with other benign/malignant cutaneous adnexal neoplasms. Histomorphological features can suggest a diagnosis of EMPSGC or PCMC, but immunohistochemistry is necessary for confirmation. A review of the literature showed variable results of antigens present in EMPSGC, and many of the positive markers only show sparse or focal immunoreactivity of tumor cells. As a result, diffusely positive markers play a crucial role in identification of these tumors, particularly with initial superficial biopsies. We present 9 cases of EMPSGC and 5 cases of PCMC with strong and diffuse immunoreactivity to renal cell carcinoma antigen. This novel finding can be useful in the diagnosis of EMPSGC and PCMC in combination with other known positive markers to differentiate them from other cutaneous neoplasms. In addition, it provides further evidence that EMPSGC could be a precursor lesion to PCMC with both existing on a spectrum.

Primary cutaneous mucinous carcinoma on the cheek arising from a probable precursor lesion of endocrine mucin－producing sweat gland carcinoma

Primary cutaneous mucinous carcinoma on the cheek arising from a probable precursor lesion of endocrine mucin－producing sweat gland carcinoma

Endocrine mucin-producing sweat gland carcinoma: Report of two cases

Endocrine mucin-producing sweat gland carcinoma: Report of two cases

Endocrine mucin-producing sweat gland carcinoma: A study of 11 cases with molecular analysis.

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare, low-grade adnexal neoplasm that most commonly involves the eyelid. Analogous to solid papillary carcinoma of the breast, it probably represents a precursor lesion to mucinous carcinoma. Here, we describe 11 cases of EMPSGC with molecular analysis. We performed a retrospective search of the Johns Hopkins Medical Institute pathology database and identified 11 cases of EMPSGC. Immunohistochemistry was performed for chromogranin, synaptophysin, neuron specific enolase, estrogen receptor (ER), epithelial membrane antigen (EMA), cytokeratin 7 (CK7), and cytokeratin 20 (CK20). Array comparative genomic hybridization (aCGH) and BRAFV600E pyrosequencing were performed on two and three cases, respectively. We observed a strong female predilection (73% females, 8/11 cases) with an average age of 66 years (range, 56-83 years). EMPSGCs were associated with adjacent benign sweat gland cysts (3/11), atypical intraductal proliferation (1/11), and mucinous carcinoma (1/11). Immunohistochemically, all tumors expressed at least one neuroendocrine marker, ER, EMA, and CK7, and were negative for CK20. aCGH demonstrated a 6p11.2 to 6q16.1 deletion (1/2 cases). All cases were negative for BRAFV600E mutation (3/3 cases). This series provides further histopathologic support that EMPSGC represents a multistage progression to mucinous carcinoma. Additional studies are needed to understand its molecular mechanisms.

Endocrine mucin-producing sweat gland carcinoma: Clinicopathologic, immunohistochemical, and molecular analysis of 11 cases with emphasis on MYB immunoexpression.

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare low-grade primary cutaneous sweat gland carcinoma with predilection for the periorbital skin in elderly female patients. We describe 11 cases of EMPSGC using a broad panel of immunohistochemical markers including BerEP4, cytokeratin 7, CAM 5.2, synaptophysin, chromogranin, cytokeratin 20, Ki67, progesterone receptor, and estrogen receptor. Calponin (1A4) and p63 were used to detect surrounding myoepithelial cells. We also examined staining with a relatively new marker, MYB. Previous studies of MYB on EMPSGC remain limited. As mucin-rich basal cell carcinoma (BCC) represents a main differential diagnosis and primary cutaneous mucinous carcinoma (PCMC) could appear synchronous with EMPSGC, these lesions were also stained for MYB. We found strong and homogenous nuclear MYB-expression in 10 EMPSGC cases stained for MYB. MYB staining was not performed in one case. Furthermore, PCMC and mucin-rich BCCs did not express MYB. The strong nuclear MYB-positivity in EMPSGC could be useful as a new surrogate marker, especially in mucin-poor EMPSGC cases. Additionally, the staining of PCMC revealed absent MYB-expression leading to the conclusion that EMPSGC might not represent a precursor lesion for primary cutaneous mucinous carcinoma.

A case of endocrine mucin-producing sweat gland carcinoma

A case of endocrine mucin-producing sweat gland carcinoma

MYB, CD117 and SOX-10 expression in cutaneous adnexal tumors.

Elevated MYB expression has been documented in adenoid cystic carcinoma (ACC), cylindroma, and spiradenoma, but the specificity of this finding is unknown. CD117 and SOX-10 expression also occurs in some cutaneous adnexal tumors. This study assesses MYB, CD117 and SOX-10 expression in cutaneous adnexal tumors. Retrospective analysis of 184 benign adnexal tumors (140 eccrine/apocrine, 40 follicular and 10 sebaceous), and 30 malignant adnexal tumors was performed with MYB, SOX-10 and CD117 immunostaining. In the benign adnexal tumors, 16% (23/140) significantly expressed MYB. MYB expression was limited to cylindromas and to a lesser extent, spiradenomas in the benign cohort. Elevated MYB expression was detected in mucinous carcinoma, endocrine mucin-producing sweat gland carcinoma and 1 and 4 cases of extramammary Paget's disease (EMPD) in the malignant cohort. CD117 and SOX-10 had similar overall positivity rates in benign apocrine and eccrine tumors (45% and 68% respectively), and were generally negative in other benign and malignant adnexal tumors. Expression of MYB appears limited to a small number of cutaneous adnexal tumors, including cylindromas, spiradenomas, ACCs, mucinous carcinoma, endocrine mucin-producing sweat gland carcinoma and some cases of EMPD.

Endocrine Mucin-Producing Sweat Gland Carcinoma, a Histological Challenge

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare adnexal tumor of the skin with low-grade cytological features and neuroendocrine differentiation. It has a predilection for the skin of the eyelid, but has also been reported in the face and rarely extra-facial locations. The tumor is seen more frequently in women and on average affects the elderly. It is histologically and immunohistochemically analogous to solid papillary carcinoma of the breast/endocrine ductal carcinoma in situ with a nodular, solid, papillary, and/or cribriforming architecture, neuroendocrine differentiation, and mucin production. Since it was first described by Flieder et al. in 1997, less than 60 cases have been reported in literature. We describe the morphological and immunohistochemical features of another case with a review of the common histological differential diagnoses and emphasize the salient features that help distinguish this rare neoplasm.

Epidemiology and Prognosis of Primary Periocular Sweat Gland Carcinomas.

To characterize the clinical presentation, management, and outcomes of patients with sweat gland carcinoma of the periocular region. To review the pathologic classification scheme, compare the clinical behavior between subtypes, and discuss treatment recommendations for periocular sweat gland carcinomas. Retrospective study from 2 tertiary centers, which are part of the American Society of Ophthalmic Plastic and Reconstructive Surgery Oncology Database. Twenty-two patients, of whom 13 (59%) were female, were included in the study. The median age was 69 years. The median follow-up time was 23 months. Twenty (91%) patients had lesions that were slow growing and were present for several months to years before diagnosis. Presentation was varied, and pathology included mucinous carcinoma (7; 32%), microcystic adnexal carcinoma (4; 18%), endocrine mucin-producing sweat gland carcinoma (4; 18%), eccrine carcinoma (2; 9%), apocrine carcinoma (2; 9%), poorly differential adnexal carcinoma (2; 9%), and hidroadenocarcinoma (1; 5%). Microcystic adnexal carcinoma tended to present with the largest tumor dimension (range: 25-32 mm) and mucinous carcinoma the smallest (range: 5-13 mm). Treatments included Mohs micrographic excision in 10 patients (45.5%), wide local excision in 10 patients (45.5%), and orbital exenteration in 2 patients (9%). No lesions ≤T2b (by AJCC 7th edition classification for eyelid carcinomas) had local recurrence or nodal metastasis. No patients had distant metastasis. Sweat gland carcinomas tend to grow slowly and be present for several months to years before diagnosis. Overall, microcystic adnexal carcinoma subtype showed more aggressive clinical behavior than mucinous subtypes. Eyelid carcinomas AJCC category T2b or less were associated with better outcomes.

Endocrine mucin-producing sweat gland carcinoma of the eyelid.

An elderly woman was incidentally noted to have a nodular mass on the upper eyelid, whilst under investigation for cataracts. Punch biopsy of this presumed basal cell carcinoma revealed it to be endocrine mucin-producing sweat gland carcinoma (EMPSGC). The tumour extended to the deep dermal layer and comprised solid nests with foci of cystic and papillary change, and additional cytoplasmic and focal extracellular mucin deposits. Immunohistochemistry confirmed epithelial lineage and neuroendocrine differentiation, and adjacent tissue invasion. The tumour was excised completely with Mohs micrographic surgery with no recurrence after 8 months. EMPSGC is a low-grade sweat gland carcinoma with variable neuroendocrine differentiation, a solid, papillary, or cystic growth pattern, and a predilection for the eyelid of elderly women [Am J Surg Pathol 29:1330-1339, 2005]. There have been 54 previously documented cases of EMPSCG. We report an additional case and review the literature.

Malignant sweat gland tumours: an update

Cutaneous adnexal tumours can be a diagnostic challenge for the pathologist. This is particularly true in the case of tumours with sweat gland differentiation, due to a large number of rare entities, a multiplicity of names to designate the same neoplasms and consequent lack of consensus regarding their classification and nomenclature. In the traditional view, sweat gland tumours were divided into eccrine and apocrine. However, this has been challenged in recent years, and in fact many of these tumours may have both eccrine and apocrine variants. Some display more complex features and defy classification, due to the presence of other lines of differentiation, namely follicular and/or sebaceous (in the case of apocrine tumours, due to the close embryological relationship between apocrine glands, hair follicles and sebaceous glands). The present paper reviews and updates the basic concepts regarding the following malignant sweat gland tumours: apocrine carcinoma, porocarcinoma, hidradenocarcinoma, spiradenocarcinoma, cylindrocarcinoma, microcystic adnexal carcinoma and related entities, squamoid eccrine ductal carcinoma, digital papillary adenocarcinoma, primary cutaneous mucinous carcinoma, endocrine mucin-producing sweat gland carcinoma and primary cutaneous signet ring cell carcinoma. Particular emphasis is put in recent findings that may have implications in the diagnosis and management of these tumours.

WT1 expression in endocrine mucin-producing sweat gland carcinoma: a study of 13 cases.

Endocrine mucin-producing sweat gland carcinoma (EMPSGC), a low-grade sweat gland carcinoma with a predilection for the eyelids, often shows areas of benign eccrine cysts, atypical intracystic proliferation and associated mucinous carcinoma, suggesting tumor progression. Wilms tumor 1 (WT1) protein, a transcription factor, is overexpressed in many tumors and plays a role in oncogenesis. A computer-based search for tumors diagnosed between 1989 and 2009 was conducted. Clinical data were obtained from pathology reports and patient records. Biopsies were reviewed for histologic features. Immunostaining was performed for WT1, chromogranin, synaptophysin, estrogen receptor (ER), epithelial membrane antigen (EMA), polyclonal carcinoembryonic antigen (P-CEA), cytokeratin 7 (CK7), cytokeratin 20 (CK20) and MIB-1. Eight women and five men (mean age: 61.2years; range: 40-77years) presented with slow-growing eyelid nodules. Cases of EMPSGC were characterized by the presence of dermal nodules with various growth patterns. Adjacent eccrine cysts were present in five patients, atypical epithelial proliferation within the cyst wall in four patients, and an associated mucinous carcinoma in one patient. All tumors were positive for WT1, CK7, ER, P-CEA and EMA and negative for CK20. Tumors were positive for synaptophysin in 12 cases and chromogranin in nine cases. The MIB-1 proliferation index was low in most cases. No WT1 staining was observed in the overlying epidermis, adnexal structures or areas of benign eccrine cyst. WT1 expression was observed in areas of atypical epithelial proliferation, and the neoplastic cells. The present study shows WT1 expression in the neoplastic epithelial cells of EMPSGC, areas of atypical intraductal proliferations, and mucinous carcinoma. The absence of WT1 expression in areas of benign eccrine cyst and cutaneous sweat glands suggests WT1 upregulation plays a role in tumor cell proliferation and progression of EMPSGC.

Primary Cutaneous Mucinous Carcinoma

Evidence-based treatment guidelines are not available for primary cutaneous mucinous carcinoma (PCMC). To assess outcomes associated with surgical treatment of PCMC. MEDLINE, Cumulative Index to Nursing and Allied Health, and Embase from 1952 to 2010. Search terms were "primary cutaneous mucinous carcinoma," "primary mucinous adenocarcinoma of the skin," "primary mucinous sweat-gland carcinoma," and "endocrine mucin-producing sweat gland carcinoma." Articles describing primary data on treatment (ie, case reports, case series, and cohort studies) of any patients with PCMC. A total of 116 articles were identified, with 90 of these assessed for eligibility and 87 used for final analysis. Each case was verified to be of primary cutaneous origin by 2 observers. Histopathologic descriptions were confirmed to be consistent with PCMC. Extracted fields included age, sex, race, lesion duration, tumor diameter, method of treatment, follow-up, and whether the lesion recurred or metastasized. Outcomes were dichotomized into good and bad depending on the presence of recurrence or metastasis during follow-up. Multivariate logistic regression analysis was performed to determine significant factors for predicting bad outcomes. RESULTS One hundred fifty-nine cases of PCMC, of whom 54.7% were male and 77.2% were white, with mean (SD) age 63.5 (13.2) years, were analyzed. Most had been treated with traditional surgical excision (85.5%), with only 9.4% of cases treated with Mohs surgery. Older (odds ratio [OR], 0.93; P = .04) and Asian (OR, 0.02; P = .01) patients had relatively better postsurgical outcomes. Larger tumors (OR, 6.71; P = .14), those persistent for longer prior to surgery (OR, 1.02; P = .11), and those located on the trunk (OR, 103.24; P = .005) also were associated with poorer outcomes. Limitations included reliance on case report data. Patient demographic characteristics and tumor-specific features may provide predictive information regarding the risk of postsurgical recurrence and metastasis after treatment of PCMC.

Endocrine mucin‐producing sweat gland carcinoma occurring on extra‐facial site: a case report

Cutaneous endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a very rare low-grade malignant neoplasm analogous to the mammary solid-papillary carcinoma. It frequently expresses neuroendocrine markers and may show mucinous differentiation. Although the nodules are circumscribed, myoepithelial cells cannot be showed in most cases and about half of the cases are associated with invasive mucinous carcinoma. Hence, it has been suggested to be invasive and the precursor lesion of some primary cutaneous mucinous carcinomas. After being recognized as a distinct entity, all cases reported to date occurred either in the periocular region or on the cheek. Two thirds of the patients were female. Herein we present an unusual case of in situ EMPSGC on the chest wall skin of a middle-aged man.

Endocrine Mucin-Producing Sweat Gland Carcinoma

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare low-grade sweat gland carcinoma with a strong predilection to the eyelid region. It is histologically analogous to endocrine ductal carcinoma/solid papillary carcinoma of the breast and is characterized by a multinodular solid cystic mucinous tumor with immunoreactivity to neuroendocrine markers. Only 20 cases of this unusual tumor have been reported. We present the clinical and histopathologic findings of 2 new cases of EMPSGC and review the relevant literature. The histological differential diagnosis is discussed and attention drawn to the role of immunohistochemistry in clarifying the nosological position of EMPSGC within the spectrum of cutaneous mucinous neoplasms.

A case of endocrine mucin‐producing sweat gland carcinoma

Endocrine mucin-producing sweat gland carcinoma (EMPSGC), which is an uncommon sweat gland tumor with a predilection for the eyelids, is morphologically analogous to solid papillary carcinoma of the breast. We report the case of a 55-year-old man with a subcutaneous tumor of the upper cheek. The pathological findings for this patient were compatible with those of reported cases of EMPSGC, and p63 staining revealed partial microinvasion into the dermis. On the basis of these findings, the patient was diagnosed with EMPSGC. It is reported that EMPSGC is a precursor of invasive mucinous carcinoma of the skin. Therefore, this patient was treated and followed up as if he had mucinous carcinoma of the skin. To the best of our knowledge, this is the first report of such a case from Japan.

Recurrent endocrine mucin‐producing sweat gland carcinoma in the eyelid

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) has recently been recognised as a low-grade carcinoma that almost always occurs on the eyelid. This carcinoma is very rare, with only 20 cases (including the present one) having been reported in the literature. EMPSGC is frequently found in association with invasive mucinous carcinoma. While EMPSGC treatments consist of a complete surgical removal, there has been no consensus regarding the surgical margin. Therefore, reports on surgical management of EMPSGC may potentially provide important therapeutic information. Here, we present a case of a 74-year-old man with EMPSGC of the eyelid that repeatedly recurred despite surgical treatments at another institution. After referral to our department, the patient underwent tumour excision. However, the specimen revealed a positive surgical margin and thus, he subsequently underwent a wider excision. There has been no sign of tumour recurrence or metastasis 6 months after his last operation. This article reviews the current literature and discusses the surgical management of EMPSGC.

5. Endocrine mucin producing sweat gland carcinoma

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is an uncommon low-grade carcinoma with a predilection for the eyelid. Only 18 other cases have been reported in the literature to date. Here, we describe three cases of EMPSGC. They occurred in the right medial canthus of a 69-year-old woman, in the left lateral canthus of a 36-year-old man and in the left lower eyelid of a 53-year-old woman. Histologically, they were well circumscribed, typically multinodular tumours with solid or partially cystic nodules, frequently showing areas of papillary architecture with fibrovascular areas. The tumour cells showed positive staining for NSE, chromogranin, synaptophysin, CK7, CEA, ER and PR. The intracytoplasmic and extracellular mucin was positive with DiPAS. Myoepithelial cells did not stain with calponin, smooth muscle actin, S100 and p63, supporting the notion that EMPSGC is an invasive carcinoma. In the largest case series to date it was observed that 50% of EMPSGC had coexisting invasive mucinous adenocarcinoma on histology, suggesting that EMPSGC is a precursor to invasive mucinous adenocarcinoma. The prognosis of EMPSGC, even in cases associated with invasive mucinous carcinoma, is favourable, but longer follow-up is needed. Patients with EMPSGC should be closely followed clinically as mucinous carcinoma has well-documented metastatic potential.

A Case of Endocrine Mucin-Producing Sweat Gland Carcinoma Co-existing with Mucinous Carcinoma - A Case Report -

A Case of Endocrine Mucin-Producing Sweat Gland Carcinoma Co-existing with Mucinous Carcinoma - A Case Report -

Endocrine Mucin-Producing Sweat Gland Carcinoma of the Eyelid

A 70-year-old woman was examined for a 4-mm bluish nodule in the left upper eyelid. The lesion was excised and pathology showed it to be endocrine mucin-producing sweat gland carcinoma with positive margins. She underwent Mohs surgery and reconstruction of the resulting defect. Clinicians should be aware of this entity given its association with invasive mucinous adenocarcinoma, a locally aggressive tumor with metastatic potential.