Eccrine Differentiation Research Articles

Chondroid Syringoma with Small Tubular Lumina

Background: Chondroid syringoma or mixed tumor of the skin is a rare epithelial tumor with eccrine differentiation. The variant with small tubular lumina is extremely uncommon. Objective: Chondroid syringoma is usually present as a single subcutaneous nodule. This report describes a man with multiple scalp lesions exhibiting features of chondroid syringoma with small tubular lumina. Methods: A Pathological examination of surgically resected multiple scalp nodules was carried out by routine histology and immunohistochemistry. The following antibodies were found: cytokeratin, epithelial membrane antigen, and vimentin. Results: Microscopic findings revealed multiple chondroid syringoma with small tubular lumina. The tumor cells were positive for high- and low-molecular-weight keratin, epithelial membrane antigen, and vimentin. Conclusion: This is the first report of multiple chondroid syringoma with small tubular lumina.

Widespread metastatic eccrine porocarcinoma

Eccrine porocarcinoma is a rare malignant sweat gland tumor arising from the acrosyringium. These lesions can show multiple epidermotropic metastases, however, systemic spread is rare. We describe the case of a 42-year-old African American man who presented in February 1998 with a lesion on the plantar surface of his right foot that was diagnosed as an eccrine porocarcinoma. Subsequently, during the next year, cutaneous metastases developed to his left shoulder and flank. Two years after his initial presentation, he was admitted with an esophageal mass and multiple gastric ulcers. Biopsy specimens of these lesions demonstrated metastatic eccrine porocarcinoma. Further evaluation revealed multiple metastases to lymph nodes, lungs, and brain. Histologic examination revealed nests of basaloid cells with eosinophilic cytoplasm and intracellular ductlike lumina lined by eosinophilic basement membranelike material. These ductular structures where highlighted by immunohistochemical stains for carcinoembryonic antigen, confirming their eccrine differentiation. There were mitotic figures and individual cell necrosis. Although rare, systemic metastases from an eccrine porocarcinoma must enter the differential diagnoses of a tumor mass in such patients.

Benign ocular adnexal tumours of a ocrine eccrine or hair follicle origin

Background: The differential diagnosis of malignant eyelid tumours, particularly basal cell carcinoma (BCC), includes tumours of skin appendages. The incidence of these adnexal tumours has not been well established. This study aimed to determine the incidence and review the main clinicopathological features of benign tumours arising from skin appendages of the eyelid with apocrine, eccrine or hair follicle differentiation.Methods: The histopathological diagnoses of 7751 ophthalmic specimens retrieved from 6967 patients between September 1993 and March 2002 at the Henry C. Witelson Ophthalmic Pathology Laboratory and Registry, McGill University, Montreal, were retrospectively reviewed. Clinical data and histopathological diagnoses were obtained for 228 benign adnexal tumours of apocrine, eccrine or hair follicle origin. New histopathological slides were made from the paraffin-embedded specimens and stained with hematoxylin-eosin and periodic acid-Schiff's reagent.Results: Of the 228 benign adnexal tumours, 182 were diagnosed as apocrine or eccrine hydrocystoma (79.80, 12 pilomatrixoma (5.3%), 12 syringoma (5.3%), 11 trichilemmoma (4.8%), 5 syringocystadenoma papilliferum (2.2%), 3 trichoepithelioma (1.3%) and 3 trichofolliculoma (1.3%). Discrepancies between clinical and histopathological diagnoses were noted in 22 cases (9.6%).Interpretation: Benign tumours originating from skin appendages of the eyelid are rare and frequently have apocrine or eccrine differentiation. These tumours, particularly those originating from the hair follicle, should be considered in the differential diagnosis of BCC of the eyelid.

Microcystic adnexal carcinoma (MAC) of the scalp with extensive pilar differentiation.

Microcystic adnexal carcinoma (MAC), or sclerosing sweat duct tumor, is an uncommon, locally aggressive tumor. It typically involves the upper lip or face of middle-aged adults and rarely involves the scalp. It is characterized histologically by both pilar and eccrine differentiation and is associated with frequent perineural invasion. MAC is frequently misdiagnosed because of its bland and asymptomatic clinical presentation. In addition, its defining histologic features may be missed with a superficial biopsy. To describe a case of MAC in the scalp treated with Mohs surgery. We describe a case with extensive pilar differentiation and clear cell changes. In addition, the clinical and histologic characteristics of this rare neoplasm are reviewed. Mohs micrographic surgery (MMS) was performed on this neoplasm. The tumor was successfully excised in one surgery (five stages) and is without evidence of recurrence at 18 months. We present a case of MAC of the scalp in a 64-year-old white man treated successfully with MMS.

Merkel Cell Carcinoma (Neuroendocrine Carcinoma of the Skin)

We review the literature on Merkel cell carcinoma (MCC; primary neuroendocrine carcinoma of the skin) and add 36 cases to the over 800 already described in the literature. Though generally considered a tumor of the elderly, MCC can also occur in young patients. Microscopically, there are dermal proliferations of small cells possessing nuclei with finely dispersed chromatin. The occasional presence of squamous or eccrine differentiation suggests a pluripotential stem cell origin. Immunohistochemistry is of great help in distinguishing these tumors from potential mimics such as malignant melanoma, lymphoma, or metastatic small cell (neuroendocrine) carcinomas. Recent chromosomal studies have enhanced our understanding of the biology of these tumors. Despite the high metastatic rate associated with Merkel cell carcinoma, spontaneous regression has been reported, and there have been some therapeutic successes. The high survival rate for stage I lesions indicates the importance of early recognition and treatment of these tumors.

Porokeratotic eccrine ostial and dermal duct nevus: A case report and review of the literature

Hamartomas with eccrine differentiation are quite rare. There are 5 accepted classifications: eccrine nevus, eccrine-centered nevus, eccrine angiomatous hamartoma, eccrine syringofibroadenoma, and porokeratotic eccrine ostial and dermal duct nevus. The latter, PEODDN, typically presents as congenital keratotic papules and plaques located on the distal extremities. We report a classic case of PEODDN that was localized to the left hand since early childhood. The literature on this rare benign tumor is also reviewed. (J Am Acad Dermatol 2000;43:364-7.)

Porokeratotic eccrine ostial and dermal duct nevus: A case report and review of the literature

Hamartomas with eccrine differentiation are quite rare. There are 5 accepted classifications: eccrine nevus, eccrine-centered nevus, eccrine angiomatous hamartoma, eccrine syringofibroadenoma, and porokeratotic eccrine ostial and dermal duct nevus. The latter, PEODDN, typically presents as congenital keratotic papules and plaques located on the distal extremities. We report a classic case of PEODDN that was localized to the left hand since early childhood. The literature on this rare benign tumor is also reviewed. (J Am Acad Dermatol 2000;43:364-7.)

Sweat Gland Adenoma with Predominant Myoepithelial Differentiated Features: Case Report and Immunohistochemical Study

A 56-year-old man presented with a subcutaneous mass adjacent to the sternoclavicular joint region, which had predominant spindle-shaped myoepithelial cells and a small number of tubular or trabecular epithelial cells that resembled eccrine differentiation under a light microscope. Immunohistochemical studies showed positive staining for muscle-specific actin and vimentin in the myoepithelial cells, but staining was negative for S-100 protein and keratin. The tubular and trabecular epithelial cells stained positively for keratin, EMA and CEA. We believe that this rare tumor had the potential for biphasic differentiation and should be differentiated from other biphasic differentiated or mixed tumors in this site. Immunohistochemical staining was useful for diagnosis and differential diagnosis.

Spiradenocylindroma-More Than a Coincidence?

To the Editor: We thank Dr. Soyer, Dr. Kerl, and Dr. Ott for their comments on our article (1). Based on a large morphologic study of spiradenomas and cylindromas they have found a remarkable overlap between these two types of sweat gland tumors. Of special interest are those tumors that combine unequivocal spiradenomatous and cylindromatous components without transition in the same lesion. They have called this tumor type spiradenocylindroma. We agree with Dr. Soyer, Dr. Kerl, and Dr. Ott that this phenomenon seems to be more than a mere coincidence of two different adnexal tumors. In our series, we studied typical spiradenomas and cylindromas without overlapping features. However, immunohistochemically we have found the same marker expression in both tumor types reflecting myoepithelial, apocrine, and possibly eccrine differentiation. Dr. Soyer, Dr. Kerl, and Dr. Ott's histologic findings and our immunohistochemical results underline the close histogenetic relationship of both neoplasms. These observations suggest that these adnexal tumors develop from cells serving as multipotential precursor cells of adnexal organs. They might have the capability to create a spiradenomatous, cylindromatous, or trichoepitheliomatous pattern (2-5). Our findings let us suppose that these multipotential cells might be sited in abortive adnexal anlagen. Dr. M. Meybehm Prof. Dr. H.-P. Fischer Department of Pathology; University Bonn; Bonn, Germany

Papillary Eccrine Adenoma: An Ultrastructural and Immunohistochemical Study

A 45-year-old African American man presented with a small, solitary, nonulcerated cutaneous lesion of the right thigh of many years duration, which was excised. Light microscopically, the lesion consisted of an oval, well-circumscribed, intradermal proliferation of tubules, which were often dilated and lined by a double layer of tumor cells with conspicuous intraluminal papillations. Ultrastructurally, there was evidence of both intradermal eccrine duct and eccrine secretory coil differentiation. Although the majority of tumor cells resembled cells of the intradermal eccrine duct, occasional myoepithelial tumor cells and rare tumor cells having secretory granules and resembling dark mucous cells were indicative of eccrine secretory coil differentiation. The positive immunoshistochemical staining for S-100 protein, CEA, EMA, and vimentin supported these ultrastructural findings.

臨床的浸潤範囲と組織学的浸潤範囲に大きな差が認められたＭｉｃｒｏｃｙｓｔｉｃ ａｄｎｅｘａｌ ｃａｒｃｉｎｏｍａの１例

A 51-year-old male was referred to our clinic in November, 1996, because of slowly progressive indurated erythematous plaque with smooth, shiny surface and small teleangiectasia for 3 years duration. In microscopic examination, the tumor cells were present in the whole dermis and showed basaloid tumor strands with inmature eccrine differentiation. This biopsy specimen was interpreted as microcystic adnexal carcinoma (MAC), and the tumor was excised with 5mm tumor-free-margin in accord to previous mapping biopsy by 4mm trephine punch. But the margin of resection were involved tumor cells in deeper dermis and subcutis. Additional wide resection was performed with 5mm wider margin and the tumor was completely excised.MAC is invasive in deeper and pathological invasion much differs from clinical apperance.

Immunohistological distribution of heparan sulfate proteoglycan and chondroitin 6-sulfate in carcinomas with eccrine differentiation

Immunohistological distribution of heparan sulfate proteoglycan and chondroitin 6-sulfate in carcinomas with eccrine differentiation

Immunohistological distribution of heparan sulfate proteoglycan and chondroitin 6-sulfate in carcinomas with eccrine differentiation.

Immunohistological distribution of heparan sulfate proteoglycan and chondroitin 6-sulfate in carcinomas with eccrine differentiation.

Benign cutaneous adnexal tumors with combined folliculosebaceous, apocrine, and eccrine differentiation. Clinicopathologic and immunohistochemical study of eight cases.

Benign cutaneous adnexal tumors displaying divergent differentiation are rare, with very few well-documented cases reported in the literature. We describe eight cases of benign adnexal tumors showing a variable combination of eccrine, apocrine, and folliculosebaceous differentiation. Clinically, all tumors presented as solitary, slowly enlarging dermal or subcutaneous nodules located in the head and neck and the extremities. Histologically, they were characterized by well-circumscribed, unencapsulated nodules composed of a lobular proliferation of epithelial cells displaying a spectrum of trichogenic, sebaceous, apocrine, and eccrine differentiation. The histological spectrum included lobules and trabeculae of basaloid cells with glandular and ductal elements, well-formed folliculosebaceous units, primitive follicles, and foci of tricholemmal keratinization. Immunohistochemical evaluation in four cases showed similar cytokeratin, carcinoembryonic antigen, and epithelial membrane antigen staining profiles as those reported for sweat gland adenomas; in addition, focal S-100 protein positivity and GCDFP-15 positivity could also be demonstrated, suggesting eccrine-apocrine differentiation. The tumors were most frequently confused histologically with other adnexal neoplasms, including sebaceoma, sebaceous adenoma, basal cell carcinoma, chondroid syringoma, and trichoepithelioma. The present series highlights the capability.

Spiradenoma Associated with Apocrine Adenoma Component

We present a case of spiradenoma associated with an apocrine adenoma component. The transitions between the spiradenoma and apocrine adenoma components were gradual. It could be observed that the small dark cells seen at the peripheries of the cell nodules of the spiradenoma component were replaced by the peripheral flat myoepithelial cell layer in the apocrine component. Similarly, the large pale cells within the centers of the cell nodules of the spiradenoma component were replaced by the brightly eosinophilic secretory cells with decapitations secretion in the apocrine component of the tumor. The reported case proves that at least some spiradenomas are tumors with distinct apocrine instead of eccrine differentiation.

Familial cylindromatosis (turban tumour syndrome) gene localised to chromosome 16q12-q13: evidence for its role as a tumour suppressor gene.

The human skin is a complex organ composed of the surface epidermis, the subjacent dermis (in which blood vessels, lymphatics and nerves are located) and the skin appendages. The latter include hair follicles, sebaceous glands (which secrete lipids that may serve as a permeability barrier, emollient or antimicrobial agent), apocrine glands (which secrete scents) and eccrine glands (which produce sweat for temperature control). Hereditary cylindromatosis (MIM 123850) is a rare autosomal dominant disease characterised by the development of multiple neoplasms originating from the skin appendages. These neoplasms have been termed cylindromas due to their characteristic microscopic architecture and are believed to exhibit apocrine or eccrine differentiation. We have carried out a genome search using two families with this disease, which has provided strong evidence for linkage of cylindromatosis to loci on chromosome 16q12-q13. Using markers close to the cylindromatosis gene, consistent loss of the wild-type allele was observed in 19 tumours from four individuals in the two families, indicating that the gene is likely to be a tumour suppressor gene.

Apokriner kutaner Mischtumor mit follikul�rer Differenzierung

An 85-year-old man presented with a firm, papillomatous nodule on the upper lip, which had been growing slowly for 30 years. Histopathologic examination revealed an apocrine mixed tumour with follicular differentiation. Mixed tumours of the skin are rare benign neoplasms, which are composed of different tissue components, e.g. epithelial and glandular elements and myxoid or chondroid components. Mixed tumours with apocrine differentiation can be discriminated from mixed tumours with eccrine differentiation. Criteria for apocrine differentiation are: decapitation secretion, elongated gland-like and duct-like structures lined with two rows of epithelial cells and branching of tubular structures. Follicular structures are another clue to the apocrine differentiation of neoplasms. The designation mixed tumour is preferable to chondroid syringoma, because most mixed tumours show apocrine differentiation. The differential diagnosis of apocrine mixed tumour includes fibroadenoma, hidradenoma, mucinous adenocarcinoma, adenoid cystic carcinoma, and rare soft tissue tumours like the myxoid chondrosarcoma.

The pathogenesis of familial multiple cylindromas, trichoepitheliomas, milia, and spiradenomas.

Early stages in the development of hereditary cylindromas associated with trichoepitheliomas, milia, and spiradenomas revealed that (a) trichoepitheliomas were derived from the bulges of hair follicles and probably represented an abnormal histogenesis from which rarely poorly differentiated trichoepitheliomas developed; (b) milia were the result of cystic alteration of the trichoepitheliomatous bulge proliferations; and (c) cylindromas and spiradenomas were different appearances of the same tumor and developed from two separate sources, follicular bulge proliferations and eccrine glands. From the bulge proliferations multiple cylindromatous buds developed that, by increasing in number, formed the classical cylindroma and, by increasing in size rather than in number, gave the spiradenomatous variant. Eccrine glands transformed into cylindromas by cylindromatous growth from the basal cell layer. Connections between the original structures and the tumors were mostly lost. Tumors from both sources revealed the same morphology, which was most indicative of eccrine differentiation including secretory and excretory elements. Apocrine differentiation was a rare and possibly secondary event. The multiplicity in derivation and differentiation suggest an adnexal progenitor cell as the most likely source.

Mixed tumours of the skin: a histopathological, enzyme-histochemical and immunohistochemical study.

Twenty-eight cases of mixed tumour of the skin were studied and subclassified into three types: eccrine (1 case), indeterminate (7), and apocrine type (20). The indeterminate type was defined as mixed tumours having tubuloalveolar patterns with two layers of epithelium, ut without apocrine secretion or pilosebaceous differentiation. Enzyme-histochemical studies were performed on four cases (one indeterminate, three apocrine): in the indeterminate type the tubular epithelial cells showed eccrine differentiation while in the apocrine type tubules were found showing the direction of differentiation to be toward the apocrine gland, but tubules with eccrine differentiation were intermingled in all three. Immunohistochemically, no differences were observed between the indeterminate and the apocrine type: hints of eccrine features were observed in both groups. Thus, the indeterminate type could be an eccrine tumour and the apocrine type showed direction of differentiation toward both eccrine and apocrine glands. It is concluded that mixed tumours of the skin are fundamentally eccrine neoplasmas, and that the apocrine features may represent apocrine metaplasia.

Clear cell eccrine carcinomas of the skin. A clinicopathologic study of nine patients.

Sweat gland carcinomas with clear cell features are extremely rare neoplasms, with few well documented cases reported in the literature. Data on nine patients with malignant eccrine adnexal neoplasms characterized by a prominent clear cell neoplastic component were studied. Immunohistochemical stains with a panel of antibodies against epithelial, stromal, and neural antigens were performed on five tumors and electron microscopic examination of one. The tumors showed a spectrum of histologic features and growth patterns that ranged from well differentiated, low grade malignant neoplasms to poorly differentiated, highly aggressive, recurrent, and metastasizing tumors. All tumors contained a varied proportion of cells with abundant clear cytoplasm, similar to those seen in a group of benign eccrine adnexal neoplasms that have been variously designated as clear cell hidradenoma, nodular hidradenoma, clear cell myoepithelioma, and eccrine acrospiroma. Immunohistochemical stains on five tumors and ultrastructural examination in one were consistent with eccrine differentiation. Clinical follow-up of eight patients showed local recurrence in six, followed by metastases in three, despite local excision, radiation, and chemotherapy. Criteria for differentiating these tumors from their benign counterparts and from other types of malignant adnexal neoplasms and metastatic lesions are presented. The findings indicate that clear cell eccrine carcinomas comprise a heterogeneous group of lesions that may range from locally recurring, low grade well differentiated tumors to highly aggressive, high grade tumors with a definite potential for uncontrollable local recurrence and metastasis. Wide surgical excision is recommended as the primary treatment for such neoplasms.