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Clear-cell syringoma. A study by conventional and electron microscopy.

The epithelial component of a syringoma is usually composed of cuboidal cells that have a moderate amount of eosinophilic cytoplasm in tissue sections stained with hematoxylin and eosin. Some syringomas have variable numbers of larger epithelial cells that have pale or clear cystoplasm. Such lesions have been called clear-cell syringomas. Examination of 18 cases by conventional microscopy revealed that their general organization is the same as ordinary syringomas but that the epithelial component constitutes a greater proportion of neoplastic volume. Histochemical studies confirmed that the clear cells contain abundant glycogen. The cells near lumina also contained a mucous substance that had a staining profile consistent with sialomucin. Examination by electron microscopy of two lesions composed entirely of clear cells revealed numerous multivesicular bodies, some in large aggregates, in periluminal cells. All cells contained numerous glycogen granules. Lesions in which there were incomplete changes in clear cells tended to have cells predominantly around the lumina. These observations suggest that the majority of clear cells result from accumulation of glycogen. The periluminal cells may be clear owing to the large number of multivesicular bodies and storage of sialomucin in them.

Adenoma↗

Detection of HPV-20, HPV-23, and HPV-DL332 in a solitary eyelid syringoma.

PURPOSE: To report evidence of many human papillomavirus types occurring in a solitary syringoma clinically appearing as a papilloma. METHODS: A 57-year-old man presented with a 10-year history of an upper eyelid tumor. Histopathology, human papillomavirus-nested polymerase chain reaction, human papillomavirus-DNA cloning into vector pCR2.1, sequencing, and computer-assisted evaluation were performed. RESULTS: Histopathology demonstrated a solitary benign syringoma. HPV-20 and HPV-23 were present in one clone each, and HPV-5-related HPV-DL332 was present in 9 clones. CONCLUSION: Many human papillomavirus types may be detected in an ocular syringoma.

DNA Primers↗

Eruptive syringoma: 27 new cases and review of the literature.

BACKGROUND: Eruptive syringomas are uncommon eccrine sweat gland tumours. Only 64 cases have been reported in the literature. OBJECTIVE: Clinical findings of 27 patients with eruptive syringomas were reviewed over a 47-year period. RESULTS: The tumours appeared as multiple yellow-brown-coloured papules localized on the neck, anterior trunk, axillae, shoulders, abdomen or pubic area. This disorder occurs more frequently among women, and is frequent in the prepubertal as well as in the postpubertal age. The diagnosis was not clinically suspected in most cases. CONCLUSIONS: Eruptive syringomas have to be considered in differential diagnosis of papular dermatosis at any age.

Adolescent↗

[Microcystic adnexal carcinoma (malignant syringoma) of the nose: case report and review of the literature].

A case of Malignant Syringoma (syn. = Microcystic Adnexal Carcinoma, Sclerosing Swat Duct Carcinoma) of the nose in a 44 year old female patient is reported. The tumor had been misdiagnosed as a Rhinophyma some 20 years before and had thus been treated under cosmetic aspects. Besides the appearance, the patient did not suffer any complaints and was referred to the ENT-department of the Klinikum Hannover for cosmetic reasons. Histopathological examination after removal revealed a malignant syringoma, which, due to its extensive size and subepithelial growth pattern, made a complete ablation of the entire nose and the adjacent soft tissue of the face necessary. Pathohistologically cellular atypia, invasive growth pattern, perineural and perivascular infiltration was characteristic. Quantitative DNA anaylsis revealed a tumor with a diploid stem line and only few aneuploid cells. Malignant syringoma is a rare differential diagnosis of face skin tumors. The present case is discussed based on a review of the literature.

Adolescent↗

Vulvar syringoma causing pruritus and carcinophobia: treatment by argon laser.

Syringomas are benign lesions most commonly appearing around the eyelids and in malar areas, but they have also been described on other body sites. Depending on the site they may cause either aesthetic disturbance (e.g. face, neck, arms) or pruritus (e.g. vulva). This is the first report of argon laser treatment of syringoma in a patient with multiple vulvar syringomas in association with pruritus vulvae and carcinophobia.

Adult↗

Unusual cases of syringoma of the forehead.

Syringoma involving the forehead is rare and can be confused with other diseases that are common on the forehead. Therefore, we describe here three patients with syringoma who developed numerous skin colored papules on the forehead. To our knowledge, syringoma of the forehead was reported previously only once by Tagami et al.

Adult↗

A case of unusual syringoma: unilateral linear distribution and plaque formation.

We describe a 25-year-old woman with syringoma, clinically manifested by unilateral linear distribution and a plaque formation. Syringoma papules are located on the extensor aspect of the left upper arm, the left antecubital fossa, and the left side of the chest. The papules distributed in linear fashion on the left upper arm, and partially formed plaques on the left upper arm and the left antecubital fossa. Syringoma must be included in the differential diagnosis of unilateral linear papular or plaque skin lesions.

Adult↗

Localized vulvar syringomas--an unusual cause of pruritus vulvae.

Genital syringomas are rare and usually asymptomatic. We report 2 young women who presented with pruritus vulvae. Examination revealed multiple bilateral skin-coloured papules over the labia majora. Histology showed syringoma. Syringomas should be considered in the differential diagnosis of pruritic papular lesions of the vulva.

Adult↗

Vulvar syringoma, report of a case and review of the literature.

Syringomas are common intraepidermal sweat gland tumors most often found in women around the time of adolescence. Frequent sites of involvement include the lower eyelids and malar areas, however vulvar involvement is relatively rare. These lesions often present as small, multiple, skin-colored-to-yellowish papules and are often associated with increased vulvar discomfort and itching. We present a case of a 29-year old female who presented to her gynecologist complaining of vulvar itching and burning. A small condylomatous-type wart observed on her vulva was biopsied and found to be a syringoma. Because of their clinical presentation and associated symptoms, vulvar syringomas should be considered in the differential diagnosis of any multicentric papular lesion of the vulva, vulvar pain syndrome, and pruritus vulvae.

Adult↗

Hereditary syringomas: a case report.

Syringoma is a benign neoplasm of eccrine origin. Clinically, it is an eruption of small translucent-to-yellowish papules. These lesions are firm, smooth, and approximately 1-3 mm in diameter. They are most commonly found around the eyes and on the upper cheeks of middle-aged women. Lesions sometimes develop on the abdomen, axillae, penis, vulva, and scalp. Involvement of the scalp may be indistinguishable from nonscarring alopecia. Familial cases have been reported, and there is an increased incidence of syringoma in adults with Down syndrome. Eruptive syringoma, a separate entity, presents mostly in adolescents as clusters of numerous papules on the upper half of the body.

Humans↗

Eruptive syringomas.

Syringomas are benign adnexal tumors derived from the intraepidermal portion of eccrine sweat ducts. Usually, they present as soft, flesh-colored to slightly yellow dermal papules on the lower eyelids of healthy individuals. We report an 18-year-old man with rare presentation of eruptive syringomas involving his trunk and extremities, with linear arrangement on the arms and forearms. A biopsy obtained from the lesions of the dorsum of the hands showed eccrine syringoma with a lymphocytic inflammatory infiltration around superficial blood vessels and eccrine ducts. We used the 585-nm and 595-nm pulsed dye laser for treatment of inflammatory lesions of forearm and trunk with no success.

Adolescent↗

Acral syringomas presenting as a photosensitive papular eruption.

A 43-year-old woman presented with a recurrent and remitting eruption of reddish-brown papules in the sun-exposed areas of the arms and forearms that had been present for 2 years. Results of a histopathologic examination revealed syringoma. To the best of our knowledge, this is the first case report of syringomas confined to sun-exposed areas with a recurrent and remitting course over time. We also review the literature on acral syringomas.

Adult↗

Unilateral multiple plaque-like syringomas.

We describe a unique presentation of multiple unilateral syringomas characterized by indurated plaques. Histologically, in addition to typical eccrine duct proliferation, desmoplasia exceeding by far that usually observed in syringoma, accounted for the hard consistency of the plaques. This unusual presentation should be added to the numerous clinical variants of syringoma.

Adult↗

Familial syringoma. Case history and application of monoclonal anti-eccrine gland antibodies.

We studied a family with dominantly inherited eruptive syringoma. The father and the older daughter had chest and neck as well as eyelid lesions. The chest lesions seemed to develop continuously by new formation of eccrine germlike budding from the epidermis. Monoclonal antikeratin antibody EKH4, which predominantly labels the basal layers of the epidermis, stained positively in the cordlike epithelial structure and peripheral cells of the walls of cystic lesions. Staining with EKH6, which recognizes normal eccrine secretory and ductal structures--particularly luminal borders--was positive along the luminal borders of the cystic lesions; staining with EKH5, which labels eccrine secretory portion, was entirely negative in the lesions. These findings further supported the theory that syringoma of the eyelids and eruptive syringoma, which mainly involves the anterior neck and upper chest, are essentially the same tumor and that these appendage tumors are of eccrine ductal differentiation.

Adenoma↗

Malignant chondroid syringoma. Report of a case with widespread metastasis and review of pertinent literature.

The authors report the autopsy case of a 73-year-old man with widespread, metastatic, malignant chondroid syringoma, with a long clinical history. Three years after resection of the primary tumor on the back, the tumor recurred at the same site, metastasized to the cervical lymph nodes 7 years later, and finally, after 13 years exhibited widespread metastases. Histologically, the tumor showed cords and nests of cuboidal or polygonal tumor cells with little cellular pleomorphism and few mitoses surrounded by a mucoid matrix. Histochemically, the matrix contained hyaluronic acid and sulfated acid mucopolysaccharides, such as chondroitin sulfate A and/or C. To the knowledge of the authors there have been nine reported cases of malignant chondroid syringoma in the English literature, four of which had distant metastasis. A review of the literature reveals that malignant chondroid syringoma is more common in women and occurs most often in trunk and extremities, which is in contrast to its benign counterpart, its histologic appearances vary greatly, and it may even have benign appearances.

Back↗

[Chondroid syringoma. Immunohistologic indications of myoepithelial differentiation].

Chondroid syringoma belongs to the group of so-called mixed tumors, like pleomorphic adenomas of the lacrimal and salivary glands. The histogenesis of this tumour is still disputed, in particular with respect to its stromal component. The distribution of cytokeratins (CKs), CEA, EMA, vimentin, S-100 protein, desmin and actin [alpha-smooth muscle actin (alpha-SMA)] was investigated by immunohistological examination of paraffin sections from a chondroid syringoma of the apocrine type. The neoplastic formations have been classified into tubuloalveolar structures, solid nests/aggregations and stromal cells of varying morphology. The inner-most cells of tubuloalveolar structures were characterized by marked expression of CKs (KL1 and MNF116), CEA and EMA, while in the outer ones there was moderate expression of vimentin, S-100 was expressed to a lesser extent and KL1, weakly but there was marked and consistent expression of MNF116. Whereas the solid nests expressed vimentin, S-100 protein, MNF116 markedly and KL1 weakly, the stromal cells were consistently positive for vimentin, S-100 protein and, focally, CKs and alpha-SMA. Anti-alpha-SMA specifically detects myoepithelial cells. In addition, the partly overlapping immunoreactivity of the intermediate filaments, membrane proteins and proteins in the different structures may indicate a common clonal origin of all neoplastic cells in chondroid syringoma.

Actins↗

Malignant syringoma of the eyelid.

Two cases of malignant syringoma of the eyelid are reported. The first patient, a 20-year-old woman, presented with a one year history of a slowly growing nodule in the left lower eyelid. The lesion recurred five months after excision, slowly grew over the ensuing two and one-half years, and was reexcised. The second case was an 18-year-old man who was first examined by an ophthalmologist because of a slowly enlarging, painless induration of his right lower eyelid for two years. Histologic examination of the excised lesions from both patients disclosed proliferated ducts lined by two layers of epithelium infiltrating the dermis and subcutaneous tissue; there was skeletal muscle and perineural invasion, but only minimal cellular atypia. Malignant syringomas are rare forms of sweat gland carcinoma, which most often occur on the upper lip and to our knowledge have not been reported on the eyelid. These tumors probably arise from eccrine ducts, are more common in women, and have a peak incidence in the fifth decade. The histologic feature that best indicates prognosis is the degree of cellular atypia. Patients with cytologically well-differentiated tumors do well following complete local excision. Features that help to distinguish malignant syringoma from its benign counterpart are the larger size, solitary nature, and subcutaneous, muscular, and perineural invasion.

Adenoma↗

A, B, and H substances in syringomas. An immunohistochemical analysis of 15 cases.

This study examines the distribution of A, B, and H substances in 15 syringomas and 11 control axillary skins. Monoclonal antibodies (MCABs) against A, B, and H substances with the avidin-biotin-peroxidase complex (ABC) method were used. Additionally, the H antigen was localized with Ulex europaeus Agglutinin-I (UEA-I) by use of the peroxidase-antiperoxidase method. The MCABs provided specific and reproducible staining of all 15 syringomas as well as the secretory coli and duct of normal eccrine glands. Contrariwise, apocrine and sebaceous glands were uniformly nonreactive. The staining by the UEA-I technique showed a similar pattern to that of the anti-H MCAB but with increased background (nonspecific) staining. These results, using state-of-the-art immunohistochemistry, confirm the eccrine origin of syringoma. Blood group substances may be useful in distinguishing other tumors of eccrine origin from those of apocrine origin.

Adenoma↗