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Immunohistochemical analysis of keratin expression in clear cell syringoma. A comparative study with conventional syringoma.

Immunophenotypes, especially expression of cytokeratins, in 9 cases of clear cell syringoma were examined using antibodies against epithelial membrane antigen (EMA), and 17 kinds of monoclonal anti-keratin antibodies to investigate its histogenesis. In addition, 7 cases of conventional syringoma were selected for parallel assessment. Conventional syringoma expressed CK1 and CK10, which exists in the acrosyringium and the transitional portion between the acrosyringium and the dermal duct. Based on immunostaining with RCK102 and 35 beta H11, syringoma was thought to express CK5. Because expression of CK5 was observed in the basal cells of sweat duct ridge (lower acrosyringium) and the outer cells of the dermal duct, but not in the acrosyringium located at upper epidermis, we speculated that syringoma differentiated toward the transitional portion between the acrosyringium and the dermal duct. A comparative study of keratin expression between conventional and clear cell syringoma showed that there was no difference in the immunoreactivities. Based on the above observations, we confirmed that clear cell syringoma is a metabolic variant of conventional syringoma, and differentiates into the transitional portion between the acrosyringium and the dermal duct.

Adolescent↗

Treatment of multiple facial syringomas with the carbon dioxide (CO2) laser.

BACKGROUND: Syringomas are benign tumors of eccrine origin most commonly found in the periorbital area. Previously reported treatments for syringomas include excision, electrodesiccation and curettage, dermabrasion, and carbon dioxide (CO2) laser resurfacing. The ideal treatment of syringomas should be destruction of the tumor with minimal scarring and no recurrence. OBJECTIVE: The objective is to present a treatment method for multiple syringomas without scarring or recurrence. METHOD: Ten patients with multiple periorbital syringomas were treated with a high energy, scanned carbon dioxide laser. Settings of 5 watts, 0.2 second scan time, and 3mm spot size were used. Two passes were performed, but some lesions required four passes. In some cases the entire lower periorbital area was treated. Results were evaluated clinically by both physicians and patients over a span of 1 to 24 months. RESULTS: Elimination of the syringomas was successful in all patients. Each patient remains free of recurrence 1 to 24 months after therapy. Prolonged erythema was the most common side effect, but no scarring was seen. Four out of the ten patients required repeat spot treatments. CONCLUSION: The CO2 laser is a dependable, safe, and nonscarring method for the treatment of periorbital syringomas.

Adult↗

Vulvar syringoma: a clinicopathologic and immunohistologic study of 18 patients and results of treatment.

BACKGROUND: Syringoma of the vulva has been rarely reported. No effective treatment modality has been documented. OBJECTIVE: The purpose of this study was to describe the clinicopathologic features of vulvar syringoma, to investigate the hormonal influence on its growth, and to establish an effective treatment modality. METHODS: A total of 18 cases of vulvar syringoma were selected from the surgical pathologic file at Chang Gung Memorial Hospital. In all, 15 formalin-fixed, paraffin-embedded specimens were examined by immunohistochemical staining for estrogen receptor and progesterone receptor. RESULTS: The age of first presentation at our vulvar clinic ranged from 21 to 60 years with a median age of 29.5 years. Of patients, 13 (72%) had vulvar pruritus and 7 noticed aggravation during summer or during menstruation. The most common clinical appearance was multiple flesh-colored or brownish papules on bilateral sides of the vulva (9 of 18). One third of our series was found to have coexisting eyelid syringoma and 4 of them also had a family history of periorbital syringoma. Immunohistochemical stainings for estrogen receptor and progesterone receptor were all negative on the 15 cases studied. Of our patients, 7 with intense pruritus were treated with carbon dioxide laser vaporization. Their lesions resolved and pruritus subsided. CONCLUSION: Vulvar syringoma is not very rare and should be considered in the differential diagnosis of vulvar pruritus. In our study, estrogen receptor and progesterone receptor were not detected in vulvar syringoma. Carbon dioxide laser was an effective therapeutic modality in treating patients with intractable symptoms.

Adult↗

Palpebral syringomas and Down's syndrome.

BACKGROUND: Palpebral syringomas have been reported to be more frequent in patients with Down's syndrome than in the normal population. OBJECTIVE: The aim of the present study was to evaluate, in a population of institutionalized patients with Down's syndrome, the prevalence of syringomas and their possible cytogenetic relationships. METHODS: Sixty-one institutionalized patients with Down's syndrome were examined in order to assess the presence of palpebral syringomas. Sixty mentally retarded non-Down's syndrome individuals were used to control group. RESULTS: Fourteen patients, 13 females and 1 male, were found to be affected. The prevalence of syringomas in both sexes was 23%; 42% of all females, and 55% when only adult females were considered, had syringomas. Thirteen of the 14 affected patients had a karyotype of Down's syndrome with free trisomy 21, 1 had a mosaicism 47,XX, +21/46,XX. Histologic examination confirmed the diagnosis in all 4 biopsied cases. A clear-cell pattern was observed only in 1 patient while, sporadically, few tubules showed a central syringial-type cuticula. CONCLUSION: The higher prevalence found in females, as compared to males, can be partially explained by their older age (mean 23.8 vs. 13.9 years). Palpebral syringomas are a common cutaneous pathology in adult females with Down's syndrome.

Adolescent↗

Temporary tattooing followed by Q-switched alexandrite laser for treatment of syringomas.

BACKGROUND: Syringomas usually develop in women as multiple skin-colored papules primarily seen on the periocular regions and cheeks. They can cause cosmetic problems and lead to poor self-esteem. Though several treatment modalities have been established, such as excision, electro/cryosurgery, chemical peeling, and CO2 laser surgery, none of them are satisfactory due to their limitations and side effects, for example, pain, prolonged healing time, postoperative erythema/pigmentary changes, and scarring. OBJECTIVE: The objective of this study was to develop a new treatment method for syringoma and to minimize the side effects through selective destruction of the tumor. METHODS: Six patients with multiple periorbital syringomas were enrolled in this study. The surface epithelium of the syringomas was vaporized by CO2 laser, and black ink was introduced in order to allow penetration to the dermis using iontophoresis. Subsequently the artificial tattoos were removed by Q-switched alexandrite laser. The results were evaluated clinically by both physicians and patients at 1, 2, 4, and 8 weeks after treatment. RESULTS: The majority of syringoma in the six patients disappeared by the first follow-up 1 week after treatment. There were no cases of prolonged erythema persisting beyond 2 weeks. Additional treatment was repeated in the same manner in order to remove the remaining syringomas in one patient. There were no recurrences during the 8-week follow-up period. CONCLUSION: Our new treatment was safer, less painful, nonscarring, and there was a quicker recovery period and less of a burden to repeat treatment when necessary.

Adult↗

[Syringoma and trisomy 21].

Two cases of syringoma associated with trisomy 21 are reported. The first case concerned a 29-year old trisomic woman with multiple syringomas of the eyelids, and the second case concerned a 44-year old trisomic woman with disseminated but not eruptive syringomas. The syringoma-trisomy 21 associated is not fortuitous, being 31 times more frequent than in the general population. The "usual" location of syringomas on the eyelids has given rise to many reports, but disseminated syringomas have been reported in only four cases.

Down Syndrome↗

Clear-cell syringoma. Immunohistochemistry and electron microscopy study.

Clear-cell syringoma is a histologic variant of syringoma that is otherwise clinically indistinguishable from ordinary syringoma. This variant is formed by cells that have pale or clear cytoplasm as a result of glycogen accumulation. There is a high association of clear-cell syringoma and diabetes mellitus. A case of clear-cell syringoma associated with diabetes mellitus is described. Electron microscopic examination revealed that periluminal cells showed intra- and extracytoplasmic multivesicular bodies that may be characteristic of the clear-cell variant. Immunohistochemistry for carcinoembryonic antigen (CEA) showed ordinary syringomas as in the presence of CEA within and surrounding duct-like spaces.

Adenoma↗

["Hyaline-cell chondroid syringoma." Morphological, immunohistochemical and ultrastructural study of a case and review of the literature].

Chondroid syringoma represents the cutaneous counterpart of mixed tumor ("pleomorphic adenoma") of salivary glands, therefore it is also termed mixed tumor of the skin. The presence of hyaline cells in mixed tumors of salivary gland is a very well known event. Tumors mainly or exclusively composed of hyaline cells are termed myoepitheliomas of hyaline type, accordingly to their alleged myoepithelial origin. Scanty components of hyaline cells in chondroid syringomas of the skin have also been observed since more than a decade. However chondroid syringomas mainly or exclusively composed of hyaline cells have been reported only very recently. If the hyaline cells of chondroid syringomas are myoepithelial or epithelial in origin is still a matter of debate, we prefer to retain the descriptive label "hyaline cell-rich chondroid syringoma" as originally conceived by JA Ferrero and AG Nascimento the patronimic authors of the entity. A case of hyaline cell-rich chondroid syringoma is reported on in a white male patient aged 64, which occurred on the external ear of 1-year duration. This case was studied immunohistochemically and ultrastructurally. Both immunoprofile which included reactivity for vimentin, low molecular weight cytokeratins, S-100 protein, GFAP (focally), alfa-smooth actin and muscle-specific actin, and ultrastructural features including evidence of intermediate filaments (non-bundling filaments, tonofilaments), desmosomes, and thin filaments of actin type sustain a myoepithelial differentiation for hyaline cells of this tumor and site. The authors also remark the importance of being aware of this new entity during the diagnostic practice for avoiding misinterpretation. A list of condition to be taken into account in differential diagnosis is also given.

Adenoma, Pleomorphic↗

Vulvar syringoma aggravated by pregnancy.

Syringoma is a benign tumors of eccrine sweat gland. They appear as multiple, tiny, firm, skin-colored papules. Vulvar involvement of syringoma is rare. Only 24 cases with vulvar syringoma have been previously reported in the literature. The majority of patients with vulvar syringomas are asymptomatic. A case of syringoma of the vulva exacerbated during pregnancy is presented. The case appears remarkable for the experienced aggravated pruritic symptoms of the patient during her pregnancy.

Adult↗

Vulvar syringoma exacerbated during pregnancy.

Although syringoma of the eyelids and the cheeks are well known and defined, vulvar syringoma is a rare clinical variant of this benign eccrine tumor. A case of syringoma of the vulva exacerbated during pregnancies with regression in the periods in between is presented. Histopathological examination showed typical features of syringomas. Only 20 patients with vulvar syringoma have been previously reported in the literature.

Female↗

'Eruptive syringoma': a misnomer for a reactive eccrine gland ductal proliferation?

BACKGROUND: Syringomas have traditionally been categorized as benign neoplasms of the eccrine gland ductal epithelium. However, the variety of clinical presentations reported in the literature and some cases recently observed by the authors cast doubt upon the neoplastic nature of eruptive syringomas. Our goal is to challenge the traditional notion that eruptive syringomas are neoplastic lesions. RESULTS: We observed two patients who presented with an eczematous process, which resolved leaving residual lesions. Biopsies of the late lesions showed features of eccrine syringoma. Yet a biopsy obtained from an incipient lesion in one of the cases showed a lymphocytic inflammatory reaction of the superficial portion of the eccrine duct resulting in tortuous hyperplastic changes. CONCLUSION: Based on our observations, some of the so-called 'eruptive syringoma' may represent a hyperplastic response of the eccrine duct to an inflammatory reaction rather than a true adnexal neoplasm. We proposed the term 'syringomatous dermatitis' for such cases.

Adult↗

A new treatment for syringoma. Combination of carbon dioxide laser and trichloroacetic acid.

BACKGROUND: Although syringoma represents a benign tumor of skin appendage, multiple and diffuse facial lesions can cause cosmetic problems for the affected individuals. Scarring, recurrence and postinflammatory hyperpigmentation can be serious troubles, especially for oriental people receiving various therapeutic modalities. OBJECTIVE: The purpose of this study was to evaluate the histopathologic and clinical efficacy of a new therapeutic approach for syringoma, consisting of combination of carbon dioxide (CO2) laser and 50% trichloroacetic acid (TCA). METHODS: Eighty skin biopsy specimens (45 patients) were evaluated to determine the depth of syringoma lesion. Among these, 28 specimens were obtained after a single test-pulse of CO2 laser (focused, superpulsed mode, power setting of 0.7-2 watts, beam diameter of 0.1 mm, pulse duration of 0.1 seconds), and 3 specimens after one pass of CO2 laser and 50% TCA. The depths of laser-induced vaporization and TCA-induced tissue necrosis were measured. Twenty patients were treated with the combination therapy of CO2 laser (two passes of laser) and 50% TCA. Clinical effectiveness and complications of combination therapy were evaluated by direct observation and photographs. RESULTS: Tumor depth: Analysis of eight specimens revealed tumor depth of 0.70 +/- 0.20 mm (mean +/- SD), ranging from 0.4-1.2 mm. There were no correlations between tumor depth and age of onset or duration of the tumor. Laser penetration depth: CO2 laser irradiation, 0.7-2 watts, induced vaporization of 0.29 +/- 0.12 mm in depth with carbonized rim of 0.1 +/- 0.03 mm width (n = 28 from 15 patients). With laser power of 1 watt, vaporization ranged from 0.17-0.45 mm in depth. Laser and TCA induced necrosis: TCA application resulted in extra tissue necrosis of 0.22-0.25 mm in depth beyond the level achieved by laser vaporization. CLINICAL FINDINGS: Among the 20 patients treated with the combination therapy, therapeutic effect for 11 patients was excellent, 6 patients showed good clinical response, and 3 patients were fair. There were no serious complications resulting from this procedure such as infection, scarring or textural change. CONCLUSION: The application of 50% TCA after CO2 laser irradiation was effective for removing deep-seated syringoma cells and for reducing the side effects, especially scarring. To the best of our knowledge, this is the first study introducing the combination of CO2 laser and TCA for removal of syringoma.

Adolescent↗

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.

Adenoma, Pleomorphic↗

Chondroid syringoma: a diagnosis more frequent than expected.

BACKGROUND: Chondroid syringoma or mixed tumor of the skin is a rare subcutaneous tumor that may be confused with various skin lesions. OBJECTIVE: To elucidate the incidence of condroid syringomas among skin lesions that were excised under local anesthesia. METHODS: The histopathologic diagnosis of 16,200 skin lesions that had been operated between 1986 and 2002 were retrospectively evaluated. The cases with condroid syringoma were histopathologically re-examined, and confirmed cases were further analyzed for preoperative diagnosis, age, gender, and lesion location. RESULTS: Sixteen patients were found to have histopathologic diagnosis of chondroid syringoma constituting 0.098% of the excised skin lesions in this series. All of these 16 cases were misdiagnosed preoperatively. The typical presentation was a solitary skin lesion located in the head and neck region in a middle-aged male patient. CONCLUSION: In the evaluation of a middle-aged male patient with a small subcutaneous nodule in the head and neck region, chondroid syringoma should be also taken into consideration for differential diagnosis. For such a lesion, excisional biopsy without destroying aesthetic and functional structures is the preferred diagnostic approach.

Adenoma, Pleomorphic↗

Chondroid syringoma associated with hidrocystoma-like changes. Possible differentiation into eccrine gland. A histologic, immunohistochemical and electron microscopic study.

A case of chondroid syringoma associated with hidrocystoma-like changes was investigated by histology, immunohistochemistry and electron microscopy. Chondroid syringoma was histologically compatible with apocrine mixed tumor, and hidrocystoma-like changes did not fulfill diagnostic criteria of either eccrine hidrocystoma or apocrine hidrocystoma. However, epithelial cellular elements composing both chondroid syringoma and hidrocystoma-like changes suggested, immunohistochemically and electron microscopically, differentiation into eccrine gland. The lesions of both had an apparent transition of ductal structures of chondroid syringoma into hidrocystoma-like changes. Therefore, chondroid syringoma and hidrocystoma-like changes in this case may be organized as a peculiar type of cutaneous appendage tumor differentiating toward eccrine gland.

Adenoma, Sweat Gland↗

Syringoma of the vulva: incidence, diagnosis, and cause of pruritus.

Syringoma of the vulva is a rare disorder. The patient presented here is the seventh case in the American literature; she is the only patient with this condition in a series of 1132 women with vulvar disease. Differential diagnosis includes epidermal cyst, cherry hemangioma, angiokeratoma, comedo, soft fibroma, Fox-Fordyce disease, steatocystoma multiplex, and lymphangioma circumstriptum. Although syringoma is usually an incidental finding, the diagnosis should be considered in patients with pruritus vulvae. In such cases, examination of the skin around the eyelids and malar areas where syringoma occurs more commonly may suggest a diagnosis of vulvar syringoma that can be confirmed by histologic examination. Inspection of the vulva in cases with extragenital involvement may lead to a more frequent diagnosis of vulvar syringoma.

Adenoma↗

[Familial syringoma: a rare clinical variant].

Syringomas usually occur either sporadically in a periorbital localized or truncal eruptive form. We report on two families with very uncommon hereditary syringomas. Multiple syringomas developed in the periorbital area of affected patients in the first family while the face, neck, trunk, and extremities were involved in the second family. The lesions first appeared during puberty and their number and distribution varied among the family members. Autosomal dominant inheritance equally affecting both sexes appears most likely. The incidence of familial syringomas may be widely underestimated. For treatment, carbon dioxide laser yielded good results while an excellent cosmetic outcome was achieved on the face with a special surgical technique using a springaction microscissors.

Adult↗

The treatment of eruptive syringomas in an African American patient with a combination of trichloroacetic acid and CO2 laser destruction.

BACKGROUND: Eruptive syringomas are uncommon benign adnexal neoplasms. They are numerous and disseminated and often have a predilection for the neck, face, chest, and axillary fossae. Because they are persistent, usually numerous, and often on exposed sites, the lesions may be disfiguring and often pose significant cosmetic concerns for patients. Many treatment modalities such as dermabrasion, electrodesiccation with curettage, and scissors excision have been tried with some success, but more recently lasers have provided good to excellent results. OBJECTIVE: To describe an approach to the treatment of eruptive syringomas in an African American patient with a combination of trichloroacetic acid (TCA) and CO2 laser resurfacing, providing acceptable cosmetic results without significant side effects. METHODS: We describe an African American patient with eruptive syringomas of the face treated with a combination of TCA and CO2 laser resurfacing with good results. RESULTS: While the syringomas were not completely ablated, the combination of TCA and CO2 laser resurfacing provided acceptable cosmetic results without significant side effects. CONCLUSION: The TCA pretreatment probably removed some of the bulk of the surface of the lesions, thereby reducing the number of laser passes required to flatten the remainder of the lesions and thus lessening the potential for thermal damage at the treated sites and of surrounding normal skin.

Carbon Dioxide↗