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[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↗

Syringoma: a review of twenty-nine cases.

The purpose of our study was to identify the clinical characteristics, epidemiologic data and histologic features in 29 cases of syringoma with a duration of lesions prior to the observation between 1 and 25 years. Only one patient complained of moderate itching. In two cases the lesion was solitary, in another the papules formed a lichenified plaque. In six patients only the eyelids were involved and in two patients a symmetrical localization on the forearms was observed. The other 18 patients showed generalized syringoma, 16 with an eruptive onset, 6 of which were familial. One of our cases showed lesions mimicking urticaria pigmentosa and two patients were affected by Down's syndrome. In two cases, histopathology showed association between syringoma and a melanocytic naevus and in one patient with a solitary lesion a clear cell syringoma was observed.

Adolescent↗

Syringomas: new approach to an old technique.

BACKGROUND: Syringomas are common, benign periorbital adnexal tumors that pose a cosmetic dilemma for both patients and physicians alike. Many therapeutic modalities can potentially cause scarring and recurrences are common. The objective was to develop a treatment method that minimizes scarring and subsequent recurrences. METHODS: Each syringoma is treated with short bursts of low voltage electrodesiccation, delivered with an epilating needle that is inserted deeply into the skin at the level of the reticular dermis. RESULTS: A patient treated by intralesional electrodesiccation of her long-standing periorbital syringomas remains lesion-free for over 24 months after therapy. CONCLUSIONS: Intralesional electrodesiccation is a safe, nonscarring and reliable method that can be used to eradicate periorbital syringomas.

Adult↗

Intralesional electrodesiccation of syringomas.

BACKGROUND: Syringomas are common, benign adnexal tumors. In the periorbital area, they pose a cosmetic dilemma for both patients and physicians alike. Many different therapeutic modalities potentially can cause scarring, and recurrences are common. OBJECTIVE: To develop a treatment method that minimizes scarring and subsequent recurrences. METHOD: Each syringoma is treated with short bursts of high frequency low voltage electrodesiccation delivered with a fine needle electrode that is inserted into the center of the syringoma, as deeply as the reticular dermis. RESULTS: Twelve patients treated over a 4-year period showed no permanent adverse effects postoperatively and no recurrences. CONCLUSIONS: Intralesional electrodesiccation is a safe, nonscarring and, reliable method that can be used to eradicate periorbital syringomas.

Adult↗

Periorbital syringoma: a pilot study of the efficacy of low-voltage electrocoagulation.

BACKGROUND: Management of periorbital syringomas is problematic and avoided by many inexperienced physicians. The medical literature presently prefers CO(2) laser resurfacing to many other modalities, but the subject of electrosurgery has not been well explored. OBJECTIVES: To evaluate the clinical efficacy of electrocoagulation at low voltages in treating periorbital syringomas. MATERIALS AND METHODS: Twenty cases were collected during the period of 2002 through 2005. All cases were photographed before treatment with a dual-frequency 4-MHz radiofrequency device (Surgitron, Ellman International, Inc., Hewlett, NY) at a power of 1 to 2 in electrocoagulation mode. Six-week follow-up visits were scheduled to discuss occurrences and expectations, observe treatment effects, and apply further electrosurgery if needed. RESULTS: Clinical improvement increased with each subsequent treatment session. All patients scored either moderate or marked clinical improvement by their final visits, with 60% (11/18) revealing a marked clinical improvement (i.e., >70% clearance). The most common encountered adverse effects consisted of periorbital burning, swelling, redness, and pigmentary changes. CONCLUSION: Low-voltage electrocoagulation is an effective therapy for periorbital syringoma and should be considered when treating this difficult condition. To our knowledge, this is the first study utilizing electrocoagulation for removal of syringomas.

Adolescent↗

Progesterone receptor positivity supports hormonal control of syringomas.

Syringomas may be at least partially under estrogen and/or progesterone influence, as they are more common in women and are known to proliferate at puberty. During pregnancy and the premenstrual period an increase in tumor size has also been described. We examined nine syringomas using immunohistochemical markers for estrogen (ER) and progesterone (PR) receptors. Scattered tumor cells displaying nuclear and cytoplasmic staining for ER were noted in one of the nine cases. Intense nuclear and cytoplasmic staining for PR was noted in most (> 80%) of the neoplastic cells in 8/9 syringoma cases. Current immunohistochemical evidence supports the theory that syringomas are under hormonal control.

Adolescent↗

Eruptive syringomas: an unusual presentation on the buttocks.

Syringomas are benign, eccrine, sweat gland tumors. Eruptive syrin-gomas are rare variants that typically develop on the body's anterior surface. We present the case of a 21-year-old black man with multiple, eruptive, asymptomatic syringomas on his buttocks that had persisted for 3 months. This case is unusual in that the patient's age, race, and sex and the location of the lesions are not considered typical for eruptive syringomas. We also present a review of eruptive syringomas and recent literature.

Adult↗

[Syringomas. Presentation of 7 cases].

OBJECTIVE: To further knowledge of syringoma and its importance within the overall condition of the patient, with a view to making it relevant in the differential diagnosis of other conditions such as xanthomata or milium cysts. DESIGN: A descriptive study of a crossover type. SETTING: Primary care and the dermatology clinic of a health centre in the metropolitan area of Madrid. PATIENTS AND OTHER PARTICIPANTS: The records of patients with syringomas confirmed by histological tests during 1990-1993 were examined. Patients with only a clinical diagnosis were discarded. The different diagnoses on referral by the primary care doctor were also studied. MEASUREMENTS AND MAIN RESULTS: Seven patients (5 women, two men) were confirmed by histological tests as suffering Syringomas. They were between 16 and 52 years old. Four had lesions only on the eyelids; in the other three they were widespread. All of them were asymptomatic, had been over three years without lesions and had no special previous history. None of them wanted to undergo treatment. The referral diagnoses cited xanthomata in five cases and milium cysts in the others. CONCLUSIONS: The primary care doctor must become better informed about syringoma, given its frequent confusion with other conditions (xanthomata and milium cysts). This confusion gives rise to unnecessary additional examinations and discomfort for the patients. These lesions should be verified histologically, as it is an nonaggressive method.

Adolescent↗

Stereographic analysis of syringomas.

To investigate biological characteristics of syringomas, three-dimensional (3-D) architectures of epithelial tumor nests and luminal structures of syringomas, hair follicles, and epidermal basement membrane in skin lesions were reconstructed using a computer stereographic analysis. Each lesion was composed of many spherical- to ovoid-shaped tumor nests, which interconnected with each other by narrow tumor strands, showing features of ginger roots or budding fungi. Small discrete tumor nests were often present in the peripheral areas of the lesions. Tumor nests developed around hair follicles and beneath epidermis, but they were never connected with hair follicles or epidermal basement membrane, although occasionally such structures were slightly bent by a pressure from the developing tumor nests. There was no relationship between tumor nests and normal sweat glands. Many tumor nests formed luminal structures; however, some small tumor nests did not. Most of the luminal structures were disconnectedly present. These findings suggest that in each lesion syringoma may occur in a single focus in the upper dermis without a relationship to the surrounding normal epithelial tissues, develop and partly swell forming lumina, and then the peripheral parts of the tumor may become discrete. The ratio of [lumen volume/tumor nest volume] measured by computer stereometry was 0.275 +/- 0.037 (n = 5); this indicates a constant ability to form luminal structures by syringoma cells.

Adenoma↗

[Chondroid syringoma: a clinical and histological review of eight cases].

INTRODUCTION: Chondroid syringoma is a rare skin tumor that presents as a slow growing, indolent lesion, that is more frequent in male adults and is usually benign. It is treated surgically and its diagnosis is histological. It can be classified as eccrine and apocrine according to its pathological characteristics. MATERIAL AND METHODS: Eight cases diagnosed of chondroid syringoma in our Department in the last eight years were reevaluated clinically and histologically and the epiluminiscence microscopy was used in one of them. RESULTS: Most of the cases occurred as well-defined nodular lesions, whose presumptive clinical diagnosis was adnexal or cystic pathology. All were treated surgically. The epiluminiscence microscopy showed a pattern of homogenous whitish-blue coloring. All the cases had a myxoid matrix and most also had a chondroid one. Five of the eight cases were apocrine, one of them with follicular and sebaceous differentiation. DISCUSSION: The chondroid syringoma is usually a small-sized skin tumor, frequently on the head and neck. The dermatoscopy does not seem to supply any specific trait to this lesion. The microscopic examination of these lesions reveals tubuloalveolar and glandular type structures positioned as islets within a fibroadipose, chondroid, myxoid or hyaline stroma. The final treatment of these tumors is surgical. As a general rule, it is a benign tumor. Some authors have proposed the term of atypical mixed tumor of the skin for those chondroid syringomas with histological traits of malignancy but without evidence of metastasis.

Adenoma, Pleomorphic↗

Localized vulvar syringomas.

Syringomas are benign appendageal tumors with differentiation toward the intraepidermal portion of the eccrine sweat duct. A 35-year-old black woman with lesions confined to the labia majora is presented. Syringomas involving the genitalia are rare, and usually the genital tumors are part of a more generalized distribution. A review of English literature showed only four reported cases of syringomas limited to the vulva. Vulvular syringomas present as multiple, bilateral, skin-colored papules over the labia majora that may or may not be pruritic. The lesions should therefore be considered in the differential diagnosis of multiple papular lesions of the vulva and are probably more common than is generally recognized.

Adenoma↗

Recurrent malignant chondroid syringoma of the foot: a case report and review of the literature.

Malignant chondroid syringoma, or mixed tumor of the skin, salivary gland type, is an uncommon neoplasm believed to originate in sweat glands. This neoplasm occurs mostly in women and is typically seen in the extremities and torso. A case of recurrent malignant chondroid syringoma of the right foot in a man aged 34 years is described with a review of pertinent literature. The surgically excised neoplasm was evaluated by routine histology, immunohistochemistry, and transmission electron microscopy. The malignant chondroid syringoma showed microscopic dermal satellite tumor nodules. Immunohistochemical staining was positive for keratin and S100 and negative for actin and p53. Ki-67 showed <10% positive staining. Ultrastructurally, the neoplasm was composed of epithelial cells with tonofilaments, cell junctions, and electron-dense amorphous keratin-like substance in the intercellular spaces. No evidence of myoepithelial differentiation was noted. Given the tumoral size, acral location, and histologic findings, the neoplasm was classified as a malignant chondroid syringoma. After reviewing the literature, it became apparent that wide surgical excision, adjuvant radiation therapy as well as patient education are critical in facilitating long-term survival.

Adenoma, Pleomorphic↗

Fine needle aspiration cytology of malignant chondroid syringoma: a case report.

BACKGROUND: Chondroid syringoma, a tumor of the eccrine glands, was previously called mixed tumor of skin as it has both mesenchymal and epithelial elements. Malignancy in this tumor is extremely rare. Although there are a few reports describing the cytomorphologic features of chondroid syringoma, the cytologic findings of its malignant counterpart have not been described. CASE: A 40-year-old female presented with a recurrent swelling on the scalp of one year's duration. Fine needle aspiration yielded blood-mixed gelatinous material. May-Grünwald-Giemsastained smears showed epithelial cells arranged in cordlike structures and ill-formed glands against a myxomatous background. The epithelial cells had scanty cytoplasm and markedly pleomorphic nuclei with prominent nucleoli. A few cells in the stroma had a halo around them and a resemblance to cartilage cells. A preoperative diagnosis of malignant chondroid syringoma was made. The tumor was excised, and the cytologic diagnosis was confirmed on histopathology. CONCLUSION: Cytomorphologic features of a rare case of malignant chondroid syringoma are reported for the first time. The presence of malignant epithelial cells against a myxoid background with a few chondroid foci helped in making a correct preoperative diagnosis.

Adenoma, Pleomorphic↗

Giant chondroid syringoma of the axilla.

BACKGROUND: Chondroid syringomas are benign uncommon tumours of controversial histogenesis that most often affect the head and neck region and usually measure > 3 cm in greatest dimension. OBJECTIVE: To describe the clinical features, histology, and differential diagnosis of an unsually large axillary chondroid syringoma. METHODS: A 64-year-old man presented with a painless, subcutaneous tumour measuring 8 3 7 3 6.5 cm on his right axilla, which had been growing slowly for several years. The tumour was completely excised under local anesthesia. No recurrence of the tumour has been observed 12 months after surgery. RESULTS: Histological examination showed cords, nests, and tubuloglandular structures composed of well-differentiated tumour cells embedded in a hyalinized stroma with abundant chondroid matrix, and the diagnosis of chondroid syringoma was established. No features suggesting malignancy were observed. CONCLUSION: As this case shows, chondroid syringoma may reach a large size, and it should be included in the differential diagnosis of slowly growing solid nodules in the skin or subcutis.

Adenoma, Pleomorphic↗

Chondroid syringoma: case report and review of the literature.

An 84-year-old man presented with an enlarging bluish, painless subcutaneous nodule on the glabella. The lesion had been excised 4 years prior and was diagnosed as chondroid syringoma, but had gradually regrown. The recurrent lesion was treated by surgical re-excision. Histopathological examination was again consistent with chondroid syringoma, and showed the following: 1) a chondroid matrix, 2) tubuloalveolar structures lined by a double epithelium, 3) ductal structures lined by a single epithelium, 4) nests of polygonal cells, and 5) the presence of keratinous cysts. Chondroid syringoma is a rare mixed tumor of the skin that was first described by Hirsch and Helwig. Characteristically, it is composed of a proliferation of epithelial cells set in a myxoid and chondroid matrix. Although chondroid syringomas are predominantly benign, malignant forms have been reported.

Adenoma, Pleomorphic↗

Eruptive syringoma.

Eruptive syringoma is a rare variant of syringoma that appears on anterior surfaces of the body, including the neck, chest, and arms. Textbooks state that this eccrine-derived tumor arises at puberty. We describe four cases of eruptive syringoma that began in childhood. We review the literature on this entity and suggest that the disorder typically presents between the ages of 4 and 10 years. Eruptive syringoma is a benign tumor that should be considered among the papular dermatoses of childhood.

Adenoma, Sweat Gland↗