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A 26-year-old woman developed papular lesions symmetrically located on both labia majora during several years duration. Histopathological examination showed typical features of syringomas. In the literature, only 15 patients have been previously described.
Hyaline cell-rich chondroid syringoma (HCRCS) is a rare benign cutaneous neoplasm composed of cells with eosinophilic hyaline cytoplasm and plasmacytoid features, the origin of which remains elusive. To the best of our knowledge, only eight cases of this entity have been reported so far, and none of them was submitted to a large panel of myoepithelial markers. We report on a case of a previously healthy 29-year-old male patient who presented with a slowly enlarging flesh-colored nodule on the palmar aspect of the tenar region of his left hand, measuring 2 cm in maximum diameter. The nodule was "shelled-out" and submitted to light microscopy, immunohistochemistry, and ultrastructural examination. Histopathologic analysis disclosed a lobulated neoplasm composed of hyaline cells with plasmacytoid features showing ovoid nuclei, with occasional invaginations, finely granular chromatin, and discrete nucleoli; the cytoplasm was deeply eosinophilic with occasional dot-shaped paranuclear hyaline inclusions. On immunohistochemical evaluation, hyaline cells were strongly and diffusely positive for S-100 protein, vimentin, pan (CAM 5.2) and high molecular weight (34betaE12) cytokeratins; these cells were focally positive for GFAP, maspin, neuron-specific enolase, and cytokeratin 14. Alpha-smooth muscle actin, epithelial membrane antigen, carcinoembryonic antigen, collagen IV, Gp100 (HMB-45), and p63 were negative in neoplastic hyaline cells. Ultrastructural analysis disclosed cells with ovoid nuclei showing occasional invaginations and nuclear pockets; the cytoplasm was rich in meshworks of non-bundling intermediate filaments and a variable amount of rough endoplasmic reticulum cisternae. Based on our findings and those previously reported, hyaline cells of HCRCS might posses an aberrant myoepithelial differentiation. Most importantly, pathologists need to be aware of the histologic and immunohistochemical features of HCRCS to avoid a misdiagnosis of highly malignant neoplams, such as malignant melanoma and extra-skeletal myxoid chondrosarcoma.
We present a rare case of malignant chondroid syringoma of the hand in a 37-year-old man. Ray amputation with coverage by an island reverse flow forearm flap gave a good final result. There have been no signs of recurrence during a follow up of five years.
This study was initiated because the histogenesis of chondroid syringomas (CSs), especially the role of the myoepithelial cells, is still controversial. Twelve cases of CS were analyzed by immunohistochemical methods with a broad panel of routinely used antibodies. The epithelial elements were classified as tubuloglandular components, stromal cells, and solid nests. The authors' results indicated the following: (1) The inner cell layers of tubuloglandular components have distinct epithelial features with cytokeratin (CK), carcinoembryonic antigen (CEA), and epithelial membrane antigen (EMA) positivity. (2) The outer cell layers had negative results for muscle-specific actin (MSA), desmin, CEA, EMA, and CK (if antibody AE1/3 was used) and had positive results for vimentin, S-100 protein, neuron-specific enolase (NSE), and in three cases for glial fibrillary acidic protein (GFAP). (3) The stromal cells and, to a lesser extent, the solid nests expressed immunophenotypes similar to those of the outer cell layers. These data suggest that the stromal components may derive from the outer cells of the tubuloglandular elements but derive from the outer cells of the tubuloglandular elements but do not confirm their exclusive myoepithelial origin.
This report describes the features of a benign chondroid syringoma removed from a foot of a 34-year-old woman. Histologic examination showed that an acidophilic hyaline cell was the predominant cell type; keratinocytes and cells with clear to foamy cytoplasms were less abundant. The hyaline cells and keratinocytes were within a chondroid and myxoid stroma. Ultrastructural study revealed large numbers of pale epithelial cells whose cytoplasms were loaded with intermediate filaments and dark cells with many tonofilaments. Intermediate transition forms between the pale and dark cells were also identified, as were myoepithelial cells with intermediate filaments, and secretory cells. These cells were also lodged in a chondroid and myxoid matrix. This study showed that acidophilic hyaline cells are of both epithelial and myoepithelial origin and that both may be responsible for laying down the matrix.
A case of malignant chondroid syringoma of skin with widespread metastases is recorded. Several features of mixed tumors are discussed, and the pertinent literature is reviewed.
The origin and significance of hyaline cells (HC) in chondroid syringomas (CS) is unclear. In a review of 20 CS, we found HC in eight cases. These were studied immunohistochemically, using antibodies to cytokeratin, vimentin, carcinoembryonic antigen, epithelial membrane antigen, desmin, muscle-specific actin, S-100 protein, myoglobin, alpha-1-antitrypsin, chromogranin, glial fibrillary acidic protein, and neuron-specific enolase. HC gave a positive reaction to cytokeratin, vimentin, S-100 protein, and neuron-specific enolase, but not to the other antibodies. Thioflavin-T stain for amyloid was negative. Ultrastructurally, HC contained intermediate filaments in hap-hazard arrangement without specific structures or densities, and had sparse intracytoplasmic organelles and rare desmosomal attachments. Intermediate forms between epithelial cells and HCs were also identified. Our results suggest that HCs are relatively common in Cs (40%) and are more frequent in CS with solid nests and myxoid stroma (relative frequency 62.5%). Immunohistochemically, HCs of CS have a specific phenotype profile; however, ultrastructurally, HCs of CS are not distinct and are similar to HCs in other organs. These results do not suggest an exclusive myoepithelial origin of HCs; it appears that HCs could derive from any epithelial cell type of sweat glands, probably via a regressive process.
Malignant chondroid syringoma, also called malignant mixed tumor of the skin, is a rare variant of a malignant tumor derived from sweat gland cells. Histologically the tumor is composed of an epithelial and a mesenchymal component. The epithelial structures show glandular differentiation and carcinomatous features. They are embedded in a mucinous stroma with spindle mesenchymal cells and areas of chondroid differentiation. The epithelial cells express cytokeratins. The luminal epithelial cells show binding to the lectin Ulex europaeus; intraluminal cells are carcinoembryonic antigen positive. The stromal cells are cytokeratin negative and sporadically positive for vimentin. Chondroid areas are S-100 protein and vimentin positive. No labeling for actin is seen.
We describe a 27-year-old Japanese female with a recurrent nodule on the left big toe and local bone invasion. Histopathologically, the tumor consisted of nests of atypical cells with few mitotic cells, which partly formed gland-like structures. Areas of myxoid degeneration, positive for Alcian blue staining and that did not stain after they were digested with hyaluronidase, were prominent in the matrix among tumor cells. Positive staining was noted in tumor cells for cytokeratin (AE1+AE3), S-100 protein, neuron specific enolase (NSE), and glial fibrillary acidic protein (GFAP). These findings, especially positive GFAP staining were characteristic and very helpful for the diagnosis of the rare tumor-malignant chondroid syringoma. Based on the previous reports, 39% of cases were found to have metastatic lesions and 22% died of this malignant tumor. There have been no reports reporting effectiveness of chemotherapy and radiotherapy, and an early wide excision with a broad margin may be the most reliable treatment to date.
Syringomas are benign adnexal tumors often located on the lower eyelid skin. Patients usually request removal for aesthetic reasons. Electrodesiccation and curettage of these lesions provides local control while preserving uninvolved eyelid skin.
A case of chondroid syringoma in the palm, probably the fourth to be reported, is presented. There was a large cystic cavity in the center of the tumor. The peculiar findings of hyaline, myxoid, or chondroid degeneration of the abundant stroma were observed histologically. This tumor should be considered in the differential diagnosis of painless, slow-growing palm tumors.
PURPOSE: To describe the clinical presentation of cutaneous benign mixed tumor of the eyelid and its management options. METHODS: Periocular cases of cutaneous benign mixed tumor were gathered from members of an oculoplastics specialty Internet discussion group. A total of 9 patients are described in this retrospective, interventional case series. The clinical presentation, histopathology, and management of these lesions is reviewed. RESULTS: Patients were typically asymptomatic, presenting with a slowly enlarging, nontender nodule of 2 to 8 years' duration. The lesions ranged from 4 mm to 17 mm in greatest dimension. Four of the lesions were on the eyelid margin, three in the sub-brow area of the upper eyelid, and two in the central lids. All six cases not involving the brow were fixed to the tarsus; one brow lesion was believed to be adherent to the skin. None of the lesions was associated with significant changes of the overlying epidermis, although one lesion showed overlying pigmentation. All patients underwent excisional biopsy for diagnostic or cosmetic reasons. On histopathologic examination, the tumors were biphasic, with an epithelial component exhibiting apocrine or hair follicle differentiation and a myxoid, adipocytic, chondroid, and/or fibrous stroma. The pathologic diagnoses were all consistent with cutaneous benign mixed tumor (chondroid syringoma, pleomorphic adenoma). Follow-up ranged from 2 weeks to 12 months, although several patients failed to keep scheduled follow-up appointments. No clinical recurrences were identified. CONCLUSIONS: Cutaneous benign mixed tumor may occur in the eyelid, and, although uncommon, should be included in the differential diagnosis of firm, nodular eyelid tumors. The histopathologic features are similar to those seen in this tumor type arising in other areas of the body. Preoperative consideration of this diagnostic possibility may allow the surgeon to plan for complete excision, thereby reducing the possibility of recurrence or malignant transformation.
Sweat-gland carcinomas are very rare, comprising only 0.01% of primary tumours of the skin. Malignant chondroid syringoma is the least common variety, of which less than twenty cases have been recorded. We report a case in which this tumour presented as an osteolytic secondary deposit.
Fifteen cases of chondroid syringoma have been studied histologically and by immunohistochemical methods in an attempt to establish their phenotypic profile and to elucidate their histogenesis. The epithelial elements were classified as tubuloglandular, solid nests and stromal cells. The inner cell layers of tubuloglandular components had distinct epithelial features, expressing cytokeratin, carcino-embryonic antigen and epithelial membrane antigen. The outer cell layers expressed vimentin, S-100 protein, neuron-specific enolase and muscle-specific actin and were negative for epithelial markers. The immunophenotypes of stromal cells and solid nests were similar to those of the outer cell layers. These data suggest that the stromal components may derive from the outer cell of tubuloglandular elements and that myo-epithelial cells have an important role in the histogenesis of these lesions and in their mesenchymal matrix production.
Generalized eruptive syringoma is a rare condition characterised by multiple papules over the anterior trunk, sometimes extending on to the flexural aspects of the arms and thighs. A 22 year old Ethiopian woman with this disorder is presented.
The technique for using the carbon dioxide laser in the vaporization mode to treat multiple lesions of syringoma of the face without adverse scarring, textural changes, or recurrence is described.
An easy, rapid, and inexpensive technique for excision of syringomas from eyelids is outlined, using a fine needle as a skin hook and a #11 scalpel blade and leaving superficial wounds for secondary closure. Possible advantages and disadvantages are discussed. We recommend this procedure as an effective alternative that provides good cosmetic results.
BACKGROUND: Malignant sweat gland neoplasms are exceedingly rare tumors. Malignant chondroid syringoma (MCS) is one of the rarest subtypes, and as such, still poorly understood. It lacks distinctive clinical features, often delaying initial diagnosis and therapeutic management. OBJECTIVE: A current case and the available literature are reviewed to determine the overall clinical course of the MCS and the potential role of adjuvant therapy. METHODS: A case of MCS was studied by light microscope, immunohistochemistry, and electron microscopy. The clinical data of this case and of other reported cases are summarized and compared. RESULTS: This tumor recurred locally after initial local excision. Subsequent re-excision and radiation therapy rendered the patient without evidence of disease. This case study and the literature review of the 20 reported cases indicate that MCS is highly recurrent with tendency toward metastasis. CONCLUSION: MCS appears to behave in an aggressive manner. An initial treatment modality is aggressive surgery. Adjuvant radiation therapy with or without chemotherapy should be tried in future cases.