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Two cases of benign chondroid syringoma of the hand are described. Both patients did not have this tumor in the differential diagnosis at the time of operation. The importance of this tumor lies in its recognition. Eighty-two percent of the malignant chondroid syringomas occur in the extremities, and the differences in benign versus malignant presentation are discussed.
We describe a patient with a benign chondroid syringoma of the little finger of the right hand. She was a 56-year-old pianist who had had the swelling for 25 years without it causing any symptoms. The tumour was excised with an excellent result. Although chondroid syringoma is rare, it should be included in the differential diagnosis of the soft tumours of the hand.
Syringomas are benign tumors of eccrine origin that occur primarily in the lower eyelid region of females. Since they do not involute and are typically refractory to conventional forms of therapy, they may grow to become considerable, chronic cosmetic and functional deficits. The current study reports on a patient with familial syringomas, previously refractory to two different standard therapies, who was successfully treated with the superpulsed CO 2 laser. A simple, accurate, and reproducible method of dosimetry of laser energy was used to treat these lesions with the Sharplan 700 CO 2 laser connected to the operating microscope. Each lesion was irradiated separately with single pulses at a calculated energy density of 1 J/cm 2. Superficial scabbing was observed 1 week postoperatively, with no edema or pain. Mild erythema persisted for 1 month, with complete healing thereafter. No recurrences were noted after a 2 year follow-up. There was no evidence of scarring, ectropion, or changes in pigmentation. The disease and methods of treatment are reviewed.
We report a case of hyaline cell-rich chondroid syringoma arising in the skin on the left side of the neck. The tumor was composed exclusively of hyaline cells arranged in a predominantly thick trabecular pattern. Ductal structures of various sizes in the tumor were composed of eosinophilic cuboidal cells showing transition to hyaline cells. Immunohistochemically, the hyaline cells were positive for cytokeratin, S-100, and vimentin, although the best marker for myoepithelial differentiation, alpha-smooth muscle actin, was negative. As is the case with plasmacytoid monomorphic adenomas of the salivary gland and some reported cases of hyaline cell-rich chondroid syringoma, hyaline cells in the present tumor similarly lacked any evidence of myoepithelial differentiation. These immunohistochemical findings reveal that the hyaline cells in the present tumor were epithelial.
We report three cases of chondroid syringoma composed almost entirely of hyaline cells and occurring in the soft tissues. These lesions were confused with a variety of malignant neoplasms, including malignant melanoma, myxoid chondrosarcoma, and alveolar soft part sarcoma. The tumors occurred in two men and one woman aged 37, 75, and 71 years, respectively, and involved the heel, great toe, and face. All cases arose in the subcutaneous tissue or fascial layer and ranged in size from 0.8 to 2.0 cm. The neoplasms were composed of plasmacytoid hyaline cells with variable amounts of myxoid stroma. Two of the chondroid syringomas contained rare duct structures. One contained cartilage lobules but no ducts. All were well circumscribed. Immunohistochemistry showed positive staining for keratin, S-100, and vimentin in all cases. Muscle markers, including desmin, smooth-muscle actin, and muscle-specific actin, were negative. Follow-up has showed no evidence of recurrence or metastasis.
Chondroid syringoma is a rare subcutaneous tumor that mainly affects the face and the neck. The authors present an occurrence in the orbit, which they believe to be the first reported at that site. Computed tomography and magnetic resonance imaging suggested that the tumor had invaded the globe, but at surgery the mass was well circumscribed and did not invade or adhere to other structures. The tumor was resected easily under general anesthesia. Epithelial cells of characteristic appearance within a chondroid matrix were demonstrated histologically. No recurrence has been evident in more than 1 year of follow-up. Most chondroid syringomas are benign, but because several cases have exhibited malignant behavior, periodic observation following complete resection is required.
Syringomas are benign epithelial tumors of the sweat glands. The diffuse nature of these raised tumors makes surgical treatment difficult. Three patients with eyelid syringomas were successfully treated with CO2 laser vaporization. CO2 laser therapy appears to be a safe and effective mode of treatment for these benign superficial eyelid tumors.
Chondroid syringoma of the vulva is an extremely rare tumor. Of the previously reported cases, only one was clinically malignant. The authors describe a case of chondroid syringoma of the right labium majus from which arose a papillary adenocarcinoma. A right inguinal lymph node was replaced with metastatic adenocarcinoma.
Syringomas localized in the genital areas are unusual. There are only three cases reported describing syringomas on the penis. Owing to this atypical localization it is necessary to consider a differential diagnosis involving other papular lesions more frequent found on the penile skin.
Four cases of chondroid syringoma containing large numbers of hyaline or plasmacytoid cells are described. Three cases occurred in the hand and one in the foot. Hyaline cells are commonly seen in mixed tumours and myoepitheliomas of salivary glands and rarely in chondroid syringomas. The hyaline-cell change in three of the cases initially caused diagnostic difficulties and the possibility of sarcoma was raised in two cases. In addition to the characteristic hyaline cells, the presence of tubulo-glandulo-ductal structures, benign squamous epithelium and myxochondroid stroma aided diagnosis. Immunohistochemically, the hyaline cells exhibited positivity for vimentin, cytokeratin, S-100 protein, carcino-embryonic antigen, focal glial fibrillary acidic protein (3 cases), neuron-specific enolase (3 cases) and focal alpha-smooth muscle actin (2 cases). Occasional cells were Ber EP4 positive (2 cases). In some cells, a striking peripheral ring-like positivity for cytokeratin and S-100 protein was noted. Ultrastructurally, desmosomes, varying numbers of tonofibrils and non-bundling intermediate filaments were seen. Scanty fine filaments with vague focal densities were detected in some cells. Our studies suggest that the hyaline cells represent modified epithelial as well as myoepithelial cells. One of our cases also exhibited collagenous spherulosis.
The clinicopathology of 13 cases of chondroid syringoma were examined. The ages of the patients ranged from 26 to 86 years, with an average of 48 years. There were eight males and five females. Ten tumors out of the thirteen appeared on the face. Only one patient out of ten was suspected of recurrence in follow-up information. Histologically, all tumors consisted of epithelial cells, chondroid or myxoid matrix, and other strumal elements. The tumors were histologically classified into two types; twelve tumors had tubular and cystic lumina lined by two layers of epithelial cells, and only one case had small lumina lined by only a single layer. By an immunohistochemical study with a PAP method, positive stainings of keratin were observed in all cases, and S-100 protein in all but one. Ultrastructurally, the tumor cells showed features of an epithelial cell. Some ultrastructural differences were noted between two types of chondroid syringoma. Type I tumor cells had many tonofilaments in cytoplasm, but cytoplasmic filaments in type II were of the intermediate-type.
We examined 4 cases of sclerosing epithelial hamartoma for the presence of carcinoembryonic antigen (CEA) by the unlabelled antibody peroxidase-antiperoxidase method but could not detect CEA in these lesions. Because syringomas contain CEA, the detection of this antigen is a useful marker for differentiating sclerosing epithelial hamartomas from syringomas.
We have reviewed a series of 585 scalp biopsies taken for histologic evaluation of hair loss and have looked for syringoma-like eccrine sweat duct proliferation. This feature was found in 6 specimens with scarring alopecia and one each with alopecia areata and female-pattern alopecia. Syringoma-like sweat duct proliferation appears to play no role in the etiology of hair loss.
A 65-year-old man presented with a 3.5 cm scrotal mass. Ultrasound showed a solid mass of indeterminate aetiology within the skin. Excisional biopsy revealed a benign chondroid syringoma. This is the first report of chondroid syringoma involving the scrotum.
Chondroid syringoma, also known as mixed tumour of the skin, is a relatively rare, usually benign tumour. A few malignant cases, especially in the lower extremities, have been published, but most of them behave in a benign fashion. A case of a middle-aged woman with surgically proved chondroid syringoma in the pretibial region is presented in this report with MRI and histological findings. Despite rapid growth over a short period of time and a location reportedly associated with malignancy, the histological features were benign.
A case of eruptive syringoma with the clinical features of urticaria pigmentosa is described. A histological specimen revealed a syringoma with an increased number of mast cells in the connective tissue.
The third documented case of chondroid syringoma occurring in a toe, involving the largest tumor of the three, along with a review of the literature, has been presented. The possibility of a mixed tumor should be kept in mind when considering the differential diagnosis of a solid nodule in the skin, particularly if it is of long duration. These tumors usually will present as simple, asymptomatic, subcutaneous nodules that are shelled out easily during surgical excision. However, there is a tendency toward local recurrence that usually is attributed to incomplete surgical removal, which easily may occur because of the lobulation of the tumor. Complete excision of the primary tumor always is recommended, as diagnosis can be made only by histopathologic examination. Six reported cases revealed that the recurrence of the lesion following primary excision resulted in malignant degeneration. In nine reported cases where metastasis had occurred, the initial site of metastasis in five patients was the regional lymph nodes, and, in four patients, there was metastasis in the lung. The period of time between the removal of the primary lesion and the malignant local recurrence or metastasis has been reported to be from 1 to 4 years. Recognition of chondroid syringoma is clinically difficult because of its rarity. Its diagnosis requires histologic examination.
The gross and histologic characteristics of chondroid syringoma were discussed. A case history of the fourth published occurrence of this rare tumor of the lower extremity was presented. Because of the rare occurrence of this tumor in the lower extremity, chondroid syringoma can easily be misdiagnosed. However, it should be included in the differential diagnosis of any slow-growing solid nodule in the skin. In the case presented, the hallux was saved by the persistence of the podiatrist and pathologist in obtaining second and third opinions on the histologic presentation of this rare tumor.