Dermatology Online Journal

Malignant chondroid syringoma: Report of a case with lymph node metastasis 12 years after local excision
Akira Watarai MD, Yasuyuki Amoh MD, Ryoichi Aki MD, Hiroshi Takasu MD, Kensei Katsuoka
Dermatology Online Journal 17 (9): 5

Kitasato University School Of Medicine, Sagamihara, Kanagawa, Japan

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Abstract

A 46-year-old man noticed a nodule on his sole. The nodule was removed and the specimen showed a lobular proliferation of tumor cells with glandular differentiation embedded in mucinous stroma. A diagnosis of chondroid syringoma was made. Twelve years later, he noted a swelling in the right inguinal region. The mass was surgically removed. The histopathological findings of the lymph node showed the more atypical tumor cells in the mucoid stroma. Upon reexamination, the primary tumor contained malignant chondroid syringoma (MCS) cells; the tumor cells metastasized to lymph node. MCS is rare with 43 reported cases in the literature. The site of the primary tumor was the lower extremity in 35 percent, the head in 28 percent, and the upper extremity in 23 percent. The percentage of malignant cases with local recurrence, nodal metastasis, and distant metastasis was 49 percent, 42 percent, and 40 percent, respectively. In these cases, the average time period until disease recurrence was 23 months, 50 months, and 66 months for local recurrence, nodal metastasis, and distant metastasis, respectively. Of these, 23 percent of the cases succombed. As MCS may progress very slowly and disease recurrence including metastasis occurs in a relatively high percentage of cases, long-term follow-up of MCS cases is required.

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Introduction

Malignant chondroid syringoma (MCS) is an extremely rare tumor arising from the sweat glands. Although MCS may progress very slowly, there is a high percentage of disease recurrence including metastasis. Here, we report a case of MCS that developed on the sole of a patient. Metastasis to the right inguinal lymph node became apparent 12 years after local excision of the primary nodule.

Case report

Figure 1	Figure 2a
Figure 1. A solitary, dome-shaped, skin-colored, and firm nodule measuring 30 mm x 30 mm was observed on the sole of the patient’s right foot. Figure 2. Pathological examination of the primary nodule on the sole showed a dermal tumor (2a: H&E, x1) with proliferation of anaplastic tumor cells embedded in a mucoid stroma (2b: H&E, x100) and the glands with few mitotic cells (2c: H&E, x200).

Figure 2b	Figure 2c

A 46-year-old man noticed an asymptomatic, enlarging nodule on the sole of his right foot for 2 years prior to presenting for a medical examination in 1996.. A biopsy had been performed biopsy at another hospital before coming to our hospital, but the tumor remained undiagnosed. On physical examination, a solitary, dome-shaped, skin-colored, and firm nodule, measuring 30 mm x 30 mm, was noted on the sole of his right foot (Figure 1). The nodule was surgically removed along with a 3 mm margin and the defect was replaced with split skin grafting. The surgical specimen showed a lobular proliferation of tumor cells with glandular differentiation associated with a few mitotic cells, embedded in a mucinous stroma (Figures 2a, 2b, and 2c). Immunohistochemistry showed that the majority of tumor cells were positive for PAS diastase, toluidine blue, cytokeratins (CAM5.2, CK7), S-100 protein, and GCDFP-15. From the above findings, although there appeared to be some atypical tumor cells, a diagnosis of chondroid syringoma was made. Surgical margins were negative. Ultrasound of the regional lymph nodes and radiography of the chest and abdomen showed no evidence of metastasis.

Figure 3a	Figure 3b
Figure 3. Pathological examination of the resected inguinal lymph node. (3a: H&E, x12.5) showed the more atypical tumor cells in the mucoid stroma compared to the primary nodule of the sole. (3b: H&E, x100)

In 2008, 12 years after the initial presentation, the patient noticed a swelling in the right inguinal region. Ultrasound of the area revealed a mass measuring 30 mm x 30 mm in the right inguinal region. The mass was surgically removed. The histopathological findings of the resected lymph node showed the more atypical tumor cells in the mucoid stroma, compared to the primary nodule of the sole (Figures 3a and 3b). From the behavior of the tumor, we reconsidered that the primary tumor contained the malignant chondroid syringoma (MCS) cells, and the tumor cells had metastasized to lymph node. MRI revealed no recurrence on the sole. CT scan of the head, thorax, abdomen, and pelvis were negative. Gallium scintigraphy revealed no abnormalities. The patient received radiation treatment in the right inguinal region and chemotherapy with tegafur, gimeracil, and oteracil potassium for one year. On follow-up 18 months after excision of the right inguinal lymph node, the patient showed no signs of local recurrence or distant metastasis.

Discussion

Chondroid syringoma was first described by Hirsch & Helwig in 1961 [1]. Chondroid syringoma is a rare, benign tumor of the skin that arises from the sweat glands. Rarely, a chondroid syringoma of many years duration may undergo sudden malignant change with subsequent widespread metastasis. The aggressiveness of MCS can vary. For example, most cases had local recurrence, but some cases showed regional lymph node or osseous metastasis. When this tumor metastasizes, it displays features of an adenocarcinoma on histopathology and often loses the tendency to form chondroid stroma. Only 43 MCS cases have been previously reported (Table 1) [1-37]. In the 43 patients, the site of the primary tumor was the lower extremity in 15 (35%), the head in 12 (28%), the upper extremity in 10 (23%), and the trunk in 6 (14%). MCS tends to follow an unpredictable clinical course. Approximately 49 percent of MCS cases have local recurrence. Nodal and distant metastasis was observed in approximately 42 percent and 40 percent of cases, respectively. These percentages suggest that disease recurrence is relatively high with MCS. Surgery was performed in all 43 MCS cases, of which 9 were given radiotherapy, 2 were treated with chemotherapy, and 5 were treated with a combination of radiotherapy and chemotherapy. In the 43 patients, 10 (23%) were dead, 20 (47%) were alive, and 13 (30%) were unknown. In those who later succumbed, the period of survival was from 6 months to 7 years (mean: 4.9 years). Barnett et al. recommended adjuvant radiotherapy for all chondroid syringomas showing signs of aggressiveness or cytologic atypia, regardless of whether it is an initial presentation or a recurrence, because of the seemingly unpredictable nature of this disease and the difficulty faced in removing it completely by excision [4].

Radiotherapy and chemotherapy have no proven value in treatment and adequate surgical excision with wide disease-free margins is the best way to achieve disease control. Chemotherapy and radiotherapy is administered to patients with advanced disease.

In the 43 cases of MCS, the average time period until disease recurrence was 23 months, 50 months, and 66 months for local recurrence, nodal metastisis, and distant metastasis, respectively. In our case, metastasis was discovered 12 years after removal of the primary tumor. The differential diagnosis of this tumor is an apocrine carcinoma and other kinds of myxoid tumors, such as extraskeletal myxoid chondrosarcoma. In our case, the tumor occurred from a sole that does not contain apocrine gland. The tumor had some chondroid stroma and it was present in the dermis and did not demonstrate any connection between the underlying muscle and bone; positive staining for cytokeratins, S-100 protein and GCDFP-15 was observed. These histopathological features, combined with the aggressive behavior of the tumor, led to the diagnosis of MCS. Although there was no evidence of other nodal or distant metastasis, we considered the possibility that neoplastic cells persisted. Therefore, the patient was given radiotherapy in the right inguinal region and chemotherapy with tegafur, gimeracil, and oteracil potassium for one year. In two reported cases, lymph node and lung metastasis was noted 11 years and 17 years after the excision of MCS, respectively [5, 6]. As MCS may progress very slowly, and disease recurrence including metastasis occurs in a relatively high percentage of cases, long-term follow-up of MCS cases is required.

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