Nicholas A. Stephens

 A 51-year-old male with Blaschkoid eccrine spiradenoma originally manifested by cutaneous nodules on the left side of his body that did not cross the midline, which remained unchanged in size or shape until approximately two years ago when he began to experience rapid growth of these lesions, prompting his presentation to our facility.

. Gross appearance of skin lesions. Note that the lesions follow a Blaschkoid pattern; they do not cross the midline.

. Initial CT scan of the lung upon presentation to our facility. Note the bibasilar nodules present.

. CT scan of the lung obtained upon patient decompensation. Note the large right sided pleural effusion as well as the enlarging basilar nodules.

. Intraoperative photograph demonstrating pleural carcinomatosis.

Initial CT scan of the chest, abdomen and pelvis revealed multiple soft tissue nodules, enlarged necrotic lymph nodes in the left axilla and supraclavicular region, and multiple subcentimeter pulmonary nodules (Figure 2a) in the bilateral lung bases suggestive of metastatic disease. Biopsies of the skin lesions were consistent with the diagnosis of eccrine spiradenoma with malignant features. Fine needle aspiration of an enlarged left axillary lymph node was consistent with carcinoma. Immunohistochemical staining of tumor cells was positive for cytokeratin (CK) 7 and MOC31 (antibody against cell surface glycoprotein) and negative for CK20, cluster of differentiation (CD) 56, gross cystic disease fluid protein and progesterone receptor. Rare cells were found to be weakly positive for estrogen receptor, so the patient was initially started on tamoxifen. This treatment did not show any benefit; he experienced a progression of the skin lesions and pulmonary disease on this regimen, requiring admission to the intensive care unit. A repeat CT scan demonstrated a right sided pleural effusion along with progression of metastatic disease (Figure 2b). A thoracentesis and subsequent tube thoracostomy were performed for symptomatic management. The patient was taken to the operating room for video-assisted diagnostic thoracoscopy. The patient had gross pleural carcinomatosis (Figure 3).

 Pleural nodule biopsy confirmed the diagnosis of metastatic eccrine spiradenoma. Talc pleurodesis was performed for symptomatic relief. Postoperatively, the patient has undergone a variety of chemotherapeutic regimens (five cycles of carboplatin and paclitaxel [Taxol]) (stopped secondary to disease progression), nine doses of gemcitabine and cisplatin (stopped secondary to concern for cerebellar neurotoxicity), and most recently capecitabine (Xeloda) (with some symptomatic improvement). The treatments have not been very successful with the most recent CT scan demonstrating continued progression of pleural and lung metastatic disease.

Malignant eccrine spiradenoma is a tumor of the sweat glands with approximately 100 cases having been reported in the English language literature. Metastatic disease to the lung is exceptionally rare. There are approximately 15 other reports of metastatic eccrine spiradenoma to the lung. Additionally, there is one reported case in the Japanese literature. 1-5 As in our patient, the transition time from the initial manifestation of symptoms to malignant disease can be long (up to 70 years has been reported) and typically presents with rapid growth of lesions. The disease is thought to spread through the lymphatics prior to distant spread. There are no standard treatment regimens given the incidence of this disease and median reported survival after the development of distant metastatic disease is approximately 16 months.


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Articles

Case History: 51-year-old male, Blaschkoid eccrine spiradenoma originally manifested by cutaneous nodules, lesions experienced rapid growth two years ago.

Nicholas A. Stephens

Articles

Metastatic Eccrine Spiradenoma