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TM Wong, KF To, T Mok Abstract We report a case of metastatic apocrine carcinoma arising from the peri-anal region. Partial response was achieved after a course of combination chemotherapy with evidence of clinical, biochemical, and radiological improvement. There are few published papers on the treatment of primary or advanced disease, in particular the role of chemotherapy is not well defined. Case Report A 49-year-old Chinese man presented in February 1995 with a subcutaneous nodule of 1 cm in the peri-anal region. Wide excision of 5 x 1.5 cm was performed. Histology revealed poorly differentiated apocrine carcinoma with hyperchromic and pleomorphic nuclei and high mitotic activity (figure 1). The resection margin was clear. The patient received no adjuvant treatment. The patient remained disease-free until June 1997 when he noticed right-sided inguinal and back pain. On examination, he had a 2 cm right inguinal node and multiple left cervical lymph nodes. Core biopsy confirmed metastatic apocrine carcinoma. His liver function was deranged, with alkaline phosphatase of 271 IU/L (normal range, 30-90 IU/L) and alanine aminotransferase of 74 IU/L (normal range, < 58 IU/L). Serum carcinoembryonic antigen (CEA) level was 410 µg/l. Chest X-ray and skeletal survey were normal. Computed axial tomography of the abdomen showed multiple liver metastases and lytic lesions in the T11 and L3 region. Extensive bony metastases were confirmed with magnetic resonance imaging of the pelvis. Figure 1. Section showed infiltrative carcinoma forming a malignant glandular pattern. Apical snouts as indicated by the arrow was typical of apocrine differentiation. (Haematoxylin and eosin stain x 200)
The patient was treated with combination chemotherapy of cisplatin 50 mg/m2, adriamycin 50 mg/m2, and cyclophosphamide 500 mg/m2 every 3 weeks. After 2 cycles of chemotherapy, he had less pain and the right inguinal lymph node resolved, together with a decrease in the size and number of the left cervical lymph nodes. The serum CEA level decreased to 25.7 µg/L. Plain radiograph showed diminished lytic lesions. On serial ultrasound, the metastatic liver nodules had also reduced in size and number. The chemotherapy was stopped after 5 cycles as there was a clinical complication of reactivation of hepatitis B. Lamivudine was commenced and the patient's liver function improved after 2 months' treatment. In summary, we achieved > 50% partial response after 5 courses of cisplatin, adriamycin, and cyclophosphamide. However, in January 1998, we noted rising CEA levels and worsening liver function. One month later, the patient had increased pain in his back and legs, and recurrent lymph nodes at the previous sites. His alkaline phosphatase and alanine aminotransferase were 1006 IU/L and 109 IU/L, respectively. The serum CEA level increased to 1725 µg/L. Plain X-ray revealed extensive osteolytic lesions in the pelvis and spine. Three cycles of taxol and carboplatin were given but the response was poor. He subsequently received palliative radiotherapy to the L1 to L5 region and the right hemi-pelvis, and died in September 1998. Discussion Apocrine carcinoma of the skin is rare. It affects males and females equally in all races. The most common sites are the axilla and scalp, although other sites such as the chest, eyelids, wrist, vulva, lips, pubic skin, finger tips, and cystic teratoma of the ovary have also been reported. The aetiology is uncertain but sunlight may play a role. The biological behaviour of apocrine carcinoma is diverse but in general, moderately- and poorly-differentiated tumours behave more aggressively. Metastases commonly involve the regional lymph nodes (100%),1 and 48% of patients have distant metastases such as bone, lung, skin, brain and kidney.2 The prognostic factors include size, histological type, and lymph node involvement. The 5- and 10-year survival rates for patients without lymph node involvement is 67% and 56%, respectively.3 The 5-year survival rate for metastatic disease is 29%, with a 10-year survival rate of 9%. The primary treatment is wide surgical excision. Lymph node dissection is indicated for recurrent disease or for patients with moderately- or poorly-differentiated carcinoma.3 Table 1. The role of chemotherapy in the treatment of metastatic apocrine carcinoma
Abbreviations: CMF = cyclophosphamide, methotrexate, 5-fluorouracil; LN = lymph nodes. Post-operative irradiation is believed to prevent recurrence or metastasis but some authors have reported that the tumour is radio-resistant.3 The role of chemotherapy is debatable and the responses in the past have generally been poor (table 1).3-7 The reason that we used cisplatin, adriamycin, and cyclophosphamide in treating metastatic apocrine carcinoma is based on our experience of treatment of adenocarcinoma of unknown primary. Cisplatin- and doxorubicin-based combination therapy have been widely studied with 63% objective response and 26% complete response achieved in one study.8 Patients treated with either cyclophosphamide or doxorubicin or both have a better median survival.9 Our centre has reported a 12.9% response rate in treating adenocarcinoma of unknown primary using cisplatin, adriamycin, and cyclophosphamide.10 Conclusion The prognosis of metastatic apocrine carcinoma is grave but our experience of using cisplatin, adriamycin, and cyclophosphamide in treating metastatic disease is encouraging. We recommend this combination therapy since a good clinical, biochemical, and radiological response was observed for our patient. We hope to use newer agents or combination therapy with better outcomes in the future. References 1. Jacobson YG, Rees TD, Grant H, Fitchett VH. Metastasizing sweat gland carcinoma. Arch Surg 1959;78:574-581. |
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