Umebayashi Yoshihiro
Department of Dermatology, Hitachi General Hospital, Ibaraki, Japan.
J Dermatol. 2004 Jan;31(1):63-5. doi: 10.1111/j.1346-8138.2004.tb00508.x.
A rare case of phalangeal metastasis of extramammary Paget's disease in a 68-year-old man is described. The patient developed an erythematous, slightly elevated area in the pubic region. A biopsy specimen demonstrated numerous, large, rounded cells with ample pale-staining cytoplasm proliferating in the epidermis. With a diagnosis of extramammary Paget's disease, he underwent wide local excision and inguinal node dissection. Eleven months postoperatively, the patient developed a tender, red, swollen right ring finger. Bone X-ray showed that the distal phalanx of the ring finger had completely dissolved. Histopathological examination demonstrated proliferation of tumor cells in the adipose tissue. They had poorer and darker cytoplasm than the Paget's cells in the epidermis of the pubic region. Immunohistochemically, these cells showed the same staining pattern as did the Paget's cells at the primary site. Accordingly, the patient was diagnosed with distal phalangeal metastasis of extramammary Paget's disease. Two weeks after the appearance of the distal phalangeal metastasis, the patient died of cancerous pleurisy. It has been reported that patients with phalangeal metastasis have a very poor prognosis.
本文描述了一例68岁男性乳腺外佩吉特病指骨转移的罕见病例。患者耻骨区域出现一个红斑、略隆起的区域。活检标本显示表皮中有大量大的圆形细胞增殖，其胞质丰富、淡染。诊断为乳腺外佩吉特病后，患者接受了广泛局部切除和腹股沟淋巴结清扫术。术后11个月，患者右手环指出现压痛、红肿。骨X线显示环指远节指骨完全溶解。组织病理学检查显示脂肪组织中有肿瘤细胞增殖。它们的细胞质比耻骨区域表皮中的佩吉特细胞更差且更暗。免疫组化显示，这些细胞的染色模式与原发部位的佩吉特细胞相同。因此，患者被诊断为乳腺外佩吉特病远节指骨转移。远节指骨转移出现两周后，患者死于癌性胸膜炎。据报道，指骨转移患者的预后非常差。
