Wilson R W, Moran C A
Department of Pulmonary and Mediastinal Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
Am J Surg Pathol. 1997 Oct;21(10):1196-202. doi: 10.1097/00000478-199710000-00010.
Primary malignant melanoma of the lung (PMML) is an uncommon neoplasm that may be confused with more conventional types of lung cancer. Although the previously proposed criteria for diagnosis, including the presence of an in situ component, are often difficult to satisfy, this lesion is characterized by a poor prognosis, ultimately leading to patient death. We report eight cases of PMML that presented as solitary, central endobronchial neoplasms, resulting in a picture that closely resembled carcinoid tumor or poorly differentiated non-small-cell carcinoma of the lung. The mean age at diagnosis was 51 years (range 45-71). The patients included one woman and seven men. The histologic growth pattern varied from organoid to fascicular and included epithelioid to spindled cells with hyperchromatic to vesicular nuclei, prominent eosinophilic nucleoli, and abundant eosinophilic to clear cytoplasm with occasional intranuclear cytoplasmic inclusions. A bronchial in situ component was present in four cases. Initial interpretations included carcinoid tumor, non-small-cell carcinoma, and malignant melanoma. Melanin was present in all neoplasms on hematoxylin and eosin staining, although very focally in one case, and was Fontana-Masson positive in all cases. Immunohistochemically, diffuse strong positivity for S-100, HMB-45, and vimentin was present in all seven tumors tested. All seven tumors were negative for cytokeratin, CAM 5.2, and chromogranin. Ultrastructural examination of the eighth case showed dysmorphic premelanosomes but no neurosecretory granules. None of the patients had disseminated disease at presentation, and all patients underwent surgical resection (seven lobectomies and one excision). In this series, primary malignant melanoma of the lung was characterized by an aggressive postoperative course, with five patients dying of metastatic disease from 4 to 32 months after resection (median 14 months). Two patients are alive with metastatic disease at 4 and 30 months after surgery, and the eighth patient is alive with no evidence of disease 108 months after surgery at last follow-up. Metastatic melanoma was identified in various sites, including the lungs, adrenal glands, liver, mesentery, brain, and bone. The cases herein presented indicate that PMML should be included in the differential diagnosis of primary bronchial tumors.
原发性肺恶性黑色素瘤（PMML）是一种罕见的肿瘤，可能会与更常见的肺癌类型相混淆。尽管先前提出的诊断标准，包括原位成分的存在，往往难以满足，但这种病变的特点是预后较差，最终导致患者死亡。我们报告了8例PMML，表现为孤立的中央支气管内肿瘤，其表现与类癌肿瘤或低分化肺非小细胞癌非常相似。诊断时的平均年龄为51岁（范围45 - 71岁）。患者包括1名女性和7名男性。组织学生长模式从类器官型到束状型不等，包括上皮样到梭形细胞，核染色质深染到泡状，嗜酸性核仁突出，胞质丰富嗜酸性到清亮，偶尔有核内胞质包涵体。4例存在支气管原位成分。最初的诊断包括类癌肿瘤、非小细胞癌和恶性黑色素瘤。苏木精和伊红染色显示所有肿瘤中均有黑色素存在，尽管1例中黑色素分布非常局限，所有病例Fontana - Masson染色均为阳性。免疫组化方面，所有检测的7个肿瘤中S - 100、HMB - 45和波形蛋白均呈弥漫性强阳性。所有7个肿瘤细胞角蛋白、CAM 5.2和嗜铬粒蛋白均为阴性。第8例的超微结构检查显示有形态异常的前黑素体，但无神经分泌颗粒。所有患者就诊时均无播散性疾病，所有患者均接受了手术切除（7例肺叶切除术和1例切除术）。在本系列中，原发性肺恶性黑色素瘤的特点是术后病程进展迅速，5例患者在切除术后4至32个月死于转移性疾病（中位时间14个月）。2例患者术后4个月和30个月有转移性疾病存活，第8例患者在最后一次随访时术后108个月无疾病证据存活。转移性黑色素瘤在多个部位被发现，包括肺、肾上腺、肝、肠系膜、脑和骨。本文报道的病例表明，PMML应纳入原发性支气管肿瘤的鉴别诊断。
